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Show 2 more Monitoring for adverse effects of medications: take into careful consideration the numerous, clinically significant side effects of medications that may be used in the context of COVID-19, as well as drug-drug interactions between medications, both of which may affect COVID-19 symptomatology (including effects on respiratory, cardiac, immune and mental and neurological function). Take into consideration both pharmacokinetic and pharmacodynamic effects. A Monitoring for thromboembolism: monitor patients with COVID-19 for signs or symptoms suggestive of thromboembolism, such as stroke, DVT, PE or acute coronary syndrome. Immediately proceed with appropriate diagnostic and management pathways, if these are clinically suspected. A https://web.pathway.md/diseases/rec2UFiNfrEGXdLl0 28/38 6/29/23, 3:24 AM COVID-19 infection Pathway 12. Quality improvement COVID-19 care pathways: Establish COVID-19 care pathways for patients with suspected or confirmed COVID-19 at local, regional and national levels. A Ensure that community health workers continue to follow usual protocols for recognition and treatment of other common illnesses and danger signs while activating the COVID-19 care pathway (including for referral as needed) for suspect cases. Refer to WHO/IFRC/UNICEF guidance on community-based healthcare, including outreach and campaigns, in the context of the COVID-19 pandemic. A Care bundles: consider using existing care bundles (defined as 3 evidence-informed practices delivered together and consistently to improve care), chosen locally by the hospital or ICU and adapted as necessary for local circumstances, in critically ill patients with COVID-19 with or without invasive mechanical ventilation. C Equipment and care availability: Ensure that all areas, where severe patients may be cared for, are equipped with pulse oximeters, functioning oxygen systems and disposable, single-use, oxygen-delivering interfaces (nasal cannula, Venturi mask and mask with reservoir bag). A Ensure palliative care interventions are accessible at each institution providing care for patients with COVID-19. A Care optimization during pandemic: ensure that hospitals and health systems at local, regional, national and global level have plans, are prepared and ready to surge clinical care capacity (staff, structure, supplies and systems) in order to be able to provide appropriate care of all COVID-19 patients and maintain essential health services. A Show 4 more International classification of diseases coding: use emergency International classification of diseases codes for mortality, as outlined in the International guidance for certification and coding of COVID-19 as cause of death. A Clinical research: As per WHO 2023 guidelines, collect standardized clinical data on all hospitalized patients to improve understanding of the natural history of the disease and contribute data to the WHO Global COVID-19 Clinical Data Platform (see website for details). A As per NIH 2021 guidelines, provide information about ongoing clinical trials of investigational therapies to eligible outpatients with COVID-19 so they can make informed decisions about participating in clinical trials. B Studies 2023 ReCOVer https://web.pathway.md/diseases/rec2UFiNfrEGXdLl0 29/38 6/29/23, 3:24 AM COVID-19 infection Pathway In patients being severely fatigued 3-12 months following COVID-19, CBT was superior to care as usual with respect to reduction in fatigue severity. Tanja A Kuut et al. Clin Infect Dis. 2023 May 8. 2023 BRACE In healthcare workers, BCG vaccine was not superior to placebo with respect to the rate of symptomatic COVID-19 by 6 months. Laure F Pittet et al. N Engl J Med. 2023 Apr 27. Show 60 more References 1. Adarsh Bhimraj, Rebecca L. Morgan, Amy Hirsch Shumaker et al. IDSA Guidelines on the Treatment and Management of Patients with COVID 19. IDSA. 2023 May 15. Open 2. Lisa K. Moore, Tobias Tritschler, Shari Brosnahan et al. Prevention, Diagnosis, and Treatment of VTE in Patients With Coronavirus Disease 2019. Chest. 2020 Sep; 158 3 1143 1163. Open 3. No authors listed. Therapeutics and COVID 19 living guideline. Geneva: World Health Organization; 2023. Open 4. John B. Lynch, Perica Davitkov, Deverick J. Anderson et al. IDSA Guidelines on Infection Prevention in Patients with Suspected or Known COVID 19. IDSA. 2021 Apr 30;Part 2:v1.0.1. Open 5. No authors listed. Living guidance for clinical management of COVID 19. WHO living guidance. 2023 Jan 13. Open 6. Amir Qaseem, Jennifer Yost, Matthew C Miller et al. Outpatient Treatment of Confirmed COVID 19 Living, Rapid Practice Points From the American College of Physicians Version 1 . 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Randomized Trial of Metformin, Ivermectin, and Fluvoxamine for Covid-19. N Engl J Med. 2022 Aug 18;387 7 599 610. Open 105. Erin A Bohula, David D Berg, Mathew S Lopes et al. Anticoagulation and Antiplatelet Therapy for Prevention of Venous and Arterial Thrombotic Events in Critically Ill Patients with COVID 19 COVID PACT. Circulation. 2022 Nov;146 18 1344 1356. Open 106. Lamontagne F et al. Corticosteroids for COVID 19. WHO Living Guidance. Sept 2020. Open 107. Theodoros Karampitsakos, Ourania Papaioannou, Panagiota Tsiri et al. Tocilizumab versus baricitinib in hospitalized patients with severe COVID 19 an open label, randomized controlled trial. Clin Microbiol Infect. 2022 Oct 20;S1198 743X 22 00529 8. Open 108. Shruti Gupta, Jake J Lee, Amber Perrin et al. Efficacy and Safety of Saline Nasal Irrigation Plus Theophylline for Treatment of COVID 19 Related Olfactory Dysfunction: The SCENT2 Phase 2 Randomized Clinical Trial. JAMA Otolaryngol Head Neck Surg. 2022 Sep 1;148 9 830 837. Open 109. Susanna Naggie, David R Boulware, Christopher J Lindsell et al. Effect of Ivermectin vs Placebo on Time to Sustained Recovery in Outpatients With Mild to Moderate COVID 19 A Randomized Clinical Trial. JAMA. 2022 Oct 25;328 16 1595 1603. Open https://web.pathway.md/diseases/rec2UFiNfrEGXdLl0 37/38 6/29/23, 3:24 AM COVID-19 infection Pathway 110. Meg J Jardine, Sradha S Kotwal, Abhinav Bassi et al. Angiotensin receptor blockers for the treatment of covid-19 pragmatic, adaptive, multicentre, phase 3, randomised controlled trial. BMJ. 2022 Nov 16;379:e072175. Open 111. Mark Loeb, Amy Bartholomew, Madiha Hashmi et al. Medical Masks Versus N95 Respirators for Preventing COVID 19 Among Health Care Workers. Ann Intern Med. 2022 Dec;175 12 1629 1638. Open 112. Christopher C Butler, F D Richard Hobbs, Oghenekome A Gbinigie et al. Molnupiravir plus usual care versus usual care alone as early treatment for adults with COVID 19 at increased risk of adverse outcomes PANORAMIC an open-label, platform-adaptive randomised controlled trial. Lancet. 2022 Dec 22;S0140 6736 22 02597 1. Open 113. Tatjana Welzel, Andrew Atkinson, Nina Sch bi et al. Methylprednisolone versus intravenous immunoglobulins in children with paediatric inflammatory multisystem syndrome temporally associated with SARS CoV 2 PIMS TS an open-label, multicentre, randomised trial. Lancet Child Adolesc Health. 2023 Feb 3;S2352 4642 23 00020 2. Open 114. writing committee for the BCG PRIME study group, Writing committee, Eva L Koekenbier et al. Bacillus Calmette-Gu rin vaccine for prevention of COVID 19 and other respiratory tract infections in older adults with comorbidities: a randomized controlled trial. Clin Microbiol Infect. 2023 Jan 31;S1198 743X 23 00044 7. Open 115. Zhujun Cao, Weiyi Gao, Hong Bao et al. VV116 versus Nirmatrelvir-Ritonavir for Oral Treatment of Covid-19. N Engl J Med. 2023 Feb 2;388 5 406 417. Open 116. Tracy Y Wang, Abdus S Wahed, Alison Morris et al. Effect of Thromboprophylaxis on Clinical Outcomes After COVID 19 Hospitalization. Ann Intern Med. 2023 Mar 21;M22 3350. Open 117. Gilmar Reis, Eduardo Augusto Dos Santos Moreira Silva, Daniela Carla Medeiros Silva et al. Oral Fluvoxamine With Inhaled Budesonide for Treatment of Early-Onset COVID 19 A Randomized Platform Trial. Ann Intern Med. 2023 May;176 5 667 675. Open 118. Laure F Pittet, Nicole L Messina, Francesca Orsini et al. Randomized Trial of BCG Vaccine to Protect against Covid-19 in Health Care Workers. N Engl J Med. 2023 Apr 27;388 17 1582 1596. Open 119. Tanja A Kuut, Fabiola M ller, Irene Csorba et al. Efficacy of cognitive behavioral therapy targeting severe fatigue following COVID 19 results of a randomized controlled trial. Clin Infect Dis. 2023 May 8;ciad257. Open 120. Yasmin Maor, Eilat Shinar, Marina Izak et al. A Randomized Controlled Study Assessing Convalescent Immunoglobulins versus Convalescent Plasma for Hospitalized COVID 19 Patients. Clin Infect Dis. 2023 May 23;ciad305. Online ahead of print. Open https://web.pathway.md/diseases/rec2UFiNfrEGXdLl0 38/38 |
Guideline sources The following summarized guidelines for the evaluation and management of COVID-19-associated pulmonary mucormycosis are prepared by our editorial team based on guidelines from the Academy of Pulmonary Sciences (APS/FISF 2022). 1 Guidelines 1. Diagnostic investigations Diagnostic imaging: obtain CT with IV contrast in the initial evaluation of patients with COVID-19- associated pulmonary mucormycosis. E Show 2 more Laboratory tests: obtain conventional microbiological testing from the lower respiratory tract samples in the initial evaluation of patients with COVID-19-associated pulmonary mucormycosis. E Show 3 more 2. Diagnostic procedures https://web.pathway.md/diseases/recVWJ0xdQoTmH9Xj 1/3 6/29/23, 3:24 AM COVID-19-associated pulmonary mucormycosis Pathway Bronchoscopy: Perform early flexible bronchoscopy in most patients with COVID-19-associated pulmonary mucormycosis due to the following advantages: visualization of airway abnormalities performing endobronchial biopsies provision of samples representing the lower respiratory tract (BAL or bronchial washings). E Biopsy: perform transthoracic tru-cut core-needle biopsy for diagnosing COVID-19-associated pulmonary mucormycosis in patients with peripheral chest lesions. E 3. Medical management Antifungal therapy (initial): administer liposomal amphotericin B 5 mg/kg/day as first-line therapy in patients with COVID-19-associated pulmonary mucormycosis. Consider escalating the dose to 10 mg/kg/day in patients with intracranial involvement. E Show 2 more Antifungal therapy (maintenance): initiate maintenance therapy with isavuconazole or posaconazole after achieving a complete or partial response. E Antifungal therapy (treatment failure): Do not combine antifungal drugs (posaconazole or isavuconazole with amphotericin) in patients with a treatment failure. D Continue liposomal amphotericin B or initiate posaconazole or isavuconazole for a longer duration until a complete or partial response is achieved, as salvage therapy in patients with treatment failure. E 4. Surgical interventions Lung resection: perform lung resection in all patients with potentially resectable lung disease. E Show 2 more Clinical findings Symptoms Past medical history Black sputum Covid-19 infection Brown sputum Diabetes mellitus Chest pain Immunocompromising condition Cough Fever Hemoptysis Shortness of breath Sputum production https://web.pathway.md/diseases/recVWJ0xdQoTmH9Xj 2/3 6/29/23, 3:24 AM COVID-19-associated pulmonary mucormycosis Pathway Imaging findings Lung consolidation Mediastinal lymphadenopathy Mycotic aneurysm Pneumothorax Pulmonary air-fluid levels Reversed halo sign Thick-walled pulmonary cavity Tree-in-bud pattern References 1. Valliappan Muthu, Ritesh Agarwal, Atul Patel et al. Definition, diagnosis, and management of COVID 19 associated pulmonary mucormycosis: Delphi consensus statement from the Fungal Infection Study Forum and Academy of Pulmonary Sciences, India. Lancet Infect Dis. 2022 Apr 04. Open https://web.pathway.md/diseases/recVWJ0xdQoTmH9Xj 3/3 |
Guideline sources The following summarized guidelines for the evaluation and management of critical illness-related corticosteroid insufficiency are prepared by our editorial team based on guidelines from the Surviving Sepsis Campaign (SSC 2021), the Infectious Diseases Society of America (IDSA/ATS 2019), the European Society of Intensive Care Medicine (ESICM/SCCM 2018; 2017), the British Medical Journal (BMJ 2018), and the European Association for the Study of the Liver (EASL 2018). 1 2 3 4 5 6 Guidelines 1. Diagnostic investigations Adrenal function testing: insufficient evidence to recommend whether to use delta cortisol (change in baseline cortisol at 60 mins of < 9 g/dL) after cosyntropin (250 g) administration or random plasma cortisol of < 10 g/dL for the diagnosis of critical illness-related corticosteroid insufficiency. Show 5 more 2. Medical management Corticosteroid administration, sepsis: https://web.pathway.md/diseases/recVKk6xYRcuppVax 1/3 6/23/23, 3:36 AM Critical illness-related corticosteroid insufficiency Pathway As per SSC 2021 guidelines, consider administering IV corticosteroids in adult patients with septic shock and an ongoing requirement for vasopressor therapy. E As per BMJ 2018 guidelines, consider administerings corticosteroids in adult and pediatric patients with sepsis, with or without shock. C As per ESICM/SCCM 2017 guidelines, avoid administering corticosteroids in adult patients with sepsis without shock. D Show 2 more Corticosteroid administration, pneumonia: As per ATS/IDSA 2019 guidelines: Do not administer corticosteroids routinely in adult patients with non-severe community- acquired pneumonia. D Avoid administering corticosteroids routinely in adult patients with severe community-acquired pneumonia D or severe influenza pneumonia. D As per ESICM/SCCM 2018 guidelines: Consider administering corticosteroids (IV hydrocortisone 400 mg/day, or equivalent) for 5-7 days in hospitalized patients with community-acquired pneumonia. C Avoid administering corticosteroids in adult patients with influenza. D Corticosteroid administration (ARDS): consider administering corticosteroids in patients with early moderate-to-severe ARDS (PaO /FiO of < 200 and within 14 days of onset). C Corticosteroid administration (meningitis): administer corticosteroids in patients with bacterial meningitis. B Corticosteroid administration (major trauma): avoid administering corticosteroids in patients with major trauma. D Corticosteroid administration (cardiopulmonary bypass surgery): consider administering corticosteroids in patients undergoing cardiopulmonary bypass surgery. C Corticosteroid administration (cardiac arrest): consider administering corticosteroids in patients with cardiac arrest. C 3. Specific circumstances Patients with liver cirrhosis: diagnose relative adrenal insufficiency based on a delta serum total cortisol of < 248 nmol/L (9 g/dL) after 250 g corticotropin injection or a random total cortisol of < 276 nmol/L (< 10 g/dL). B Show 2 more References https://web.pathway.md/diseases/recVKk6xYRcuppVax 2/3 6/23/23, 3:36 AM Critical illness-related corticosteroid insufficiency Pathway 1. Stephen M Pastores, Djillali Annane, Bram Rochwerg et al. Guidelines for the diagnosis and management of critical illness-related corticosteroid insufficiency CIRCI in critically ill patients Part II Society of Critical Care Medicine SCCM and European Society of Intensive Care Medicine ESICM 2017. Intensive Care Med. 2018 Apr;44 4 474 477. Open 2. Lamontagne F, Rochwerg B, Lytvyn L et al. Corticosteroid therapy for sepsis: a clinical practice guideline. BMJ. 2018 Aug 10;362:k3284. Open 3. Metlay JP, Waterer GW, Long AC et al. Diagnosis and Treatment of Adults with Community-acquired Pneumonia. An Official Clinical Practice Guideline of the American Thoracic Society and Infectious Diseases Society of America. Am J Respir Crit Care Med. 2019 Oct 1;200 7):e45-e67. Open 4. Djillali Annane, Stephen M Pastores, Bram Rochwerg et al. Guidelines for the Diagnosis and Management of Critical Illness-Related Corticosteroid Insufficiency CIRCI in Critically Ill Patients Part I Society of Critical Care Medicine SCCM and European Society of Intensive Care Medicine ESICM 2017. Crit Care Med. 2017 Dec;45 12 2078 2088. Open 5. Laura Evans, Andrew Rhodes, Waleed Alhazzani et al. Surviving sepsis campaign: international guidelines for management of sepsis and septic shock 2021. Intensive Care Med. 2021 Nov;47 11 1181 1247. Open 6. European Association for the Study of the Liver. EASL Clinical Practice Guidelines for the management of patients with decompensated cirrhosis. J Hepatol. 2018 Aug;69 2 406 460. Open https://web.pathway.md/diseases/recVKk6xYRcuppVax 3/3 |
Guideline sources The following summarized guidelines for the evaluation and management of Crohn's disease (CD) are prepared by our editorial team based on guidelines from the European Society for Clinical Nutrition and Metabolism (ESPEN 2023), the European Society of Gastrointestinal Endoscopy (ESGE 2023), the World Society of Emergency Surgery (WSES/AAST 2021), the American Gastroenterological Association (AGA 2021; 2020; 2018; 2017; 2013), the American College of Gastroenterology (ACG 2021; 2018; 2017), the American College of Radiology (ACR 2021), the American Society of Colon and Rectal Surgeons (ASCRS 2020; 2015), the European Crohn's and Colitis Organisation (ECCO 2020; 2019; 2015), the European Society for Paediatric Gastroenterology Hepatology and Nutrition (ESPGHAN/ECCO 2020), the Canadian Association of Gastroenterologists (CAG 2019), the British Society of Gastroenterology (BSG 2019), the https://web.pathway.md/diseases/recFEepjgYMnE2bAn 1/28 6/23/23, 3:37 AM Crohn's disease Pathway Healthcare Infection Society (HIS/BSG 2018), the Latin American Federation of Nutritional Therapy, Clinical Nutrition and Metabolism (FELANPE/ASPEN 2017), and the Clinical Pharmacogenetics Implementation Consortium (CPIC 2011). 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 17 18 19 20 21 22 23 24 25 26 27 27 27 28 Definition CD is a chronic relapsing IBD characterized by a transmural granulomatous inflammation most commonly affecting the ileum, colon, or both. 27 Epidemiology The exact cause of CD is unknown; however, a complex interplay of genetic factors, environmental factors, immune dysregulation to intestinal microbiota have been implicated. 27 Disease course The complex interplay of genetic, environmental factors and immune dysregulation to intestinal microbiota result in CD, which causes clinical manifestations of chronic diarrhea, fatigue, abdominal pain, weight loss, bloody stools, mucinous stools, peripheral arthritis, aphthous stomatitis, uveitis, erythema nodosum, ankylosing spondylitis, pyoderma, gangrenosum, psoriasis, PSC. Disease progression may lead to fistulae and decreased quality of life. 27 Prognosis and risk of recurrence The annual mortality rate in CD is 1.6%. 28 Guidelines 1. Screening and diagnosis Clinical presentation: Recognize that hallmark/cardinal symptoms of CD include abdominal pain, diarrhea, and fatigue; weight loss, fever, growth failure, anemia, recurrent fistulas, or extraintestinal manifestations can also be presenting features. E Recognize that extraintestinal manifestations of CD include: seronegative arthritis (both axial and peripheral) dermatological pathology (including pyoderma gangrenosum and erythema nodosum) ocular pathology (including uveitis, scleritis, and episcleritis) hepatobiliary disease (PSC) thromboembolic complications (both venous and arterial) bone disease (osteoporosis, osteonecrosis) cholelithiasis and nephrolithiasis https://web.pathway.md/diseases/recFEepjgYMnE2bAn 2/28 6/23/23, 3:37 AM Crohn's disease Pathway immune-mediated diseases (asthma, chronic bronchitis, pericarditis, psoriasis, celiac disease, rheumatoid arthritis, and multiple sclerosis). (Summary statement). E Natural history: Recognize that: CD, in most cases, is a chronic, progressive, destructive disease the location of CD tends to be stable, but can occasionally extend most, but not all, patients with CD will present with non-penetrating, non-stricturing disease behavior, but up to half of the patients would have developed an intestinal complication (stricture, abscess, fistula, or phlegmon) within 20 years of diagnosis patients with ileal, ileocolonic, or proximal gastrointestinal involvement are significantly more likely than patients with an isolated colonic disease to progress to an intestinal complication extensive anatomic involvement and deep ulcerations are other risk factors for progression to intestinal complications over long periods of observation, only 20-30% of patients with CD will have a non-progressive or indolent course, therefore, the majority of patients will require active effort to identify therapies that achieve adequate control of bowel inflammation features associated with a high risk for progressive disease burden include young age at diagnosis, initial extensive bowel involvement, ileal/ileocolonic involvement, perianal/severe rectal disease, and patients presenting with a penetrating or stenosis disease phenotype visceral adiposity may be a marker for increased risk of penetrating disease symptoms of CD do not correlate well with the presence of active inflammation perianal fistulizing CD occurs in up to one-quarter of patients symptoms of CD occur in most cases as a chronic, intermittent course; only a minority of patients will have continuously active symptomatic disease or prolonged symptomatic remission in the absence of immunomodulator or biologic treatment, corticosteroid dependency and/or resistance occurs in up to half of patients up to 80% of patients require hospitalization at some point during their clinical course, but the annual hospitalization rate decreases in later years after diagnosis the 10-year cumulative risk of major abdominal surgery in CD is 40-55%, although recent studies performed in the biologic era suggest that the 10-year risk may have decreased to 30%; the 10-year risk of a second resection after the first is 35%, although again more recent studies suggest that this may have dropped to closer to 30% the 5-year rate of symptomatic postoperative recurrence is 50% overall mortality is slightly increased, with a standardized mortality ratio of 1.4 times that of the general population; causes of excess mortality include gastrointestinal disease, gastrointestinal cancer, lung disease, and lung cancer. E Diagnosis: As per WSES 2021 guidelines, diagnose CD based on a set of modalities including clinical, biochemical, endoscopic, radiological, and histological diagnostics rather than a single reference standard. B https://web.pathway.md/diseases/recFEepjgYMnE2bAn 3/28 6/23/23, 3:37 AM Crohn's disease Pathway As per ACG 2018 guidelines, diagnose CD clinically, even though there are no truly pathognomonic features, with the support of endoscopic, radiographic, and histologic criteria with evidence of chronic intestinal inflammation. E 2. Diagnostic investigations Routine blood tests: Obtain laboratory investigations for inflammation, anemia, dehydration, and malnutrition as part of the initial evaluation of patients with CD. E Do not obtain serologic markers of IBD routinely to establish the diagnosis of CD. D Stool tests (fecal calprotectin): obtain fecal calprotectin to help differentiate the presence of IBD from IBS. B Stool tests, fecal pathogens: As per ACG 2021 guidelines, obtain stool testing for C. difficile infection in patients with IBD presenting with an acute flare associated with diarrhea. B As per ACG 2018 guidelines, obtain stool testing for fecal pathogens and C. difficile in patients with symptoms of active CD. E As per AGA 2017 guidelines, obtain stool testing for C. difficile infection in patients presenting with a flare of underlying IBD. B Emergency laboratory workup: Obtain the following laboratory tests, if possible, for the assessment of disease activity in patients with CD in urgent clinical situations: CBC, including Hgb, WBC count, and platelet count inflammatory markers, including serum CRP and ESR serum electrolytes liver enzymes serum albumin renal function tests blood workup for infectious causes. B Show 4 more Genetic testing: do not obtain genetic testing to establish the diagnosis of CD, albeit certain genetic variants are associated with different phenotypic expressions. D Diagnostic imaging, general approach: As per ESGE 2023 guidelines, obtain dedicated cross-sectional imaging for small-bowel evaluation to assess the extent and location of any CD lesions, to identify strictures, and to assess for extraluminal disease in patients with established CD based on ileocolonoscopy findings. A As per ACG 2018 guidelines, obtain small bowel imaging as part of the initial diagnostic workup in patients with suspected CD. E https://web.pathway.md/diseases/recFEepjgYMnE2bAn 4/28 6/23/23, 3:37 AM Crohn's disease Pathway Show 2 more As per AGA 2018 guidelines, obtain cross-sectional enterography at diagnosis of CD to detect small bowel inflammation and penetrating complications beyond the reach of standard ileocolonoscopy. A Show 6 more Diagnostic imaging, perianal disease: As per ACR 2021 guidelines, obtain any of the following modalities as the initial imaging of suspected proctitis or pouchitis: MR enterography CT enterography pelvic MRI without and with IV contrast pelvic CT with IV contrast. B As per CAG 2019 guidelines, obtain imaging MRI or EUS (based on availability and local expertise) in patients with CD and signs and/or symptoms of active fistulizing disease, to delineate the anatomy of the fistula tracts. B As per ACG 2018 guidelines, consider obtaining cross-sectional imaging with MRI of the pelvis and/or EUS to further characterize perianal CD and perirectal abscesses. E As per AGA 2018 guidelines, obtain dedicated pelvic MRI (perianal fistula MRI protocol) for the adequate preoperative assessment of perianal CD and its complications (number of fistula tracts, location and relationship to anal sphincter muscle complex, and presence of abscess). A Diagnostic imaging, complications: As per ESGE 2023 guidelines, obtain dedicated small-bowel cross-sectional imaging in patients with suspected CD and obstructive symptoms or known bowel stenosis. B As per AAST/WSES 2021 guidelines, obtain IV contrast-enhanced CT in the emergency setting in patients with IBD presenting with acute abdomen, to exclude intestinal perforation, stenosis, bleeding, and abscesses and to help guide decision-making for immediate surgery or initial conservative management. B Show 5 more As per ACG 2018 guidelines, obtain cross-sectional imaging of the abdomen and pelvis if an intra-abdominal abscess is suspected. E Evaluation of anemia: obtain anemia workup (including at least RBC indices such as red cell distribution width and MCV, reticulocyte count, differential blood cell count, serum ferritin, transferrin saturation, and CRP concentration) if the Hgb is below normal. Include the following in a more extensive workup: serum concentrations of vitamin B12, folic acid, haptoglobin, the percentage of hypochromic red cells, reticulocyte Hgb, LDH, soluble transferrin receptor, creatinine, and urea. B Show 8 more Screening for osteoporosis: https://web.pathway.md/diseases/recFEepjgYMnE2bAn 5/28 6/23/23, 3:37 AM Crohn's disease Pathway As per ESPEN 2023 guidelines, monitor serum 25-hydroxyvitamin D status in adult and pediatric patients with active IBD on corticosteroid treatment or suspected vitamin D deficiency. Initiate calcium/vitamin D supplementation, if required, to prevent low bone mineral density. Manage osteopenia and osteoporosis according to current osteoporosis guidelines. B As per ACG 2017 guidelines, screen for osteoporosis with bone mineral density testing at the time of diagnosis and periodically after diagnosis in patients with CD and conventional risk factors for abnormal bone mineral density. B Screening for depression and anxiety: Screen patients with IBD for depression and anxiety. B Assess and manage stress, depression, and anxiety as part of the comprehensive care of patients with CD. B 3. Diagnostic procedures Lower gastrointestinal endoscopy and biopsy: As per ESGE 2023 guidelines: Perform ileocolonoscopy as the first endoscopic examination in patients with suspected CD. A Perform device-assisted enteroscopy with small-bowel biopsies in patients with noncontributory ileocolonoscopy and suspected CD on small-bowel cross-sectional imaging or small-bowel capsule endoscopy. A As per WSES 2021 guidelines, perform sigmoidoscopy in an acute setting, if available, for intra- luminal assessment of distal disease activity, bleeding source identification, and biopsies. B As per ASCRS 2020 guidelines, perform biopsies of suspicious lesions (such as mass, ulcer) identified in patients with CD, especially when considering small bowel or colonic strictureplasty. B As per ACG 2018 guidelines, perform lower gastrointestinal endoscopy (ileocolonoscopy) with biopsies for the assessment of patients with suspected CD. E Show 2 more Upper gastrointestinal endoscopy: Perform upper gastrointestinal endoscopy only in patients with upper gastrointestinal signs and symptoms. E Consider performing deep enteroscopy to provide additional information in patients with suspected CD requiring biopsy/sampling of small bowel tissue to make a diagnosis. E Video capsule endoscopy, indications: As per ESGE 2023 guidelines, perform small bowel capsule endoscopy as the initial diagnostic modality for investigating the small bowel, in the absence of obstructive symptoms or known bowel stenosis, in patients with suspected CD and negative ileocolonoscopy findings. A Show 2 more https://web.pathway.md/diseases/recFEepjgYMnE2bAn 6/28 6/23/23, 3:37 AM Crohn's disease Pathway As per ACG 2018 guidelines, consider performing video capsule endoscopy as an adjunct in the diagnosis of small bowel CD in patients with a high index of suspicion of small bowel disease. E Video capsule endoscopy, preparation: As per ESGE 2023 guidelines, administer a patency capsule before small bowel capsule endoscopy to decrease the capsule retention rate in patients with suspected CD and obstructive symptoms B and in patients with established CD. B Show 2 more As per ACG 2018 guidelines, obtain small bowel imaging and/or patency capsule evaluation before video capsule endoscopy in patients with obstructive symptoms, in order to decrease the risk of capsule retention. E Video capsule endoscopy (capsule retention): offer initial conservative treatment in case of capsule retention. Perform device-assisted enteroscopy if medical therapy fails to achieve spontaneous capsule passage. A 4. Medical management Induction of remission, mild-to-moderate disease, 5-ASAs: As per ECCO 2020 guidelines, avoid using 5-aminosalicylic acid for induction of remission in patients with CD. D As per ACG 2018 guidelines: Consider initiating sulfasalazine for the treatment of patients with mild-to-moderately active colonic CD. C Do not use oral mesalamine for induction of remission and achieving mucosal healing in patients with active CD. D Induction of remission, mild-to-moderate disease, corticosteroids: As per ECCO 2020 guidelines, initiate budesonide for induction of clinical remission in patients with active mild-to-moderate CD limited to the ileum and/or ascending colon. B As per ACG 2018 guidelines, administer controlled ileal release budesonide at a dose of 9 mg once daily for induction of symptomatic remission in patients with mild-to-moderate ileocecal CD. B Induction of remission, mild-to-moderate disease, antibiotics: As per ESPEN 2023 guidelines, insufficient evidence to recommend an antibiotic regimen for the management of active CD. I As per ACG 2018 guidelines: Do not use metronidazole D or ciprofloxacin as primary therapy for induction of remission in patients with luminal inflammatory CD. D Do not use antimycobacterial agents as primary therapy for induction of remission or mucosal healing in patients with CD. D https://web.pathway.md/diseases/recFEepjgYMnE2bAn 7/28 6/23/23, 3:37 AM Crohn's disease Pathway Induction of remission, moderate-to-severe disease (5-ASAs): do not use 5-ASA or sulfasalazine for induction of remission in adult outpatients with moderate-to-severe CD. D Induction of remission, moderate-to-severe disease, corticosteroids: As per AGA 2021 guidelines, consider initiating corticosteroids for induction of remission in adult outpatients with moderate-to-severe CD. C As per ECCO 2020 guidelines, consider initiating systemic corticosteroids for induction of clinical response and remission in patients with active moderate-to-severe CD. C As per ACG 2018 guidelines, initiate a short-term course of oral corticosteroids to alleviate signs and symptoms in patients with moderate-to-severely active CD. B Show 2 more Induction of remission, moderate-to-severe disease, other immunosuppressants: As per AGA 2021 guidelines: Consider initiating methotrexate monotherapy (SC or intramuscularly) for induction of remission in adult outpatients with moderate-to-severe CD. C Avoid using oral methotrexate monotherapy for induction of remission in adult outpatients with moderate-to-severe CD. D As per ACG 2018 guidelines: Consider initiating methotrexate (up to 25 mg once weekly intramuscularly or SC) to alleviate signs and symptoms in patients with corticosteroid-dependent CD. C Do not use cyclosporine, mycophenolate mofetil, or tacrolimus in patients with CD. D As per AGA 2013 guidelines, avoid using methotrexate for induction of remission in patients with moderately severe CD. D Induction of remission, moderate-to-severe disease, thiopurines: As per AGA 2021 guidelines, avoid using thiopurine monotherapy for achieving remission in adult outpatients with moderate-to-severe CD. D As per ECCO 2020 guidelines, avoid using thiopurines as monotherapy for induction of remission in patients with moderate-to-severe luminal CD. D As per ACG 2018 guidelines: Do not use thiopurines (azathioprine or 6-mercaptopurine) for induction of short-term symptomatic remission in patients with CD unless as corticosteroid-sparing agents. D Consider obtaining TPMT testing before using azathioprine or 6-mercaptopurine in patients with CD. B As per AGA 2013 guidelines, avoid using thiopurine monotherapy for induction of remission in patients with moderately severe CD. D Induction of remission, moderate-to-severe disease, anti-TNFs: As per AGA 2021 guidelines, initiate anti-TNFs for induction of remission in adult outpatients with moderate-to-severe CD. B Show 6 more https://web.pathway.md/diseases/recFEepjgYMnE2bAn 8/28 6/23/23, 3:37 AM Crohn's disease Pathway As per ECCO 2020 guidelines: Initiate anti-TNF agents (infliximab, adalimumab, or certolizumab pegol) for induction of remission in patients with moderate-to-severe CD not responding to conventional therapy. B Avoid combining adalimumab with thiopurines to achieve clinical remission and response. D As per ACG 2018 guidelines, initiate anti-TNF agents (infliximab, adalimumab, certolizumab pegol) for the treatment of patients with corticosteroid-resistant CD. B Show 4 more As per AGA 2013 guidelines: Initiate anti-TNF agents for induction of remission in patients with moderately severe CD. Prefer anti-TNF monotherapy B or anti-TNF agents in combination with thiopurines over thiopurine monotherapy. A Consider initiating anti-TNF agents in combination with thiopurines over anti-TNF monotherapy for induction of remission in patients with moderately severe CD. C Induction of remission, moderate-to-severe disease, other biologics: As per AGA 2021 guidelines, initiate ustekinumab (Strong recommendation, moderate certainty evidence) or consider initiating for induction of remission in adult outpatients with moderate-to- severe CD never responded to anti-TNFs (primary nonresponse). B Show 4 more As per ECCO 2020 guidelines, initiate ustekinumab B or vedolizumab for induction of remission in patients with moderate-to-severe CD with inadequate response to conventional therapy and/or anti-TNF therapy. B As per ACG 2018 guidelines, consider initiating anti-integrin therapy (with vedolizumab) with or without an immunomodulator for induction of symptomatic remission in patients with moderate- to-severely active CD and objective evidence of active disease. B Show 2 more Maintenance therapy, medically induced remission, thiopurines: As per AGA 2021 guidelines, consider initiating thiopurine monotherapy for maintenance of remission in adult outpatients with quiescent moderate-to-severe CD (or patients in corticosteroid-induced remission). C As per ECCO 2020 guidelines, INITIATE thiopurines for maintenance of remission in patients with corticosteroid-dependent CD. B Show 2 more As per ACG 2018 guidelines: Consider initiating a thiopurine (azathioprine or 6-mercaptourine) or methotrexate once remission is induced with corticosteroids. B Initiate a thiopurine or methotrexate with or without anti-TNF therapy in corticosteroid- dependent patients. B As per AGA 2013 guidelines, initiate thiopurines for maintenance of corticosteroid-induced remission. B https://web.pathway.md/diseases/recFEepjgYMnE2bAn 9/28 6/23/23, 3:37 AM Crohn's disease Pathway Maintenance therapy, medically induced remission, methotrexate: As per AGA 2021 guidelines: Consider initiating methotrexate monotherapy (SC or intramuscularly) for maintenance of remission in adult outpatients with moderate-to-severe CD. C Avoid using oral methotrexate monotherapy for maintenance of remission in adult outpatients with moderate-to-severe CD. D As per ECCO 2020 guidelines, initiate parenteral methotrexate for maintenance of remission in patients with corticosteroid-dependent CD. B As per ACG 2018 guidelines, consider initiating a thiopurine or methotrexate once remission is induced with corticosteroids. B Show 2 more As per AGA 2013 guidelines, consider initiating methotrexate for maintenance of corticosteroid- induced remission. C Maintenance therapy, medically induced remission, anti-TNFs: As per AGA 2021 guidelines, initiate anti-TNFs for maintenance of remission in adult outpatients with moderate-to-severe CD. B Show 3 more As per ECCO 2020 guidelines, continue the same treatment for maintenance of remission in patients with CD achieved remission with anti-TNF agents. B Show 2 more As per ACG 2018 guidelines: Consider initiating anti-TNF therapy, specifically infliximab, adalimumab, or certolizumab pegol, for maintenance of anti-TNF-induced remission. B Consider combining anti-TNFs with azathioprine/6-mercaptopurine or methotrexate for maintenance of anti-TNF-induced remission because of the potential for immunogenicity and loss of response with anti-TNF monotherapy. B As per AGA 2013 guidelines: Initiate anti-TNF agents for maintenance of corticosteroid- or anti-TNF-induced remission. A Insufficient evidence to recommend for or against the combination of an anti-TNF agent and a thiopurine versus an anti-TNF agent alone for maintenance of remission induced by a combination of these agents in patients with CD. I Maintenance therapy, medically induced remission, other biologics: As per AGA 2021 guidelines, initiate ustekinumab B or consider initiating vedolizumab for maintenance of remission in adult outpatients with moderate-to-severe CD. B Show 2 more As per ECCO 2020 guidelines: Initiate vedolizumab for maintaining clinical remission in patients with moderate-to-severe CD achieved remission with vedolizumab. B https://web.pathway.md/diseases/recFEepjgYMnE2bAn 10/28 6/23/23, 3:37 AM Crohn's disease Pathway Initiate ustekinumab for maintaining clinical remission in patients with CD achieved remission with ustekinumab. B As per ACG 2018 guidelines, consider initiating vedolizumab for maintenance of vedolizumab- induced remission. C Show 2 more Maintenance therapy, medically induced remission, 5-ASAs: As per AGA 2021 guidelines, do not use 5-ASA or sulfasalazine for maintenance of remission in adult outpatients with moderate-to-severe CD. D As per ECCO 2020 guidelines, do not use oral 5-aminosalicylic acid for maintenance of medically induced remission in patients with CD. D As per ACG 2018 guidelines, do not use long-term oral 5-aminosalicylic acid for maintenance of medically induced remission in patients with CD. D Maintenance therapy, medically induced remission, corticosteroids: As per AGA 2021 guidelines, do not use corticosteroids for maintenance of remission in adult outpatients with moderate-to-severe CD. D As per ACG 2018 guidelines: Do not use long-term corticosteroids for maintenance of medically induced remission in patients with CD. D Do not use budesonide beyond 4 months for maintenance of remission in patients with CD. D Maintenance therapy, medically induced remission, antibiotics: As per ESPEN 2023 guidelines, insufficient evidence to recommend an antibiotic regimen for the maintenance of medically-induced remission. I As per ACG 2018 guidelines, do not use antimycobacterial agents for maintenance of remission in patients with CD. D Maintenance therapy, surgically induced remission: As per ACG 2018 guidelines, initiate prophylactic treatment after small intestinal resection in patients with risk factors for recurrence. E Show 6 more As per AGA 2017 guidelines, consider initiating early pharmacological prophylaxis, rather than endoscopy-guided pharmacological treatment, in patients with surgically induced remission. C Show 2 more Symptomatic therapy: Offer antidiarrheals, other nonspecific medications, and dietary manipulation for the treatment of symptoms in patients with low risk of progression, along with careful observation for inadequate symptom relief, worsening inflammation, or disease progression. B Avoid using NSAIDs in patients with CD, when possible, as they may exacerbate disease activity. D https://web.pathway.md/diseases/recFEepjgYMnE2bAn 11/28 6/23/23, 3:37 AM Crohn's disease Pathway Management of acute abdomen: ensure a multidisciplinary approach, involving a gastroenterologist and an acute care surgeon, B to decide on medical treatment in patients with IBD presenting with acute abdominal pain. B Show 3 more Management of intra-abdominal abscesses, antibiotics and drainage: As per ASCRS 2020 guidelines, consider administering antibiotics with or without drainage in patients with penetrating CD with abscess formation, followed by interval elective resection or medical therapy depending on the clinical situation and patient preferences. C As per ACG 2018 guidelines, administer antibiotics and perform a drainage procedure, either radiographically or surgically, in patients with an intra-abdominal abscess. B Management of intra-abdominal abscesses (anti-inflammatory therapy): consider administering infliximab if anti-inflammatory therapy for penetrating ileocecal CD is required, following adequate resolution of intra-abdominal abscesses in a multidisciplinary approach. C Management of anemia, iron supplementation: As per ESPEN 2023 guidelines, initiate iron supplementation in all patients with IBD and iron deficiency anemia, to correct anemia and normalize iron stores. B Show 2 more As per ECCO 2015 guidelines, initiate iron supplementation in all patients with IBD and iron deficiency anemia. A Show 4 more Management of anemia (RBC transfusion): consider administering RBC transfusion in patients with anemia with Hgb levels < 7 g/dL, or > 7 g/dL if symptoms or particular risk factors are present, followed by subsequent IV iron supplementation. C Management of anemia (monitoring of recurrence): Monitor patients with IBD for recurrent iron deficiency every 3 months for at least a year after correction, and 6-12 months thereafter. B Consider suspecting persistent intestinal disease activity in cases of recurrent anemia, even if there is clinical remission and inflammatory parameters (such as CRP) are normal. C Management of anemia (management of recurrence): Recognize that IBD-associated iron deficiency and anemia recur frequently and fast, even after treatment with IV iron, while the recurrence of iron deficiency is lower in patients with higher post-treatment ferritin levels. B Initiate re-treatment with IV iron as soon as serum ferritin drops < 100 g/L or Hgb < 12-13 g/dL (according to gender) after successful treatment of iron deficiency anemia with IV iron. B Management of anemia (management of non-IDA anemia): exclude other possible concomitant diseases, such as infections, malignancies, and side effects of medications, in the management of non-iron deficiency anemia in patients with IBD. B Show 4 more https://web.pathway.md/diseases/recFEepjgYMnE2bAn 12/28 6/23/23, 3:37 AM Crohn's disease Pathway 5. Inpatient care Thromboprophylaxis: administer VTE prophylaxis with LMWH as soon as possible in the emergency setting for the high risk of thrombotic events related to complicated IBD. A 6. Nonpharmacologic interventions Smoking cessation: advise patients with CD to quit smoking as it exacerbates disease activity and accelerates disease recurrence. Encourage active smoking cessation programs. B Physical activity: encourage endurance training in all patients with IBD. Advise appropriate physical activity (mainly resistance training) in patients with IBD with decreased muscle mass and/or muscle performance. B Nutritional support (counseling): Provide individual counseling by a dietitian as part of the multidisciplinary approach to improve nutritional therapy and avoid malnutrition and nutrition-related disorders in all patients with IBD, B as well as regarding malabsorption or maldigestion in patients with CD. B Counsel patients with IBD with hyperoxaluria regarding fat malabsorption as they often also have fat malabsorption. B Nutritional support, general principles: As per ESPEN 2023 guidelines: Obtain nutritional screening and deliver dietary management by a multidisciplinary IBD team including nurses. B Insufficient evidence to recommend an "IBD diet" to promote remission in patients with IBD with active disease. I As per AAST/WSES 2021 guidelines, provide nutritional support (parenteral or enteral, according to gastrointestinal function and in conjunction with a dietitian/nutrition team) as soon as possible. B Nutritional support (exclusion diet): Consider offering a CD exclusion diet, with or without enteral nutrition, in adult patients with mild-to-moderately active CD. C Consider offering a CD exclusion diet with partial enteral nutrition as an alternative to exclusive enteral nutrition to achieve remission in pediatric patients with mild-to-moderately active CD. C Nutritional support (oral nutritional supplements): Offer oral nutritional supplements as the first step when medical nutrition is indicated in patients with IBD, as supportive therapy in addition to regular food. B Consider offering oral nutritional supplements or enteral nutrition in patients with CD in remission if malnutrition cannot be treated sufficiently by dietary counseling. C Nutritional support (enteral nutrition): consider initiating enteral nutrition as supportive therapy if oral feeding is insufficient. Consider preferring enteral nutrition using formulas or liquids over https://web.pathway.md/diseases/recFEepjgYMnE2bAn 13/28 6/23/23, 3:37 AM Crohn's disease Pathway parenteral nutrition unless completely contraindicated. C Show 8 more Nutritional support, parenteral nutrition: As per ESPEN 2023 guidelines, initiate parenteral nutrition in patients with IBD in the following situations: oral or enteral nutrition is not sufficiently possible, such as dysfunctional gastrointestinal tract or CD with short bowel bowel obstruction with no possibility of placement of a feeding tube beyond the obstruction, or if these attempts have failed other complications, such as an anastomotic leak or a high output intestinal fistula. B Show 3 more As per AAST/WSES 2021 guidelines: Reserve TPN for nutritionally deficient patients unable to tolerate enteral nutrition and when the enteral route is contraindicated, in critically ill patients presenting with signs of shock, intestinal ischemia, high output fistula, and/or severe intestinal hemorrhage. B Initiate TPN as the mode of choice in patients with complicated IBD requiring emergency surgery. A Nutritional support (energy requirements): ensure energy intake of 30-35 kcal/kg/day in patients with IBD, as their requirements are similar to the healthy population. Determine individual energy requirements using indirect calorimetry and an individual physical activity factor if there is a clinical suspicion of a different energy requirement in particular disease states. B Nutritional support (protein requirements): Ensure protein intake of ~ 1 g/kg/day in adult patients with IBD in remission, as their requirements are similar to the general population. B Increase protein intake to 1.2-1.5 g/kg/day in adult patients with active IBD. B Nutritional support (micronutrient requirements): assess for micronutrient deficiencies regularly in patients with IBD, including in the remission phase, and correct specific deficits appropriately. B Nutritional support (vitamin supplements): initiate vitamin B12 supplementation in patients with CD when > 20 cm of the distal ileum is resected, whether or not in combination with the ileocecal valve, or when vitamin B12 deficiency is documented. B Show 2 more Nutritional support (management of malnutrition): screen for malnutrition at the time of diagnosis and regularly thereafter in patients with IBD. B Show 2 more Nutritional support (avoidance of dehydration): make every effort to avoid dehydration in patients with IBD. B Nutritional support (avoidance of refeeding syndrome): consider undertaking standard precautions and interventions to prevent the refeeding syndrome, particularly concerning https://web.pathway.md/diseases/recFEepjgYMnE2bAn 14/28 6/23/23, 3:37 AM Crohn's disease Pathway phosphate and thiamine, in patients with IBD and nutritional deprivation extended over many days. C Probiotics: As per ESPEN 2023 guidelines: Do not offer probiotics for the treatment of active disease or for the prevention of relapse in the remission phase or postoperative recurrence of disease in patients with CD. D Do not offer prebiotics for the treatment of CD, neither in active disease nor for maintenance of remission. D As per AGA 2020 guidelines, offer probiotics in patients with CD only in the context of a clinical trial. B 7. Therapeutic procedures Fecal microbiota transplantation: insufficient evidence to recommend for or against fecal microbiota transplantation in patients with IBD. I 8. Perioperative care Preoperative medications: As per WSES 2021 guidelines: Wean off corticosteroids (preoperatively, ideally 4 weeks) and stop anti-TNFs before surgery, as soon as possible to decrease the risk of postoperative complications, in accordance with a gastroenterologist. B Recognize that preoperative anti-TNFs and corticosteroids are risk factors for intra-abdominal sepsis in patients requiring emergency resectional surgery. B As per ASCRS 2020 guidelines, attempt weaning corticosteroids before surgical intervention as preoperative high-dose corticosteroids increase the risk of postoperative infectious complications. B Show 2 more As per ECCO 2019 guidelines, consider reducing the dose of corticosteroids preoperatively, with a careful monitoring to avoid increasing disease burden, in order to reduce the rates of postoperative complications. C Show 2 more Perioperative nutrition: As per ESPEN 2023 guidelines, assess nutritional status before planned surgery. Administer dietetic interventions including nutritional therapy in patients with malnutrition or at nutritional risk. B Show 10 more As per WSES 2021 guidelines, provide preoperative nutritional support in severely undernourished patients. A https://web.pathway.md/diseases/recFEepjgYMnE2bAn 15/28 6/23/23, 3:37 AM Crohn's disease Pathway As per ASCRS 2020 guidelines, consider providing preoperative nutritional support to decrease postoperative morbidity in patients with malnutrition. C As per ECCO 2019 guidelines, obtain preoperative nutritional assessment in all patients with CD planned to undergo surgery. Optimize nutritional status with enteral or parenteral nutrition before surgery in patients with nutritional deficiencies. B Preoperative smoking cessation: advise smoking cessation to reduce postoperative morbidity in patients with CD. B 9. Surgical interventions Indications for surgery, refractory disease: As per AAST/WSES 2021 guidelines: Perform surgical exploration by laparotomy in hemodynamically unstable patients with severe colitis not responding to medical treatment, taking into consideration damage control surgery principles with or without an open abdomen. B Perform subtotal colectomy with ileostomy in patients presenting with acute severe refractory colitis, and massive colorectal bleeding not responding to medical treatment, in a laparoscopic or open approach according to patient's hemodynamic stability and surgeon's skills. B As per ASCRS 2020 guidelines: Perform surgery in patients with severe acute colitis not adequately responding to medical therapy. B Consider performing surgery in patients with an inadequate response to, developing complications from, or being nonadherent with medical therapy. B Indications for surgery, hemorrhage: As per AAST/WSES 2021 guidelines, consider performing sigmoidoscopy and upper gastrointestinal endoscopy in hemodynamically stable patients with IBD presenting with gastrointestinal bleeding. C Attempt preoperative localization of the bleeding site with the aim of excluding upper gastrointestinal or anorectal bleeding to allow better planning of the surgical strategy. C Show 6 more As per ASCRS 2020 guidelines, consider evaluating and treating stable patients with gastrointestinal hemorrhage by endoscopic and/or interventional radiologic techniques. Perform operative exploration in unstable patients despite resuscitation efforts. B Indications for surgery, perforation: As per AAST/WSES 2021 guidelines, perform surgical exploration in the presence of radiological signs of pneumoperitoneum and free fluid within the peritoneal cavity in acutely unwell patients presenting with complicated CD or acute severe ulcerative colitis. B Show 4 more As per ASCRS 2020 guidelines: Perform surgery in patients with signs or symptoms of impending or actual perforation. B https://web.pathway.md/diseases/recFEepjgYMnE2bAn 16/28 6/23/23, 3:37 AM Crohn's disease Pathway Perform surgical resection of the perforated segment in patients with free perforation. B Indications for surgery, strictures: As per AAST/WSES 2021 guidelines, perform surgery in patients with CD with symptomatic intestinal strictures not responding to medical therapy and not amenable to endoscopic dilatation. B Show 3 more As per ASCRS 2020 guidelines, consider performing endoscopic dilation in patients with short- segment, non-inflammatory, symptomatic small bowel or anastomotic strictures. B Show 4 more As per ECCO 2019 guidelines, defer surgery in adult patients with CD presenting with acute small bowel obstruction without bowel ischemia or peritonitis. B Show 2 more Indications for surgery (toxic megacolon): perform surgical exploration by laparotomy in hemodynamically unstable patients presenting with toxic megacolon, taking into consideration damage control surgery principles with or without an open abdomen. B Show 3 more Indications for surgery, abdominal abscess: |
bowel obstruction with no possibility of placement of a feeding tube beyond the obstruction, or if these attempts have failed other complications, such as an anastomotic leak or a high output intestinal fistula. B Show 3 more As per AAST/WSES 2021 guidelines: Reserve TPN for nutritionally deficient patients unable to tolerate enteral nutrition and when the enteral route is contraindicated, in critically ill patients presenting with signs of shock, intestinal ischemia, high output fistula, and/or severe intestinal hemorrhage. B Initiate TPN as the mode of choice in patients with complicated IBD requiring emergency surgery. A Nutritional support (energy requirements): ensure energy intake of 30-35 kcal/kg/day in patients with IBD, as their requirements are similar to the healthy population. Determine individual energy requirements using indirect calorimetry and an individual physical activity factor if there is a clinical suspicion of a different energy requirement in particular disease states. B Nutritional support (protein requirements): Ensure protein intake of ~ 1 g/kg/day in adult patients with IBD in remission, as their requirements are similar to the general population. B Increase protein intake to 1.2-1.5 g/kg/day in adult patients with active IBD. B Nutritional support (micronutrient requirements): assess for micronutrient deficiencies regularly in patients with IBD, including in the remission phase, and correct specific deficits appropriately. B Nutritional support (vitamin supplements): initiate vitamin B12 supplementation in patients with CD when > 20 cm of the distal ileum is resected, whether or not in combination with the ileocecal valve, or when vitamin B12 deficiency is documented. B Show 2 more Nutritional support (management of malnutrition): screen for malnutrition at the time of diagnosis and regularly thereafter in patients with IBD. B Show 2 more Nutritional support (avoidance of dehydration): make every effort to avoid dehydration in patients with IBD. B Nutritional support (avoidance of refeeding syndrome): consider undertaking standard precautions and interventions to prevent the refeeding syndrome, particularly concerning https://web.pathway.md/diseases/recFEepjgYMnE2bAn 14/28 6/23/23, 3:37 AM Crohn's disease Pathway phosphate and thiamine, in patients with IBD and nutritional deprivation extended over many days. C Probiotics: As per ESPEN 2023 guidelines: Do not offer probiotics for the treatment of active disease or for the prevention of relapse in the remission phase or postoperative recurrence of disease in patients with CD. D Do not offer prebiotics for the treatment of CD, neither in active disease nor for maintenance of remission. D As per AGA 2020 guidelines, offer probiotics in patients with CD only in the context of a clinical trial. B 7. Therapeutic procedures Fecal microbiota transplantation: insufficient evidence to recommend for or against fecal microbiota transplantation in patients with IBD. I 8. Perioperative care Preoperative medications: As per WSES 2021 guidelines: Wean off corticosteroids (preoperatively, ideally 4 weeks) and stop anti-TNFs before surgery, as soon as possible to decrease the risk of postoperative complications, in accordance with a gastroenterologist. B Recognize that preoperative anti-TNFs and corticosteroids are risk factors for intra-abdominal sepsis in patients requiring emergency resectional surgery. B As per ASCRS 2020 guidelines, attempt weaning corticosteroids before surgical intervention as preoperative high-dose corticosteroids increase the risk of postoperative infectious complications. B Show 2 more As per ECCO 2019 guidelines, consider reducing the dose of corticosteroids preoperatively, with a careful monitoring to avoid increasing disease burden, in order to reduce the rates of postoperative complications. C Show 2 more Perioperative nutrition: As per ESPEN 2023 guidelines, assess nutritional status before planned surgery. Administer dietetic interventions including nutritional therapy in patients with malnutrition or at nutritional risk. B Show 10 more As per WSES 2021 guidelines, provide preoperative nutritional support in severely undernourished patients. A https://web.pathway.md/diseases/recFEepjgYMnE2bAn 15/28 6/23/23, 3:37 AM Crohn's disease Pathway As per ASCRS 2020 guidelines, consider providing preoperative nutritional support to decrease postoperative morbidity in patients with malnutrition. C As per ECCO 2019 guidelines, obtain preoperative nutritional assessment in all patients with CD planned to undergo surgery. Optimize nutritional status with enteral or parenteral nutrition before surgery in patients with nutritional deficiencies. B Preoperative smoking cessation: advise smoking cessation to reduce postoperative morbidity in patients with CD. B 9. Surgical interventions Indications for surgery, refractory disease: As per AAST/WSES 2021 guidelines: Perform surgical exploration by laparotomy in hemodynamically unstable patients with severe colitis not responding to medical treatment, taking into consideration damage control surgery principles with or without an open abdomen. B Perform subtotal colectomy with ileostomy in patients presenting with acute severe refractory colitis, and massive colorectal bleeding not responding to medical treatment, in a laparoscopic or open approach according to patient's hemodynamic stability and surgeon's skills. B As per ASCRS 2020 guidelines: Perform surgery in patients with severe acute colitis not adequately responding to medical therapy. B Consider performing surgery in patients with an inadequate response to, developing complications from, or being nonadherent with medical therapy. B Indications for surgery, hemorrhage: As per AAST/WSES 2021 guidelines, consider performing sigmoidoscopy and upper gastrointestinal endoscopy in hemodynamically stable patients with IBD presenting with gastrointestinal bleeding. C Attempt preoperative localization of the bleeding site with the aim of excluding upper gastrointestinal or anorectal bleeding to allow better planning of the surgical strategy. C Show 6 more As per ASCRS 2020 guidelines, consider evaluating and treating stable patients with gastrointestinal hemorrhage by endoscopic and/or interventional radiologic techniques. Perform operative exploration in unstable patients despite resuscitation efforts. B Indications for surgery, perforation: As per AAST/WSES 2021 guidelines, perform surgical exploration in the presence of radiological signs of pneumoperitoneum and free fluid within the peritoneal cavity in acutely unwell patients presenting with complicated CD or acute severe ulcerative colitis. B Show 4 more As per ASCRS 2020 guidelines: Perform surgery in patients with signs or symptoms of impending or actual perforation. B https://web.pathway.md/diseases/recFEepjgYMnE2bAn 16/28 6/23/23, 3:37 AM Crohn's disease Pathway Perform surgical resection of the perforated segment in patients with free perforation. B Indications for surgery, strictures: As per AAST/WSES 2021 guidelines, perform surgery in patients with CD with symptomatic intestinal strictures not responding to medical therapy and not amenable to endoscopic dilatation. B Show 3 more As per ASCRS 2020 guidelines, consider performing endoscopic dilation in patients with short- segment, non-inflammatory, symptomatic small bowel or anastomotic strictures. B Show 4 more As per ECCO 2019 guidelines, defer surgery in adult patients with CD presenting with acute small bowel obstruction without bowel ischemia or peritonitis. B Show 2 more Indications for surgery (toxic megacolon): perform surgical exploration by laparotomy in hemodynamically unstable patients presenting with toxic megacolon, taking into consideration damage control surgery principles with or without an open abdomen. B Show 3 more Indications for surgery, abdominal abscess: As per AAST/WSES 2021 guidelines, perform image-guided percutaneous drainage of intra- abdominal abscesses > 3 cm and administer early empiric antibiotic therapy and adapt antibiotics as soon as possible to microbiological cultures results. Reevaluate antibiotic therapy according to the patient's clinical and biochemical features. B Show 3 more As per ASCRS 2020 guidelines, consider administering antibiotics with or without drainage in patients with penetrating CD with abscess formation, followed by interval elective resection or medical therapy depending on the clinical situation and patient preferences. C As per ECCO 2019 guidelines: Perform image-guided percutaneous drainage as the primary treatment approach in patients with well-defined accessible intra-abdominal abscesses. B Consider initiating medical management following successful image-guided drainage of an intra-abdominal abscess. Set a low threshold for surgery in the event of failure of medical therapy. C As per ACG 2018 guidelines, perform surgical or image-guided percutaneous drainage, along with antibiotic administration, for the treatment of patients with an intra-abdominal abscess. B Indications for surgery, anorectal abscess: As per AAST/WSES 2021 guidelines, perform adequate surgical drainage of a perianal abscess in patients with CD without searching for an associated fistula. B Show 3 more As per ACG 2018 guidelines, drain abscesses (surgically or percutaneously) before initiating anti-TNFs in patients with fistulizing CD. B https://web.pathway.md/diseases/recFEepjgYMnE2bAn 17/28 6/23/23, 3:37 AM Crohn's disease Pathway Indications for surgery (enteric fistulas): consider surgery in patients with enteric fistulas that persist despite appropriate medical therapy. B Indications for surgery (carcinoma or dysplasia): obtain endoscopic surveillance in patients with visible dysplasia undergone complete endoscopic excision. Perform total colectomy or total proctocolectomy if dysplasia is not amenable to endoscopic excision, is also found in the surrounding flat mucosa, or is multifocal, or if colorectal adenocarcinoma is diagnosed. B Show 2 more Choice of surgical intervention: As per WSES 2021 guidelines, use laparoscopic approach in hemodynamically stable patients presenting with complications related to IBD, when skills are available, in order to decrease morbidity and length of hospital stay. B Show 3 more As per ASCRS 2020 guidelines, consider performing endoscopic dilation, bypass, or strictureplasty in patients with symptomatic disease of the stomach or duodenum despite medical therapy. B Show 11 more As per ECCO 2019 guidelines, use laparoscopic approach as the first-line approach in surgery for CD, dependent on appropriate expertise. B Show 7 more 10. Specific circumstances Pediatric patients (nutritional therapy): Offer dietary therapy with exclusive enteral nutrition as first-line therapy for induction of remission in pediatric patients with active luminal CD. B Offer monotherapy with maintenance enteral nutrition (at least 50% of daily energy requirements) to prolong remission in pediatric patients with low-risk CD reached clinical remission. B Pediatric patients (induction of remission, corticosteroids): consider initiating corticosteroids for induction of remission in pediatric patients with active luminal CD if exclusive enteral nutrition is not an option. C Pediatric patients (induction of remission, TNF inhibitors): initiate anti-TNF therapy for induction of remission in pediatric patients with a new-onset disease at high risk for a complicated disease course. B Show 6 more Pediatric patients (induction of remission, monoclonal antibodies): consider initiating ustekinumab or vedolizumab in patients failed to achieve clinical remission on anti-TNF agents, despite anti-TNF dose optimization and immunomodulator use. C Pediatric patients (maintenance of remission, DMARDs): https://web.pathway.md/diseases/recFEepjgYMnE2bAn 18/28 6/23/23, 3:37 AM Crohn's disease Pathway Consider initiating methotrexate as a first-choice immunomodulator, or after thiopurine failure or intolerance, to maintain clinical remission in pediatric patients with CD. C Consider initiating thiopurines (azathioprine or 6-mercaptopurine) to maintain remission in pediatric patients reached remission. C Pediatric patients (maintenance of remission, TNF inhibitors): initiate anti-TNF agents for maintenance of remission in pediatric patients with active CD failed to maintain remission with an immunomodulator. B Show 2 more Pediatric patients (maintenance of remission, monoclonal antibodies): consider initiating ustekinumab or vedolizumab in patients failed to maintain clinical remission on anti-TNF agents, despite anti-TNF dose optimization and immunomodulator use. C Pediatric patients (therapies to avoid): do not use thiopurine monotherapy for induction of remission in pediatric patients with active CD. D Show 2 more Pediatric patients (monitoring of treatment response): consider assessing for a decrease of fecal calprotectin in the context of clinical improvement as a marker of treatment response in pediatric patients with luminal CD following induction therapy. C Show 5 more Pregnant patients: Monitor iron status and folate levels regularly in pregnant patients with IBD and supplement iron and folic acid in case of deficiencies. B Monitor nutritional status regularly in patients with IBD when breastfeeding and supplement in case of deficiencies. B Patients with obesity: as per ESPEN 2023 guidelines, advise weight loss in patients with IBD and obesity only in phases of stable remission and then according to current obesity guidelines. B Patients with TPMT deficiency (adjustment of azathioprine): Reduce doses of azathioprine in patients with a heterozygous variant TPMT genotype. Start at 30-70% of the full dose (such as 1-1.5 mg/kg/day) and adjust doses based on patient tolerance. Allow 2-4 weeks to reach a steady state after each dose adjustment. A Consider initiating alternative nonthiopurine immunosuppressant therapy, rather than azathioprine, in patients with a homozygous variant TPMT genotype. Start with drastically reduced doses of azathioprine (reduce daily dose by 10-fold and dose three times weekly, instead of daily), and adjust doses based on the degree of myelosuppression and disease- specific guidelines. Allow 4-6 weeks to reach a steady state after each dose adjustment. B Patients with TPMT deficiency (adjustment of 6-mercaptopurine): Reduce doses of 6-mercaptopurine in patients with a heterozygous variant TPMT genotype. Start at 30-70% of the full dose (such as 0.75 mg/kg/day) and adjust doses based on the degree of myelosuppression and disease-specific guidelines. Allow 2-4 weeks to reach a steady state after each dose adjustment. A https://web.pathway.md/diseases/recFEepjgYMnE2bAn 19/28 6/23/23, 3:37 AM Crohn's disease Pathway Consider initiating alternative nonthiopurine immunosuppressant therapy, rather than 6- mercaptourine, in patients with a homozygous variant TPMT genotype being treated for nonmalignant conditions. B Patients with fistulizing disease, anti-TNFs: As per AAST/WSES 2021 guidelines, consider initiating infliximab or adalimumab as first-line therapy in combination with azathioprine following adequate surgical drainage, if indicated, in patients with complex perianal fistulizing disease. Consider combining anti-TNF therapy with ciprofloxacin for improved short-term outcomes. B As per AGA 2021 guidelines, initiate infliximab B or consider initiating adalimumab for induction and maintenance of fistula remission in adult outpatients with CD and active perianal fistula. B As per ECCO 2020 guidelines: Initiate infliximab B or consider initiating adalimumab for induction and maintenance of remission in patients with complex perianal fistulas. B Insufficient evidence regarding the effect of adding immunomodulators to anti-TNF therapy on fistula healing in patients with complex perianal fistulas. I As per CAG 2019 guidelines, initiate anti-TNF therapy to induce a symptomatic response in patients with CD and evidence of fistulizing disease. B Show 2 more As per ACG 2018 guidelines: Consider initiating infliximab for the treatment of perianal, enterocutaneous and rectovaginal fistulas in patients with CD. Consider adding antibiotics to infliximab therapy for better outcomes in patients with perianal fistulas. B Consider initiating adalimumab or certolizumab pegol for the treatment of perianal fistulas in patients with CD. B Patients with fistulizing disease, other biologics: As per AGA 2021 guidelines, consider initiating ustekinumab or vedolizumab for induction or maintenance of fistula remission in adult outpatients with CD and active perianal fistula. C As per ECCO 2020 guidelines, insufficient evidence to support the use of ustekinumab I or vedolizumab for fistula healing in patients with CD and complex perianal fistula. I Patients with fistulizing disease, thiopurines: As per ECCO 2020 guidelines, avoid using thiopurine monotherapy (azathioprine, mercaptopurine) for fistula closure in patients with CD and complex perianal fistula. D As per ACG 2018 guidelines, consider initiating thiopurines (azathioprine or 6-mercaptopurine) for the treatment of patients with fistulizing CD. B Patients with fistulizing disease (tacrolimus): consider initiating a short-term course of tacrolimus for the treatment of perianal and cutaneous fistulas in patients with CD. B Patients with fistulizing disease, antibiotics: As per AGA 2021 guidelines: https://web.pathway.md/diseases/recFEepjgYMnE2bAn 20/28 6/23/23, 3:37 AM Crohn's disease Pathway Avoid using antibiotics alone for induction of fistula remission in adult outpatients with CD and active perianal fistula without perianal abscess. D Initiate biologic agents in combination with an antibiotic for induction of fistula remission in adult outpatients with CD and active perianal fistula without perianal abscess. B As per ECCO 2020 guidelines, avoid using antibiotics alone for fistula closure in patients with CD and complex perianal fistula. D As per CAG 2019 guidelines, consider initiating antibiotic therapy for initial management to achieve symptomatic response in patients with CD and evidence of fistulizing disease. C As per ACG 2018 guidelines, consider initiating antibiotics (imidazoles) for the treatment of simple perianal fistulas. B Patients with fistulizing disease (stem cell therapy): consider offering allogeneic C or autologous adipose-derived stem cell therapy in patients with complex perianal fistulas. C Patients with fistulizing disease (seton placement): consider placing setons for the treatment of patients with perianal fistulas, and to increase the efficacy of infliximab. B Patients with fistulizing disease (fibrin glue injection): consider administering fibrin glue injection in patients with complex perianal CD. C Patients with fistulizing disease (anal fistula plug): avoid using anal fistula plugs for ano- perineal fistula closure in patients with CD, as seton removal alone is equally effective. D Patients with fistulizing disease, abscess drainage: As per AAST/WSES 2021 guidelines, perform adequate surgical drainage of a perianal abscess without searching for an associated fistula in patients with CD. B Show 3 more As per ACG 2018 guidelines: Perform prompt surgical drainage of a perianal abscess in patients with CD. E Perform drainage of abscesses (surgically or percutaneously) before initiating anti-TNF treatment. B Patients with fistulizing disease, surgical management: As per CAG 2019 guidelines: Consider obtaining surgical consultation in patients with CD and evidence of complicated fistulizing disease. C Consider referring patients with CD and evidence of fistulizing disease for surgical management if there is an inadequate symptomatic response to medical management strategies. C As per ECCO 2019 guidelines, consider performing advancement flap repair as a therapeutic option in patients with CD and complex perianal fistulas. C Show 2 more Patients with fistulizing disease (nutritional support): https://web.pathway.md/diseases/recFEepjgYMnE2bAn 21/28 6/23/23, 3:37 AM Crohn's disease Pathway Consider administering all nutritional support via the enteral route (generally as food) in patients with a distal (low ileal or colonic) fistula and low output. C Administer nutritional support by partial or exclusive parenteral nutrition in patients with a proximal fistula and/or very high output. B Patients with enterocutaneous fistula (nutritional assessment): assess patients with enterocutaneous fistula for malnutrition at the time of diagnosis. Obtain periodic nutrition assessment if malnutrition is not present at baseline as patients with fistulas have a high likelihood of becoming malnourished due to nutrient malabsorption, fluid and electrolyte losses, and sepsis. B Show 2 more Patients with enterocutaneous fistula, medical therapy: As per BSG 2019 guidelines, consider initiating immunomodulator and biological therapy for the management of patients with IBD and low volume enterocutaneous fistulas. C Show 2 more As per ACG 2018 guidelines, consider initiating infliximab for the treatment of enterocutaneous fistulas in patients with CD. B Patients with enterocutaneous fistula, surgical management: As per ASCRS 2020 guidelines, consider surgery in patients with enteric fistulas that persist despite appropriate medical therapy. B As per BSG 2019 guidelines, offer surgery to achieve symptom control in patients with IBD and high-volume fistulas. B Patients with ostomy: Monitor fluid output and urine sodium and adapt fluid input accordingly (decrease hypotonic fluids, increase saline solutions, limit hypertonic fluids), taking into account food intolerances that may enhance fluid output, in patients with IBD with severe diarrhea or a high output jejunostomy or ileostomy. B Consider administering parenteral infusions (fluid and electrolytes) in patients with ongoing high- output stomas. C Patients with pouchitis: consider offering multistrain probiotics to prevent pouchitis. C Insufficient evidence regarding prebiotics for pouchitis. C Show 2 more Patients with Clostridioides difficile infection, antibiotic therapy: As per ACG 2021 guidelines, consider administering oral vancomycin (125 mg QID for a minimum of 14 days) in patients with IBD and C. difficile infection. B As per AGA 2017 guidelines, consider administering vancomycin rather than metronidazole for the treatment of C. difficile infection in patients with IBD. C Show 2 more Patients with Clostridioides difficile infection, withholding immunosuppressive therapy: https://web.pathway.md/diseases/recFEepjgYMnE2bAn 22/28 6/23/23, 3:37 AM Crohn's disease Pathway As per ACG 2021 guidelines, do not hold immunosuppressive IBD therapy during anti-C. difficile infection treatment in the setting of disease flare, and consider escalating therapy if there is no symptomatic improvement with treatment of C. difficile infection. D As per AGA 2017 guidelines, consider postponing the escalation of corticosteroids and other immunosuppression agents during acute C. difficile infection until therapy for C. difficile infection has been initiated. Decide on withholding or continuing immunosuppression in patients with IBD with C. difficile infection on an individual basis. C Patients with Clostridioides difficile infection, fecal microbiota transplantation: As per ACG 2021 guidelines, consider performing fecal microbiota transplantation in patients with IBD and recurrent C. difficile infection. B As per HIS/BSG 2018 guidelines: Offer fecal microbiota transplantation in patients with IBD and recurrent C. difficile infection. Counsel patients about a small but recognized risk of exacerbating IBD. B Do not offer fecal microbiota transplantation for the treatment of IBD. Insufficient evidence to recommend fecal microbiota transplantation for any other gastrointestinal or non- gastrointestinal disease apart from C. difficile infection. D As per AGA 2017 guidelines, offer a referral for fecal microbiota transplantation in patients with IBD and recurrent C. difficile infection. B 11. Preventative measures Routine immunizations: provide annual vaccination against influenza in all adult patients with IBD. Administer the non-live trivalent inactivated influenza vaccine, and not the live inhaled influenza vaccine, in patients on immunosuppressive therapies and their household contacts. B Show 7 more Healthy diet: advise following the principles of healthy dietary patterns and avoidance of individual nutritional triggers. Adjust the diet accordingly if particular clinical problems are still present during the remission phase. B Show 2 more Breastfeeding: advise breastfeeding as it is the optimal food for infants and reduces the risk of IBD. B 12. Follow-up and surveillance Therapeutic drug monitoring: As per ECCO 2020 guidelines: Insufficient evidence to recommend for or against proactive therapeutic drug monitoring in patients with CD in clinical remission under anti-TNF treatment. I Insufficient evidence to recommend for or against reactive therapeutic drug monitoring in patients with CD lost response to an anti-TNF agent. I https://web.pathway.md/diseases/recFEepjgYMnE2bAn 23/28 6/23/23, 3:37 AM Crohn's disease Pathway As per AGA 2017 guidelines, consider obtaining reactive therapeutic drug monitoring to guide treatment changes in adult patients with active IBD treated with anti-TNF agents. C Show 4 more Serial laboratory assessment: Consider measuring fecal calprotectin and fecal lactoferrin as an adjunct to monitor disease activity in patients with CD. E Consider obtaining serial CRP level measurements as an adjunct to monitor disease activity and response to therapy in selected patients with CD, recognizing that this test is relatively nonspecific to CD inflammation. E Serial imaging assessment: As per ACG 2018 guidelines, consider obtaining periodic cross-sectional imaging (CT enterography, MR enterography) to monitor response to therapy in selected patients with small bowel CD. E As per AGA 2018 guidelines: Consider obtaining cross-sectional enterography in disease monitoring paradigms when small bowel disease or penetrating disease complications are present. B Prefer MR enterography over CT enterography, when possible, for estimating response to medical treatment in asymptomatic patients with CD, as its multiparametric nature permits evaluation of multiple imaging parameters reflecting inflammation and avoids radiation. B Postoperative imaging (early): Obtain any of the following modalities as the initial imaging of a suspected complication after proctectomy, coloproctectomy or colectomy with pouch or other anastomosis: MRI of the pelvis without and with IV contrast CT of the abdomen and pelvis with IV contrast CT of the pelvis with IV contrast. B Postoperative imaging (late): Obtain postoperative endoscopic monitoring at 6-12 months after surgical resection in patients with surgically-induced remission of CD not receiving pharmacological prophylaxis. B Consider obtaining postoperative endoscopic monitoring at 6-12 months after surgical resection in patients with surgically-induced remission of CD receiving pharmacological prophylaxis. C Endoscopic surveillance, ileocolonscopy: As per ASCRS 2020 guidelines, obtain endoscopic surveillance at regular intervals in patients with long-standing Crohn's colitis involving at least one-third of the colon or > 1 segment. B Show 3 more As per ACG 2018 guidelines: Do not use narrow-band imaging during colorectal neoplasia surveillance examinations for CD. D Insufficient evidence to recommend routine use of chromoendoscopy in patients with IBD undergoing surveillance colonoscopy if the endoscopist has access to high-definition white https://web.pathway.md/diseases/recFEepjgYMnE2bAn 24/28 6/23/23, 3:37 AM Crohn's disease Pathway light endoscopy. I As per ASCRS 2015 guidelines: Obtain endoscopic surveillance of the large bowel in patients with long-standing CD of the ileocolon or colon. B Perform biopsies of suspicious lesions (mass, ulcer) identified in patients with Crohn's, especially when considering small-bowel strictureplasty. B Endoscopic surveillance (video capsule endoscopy): Consider performing small bowel capsule endoscopy for the assessment of CD extent and for monitoring and guiding the "treat-to-target" strategy. C Use activity scores (such as the Lewis score and the CECDAI) to facilitate prospective small- bowel capsule endoscopy follow-up of patients for longitudinal assessment of small bowel CD and its response to medical therapy (using mucosal healing as an endpoint). B Cervical cancer surveillance: obtain annual cervical cancer screening in female patients with IBD on immunosuppressive therapy. B Skin cancer surveillance: Obtain screening for melanoma in patients with IBD independent of the use of biologic therapy. B Obtain screening for non-melanoma squamous cell cancer in patients with IBD receiving immunomodulators (6-mercaptopurine or azathioprine), particularly > 50 years of age. B 13. Quality improvement Documentation: document the type of IBD in the medical record, along with the location and level of activity. E References 1. Stephan C Bischoff, Palle Bager, Johanna Escher et al. ESPEN guideline on Clinical Nutrition in inflammatory bowel disease. Clin Nutr. 2023 Jan 13;42 3 352 379. Open 2. Adamina M, Bonovas S, Raine T et al. ECCO guidelines on therapeutics in Crohn's disease: surgical treatment. J Crohns Colitis. 2019 Nov 19. pii: jjz187. Open 3. Steinhart AH, Panaccione R, Targownik L et al. Clinical Practice Guideline for the Medical Management of Perianal Fistulizing Crohn's Disease: The Toronto Consensus. Inflamm Bowel Dis. 2019 Jan 1;25 1 1 13. Open 4. Strong S, Steele SR, Boutrous M et al. Clinical Practice Guideline for the Surgical Management of Crohn's Disease. Dis Colon Rectum. 2015 Nov;58 11 1021 36. Open 5. Amy L Lightner, Jon D Vogel, Joseph C Carmichael et al. The American Society of Colon and Rectal Surgeons Clinical Practice Guidelines for the Surgical Management of Crohn's Disease. Dis Colon Rectum. 2020 Aug;63 8 1028 1052. Open https://web.pathway.md/diseases/recFEepjgYMnE2bAn 25/28 6/23/23, 3:37 AM Crohn's disease Pathway 6. Joana Torres, Stefanos Bonovas, Glen Doherty et al. ECCO Guidelines on Therapeutics in Crohn's Disease: Medical Treatment. J Crohns Colitis. 2020 Jan 1;14 1 4 22. Open 7. Marco Pennazio, Emanuele Rondonotti, Edward J Despott et al. Small-bowel capsule endoscopy and device-assisted enteroscopy for diagnosis and treatment of small-bowel disorders: European Society of Gastrointestinal Endoscopy ESGE Guideline Update 2022. Endoscopy. 2023 Jan;55 1 58 95. Open 8. Lichtenstein GR, Loftus EV, Isaacs KL et al. ACG Clinical Guideline: Management of Crohn's Disease in Adults. Am J Gastroenterol. 2018 Apr;113 4 481 517. Open 9. Terdiman JP, Gruss CB, Heidelbaugh JJ et al. American Gastroenterological Association Institute guideline on the use of thiopurines, methotrexate, and anti-TNF biologic drugs for the induction and maintenance of remission in inflammatory Crohn's disease. Gastroenterology. 2013 Dec;145 6 1459 63. Open 10. Belinda De Simone, Justin Davies, Elie Chouillard et al. WSES AAST guidelines: management of inflammatory bowel disease in the emergency setting. World J Emerg Surg. 2021; 16 23. Open 11. Sahil Khanna, Andrea Shin, Ciar n P Kelly. Management of Clostridium difficile Infection in Inflammatory Bowel Disease: Expert Review from the Clinical Practice Updates Committee of the AGA Institute. Clin Gastroenterol Hepatol. 2017 Feb;15 2 166 174. Open 12. Francis A Farraye, Gil Y Melmed, Gary R Lichtenstein et al. ACG Clinical Guideline: Preventive Care in Inflammatory Bowel Disease. Am J Gastroenterol. 2017 Feb;112 2 241 258. Open 13. Patrick F van Rheenen, Marina Aloi, Amit Assa et al. The Medical Management of Paediatric Crohn's Disease: an ECCO ESPGHAN Guideline Update. J Crohns Colitis. 2020 Oct 7;jjaa161. Open 14. Bruining DH, Zimmermann EM, Loftus EV Jr et al. Consensus Recommendations for Evaluation, Interpretation, and Utilization of Computed Tomography and Magnetic Resonance Enterography in Patients With Small Bowel Crohn's Disease. Radiology. 2018 Mar;286 3 776 799. Open 15. Joseph D Feuerstein, Edith Y Ho, Eugenia Shmidt et al. AGA Clinical Practice Guidelines on the Medical Management of Moderate to Severe Luminal and Perianal Fistulizing Crohn's Disease. Gastroenterology. 2021 Jun;160 7 2496 2508. Open 16. Stephan C Bischoff, Johann Ockenga, Ahad Eshraghian et al. Practical guideline on obesity care in patients with gastrointestinal and liver diseases Joint ESPEN/UEG guideline. Clin Nutr. 2023 Apr 10;42 6 987 1024. Open 17. Mullish BH, Quraishi MN, Segal JP et al. The use of faecal microbiota transplant as treatment for recurrent or refractory Clostridium difficile infection and other potential indications: joint British Society of Gastroenterology BSG and Healthcare Infection Society HIS guidelines. Gut. 2018 Nov;67 11 1920 1941. Open 18. Lamb CA, Kennedy NA, Raine T et al. British Society of Gastroenterology consensus guidelines on the management of inflammatory bowel disease in adults. Gut. 2019 Dec;68 Suppl 3):s1-s106. Open 19. Nguyen GC, Loftus EV Jr, Hirano I et al. American Gastroenterological Association Institute Guideline on the Management of Crohn's Disease After Surgical Resection. Gastroenterology. 2017 Jan;152 1 271 275. Open 20. Grace L Su, Cynthia W Ko, Premysl Bercik et al. AGA Clinical Practice Guidelines on the Role of Probiotics in the Management of Gastrointestinal Disorders. Gastroenterology. 2020 Aug;159 2 697 705. Open https://web.pathway.md/diseases/recFEepjgYMnE2bAn 26/28 6/23/23, 3:37 AM Crohn's disease Pathway 21. Vanessa J Kumpf, Jose Eduardo de Aguilar-Nascimento, Jose Ignacio Diaz-Pizarro Graf et al. ASPEN FELANPE Clinical Guidelines. JPEN J Parenter Enteral Nutr. 2017 Jan;41 1 104 112. Open 22. Colleen R Kelly, Monika Fischer, Jessica R Allegretti et al. ACG Clinical Guidelines: Prevention, Diagnosis, and Treatment of Clostridioides difficile Infections. Am J Gastroenterol. 2021 Jun 1;116 6 1124 1147. Open 23. Axel U Dignass, Christoph Gasche, Dominik Bettenworth et al. European consensus on the diagnosis and management of iron deficiency and anaemia in inflammatory bowel diseases. J Crohns Colitis. 2015 Mar;9 3 211 22. Open 24. Expert Panel on Gastrointestinal Imaging: Angela D. Levy, MD, Peter S. Liu et al. ACR Appropriateness Criteria Anorectal Disease. ACR. 2021. Open 25. Feuerstein JD, Nguyen GC, Kupfer SS et al. American Gastroenterological Association Institute Guideline on Therapeutic Drug Monitoring in Inflammatory Bowel Disease. Gastroenterology. 2017 Sep;153 3 827 834. Open 26. Relling MV, Gardner EE, Sandborn WJ et al. Clinical Pharmacogenetics Implementation Consortium guidelines for thiopurine methyltransferase genotype and thiopurine dosing. Clin Pharmacol Ther. 2011 Mar;89 3 387 91. Open 27. Francis Ha, Hanan Khalil. Crohn's disease: a clinical update. 2015 Nov;8 6 352 9.2015 Nov;8 6 352 9. Open 28. E V Loftus Jr. Crohn's disease: why the disparity in mortality?. 2006 Apr;55 4 447 9.2006 Apr;55 4 447 9. Open 29. Brian Veauthier, Jaime R Hornecker. Crohn's Disease: Diagnosis and Management. Am Fam Physician. 2018 Dec 1;98 11 661 669. Open 30. Joana Torres, Saurabh Mehandru, Jean-Fr d ric Colombel et al. Crohn's disease. Lancet. 2017 Apr 29;389 10080 1741 1755. Open 31. Tun GS, Cripps S, Lobo AJ. Crohn's disease: management in adults, children and young people - concise guidance . Clin Med Lond). 2018 Jun;18 3 231 236. Open 32. E V Loftus, Jr. Crohn's disease: why the disparity in mortality?. Gut. 2006 Apr; 55 4 447 449. Open 33. Francis Ha and Hanan Khalil. Crohn s disease: a clinical update. Therap Adv Gastroenterol. 2015 Nov; 8 6 352 359. Open 34. Kathryn A. Myers, MD, EdM et al. Does This Patient Have Clubbing?. JAMA. 2001;286 3 341 347. Open 35. Anand Padmanabhan, Laura Connelly-Smith, Nicole Aqui et al. Guidelines on the Use of Therapeutic Apheresis in Clinical Practice Evidence-Based Approach from the Writing Committee of the American Society for Apheresis: The Eighth Special Issue. J Clin Apher. 2019 Jun;34 3 171 354. Open 36. Jean-Fr d ric Colombel, William J Sandborn, Paul Rutgeerts et al. Adalimumab for maintenance of clinical response and remission in patients with Crohn's disease: the CHARM trial. Gastroenterology. 2007 Jan;132 1 52 65. Open 37. Brian G Feagan, William J Sandborn, Christopher Gasink et al. Ustekinumab as Induction and Maintenance Therapy for Crohn's Disease. N Engl J Med. 2016 Nov 17;375 20 1946 1960. Open https://web.pathway.md/diseases/recFEepjgYMnE2bAn 27/28 6/23/23, 3:37 AM Crohn's disease Pathway https://web.pathway.md/diseases/recFEepjgYMnE2bAn 28/28 |
Guideline sources The following summarized guidelines for the evaluation and management of cryptococcal meningitis (CM) are prepared by our editorial team based on guidelines from the Infectious Diseases Society of America (IDSA/CDC/NIH/HIVMA 2023), the World Health Organization (WHO 2022), and the Infectious Diseases Society of America (IDSA 2010). 1 2 3 Guidelines 1. Screening and diagnosis Indications for screening: As per CDC 2023 guidelines: Obtain routine surveillance testing for serum cryptococcal antigen in patients with newly diagnosed human immunodeficiency virus with no overt clinical signs of meningitis, if the CD4 counts are 100 cells/mm , especially if 50 cells/mm . B Obtain prompt CSF evaluation for CNS infection in patients with a positive test (BIII), particularly when the serum cryptococcal antigen lateral flow assay titer is 1:160. B https://web.pathway.md/diseases/rec8qZXQuHUGCY1CX 1/8 6/23/23, 3:37 AM Cryptococcal meningitis Pathway As per WHO 2022 guidelines, consider screening for plasma, serum or whole-blood cryptococcal antigen as the optimal approach for guiding resources in a public health approach and as the preferred approach for identifying infection when managing 10 years old patients presenting with advanced human immunodeficiency virus disease. E Show 2 more 2. Diagnostic investigations Antigen testing: Obtain rapid serum, plasma or whole-blood cryptococcal antigen testing as the preferred diagnostic approach if both access to a cryptococcal antigen assay and rapid results (< 24 hours) are available in settings without immediate access to lumbar puncture or when lumbar puncture is clinically contraindicated. B Consider referring for further investigation and treatment promptly if a cryptococcal antigen assay is not available and/or rapid access to results is not ensured in settings without immediate access to lumbar puncture or when lumbar puncture is clinically contraindicated. B 3. Diagnostic procedures Lumbar puncture: As per WHO 2022 guidelines, perform s prompt lumbar puncture with measurement of CSF opening pressure and rapid cryptococcal antigen assay as the preferred diagnostic approach in adult, adolescent B and pediatric patients with human immunodeficiency virus suspected of having a first episode of CM. B Show 2 more As per IDSA 2010 guidelines, consider performing a prompt baseline lumbar puncture to assess CSF pressure at baseline. C Show 2 more 4. Medical management Antifungal therapy, induction, HIV-positive: As per IDSA/HIVMA/CDC/NIH 2023 guidelines, initiate IV amphotericin B in combination with oral flucytosine as induction therapy in patients with CM. A Show 5 more As per WHO 2022 guidelines, administer a single high-dose (10 mg/kg) of liposomal amphotericin B and initiate flucytosine (100 mg/kg/day divided into 4 doses per day) and fluconazole (1,200 mg/daily in adult patients and 12 mg/kg/day in pediatric and adolescent patients up to a maximum of 800 mg daily) for 14 days as the first-line induction regimen for the treatment of patients with CM. B Show 5 more https://web.pathway.md/diseases/rec8qZXQuHUGCY1CX 2/8 6/23/23, 3:37 AM Cryptococcal meningitis Pathway As per IDSA 2010 guidelines, initiate amphotericin B deoxycholate 0.7-1.0 mg/kg/day IV plus flucytosine 100 mg/kg/day PO in 4 divided doses (consider initiating IV formulations in severe cases and in patients without oral intake where the preparation is available) for at least 2 weeks as first-line induction therapy in human immunodeficiency virus-infected patients with CM. A Show 2 more Antifungal therapy, induction (organ transplant recipients): initiate liposomal amphotericin B (3-4 mg/kg/day IV) or amphotericin B lipid complex (5 mg/kg/day IV) plus flucytosine (100 mg/kg/day in 4 divided doses) for at least 2 weeks as induction therapy in organ transplant recipients with CM. B Show 2 more Antifungal therapy, induction (HIV-negative, non-transplant recipients): initiate amphotericin B deoxycholate 0.7-1.0 mg/kg/day IV plus flucytosine 100 mg/kg/day PO in 4 divided doses for at least 4 weeks as induction therapy in human immunodeficiency virus-negative, non-transplant patients with CM. B Show 6 more Antifungal therapy, consolidation, HIV-positive: As per IDSA/HIVMA/CDC/NIH 2023 guidelines, initiate fluconazole 800 mg/day as consolidation therapy. A Show 7 more As per WHO 2022 guidelines, initiate fluconazole (800 mg/day for adult patients and 6-12 mg/kg/day for pediatric and adolescent patients up to a maximum of 800 mg/day) for the consolidation phase (for 8 weeks following the induction phase). B As per IDSA 2010 guidelines: Initiate fluconazole 400 mg (6 mg/kg) per day PO for a minimum of 8 weeks following an induction therapy with amphotericin B and flucytosine as first-line consolidation therapy in human immunodeficiency virus-infected patients with CM. A Initiate fluconazole 800 mg/day PO for a minimum of 8 weeks following an induction therapy with amphotericin B and fluconazole as consolidation therapy in human immunodeficiency virus-infected patients with CM. B Antifungal therapy, consolidation (organ transplant recipients): initiate fluconazole 400-800 mg (6-12 mg/kg) per day PO for 8 weeks following an induction therapy with liposomal amphotericin B or amphotericin B lipid complex plus flucytosine as consolidation therapy in organ transplant recipients with CM. B Antifungal therapy, consolidation (HIV-negative, non-transplant recipients): Initiate fluconazole 400 mg/day for 8 weeks following an induction therapy with amphotericin B and flucytosine as consolidation therapy in human immunodeficiency virus-negative, non- transplant patients with CM. B Initiate fluconazole 800 mg (12 mg/kg) per day PO for 8 weeks following a 2-week induction therapy with amphotericin B and flucytosine as consolidation therapy in patients at low risk for therapeutic failure (an early diagnosis by history, no uncontrolled underlying disease or https://web.pathway.md/diseases/rec8qZXQuHUGCY1CX 3/8 6/23/23, 3:37 AM Cryptococcal meningitis Pathway immunocompromised state and excellent clinical response to initial 2-week antifungal combination course). B Antifungal therapy, maintenance, HIV-positive: As per IDSA/HIVMA/CDC/NIH 2023 guidelines, initiate fluconazole 200 mg/day as maintenance therapy and continue until at least 1 year from initiation of antifungal therapy. A Show 4 more As per WHO 2022 guidelines, initiate fluconazole (200 mg/day for adult patients and 6 mg/kg/day for adolescent and pediatric patients) for the maintenance phase until immune reconstitution (CD4 > 200 cells/mm ) and suppression of viral loads on ART. A As per IDSA 2010 guidelines, initiate the following regimens as maintenance therapy in human immunodeficiency virus-infected patients with CM: fluconazole 200 mg/day PO A traconazole 200 mg BID PO with drug-level monitoring B amphotericin B deoxycholate 1 mg/kg/week IV in azole-intolerant patients, as it is less effective than azoles and is associated with IV catheter-related infections. B Antifungal therapy, maintenance (organ transplant recipients): Continue fluconazole 200-400 mg/day PO for at least 6-12 months as maintenance therapy in organ transplant recipients with CM. B Reduce immunosuppressants sequentially or in a stepwise manner, with consideration of lowering the corticosteroid dose first. B Antifungal therapy, maintenance (HIV-negative, non-transplant recipients): initiate fluconazole 200 mg (3 mg/kg) per day PO for 6-12 months as maintenance therapy in human immunodeficiency virus-negative, non-transplant patients with CM. B Antiretroviral therapy: As per CDC 2023 guidelines, defer initiation of ART 4-6 weeks after antifungal agents are started. A As per WHO 2022 guidelines, do not initiate immediate ART in adult, (Strong recommendation; low-certainty evidence) adolescent and pediatric patients with human immunodeficiency virus and CM because of the risk of increased mortality and defer it 4-6 weeks from the initiation of antifungal treatment. D As per IDSA 2010 guidelines: Initiate HAART 2-10 weeks after commencement of initial antifungal treatment. B Consider discontinuing suppressive therapy during HAART in patients with a CD4 cell count > 100 cells/ L and an undetectable or very low human immunodeficiency virus RNA level sustained for 3 months, minimum of 12 months of antifungal therapy. C Consider resuming maintenance therapy if the CD4 cell count decreases to < 100 cells/ L. C Diuretics and corticosteroids: As per CDC 2023 guidelines: https://web.pathway.md/diseases/rec8qZXQuHUGCY1CX 4/8 6/23/23, 3:37 AM Cryptococcal meningitis Pathway Do not use mannitol or corticosteroids for elevated ICP because of the ineffectiveness. D Do not use acetazolamide for elevated ICP because it is ineffective and may exacerbate hyperchloremic acidosis from amphotericin B. D Do not use corticosteroids during induction therapy for ICP control in patients with human immunodeficiency virus-associated CM unless for immune reconstitution inflammatory syndrome. D As per WHO 2022 guidelines, do not use adjunctive corticosteroids routinely during the induction phase in treating adult, adolescent D and pediatric patients with human immunodeficiency virus-associated CM. D As per IDSA 2010 guidelines: Do not use routine mannitol for ICP. D Avoid using acetazolamide and corticosteroids (unless part of immune reconstitution inflammatory syndrome treatment) to control increased ICP. D Management of medication adverse effects: consider administering acetaminophen 650 mg and diphenhydramine 25-50 mg or hydrocortisone 50-100 mg 30 minutes before the infusion of amphotericin to reduce the severity of infusion reactions in patients with severe amphotericin infusion-related adverse reactions. C Show 4 more Management of treatment failure: As per CDC 2023 guidelines, switch to amphotericin B, with or without flucytosine, in patients not responding to induction with fluconazole monotherapy. B Show 4 more As per IDSA 2010 guidelines, take adequate measures to improve immune status (such as decrease immunosuppressants and introduce HAART) and optimize management of increased ICP. B Show 8 more Management of relapse: As per CDC 2023 guidelines, confirm CSF culture sterility at the completion of re-induction therapy for treatment failure or relapse. B Show 2 more As per IDSA 2010 guidelines, restart induction phase therapy as in patients with persistence disease. B Show 2 more 5. Therapeutic procedures CSF drainage: As per CDC 2023 guidelines, take measures to decrease ICP in all patients with confusion, blurred vision, papilledema, lower extremity clonus, or other neurologic signs indicative of increased ICP. Perform drainage of CSF via lumbar puncture for initial management. B https://web.pathway.md/diseases/rec8qZXQuHUGCY1CX 5/8 6/23/23, 3:37 AM Cryptococcal meningitis Pathway Show 4 more As per IDSA 2010 guidelines, perform CSF drainage by lumbar puncture to relieve ICP (reduce the opening pressure by 50% if it is extremely high or to a normal pressure of 20 cm of CSF) if the CSF pressure is 25 cm and there are symptoms of increased ICP during induction therapy. B Show 2 more Ventriculoperitoneal shunting: consider placing permanent ventriculoperitoneal shunts only if the patient is receiving or has received appropriate antifungal therapy and if more conservative measures to control increased ICP have failed. C Show 2 more 6. Specific circumstances Pediatric patients: initiate amphotericin B deoxycholate 1 mg/kg/day IV plus flucytosine 100 mg/kg/day PO in 4 divided doses for 2 weeks (follow the treatment length schedule for adults in non-human immunodeficiency virus-infected, non-transplant patients) followed by fluconazole 10- 12 mg/kg/day PO for 8 weeks as induction and consolidation therapy in pediatric patients with CNS disease. B Show 2 more Pregnant patients: As per CDC 2023 guidelines, consider using flucytosine during pregnancy only when the benefits outweigh the risks to the fetus and only in the third trimester. B Show 2 more As per IDSA 2010 guidelines, initiate amphotericin B deoxycholate or lipid formulations of amphotericin B with or without flucytosine in pregnant patients with CNS disease. B Consider using flucytosine in a relationship to benefit versus risk because it is a category C drug for pregnancy. Show 2 more Patients with immune reconstitution inflammatory syndrome: Do not alter direct antifungal therapy in patients with immune reconstitution inflammatory syndrome. D Show 3 more Do not alter direct antifungal therapy in patients with immune reconstitution inflammatory syndrome. D Show 3 more Patients with cerebral cyptococcomas: initiate amphotericin B deoxycholate 0.7-1 mg/kg/day IV, liposomal amphotericin B 3-4 mg/kg/day IV or amphotericin B lipid complex 5 mg/kg/day IV plus flucytosine 100 mg/kg/day PO in 4 divided doses for at least 6 weeks as induction therapy in patients with cerebral cyptococcomas. B Show 4 more https://web.pathway.md/diseases/rec8qZXQuHUGCY1CX 6/8 6/23/23, 3:37 AM Cryptococcal meningitis Pathway Patients with Cryptococcus gattii infection: Initiate the same induction, consolidation and suppressive therapy in patients with CNS disease due to Cryptococcus gattii as for Cryptococcus neoformans. B Pay more diagnostic attention by radiology and follow-up examinations for cryptococcomas/hydrocephalus due to Cryptococcus gattii than that due to Cryptococcus neoformans, but recognize that the management principles are the same. B Resource-limited healthcare environments: As per CDC 2023 guidelines, prefer 1-week amphotericin B deoxycholate with flucytosine regimen followed by high-dose fluconazole as induction and consolidation therapy in resource- limited settings. B As per IDSA 2010 guidelines, initiate amphotericin B deoxycholate 1 mg/kg/day IV for 2 weeks or amphotericin deoxycholate 0.7 mg/kg/day IV plus fluconazole 800 mg/day PO for 2 weeks followed by consolidation therapy with fluconazole 800 mg/day PO for 8 weeks as induction and consolidation therapy in patients with CNS disease in areas where flucytosine is not available. A Show 5 more 7. Preventative measures Primary prophylaxis, HIV-positive: As per IDSA/HIVMA/CDC/NIH 2023 guidelines, do not initiate primary prophylaxis in human immunodeficiency virus-positive patients in the United States in the absence of a positive serum cryptococcal antigen test because of the relative infrequency of cryptococcal disease, lack of survival benefit associated with prophylaxis, possibility of drug-drug interactions, potential development of antifungal drug resistance, and costs. D Show 4 more As per WHO 2022 guidelines: Initiate fluconazole primary prophylaxis in adult and adolescent patients with human immunodeficiency virus having a CD4 cell count < 100 cells/mm when cryptococcal antigen screening is not available. B Consider initiating fluconazole primary prophylaxis at a higher CD4 cell count threshold of < 200 cells/mm . C As per IDSA 2010 guidelines, do not initiate routine primary antifungal prophylaxis for cryptococcosis in patients with human immunodeficiency virus in the United States and Europe. D Show 2 more 8. Follow-up and surveillance https://web.pathway.md/diseases/rec8qZXQuHUGCY1CX 7/8 6/23/23, 3:37 AM Cryptococcal meningitis Pathway Assessment of treatment response: do not monitor serum or CSF cryptococcal antigen titers for determination of initial response to therapy. D References 1. Perfect JR, Dismukes WE, Dromer F et al. Clinical practice guidelines for the management of cryptococcal disease: 2010 update by the infectious diseases society of america. Clin Infect Dis. 2010 Feb 1;50 3 291 322. Open 2. Panel on Opportunistic Infections in Adults and Adolescents with HIV. Guidelines for the Prevention and Treatment of Opportunistic Infections in Adults and Adolescents with HIV. Department of Health and Human Services. 2023. Open 3. No authors listed. Guidelines for Diagnosing, Preventing and Managing Cryptococcal Disease Among Adults, Adolescents and Children Living with HIV. Geneva: World Health Organization; 2022. Open 4. Saag MS, Graybill RJ, Larsen RA et al. Practice guidelines for the management of cryptococcal disease. Infectious Diseases Society of America. Clin Infect Dis. 2000 Apr;30 4 710 8. Open 5. Joseph N Jarvis, David S Lawrence, David B Meya et al. Single-Dose Liposomal Amphotericin B Treatment for Cryptococcal Meningitis. N Engl J Med. 2022 Mar 24;386 12 1109 1120. Open https://web.pathway.md/diseases/rec8qZXQuHUGCY1CX 8/8 |
Guideline sources The following summarized guidelines for the evaluation and management of cryptorchidism are prepared by our editorial team based on guidelines from the European Association of Urology (EAU 2023; 2022), the European Thyroid Association (ETA 2018), the Canadian Urological Association (CUA 2017), and the American Urological Association (AUA 2014). 1 2 3 4 5 6 6 6 7 Definition Cryptorchidism, also known as undescended testis, is a common congenital abnormality among males that is characterized by the failure of one or both testes to permanently descend. 6 Epidemiology Cryptorchidism is caused due to a disruption in any phase of testicular descent triggered by hereditary, hormonal, anatomical, environmental, or social factors. 6 Disease course The disruption in any phase of testicular descent results in cryptorchidism, which causes an increased chance of inguinal hernia, torsion, and trauma that leads to psychological distress in parents. It may also lead to reduced fertility and testicular cancer in adult males. 6 Prognosis and risk of recurrence https://web.pathway.md/diseases/recR13KRftHx2eXVw 1/5 6/23/23, 3:37 AM Cryptorchidism Pathway g Orchiectomy for ASA 3 and ASA 4 is associated with 0.49% and 3.31% of mortality risk. 7 Guidelines 1. Diagnostic investigations Clinical history and physical examination: obtain a gestational history as part of the initial evaluation of boys with suspected cryptorchidism. B Evaluation for disorders of sex development: As per EAU 2022 guidelines, evaluate male neonates with bilateral non-palpable testes for possible disorders of sex development. B As per CUA 2017 guidelines, do not obtain routine karyotype or genetic testing in patients with cryptorchidism. D Show 2 more As per AUA 2014 guidelines, assess for the possibility of a disorder of sex development in patients with cryptorchidism and severe hypospadias. B Hormone testing: measure anti-M llerian hormone levels, and consider additional hormone testing, to evaluate for anorchia, in boys with bilateral, nonpalpable testes who do not have congenital adrenal hyperplasia. B Diagnostic imaging: As per CUA 2017 guidelines, do not obtain imaging as a standard adjunct to preoperative assessment of pediatric patients with cryptorchidism, as it is not cost-effective, and may delay referral and surgical treatment. D As per AUA 2014 guidelines, avoid ultrasound or other imaging modalities in the evaluation of boys with cryptorchidism prior to referral, as these studies rarely assist in decision making. D Evaluation for central hypothyroidism: obtain screening for central hypothyroidism in infants with signs of congenital hypopituitarism, such as micropenis with undescended testes. A 2. Medical management Hormonal therapy: As per EAU 2022 guidelines: Do not offer hormonal therapy in patients with unilateral cryptorchidism, as it is of no benefit for future paternity. D Offer GnRH analogs at a typical dose of 1.2 mg/day in 3 divided doses for 4 weeks to preserve the fertility potential in patients with bilateral cryptorchidism. B As per CUA 2017 guidelines, do not offer hormonal therapy as first-line therapy in patients with cryptorchidism. D https://web.pathway.md/diseases/recR13KRftHx2eXVw 2/5 6/23/23, 3:37 AM Cryptorchidism Pathway As per AUA 2014 guidelines, avoid hormonal therapy to induce testicular descent, given low response rates and lack of evidence for long-term efficacy. D 3. Surgical interventions Orchidopexy, pre-pubertal: As per EAU 2022 guidelines, perform surgical orchidolysis and orchidopexy before the age of 12 months and by 18 months at the latest. B Show 2 more As per CUA 2017 guidelines, perform orchidopexy between 6 and 18 months of age. B Show 4 more As per AUA 2014 guidelines, perform scrotal or inguinal orchidopexy in prepubertal boys with palpable cryptorchid testes. B Orchidopexy (post-pubertal): consider offering unilateral or bilateral orchidopexy, if technically feasible, in patients with unilateral or bilateral undescended testis with biochemical hypogonadism and/or spermatogenic failure (infertility). C Surgical or laparoscopic exploration: As per EAU 2022 guidelines, perform a diagnostic laparoscopy to locate an intra-abdominal testicle. A As per CUA 2017 guidelines, consider performing laparoscopic exploration in uncertain cases or when tissue analysis is not consistent with atrophic testicular tissue. C As per AUA 2014 guidelines, consider performing surgery within the next year in the absence of spontaneous testicular descent by 6 months (corrected for gestational age). B Orchiectomy: As per EAU 2023 guidelines, offer orchidectomy in adult patients with unilateral undescended testis and normal hormonal function/spermatogenesis. A As per CUA 2017 guidelines: Consider performing orchiectomy in < 50 years old postpubertal patients with hypotrophic/atrophic undescended testicles. C Offer orchiectomy as an option in patients with unilateral intra-abdominal or inguinal hypotrophic testes identified after puberty. B As per AUA 2014 guidelines, consider performing orchiectomy in patients with a normal contralateral testis meeting 1 of the following criteria: very short testicular vessels and vas deferens dysmorphic or very hypoplastic affected testis postpubertal age. C 4. Patient education https://web.pathway.md/diseases/recR13KRftHx2eXVw 3/5 6/23/23, 3:37 AM Cryptorchidism Pathway General counseling: As per EAU 2022 guidelines, inform the patient/caregivers about: the increased risk of later malignancy with an undescended testis in post-pubertal or older age; discuss removal in case of contralateral normal testis in a scrotal position B the increased risk of later malignancy with a failed or delayed orchidopexy; the earlier the treatment, the lower the risk of impaired fertility and testicular cancer B the reduced rates of fertility with unilateral undescended testis B the reduced rates of fertility and paternity with bilateral undescended testes. B As per CUA 2017 guidelines, inform the patient/caregivers that only one-third to two-thirds of males with bilateral cryptorchidism will be able to father a child. B As per AUA 2014 guidelines, counsel patients with a history of cryptorchidism and/or monorchidism and their parents regarding potential long-term risks, including infertility and testicular cancer. B 5. Preventative measures Prophylatic contralateral orchidopexy: decide on performing prophylactic orchidopexy based on informed discussion of options with the patient parents or legal guardian. B 6. Follow-up and surveillance Indications for specialist referral: refer infants with a history of cryptorchidism detected at birth who do not have spontaneous testicular descent by six months to an appropriate surgical specialist for timely evaluation. B Follow-up: As per EAU 2022 guidelines, obtain close follow-up on a yearly basis until puberty in patients with retractile testes instead of offering medical or surgical treatment. B As per CUA 2017 guidelines, consider offering observation in > 50 years old patients with hypotrophic/atrophic undescended testicles. C References 1. Kolon TF, Herndon CD, Baker LA et al. Evaluation and treatment of cryptorchidism: AUA guideline. J Urol. 2014 Aug;192 2 337 45. Open 2. Persani L, Brabant G, Dattani M et al. 2018 European Thyroid Association ETA Guidelines on the Diagnosis and Management of Central Hypothyroidism. Eur Thyroid J. 2018 Oct;7 5 225 237. Open 3. Luis H Braga, Armando J Lorenzo, Rodrigo L P Romao. Canadian Urological Association-Pediatric Urologists of Canada CUA PUC guideline for the diagnosis, management, and followup of cryptorchidism. Can Urol Assoc J. 2017 Jul;11 7 E251 E260. Open https://web.pathway.md/diseases/recR13KRftHx2eXVw 4/5 6/23/23, 3:37 AM Cryptorchidism Pathway 4. Lisette A 't Hoen, Guy Bogaert, Christian Radmayr et al. EAU guidelines on Paediatric Urology. EAU. 2022 Mar. Open 5. A. Salonia, C. Bettocchi, J. Carvalho et al. EAU Guidelines on Sexual and Reproductive Health. EAU. 2023. Open 6. Luis H Braga, Armando J Lorenzo. Cryptorchidism: A practical review for all community healthcare providers. Jan-Feb 2017;11 1 2Suppl1 S26 S32.Jan-Feb 2017;11 1 2Suppl1 S26 S32. Open 7. Ankur Shah, Paul J Feustel, Jennifer Knuth et al. An updated mortality risk analysis of the post-pubertal undescended testis. 2018 Jul 24;13 1 E1 E6.2018 Jul 24;13 1 E1 E6. Open 8. American Urological Association. Choosing Wisely AUA recommendations. Choosing Wisely. 2017. Open 9. Ankur Shah, Paul J. Feustel, Jennifer Knuth et al. An updated mortality risk analysis of the post-pubertal undescended testis. Can Urol Assoc J. 2019 Jan; 13 1 E1 E6. Open 10. Luis H. Braga and Armando J. Lorenzo. Cryptorchidism: A practical review for all community healthcare providers. Can Urol Assoc J. 2017 Jan-Feb; 11 1 2Suppl1 S26 S32. Open https://web.pathway.md/diseases/recR13KRftHx2eXVw 5/5 |
Guideline sources The following summarized guidelines for the evaluation and management of Cushing's syndrome (CS) are prepared by our editorial team based on guidelines from the American Association of Endocrine Surgeons (AAES 2022), the European Society of Endocrinology (ESE 2021), and the Endocrine Society (ES 2020; 2015; 2008). 1 2 3 4 5 6 6 6 6 Definition CS is a rare disorder resulting from chronic exposure to excess glucocorticoids characterized by obesity, moon face, facial plethora, and hypertension. 6 Epidemiology CS is caused by ACTH production from a pituitary adenoma (Cushing's disease; 75-80%), ACTH production from nonpituitary tumors (ectopic ACTH syndrome; 15-20%), and corticotropin-releasing hormone (CRH)-producing tumors (<1%). 6 https://web.pathway.md/diseases/recTgTOpnhjZgalO3 1/8 6/23/23, 3:37 AM Cushing's syndrome Pathway Disease course Glucocorticoid excess results in CS, which causes clinical manifestations of obesity, moon face, supraclavicular/dorsocervical fat pads, hirsutism, alopecia, facial plethora, violaceous striae, acne, easy bruising, menstrual irregularity, decreased libido, emotional lability, depression, psychosis, cognitive dysfunction, muscle weakness, muscular atrophy, osteopenia, fractures, decreased linear growth in children, hypertension, glucose intolerance, hyperlipidemia, hepatic steatosis, and nephrolithiasis. 6 Prognosis and risk of recurrence CS is associated with a standard mortality ratio of 3.7 (95% CI 2.3-5.3). 6 Calculator Diagnostic criteria for Cushing's Guidelines 1. Screening and diagnosis Indications for testing: test for CS in patients with an adrenal adenoma. B Show 3 more 2. Diagnostic investigations Corticosteroid exposure history: obtain a thorough drug history to exclude iatrogenic CS (due to excessive exogenous corticosteroid exposure) prior to undertaking further diagnostic investigations. A Initial laboratory testing: Obtain one of the following tests to evaluate for CS, based on its suitability for a given patient: urinary free cortisol late-night salivary cortisol 1-mg overnight dexamethasone suppression test longer low-dose dexamethasone suppression test. B Subsequent laboratory testing: confirm abnormal results on initial laboratory testing using a different test drawn from the recommended initial tests (urine free cortisol, late-night salivary cortisol, 1-mg overnight dexamethasone suppression test, or longer low-dose dexamethasone suppression test). B https://web.pathway.md/diseases/recTgTOpnhjZgalO3 2/8 6/23/23, 3:37 AM Cushing's syndrome Pathway Show 2 more Adrenal gland imaging: consider evaluating patients with an adrenal mass that has a radiodensity of 10 Hounsfield on noncontrast CT scan for malignancy, using further imaging. C Dexamethasone-CRH test: Consider obtaining the dexamethasone-CRH test only in specific clinical situations. B The dexamethasone-CRH test can be useful in patients with equivocal results for urinary free cortisol. Midnight serum cortisol test: Consider obtaining the midnight serum cortisol test only in specific situations. B The midnight serum cortisol test may be useful in the following situations: patients with a high clinical index of suspicion of CS, but normal urinary free cortisol and dexamethasone suppression testing patients with a low clinical index of suspicion of CS, but abnormal dexamethasone suppression testing and mildly elevated urinary free cortisol patients who fail to suppress on dexamethasone testing due to anticonvulsant medication. Desmopressin test: consider avoiding the use of the desmopressin test, except in research studies, until additional data validate its utility. D Evaluation of cardiovascular risks: obtain cardiovascular risk assessment and initiate treatment in adult patients with cured CS as in the general population. B Show 2 more 3. Medical management General principles: perform surgical resection of any primary lesion identified as the cause of cortisol excess (pituitary, ectopic or adrenal) as the first-line and definitive treatment for CS, unless surgery is not possible or is unlikely to significantly reduce corticosteroid excess. A Show 2 more Indications for treatment: treat patients with overt CS by normalizing cortisol levels, or blocking the action of cortisol at its receptors, in order to eliminate the signs and symptoms of CS. B Show 2 more Corticosteroid antagonists: consider initiating a corticosteroid antagonist (mifepristone) in patients with diabetes or glucose intolerance who are not surgical candidates or who have persistent disease after transsphenoidal selective adenomectomy. C Pituitary-directed therapy: consider pituitary-directed medical treatments (cabergoline, pasireotide) in patients with Cushing's disease who are not surgical candidates or who have persistent disease after transsphenoidal surgery. C Steroidogenesis inhibitors: Initiate steroidogenesis inhibitors (such as ketoconazole, metyrapone, mitotane, etomidate) in the following clinical scenarios: https://web.pathway.md/diseases/recTgTOpnhjZgalO3 3/8 6/23/23, 3:37 AM Cushing's syndrome Pathway as second-line treatment after transsphenoidal surgery in patients with Cushing's disease, either with or without RT/radiosurgery as primary treatment of ectopic ACTH secretion in patients with occult or metastatic ectopic ACTH secretion as adjunctive treatment to reduce cortisol levels in adrenocortical carcinoma. B Management of ectopic ACTH syndrome: consider targeted therapies (octreotide, cabergoline, vandetanib, sorafenib) to treat ectopic ACTH syndrome. C Statin therapy: consider initiating statin therapy as adjunct to lifestyle modification, to reduce cardiovascular risk, irrespective of the cardiovascular risk score, in adult patients with persistent endogenous CS. C 4. Therapeutic procedures Radiotherapy: Consider radiation therapy / radiosurgery in patients with Cushing's disease who have failed transsphenoidal surgery or display evidence of disease recurrence. C Confirm that medical therapy is effective in normalizing cortisol prior to initiating radiation therapy for Cushing's disease, given that a response to medical therapy is required for disease control while awaiting the effect of radiation. B 5. Perioperative care Perioperative thromboprophylaxis: consider administering perioperative prophylaxis for VTE in patients with CS undergoing surgery. C Perioperative sodium monitoring: measure serum sodium frequently during the first 5-14 days after transsphenoidal surgery. B 6. Surgical interventions Adrenal gland resection, adrenal gland disease: As per AAES 2022 guidelines: Consider performing unilateral laparoscopic adrenalectomy in patients with bilateral macronodular hyperplasia with CS as an attempt to achieve biochemical remission of hypercortisolism without causing permanent adrenal insufficiency. C Administer empirical postoperative corticosteroid replacement therapy in all patients with overt CS after unilateral adrenalectomy. Obtain morning cortisol or corticotropin stimulation testing on the first postoperative day to determine the need for corticosteroid replacement therapy in patients with mild autonomous cortisol secretion. B As per ES 2015 guidelines, perform surgical resection as the treatment of choice for CS in patients with unilateral cortisol-secreting adrenal disease. B https://web.pathway.md/diseases/recTgTOpnhjZgalO3 4/8 6/23/23, 3:37 AM Cushing's syndrome Pathway Show 2 more Adrenal gland resection, occult ACTH secreting-tumors: As per AAES 2022 guidelines, perform laparoscopic adrenalectomy for anticipated significant improvements in cardiometabolic comorbidities in patients with mild autonomous cortisol secretion secondary to a unilateral adenoma. B Show 2 more As per ES 2015 guidelines, consider performing bilateral adrenalectomy for occult or metastatic ectopic ACTH secretion, or as a life-preserving emergency treatment in patients with very severe ACTH-dependent disease who cannot be promptly controlled by medical therapy. C Transsphenoidal selective adenomectomy: perform transsphenoidal selective adenomectomy as the treatment of choice for Cushing's disease in patients who are fit for surgery. A Ectopic ACTH-secreting tumor resection: perform surgical resection (with node dissection as appropriate) in patients with ectopic ACTH-secreting tumors. A 7. Specific circumstances Pregnant patients, evaluation: As per ESE 2021 guidelines: Consider obtaining testing for a new diagnosis of Cushing's disease during pregnancy only in case of high clinical suspicion. C Obtain neuro-ophthalmologic evaluation in pregnant patients with pituitary adenomas impinging visual pathways or in case of suspected tumor progression or pituitary apoplexy. Obtain MRI without contrast in case of symptoms of tumor progression or apoplexy. B As per ES 2008 guidelines: Assess urine free cortisol as the initial diagnostic test in pregnant patients with suspected CS. B Do not obtain dexamethasone testing in the initial evaluation of pregnant patients with suspected CS. D Pregnant patients (management): manage pregnant patients with active or medically treated Cushing's disease by a multidisciplinary team expert in high-risk pregnancies. B Show 3 more Pregnant patients (monitoring): monitor pregnant patients with a known pituitary adenoma, particularly in large sizes (> 1 cm) and with Cushing's disease, by an endocrinologist and an advanced nurse practitioner where relevant. Decide on the frequency based on the underlying condition and individualized needs. B Pregnant patients (delivery and breastfeeding): consider providing standard obstetrical care with close maternal and fetal surveillance in pregnant patients with pituitary adenomas. C Show 2 more https://web.pathway.md/diseases/recTgTOpnhjZgalO3 5/8 6/23/23, 3:37 AM Cushing's syndrome Pathway Patients with suspected cyclic Cushing's syndrome: consider using urinary free cortisol or midnight salivary cortisol, rather than performing a dexamethasone suppression test, as the diagnostic tests of choice in patients suspected of having cyclic CS. C Patients with renal failure: consider using the 1-mg overnight dexamethasone suppression test, rather than urinary free cortisol, as the diagnostic test of choice for in patients with severe renal failure who are suspected of having CS. C Patients with epilepsy: Use measurements of cortisol in blood, saliva, or urine as the diagnostic tests of choice in patients with suspected CS who are receiving antiepileptic drugs known to enhance dexamethasone clearance. B Avoid the use of dexamethasone testing in patients receiving antiepileptic drugs known to enhance dexamethasone clearance. D Patients with life-threatening complications: treat hypercortisolism urgently (within 24-48 hours) in patients with life-threatening complications of CS, including cardiovascular, infectious, or thromboembolic complications, as well as acute psychosis. B 8. Patient education General counseling: Counsel patients on the nature of their disease, available treatment options, and what to expect after remission. B Provide education about adrenal insufficiency to hypocortisolemic patients. A Preconception counseling: advise patients with active CS not to get pregnant. B Show 5 more 9. Preventative measures Immunizations: provide age-appropriate immunizations to patients with CS - particularly influenza, Herpes zoster, and pneumococcal immunizations - due to an increased risk of infection. B 10. Follow-up and surveillance Indications for specialist referral: refer patients with a high pretest probability of CS in whom biochemical testing is negative for further evaluation by an endocrinologist. B Serial clinical assessment: manage the specific comorbidities associated with CS (including cardiovascular risk factors, osteoporosis and psychiatric symptoms) on a lifelong basis, until resolution. B https://web.pathway.md/diseases/recTgTOpnhjZgalO3 6/8 6/23/23, 3:37 AM Cushing's syndrome Pathway Serial laboratory assessment: obtain testing for recurrent disease throughout life, except in patients who underwent resection of an adrenal adenoma with a CT density of < 10 HUs. B Serial imaging assessment: Obtain a pituitary MRI within 1-3 months of surgery in patients who have undergone successful transsphenoidal selective adenomectomy. B Obtain follow-up tests for cardiac myxoma and other associated disease (testicular tumors, acromegaly, thyroid lesions) on a lifelong basis in patients with Carney complex. A Serial endocrine assessment: measure free T4 and prolactin within 1-2 weeks of surgery, to evaluate for overt hypopituitarism. B Show 2 more Management of recurrent disease: provide an individualized management approach in patients with recurrent disease, based upon whether the postoperative serum cortisol values categorize the patient's condition as hypocortisolism, hypercortisolism, or eucortisolism. B Likelihood Ratios Pertinent positives The following findings increase the probability of Cushing's syndrome in adults. 1 1 1 Finding LR+ Value Increased urine free cortisol 15.7 Increased saliva late-night salivary cortisol (> 7.5 g/dL) 13.1 Abnormal overnight dexamethasone suppression test (plasma cortisol < 1.8 g/dL at 48h after 2 mg dex) 11.5 Abnormal low-dose dexamethasone suppression test (cortisol < 1.4 mg/dL at 12 h after 1 mg dex) 3 Show 1 more Pertinent negatives The following findings decrease the probability of Cushing's syndrome in adults. 1 1 1 Finding LR- Value Normal low-dose dexamethasone suppression test (cortisol 1.4 mg/dL at 12 h after 1 mg dex) 0.01 urine free cortisol not increased 0.1 saliva late-night salivary cortisol not increased ( 7.5 g/dL) 0.1 Normal overnight dexamethasone suppression test (plasma cortisol 1.8 g/dL at 48h after 2 mg dex) 0.1 https://web.pathway.md/diseases/recTgTOpnhjZgalO3 7/8 6/23/23, 3:37 AM Cushing's syndrome Pathway Show 1 more References 1. Nieman LK, Biller BM, Findling JW et al. The Diagnosis of Cushing's Syndrome: An Endocrine Society Clinical Practice Guideline. J Clin Endocrinol Metab. 2008 May;93 5 1526 40. Open 2. Nieman LK, Biller BM, Findling JW et al. Treatment of Cushing's Syndrome: An Endocrine Society Clinical Practice Guideline. J Clin Endocrinol Metab. 2015 Aug;100 8 2807 31. Open 3. A Luger, L H A Broersen, N R Biermasz et al. ESE Clinical Practice Guideline on functioning and nonfunctioning pituitary adenomas in pregnancy. Eur J Endocrinol. 2021 Aug 23;185 3 G1 G33. Open 4. Linwah Yip, Quan-Yang Duh, Heather Wachtel et al. American Association of Endocrine Surgeons Guidelines for Adrenalectomy: Executive Summary. JAMA Surg. 2022 Aug 17. Open 5. Connie B Newman, Michael J Blaha, Jeffrey B Boord et al. Lipid Management in Patients with Endocrine Disorders: An Endocrine Society Clinical Practice Guideline. J Clin Endocrinol Metab. 2020 Dec 1;105 12):dgaa674. Open 6. Susmeeta T Sharma, Lynnette K Nieman, and Richard A Feelders. Cushing s syndrome: epidemiology and developments in disease management. Clin Epidemiol. 2015; 7 281 293. Open https://web.pathway.md/diseases/recTgTOpnhjZgalO3 8/8 |
Guideline sources The following summarized guidelines for the evaluation and management of cutaneous and mucocutaneous leishmaniasis are prepared by our editorial team based on guidelines from the Infectious Diseases Society of America (IDSA/ASTMH 2017). 1 Guidelines 1. Diagnostic investigations Clinical examination: assess for mucosal symptoms snd signs, even during initial evaluation, in all patients with cutaneous leishmaniasis at risk for mucocutaneous leishmaniasis on the basis of the etiologic agent of Leishmania infection, if known, and the region in the New World in which infection was acquired. B Show 3 more Laboratory testing (general principles): Attempt multiple diagnostic approaches to maximize the likelihood of a positive Leishmania result, using methods such as: visualization of the characteristic amastigote in smears or tissue (histopathology) parasite isolation by culture molecular detection of parasite DNA. B https://web.pathway.md/diseases/recVQKiNkLRDIHqBS 1/4 6/23/23, 3:36 AM Cutaneous and mucocutaneous leishmaniasis Pathway Show 2 more Laboratory testing (deoxyribonucleic acid-based assays): Obtain DNA-based assays, especially if other diagnostic testing is unrevealing. Recognize that they are emerging as the most sensitive assays for the diagnosis of leishmaniasis. B Obtain molecular amplification assays because they are the most sensitive Leishmania tests currently available. B Laboratory testing (serology): do not obtain serologic testing as part of the diagnostic evaluation for cutaneous leishmaniasis. Recognize that the currently available serologic assays are neither sensitive nor specific for the diagnosis of cutaneous leishmaniasis. D Skin testing: do not obtain Leishmania skin testing for any form of leishmaniasis. Recognize that there are no standardized, approved, or commercially available skin-test products in North America. D 2. Diagnostic procedures Biopsy: collect tissue specimens from lesions when cutaneous leishmaniasis is clinically suspected. Recognize that full-thickness skin biopsy specimens allow for simultaneous testing for other diagnoses, such as by histopathology and cultures. B Show 2 more 3. Medical management General principles: consider offering empiric treatment based on individualized risk-benefit assessment after a careful diagnostic evaluation in which neither leishmaniasis nor another diagnosis is confirmed. C Show 4 more Systemic therapy (indications): administer systemic anti-leishmanial therapy in all patients with clinically manifest, metastatic, American mucocutaneous leishmaniasis, with the goals of preventing morbidity (such as disfigurement) and mortality (such as from aspiration pneumonia or respiratory obstruction). B Show 4 more Systemic therapy (choice of agent, dose and duration): individualize the choice of agent, dose, and duration of therapy in patients with cutaneous/mucocutaneous leishmaniasis to maximize effectiveness and to minimize toxicity. B Show 3 more Local therapy: Prefer local therapy for the treatment of clinically simple Old World cutaneous leishmaniasis lesions. Consider using local therapy for localized New World cutaneous leishmaniasis caused by Leishmania species not associated with increased risk for mucocutaneous leishmaniasis. B https://web.pathway.md/diseases/recVQKiNkLRDIHqBS 2/4 6/23/23, 3:36 AM Cutaneous and mucocutaneous leishmaniasis Pathway Perform debridement of eschars overlying ulcers before administering local therapy in patients with cutaneous leishmaniasis and treat any secondary infection to maximize treatment effect. B Corticosteroids: administer prophylactic corticosteroid therapy in patients with mucocutaneous leishmaniasis with laryngeal/pharyngeal involvement and increased risk for respiratory obstruction, as indicated by symptoms and otolaryngologic/radiologic examinations, because of the potential for inflammatory reactions after initiation of anti-leishmanial therapy. B 4. Specific circumstances Patients with human immunodeficiency virus/acquired immunodeficiency syndrome: offer systemic anti-leishmanial therapy in patients with human immunodeficiency virus/AIDS-associated cutaneous/mucocutaneous leishmaniasis, particularly in moderately to severely immunosuppressed patients (such as having CD4+ T-lymphocyte counts < 200-350 cells/mm ), possibly being at increased risk for suboptimal therapeutic responses, for post-treatment relapses, and for cutaneous, mucosal, or visceral dissemination. B Show 2 more Patients with other causes of immunosuppression: Do not obtain routine serologic screening of organ donors from leishmaniasis-endemic areas. Obtain clinical and laboratory monitoring of the organ recipient in the post-transplant period if an available donor is known to be seropositive, rather than to reject the organ for transplant. D Consider offering systemic therapy and standard drug regimens for the pertinent setting/species (such as geographic area where the infection was acquired) in patients with cutaneous/mucocutaneous leishmaniasis associated with the use of TNF- antagonist therapy. C 5. Patient education Patient education: Educate and provide patients with cutaneous leishmaniasis at risk for mucocutaneous leishmaniasis with personalized documentation about the importance of seeking medical attention for possible mucocutaneous leishmaniasis if they ever develop persistent, atypical (unusual for the patient) naso-oropharyngeal/laryngeal manifestations not having a clear etiology. B Educate patients with cutaneous leishmaniasis about the signs and symptoms of relapse and mucocutaneous leishmaniasis and instruct them to seek medical attention anytime these appear. B 6. Follow-up and surveillance Observation: offer clinical observation alone for spontaneously healing skin lesions in immunocompetent patients with cutaneous leishmaniasis caused by infection with Leishmania https://web.pathway.md/diseases/recVQKiNkLRDIHqBS 3/4 6/23/23, 3:36 AM Cutaneous and mucocutaneous leishmaniasis Pathway species not associated with increased risk for mucocutaneous leishmaniasis, if the patient concurs with this management. B Show 2 more Indications for referral: refer for consultation with a leishmaniasis expert about other treatment options for the management of patients with cutaneous leishmaniasis with lesions associated with therapeutic failure. B Show 2 more Monitoring of treatment response: assess patients with cutaneous leishmaniasis for treatment response by clinical criteria. Do not obtain repeated parasitologic testing if the skin lesion appears to be healing. B Show 3 more Monitoring for progression: Obtain active clinical monitoring in patients with cutaneous leishmaniasis, including by performing a careful nasal and oropharyngeal examination periodically up to 1 year, or at least 2 years if at increased risk for mucocutaneous leishmaniasis. Educate patients with cutaneous leishmaniasis about the signs and symptoms of relapse and mucocutaneous leishmaniasis and instruct them to seek medical attention anytime these appear. B Evaluate patients with cutaneous leishmaniasis for mucocutaneous leishmaniasis, including performing fiberoptic examination of the affected area if relevant, if symptoms such as chronic nasal stuffiness, epistaxis, or hoarseness or findings such as septal perforation occur anytime in patients with a prior or current diagnosis of cutaneous leishmaniasis or a scar consistent with prior cutaneous leishmaniasis. B Management of treatment failure: offer additional therapy (but not necessarily always a different agent or approach) in patients with cutaneous leishmaniasis if new skin lesions develop or existing lesions worsen. Offer additional therapy if there is incomplete healing by 3 months after completion of the treatment course. B References 1. Naomi Aronson, Barbara L Herwaldt, Michael Libman et al. Diagnosis and Treatment of Leishmaniasis: Clinical Practice Guidelines by the Infectious Diseases Society of America IDSA and the American Society of Tropical Medicine and Hygiene ASTMH . Am J Trop Med Hyg. 2017 Jan 11; 96 1 24 45. Open https://web.pathway.md/diseases/recVQKiNkLRDIHqBS 4/4 |
Guideline sources The following summarized guidelines for the evaluation and management of cutaneous lupus erythematosus (CLE) are prepared by our editorial team based on guidelines from the American College of Rheumatology (ACR 2023), the European League Against Rheumatism (EULAR 2022), the British Association of Dermatologists (BAD 2021), the German Society of Dermatology (DDG/GKJR/DGRh 2021), the Chinese Society of Dermatology (CSD/ADA/AADV 2021), the Royal College of Ophthalmologists (RCOphth 2020), and the European Academy of Dermatology and Venereology (EADV 2017). 1 2 3 4 5 6 7 8 CalculatorEULAR/ACR classification criteri https://web.pathway.md/diseases/rechQtCFQ0qxf8UQv 1/10 6/23/23, 3:37 AM Cutaneous lupus erythematosus Pathway Guidelines 1. Screening and diagnosis Diagnostic criteria: Use the 2019 EULAR/ACR criteria to differentiate CLE from SLE. A Consider reassessing the 2019 EULAR/ACR criteria either once a year and/or in case of clinical/laboratory changes in any patient with CLE. C Differential diagnosis: as per BAD 2021 guidelines, suspect drug-induced CLE in patients with CLE, particularly subacute CLE, and discontinue any potential causative drug. B 2. Classification and risk stratification Severity assessment: As per DDG 2021 guidelines: Consider using the CLASI or revised CLASI to assess disease activity and intensity in patients with CLE. C Consider using the DLQI or Skindex-29 (skin-specific methods for assessing the quality of life) to assess the quality of life in patients with CLE. C 3. Diagnostic investigations History and physical examination: As per BAD 2021 guidelines, evaluate for SLE by history, examination and targeted laboratory investigations in patients with CLE. B As per DDG 2021 guidelines, assess the patient's medication in cases of drug-induced subacute CLE or deterioration of established subacute CLE. A As per EADV 2017 guidelines, elicit patient's past and present drug history, particularly in patients with subacute CLE. B Evaluation for malignancy: evaluate for a tumor in cases of treatment-refractory subacute CLE, a late manifestation of subacute CLE (> 60 years of age), and symptoms suggestive of carcinoma. A Photoprovocation testing: consider obtaining standardized photoprovocation testing by experienced investigators in special cases, for example, to exclude CLE or to differentiate between CLE and polymorphous light eruption. C Laboratory testing: Do not obtain a routine assessment of glucose-6 phosphate dehydrogenase activity. D Do not obtain a pregnancy test as part of the basic evaluation. D https://web.pathway.md/diseases/rechQtCFQ0qxf8UQv 2/10 6/23/23, 3:37 AM Cutaneous lupus erythematosus Pathway Pretreatment evaluation: screen for HBV infection in all patients eligible for treatment with conventional synthetic, biological, or targeted synthetic DMARDs, immunosuppressants, or corticosteroids (according to dose and duration). B Show 3 more 4. Diagnostic procedures Biopsy and histopathology: perform lesional biopsy for histological confirmation of a clinical diagnosis of CLE, while omitting in cases of butterfly rash or mucosal lesions. A Show 2 more 5. Medical management General principles: manage patients with CLE in the setting of SLE jointly with a rheumatologist. E Topical therapy: As per BAD 2021 guidelines, initiate very potent/potent topical corticosteroids as first-line monotherapy option in patients with localized CLE (including the face) for up to 4 weeks, and as an adjuvant to systemic therapy when there is widespread cutaneous and/or SLE involvement. A Show 3 more As per DDG 2021 guidelines, initiate topical corticosteroids in patients with circumscribed CLE lesions. A Show 4 more As per EADV 2017 guidelines, initiate topical corticosteroids as first-line therapy for several weeks in patients with CLE. B Show 4 more Antimalarial agents: As per BAD 2021 guidelines, initiate antimalarials, either alone or with adjunctive topical corticosteroids, as first-line systemic therapy in patients with CLE. A Show 14 more As per DDG 2021 guidelines, initiate combination treatment with antimalarial drugs in patients with extensive lesions, an inclination to scarring, or insufficient response. A Show 3 more As per EADV 2017 guidelines, initiate antimalarials as first-line and long-term systemic therapy in all patients with CLE with severe or widespread skin lesions, particularly in patients with the risk of scarring and development of systemic disease. B Show 7 more Systemic corticosteroids: https://web.pathway.md/diseases/rechQtCFQ0qxf8UQv 3/10 6/23/23, 3:37 AM Cutaneous lupus erythematosus Pathway As per BAD 2021 guidelines, consider initiating concomitant tapering courses of oral or IV corticosteroids (such as methylprednisolone) in patients with severe or disseminated CLE, or subtypes with the greatest risk of scarring (such as discoid lupus erythematosus with a high risk of permanent damage, high disease burden). E Show 2 more As per DDG 2021 guidelines, initiate systemic corticosteroids, in addition to antimalarial drugs, as first-line therapy for a limited period of time in patients with severe or disseminated CLE lesions. Taper off systemic corticosteroids as soon as possible. A As per EADV 2017 guidelines, initiate systemic corticosteroids first-line therapy in addition to antimalarials in patients with severe or widespread active CLE lesions. B Show 2 more Methotrexate: As per BAD 2021 guidelines, consider initiating methotrexate up to 25 mg once weekly in patients with CLE with an inadequate response to topical therapy and antimalarials. C Show 2 more As per DDG 2021 guidelines, initiate methotrexate as systemic second-line therapy at doses of up to 25 mg per week and, if possible, in combination with antimalarial drugs. A As per EADV 2017 guidelines, initiate methotrexate up to 20 mg/week as second-line therapy, primarily in patients with subacute CLE, preferably SC and in addition to antimalarials. B Systemic retinoids: As per BAD 2021 guidelines, consider initiating acitretin 25-50 mg daily as second-line systemic therapy in patients with CLE. C Show 2 more As per DDG 2021 guidelines, initiate retinoids, preferably in combination with antimalarial drugs, as second-line systemic therapy in patients with hypertrophic CLE lesions. A Consider initiating retinoids as second-line systemic therapy in all other forms of CLE. B As per EADV 2017 guidelines, initiate systemic retinoids as second-line therapy in selected patients with CLE unresponsive to other treatments, preferably in addition to antimalarials. B Dapsone: As per BAD 2021 guidelines, consider initiating dapsone (typically at 50 mg daily and escalated to 150 mg daily, depending on response and tolerability) as first-line systemic therapy in patients with subacute CLE and bullous SLE. E Show 2 more As per DDG 2021 guidelines: Consider initiating dapsone as first-line therapy in patients with bullous CLE and as second- line therapy, preferably in combination with antimalarial drugs, in patients with refractory CLE. C Start dapsone at low doses (50 mg/day) and increase the dose to a maximum of 1.5 mg/kg body weight depending on clinical response and side effects. Monitor glucose-6 phosphate dehydrogenase activity before initiating treatment. A https://web.pathway.md/diseases/rechQtCFQ0qxf8UQv 4/10 6/23/23, 3:37 AM Cutaneous lupus erythematosus Pathway As per EADV 2017 guidelines, consider initiating dapsone as first-line therapy in patients with bullous lupus erythematosus. C Show 3 more Mycophenolate mofetil: As per BAD 2021 guidelines, consider initiating oral mycophenolate mofetil (typically at 500 mg BID and escalated to 1.5 g BID, dependent on response and tolerability) in pateints with CLE with an inadequate response to topical therapy and antimalarials. C Show 2 more As per DDG 2021 guidelines: Consider initiating mycophenolate mofetil, preferably in combination with antimalarial drugs, as third-line therapy in patients with refractory CLE lesions. Start mycophenolate mofetil at a dose of 500 mg BID with a subsequent dose increase to 2 g/day. B Consider initiating mycophenolic acid as an alternative to mycophenolate mofetil. C As per EADV 2017 guidelines, initiate mycophenolate mofetil as third-line therapy in patients with refractory CLE, preferably in addition to antimalarials. B Show 2 more Other immunosuppressants: As per DDG 2021 guidelines: Consider offering azathioprine or cyclosporin for the treatment of patients with CLE. (No recommendation. Do not use cyclophosphamide for the treatment of patients with CLE. D As per EADV 2017 guidelines: Avoid using azathioprine, cyclophosphamide and cyclosporine for the treatment of patients with CLE without systemic involvement. D Do not use leflunomide for the treatment of patients with CLE. D Thalidomide and lenalidomide: As per BAD 2021 guidelines, consider initiating thalidomide as third-line therapy in patients with treatment-resistant CLE. C Show 4 more As per DDG 2021 guidelines, consider initiating thalidomide, preferably in combination with antimalarial drugs, in selected patients with refractory CLE lesions. As per EADV 2017 guidelines, initiate thalidomide in selected patients with refractory CLE, preferably in addition to antimalarials. B Show 4 more Monoclonal antibodies: As per BAD 2021 guidelines: Consider initiating rituximab on a case-by-case basis in patients with treatment-resistant CLE, if conventional systemic therapies have failed. C https://web.pathway.md/diseases/rechQtCFQ0qxf8UQv 5/10 6/23/23, 3:37 AM Cutaneous lupus erythematosus Pathway Consider initiating belimumab in eligible patients with SLE with cutaneous involvement, if conventional systemic therapies have failed. C As per DDG 2021 guidelines, consider initiating belimumab or rituximab in patients with CLE. As per EADV 2017 guidelines, avoid using belimumab for the treatment of patients with CLE without systemic involvement. D Show 2 more Other agents: As per BAD 2021 guidelines, consider administering IVIG only on a case-by-case basis in patients with treatment-resistant CLE, if conventional systemic therapies have failed. C Show 2 more As per DDG 2021 guidelines: Consider administering IVIG in patients with CLE. Do not use antibiotics for the treatment of patients with CLE. D As per EADV 2017 guidelines: Do not use antibiotics/antimicrobials (clofazimine, sulfasalazine, cefuroxime axetil) and interferon-alpha for the treatment of patients with CLE. D Avoid using intravenous IgAnd danazol for the treatment of patients with CLE. D 6. Nonpharmacologic interventions Lifestyle modifications: As per BAD 2021 guidelines, counsel patients with CLE about the importance of lifestyle changes on disease activity and treatment response, such as smoking cessation and the need for a range of photoprotective measures, including the use of a broad-spectrum sunscreen. A As per DDG 2021 guidelines, advise using sunscreen in exposed areas in addition to sun protective clothing in patients of all stages irrespective of the extent and the topical or systemic medication used. A As per DDG 2021 guidelines, advise avoiding active and passive exposure to tobacco smoke in patients with CLE. A As per EADV 2017 guidelines, advise avoiding unprotected UV exposure and using daily preventive (chemical and physical) measures in all patients with CLE. B Show 2 more Vitamin D supplementation: As per BAD 2021 guidelines, consider offering vitamin D supplementation in patients with CLE. C As per DDG 2021 guidelines, consider offering vitamin D3 supplementation in patients with CLE. https://web.pathway.md/diseases/rechQtCFQ0qxf8UQv 6/10 6/23/23, 3:37 AM Cutaneous lupus erythematosus Pathway As per EADV 2017 guidelines, consider offering vitamin D supplementation in all patients with CLE. C Herbal products: caution patients with CLE against herbal supplements and traditional medicines as some may contain corticosteroids or induce disease exacerbation by immune stimulation. E Psychological support: consider referring patients to available psychological support services (such as a psychiatrist, clinical psychologist or patient support group) if the psychological impact of CLE is significant. E 7. Therapeutic procedures Intralesional corticosteroids: as per BAD 2021 guidelines, consider administering intralesional triamcinolone (0.1 mL/cm field, starting at 2.5-5 mg/mL for sites at higher risk of atrophy including the face and 10 mg/mL for other sites) as a local treatment option in patients with localized discoid lupus erythematosus or as an adjunctive therapy for persistent lesions. E Physical modalities: As per BAD 2021 guidelines, insufficient evidence to recommend pulsed dye laser for the treatment of patients with CLE. I As per DDG 2021 guidelines, do not offer terapeutic UV irradiation in patients with CLE. D Show 2 more As per EADV 2017 guidelines, do not offer any UV light therapy in patients with CLE. D Show 3 more 8. Specific circumstances Considerations for contraception: As per DDG 2021 guidelines, consider preferring non-hormonal contraceptives or progestin-only contraceptives. C Show 2 more As per EADV 2017 guidelines: Do not use estrogen-containing hormonal contraception in patients with CLE and associated antiphospholipid syndrome. D Avoid using estrogen replacement therapy in patients with CLE. D Patients contemplating pregnancy: counsel female patients of reproductive age with CLE about possible difficulties with pregnancy as a precautionary measure. A Show 3 more Pregnant patients: As per BAD 2021 guidelines, obtain additional monitoring by the obstetric team to identify congenital heart block in the fetuses of female patients with CLE with anti-Ro/SSA and/or anti- La/SSB antibodies becoming pregnant. E https://web.pathway.md/diseases/rechQtCFQ0qxf8UQv 7/10 6/23/23, 3:37 AM Cutaneous lupus erythematosus Pathway Show 2 more As per DDG 2021 guidelines, obtain serial fetal echocardiography starting at the 16th week of gestation in patients positive for anti-Ro/SSA and/or anti-La/SSB antibodies with a previous congenital heart block pregnancy. A Show 4 more As per EADV 2017 guidelines, initiate hydroxychloroquine at usual dosage as first-line therapy in patients with active CLE during pregnancy or breastfeeding. B Show 5 more 9. Patient education General counseling: As per BAD 2021 guidelines: Offer obtaining a pregnancy test in female patients of child-bearing potential before initiating methotrexate, mycophenolate mofetil, acitretin, rituximab, belimumab, cyclophosphamide, thalidomide or lenalidomide therapy. Counsel them regarding the risk of teratogenicity, advise pregnancy prevention and instigate the pregnancy prevention program. A Advise using reliable contraception as a precautionary measure in sexually active male patients during methotrexate, mycophenolate mofetil, thalidomide and lenalidomide therapy, during dose interruption (where applicable) and for periods of time following the cessation of treatment. A As per DDG 2021 guidelines, inform patients with CLE about the possibility of Koebner phenomenon. A Show 2 more 10. Preventative measures Routine immunizations: As per ACR 2023 guidelines, consider offering high-dose or adjuvanted influenza vaccination, rather than regular-dose influenza vaccination, in 65 years old patients with rheumatic or musculoskeletal diseases and in 18-65 years old patients with rheumatic or musculoskeletal diseases on immunosuppressive medications. C Show 17 more As per DDG 2021 guidelines, evaluate and vaccinate patients before and during immunosuppressive/immunomodulating treatment according to current guidelines. Offer annual influenza vaccination. A Prophylaxis for Pneumocystis jirovecii pneumonia: consider administering prophylaxis against P. jirovecii pneumonia in patients initiating high-dose corticosteroids, especially in combination with immunosuppressants and depending on the risk-benefit ratio. C https://web.pathway.md/diseases/rechQtCFQ0qxf8UQv 8/10 6/23/23, 3:37 AM Cutaneous lupus erythematosus Pathway 11. Follow-up and surveillance Assessment of treatment response: As per DDG 2021 guidelines, consider using the CLASI or the revised CLASI for monitoring therapeutic response. C As per DDG 2021 guidelines: Assess the efficacy of systemic treatment for CLE after a minimum of 3 months and a maximum of 6 months (except for corticosteroids). A Continue systemic treatment for up to 1 year after the skin lesions have resolved in patients without any immunological disorders or systemic symptoms. Individualize the decision on treatment continuation for all other patients. A Monitoring of chloroquine/chloroquine treatment: As per DDG 2021 guidelines: Obtain ophthalmological evaluation in all patients with CLE during the first year of treatment with antimalarial drugs, and every year after five years of treatment. A Obtain ophthalmological evaluation before initiating treatment with antimalarial drugs in patients with preexisting ocular disease. Obtain yearly ophthalmological monitoring in patients with risk factors (especially renal failure). A Follow-up: As per DDG 2021 guidelines, consider reassessing the 2019 EULAR/ACR criteria either once a year and/or in case of clinical/laboratory changes in patients with CLE. C Show 3 more As per DDG 2021 guidelines: Obtain routine evaluation of basic laboratory parameters used in normal patient care during chloroquine/hydroxychloroquine treatment. Do not obtain additional monitoring. Monitor all other treatments according to the guideline for bullous dermatitis/psoriasis. A Evaluate and monitor patients before and during immunosuppressive/immunomodulating treatment according to current guidelines. A Surveillance for malignancy: advise participation in the generally recommended cancer screening examinations (skin, colon, gynecology, prostate) in patients with CLE. A Monitoring of hydroxychloroquine/chloroquine treatment: as per RCOphth 2020 guidelines, obtain annual monitoring for retinopathy in all patients receiving hydroxychloroquine for > 5 years or chloroquine for > 1 year. B Show 17 more References 1. Anne R Bass, Eliza Chakravarty, Elie A Akl et al. 2022 American College of Rheumatology Guideline for Vaccinations in Patients With Rheumatic and Musculoskeletal Diseases. Arthritis Rheumatol. 2023 Jan 4. Online ahead of print. Open https://web.pathway.md/diseases/rechQtCFQ0qxf8UQv 9/10 6/23/23, 3:37 AM Cutaneous lupus erythematosus Pathway 2. D O'Kane, C McCourt, S Meggitt et al. British Association of Dermatologists guidelines for the management of people with cutaneous lupus erythematosus 2021. Br J Dermatol. 2021 Dec;185 6 1112 1123. Open 3. A Kuhn, E Aberer, Z Bata-Cs rg et al. S2k guideline for treatment of cutaneous lupus erythematosus - guided by the European Dermatology Forum EDF in cooperation with the European Academy of Dermatology and Venereology EADV . J Eur Acad Dermatol Venereol. 2017 Mar;31 3 389 404. Open 4. Margitta Worm, Miriam Zidane, Lisa Eisert et al. S2k guideline: Diagnosis and management of cutaneous lupus erythematosus Part 2 Therapy, risk factors and other special topics. J Dtsch Dermatol Ges. 2021 Sep;19 9 1371 1395. Open 5. Qianjin Lu, Hai Long, Steven Chow et al. Guideline for the diagnosis, treatment and long-term management of cutaneous lupus erythematosus. J Autoimmun. 2021 Sep;123 102707. Open 6. Margitta Worm, Miriam Zidane, Lisa Eisert et al. S2k guideline: Diagnosis and management of cutaneous lupus erythematosus Part 1 Classification, diagnosis, prevention, activity scores. J Dtsch Dermatol Ges. 2021 Aug;19 8 1236 1247. Open 7. George E Fragoulis, Elena Nikiphorou, Mrinalini Dey et al. 2022 EULAR recommendations for screening and prophylaxis of chronic and opportunistic infections in adults with autoimmune inflammatory rheumatic diseases. Ann Rheum Dis. 2022 Nov 3;ard-2022 223335. Open 8. The Royal College of Ophthalmologists. Hydroxychloroquine and Chloroquine Retinopathy: Recommendations on Monitoring. RCOphth. 2020 Dec. Open https://web.pathway.md/diseases/rechQtCFQ0qxf8UQv 10/10 |
Guideline sources The following summarized guidelines for the evaluation and management of cutaneous melanoma (CM) are prepared by our editorial team based on guidelines from the U.S. Preventive Services Task Force (USPSTF 2023), the American Society of Plastic Surgeons (ASPS 2021), the European Society of Medical Oncology (ESMO 2020; 2019), the American Society of Clinical Oncology (ASCO 2020), and the American Academy of Dermatology (AAD 2019). 1 2 3 4 5 7 Calculator Calculator Calculator 7-point dermoscopy checklist fo Eastern Cooperative Oncology G Karnofsky Guidelines https://web.pathway.md/diseases/recGM7euHf4Lsuh55 1/8 6/23/23, 3:36 AM Cutaneous melanoma Pathway 1. Screening and diagnosis Indications for screening: insufficient evidence to assess the balance of benefits and harms of visual skin examination by a clinician to screen for skin cancer in adolescents and adults. I 2. Diagnostic investigations Physical examination: Obtain dermatoscopy by an experienced physician to enhance the diagnostic accuracy. B Perform physical examination with special attention to other suspicious pigmented lesions, tumor satellites, in-transit metastases, regional lymph nodes, and distant metastases. B Baseline imaging: As per AAD 2019 guidelines, do not obtain baseline imaging in asymptomatic patients with newly diagnosed stage 0-II primary CM. D Show 2 more As per ESMO 2019 guidelines, do not obtain additional investigations in patients with low-risk melanomas (pT1a). D Show 2 more Baseline laboratory tests: Do not obtain baseline laboratory studies in asymptomatic patients with newly diagnosed stage 0-II primary CM. D Obtain baseline laboratory studies only for the evaluation of specific signs or symptoms of synchronous metastasis (regional nodal or distant) in patients with CM. E 3. Diagnostic procedures Skin biopsy: As per AAD 2019 guidelines, perform narrow excisional/complete biopsy with 1-3 mm margins encompassing the entire breadth of lesion and of sufficient depth to prevent transection at the base as the preferred biopsy technique. Perform excisional biopsy by any of the following methods: fusiform/elliptical excision punch excision around the clinical lesion deep shave/saucerization removal to depth below the anticipated plane of the lesion. E Show 2 more As per ESMO 2019 guidelines: Perform full thickness excisional biopsy with a minimal side margin for the diagnosis of CM. B Follow the eighth edition of the AJCC tumor, node, metastasis classification for histology reporting and include the following in the report: https://web.pathway.md/diseases/recGM7euHf4Lsuh55 2/8 6/23/23, 3:36 AM Cutaneous melanoma Pathway the maximum thickness in millimetres (Breslow) reported to the nearest 0.1 mm (rounding up starting at 0.05) presence of ulceration information on mitotic rate presence and extent of regression actinic damage clearance of the surgical margins. B Sentinel lymph node biopsy: As per ESMO 2020 guidelines, perform sentinel lymph node biopsy for staging of melanomas of stage pT2a or higher (> 1.0 mm Breslow thickness). A Show 3 more As per AAD 2019 guidelines, discuss carefully the risks and benefits of the procedure involving surgical oncology input with all patients eligible for sentinel lymph node biopsy. E Show 5 more As per ESMO 2019 guidelines, perform sentinel lymph node biopsy for precise staging of melanoma in patients with stage pT1b or higher, with a tumor thickness > 0.8 mm or with a tumor thickness with < 0.8 mm with ulceration. B Molecular testing: As per AAD 2019 guidelines, consider obtaining ancillary diagnostic molecular tests, such as comparative genomic hybridization, FISH, gene expression profiling, in patients with equivocal melanocytic neoplasms. E Show 2 more As per ESMO 2019 guidelines, obtain mutation testing for actionable mutations (including BRAF) in patients with: resectable or unresectable stage III or stage IV disease high-risk resected stage IIC disease. A Show 4 more 4. Medical management Neoadjuvant therapy: As per ASCO 2020 guidelines, insufficient evidence to recommend for or against the routine use of neoadjuvant therapy in adult patients with resectable regional or distant metastatic CM. Offer neoadjuvant therapy or refer patients for enrollment in clinical trials where possible. I As per ESMO 2020 guidelines: Consider offering neoadjuvant therapy in patients with easily resectable stage III disease with acceptable surgical morbidity only in the context of a clinical trial. C Consider offering neoadjuvant therapy outside of the context of a clinical trial in patients with technically resectable but bulky nodal and/or in-transit disease when surgery will be https://web.pathway.md/diseases/recGM7euHf4Lsuh55 3/8 6/23/23, 3:36 AM Cutaneous melanoma Pathway associated with significant morbidity, likely to result in positive resection margin status or necessitate the need for postoperative radiotherapy. C Adjuvant therapy: Do not offer adjuvant pembrolizumab, nivolumab, or combination dabrafenib and trametinib therapy in patients with resected stage II melanoma outside of enrollment in a clinical trial. D Show 6 more Consider continuing treatment after surgery based on the pathological response evaluation of the surgical specimen. C Show 9 more Consider offering topical imiquimod 5% cream as second-line therapy in patients with melanoma in situ, lentigo maligna type, after optimal surgery (adjuvant setting). E Offer anti-PD-1 adjuvant therapy or dabrafenib/trametinib as the preferred treatment options in patients with local/locoregional melanoma. A Recognize that the European Medicines Agency has approved nivolumab, pembrolizumab, and the combination of dabrafenib/trametinib for melanoma in the adjuvant setting. A Management of unresectable/metastatic disease: As per ASCO 2020 guidelines, offer the following regimens (in no particular order) in patients with BRAF wild-type unresectable/metastatic CM: ipilimumab plus nivolumab followed by nivolumab nivolumab pembrolizumab. A Show 7 more As per AAD 2019 guidelines, consider offering topical imiquimod 5% cream as second-line therapy in patients with melanoma in situ, lentigo maligna type, if surgery is not possible at the outset (primary setting). E As per ESMO 2019 guidelines, offer programmed cell death protein-1 blockade as first-line standard of care in all patients, regardless of BRAF status. A Show 11 more 5. Therapeutic procedures Radiotherapy: Reserve radiotherapy for patients with recurrent disease. B Consider offering adjuvant radiotherapy in high-risk patients if regional control is a major issue and/or systemic therapy is not possible. C Consider offering radiotherapy as second-line therapy in non-surgical candidates with melanoma in situ, lentigo maligna type. E Show 2 more Do not perform primary elective irradiation in patients with locoregional disease. D https://web.pathway.md/diseases/recGM7euHf4Lsuh55 4/8 6/23/23, 3:36 AM Cutaneous melanoma Pathway Show 3 more 6. Surgical interventions Wide-local excision: As per ESMO 2020 guidelines: Remove primary melanomas with clear margins to ensure local control in the context of resectable clinical stage III disease. Consider performing wide local excision with primary closure to avoid reconstruction whenever possible, ideally with a clinical 1 cm margin, with reconstruction avoided whenever possible. B Do not resect the primary tumor in the absence of symptoms or need for diagnostic tissue in the context of clinical stage IV disease. Resect with clear margins but without additional safety margins if there is an indication to resect the primary lesion. D As per AAD 2019 guidelines, perform surgical excision with histologically negative margins as first-line treatment in patients with CM of any thickness and melanoma in situ. Decide on surgical margins based on tumor thickness. E Show 5 more Lymph node dissection: Perform radical lymph node dissection in patients with clinically-detected lymph node metastases in resectable stage III disease after pathological assessment (cytology or histology of lesion preoperatively) and adequate staging. B Show 5 more Ensure interdisciplinary collaboration involving surgical and medical oncologists for discussion of possible completion of lymph node dissection or obtaining regional nodal ultrasound surveillance in patients with positive sentinel lymph node biopsy. E Do not perform elective lymph node dissection in patients with locoregional disease. D Show 2 more Resection of metastases: Perform resection with clear margins, but without additional safety margins, in patients with in- transit metastases regarded as few, small and non-rapidly recurrent lesions. Avoid performing extensive and multiple repeated resections and reconstructions. B Consider performing surgical removal of locoregional recurrence or single distant metastasis in fit patients, as a therapeutic option for long-term disease control. C 7. Specific circumstances Pregnant patients: ensure a tailored, multidisciplinary approach of care for pregnant patients with CM, involving obstetrician and CM specialist relevant to the patient's stage of disease. Recognize that the diagnosis of CM during pregnancy does not alter prognosis or outcome for the patient, however take the safety of the fetus into consideration for work-up and treatment. E https://web.pathway.md/diseases/recGM7euHf4Lsuh55 5/8 6/23/23, 3:36 AM Cutaneous melanoma Pathway Show 2 more Concurrent use of exogenous hormones: recognize that exogenous hormones (such as oral contraceptives and hormone-containing contraceptive devices/implants, postmenopausal HRT, or hormones associated with assisted reproductive technology) may be used in female patients diagnosed with CM. E 8. Patient education General counseling: As per AAD 2019 guidelines: Discuss carefully the associated risks, benefits and uncertainties of non-surgical treatment with the patient and family. E Educate patients on self-examination of the skin and lymph node for the detection of recurrent disease or new primary CM. E As per ESMO 2019 guidelines: Provide patients with CM with instructions in avoidance of sunburns, extended unprotected solar or artificial UV exposure, and in lifelong regular self-examinations of the skin and peripheral lymph nodes. B Inform patients that their family members have an increased risk of melanoma. B Genetic counseling: as per AAD 2019 guidelines, provide cancer risk counseling by a qualified genetic counselor in patients with CM with any of the following: a family history of invasive CM or pancreatic cancer ( 3 affected members on one side of the family) multiple primary invasive CM ( 3), including one early-onset tumor (at age < 45 years) 1 melanocytic BAP1-mutated atypical intradermal tumor and a family history of mesothelioma, meningioma, and/or uveal melanoma 2 melanocytic BAP1-mutated atypical intradermal tumors. E 9. Follow-up and surveillance Assessment for cutaneous toxicity: ensure collaboration between dermatologists and oncologists for the management of cutaneous toxicity during BRAF/MEK kinase or immune checkpoint inhibitor therapy because appropriate recognition and control of skin side effects may improve the quality of life of patients with CM and avoid unnecessary interruption of medication. E Show 4 more Skin reconstruction after tumor resection (timing): consider performing reconstructive surgery in a delayed (asynchronous) fashion in adult patients after skin cancer resection. C Skin reconstruction after tumor resection (perioperative antibiotics): consider discussing the management of antibiotics with adult patients undergoing reconstruction after skin cancer https://web.pathway.md/diseases/recGM7euHf4Lsuh55 6/8 6/23/23, 3:36 AM Cutaneous melanoma Pathway resection when relevant. C Show 3 more Skin reconstruction after tumor resection (perioperative antithrombotics): consider discussing the management of anticlotting agents with adult patients undergoing reconstruction after skin cancer resection when relevant. C Show 2 more Skin reconstruction after tumor resection (perioperative analgesics): consider discussing the management of pain with adult patients undergoing reconstruction after skin cancer resection when relevant. C Show 2 more Skin reconstruction after tumor resection (follow-up): consider obtaining postoperative follow- up assessment in adult patients undergoing reconstruction after skin cancer resection. C Follow-up: As per AAD 2019 guidelines, obtain regular clinical follow-up as the most important means of detecting CM recurrence. Include history (review of systems) and physical examination in the follow-up assessment to direct the need for further radiologic or laboratory studies to detect local, regional, and distant metastatic disease. E Show 4 more As per ESMO 2019 guidelines: Monitor patients with CM to detect a relapse and to recognize additional skin tumors, especially secondary melanomas as early as possible. B Obtain serum S100 protein rather than LDH, if any blood test is required, as the most accurate blood test for monitoring disease progression in patients with CM. B Likelihood Ratios Pertinent positives The following findings increase the probability of cutaneous melanoma in adults. -1 Finding LR+ Value History of lesion enlargement 11 (8.5-14) Presence of lesion greater than 6 mm in diameter 2.3 (2.1-2.5) Presence of asymmetric lesion 2.1 (1.9-2.5) Presence of uneven or ragged borders of lesion 2.1 (1.8-2.4) Show 2 more Pertinent negatives The following findings decrease the probability of cutaneous melanoma in adults. -1 Finding LR- Value Absence of ABCDE score (< 1) 0.07 (0.04-0.13) https://web.pathway.md/diseases/recGM7euHf4Lsuh55 7/8 6/23/23, 3:36 AM Cutaneous melanoma Pathway Absence of lesion greater than 6 mm in diameter No history of lesion enlargement 0.17 (0.13-0.22) 0.18 (0.15-0.22) Absence of more than 1 shade of pigment in lesion 0.59 (0.52-0.68) Show 2 more References 1. Swetter SM, Tsao H, Bichakjian CK et al. Guidelines of care for the management of primary cutaneous melanoma. J Am Acad Dermatol. 2019 Jan;80 1 208 250. Open 2. O Michielin, A C J van Akkooi, P A Ascierto et al. Cutaneous melanoma: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2019 Dec 1;30 12 1884 1901. Open 3. Andrew Chen, John G Albertini, Jeremy S Bordeaux et al. Evidence-Based Clinical Practice Guideline: Reconstruction after Skin Cancer Resection. Plast Reconstr Surg. 2021 May 1;147 5 812e-829e. Open 4. O Michielin, A van Akkooi, P Lorigan et al. ESMO consensus conference recommendations on the management of locoregional melanoma: under the auspices of the ESMO Guidelines Committee. Ann Oncol. 2020 Nov;31 11 1449 1461. Open 5. Rahul Seth, Hans Messersmith, Varinder Kaur et al. Systemic Therapy for Melanoma: ASCO Guideline. J Clin Oncol. 2020 Nov 20;38 33 3947 3970. Open 6. Wong SL, Faries MB, Kennedy EB et al. Sentinel Lymph Node Biopsy and Management of Regional Lymph Nodes in Melanoma: American Society of Clinical Oncology and Society of Surgical Oncology Clinical Practice Guideline Update. J Clin Oncol. 2018 Feb 1;36 4 399 413. Open 7. US Preventive Services Task Force, Carol M Mangione, Michael J Barry et al. Screening for Skin Cancer: US Preventive Services Task Force Recommendation Statement. JAMA. 2023 Apr 18;329 15 1290 1295. Open 8. Dummer R, Hauschild A, Guggenheim M et al. Cutaneous melanoma: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2012 Oct;23 Suppl 7:vii86 91. Open 9. Giulia C Leonardi, Luca Falzone, Rossella Salemi et al. Cutaneous melanoma: From pathogenesis to therapy Review). Int J Oncol. 2018 Apr;52 4 1071 1080. Open 10. David E Elder, Boris C Bastian, Ian A Cree et al. The 2018 World Health Organization Classification of Cutaneous, Mucosal, and Uveal Melanoma: Detailed Analysis of 9 Distinct Subtypes Defined by Their Evolutionary Pathway. Arch Pathol Lab Med. 2020 Apr;144 4 500 522. Open 11. G Argenziano, G Fabbrocini, P Carli et al. Epiluminescence microscopy for the diagnosis of doubtful melanocytic skin lesions. Comparison of the ABCD rule of dermatoscopy and a new 7-point checklist based on pattern analysis. Arch Dermatol. 1998 Dec;134 12 1563 70. Open 12. G Argenziano, C Catrical , M Ardigo et al. Seven-point checklist of dermoscopy revisited. Br J Dermatol. 2011 Apr;164 4 785 90. Open 13. Rahul Seth, Hans Messersmith, Pauline Funchain et al. Systemic Therapy for Melanoma: ASCO Guideline Rapid Recommendation Update. J Clin Oncol. 2022 Jul 20;40 21 2375 2377. Open https://web.pathway.md/diseases/recGM7euHf4Lsuh55 8/8 |
Guideline sources The following summarized guidelines for the evaluation and management of cutaneous squamous cell carcinoma (CSCC) are prepared by our editorial team based on guidelines from the U.S. Preventive Services Task Force (USPSTF 2023; 2018), the American Society of Plastic Surgeons (ASPS 2021), the British Association of Dermatologists (BAD 2021), the European Association of Dermato-Oncology (EADO/EORTC 2020), the American Society for Radiation Oncology (ASTRO 2020), the British Photodermatology Group (BPG/BAD 2019), and the American Academy of Dermatology (AAD 2018). 1 2 3 4 5 6 7 8 Calculator Calculator Calculator Brigham and Women's Hospital t Eastern Cooperative Oncology G Karnofsky Guidelines https://web.pathway.md/diseases/recYAu9FO4o5hoaAg 1/11 6/23/23, 3:36 AM Cutaneous squamous cell carcinoma Pathway 1. Screening and diagnosis Indications for screening: As per USPSTF 2023 guidelines, insufficient evidence to assess the balance of benefits and harms of visual skin examination by a clinician to screen for skin cancer in adolescents and adults. I As per USPSTF 2018 guidelines, insufficient evidence to assess the balance of benefits and harms of counseling adult persons about skin self-examination to prevent skin cancer. I 2. Classification and risk stratification Staging: use the Brigham and Women's Hospital tumor classification system to obtain the most accurate prognostication of patients with localized cutaneous SCC. B Risk classification: document the risk status of cutaneous SCC as low risk, high risk, or very high risk in the patient notes. E 3. Diagnostic investigations Imaging for staging: As per BAD 2021 guidelines, do not obtain routine imaging of the draining nodal basin in patients with cutaneous SCC without suspected or clinically detectable regional nodal involvement. D Show 3 more As per ASTRO 2020 guidelines, consider obtaining imaging to guide the need for and target of lymph node basin radiotherapy in patients with cutaneous SCC at high risk of regional nodal metastasis. E 4. Diagnostic procedures Skin biopsy: As per BAD 2021 guidelines, obtain histological confirmation of cutaneous SCC lesions in case of diagnostic uncertainty before planning definitive treatment. Perform full-thickness incisional biopsy to collect a representative tumor sample, ideally incorporating both the peripheral and the deep margins. A Show 3 more As per EADO 2020 guidelines: Perform an excision to obtain histological confirmation of peripheral and deep excision margins. B Consider performing a punch or incisional biopsy (with planned subsequent complete excision) for histological confirmation of large tumors or tumors on high-risk sites. B https://web.pathway.md/diseases/recYAu9FO4o5hoaAg 2/11 6/23/23, 3:36 AM Cutaneous squamous cell carcinoma Pathway As per AAD 2018 guidelines, perform punch biopsy, shave biopsy, or excisional biopsy depending on the characteristics of the suspected malignancy (morphology, location) and the physician's judgment. B Show 4 more Sentinel lymph node biopsy: As per BAD 2021 guidelines: Consider performing sentinel lymph node biopsy only in specific, high-risk cases of primary cutaneous SCC in the context of a clinical trial or specialist skin cancer multidisciplinary team. E Obtain US-guided FNA cytology in patients with cutaneous SCC with clinically suspicious nodes. Consider repeating US-guided FNA cytology in case of negative results but remaining suspicion, although core or open-biopsy histology may be required. E As per ASTRO 2020 guidelines, consider performing sentinel lymph node biopsy to guide the need for and target of lymph node basin radiotherapy in patients with cutaneous SCC at high risk of regional nodal metastasis. E 5. Medical management Management of local disease, general principles: As per BAD 2021 guidelines: Take into consideration the risk factors for the patient and the margin, site, and tumor stage in patients with cutaneous SCC with 1 clear-but-close margins (< 1 mm). Consider offering observation in immunocompetent patients with cutaneous SCC with a low-risk tumor. B Offer active treatment in immunosuppressed patients with cutaneous SCC with 1 clear-but- close (< 1 mm) or involved margins with structured follow-up and surveillance. E As per AAD 2018 guidelines, develop a treatment plan taking into consideration recurrence rate, preservation of function, patient expectations, and potential adverse effects. B Management of local disease, topical therapies: As per BAD 2021 guidelines, insufficient evidence to recommend topical therapies in the treatment of cutaneous SCC. I As per AAD 2018 guidelines, do not offer topical therapies (imiquimod or 5-fluorouracil) in patients with cutaneous SCC. D Management of local disease, curettage and cautery: As per BAD 2021 guidelines: Consider performing curettage and cautery with curative intent in immunocompetent patients with small (< 1 cm), well-defined, nonrecurrent, clinically low-risk cutaneous SCC. C Review the histology of cutaneous SCC removed by curettage and cautery to identify high-risk or very high-risk features and, if identified, discuss with an appropriate multidisciplinary team regarding further management. E https://web.pathway.md/diseases/recYAu9FO4o5hoaAg 3/11 6/23/23, 3:36 AM Cutaneous squamous cell carcinoma Pathway As per EORTC/EADO 2020 guidelines, consider performing curettage and electrodesiccation in patients with small-sized and/or multiple invasive cutaneous SCCs in low-risk areas where surgery is impossible or has unacceptable consequences. C As per AAD 2018 guidelines, consider performing curettage and electrodesiccation in patients with low-risk primary cutaneous SCC in non-terminal hair-bearing locations. C Management of local disease, surgical excision: As per BAD 2021 guidelines, perform standard surgical excision as the first-line treatment option in patients with resectable primary cutaneous SCC. A Show 5 more As per EORTC/EADO 2020 guidelines, perform an excision with histological control as standard therapy aiming for complete excision (R0) with histological confirmation of peripheral and deep excision margins. B Show 4 more As per AAD 2018 guidelines: Perform standard excision with a 4-6 mm margin to a depth of the mid-subcutaneous adipose tissue with histologic margin assessment in patients with low-risk primary cutaneous SCC. B Consider performing standard excision in selected patients with high-risk tumors. Exercise strong caution when selecting a treatment modality for high-risk tumors without a complete margin assessment. C Management of local disease, Mohs micrographic surgery: As per BAD 2021 guidelines: Perform Mohs micrographic surgery in patients with cutaneous SCC with 1 involved margins or close margins (< 1 mm) where patient or tumor factors confer higher risk. A Consider performing Mohs micrographic surgery in selected patients with cutaneous SCC following specialist skin cancer multidisciplinary team discussion, particularly where tumor margins are difficult to delineate or in sites where tissue conservation is important for function. C As per EORTC/EADO 2020 guidelines, perform Mohs micrographic surgery/micrographically controlled surgery in patients with cutaneous SCC with high-risk factors. B As per AAD 2018 guidelines, perform Mohs micrographic surgery in patients with high-risk cutaneous SCC. B Management of local disease (elective lymph node dissection): do not perform elective lymph node dissection in patients with cutaneous SCC. D Management of local disease, cryotherapy: As per BAD 2021 guidelines, insufficient evidence to recommend cryotherapy in the treatment of cutaneous SCC. I As per EORTC/EADO 2020 guidelines, consider offering cryotherapy in patients with small- sized and/or multiple invasive cutaneous SCCs in low-risk areas where surgery is impossible or has unacceptable consequences. C https://web.pathway.md/diseases/recYAu9FO4o5hoaAg 4/11 6/23/23, 3:36 AM Cutaneous squamous cell carcinoma Pathway As per AAD 2018 guidelines, consider performing cryosurgery in patients with low-risk cutaneous SCC when more effective therapies are contraindicated or impractical. C Management of local disease, laser therapy: As per BAD 2021 guidelines, insufficient evidence to recommend CO laser therapy in the treatment of cutaneous SCC. I As per EORTC/EADO 2020 guidelines, consider offering laser therapy in patients with small- sized and/or multiple invasive cutaneous SCCs in low-risk areas where surgery is impossible or has unacceptable consequences. C As per AAD 2018 guidelines, insufficient evidence to recommend laser therapies or electronic surface brachytherapy in patients with cutaneous SCC. I Management of local disease, photodynamic therapy: As per EORTC/EADO 2020 guidelines, consider offering photodynamic therapy in patients with small-sized and/or multiple invasive cutaneous SCCs in low-risk areas where surgery is impossible or has unacceptable consequences. C As per BPG/BAD 2019 guidelines, consider offering photodynamic therapy as a treatment option in patients with microinvasive cutaneous SCC if surgery is contraindicated. C As per AAD 2018 guidelines, do not offer photodynamic therapy in patients with cutaneous SCC. D Management of local disease (adjuvant radiotherapy): consider offering adjuvant radiotherapy in patients with cutaneous SCC with clear-but-close (< 1 mm) pathological excision margins following a discussion at a skin cancer multidisciplinary team involving a clinical oncologist. C Show 3 more Management of local disease, definitive radiotherapy: As per BAD 2021 guidelines: Offer primary radiotherapy as treatment option in selected patients with cutaneous SCC, factoring in patient preference, following appropriate discussion at skin cancer multidisciplinary team and/or with a clinical or radiation oncologist. A Offer primary radiotherapy in patients with cutaneous SCC when surgery is not feasible or would be challenging or likely to result in an unacceptable functional or esthetic outcome. A As per ASTRO 2020 guidelines, offer definitive radiotherapy as a curative treatment modality in patients with cutaneous SCC unable to undergo or declining surgical resection. B Show 3 more As per AAD 2018 guidelines, consider offering radiotherapy, such as superficial radiotherapy, brachytherapy, external electron beam therapy, C and other traditional radiotherapy forms in patients with low-risk tumors if surgical therapy is not feasible or preferred, recognizing that the cure rate may be lower. C Management of locally advanced/metastatic disease, general principles: As per BAD 2021 guidelines: https://web.pathway.md/diseases/recYAu9FO4o5hoaAg 5/11 6/23/23, 3:36 AM Cutaneous squamous cell carcinoma Pathway Discuss cases of cutaneous SCC with symptomatic perineural invasion and/or radiological evidence of perineural invasion at a specialist skin cancer multidisciplinary. Consider obtaining skull base or head and neck multidisciplinary team opinion if the case is discussed at a skin multidisciplinary team. A Compose an individualized specialist skin cancer multidisciplinary team, multimodality, and imaging treatment plan for patients with any of the following: regional lymph node metastasis immunocompromised state and locally advanced and/or metastatic cutaneous SCC in-transit metastases from cutaneous SCC metastatic cutaneous SCC with further locoregional relapse following lymphadenectomy. A As per AAD 2018 guidelines, provide multidisciplinary consultation and management in patients with locoregional or distant metastases, particularly in patients with immunosuppression. Consider providing such consultation in patients with locally advanced disease without known metastases in some cases. B Management of locally advanced/metastatic disease, surgical resection: As per BAD 2021 guidelines: Consider performing surgical resection (with or without adjuvant radiotherapy) or primary radiotherapy in patients with locally recurrent cutaneous SCC. E Perform aggressive surgical excision of the involved nerve as the first step, where technically possible, followed by consideration of adjuvant radiotherapy in patients with cutaneous SCC with perineural invasion. A As per AAD 2018 guidelines, perform surgical resection, with or without adjuvant radiotherapy and possible systemic therapy, for regional lymph node metastases. Consider offering combination chemoradiotherapy in patients with inoperable disease. B Management of locally advanced/metastatic disease, lymph node dissection, general principles: As per BAD 2021 guidelines: Perform therapeutic regional lymphadenectomy in patients with cutaneous SCC with regional lymph node metastasis. A Consider performing regional lymphadenectomy or regional lymph node basin irradiation for disease control in selected patients, even in the presence of distant metastases, especially in patients undergoing multimodality treatment. E Perform regional lymphadenectomy by a designated surgeon of the specialist skin cancer multidisciplinary team pathway compliant with prevailing multispecialty guidance. A As per ASTRO 2020 guidelines, perform therapeutic lymphadenectomy followed by adjuvant radiotherapy in patients with cutaneous SCC metastasized to clinically apparent regional lymph nodes, except for patients having a single, small (< 3 cm) cervical lymph node harboring carcinoma without extracapsular extension. B As per EORTC/EADO 2020 guidelines, perform regional therapeutic lymph node dissection in clinically or radiologically detected lymph node metastasis confirmed with cytology or biopsy. B Show 2 more https://web.pathway.md/diseases/recYAu9FO4o5hoaAg 6/11 6/23/23, 3:36 AM Cutaneous squamous cell carcinoma Pathway Management of locally advanced/metastatic disease (lymph node dissection, head and neck region): perform therapeutic regional lymphadenectomy in patients with head and neck cutaneous SCC with regional lymph node metastasis. A Show 4 more Management of locally advanced/metastatic disease (lymph node dissection, non-head and neck region): perform therapeutic regional lymphadenectomy in patients with non-head and neck cutaneous SCC with regional lymph node metastases in axillary, inguinofemoral, or other peripheral draining nodes. A Show 3 more Management of locally advanced/metastatic disease, adjuvant radiotherapy: As per BAD 2021 guidelines: Offer adjuvant radiotherapy following therapeutic regional lymphadenectomy in patients with cutaneous SCC with high-risk pathology ( pN1; such as 2 nodes, large nodes, and extracapsular extension). A Consider offering adjuvant radiotherapy following surgical excision of the involved nerve in patients with cutaneous SCC with perineural invasion. B As per ASTRO 2020 guidelines, consider offering postoperative radiotherapy in patients with cutaneous SCC with close or positive margins that cannot be corrected with further surgery (secondary to morbidity or adverse cosmetic outcome). B Show 6 more As per EORTC/EADO 2020 guidelines: Consider offering adjuvant radiotherapy in patients with cutaneous SCC of the head and neck with regional nodal metastases and extracapsular extension. C Consider offering postoperative radiotherapy after surgical excision of tumors with positive margins if re-excision is not possible. C Management of locally advanced/metastatic disease, definitive radiotherapy: As per BAD 2021 guidelines, consider offering conformal radiotherapy including the entire course of the involved nerve in patients with cutaneous SCC with symptomatic perineural invasion and/or radiological evidence of perineural invasion if surgery is inappropriate, after carefully weighing the benefits and side effects from radiotherapy to such an extensive radiotherapy treatment field. C As per ASTRO 2020 guidelines, consider offering elective lymph node basin irradiation only in patients with cutaneous SCC at high risk of regional nodal metastasis (thickness > 6 mm) undergoing radiotherapy to the primary site with an overlap of the adjacent nodal basin. C Show 3 more As per EORTC/EADO 2020 guidelines, consider offering primary radiotherapy as an alternative to surgery in patients with inoperable or difficult-to-operate tumors or in the absence of consent to surgical excision. C Management of locally advanced/metastatic disease, systemic therapy: As per BAD 2021 guidelines: https://web.pathway.md/diseases/recYAu9FO4o5hoaAg 7/11 6/23/23, 3:36 AM Cutaneous squamous cell carcinoma Pathway Consider offering immune checkpoint inhibitors in patients with locally advanced cutaneous SCC if curative surgery or radiotherapy is not reasonable and in patients with metastatic cutaneous SCC, except patients with organ transplants or having significant autoimmune conditions. C Consider offering systemic chemotherapy or epidermal growth factor receptor inhibitors in patients with metastatic cutaneous SCC with contraindications to immune checkpoint inhibitors. C As per ASTRO 2020 guidelines: Do not offer concurrent carboplatin in addition to adjuvant radiotherapy in patients with resected locally advanced cutaneous SCC. D Consider offering concurrent drug therapies in addition to definitive radiotherapy in patients with unresected locally advanced cutaneous SCC. C As per EORTC/EADO 2020 guidelines, offer a PD-1 antibody as first-line therapy in patients with locally advanced or metastatic cutaneous SCC ineligible for curative surgery or radiotherapy. B Show 2 more As per AAD 2018 guidelines, consider offering epidermal growth factor inhibitors and cisplatin, as a single agent or in combination therapy, in patients with metastatic disease. C Management of locally advanced/metastatic disease (laser therapy): insufficient evidence to recommend laser therapies or electronic surface brachytherapy in patients with cutaneous SCC. I Management of locally advanced/metastatic disease, photodynamic therapy: As per BPG/BAD 2019 guidelines, do not offer photodynamic therapy as a treatment option in patients with invasive cutaneous SCC. As per AAD 2018 guidelines, do not offer photodynamic therapy in patients with cutaneous SCC. D Management of locally advanced/metastatic disease, palliative care: As per BAD 2021 guidelines: Consider offering electrochemotherapy in patients with locally advanced cutaneous SCC in palliative settings if other local or systemic therapies are not appropriate. E Consider offering best supportive care in elderly and frail patients with metastatic cutaneous SCC since responses to systemic therapy are generally short-lived, and chemotherapy is poorly tolerated. C As per EORTC/EADO 2020 guidelines: Assess nutritional, psychological, social, and existential needs to improve the overall quality of life in the palliative setting. B Provide supportive care to all patients with skin cancer, including reduction of symptoms, prevention/treatment of infection, control of bleeding, and adequate pain management. B https://web.pathway.md/diseases/recYAu9FO4o5hoaAg 8/11 6/23/23, 3:36 AM Cutaneous squamous cell carcinoma Pathway As per AAD 2018 guidelines, provide palliative and best supportive care to optimize symptom management and maximize the quality of life in patients with advanced disease. B 6. Patient education Pretreatment counseling: Discuss the risks and benefits of all treatment options (outcomes, function, cosmesis) with patients with cutaneous SCC and their families/carers and ensure making treatment decisions together. E Inform younger patients with cutaneous SCC (age < 60 years), especially organ transplant recipients, about the very low risk of radiation-induced, in-field malignancy in the future. Take this risk into account when making any treatment decision. E 7. Preventative measures Primary prevention: As per AAD 2018 guidelines: Counsel persons with fair skin types aged 6 months to 24 years and parents of young children about minimizing exposure to UV radiation to reduce the risk of skin cancer. B Offer counseling selectively in adults > 24 years with fair skin types about minimizing exposure to UV radiation to reduce the risk of skin cancer, taking into consideration the presence of risk factors for skin cancer. B Primary prevention (EDA): consider offering field photodynamic therapy as prophylaxis to reduce the emergence of new lesions in patients with actinic keratosis or non-melanoma skin cancer, including patients with organ transplantation. C Secondary prevention: do not offer topical and oral retinoids, such as tretinoin, D retinol, D acitretin, D and isotretinoin D , to reduce the incidence of keratinocyte cancers in patients with a history of cutaneous SCC, unless they are solid organ transplant recipients. Consider offering acitretin only in solid organ transplant recipients. D Show 2 more 8. Follow-up and surveillance Skin reconstruction after tumor resection (timing): consider performing reconstructive surgery in a delayed (asynchronous) fashion in adult patients after skin cancer resection. C Skin reconstruction after tumor resection (perioperative antibiotics): consider discussing the management of antibiotics with adult patients undergoing reconstruction after skin cancer resection when relevant. C Show 3 more https://web.pathway.md/diseases/recYAu9FO4o5hoaAg 9/11 6/23/23, 3:36 AM Cutaneous squamous cell carcinoma Pathway Skin reconstruction after tumor resection (perioperative antithrombotics): consider discussing the management of anticlotting agents with adult patients undergoing reconstruction after skin cancer resection when relevant. C Show 2 more Skin reconstruction after tumor resection (perioperative analgesics): consider discussing the management of pain with adult patients undergoing reconstruction after skin cancer resection when relevant. C Show 2 more Skin reconstruction after tumor resection (follow-up): consider obtaining postoperative follow- up assessment in adult patients undergoing reconstruction after skin cancer resection. C Surveillance after curative-intent therapy: As per BAD 2021 guidelines, do not obtain a routine discussion at a multidisciplinary team for cases of T1 cutaneous SCC excised with histologically clear margins of at least 1 mm without other high-risk factors. D Show 5 more As per EADO 2020 guidelines: Perform regular clinical examinations, including inspection of the entire skin and inspection and palpation of the excision site, the in-transit route, and the regional lymph nodes, during follow-up of all patients. B Decide on the frequency of follow-up visits and imaging depending on underlying risk characteristics of cutaneous SCC, including low-risk or high-risk common primary, advanced or regional disease, immunosuppression setting. B Surveillance for future malignancies: As per BAD 2021 guidelines, educate patients with cutaneous SCC on self-examination (skin and lymph nodes) and sun protection by providing appropriate verbal and written information. E As per EADO 2020 guidelines, obtain surveillance for recurrences and development of new non- melanoma skin cancer and melanoma in patients with a history of cutaneous SCC. B As per AAD 2018 guidelines: Counsel patients with a history of cutaneous SCC on skin self-examination and sun protection. B Obtain screening for new keratinocyte cancers (basal cell carcinoma or cutaneous SCC) and melanoma on at least an annual basis after diagnosis of first SCC. A References 1. Andrew Chen, John G Albertini, Jeremy S Bordeaux et al. Evidence-Based Clinical Practice Guideline: Reconstruction after Skin Cancer Resection. Plast Reconstr Surg. 2021 May 1;147 5 812e-829e. Open https://web.pathway.md/diseases/recYAu9FO4o5hoaAg 10/11 6/23/23, 3:36 AM Cutaneous squamous cell carcinoma Pathway 2. Alexander J Stratigos, Claus Garbe, Clio Dessinioti et al. European interdisciplinary guideline on invasive squamous cell carcinoma of the skin: Part 2. Treatment. Eur J Cancer. 2020 Mar;128 83 102. Open 3. Work Group, Invited Reviewers, John Y S Kim et al. Guidelines of care for the management of cutaneous squamous cell carcinoma. J Am Acad Dermatol. 2018 Mar;78 3 560 578. Open 4. S G Keohane, J Botting, P G Budny et al. British Association of Dermatologists guidelines for the management of people with cutaneous squamous cell carcinoma 2020. Br J Dermatol. 2021 Mar;184 3 401 414. Open 5. T H Wong, C A Morton, N Collier et al. British Association of Dermatologists and British Photodermatology Group guidelines for topical photodynamic therapy 2018. Br J Dermatol. 2019 Apr;180 4 730 739. Open 6. Anna Likhacheva, Musaddiq Awan, Christopher A Barker et al. Definitive and Postoperative Radiation Therapy for Basal and Squamous Cell Cancers of the Skin: Executive Summary of an American Society for Radiation Oncology Clinical Practice Guideline. Pract Radiat Oncol. Jan-Feb 2020;10 1 8 20. Open 7. US Preventive Services Task Force, Grossman DC, Curry SJ et al. Behavioral Counseling to Prevent Skin Cancer: US Preventive Services Task Force Recommendation Statement. JAMA. 2018 Mar 20;319 11 1134 1142. Open 8. US Preventive Services Task Force, Carol M Mangione, Michael J Barry et al. Screening for Skin Cancer: US Preventive Services Task Force Recommendation Statement. JAMA. 2023 Apr 18;329 15 1290 1295. Open 9. Ashish Sharma, Andrew J Birnie, Cristina Bordea et al. British Association of Dermatologists guidelines for the management of people with cutaneous squamous cell carcinoma in situ Bowen disease) 2022. Br J Dermatol. 2023 Feb 10;188 2 186 194. Open 10. C A Morton, R M Szeimies, N Basset-Seguin et al. European Dermatology Forum guidelines on topical photodynamic therapy 2019 Part 1 treatment delivery and established indications - actinic keratoses, Bowen's disease and basal cell carcinomas. J Eur Acad Dermatol Venereol. 2019 Dec;33 12 2225 2238. Open 11. Anokhi Jambusaria-Pahlajani, Peter A Kanetsky, Pritesh S Karia et al. Evaluation of AJCC tumor staging for cutaneous squamous cell carcinoma and a proposed alternative tumor staging system. JAMA Dermatol. 2013 Apr;149 4 402 10. Open https://web.pathway.md/diseases/recYAu9FO4o5hoaAg 11/11 |
Guideline sources The following summarized guidelines for the management of cutaneous warts are prepared by our editorial team based on guidelines from the British Association of Dermatologists (BAD 2014) and the American Association of Family Physicians (AAFP 2011). 1 2 Guidelines 1. Medical management Salicylic acid: As per BAD 2014 guidelines, apply salicylic acid for the treatment of patients with cutaneous warts. B As per AAFP 2011 guidelines, apply salicylic acid as first-line therapy in patients with cutaneous warts. B Other destructive agents: Insufficient evidence to support the use of other destructive agents for the treatment of patients with cutaneous warts, such as: phenol I cantharidin I glycolic acid 5% I pyruvic acid I trichloroacetic and monochloroacetic acid I https://web.pathway.md/diseases/rec0CDKIPO2wSvEgm 1/4 6/23/23, 3:36 AM Cutaneous warts Pathway trichloroacetic and monochloroacetic acid I silver nitrate I citric acid 50% I formic acid. I Virucidal agents: insufficient evidence to support the use of virucidal agents, such as formaldehyde or glutaraldehyde, for the treatment of patients with cutaneous warts. I Antiproliferative agents: Consider applying topical 5-fluorouracil or bleomycin for the treatment of patients with cutaneous warts. C Insufficient evidence to support the use of other antiproliferative agents for the treatment of patients with cutaneous warts, such as: dithranol I topical retinoids I systemic retinoids I vitamin D analogues I podophyllin and podophyllotoxin I cidofovir. I Immunological therapy: Insufficient evidence to support the use of the following immunological agents for the treatment of patients with cutaneous warts: H2RAs I zinc oxide and zinc sulfate I imiquimod I systemic immunotherapy agents such as interferon, IgAnd valaciclovir. I 2. Nonpharmacologic interventions Duct tape: As per BAD 2014 guidelines, insufficient evidence to support the use of occlusotherapy in patients with cutaneous warts. I As per AAFP 2011 guidelines, do not use duct tapes for the treatment of patients with cutaneous warts. D Alternative and complementary therapies: Insufficient evidence to support the use of complementary and alternative treatment options in patients with cutaneous warts, such as: psychological treatments I herbal treatments I homeopathy I acupuncture I https://web.pathway.md/diseases/rec0CDKIPO2wSvEgm 2/4 6/23/23, 3:36 AM Cutaneous warts Pathway 3. Therapeutic procedures Cryotherapy: As per BAD 2014 guidelines, consider performing cryotherapy for the treatment of patients with cutaneous warts. C As per AAFP 2011 guidelines, perform cryotherapy with liquid nitrogen as first-line therapy in patients with cutaneous warts. B Show 2 more Intralesional injections: As per BAD 2014 guidelines: Consider performing intralesional injections of 5-fluorouracil or bleomycin for the treatment of patients with cutaneous warts. C Insufficient evidence to support the use of intralesional immunotherapy in patients with cutaneous warts. I As per AAFP 2011 guidelines: Consider performing intralesional injections with Candida or mumps skin antigen for the treatment of patients with recalcitrant warts with a positive skin antigen pretest. B Consider performing intralesional injections with bleomycin for the treatment of patients with recalcitrant warts, although the effectiveness is unproven. B Photodynamic therapy: As per BAD 2014 guidelines, insufficient evidence to support the use of photodynamic therapy for the treatment of patients with cutaneous warts. I As per AAFP 2011 guidelines, consider performing photodynamic therapy with ALA plus applying topical salicylic acid for the treatment of patients with recalcitrant warts. B Pulsed dye laser: As per BAD 2014 guidelines, consider performing pulsed dye laser for the treatment of patients with cutaneous warts. C As per AAFP 2011 guidelines, consider performing pulsed dye laser for the treatment of patients with recalcitrant warts, although the effectiveness is unproven. B Hyperthermia: insufficient evidence to support the use of hyperthermia in patients with cutaneous warts. I Contact immunotherapy: consider offering contact immunotherapy for the treatment of patients with cutaneous warts. C 4. Surgical interventions Surgical methods: insufficient evidence to support the use of surgical interventions in patients with cutaneous warts. I https://web.pathway.md/diseases/rec0CDKIPO2wSvEgm 3/4 6/23/23, 3:36 AM Cutaneous warts Pathway References 1. J C Sterling, S Gibbs, S S Haque Hussain et al. British Association of Dermatologists' guidelines for the management of cutaneous warts 2014. Br J Dermatol. 2014 Oct;171 4 696 712. Open 2. Elie Mulhem, Susanna Pinelis. Treatment of nongenital cutaneous warts. Am Fam Physician. 2011 Aug 1;84 3 288 293. Open https://web.pathway.md/diseases/rec0CDKIPO2wSvEgm 4/4 |
Guideline sources The following summarized guidelines for the evaluation and management of cystic fibrosis (CF) are prepared by our editorial team based on guidelines from the American Diabetes Association (ADA 2023), the National Society of Genetic Counselors (NSGC 2014), the Clinical Pharmacogenetics Implementation Consortium (CPIC 2014), and the Cystic Fibrosis Foundation (CFF 2013). 1 2 3 4 5 5 5 6 Definition CF is an inherited multiorgan disease characterized by recurrent cough, wheeze, and pneumonia, sinusitis, and male infertility. 5 Epidemiology CF is caused by a mutation in the CF transmembrane conductance regulator gene. 5 Disease course Genetic mutations in CF transmembrane conductance regulator gene result in CF, which causes clinical manifestations of recurrent respiratory symptoms of cough, wheeze, and pneumonias, nasal polyps, sinusitis, finger clubbing, male infertility, exocrine pancreatic insufficiency (steatorrhea, diarrhea, abdominal distension, poor growth in infants, pancreatitis), GERD, meconium ileus, distal intestinal obstruction syndrome, celiac disease, malabsorption, Crohn's disease, constipation, rectal https://web.pathway.md/diseases/recQObj753riIIE2o 1/5 6/23/23, 3:40 AM Cystic fibrosis Pathway prolapse, fatty liver, cirrhosis, diabetes, osteopenia, acute collapse, vaginal candidiasis, and stress incontinence. Disease progression may lead to death. 5 Prognosis and risk of recurrence The in-hospital mortality of CF is 0.71%. 6 Guidelines 1. Screening and diagnosis Carrier testing: Offer carrier testing for CF to: women of reproductive age, regardless of ancestry, and preferably preconceptionally individuals with a family history of CF partners of mutation carriers and people with CF. 2. Diagnostic investigations Genetic testing: review and re-interpret prior carrier screening results in light of current knowledge when individuals present for genetic counseling. The inclusion and exclusion of mutations on available CFTR mutation screening panels remains a dynamic process as new information is learned about the pathogenicity of CFTR mutations. Screening for diabetes: Start annual screening for CF-related diabetes with an oral glucose tolerance by age 10 years in all patients with CF not previously diagnosed with CF-related diabetes. B Do not obtain Hgb A1C as a screening test for CF-related diabetes. D 3. Medical management Ivacaftor: As per CPIC 2014 guidelines, use ivacaftor according to the product label (150 mg every 12 h) for patients 6 years without other diseases; adjust dosing as appropriate in patients with hepatic impairment. A As per CFF 2013 guidelines, administer of ivacaftor to improve lung function and quality of life and reduce exacerbations in patients with CF 6 years of age with at least one G551D CFTR mutation. A Dornase alfa: offer dornase alfa to improve lung function and quality of life, and reduce exacerbations, for individuals with CF, 6 years of age, and with moderate-to-severe lung disease. A https://web.pathway.md/diseases/recQObj753riIIE2o 2/5 6/23/23, 3:40 AM Cystic fibrosis Pathway Inhaled hypertonic saline: implement chronic inhaled hypertonic saline therapy to improve lung function and quality of life and reduce exacerbations in individuals with CF, 6 years of age. B Inhaled tobramycin: offer the chronic use of inhaled tobramycin to improve lung function and quality of life, and reduce exacerbations for patients with CF, 6 years of age, with moderate-to- severe lung disease and P. aeruginosa persistently present in cultures of the airways. A Inhaled aztreonam: initiate chronic inhaled aztreonam therapy to improve lung function and quality of life in patients with CF 6 years of age with moderate-to-severe lung disease and P. aeruginosa persistently present in cultures of the airways. A Other inhaled antibiotics: insufficient evidence to recommend for or against the chronic use of other inhaled antibiotics including carbenicillin, ceftazidime, colistin, or gentamicin to improve lung function and quality of life or reduce exacerbations, in patients with CF 6 years of age, with P. aeruginosa persistently present in cultures of the airways. I Prophylactic azithromycin: implement chronic azithromycin therapy to improve lung function and reduce exacerbations for patients 6 years of age with CF and P. aeruginosa persistently present in cultures of the airways. B Other prophylactic oral antibiotics: insufficient evidence to recommend for or against the routine use of chronic oral antipseudomonal antibiotics to improve lung function and quality of life or reduce exacerbations, in patients with CF 6 years of age with P. aeruginosa persistently present in cultures of the airways. I Inhaled corticosteroids: avoid routine use of ICSs to improve lung function or quality of life and reduce pulmonary exacerbations, for individuals with CF, 6 years of age, without asthma or allergic bronchopulmonary aspergillosis. D Oral corticosteroids: avoid the chronic use of oral corticosteroids to improve lung function, quality of life, or reduce exacerbations for individuals with CF 6 years of age without asthma or allergic bronchopulmonary aspergillosis. D Leukotriene modifiers: insufficient evidence to recommend for or against the routine chronic use of leukotriene modifiers to improve lung function and quality of life or reduce exacerbations, in patients with CF 6 years of age. I Inhaled anticholinergics: insufficient evidence to recommend for or against the chronic use of inhaled anticholinergic bronchodilators to improve lung function and quality of life or reduce exacerbations in patients with CF 6 years of age. I Inhaled beta-2 adrenergic receptor agonists: insufficient evidence to recommend for or against chronic use of inhaled -2 adrenergic receptor agonists to improve lung function and quality of life or reduce exacerbations in patients with CF 6 years of age. I Oral antistaphylococcal antibiotics: insufficient evidence to recommend for or against the chronic use of oral antistaphylococcal antibiotics to improve lung function and quality of life or reduce exacerbations in individuals with CF, 6 years of age and older, with S. aureus persistently present in cultures of the airways. I https://web.pathway.md/diseases/recQObj753riIIE2o 3/5 6/23/23, 3:40 AM Cystic fibrosis Pathway N-acetylcysteine and glutathione: insufficient evidence to recommend for or against the chronic use of inhaled or oral N-acetylcysteine or inhaled glutathione to improve lung function and quality of life or reduce exacerbations in patients with CF 6 years of age. I Ibuprofen: initiate chronic oral ibuprofen, at a peak plasma concentration of 50-100 mg/mL, to slow the loss of lung function, for patients with CF 6-17 years of age with an FEV in 1 second 60% of predicted. B 4. Specific circumstances Patients with cystic fibrosis-related diabetes: Initiate insulin to attain individualized glycemic goals in patients with CF-related diabetes. A Obtain annual monitoring for complications beginning 5 years after the diagnosis of CF-related diabetes. B 5. Patient education Post-carrier screening counseling: genetic counselors offering CF carrier screening should ensure that they are providing the most current information to patients regarding the range of symptoms, potential treatment options, and quality of life issues associated with CF and the CFTR-related disease spectrum. References 1. Mogayzel PJ Jr, Naureckas ET, Robinson KA et al. Cystic fibrosis pulmonary guidelines. Chronic medications for maintenance of lung health. Am J Respir Crit Care Med. 2013 Apr 1;187 7 680 9. Open 2. Steven E. Kahn, Cheryl A.M. Anderson, John B. Buse et al. Standards of Care in Diabetes 2023. Diabetes Care. 2023 Jan;46 Supplement_1 S1 S291. Open 3. Langfelder-Schwind E, Karczeski B, Strecker MN et al. Molecular testing for cystic fibrosis carrier status practice guidelines: recommendations of the National Society of Genetic Counselors. J Genet Couns. 2014 Feb;23 1 5 15. Open 4. Clancy JP, Johnson SG, Yee SW et al. Clinical Pharmacogenetics Implementation Consortium CPIC guidelines for ivacaftor therapy in the context of CFTR genotype. Clin Pharmacol Ther. 2014 Jun;95 6 592 7. Open 5. Jane C Davies, Eric W F W Alton, Andrew Bush. Cystic fibrosis. 2007 Dec 15;335 7632 1255 9.2007 Dec 15;335 7632 1255 9. Open 6. Abhinav Agrawal, Abhishek Agarwal, Dhruv Mehta et al. Nationwide trends of hospitalizations for cystic fibrosis in the United States from 2003 to 2013. 2017 Aug;6 3 191 198.2017 Aug;6 3 191 198. Open 7. Mogayzel PJ Jr, Naureckas ET, Robinson KA et al. Cystic Fibrosis Foundation pulmonary guideline. pharmacologic approaches to prevention and eradication of initial Pseudomonas aeruginosa infection. Ann Am Thorac Soc. 2014 Dec;11 10 1640 50. Open https://web.pathway.md/diseases/recQObj753riIIE2o 4/5 6/23/23, 3:40 AM Cystic fibrosis Pathway 8. Saiman L, Siegel JD, LiPuma JJ et al. Infection prevention and control guideline for cystic fibrosis: 2013 update. Infect Control Hosp Epidemiol. 2014 Aug;35 Suppl 1 S1 S67. Open 9. Lahiri T, Hempstead SE, Brady C et al. Clinical Practice Guidelines From the Cystic Fibrosis Foundation for Preschoolers With Cystic Fibrosis. Pediatrics. 2016 Apr;137 4 . pii: e20151784. Open 10. Abhinav Agrawal, Abhishek Agarwal, Dhruv Mehta et al. Nationwide trends of hospitalizations for cystic fibrosis in the United States from 2003 to 2013. Intractable Rare Dis Res. 2017 Aug; 6 3 191 198. Open 11. Jane C Davies, Eric W F W Alton, and Andrew Bush. Cystic fibrosis. BMJ. 2007 Dec 15; 335 7632 1255 1259. Open 12. Philip M Farrell, Terry B White, Clement L Ren et al. Diagnosis of Cystic Fibrosis: Consensus Guidelines from the Cystic Fibrosis Foundation. J Pediatr. 2017 Feb;181S S4 S15.e1. Open 13. Clement L Ren, Rebecca L Morgan, Christopher Oermann et al. Cystic Fibrosis Foundation Pulmonary Guidelines. Use of Cystic Fibrosis Transmembrane Conductance Regulator Modulator Therapy in Patients with Cystic Fibrosis. Ann Am Thorac Soc. 2018 Mar;15 3 271 280. Open 14. R Andres Floto, Kenneth N Olivier, Lisa Saiman et al. US Cystic Fibrosis Foundation and European Cystic Fibrosis Society consensus recommendations for the management of non-tuberculous mycobacteria in individuals with cystic fibrosis. Thorax. 2016 Jan;71 Suppl 1 Suppl 1):i1 22. Open https://web.pathway.md/diseases/recQObj753riIIE2o 5/5 |
Guideline sources The following summarized guidelines for the evaluation and management of deep vein thrombosis are prepared by our editorial team based on guidelines from the American Society of Hematology (ASH 2023; 2020; 2018), the European Association for the Study of the Liver (EASL 2022), the European Society for Vascular Surgery (ESVS 2021), the American College of Chest Physicians (ACCP 2021; 2016), the Society for Vascular Medicine (SVM/AHA/ACC/ACS/ACCP/SIR 2020), the International Initiative on Thrombosis and Cancer (ITAC 2019), the Interdisciplinary Expert Panel on Iliofemoral Deep Vein Thrombosis (InterEPID 2015), the British Committee for Standards In Haematology (BCSH 2015), the International Society on Thrombosis and Haemostasis (ISTH 2014), the American Association of Family Physicians (AAFP 2013), and the Society for Vascular Surgery (SVS 2012). 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 15 15 16 Definition Deep vein thrombosis is the formation of thrombus within the deep veins of lower extremity characterized by lower extremity pain and swelling and calf tenderness. 15 https://web.pathway.md/diseases/recy7Y18EJ5OGaopy 1/17 6/29/23, 10:06 PM Deep vein thrombosis Pathway Epidemiology Deep vein thrombosis is caused by thrombus formation due to venous stasis, endothelial injury, and blood hypercoagulability. 15 Disease course Thrombus formation due to venous stasis, endothelial injury, and blood hypercoagulability in the lower extremities results in deep vein thrombosis, which causes clinical manifestations of lower extremity pain, swelling, and calf tenderness. Disease progression may lead to postphlebitis syndrome and pulmonary hypertension; acute complication may result in PE that might prove fatal. 15 Prognosis and risk of recurrence The 90-day mortality rates of asymptomatic proximal deep vein thrombosis and asymptomatic distal deep vein thrombosis are 13.75% and 3.39%, respectively. 16 Calculator Calculator Calculat Caprini Score for venous thromb DASH score for recurrent VTE Geneva Guidelines 1. Classification and risk stratification Anatomic classification: use precise anatomic terminology to characterize the most proximal extent of venous thrombosis as involving the iliofemoral veins, with or without extension into the IVC, the femoropopliteal veins, or isolated to the calf veins, rather than a simple characterization of a thrombus as proximal or distal. A 2. Diagnostic investigations Assessment of pretest probability: obtain clinical assessment of the pretest probability as part of the diagnostic process in patients with suspected deep vein thrombosis. B Diagnostic imaging: As per ESVS 2021 guidelines, obtain ultrasound as the first-line modality in patients with suspected deep vein thrombosis requiring imaging. B Show 4 more As per AAFP 2013 guidelines, obtain MRV of the lower extremity and pelvis in patients with unilateral left leg swelling and negative results on duplex ultrasound if the clinical suspicion for DVT is high (in the context of suspected May-Thurner syndrome). B https://web.pathway.md/diseases/recy7Y18EJ5OGaopy 2/17 6/29/23, 10:06 PM Deep vein thrombosis Pathway As per SVS 2012 guidelines, obtain adjunctive imaging modalities (such as CTV or MRV) in patients with suspected iliofemoral venous thrombosis not confirmed with standard diagnostic modalities such as venous ultrasound imaging. B Evaluation for occult pulmonary embolism: do not evaluate for occult PE routinely in patients with deep vein thrombosis in the absence of symptoms or signs. D Evaluation for occult malignancy: As per ESVS 2021 guidelines, perform clinical examination and obtain sex-specific cancer screening, as opposed to routine extensive screening, for occult malignancy in patients with unprovoked deep vein thrombosis. A As per BCSH 2015 guidelines, consider obtaining CT (and mammography in females) for screening of cancer in > 40 years old patients with unprovoked VTE. Set a lower threshold for screening patients with bilateral deep vein thrombosis, very high D-dimers, or an early recurrence of VTE. C Landmark trials: SOME In patients who had a first unprovoked VTE, limited occult-cancer screening plus CT was not superior to limited occult-cancer screening with respect to occult cancer diagnosis at 1 year. Carrier M et al. N Engl J Med. 2015 Aug 20. Evaluation for thrombophilia: do not obtain thrombophilia testing in patients with provoked deep vein thrombosis. D Show 3 more 3. Medical management Setting of care: As per ESVS 2021 guidelines, offer outpatient management in most patients with deep vein thrombosis. A As per ASH 2020 guidelines, consider offering home-based rather than hospital treatment in patients with uncomplicated deep vein thrombosis. C Indications for anticoagulation: As per ACCP 2021 guidelines, consider obtaining serial imaging (repeated ultrasound once weekly or with worsening symptoms) of the deep veins for 2 weeks rather than initiating anticoagulation in patients with acute isolated distal deep vein thrombosis of the leg and without severe symptoms or risk factors for extension. C Show 3 more As per ESVS 2021 guidelines, consider deciding on the initiation of anticoagulation treatment in patients with calf deep vein thrombosis based on the patient's symptoms, risk factors for progression, and bleeding risk. C Administer a 3-month anticoagulant therapy in patients with symptomatic calf deep vein thrombosis requiring anticoagulation. B https://web.pathway.md/diseases/recy7Y18EJ5OGaopy 3/17 6/29/23, 10:06 PM Deep vein thrombosis Pathway Choice of anticoagulation, initial management, provoked thrombosis: As per ACCP 2021 guidelines, administer apixaban, dabigatran, edoxaban, or rivaroxaban rather than vitamin K antagonists for the treatment phase (first 3 months) in patients with deep vein thrombosis of the leg. B As per ESVS 2021 guidelines: Administer direct OACs rather than vitamin K antagonists for the principal treatment phase of patients with provoked proximal deep vein thrombosis. A Administer direct OACs rather than LMWH followed by vitamin K antagonists in patients with calf deep vein thrombosis requiring anticoagulation. B As per ASH 2020 guidelines: Consider administering direct OACs rather than vitamin K antagonists in patients with deep vein thrombosis and/or PE. C Insufficient evidence to recommend one direct OAC over another in patients with deep vein thrombosis and/or PE. I Choice of anticoagulation, initial management, unprovoked thrombosis: As per ACCP 2021 guidelines, administer oral Xa inhibitors (apixaban, edoxaban, rivaroxaban) rather than LMWH for the initiation and treatment phases in patients with acute deep vein thrombosis and cancer (cancer-associated thrombosis). B Show 2 more Landmark trials: CLOT In patients with cancer who had acute, symptomatic proximal deep vein thrombosis, PE, or both, subcutaneous dalteparin was superior to oral anticoagulation with respect to recurrent VTE at 6 months. Lee AY et al. N Engl J Med. 2003 Jul 10. As per ESVS 2021 guidelines: Administer LMWH for the initial and principal treatment in patients with cancer-associated deep vein thrombosis. A Consider administering approved direct OACs for the initial and principal treatment in selected patients with cancer-associated deep vein thrombosis, with the malignancy not located in the gastrointestinal or genitourinary systems. B As per ESVS 2021 guidelines, administer direct OACs rather than LMWH followed by a vitamin K antagonist for the principal treatment phase in patients with unprovoked proximal deep vein thrombosis. A Choice of anticoagulation, extended therapy: As per ACCP 2021 guidelines, administer reduced-dose direct OACs rather than aspirin in patients requiring extended-phase anticoagulation. B Show 3 more As per ESVS 2021 guidelines, consider administering direct OACs rather than vitamin K antagonists in patients with unprovoked proximal deep vein thrombosis requiring extended https://web.pathway.md/diseases/recy7Y18EJ5OGaopy 4/17 6/29/23, 10:06 PM Deep vein thrombosis Pathway anticoagulation beyond 3 months. C Show 2 more As per ASH 2020 guidelines: Target an INR range of 2-3 over a lower range (such as 1.5-1.9) in patients with deep vein thrombosis and/or PE completed primary treatment and continuing vitamin K antagonist therapy for secondary prevention. B Consider using a standard- or a lower-dose direct OAC in patients with deep vein thrombosis and/or PE completed primary treatment and continuing direct OAC for secondary prevention. C Duration of anticoagulation, provoked thrombosis: As per ASH 2023 guidelines, avoid obtaining thrombophilia testing to determine the duration of anticoagulant treatment after primary short-term treatment in patients with VTE provoked by surgery. D Show 2 more As per ACCP 2021 guidelines, complete a 3-month treatment phase of anticoagulation in patients with acute deep vein thrombosis in the absence of contraindications. Assess the need for extended-phase therapy upon completion of the 3-month treatment phase of therapy. B Show 2 more As per ESVS 2021 guidelines, complete a 3-month rather than a shorter A or longer duration of anticoagulation treatment in patients with provoked proximal deep vein thrombosis and a major transient risk factor. B Show 2 more As per ASH 2020 guidelines, consider completing a shorter (3-6 months) over a longer (6-12 months) course of anticoagulation for primary treatment in patients with deep vein thrombosis and/or PE, whether provoked by a transient risk factor or by a chronic risk factor. C Show 3 more Duration of anticoagulation, unprovoked thrombosis: As per ASH 2023 guidelines: Avoid obtaining thrombophilia testing to guide the duration of anticoagulant treatment after primary short-term treatment in patients with unprovoked VTE. D Avoid obtaining thrombophilia testing to guide anticoagulant treatment duration after primary short-term treatment in patients with an unspecified type of VTE. D As per ACCP 2021 guidelines, complete a 3-month treatment phase of anticoagulation in patients with acute deep vein thrombosis in the absence of contraindications. Assess the need for extended-phase therapy upon completion of the 3-month treatment phase of therapy. B Show 3 more As per ESVS 2021 guidelines, reassess the bleeding risk before continuing anticoagulation beyond 3 months in patients with unprovoked deep vein thrombosis. B Show 3 more https://web.pathway.md/diseases/recy7Y18EJ5OGaopy 5/17 6/29/23, 10:06 PM Deep vein thrombosis Pathway As per ASH 2020 guidelines, consider completing a shorter (3-6 months) over a longer (6-12 months) course of anticoagulation for primary treatment in patients with unprovoked deep vein thrombosis and/or PE. C Show 2 more Aspirin for extended treatment: As per ACCP 2021 guidelines, consider initiating aspirin to prevent recurrent VTE in patients with unprovoked proximal deep vein thrombosis after stopping anticoagulant therapy and not having contraindications to aspirin. C Landmark trials: ASPIRE In patients who had completed initial anticoagulant therapy after a first episode of unprovoked VTE, aspirin was not superior to placebo with respect to the incidence of recurrent VTE. Brighton TA et al. N Engl J Med. 2012 Nov 22. As per ESVS 2021 guidelines, do not use aspirin for extended antithrombotic therapy in patients with unprovoked deep vein thrombosis. D As per ASH 2020 guidelines: Consider initiating anticoagulation over aspirin in patients with deep vein thrombosis and/or PE completed primary treatment and continuing to receive secondary prevention. C Consider suspending aspirin for the duration of anticoagulation therapy in patients with deep vein thrombosis and/or PE with stable CVD initiating anticoagulation and previously taking aspirin for cardiovascular risk modification. C Thrombolytic therapy: As per ACCP 2021 guidelines, consider initiating anticoagulant therapy alone over interventional (thrombolytic, mechanical, or pharmacomechanical) therapy in patients with acute deep vein thrombosis of the leg. C As per ASH 2020 guidelines: Consider initiating anticoagulation therapy alone over thrombolytic therapy in addition to anticoagulation in most patients with proximal deep vein thrombosis. C Consider performing catheter-directed thrombolysis over systemic thrombolysis in patients with extensive deep vein thrombosis, if thrombolysis is performed. C 4. Nonpharmacologic interventions Compression stockings (early use): offer early compression at 30-40 mmHg with either multilayer bandaging or compression hosiery, applied within 24 hours, to reduce pain, edema, and residual venous obstruction in patients with proximal deep vein thrombosis. A Compression stockings, medically managed patients: As per ACCP 2021 guidelines, avoid using compression stockings routinely for the prevention of post-thrombotic syndrome in patients with acute deep vein thrombosis of the https://web.pathway.md/diseases/recy7Y18EJ5OGaopy 6/17 6/29/23, 10:06 PM Deep vein thrombosis Pathway leg. D As per ESVS 2021 guidelines: Consider offering below-knee compression stockings in patients with proximal deep vein thrombosis to reduce the risk of post-thrombotic syndrome. B Limit the use of below-knee stockings to 6 or 12 months in patients with proximal deep vein thrombosis and limited symptoms and signs, as described in the Villalta score. A As per ASH 2020 guidelines, avoid using compression stockings routinely in patients with deep vein thrombosis, with or without an increased risk for post-thrombotic syndrome. D Compression stockings, after thrombus removal: As per AAFP 2013 guidelines, offer compression stockings in patients following deep vein thrombosis to prevent post-thrombotic syndrome. A As per SVS 2012 guidelines, offer knee-high compression stockings (30-40 mmHg), to be worn for 2 years after the procedure, in patients with deep vein thrombosis managed with thrombus removal techniques. B 5. Therapeutic procedures Thrombus removal and venous stenting: As per ESVS 2021 guidelines: Consider performing early thrombus removal in selected patients with symptomatic iliofemoral deep vein thrombosis. B Do not perform early thrombus removal in patients with deep vein thrombosis limited to femoral, popliteal, or calf veins. D As per SVS 2012 guidelines, perform early thrombus removal as the treatment of choice in patients with limb-threatening venous ischemia due to iliofemoral deep vein thrombosis (phlegmasia cerulea dolens), with or without associated femoropopliteal vein thrombosis. A Show 3 more Anticoagulation after thrombus removal: As per ESVS 2021 guidelines: Initiate anticoagulation in patients with deep vein thrombosis treated by early thrombus removal, with or without stenting, with a duration of at least as long as if the patients were treated by anticoagulation alone and at the discretion of the treating physician. B Decide on the choice of therapy in patients with iliofemoral deep vein thrombosis undergoing early thrombus removal based on the judgment of the treating physician. B As per SVS 2012 guidelines, administer a standard course of conventional anticoagulation in patients managed with early thrombus removal. A Inferior vena cava filter placement: As per ACCP 2021 guidelines: Do not use IVC filters in addition to anticoagulation therapy in patients with acute deep vein thrombosis of the leg. D Place an IVC filter in patients with acute proximal deep vein thrombosis of the leg and a contraindication to anticoagulation. B https://web.pathway.md/diseases/recy7Y18EJ5OGaopy 7/17 6/29/23, 10:06 PM Deep vein thrombosis Pathway Landmark trials: PREPIC In patients with proximal deep vein thrombosis who were at risk for PE, IVC filter implantation was superior to no IVC filter implantation with respect to symptomatic or asymptomatic PE at 12 days. Decousus H et al. N Engl J Med. 1998 Feb 12. As per ESVS 2021 guidelines: Place a temporary IVC filter in patients with proximal deep vein thrombosis having contraindications to anticoagulation during the initial or principal treatment phase. B Do not use IVC filters routinely in patients anticoagulated for deep vein thrombosis. D As per ASH 2020 guidelines, consider initiating anticoagulation alone rather than anticoagulation plus placement of an IVC filter in patients with proximal deep vein thrombosis and significant preexisting cardiopulmonary disease, as well as in patients with PE and hemodynamic compromise. C As per SIR 2020 guidelines, consider placing an IVC filter based on various clinical risk factors in patients with deep vein thrombosis without PE, if anticoagulation is contraindicated. C Show 8 more As per SVS 2012 guidelines: Do not use IVC filters (permanent or temporary) routinely in conjunction with catheter- directed pharmacologic thrombolysis of the iliofemoral venous segments. D Discuss the relative risks and benefits of periprocedural retrievable IVC filters in patients: undergoing pharmacomechanical thrombolysis with thrombus extending into the IVC with markedly limited cardiopulmonary reserve. B Anticoagulation after IVC filter placement: As per SIR 2020 guidelines, insufficient evidence to recommend for or against anticoagulation in patients having indwelling IVC filters with no other indication for anticoagulation. I As per ACCP 2016 guidelines, consider administering a conventional course of anticoagulant therapy in patients with acute proximal deep vein thrombosis of the leg and an IVC filter inserted as an alternative to anticoagulation, if the risk of bleeding resolves. C Inferior vena cava filter removal: consider removing/converting IVC filters routinely in patients with indwelling retrievable/convertible filters if the risk of PE has been mitigated or if patients are no longer at risk for PE unless risk outweighs benefit. E Show 5 more 6. Surgical interventions https://web.pathway.md/diseases/recy7Y18EJ5OGaopy 8/17 6/29/23, 10:06 PM Deep vein thrombosis Pathway Surgical thrombectomy: consider performing open surgical venous thrombectomy in selected patients being candidates for anticoagulation, if thrombolytic therapy is contraindicated. C 7. Specific circumstances Pregnant patients (antepartum thromboprophylaxis): Consider obtaining testing for the known familial thrombophilia in females with a family history of VTE and known homozygous factor V Leiden, a combination of factor V Leiden and prothrombin G20210A, or antithrombin deficiency in the family. Consider administering antepartum thromboprophylaxis in patients with the same familial thrombophilia. C Consider either obtaining testing for the known familial thrombophilia or omitting testing for thrombophilia to guide antepartum prophylaxis in females with a family history of VTE and known protein C or protein S deficiency in the family. C Pregnant patients (postpartum thromboprophylaxis): consider obtaining testing for the known familial thrombophilia in females with a first-degree family history of VTE and known homozygous factor V Leiden, a combination of factor V Leiden and prothrombin G20210A, antithrombin deficiency, protein C deficiency, or protein S deficiency in the family. Consider administering postpartum thromboprophylaxis in patients with the same familial thrombophilia. C Show 2 more Pregnant patients, evaluation: As per ESVS 2021 guidelines, do not measure D-dimer levels and use the Wells score in pregnant patients with suspected deep vein thrombosis. D As per ASH 2018 guidelines, consider obtaining additional investigations, including serial compression ultrasound or MRV, after an initial negative ultrasound of the iliac veins in pregnant patients with suspected deep vein thrombosis. C Pregnant patients (setting of care): consider managing pregnant patients with low-risk acute VTE initially with outpatient therapy. C Pregnant patients, anticoagulation: As per ASH 2023 guidelines, consider obtaining thrombophilia testing to guide anticoagulant treatment duration after primary treatment in patients with VTE provoked by pregnancy or postpartum. Consider continuing anticoagulant treatment indefinitely in patients with thrombophilia and discontinuing it in patients without thrombophilia. C As per ESVS 2021 guidelines, initiate therapeutic doses of LMWH in pregnant patients with deep vein thrombosis during pregnancy for at least 3 months and for at least 6 weeks postpartum. B As per ASH 2018 guidelines: Initiate anticoagulant therapy with LMWH B , either once daily or BID, B in pregnant patients with acute VTE. A Avoid obtaining routine monitoring of anti-factor Xa levels to guide dosing in pregnant patients receiving therapeutic doses of LMWH. D https://web.pathway.md/diseases/recy7Y18EJ5OGaopy 9/17 6/29/23, 10:06 PM Deep vein thrombosis Pathway Pregnant patients (catheter-directed thrombolysis): avoid performing catheter-directed thrombolysis in addition to anticoagulation in pregnant patients with acute lower extremity deep vein thrombosis. D Pregnant patients (IVC filter placement): consider placing a temporary IVC filter in pregnant patients with deep vein thrombosis < 2 weeks before the anticipated date of delivery. C Pediatric patients: refer pediatric patients with deep vein thrombosis to clinicians with specific expertise in pediatric thrombosis and hemostasis. B Underweight or overweight patients: adjust the dose of UFH, LMWHs, and fondaparinux in underweight or overweight patients with deep vein thrombosis requiring anticoagulation. B Patients with chronic kidney disease: Consider monitoring anticoagulation levels and reducing the dosage in patients with deep vein thrombosis and CKD as most anticoagulants are excreted through kidneys. C Obtain periodic reassessments of renal function in patients with deep vein thrombosis and CKD treated with LMWH, fondaparinux, or a direct OAC. B Patients with liver cirrhosis: recognize that the risk of deep vein thrombosis/PE is at least as high in patients with cirrhosis as in the general population. B Show 6 more Patients with thrombophilia: As per ACCP 2021 guidelines, consider administering adjusted-dose vitamin K antagonists (target INR 2.5, with an overlapping period of parenteral anticoagulation) over direct OAC therapy during the treatment phase in patients with confirmed antiphospholipid syndrome being treated with anticoagulant therapy. C As per ESVS 2021 guidelines, initiate full-dose extended anticoagulant therapy with periodic reevaluation in patients with deep vein thrombosis and high-risk thrombophilia (such as antiphospholipid syndrome, homozygous FVL mutation, or deficiencies of protein C or S, or antithrombin). B Show 3 more Patients with iliofemoral DVT (evaluation): decide on the initial choice of tests based on the pretest probability of deep vein thrombosis. B Show 3 more Patients with cancer-associated thrombosis, initial management: As per ACCP 2021 guidelines: Administer oral Xa inhibitors (apixaban, edoxaban, rivaroxaban) rather than LMWH for the initiation and treatment phases in patients with acute deep vein thrombosis and cancer (cancer-associated thrombosis). B Consider preferring apixaban and LMWH in patients with luminal gastrointestinal malignancies due to the lower risk of major gastrointestinal bleeding. As per ESVS 2021 guidelines: Administer LMWH for the initial and principal treatment in patients with cancer-associated deep vein thrombosis. A Consider administering approved direct OACs for the initial and principal treatment in selected patients with cancer-associated deep vein thrombosis, with the malignancy not https://web.pathway.md/diseases/recy7Y18EJ5OGaopy 10/17 6/29/23, 10:06 PM Deep vein thrombosis Pathway located in the gastrointestinal or genitourinary systems. B Patients with cancer-associated thrombosis (extended therapy): Consider continuing anticoagulation beyond 3 months in patients with symptomatic calf deep vein thrombosis and active cancer. C Switch from LMWH to an OAC after 3-6 months of treatment for extended treatment in patients with active cancer-associated deep vein thrombosis. B Consider administering approved direct OACs for the extended treatment in selected patients with cancer-associated deep vein thrombosis, with the malignancy not located in the gastrointestinal or genitourinary systems. B Patients with catheter-related thrombosis, catheter removal: As per ESVS 2021 guidelines, consider removing the catheter in patients with catheter- related thrombosis when: it is not needed it is not functional anticoagulation is contraindicated symptoms are not resolving with anticoagulation the thrombosis is limb- or life-threatening. C As per ITAC 2019 guidelines, consider keeping the central venous catheter in place if it is functional, well positioned, and not infected, under close surveillance while administering anticoagulation therapy. Insufficient evidence to recommend specific duration of anticoagulation. C As per ISTH 2014 guidelines: Remove the non-functional, infected or incorrectly positioned catheter and consider initiating anticoagulation with LMWH for catheter-related thrombosis in patients with cancer. E Consider removing the central venous catheter without anticoagulation if therapeutic anticoagulation cannot be safely administered due to the active risk of hemorrhage. E Patients with iliofemoral DVT (anticoagulation): initiate anticoagulation for a minimum of 3 months in all patients in the acute care setting. A Show 4 more Patients with iliofemoral DVT (IVC filter placement): consider placing an IVC filter in patients with acute iliofemoral deep vein thrombosis with contraindications to systemic anticoagulation. B Show 2 more Patients with iliofemoral DVT (systemic thrombolysis): do not administer systemic thrombolysis in patients with iliofemoral deep vein thrombosis. D Patients with iliofemoral DVT (endovascular thrombectomy): Consider performing clot removal by endovenous techniques in patients with symptomatic acute iliofemoral deep vein thrombosis, to prevent or reduce post-thrombotic syndrome, ideally in patients with onset of symptoms within 21 days, good functional status, reasonable life expectancy, and low risk of bleeding. C Consider using endovenous techniques as first-line therapy for early thrombus removal. C https://web.pathway.md/diseases/recy7Y18EJ5OGaopy 11/17 6/29/23, 10:06 PM Deep vein thrombosis Pathway Patients with iliofemoral DVT (surgical thrombectomy): Perform urgent surgical thrombectomy, B or endovascular thrombus removal as an alternative option, in patients with phlegmasia cerulea dolens. B Consider performing open surgical venous thrombectomy in selected patients with iliofemoral deep vein thrombosis without phlegmasia cerulea dolens if the patient is a candidate for thrombus removal but has contraindications to thrombolytic therapy. C Patients with iliofemoral DVT (venous stenting): Consider performing stenting of the iliac venous system with self-expanding metallic stents at the time of clot removal in cases of clinically significant stenosis or extrinsic compression. C Do not place stents in the infrainguinal veins. D Patients with iliofemoral DVT (management of post-thrombotic syndrome): consider offering class II (30-40 mmHg) below-knee elastic compression stockings as soon as possible following initiation of anticoagulant therapy and continue for a minimum of 2 years. B Show 2 more Patients with iliofemoral DVT (follow-up): Obtain periodic clinical assessments in patients receiving extended anticoagulant therapy to reassess the risks and benefits of continuing the therapy. B Obtain longitudinal follow-up in all patients with an inserted optional recovery IVC filter. B Patients with upper extremity DVT: As per ESVS 2021 guidelines, obtain ultrasound as the initial imaging modality in patients with suspected upper extremity deep vein thrombosis. B Show 4 more As per ACCP 2016 guidelines: Consider initiating anticoagulant therapy alone over thrombolysis in patients with acute deep vein thrombosis of the upper extremity involving the axillary or more proximal veins. C Administer the same intensity and duration of anticoagulant therapy in patients with acute DVT of the upper extremity undergoing thrombolysis as in patients not undergoing thrombolysis. B Patients with catheter-related thrombosis, anticoagulation: As per ESVS 2021 guidelines, consider initiating LMWH or LMWH followed by vitamin K antagonists for a minimum of 3 months in patients with catheter-related thrombosis. C As per ITAC 2019 guidelines, initiate anticoagulation with LMWH for a minimum of 3 months and as long as the central venous catheter is in place for the treatment of symptomatic catheter-related thrombosis in patients with cancer. B As per ISTH 2014 guidelines, consider initiating anticoagulation with LMWH without removal of the catheter if the central venous catheter is functional and required for ongoing therapy. E Show 7 more 8. Preventative measures https://web.pathway.md/diseases/recy7Y18EJ5OGaopy 12/17 6/29/23, 10:06 PM Deep vein thrombosis Pathway Thrombophilia testing for minor provoking factors: avoid obtaining testing for factor V Leiden or prothrombin G20210A (low-risk thrombophilia) to guide thromboprophylaxis in patients with a family history of factor V Leiden (with or without VTE) and having a minor provoking risk factor for VTE, such as immobility or minor injury, illness, or infection. D Show 4 more Thrombophilia testing before hormone therapy (combined oral contraceptives): do not obtain thrombophilia testing to guide the use of combined oral contraceptives in females in the general population. D Show 3 more Thrombophilia testing before hormone therapy (hormone-replacement therapy): avoid obtaining thrombophilia testing to guide the use of hormone-replacement therapy in patients from the general population. D Show 3 more 9. Follow-up and surveillance Surveillance imaging: consider obtaining repeated ultrasound assessment after 5-7 days in patients with suspected deep vein thrombosis with a likely pretest probability and negative compression ultrasound. C Show 3 more Management of recurrent thrombosis: As per ESVS 2021 guidelines, consider switching the type of anticoagulation, increasing the dose of LMWH or direct OAC to a therapeutic dose, or switching to vitamin K antagonists with a higher INR target in patients with recurrent deep vein thrombosis occurring while compliant with treatment. C As per ASH 2020 guidelines: Consider administering LMWH rather than direct OACs in patients with breakthrough deep vein thrombosis and/or PE occurring during therapeutic vitamin K antagonist therapy. C Continue antithrombotic therapy indefinitely rather than stopping anticoagulation after completion of primary treatment in patients with a recurrent unprovoked deep vein thrombosis and/or PE. B As per ACCP 2016 guidelines: Switch to LMWH, at least temporarily, in patients with recurrent VTE while on OACs (vitamin K antagonists, dabigatran, rivaroxaban, apixaban, or edoxaban) deemed to be compliant with treatment. B Increase the dose of LMWH by about one-quarter to one-third in patients with recurrent VTE while on long-term LMWH deemed to be compliant with treatment. B Clinical findings Symptoms Past medical history Buttock pain Antiphospholipid syndrome https://web.pathway.md/diseases/recy7Y18EJ5OGaopy 13/17 6/29/23, 10:06 PM Deep vein thrombosis Pathway Calf pain Beh et's syndrome Fever Celiac disease Groin pain FVL mutation Leg cramping Granulomatosis with polyangiitis Leg pain IBD Leg swelling Immobility Inserted central venous catheter Medication history Malignancy Obesity Hormonal contraceptives PE Past surgical history Protein C deficiency Protein S deficiency Recent surgery Prothrombin G20210A mutation Rheumatoid arthritis Vital signs Superficial vein thrombosis Tachycardia Thromboangiitis obliterans Integument exam Past obstetric history Leg discoloration Pregnancy Leg telangiectasia Social history Tobacco use Vascular exam Bancroft's sign Homans' sign Leg edema Lisker's sign Louvel's sign Lowenberg's sign Peabody's sign Pratt's sign Rose's sign Lab findings serum D dimer Studies 2019 PREVENT In adults patients within 48 hours after admission to an ICU, pneumatic compression was not superior to control with respect to incident proximal deep vein thrombosis. Yaseen M Arabi et al. N Engl J Med. 2019 Apr 4. 2019 AVERT https://web.pathway.md/diseases/recy7Y18EJ5OGaopy 14/17 6/29/23, 10:06 PM Deep vein thrombosis Pathway In ambulatory patients with cancer who were at intermediate-to-high risk for VTE and were initiating chemotherapy, apixaban was superior to placebo with respect to the rate of VTE within 180 days. Carrier M et al. N Engl J Med. 2019 Feb 21. Show 22 more References 1. Saskia Middeldorp, Robby Nieuwlaat, Lisa Baumann Kreuziger et al. American Society of Hematology 2023 Guidelines for Management of Venous Thromboembolism: Thrombophilia Testing. Blood Adv. 2023 May 17;bloodadvances.2023010177. Open 2. Kathryn P Trayes, James S Studdiford, Sarah Pickle et al. Edema: Diagnosis and Management. Am Fam Physician. 2013 Jul 15;88 2 102 10. Open 3. Stavros K Kakkos, Manjit Gohel, Niels Baekgaard et al. Editor's Choice European Society for Vascular Surgery ESVS 2021 Clinical Practice Guidelines on the Management of Venous Thrombosis. Eur J Vasc Endovasc Surg. 2021 Jan;61 1 9 82. Open 4. Scott M Stevens, Scott C Woller, Lisa Baumann Kreuziger et al. Antithrombotic Therapy for VTE Disease: Second Update of the CHEST Guideline and Expert Panel Report. Chest. 2021 Dec;160 6):e545-e608. Open 5. Kearon C, Akl EA, Ornelas J et al. Antithrombotic Therapy for VTE Disease: CHEST Guideline and Expert Panel Report. Chest. 2016 Feb;149 2 315 352. Open 6. Meissner MH, Gloviczki P, Comerota AJ et al. Early thrombus removal strategies for acute deep venous thrombosis: clinical practice guidelines of the Society for Vascular Surgery and the American Venous Forum. J Vasc Surg. 2012 May;55 5 1449 62. Open 7. John A Kaufman, Geoffrey D Barnes, Rabih A Chaer et al. Society of Interventional Radiology Clinical Practice Guideline for Inferior Vena Cava Filters in the Treatment of Patients with Venous Thromboembolic Disease: Developed in collaboration with the American College of Cardiology, American College of Chest Physicians, American College of Surgeons Committee on Trauma, American Heart Association, Society for Vascular Surgery, and Society for Vascular Medicine. J Vasc Interv Radiol. 2020 Oct;31 10 1529 1544. Open 8. David Liu, Erica Peterson, James Dooner et al. Diagnosis and management of iliofemoral deep vein thrombosis: clinical practice guideline. CMAJ. 2015 Nov 17;187 17 1288 1296. Open 9. Thomas L Ortel, Ignacio Neumann, Walter Ageno et al. American Society of Hematology 2020 guidelines for management of venous thromboembolism: treatment of deep vein thrombosis and pulmonary embolism. Blood Adv. 2020 Oct 13;4 19 4693 4738. Open 10. Henry G Watson, David M Keeling, Mike Laffan et al. Guideline on aspects of cancer-related venous thrombosis. Br J Haematol. 2015 Sep;170 5 640 8. Open 11. J I Zwicker, G Connolly, M Carrier et al. Catheter-associated deep vein thrombosis of the upper extremity in cancer patients: guidance from the SSC of the ISTH. J Thromb Haemost. 2014 May;12 5 796 800. Open 12. Bates SM, Rajasekhar A, Middeldorp S et al. American Society of Hematology 2018 guidelines for management of venous thromboembolism: venous thromboembolism in the context of pregnancy. Blood Adv. 2018 Nov 27;2 22 3317 3359. Open https://web.pathway.md/diseases/recy7Y18EJ5OGaopy 15/17 6/29/23, 10:06 PM Deep vein thrombosis Pathway 13. European Association for the Study of the Liver. EASL Clinical Practice Guidelines on prevention and management of bleeding and thrombosis in patients with cirrhosis. J Hepatol. 2022 May;76 5 1151 1184. Open 14. Dominique Farge, Corinne Frere, Jean M Connors et al. 2019 international clinical practice guidelines for the treatment and prophylaxis of venous thromboembolism in patients with cancer. Lancet Oncol. 2019 Oct;20 10):e566-e581. Open 15. Emeka Kesieme, Chinenye Kesieme, Nze Jebbin et al. Deep vein thrombosis: a clinical review. J Blood Med. 2011; 2 59 69. Open 16. Paul T Vaitkus, Alain Leizorovicz, Alexander T Cohen et al. Mortality rates and risk factors for asymptomatic deep vein thrombosis in medical patients. Thromb Haemost. 2005 Jan;93 1 76 9. Open 17. Dhall SS, Hadley MN, Aarabi B et al. Deep venous thrombosis and thromboembolism in patients with cervical spinal cord injuries. Neurosurgery. 2013 Mar;72 Suppl 2 244 54. Open 18. Mahajerin A, Petty JK, Hanson SJ et al. Prophylaxis against venous thromboembolism in pediatric trauma: A practice management guideline from the Eastern Association for the Surgery of Trauma and the Pediatric Trauma Society. J Trauma Acute Care Surg. 2017 Mar;82 3 627 636. Open 19. Gould MK, Garcia DA, Wren SM et al. Prevention of VTE in nonorthopedic surgical patients: Antithrombotic Therapy and Prevention of Thrombosis, 9th ed: American College of Chest Physicians Evidence-Based Clinical Practice Guidelines. Chest. 2012 Feb;141 2 Suppl):e227S-e277S. Open 20. Falck-Ytter Y, Francis CW, Johanson NA et al. Prevention of VTE in orthopedic surgery patients: Antithrombotic Therapy and Prevention of Thrombosis, 9th ed: American College of Chest Physicians Evidence-Based Clinical Practice Guidelines. Chest. 2012 Feb;141 2 Suppl):e278S-e325S. Open 21. Practice Committee of the American Society for Reproductive Medicine. Combined hormonal contraception and the risk of venous thromboembolism: a guideline. Fertil Steril. 2017 Jan;107 1 43 51. Open 22. American Physical Therapy Association. Choosing Wisely APTA recommendations. Choosing Wisely. 2014. Open 23. Society for Vascular Medicine. Choosing Wisely SVM recommendations. Choosing Wisely. 2013. Open 24. Shrey Modi, Ryan Deisler, Karen Gozel et al. Wells criteria for DVT is a reliable clinical tool to assess the risk of deep venous thrombosis in trauma patients. World J Emerg Surg. 2016 Jun 8;11 24. Open 25. Shannon M Bates, Ian A Greer, Saskia Middeldorp et al. VTE, thrombophilia, antithrombotic therapy, and pregnancy: Antithrombotic Therapy and Prevention of Thrombosis, 9th ed: American College of Chest Physicians Evidence-Based Clinical Practice Guidelines. Chest. 2012 Feb;141 2 Suppl):e691S- e736S. Open 26. Shrey Modi, Ryan Deisler, Karen Gozel et al. Wells criteria for DVT is a reliable clinical tool to assess the risk of deep venous thrombosis in trauma patients. World J Emerg Surg. 2016 Jun 8;11 24. Open 27. J P Galanaud, C A Holcroft, M A Rodger et al. Comparison of the Villalta post-thrombotic syndrome score in the ipsilateral vs. contralateral leg after a first unprovoked deep vein thrombosis. J Thromb Haemost. 2012 Jun;10 6 1036 42. Open |
Increase the dose of LMWH by about one-quarter to one-third in patients with recurrent VTE while on long-term LMWH deemed to be compliant with treatment. B Clinical findings Symptoms Past medical history Buttock pain Antiphospholipid syndrome https://web.pathway.md/diseases/recy7Y18EJ5OGaopy 13/17 6/29/23, 10:06 PM Deep vein thrombosis Pathway Calf pain Beh et's syndrome Fever Celiac disease Groin pain FVL mutation Leg cramping Granulomatosis with polyangiitis Leg pain IBD Leg swelling Immobility Inserted central venous catheter Medication history Malignancy Obesity Hormonal contraceptives PE Past surgical history Protein C deficiency Protein S deficiency Recent surgery Prothrombin G20210A mutation Rheumatoid arthritis Vital signs Superficial vein thrombosis Tachycardia Thromboangiitis obliterans Integument exam Past obstetric history Leg discoloration Pregnancy Leg telangiectasia Social history Tobacco use Vascular exam Bancroft's sign Homans' sign Leg edema Lisker's sign Louvel's sign Lowenberg's sign Peabody's sign Pratt's sign Rose's sign Lab findings serum D dimer Studies 2019 PREVENT In adults patients within 48 hours after admission to an ICU, pneumatic compression was not superior to control with respect to incident proximal deep vein thrombosis. Yaseen M Arabi et al. N Engl J Med. 2019 Apr 4. 2019 AVERT https://web.pathway.md/diseases/recy7Y18EJ5OGaopy 14/17 6/29/23, 10:06 PM Deep vein thrombosis Pathway In ambulatory patients with cancer who were at intermediate-to-high risk for VTE and were initiating chemotherapy, apixaban was superior to placebo with respect to the rate of VTE within 180 days. Carrier M et al. N Engl J Med. 2019 Feb 21. Show 22 more References 1. Saskia Middeldorp, Robby Nieuwlaat, Lisa Baumann Kreuziger et al. American Society of Hematology 2023 Guidelines for Management of Venous Thromboembolism: Thrombophilia Testing. Blood Adv. 2023 May 17;bloodadvances.2023010177. Open 2. Kathryn P Trayes, James S Studdiford, Sarah Pickle et al. Edema: Diagnosis and Management. Am Fam Physician. 2013 Jul 15;88 2 102 10. Open 3. Stavros K Kakkos, Manjit Gohel, Niels Baekgaard et al. Editor's Choice European Society for Vascular Surgery ESVS 2021 Clinical Practice Guidelines on the Management of Venous Thrombosis. Eur J Vasc Endovasc Surg. 2021 Jan;61 1 9 82. Open 4. Scott M Stevens, Scott C Woller, Lisa Baumann Kreuziger et al. Antithrombotic Therapy for VTE Disease: Second Update of the CHEST Guideline and Expert Panel Report. Chest. 2021 Dec;160 6):e545-e608. Open 5. Kearon C, Akl EA, Ornelas J et al. Antithrombotic Therapy for VTE Disease: CHEST Guideline and Expert Panel Report. Chest. 2016 Feb;149 2 315 352. Open 6. Meissner MH, Gloviczki P, Comerota AJ et al. Early thrombus removal strategies for acute deep venous thrombosis: clinical practice guidelines of the Society for Vascular Surgery and the American Venous Forum. J Vasc Surg. 2012 May;55 5 1449 62. Open 7. John A Kaufman, Geoffrey D Barnes, Rabih A Chaer et al. Society of Interventional Radiology Clinical Practice Guideline for Inferior Vena Cava Filters in the Treatment of Patients with Venous Thromboembolic Disease: Developed in collaboration with the American College of Cardiology, American College of Chest Physicians, American College of Surgeons Committee on Trauma, American Heart Association, Society for Vascular Surgery, and Society for Vascular Medicine. J Vasc Interv Radiol. 2020 Oct;31 10 1529 1544. Open 8. David Liu, Erica Peterson, James Dooner et al. Diagnosis and management of iliofemoral deep vein thrombosis: clinical practice guideline. CMAJ. 2015 Nov 17;187 17 1288 1296. Open 9. Thomas L Ortel, Ignacio Neumann, Walter Ageno et al. American Society of Hematology 2020 guidelines for management of venous thromboembolism: treatment of deep vein thrombosis and pulmonary embolism. Blood Adv. 2020 Oct 13;4 19 4693 4738. Open 10. Henry G Watson, David M Keeling, Mike Laffan et al. Guideline on aspects of cancer-related venous thrombosis. Br J Haematol. 2015 Sep;170 5 640 8. Open 11. J I Zwicker, G Connolly, M Carrier et al. Catheter-associated deep vein thrombosis of the upper extremity in cancer patients: guidance from the SSC of the ISTH. J Thromb Haemost. 2014 May;12 5 796 800. Open 12. Bates SM, Rajasekhar A, Middeldorp S et al. American Society of Hematology 2018 guidelines for management of venous thromboembolism: venous thromboembolism in the context of pregnancy. Blood Adv. 2018 Nov 27;2 22 3317 3359. Open https://web.pathway.md/diseases/recy7Y18EJ5OGaopy 15/17 6/29/23, 10:06 PM Deep vein thrombosis Pathway 13. European Association for the Study of the Liver. EASL Clinical Practice Guidelines on prevention and management of bleeding and thrombosis in patients with cirrhosis. J Hepatol. 2022 May;76 5 1151 1184. Open 14. Dominique Farge, Corinne Frere, Jean M Connors et al. 2019 international clinical practice guidelines for the treatment and prophylaxis of venous thromboembolism in patients with cancer. Lancet Oncol. 2019 Oct;20 10):e566-e581. Open 15. Emeka Kesieme, Chinenye Kesieme, Nze Jebbin et al. Deep vein thrombosis: a clinical review. J Blood Med. 2011; 2 59 69. Open 16. Paul T Vaitkus, Alain Leizorovicz, Alexander T Cohen et al. Mortality rates and risk factors for asymptomatic deep vein thrombosis in medical patients. Thromb Haemost. 2005 Jan;93 1 76 9. Open 17. Dhall SS, Hadley MN, Aarabi B et al. Deep venous thrombosis and thromboembolism in patients with cervical spinal cord injuries. Neurosurgery. 2013 Mar;72 Suppl 2 244 54. Open 18. Mahajerin A, Petty JK, Hanson SJ et al. Prophylaxis against venous thromboembolism in pediatric trauma: A practice management guideline from the Eastern Association for the Surgery of Trauma and the Pediatric Trauma Society. J Trauma Acute Care Surg. 2017 Mar;82 3 627 636. Open 19. Gould MK, Garcia DA, Wren SM et al. Prevention of VTE in nonorthopedic surgical patients: Antithrombotic Therapy and Prevention of Thrombosis, 9th ed: American College of Chest Physicians Evidence-Based Clinical Practice Guidelines. Chest. 2012 Feb;141 2 Suppl):e227S-e277S. Open 20. Falck-Ytter Y, Francis CW, Johanson NA et al. Prevention of VTE in orthopedic surgery patients: Antithrombotic Therapy and Prevention of Thrombosis, 9th ed: American College of Chest Physicians Evidence-Based Clinical Practice Guidelines. Chest. 2012 Feb;141 2 Suppl):e278S-e325S. Open 21. Practice Committee of the American Society for Reproductive Medicine. Combined hormonal contraception and the risk of venous thromboembolism: a guideline. Fertil Steril. 2017 Jan;107 1 43 51. Open 22. American Physical Therapy Association. Choosing Wisely APTA recommendations. Choosing Wisely. 2014. Open 23. Society for Vascular Medicine. Choosing Wisely SVM recommendations. Choosing Wisely. 2013. Open 24. Shrey Modi, Ryan Deisler, Karen Gozel et al. Wells criteria for DVT is a reliable clinical tool to assess the risk of deep venous thrombosis in trauma patients. World J Emerg Surg. 2016 Jun 8;11 24. Open 25. Shannon M Bates, Ian A Greer, Saskia Middeldorp et al. VTE, thrombophilia, antithrombotic therapy, and pregnancy: Antithrombotic Therapy and Prevention of Thrombosis, 9th ed: American College of Chest Physicians Evidence-Based Clinical Practice Guidelines. Chest. 2012 Feb;141 2 Suppl):e691S- e736S. Open 26. Shrey Modi, Ryan Deisler, Karen Gozel et al. Wells criteria for DVT is a reliable clinical tool to assess the risk of deep venous thrombosis in trauma patients. World J Emerg Surg. 2016 Jun 8;11 24. Open 27. J P Galanaud, C A Holcroft, M A Rodger et al. Comparison of the Villalta post-thrombotic syndrome score in the ipsilateral vs. contralateral leg after a first unprovoked deep vein thrombosis. J Thromb Haemost. 2012 Jun;10 6 1036 42. Open 28. Yaseen M Arabi, Fahad Al-Hameed, Karen E A Burns et al. Adjunctive Intermittent Pneumatic Compression for Venous Thromboprophylaxis. N Engl J Med. 2019 Apr 4;380 14 1305 1315. Open https://web.pathway.md/diseases/recy7Y18EJ5OGaopy 16/17 6/29/23, 10:06 PM Deep vein thrombosis Pathway 29. Clive Kearon, Elie A Akl, Anthony J Comerota et al. Antithrombotic therapy for VTE disease: Antithrombotic Therapy and Prevention of Thrombosis, 9th ed: American College of Chest Physicians Evidence-Based Clinical Practice Guidelines. Chest. 2012 Feb;141 2 Suppl):e419S-e496S. Open 30. Paul Monagle, Anthony K C Chan, Neil A Goldenberg et al. Antithrombotic therapy in neonates and children: Antithrombotic Therapy and Prevention of Thrombosis, 9th ed: American College of Chest Physicians Evidence-Based Clinical Practice Guidelines. Chest. 2012 Feb;141 2 Suppl):e737S-e801S. Open 31. American College of Obstetricians and Gynecologists Committee on Practice Bulletins Gynecology. Prevention of Venous Thromboembolism in Gynecologic Surgery: ACOG Practice Bulletin, Number 232. Obstet Gynecol. 2021 Jul 1;138 1):e1-e15. Open 32. Paul Monagle, Carlos A Cuello, Caitlin Augustine et al. American Society of Hematology 2018 Guidelines for management of venous thromboembolism: treatment of pediatric venous thromboembolism. Blood Adv. 2018 Nov 27;2 22 3292 3316. Open 33. David R Anderson, Gian Paolo Morgano, Carole Bennett et al. American Society of Hematology 2019 guidelines for management of venous thromboembolism: prevention of venous thromboembolism in surgical hospitalized patients. Blood Adv. 2019 Dec 10;3 23 3898 3944. Open https://web.pathway.md/diseases/recy7Y18EJ5OGaopy 17/17 |
Guideline sources The following summarized guidelines for the evaluation and management of delayed ejaculation (DE) are prepared by our editorial team based on guidelines from the American Urological Association (AUA/SMSNA 2022). 1 Guidelines 1. Screening and diagnosis Definitions: Recognize that lifelong DE is defined as lifelong, consistent, bothersome inability to achieve ejaculation, or excessive latency of ejaculation, despite adequate sexual stimulation and the desire to ejaculate. E Recognize that acquired DE is defined as an acquired, consistent, bothersome inability to achieve ejaculation, or an increased latency of ejaculation, despite adequate sexual stimulation and the desire to ejaculate. E 2. Diagnostic investigations Initial assessment: Elicit a medical, relationship, and sexual history and perform a focused physical examination for the evaluation of patients with DE. B https://web.pathway.md/diseases/rec6I6QVeDF2MvvpN 1/2 6/29/23, 10:06 PM Delayed ejaculation Pathway Consider obtaining additional testing as clinically indicated for the evaluation of patients with DE. C 3. Medical management Modification in precipitating medications: consider offering replacement, dose adjustment, or staged cessation of medications likely to contribute to DE in patients with DE. C Management of erectile dysfunction: treat patients with DE and comorbid erectile dysfunction according to the erectile dysfunction guidelines. E Management of testosterone deficiency: consider offering treatment to normalize serum testosterone levels in patients with DE and testosterone deficiency. E Specific therapies: insufficient evidence to recommend oral pharmacotherapy or invasive nonpharmacological strategies for the management of patients with DE. I 4. Patient education General counseling: consider advising patients with DE to modify sexual positions or practices to increase arousal. E 5. Follow-up and surveillance Indications for referral: consider referring patients with lifelong or acquired DE to a mental health professional with expertise in sexual health. E References 1. Alan W Shindel, Stanley E Althof, Serge Carrier et al. Disorders of Ejaculation: An AUA/SMSNA Guideline. J Urol. 2022 Mar;207 3 504 512. Open https://web.pathway.md/diseases/rec6I6QVeDF2MvvpN 2/2 |
Guideline sources The following summarized guidelines for the evaluation and management of dementia are prepared by our editorial team based on guidelines from the American College of Preventive Medicine (ACPM/PCNA/ABC/ASPC/ASH/AAPA/AGS/AHA/NMA/ACC/APhA 2018), the Canadian Task Force on Preventive Health Care (CTFPHC 2016), the American Geriatrics Society (AGS 2015; 2014), the U.S. Preventive Services Task Force (USPSTF 2014), the Canadian Geriatrics Society (CGS 2012), the American Academy of Neurology (AAN 2010), and the American Psychiatric Association (APA 2007). 1 2 3 4 5 6 7 8 9 9 9 10 Definition Dementia is a clinical syndrome characterized by a progressive cognitive decline that interferes with the ability to function independently. 9 Epidemiology Dementia is mostly caused by Alzheimer's disease (60-80%), vascular injury (stroke; 20%), Lewy bodies (5-15%), and frontotemporal impairment (Pick's disease). 9 https://web.pathway.md/diseases/recqgGsIww8nnHUqF 1/9 6/29/23, 10:08 PM Dementia Pathway Disease course Alzheimer's disease, vascular injury, Lewy body deposition, and frontotemporal lobe impairment result in dementia, which causes clinical manifestations of gradual, persistent, and progressive decline in cognition and function. Cognitive deficits may present as memory loss, communication and language impairments, agnosia, apraxia, and impaired executive function (reasoning, judgment, and planning). Dementia progression may lead to behavioral and psychological symptoms including wandering, hoarding, sexual disinhibition, eating inappropriate objects, repetitive behaviors, restlessness, agitation, apathy, aggression, psychosis, hallucinations, and delusions causing considerable distress and safety risk for the patient and their caregivers. 9 Prognosis and risk of recurrence Annual mortality associated with dementia in men and women is 38.3% and 30.5%, respectively. 10 Calculator Calculator Calculat Abbey pain scale for patients wit Abbreviated mental test AMT 10 Abbrev Guidelines 1. Screening and diagnosis Indications for screening: As per CTFPHC 2016 guidelines, avoid screening asymptomatic adults 65 years of age for cognitive impairment. D As per USPSTF 2014 guidelines, insufficient evidence to assess the balance of benefits and harms of screening for cognitive impairment. I Diagnostic criteria: Adopt the criteria for dementia proposed by the National Institute on Aging-Alzheimer's Association working group in 2011. E Adopt the criteria for possible and probable Alzheimer's disease proposed by the National Institute on Aging-Alzheimer's Association working group in 2011. E 2. Diagnostic investigations Indications for head CT: Obtain a CT scan in patients with rapid unexplained decline in cognition or function duration of dementia < 2 years recent and significant head trauma unexplained neurological symptoms such as new onset of severe headache or seizures https://web.pathway.md/diseases/recqgGsIww8nnHUqF 2/9 6/29/23, 10:08 PM Dementia Pathway a history of cancer especially in sites and types that metastasize to the brain a history of anticoagulant use or bleeding disorder a history of urinary incontinence and gait disorder early in the course of dementia any new localizing sign such as hemiparesis or a Babinski reflex unusual or atypical cognitive symptoms or presentation, such as progressive aphasia gait disturbance. E Indications for brain magnetic resonance imaging: obtain MRI of the brain when a radiologist/neuroradiologist and/or a cognitive specialist can interpret patterns of atrophy and other radiological features that may provide added diagnostic and predictive value in the evaluation of patients with dementia. B 18F-fluorodeoxyglucose positron emission tomography and single positron emission computed tomography: obtain a 18F-labeled FDG-positron emission tomography scan in patients with a diagnosis of dementia who have undergone the recommended baseline clinical and structural brain imaging evaluation and have been evaluated by a dementia specialist, but whose underlying pathological process is still unclear, preventing adequate clinical management. B Functional magnetic resonance imaging: avoid functional MRI for the investigation of patients presenting with cognitive complaints. D Positron emission tomography amyloid imaging: avoid the use of amyloid imaging outside of research settings, because even though it represents a promising technique in the evaluation of dementia, there are many unknowns that could impact its diagnostic utility. D Magnetic resonance spectroscopy: avoid magnetic resonance spectroscopy to diagnose dementia or differentiate dementia from mild cognitive impairment. D Other investigations: avoid obtaining imaging biomarkers of neuro-inflammation or tau pathology in dementia patients. D Evaluation of driving risk: Identify patients with dementia at increased risk for unsafe driving by considering the following characteristics: the Clinical Dementia Rating scale A a caregiver's rating of a patient's driving ability as marginal or unsafe B a history of crashes or traffic citations B reduced driving mileage or self-reported situational avoidance B mini-Mental State Examination scores of 24 or less B aggressive or impulsive personality characteristics B 3. Medical management General principles: individualize treatment plans, because patients with dementia display a broad range of cognitive impairments and neuropsychiatric symptoms that can cause significant distress to themselves and caregivers. A Cholinesterase inhibitors: https://web.pathway.md/diseases/recqgGsIww8nnHUqF 3/9 6/29/23, 10:08 PM Dementia Pathway As per CGS 2012 guidelines, offer a trial of a cholinesterase inhibitor for most patients with Alzheimer's dementia. A Show 3 more As per APA 2007 guidelines: Offer cholinesterase inhibitors to patients with mild-to-moderate Alzheimer's disease after a thorough discussion of their potential risks and benefits. B Consider cholinesterase inhibitors in patients with severe Alzheimer's disease. C NMDA antagonists: As per CGS 2012 guidelines, insufficient evidence to recommend for or against combination therapy of a cholinesterase inhibitor and memantine. I As per APA 2007 guidelines, consider memantine, a noncompetitive NMDA antagonist, in patients with moderate and severe Alzheimer's disease. B Antidepressant trial: as per CGS 2012 guidelines, consider a trial of antidepressants in patients with major depressive disorder, severe dysthymia, or severe emotional liability. B Other pharmacological treatments: avoid vitamin E for the treatment of cognitive symptoms of dementia, because of limited evidence for its efficacy as well as safety concerns. D Management of sleep disturbances: manage sleep disturbances in patients with dementia with interventions including maintaining daytime activities and giving careful attention to sleep hygiene. B Show 3 more 4. Nonpharmacologic interventions Long-term care issues: organize care to meet the needs of patients, including those with behavioral problems. A Psychiatric management: Educate patients and families about the illness, its treatment, and sources of additional care and support. A Advise patients and their families of the need for financial and legal planning due to the patient's eventual incapacity. A Psychotherapy: large randomized clinical trials on behavioral approaches have not been performed, but their widespread clinical use is supported by small trials and case studies. B Driving cessation: inform all patients and families that even mild dementia increases the risk of motor vehicle accidents. A 5. Therapeutic procedures Tube feeding: As per Choosing Wisely 2015 guidelines, avoid percutaneous feeding tubes in patients with advanced dementia; offer oral assisted feeding instead. D https://web.pathway.md/diseases/recqgGsIww8nnHUqF 4/9 6/29/23, 10:08 PM Dementia Pathway As per AGS 2014 guidelines, avoid using feeding tubes for older adults with advanced dementia. Offer careful hand feeding; for persons with advanced dementia, hand feeding is at least as good as tube feeding for the outcomes of death, aspiration pneumonia, functional status, and comfort. 6. Specific circumstances Patients with rapidly progressive dementia: define rapidly progressive dementia as a dementia which develops within 12 months after the appearance of first cognitive symptoms. B Show 3 more Patients with early onset dementia: refer all patients with early onset dementia to a memory clinic, preferably one with access to genetic counseling and testing when appropriate. E 7. Preventative measures Management of blood pressure: consider BP lowering to prevent cognitive decline and dementia in adults with hypertension. C 8. Follow-up and surveillance Monitoring for psychiatric symptoms: monitor periodically for the development and evolution of cognitive and non-cognitive psychiatric symptoms, and their response to intervention. A Frequency of follow-up visits: follow-up patients at least every 3-6 months to adjust treatment, enhance safety, and provide timely advice to the patient and family. B Indications for specialist referral: obtain head MRI when a radiologist/neuroradiologist and/or a cognitive specialist can interpret patterns of atrophy and other features that may provide added diagnostic and predictive value. B Discontinuation of cholinesterase inhibitors: Consider discontinuing cholinesterase inhibitors when: the patient and/or their proxy decision-maker decide to stop after a reappraisal of risks and benefits the patient is sufficiently non-adherent with the medication that continued prescription of it would be useless, and it is not possible to establish a system for the administration of the medication to rectify the problem the patient's rate of cognitive, functional, and/or behavioral decline is greater on treatment compared to that prior to being treated the patient experiences intolerable side effects that are definitely or probably related to the cholinesterase inhibitor the comorbidities of the patient make continued use of the agent either unacceptably risky or futile https://web.pathway.md/diseases/recqgGsIww8nnHUqF 5/9 6/29/23, 10:08 PM Dementia Pathway the patient's dementia progresses to a stage where there would be no clinically meaningful benefit from continued therapy. E Clinical findings Patient demographics Symptoms Age > 65 Apathy Being unable to concentrate on tasks Past medical history Confusion Hyperthyroidism Depression Hypothyroidism Dysarthria Medications Loss of ability to do everyday tasks Neurological exam Memory impairment Disorientation Personality or behavioral changes Mood alteration Social history Alcohol consumption Likelihood Ratios Pertinent positives The following findings increase the probability of dementia in adults. 10 Finding LR+ Value Positive 7-Minute Screen 47 (3-730) Positive memory Impairment Screen 33 (15-72) Positive brief Alzheimer Screen 25 (17-35) Positive Mini-Cog 13.0 (9.9-17.0) Show 1 more Pertinent negatives The following findings decrease the probability of dementia in adults. 10 Finding LR- Value Negative brief Alzheimer Screen 0.02 (0.01-0.04) Negative memory Impairment Screen 0.08 (0.02-0.30) Negative 7-Minute Screen 0.09 (0.01-0.59) Negative 6-Item Screen 0.15 (0.04-0.14) Show 1 more Studies https://web.pathway.md/diseases/recqgGsIww8nnHUqF 6/9 6/29/23, 10:08 PM Dementia Pathway 2019 SPRINT-MIND In ambulatory adult patients ( 50 years of age) with hypertension but without diabetes or history of stroke who had an increased cardiovascular risk, intensive BP reduction was not superior to standard BP management with respect to the incidence of adjudicated probable dementia at a median follow-up of 5.11 years. SPRINT MIND Investigators for the SPRINT Research Group et al. JAMA. 2019 Feb 12. 2018 EXPEDITION3 In patients with mild dementia due to Alzheimer's disease and with amyloid deposition shown by means of florbetapir positron-emission tomography or Abeta1-42 measurements in CSF, solanezumab was not superior to placebo with respect to a ADAS-cog14 score at 80 weeks. Honig LS et al. N Engl J Med. 2018 Jan 25. References 1. APA Work Group on Alzheimer's Disease and other Dementias, Rabins PV, Blacker D et al. American Psychiatric Association practice guideline for the treatment of patients with Alzheimer's disease and other dementias. Second edition. Am J Psychiatry. 2007 Dec;164 12 Suppl):5 56. Open 2. Iverson DJ, Gronseth GS, Reger MA et al. Practice parameter update: evaluation and management of driving risk in dementia: report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology. 2010 Apr 20;74 16 1316 24. Open 3. Gauthier S, Patterson C, Chertkow H et al. Recommendations of the 4th Canadian Consensus Conference on the Diagnosis and Treatment of Dementia CCCDTD4 . Can Geriatr J. 2012 Dec;15 4 120 6. Open 4. Paul K Whelton, Robert M Carey, Wilbert S Aronow et al. 2017 ACC/AHA/AAPA/ABC/ACPM/AGS/APhA/ASH/ASPC/NMA/PCNA Guideline for the Prevention, Detection, Evaluation, and Management of High Blood Pressure in Adults: Executive Summary: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. Hypertension. 2018 Jun;71 6):e13-e115. Open 5. American Geriatrics Society. Choosing Wisely: Recommendations of the American Geriatrics Society. Choosing Wisely. 2015 Apr. Open 6. Moyer VA, U.S. Preventive Services Task Force. Screening for cognitive impairment in older adults: U.S. Preventive Services Task Force recommendation statement. Ann Intern Med. 2014 Jun 3;160 11 791 7. Open 7. American Geriatrics Society Ethics Committee and Clinical Practice and Models of Care Committee. American Geriatrics Society feeding tubes in advanced dementia position statement. J Am Geriatr Soc. 2014 Aug;62 8 1590 3. Open 8. Pottie K, Rahal R, Jaramillo A et al. Recommendations on screening for cognitive impairment in older adults. CMAJ. 2016 Jan 5;188 1 37 46. Open 9. Silvia Duong, Tejal Patel, and Feng Chang. Dementia What pharmacists need to know. Can Pharm J (Ott). 2017 Mar-Apr; 150 2 118 129. Open https://web.pathway.md/diseases/recqgGsIww8nnHUqF 7/9 6/29/23, 10:08 PM Dementia Pathway 10. Irene E van de Vorst, Ilonca Vaartjes, Mirjam I Geerlings et al. Prognosis of patients with dementia: results from a prospective nationwide registry linkage study in the Netherlands. BMJ Open. 2015; 5 10 e008897. Open 11. Bang J, Spina S, Miller BL. Frontotemporal dementia. Lancet. 2015 Oct 24;386 10004 1672 82. Open 12. Crum RM, Anthony JC, Bassett SS et al. Population-based norms for the Mini-Mental State Examination by age and educational level. JAMA. 1993 May 12;269 18 2386 91. Open 13. Cordell CB, Borson S, Boustani M et al. Alzheimer's Association recommendations for operationalizing the detection of cognitive impairment during the Medicare Annual Wellness Visit in a primary care setting. Alzheimers Dement. 2013 Mar;9 2 141 50. Open 14. Shaji KS, Sivakumar PT, Rao GP et al. Clinical Practice Guidelines for Management of Dementia. Indian J Psychiatry. 2018 Feb;60 Suppl 3 S312 S328. Open 15. American Geriatrics Society. Choosing Wisely AGS recommendations. Choosing Wisely. 2015. Open 16. American Geriatrics Society. Choosing Wisely AGS recommendations. Choosing Wisely. 2013. Open 17. American Academy of Hospice and Palliative Medicine. Choosing Wisely AAHPM recommendations. Choosing Wisely. 2013. Open 18. American Academy of Neurology. Choosing Wisely AAN recommendations. Choosing Wisely. 2016. Open 19. Society of Nuclear Medicine and Molecular Imaging. Choosing Wisely SNMMI recommendations. Choosing Wisely. 2013. Open 20. Society for Post-Acute and Long-Term Care Medicine. Choosing Wisely AMDA recommendations. Choosing Wisely. 2013. Open 21. American Academy of Neurology. Choosing Wisely AAN recommendations. Choosing Wisely. 2018. Open 22. American Psychiatric Association. Choosing Wisely APA recommendations. Choosing Wisely. 2015. Open 23. Johnson KA, Minoshima S, Bohnen NI et al. Appropriate use criteria for amyloid PET a report of the Amyloid Imaging Task Force, the Society of Nuclear Medicine and Molecular Imaging, and the Alzheimer's Association. J Nucl Med. 2013 Mar;54 3 476 90. Open 24. EL Cunningham, B McGuinness, B Herron et al. Dementia. Ulster Med J. 2015 May; 84 2 79 87. Open 25. J Hort, J T O'Brien, G Gainotti et al. EFNS guidelines for the diagnosis and management of Alzheimers disease. Eur J Neurol. 2010 Oct;17 10 1236 48. Open 26. Valentina Lichtner, Dawn Dowding, Philip Esterhuizen et al. Pain assessment for people with dementia: a systematic review of systematic reviews of pain assessment tools. BMC Geriatr. 2014; 14 138. Open 27. L Berg. Clinical Dementia Rating CDR . Psychopharmacol Bull. 1988;24 4 637 9. Open 28. Albert Lukas, Ulrich Hagg-Gr n, Benjamin Mayer et al. Pain assessment in advanced dementia. Validity of the German PAINAD-a prospective double-blind randomised placebo-controlled trial. Pain. 2019 Mar;160 3 742 753. Open https://web.pathway.md/diseases/recqgGsIww8nnHUqF 8/9 6/29/23, 10:08 PM Dementia Pathway 29. Jennifer Abbey, Neil Piller, Anita De Bellis et al. The Abbey pain scale: a 1-minute numerical indicator for people with end-stage dementia. Int J Palliat Nurs. 2004 Jan;10 1 6 13. Open 30. M Mosele, E M Inelmen, E D Toffanello et al. Psychometric properties of the pain assessment in advanced dementia scale compared to self assessment of pain in elderly patients. Dement Geriatr Cogn Disord. 2012;34 1 38 43. Open 31. Victor I Reus, Laura J Fochtmann, A Evan Eyler et al. The American Psychiatric Association Practice Guideline on the Use of Antipsychotics to Treat Agitation or Psychosis in Patients With Dementia. Am J Psychiatry. 2016 May 1;173 5 543 6. Open 32. John T O'Brien, Clive Holmes, Matthew Jones et al. Clinical practice with anti-dementia drugs: A revised (third) consensus statement from the British Association for Psychopharmacology. J Psychopharmacol. 2017 Feb;31 2 147 168. Open 33. Philip B Gorelick, Angelo Scuteri, Sandra E Black et al. Vascular Contributions to Cognitive Impairment and Dementia: A Statement for Healthcare Professionals From the American Heart Association/American Stroke Association. Stroke. 2011 Sep;42 9 2672 713. Open 34. Eric E Smith, Philip Barber, Thalia S Field et al. Canadian Consensus Conference on Diagnosis and Treatment of Dementia CCCDTD 5 Guidelines for management of vascular cognitive impairment. Alzheimers Dement N Y . 2020 Nov 11;6 1):e12056. Open 35. M Filippi, F Agosta, F Barkhof et al. EFNS task force: the use of neuroimaging in the diagnosis of dementia. Eur J Neurol. 2012 Dec;19 12):e131 40, 1487 501. Open 36. S Sorbi, J Hort, T Erkinjuntti et al. EFNS ENS Guidelines on the diagnosis and management of disorders associated with dementia. Eur J Neurol. 2012 Sep;19 9 1159 79. Open https://web.pathway.md/diseases/recqgGsIww8nnHUqF 9/9 |
Guideline sources The following summarized guidelines for the evaluation and management of dengue are prepared by our editorial team based on guidelines from the World Health Organization (WHO 2023), the Pan American Health Organization (PAHO/WHO 2022), the Center for Disease Control (CDC 2021), the Philippine Pediatric Society (PPS/PIDSP 2017), the European Academy of Neurology (EAN 2017), and the Association of British Neurologists (ABN/BIANG 2012). 1 2 3 4 5 6 7 7 7 8 9 9 Definition Dengue fever is an acute arthropod-borne viral illness caused by an RNA virus of the Flaviviridae family. 7 Epidemiology Dengue fever is caused by the dengue virus of the family Flaviviridae that includes four different serotypes (DEN-1, DEN-2, DEN-3, and DEN-4) and is transmitted through Aedes aegypti mosquito. 7 Pathophysiology https://web.pathway.md/diseases/rec7ykc9vTLUsq4fr 1/8 6/29/23, 10:08 PM Dengue Pathway The average incidence of dengue in the Americas is 58.02 per 100,000 person-years. 8 Disease course The host-viral interaction, viral replication, and/or direct skin infection by the virus trigger humoral, cellular, and innate host immune response that leads to alterations in endothelial microvascular permeability and thromboregulatory mechanisms causing increased plasma and protein loss resulting in thrombocytopenia associated with platelet dysfunction, damage, or depletion causing significant hemorrhages, breakbone fever, multiorgan damage and DIC. 7 Prognosis and risk of recurrence The mortality associated with a lack of early appropriate intervention is around 10-20%. The recurrence rate associated with shock is approximately 30%. 9 9 Guidelines 1. Screening and diagnosis Differential diagnosis (arbovirus infections): Recognize clinical manifestations of different arbovirus infections to help differentiate them: Situation Guidance Findings that help in differentiation - thrombocytopenia, progressive increase in hematocrit, leukopenia Dengue Findings that probably help in differentiation - anorexia or hyporexia, vomiting, abdominal pain, chills, hemorrhages, including bleeding on the skin, mucous membranes, or both Findings that may help in differentiation - retro-ocular pain, hepatomegaly, headache, diarrhea, dysgeusia, cough, elevated transaminases, positive tourniquet test B Findings that help in differentiation - arthralgias Chikungunya Findings that probably help in differentiation - eruption, conjunctivitis, arthritis, myalgias, bone pain Findings that may help in differentiation - hemorrhages, including bleeding on the skin, mucous membranes, or both B Findings that help in differentiation - pruritus Zika https://web.pathway.md/diseases/rec7ykc9vTLUsq4fr 2/8 6/29/23, 10:08 PM Dengue Pathway Findings that probably help in differentiation - eruption, conjunctivitis Findings that may help in differentiation - lymphadenopathies, pharyngitis, odynophagia B Differential diagnosis (COVID-19 infection): Test for dengue fever as appropriate based on the local epidemiology and clinical symptoms in patients with suspected COVID-19. A Include arbovirus infections (including dengue and chikungunya) in the differential diagnosis of undifferentiated febrile illness in endemic areas, particularly in the presence of thrombocytopenia. Test for dengue in patients with a febrile illness in endemic areas, irrespective of the presence of respiratory signs and symptoms. Recognize that coinfection with COVID-19 may also occur, and a positive diagnostic test for dengue (such as dengue rapid diagnostic tests) does not exclude the testing for COVID-19 virus. 2. Classification and risk stratification Severity assessment: Consider assessing for the following warning signs to identify patients with an increased risk of progression to severe disease: Situation Guidance Progressive until it is continuous or sustained and intense, and at the end of the febrile stage Abdominal pain Irritability, drowsiness, and lethargy Sensory disorder Gingivorrhagia, epistaxis, vaginal bleeding not associated with menstruation or more menstrual bleeding than usual, and hematuria Mucosal bleeding Clinical, on imaging, or both, at the end of the febrile stage Fluid accumulation > 2 cm below the costal margin and abrupt onset Hepatomegaly Persistent ( 3 episodes in 1 hour or 4 episodes in 6 hours) Vomiting On at least 2 consecutive measurements during patient monitoring. C Progressive increase in hematocrit 3. Diagnostic investigations https://web.pathway.md/diseases/rec7ykc9vTLUsq4fr 3/8 6/29/23, 10:08 PM Dengue Pathway Evaluation for encephalitis (flavivirus): Obtain acute and convalescent blood samples in patients with suspected arbovirus encephalitis. B Test CSF for IgM antibodies in patients with suspected flavivirus encephalitis. B Evaluation for encephalitis (tick-borne encephalitis virus): obtain virus neutralization test in areas with possible exposure to other pathogenic flaviviruses (including dengue) to assess the specific immunity against tick-borne encephalitis virus. A 4. Medical management Setting of care: Consider using the following criteria for the hospitalization of patients with dengue: presence of warning signs (abdominal pain, sensory disorder, mucosal bleeding, fluid accumulation, hepatomegaly, persistent vomiting, progressive increase in hematocrit) meeting criteria for severe disease, according to the WHO 2009 definition oral intolerance difficulty breathing narrowing pulse pressure arterial hypotension acute renal failure prolonged capillary refill time pregnancy coagulopathy. C Fluid resuscitation: initiate intense oral hydration in patients with dengue to decrease the progression to severe forms and the appearance of disease complications. B Show 2 more Antipyretics: consider offering acetaminophen or metamizole over NSAIDs, antihistamines, or corticosteroids for the initial symptomatic management of patients with arboviral infection. C Corticosteroids: avoid administering systemic corticosteroids in patients with dengue shock. D Intravenous immunoglobulin: avoid administering immunoglobulins in patients with severe dengue. D 5. Therapeutic procedures Blood transfusion: do not administer blood components (platelet concentrate, FFP) in patients with thrombocytopenia, regardless of platelet count. Consider administering blood component transfusion in patients with bleeding or additional conditions predisposing to bleeding (such as pregnancy). D 6. Specific circumstances https://web.pathway.md/diseases/rec7ykc9vTLUsq4fr 4/8 6/29/23, 10:08 PM Dengue Pathway Pediatric patients (indications for hospital admission): Admit patients with a confirmed or presumptive diagnosis of dengue in an outpatient setting to a healthcare facility for closer monitoring and observation in the presence of the following signs and symptoms: shortness of breath irritability or drowsiness pleural effusion abdominal pain melena elevated hematocrit decreased or decreasing platelet count. B Show 2 more Pediatric patients (risk of bleeding): Recognize that hospitalized patients with dengue having 1 of the following clinical or laboratory findings may increase the risk of bleeding: hypotension narrow pulse pressure platelet count < 50, 000/mm WBC count < 5, 000/mm hepatomegaly elevated ALT (> 3 times the ULN). B Show 2 more Pediatric patients (risk of mortality): Recognize that patients with dengue presenting with any of the following clinical findings may be at increased risk for mortality: hypotension on admission narrow pulse pressure on admission dengue hemorrhagic fever stage 3 and 4 (severe dengue) history of previous dengue prolonged shock respiratory failure liver failure (AST > 200 U/L and INR > 1.3) renal failure (BUN > 20 mg/dL and serum creatinine > 1.0 mg/dL) significant bleeding, including gastrointestinal bleeding severe plasma leakage in multiple sites (pleural effusion, pericardial effusion, and ascites). B Show 2 more Pediatric patients (fluid resuscitation): Insufficient evidence regarding the tonicity of IV fluid influencing mortality in patients with dengue without shock. Consider administering isotonic fluids as maintenance for patients with dengue without shock, recognizing that hypotonic IV fluids are associated with hyponatremia in hospitalized pediatric patients. I Consider administering either crystalloids or colloids for fluid resuscitation in patients with dengue with shock. Insufficient evidence to recommend colloids over crystalloids to improve https://web.pathway.md/diseases/rec7ykc9vTLUsq4fr 5/8 6/29/23, 10:08 PM Dengue Pathway mortality, recognizing that colloids may be associated with more adverse reactions (such as bleeding and allergic reactions) compared to crystalloids. B Pediatric patients (blood transfusion): insufficient evidence to recommend administering prophylactic platelet transfusion to prevent bleeding and reduce mortality in patients with minimal or no active bleeding. Do not administer prophylactic platelet transfusion in pediatric patients with platelet count < 50, 000/mm with minimal or no active bleeding. I Show 2 more Pediatric patients (mosquito repellents): insufficient evidence to recommend using citronella-based repellents over DEET-based repellents to reduce dengue transmission. I 7. Preventative measures Vaccination: offer vaccination against dengue (3-dose vaccination series, administered 6 months apart at 0, 6, and 12 months) in 9-16 years old pediatric patients with evidence of previous dengue infection (such as confirmation with previous laboratory-confirmed infection or a highly specific serodiagnostic test) and living in dengue-endemic areas. E Clinical findings Symptoms Social history Abdominal pain Exposure to aedes mosquitoes Arthralgia Living in or travel to subtropical regions Bone pain Living in or travel to tropical regions Change in taste Chills Confusion Vascular exam Drowsiness Cold extremities Fever Narrow pulse pressure Gingival bleeding Headache Ocular exam Hematemesis Hematuria Conjunctival injection Irritability Cotton wool spots Malaise Macular edema Melena Retinal hemorrhage Myalgia Abdominal exam Nausea Nosebleed Abdominal tenderness Restlessness Ascites Retro-orbital pain Hepatomegaly Seizure Skin flushing Integument exam Skin rash Dehydration Throat pain https://web.pathway.md/diseases/rec7ykc9vTLUsq4fr 6/8 6/29/23, 10:08 PM Dengue Pathway Vaginal bleeding Ecchymosis Vomiting Jaundice Maculopapular rash Vital signs Morbilliform rash Mucosal bleeding Hypotension Petechiae Signs of shock Purpura Neurological exam Skin erythema Altered mental status Hematological findings Respiratory exam hematocrit WBC count Respiratory distress blood platelet count Diagnostic maneuvers Imaging findings tourniquet test Pericardial effusion Pleural effusion Lab findings gallbladder wall thickness serum creatinine serum transaminases dengue virus NS1 antigen dengue virus RNA Serological findings dengue virus IgA d i I M Likelihood Ratios Pertinent positives The following findings increase the probability of dengue in adults. 9 10 Finding LR+ Value Positive dengue virus NS1 antigen and IgM 27.5 Positive dengue virus IgA 22 Positive dengue virus NS1 antigen 14.4 Positive dengue virus IgM 8.24 Pertinent negatives The following findings decrease the probability of dengue in adults. 9 10 Finding LR- Value Negative dengue virus NS1 antigen 0.19 Negative dengue virus NS1 antigen or IgM 0.22 Negative dengue virus IgA 0.5 Negative dengue virus IgM 0.79 https://web.pathway.md/diseases/rec7ykc9vTLUsq4fr 7/8 6/29/23, 10:08 PM Dengue Pathway References 1. Jaime Santos, Ma. Liza Antoinette Gonzales, Mary Antonette Madrid et al. Clinical Practice Guidelines on Dengue in Children. PIDSP. 2017. Open 2. Gabriela Paz-Bailey, Laura Adams, Joshua M Wong et al. Dengue Vaccine: Recommendations of the Advisory Committee on Immunization Practices, United States, 2021. MMWR Recomm Rep. 2021 Dec 17;70 6 1 16. Open 3. No authors listed. Guidelines for the Clinical Diagnosis and Treatment of Dengue, Chikungunya, and Zika. PAHO. 2022. Open 4. T Solomon, B D Michael, P E Smith et al. Management of suspected viral encephalitis in adults Association of British Neurologists and British Infection Association National Guidelines. J Infect. 2012 Apr;64 4 347 73. Open 5. Taba P, Schmutzhard E, Forsberg P et al. EAN consensus review on prevention, diagnosis and management of tick-borne encephalitis. Eur J Neurol. 2017 Oct;24 10 1214-e61. Open 6. No authors listed. Living guidance for clinical management of COVID 19. WHO living guidance. 2023 Jan 13. Open 7. Hasan S, Jamdar SF, Alalowi M et al. Dengue virus: A global human threat: Review of literature. J Int Soc Prev Community Dent. 2016 Jan-Feb;6 1 1 6. Open 8. Salles TS, da Encarnacao Sa-Guimaraes T, de Alvarenga ESL et al. History, epidemiology and diagnostics of dengue in the American and Brazilian contexts: a review. Parasit Vectors. 2018 Apr 24;11 1 264. Open 9. Huy NT, Thao NT, Ha TT et al. Development of clinical decision rules to predict recurrent shock in dengue. Crit Care. 2013 Dec 2;17 6 R280. Open 10. Viravarn Luvira, Charin Thawornkuno, Saranath Lawpoolsri et al. Diagnostic Performance of Dengue NS1 and Antibodies by Serum Concentration Technique. Trop Med Infect Dis. 2023 Feb 14;8 2 117. Open 11. K Alagarasu, A M Walimbe, S M Jadhav et al. A meta-analysis of the diagnostic accuracy of dengue virus-specific IgA antibody-based tests for detection of dengue infection. Epidemiol Infect. 2016 Mar;144 4 876 86. Open https://web.pathway.md/diseases/rec7ykc9vTLUsq4fr 8/8 |
Guideline sources The following summarized guidelines for the management of deprescribing proton-pump inhibitors are prepared by our editorial team based on guidelines from the American Gastroenterological Association (AGA 2022) and the College of Family Physicians of Canada (CFPC 2017). 1 2 Guidelines 1. Medical management Indications for discontinuation: consider discontinuing PPIs in all patients without a definitive indication for chronic use. E Show 2 more Considerations for discontinuation: As per AGA 2022 guidelines, assess PPI users for upper gastrointestinal bleeding risk using an evidence-based strategy before deprescribing. E Show 2 more As per CFPC 2017 guidelines: Decrease the daily dose or discontinue PPIs and change to on-demand (as needed) use in adult patients with upper gastrointestinal symptoms completed a minimum 4-week course of PPI treatment, resulting in resolution of upper gastrointestinal symptoms. B Consider prescribing an H2RA as an alternative to PPIs in adult patients with upper gastrointestinal symptoms completed a minimum 4-week course of PPI treatment, resulting in resolution of upper gastrointestinal symptoms. C https://web.pathway.md/diseases/rec3UPQpMnyaeoVab 1/2 6/29/23, 10:08 PM Deprescribing proton-pump inhibitors Pathway 2. Patient education General counseling: counsel patients discontinuing long-term PPI therapy that they may develop transient upper gastrointestinal symptoms due to rebound acid hypersecretion. E 3. Follow-up and surveillance Monitoring for indications: obtain a regular review (by the patient's primary care provider) of the ongoing indications for use and document that indication in all patients taking a PPI. E References 1. Laura E Targownik, Deborah A Fisher, Sameer D Saini. AGA Clinical Practice Update on De- Prescribing of Proton Pump Inhibitors: Expert Review. Gastroenterology. 2022 Feb 16;S0016 5085 21 04083 X. Open 2. Barbara Farrell, Kevin Pottie, Wade Thompson et al. Deprescribing proton pump inhibitors: Evidence- based clinical practice guideline. Can Fam Physician. 2017 May;63 5 354 364. Open https://web.pathway.md/diseases/rec3UPQpMnyaeoVab 2/2 |
Guideline sources The following summarized guidelines for the evaluation and management of dermatitis herpetiformis are prepared by our editorial team based on guidelines from the European Academy of Dermatology and Venereology (EADV 2021) and the European Society for the Study of Coeliac Disease (ESsCD 2019). 1 2 Guidelines 1. Screening and diagnosis Diagnosis: As per EADV 2021 guidelines: Diagnose dermatitis herpetiformis if both major diagnostic criteria are fulfilled - clinical manifestation compatible with dermatitis herpetiformis and positive DIF microscopy. B Recognize that HLA-DQ2/DQ8 positivity does not confirm the diagnosis of dermatitis herpetiformis. B As per ESsCD 2019 guidelines: Confirm the diagnosis of dermatitis herpetiformis by DIF examination of perilesional skin showing granular IgA deposits in the papillary dermis. A https://web.pathway.md/diseases/recomDblZ3foWk3l5 1/6 6/29/23, 10:08 PM Dermatitis herpetiformis Pathway Consider confirming the diagnosis of celiac disease and dermatitis herpetiformis by typical results from DIF examination of perilesional skin and positive anti-tissue transglutaminase. B Differential diagnosis: Differentiate dermatitis herpetiformis from the following autoimmune bullous diseases: linear IgA dermatosis bullous pemphigoid anti-laminin gamma-1 pemphigoid epidermolysis bullosa acquisita bullous SLE pemphigus herpetiformis igA pemphigus. Differentiate dermatitis herpetiformis from the following non-autoimmune disease: atopic dermatitis and other types of eczema multiple folliculitis nodular or subacute prurigo scabies arthropod bite reactions (papular urticaria, strophulus). 2. Diagnostic investigations History and physical examination: elicit a medical history, perform a complete physical examination and obtain gastroenterological assessment in patients with suspected dermatitis herpetiformis. B Show 3 more Serologic testing (general principles): Recognize that: there are circulating IgA antibodies directed against two different transglutaminase isoenzymes (tissue transglutaminase-2 and tissue transglutaminase-3) only IgA antibody-based serological immunoassays (IDIF microscopy or ELISA) play a significant role in the diagnosis of dermatitis herpetiformis. Show 4 more Serologic testing (endomysial antibodies): recognize that IDIF microscopy is used for qualitative and semi-quantitative detection of endomysial antibodies in the sera of patients with dermatitis herpetiformis. Show 2 more Serologic testing (tissue transglutaminase antibodies): obtain IgA antibodies against tissue transglutaminase-2 in all patients with suspected dermatitis herpetiformis, as they are specific markers of gluten-induced enteropathy in patients with dermatitis herpetiformis and celiac disease. B Show 5 more Serologic testing (epidermal transglutaminase antibodies): consider obtaining epidermal transglutaminase ELISA in addition to tissue transglutaminase-2. C https://web.pathway.md/diseases/recomDblZ3foWk3l5 2/6 6/29/23, 10:08 PM Dermatitis herpetiformis Pathway Show 2 more Serologic testing (gliadin antibodies): do not obtain antibodies against gliadin or deamidated gliadin as primary tests in the diagnostic work-up for dermatitis herpetiformis or celiac disease. D HLA genotyping: Do not obtain routine HLA-DQ2/DQ8 typing for the diagnosis of dermatitis herpetiformis. D Consider obtaining HLA-DQ2/DQ8 to exclude dermatitis herpetiformis or celiac disease in selected situations due to its high negative predictive value. C Baseline laboratory tests: Obtain the following laboratory tests at baseline: CBC including reticulocyte count LFTs renal function tests serum G6PD level (if not available, reduce starting dapsone dose). B Evaluation for comorbidities: consider assessing thyroid function and thyroid autoimmunity at diagnosis and during follow-up. C Show 4 more 3. Diagnostic procedures Skin biopsy: As per EADV 2021 guidelines: Perform lesional biopsy for histopathology in patients with suspected dermatitis herpetiformis. B Use a standardized (buffered) 4% formaldehyde (10% formalin) solution for storage and transport. B As per ESsCD 2019 guidelines: Perform perilesional skin biopsy with DIF examination to identify granular IgA deposits in the papillary dermis for the diagnosis of dermatitis herpetiformis. A Perform perilesional skin biopsy after at least 1 month of initiating gluten-containing diet in patients already following a gluten-free diet. B Direct immunofluorescence microscopy: perform DIF microscopy as the gold-standard for diagnosis in all patients with suspected dermatitis herpetiformis. B Show 2 more Small bowel biopsy: Perform small bowel biopsy in patients with dermatitis herpetiformis to evaluate the degree of enteropathy. B Consider taking at least four specimen from the distal duodenum and at least one from the duodenal bulb by upper gastrointestinal endoscopy. C 4. Medical management https://web.pathway.md/diseases/recomDblZ3foWk3l5 3/6 6/29/23, 10:08 PM Dermatitis herpetiformis Pathway Dapsone: As per EADV 2021 guidelines: Initiate dapsone in patients with dermatitis herpetiformis in the following situations: intolerable and/or severe skin involvement skin manifestation not responding to a correct gluten-free diet patients not accepting or unable to adhere to a gluten-free diet. B Do not use dapsone in patients with any of the following: G6PD deficiency low blood cell counts, especially anemia or neutropenia any cardiac or pulmonary disease leading to significantly impaired tissue oxygenation. D As per ESsCD 2019 guidelines, consider initiating dapsone for the management of patients with dermatitis herpetiformis during the 6-24 month period until the gluten-free diet is effective. B Second-line therapy: Offer the following agents as additional options in patients with dermatitis herpetiformis: sulfasalazine potent topical corticosteroids antihistamines. B Offer the following agents as alternative therapies in patients with dermatitis herpetiformis: sulfasalazine (1-4 g/day), sulfamethoxipyridazine (0.25-1.5 g/day), sulfapyridine tetracycline plus nicotinamide (500 mg/500 mg QID) potent topical corticosteroids. B Agents with no evidence for benefit: Do not use the following agents in patients with dermatitis herpetiformis: cyclosporine systemic corticosteroids. D Insufficient evidence to suggest the use of colchicine in patients with dermatitis herpetiformis. I 5. Nonpharmacologic interventions Gluten-free diet: As per EADV 2021 guidelines, offer lifelong gluten-free diet with/without dapsone as the main therapeutic option in patients with dermatitis herpetiformis. B Show 4 more As per ESsCD 2019 guidelines, offer a gluten-free diet for the management of patients with dermatitis herpetiformis. A Supplements: consider offering micronutrient and vitamin supplementation case-by-case. C 6. Follow-up and surveillance https://web.pathway.md/diseases/recomDblZ3foWk3l5 4/6 6/29/23, 10:08 PM Dermatitis herpetiformis Pathway Follow-up: Obtain the following at according intervals: Situation Guidance History and clinical review Weekly at 1st month CBC including reticulocyte count Every 2 weeks at 2nd and 3rd months MetHgb if the daily dose of dapsone is > 150 mg weekly LFTs Every 2 weeks at 1st 3 months Renal function tests History and clinical review including peripheral motor neurological examination Every 3rd month CBC including reticulocyte count MetHgb if the daily dose of dapsone is > 150 mg LFTs Renal function tests. B 7. Quality improvement Labelling regulations: Recognize the following regulations on gluten-free diets in Europe: gluten-free labels guarantee safety for consumers with dermatitis herpetiformis and celiac disease foods labeled as "very low gluten content" are not suitable for consumers with dermatitis herpetiformis and celiac disease hidden gluten contamination should be avoided as it is easy to reach toxicity thresholds. Clinical findings Symptoms Past medical history Abdominal pain Celiac disease Diarrhea Gluten-sensitive enteropathy Itching Iron deficiency anemia Integument exam Serological findings Hyperpigmentation anti-epidermal transglutaminase Hypopigmentation anti-gliadin antibodies Papulovesicular rash anti-tissue transglutaminase Skin blisters antiendomysial antibody Skin erythema Skin excoriations https://web.pathway.md/diseases/recomDblZ3foWk3l5 5/6 6/29/23, 10:08 PM Dermatitis herpetiformis Pathway Skin plaques References 1. A G r g, E Antiga, M Caproni et al. S2k guidelines (consensus statement) for diagnosis and therapy of dermatitis herpetiformis initiated by the European Academy of Dermatology and Venereology EADV . J Eur Acad Dermatol Venereol. 2021 Jun;35 6 1251 1277. Open 2. Abdulbaqi Al-Toma, Umberto Volta, Renata Auricchio et al. European Society for the Study of Coeliac Disease ESsCD guideline for coeliac disease and other gluten-related disorders. United European Gastroenterol J. 2019 Jun;7 5 583 613. Open https://web.pathway.md/diseases/recomDblZ3foWk3l5 6/6 |
Guideline sources The following summarized guidelines for the evaluation of developmental delay (DD) are prepared by our editorial team based on guidelines from the Canadian Task Force on Preventive Health Care (CTFPHC 2016). 1 2 2 2 2 Definition DD in a child is an inability to achieve developmental milestones in comparison to peers of the same age range. 2 Epidemiology DD is caused by genetic disorders (Down syndrome, fragile X syndrome), cerebral dysgenesis (microcephaly, hydrocephalus), vascular (occlusion, hemorrhage), drugs (cytotoxic, anti- epileptic), toxins (alcohol, smoking), early maternal infections (rubella, CMV), late maternal infections (varicella, HIV, malaria), prematurity, intrauterine growth retardation, perinatal asphyxia, metabolic dysfunction, postnatal infections, head injury, stroke, maltreatment, malnutrition, and maternal mental health disorder. 2 Disease course Various prenatal, perinatal, and postnatal causes may lead to mild, moderate, and severe DD ranging from a single domain to multiple domains including growth (weight, height), occipitofrontal circumference, speech, fine motor skills, visual, auditory, and bone development. Late detection of DD may result in poor outcomes resulting in learning difficulties, behavior problems, and functional impairments later on in life. 2 Prognosis and risk of recurrence DD is not associated with increased mortality. 2 https://web.pathway.md/diseases/recpbGoiBbwFo9sLA 1/2 6/29/23, 10:10 PM Developmental delay Pathway Guidelines 1. Screening and diagnosis Indications for screening: avoid screening for DD in children 1-4 years of age who have no apparent signs of DD, and whose parents and clinicians have no concerns about development. D Clinical findings Symptoms Past medical history Difficulties talking or talking late Genetic abnormality Difficulty communicating or socializing with others Metabolic disorders Phenylketonuria Difficulty with problem-solving or logical thinking Toxins Traumatic injury Having problems remembering things Inability to connect actions with consequences Inability to groom or dress oneself Learning and developing more slowly than other children same age Learning difficulties in school Lower than average scores on IQ tests References 1. Tonelli M, Parkin P, Brauer P et al. Recommendations on screening for developmental delay. CMAJ. 2016 May 17;188 8 579 87. Open 2. Ying Ying Choo, Pratibha Agarwal, Choon How How et al. Developmental delay: identification and management at primary care level. Singapore Med J. 2019 Mar; 60 3 119 123. Open https://web.pathway.md/diseases/recpbGoiBbwFo9sLA 2/2 |
Guideline sources The following summarized guidelines for the evaluation and management of developmental dysplasia of the hip (DDH) are prepared by our editorial team based on guidelines from the American Academy of Orthopaedic Surgeons (AAOS 2022), the Dutch Orthopaedic Society (DOS 2022), the American College of Radiology (ACR 2019), the Pediatric Orthopaedic Society of North America (POSNA 2007), and the U.S. Preventive Services Task Force (USPSTF 2006). 1 2 3 4 5 Guidelines 1. Screening and diagnosis Indications for screening: As per AAOS 2022 guidelines, avoid obtaining universal ultrasound screening of newborn infants. D As per POSNA 2007 guidelines, perform physical examination for hip instability at birth, with periodic follow-up exams until the child is walking. E As per USPSTF 2006 guidelines, insufficient evidence to recommend routine screening for DDH in infants as a means to prevent adverse outcomes. I https://web.pathway.md/diseases/rec5R6XC2IWHO9t1Z 1/5 6/29/23, 10:10 PM Developmental dysplasia of the hip Pathway 2. Diagnostic investigations Hip ultrasound: As per AAOS 2022 guidelines: Obtain imaging before 6 months of age in pediatric patients with 1 of the following risk factors: breech presentation family history history of clinical instability. A Consider obtaining an ultrasound in pediatric patients < 6 weeks of age with a positive instability examination to guide the decision to initiate brace treatment. C As per ACR 2019 guidelines, obtain hip ultrasound as the initial imaging in pediatric patients < 4 months of age with physical findings of DDH at initial imaging. B Show 2 more As per POSNA 2007 guidelines, consider obtaining ultrasound as an adjunct in evaluation for patients at risk for hip dysplasia and/or when the diagnosis is in question. E Pelvic radiography: As per AAOS 2022 guidelines, consider obtaining an anterioposterior pelvis radiograph instead of an ultrasound for the assessment of DDH in pediatric patients beginning at 4 months of age. C As per ACR 2019 guidelines: Obtain pelvic XR as the initial imaging in pediatric patients 4-6 months of age with a concern for DDH at initial imaging. B Obtain pelvic XR as the initial imaging in pediatric patients > 6 months of age with a concern for DDH. B As per POSNA 2007 guidelines, consider obtaining a pelvic XR for the evaluation of pediatric patients 4-5 months of age, if high-quality ultrasound is not available. E 3. Medical management Observation: As per AAOS 2022 guidelines, consider offering observation without a brace in pediatric patients with a clinically stable hip with morphologic ultrasound imaging abnormalities. C As per DOS 2022 guidelines, offer initial observation in 3 months old patients with centered DDH. B 4. Nonpharmacologic interventions Hip abduction braces: As per AAOS 2022 guidelines, consider offering either immediate or delayed (2-9 weeks) brace treatment for hips with a positive instability exam. C As per DOS 2022 guidelines: https://web.pathway.md/diseases/rec5R6XC2IWHO9t1Z 2/5 6/29/23, 10:10 PM Developmental dysplasia of the hip Pathway Consider offering abduction treatment if the hip does not normalize after 6-12 weeks. C Discontinue the abduction device when the hip has normalized or when the child is 1 year old. B Splinting: As per AAOS 2022 guidelines, consider offering the von Rosen splint over Pavlik, Craig, or Frejka splints for the initial treatment of an unstable hip. C As per DOS 2022 guidelines, offer a Pavlik harness in < 6 months old patients with centered DDH on repeated ultrasound. Consider offering another abduction device in > 6 months old patients. E As per POSNA 2007 guidelines: Offer early treatment with a Pavlik harness or similarly effective orthosis in patients with DDH. E Offer splinting for hips not stabilized after flexion/abduction. E Closed reduction: offer closed reduction for hips not stabilized after flexion/abduction. E Hip spica cast: offer spica cast immobilization for hips not stabilized after flexion/abduction. E 5. Follow-up and surveillance Indications for referral: refer patients to an experienced orthopedic surgeon for confirmation of the suggested diagnosis. E Serial clinical and imaging assessment: As per AAOS 2022 guidelines: Consider re-examining patients previously screened as having a normal hip examination on subsequent visits before 6 months of age. C Obtain serial physical examinations and periodic imaging assessments (ultrasound or radiograph based on age) during the management of patients with unstable hips. B As per DOS 2022 guidelines, assess < 1-year-old patients with centered DDH in the outpatient clinic and with imaging every 6 weeks. E As per ACR 2019 guidelines, obtain hip ultrasound in pediatric patients < 6 months of age with a known diagnosis of DDH during nonoperative surveillance imaging in harness. B Clinical findings Patient demographics Past obstetric history Infants Breech presentation Musculoskeletal exam Asymmetric thigh skin folds Hamstring stretch sign Hip click Hip dislocation https://web.pathway.md/diseases/rec5R6XC2IWHO9t1Z 3/5 6/29/23, 10:10 PM Developmental dysplasia of the hip Pathway Leg length discrepancy Limited hip abduction in flexion Limping Lumbar hyperlordosis Waddling gait Barlow test Galeazzi test Ortolani test telescoping test Likelihood Ratios Pertinent positives The following findings increase the probability of developmental dysplasia of the hip in adults. 5 Finding LR+ Value Positive telescoping test 25.5 Positive Trendelenburg's sign 14 Presence of limping 13.6 Presence of hamstring stretch sign 5.9 Show 2 more Pertinent negatives The following findings decrease the probability of developmental dysplasia of the hip in adults. 5 Finding LR- Value Absence of limping 0.1 Absence of hamstring stretch sign 0.1 Absence of leg length discrepancy 0.2 Negative Trendelenburg's sign 0.2 Show 2 more References 1. AAOS. Detection and Nonoperative Management of Pediatric Developmental Dysplasia of the Hip in Infants up to Six Months of Age. AAOS. 21 Mar 2022. Open 2. Richard M Schwend, Perry Schoenecker, B Stephens Richards et al. Screening the newborn for developmental dysplasia of the hip: now what do we do?. J Pediatr Orthop. 2007 Sep;27 6 607 10. Open 3. Christiaan J A van Bergen, Pieter Bas de Witte, Floor Willeboordse et al. Treatment of centered developmental dysplasia of the hip under the age of 1 year: an evidence-based clinical practice guideline Part 1. EFORT Open Rev. 2022 Jul 5;7 7 498 505. Open 4. US Preventive Services Task Force. Screening for developmental dysplasia of the hip: recommendation statement. Pediatrics. 2006 Mar;117 3 898 902. Open https://web.pathway.md/diseases/rec5R6XC2IWHO9t1Z 4/5 6/29/23, 10:10 PM Developmental dysplasia of the hip Pathway 5. Expert Panel on Pediatric Imaging:, Jie C Nguyen, Scott R Dorfman et al. ACR Appropriateness Criteria Developmental Dysplasia of the Hip-Child. J Am Coll Radiol. 2019 May;16 5S S94 S103. Open 6. Kremli MK, Khoshhal KI, Zamzam M et al. Evaluation of clinical signs and tests of congenital dysplasia of the hip. Ann Saudi Med. 2002 Jan-Mar;22 1 2 102 4. Open 7. French LM, Dietz FR. Screening for developmental dysplasia of the hip. Am Fam Physician. 1999 Jul;60 1 177 84, 187 8. Open https://web.pathway.md/diseases/rec5R6XC2IWHO9t1Z 5/5 |
Guideline sources The following summarized guidelines for the evaluation and management of diabetes during pregnancy are prepared by our editorial team based on guidelines from the American Diabetes Association (ADA 2023), the Society of Obstetricians and Gynaecologists of Canada (SOGC 2019), the U.S. Preventive Services Task Force (USPSTF 2014), and the Endocrine Society (ES 2013). 1 2 3 4 5 5 6 7 Definition Diabetes during pregnancy is characterized by newly recognized hyperglycemia during pregnancy associated with an increased risk of maternal and neonatal morbidities. 5 Epidemiology Diabetes during pregnancy is caused due to insulin resistance (due to maternal obesity or increased production of diabetogenic placental hormone) in pregnancy and pancreatic -cell dysfunction. 6 Disease course https://web.pathway.md/diseases/rec8f7VCPdwpi3vjw 1/8 6/29/23, 10:10 PM Diabetes during pregnancy Pathway Diabetes during pregnancy is related to several maternal complications including diabetic ketoacidosis, hypoglycemia, retinopathy, deterioration of nephropathy, gastric neuropathy, miscarriages, pre-eclampsia, polyhydramnios, premature delivery; and perinatal complications including stillbirth, neonatal death, shoulder dystocia, bone fracture, nerve palsy, neonatal hypoglycemia. 7 Prognosis and risk of recurrence There is an increased risk of stillbirth from 36-42 weeks in women with diabetes during pregnancy with a relative risk of 1.34 (95% CI 1.2-1.5) and 17.1 per 10,000 deliveries. 5 Guidelines 1. Screening and diagnosis Indications for screening: As per ADA 2023 guidelines, obtain screening for undiagnosed diabetes in individuals with risk factors planning pregnancy. B Consider testing all individuals of childbearing potential for undiagnosed diabetes. B Show 4 more As per SOGC 2019 guidelines, obtain screening for diabetes in all pregnant individuals between 24-28 weeks of gestation, using a standardized non-fasting 50-g glucose challenge screening test with plasma glucose measured 1 hour later. Do not obtain further testing if the value is < 7.8 mmol/L. Obtain a 2-hour 75-g oral glucose tolerance test with fasting plasma glucose, 1-hour plasma glucose, and 2-hour plasma glucose if the value of the glucose challenge screening is 7.8-11.0. B Show 2 more As per USPSTF 2014 guidelines: Obtain screening for gestational diabetes in asymptomatic pregnant individuals after 24 weeks of gestation. B Insufficient evidence to assess the balance of benefits and harms of screening for gestational diabetes in asymptomatic pregnant individuals before 24 weeks of gestation. I As per ES 2013 guidelines: Obtain screening for diabetes in pregnant individuals at the first prenatal visit, with fasting plasma glucose, HbA1c, or untimed random plasma glucose. B Obtain screening for diabetes in pregnant individuals at 24-28 weeks of gestation, with a 2- hour, 75-g oral glucose tolerance test. B 2. Diagnostic investigations Renal function assessment: consider obtaining renal function assessment (urine albumin-to- creatinine ratio, serum creatinine, and eGFR) in all individuals with diabetes planning conception, before withdrawing contraceptive measures or otherwise trying to conceive. https://web.pathway.md/diseases/rec8f7VCPdwpi3vjw 2/8 6/29/23, 10:10 PM Diabetes during pregnancy Pathway Show 2 more Thyroid function assessment: measure serum TSH and thyroid peroxidase antibodies, if the thyroid status is unknown, in patients with T1DM planning conception, before withdrawing contraceptive measures or otherwise trying to conceive. B Screening for coronary artery disease: Screen for coronary artery disease in patients with diabetes and several vascular risk factors (particularly the duration of the patient's diabetes and age) planning conception, before withdrawing contraceptive measures or otherwise trying to conceive. B Assess disease severity in patients with diabetes and known coronary artery disease planning conception. Initiate appropriate treatment and provide counseling as to the potential risks of pregnancy to the patient and fetus. A Screening for diabetic retinopathy: As per ADA 2023 guidelines, counsel patients with preexisting T2DM planning pregnancy or who have become pregnant on the risk of development and/or progression of diabetic retinopathy. Obtain dilated eye examinations ideally before pregnancy or in the first trimester, and then monitor patients every trimester and for 1 year postpartum as indicated by the degree of retinopathy and as recommended by the eye care healthcare professional. B As per ES 2013 guidelines, obtain a detailed ocular assessment by a qualified eye care professional in all patients with diabetes planning conception, before withdrawing contraceptive measures or otherwise trying to conceive. A Show 4 more 3. Medical management Setting of care: Manage patients with preexisting diabetes planning a pregnancy ideally beginning in preconception in a multidisciplinary clinic including an endocrinologist, maternal-fetal medicine specialist, registered dietitian nutritionist, and diabetes care and education specialist, when available. B Recognize that telehealth visits for pregnant patients with gestational diabetes mellitus improve outcomes compared with standard in-person care. A Glycemic targets, before pregnancy: consider targeting blood glucose and HbA1c levels as close to normal as possible in patients with diabetes planning to conceive, without causing undue hypoglycemia. C Glycemic targets, during pregnancy: As per ADA 2023 guidelines: Obtain fasting and postprandial blood glucose monitoring in both gestational diabetes mellitus and preexisting diabetes in pregnancy, to achieve optimal glucose levels. Set the following glucose targets: fasting plasma glucose < 95 mg/dL (5.3 mmol/L) and either 1- hour postprandial glucose < 140 mg/dL (7.8 mmol/L) or 2-hour postprandial glucose < 120 mg/dL (6.7 mmol/L). Obtain blood glucose preprandially in some patients with preexisting diabetes. B https://web.pathway.md/diseases/rec8f7VCPdwpi3vjw 3/8 6/29/23, 10:10 PM Diabetes during pregnancy Pathway Recognize that HbA1c is slightly lower during pregnancy in individuals with or without diabetes due to increased RBC turnover. Set a HbA1c target in pregnancy of < 6% (42 mmol/mol) if this can be achieved without significant hypoglycemia. Consider relaxing the target to < 7% (53 mmol/mol) if necessary to prevent hypoglycemia. B As per ES 2013 guidelines, target blood glucose levels as close to normal as possible in patients with gestational diabetes. B Show 4 more Glycemic targets, after pregnancy: consider targeting blood glucose levels of 72-126 mg/dL (4.0-7.0 mmol/L) during labor and delivery in patients with overt or gestational diabetes. C Initial management, gestational diabetes: As per ADA 2023 guidelines, offer lifestyle behavior changes for the management of gestational diabetes mellitus. Add insulin if needed to achieve glycemic targets. A As per ES 2013 guidelines: Offer lifestyle measures (medical nutrition therapy and daily moderate exercise for 30 minutes) as first-line treatment for gestational diabetes. B Initiate pharmacological therapy if lifestyle therapy is insufficient to maintain normoglycemia in patients with gestational diabetes. A Initial management, preexisting diabetes: Prescribe insulin as the preferred therapy for both T1DM A and T2DM during pregnancy. B Consider prescribing either multiple daily injections or insulin pump technology in pregnancy complicated by T1DM. C Insulin therapy (before pregnancy): prescribe multiple daily doses of insulin or continuous subcutaneous insulin infusion in preference to split-dose, premixed insulin therapy in insulin- treated patients with diabetes planning to conceive because the former are more likely to allow for the achievement and maintenance of target blood glucose levels preconceptionally and, in the event of pregnancy, are more likely to allow for sufficient flexibility or precise adjustment of insulin therapy. B Show 3 more Insulin therapy (during pregnancy): consider prescribing long-acting insulin analog detemir during pregnancy in patients requiring basal insulin, if NPH insulin, in appropriate doses, has previously resulted in problematic hypoglycemia, or would result in a high risk of problematic hypoglycemia. Consider continuing insulin detemir in patients already successfully taking insulin detemir before pregnancy. B Show 3 more Oral antihyperglycemics: As per ADA 2023 guidelines: Do not use metformin and glyburide as first-line therapy, as both cross the placenta to the fetus. Recognize that other oral and non-insulin injectable glucose-lowering medications lack long-term safety data. D Discontinue metformin by the end of the first trimester, when used for the treatment of PCOS and induction of ovulation. A https://web.pathway.md/diseases/rec8f7VCPdwpi3vjw 4/8 6/29/23, 10:10 PM Diabetes during pregnancy Pathway As per ES 2013 guidelines, consider offering metformin for glycemic control only in patients with gestational diabetes meeting the following criteria: inadequate glycemic control despite medical nutrition therapy refusal of insulin therapy, or inability to use insulin pregnancy in the second or third trimester. C Show 2 more Low-dose aspirin: initiate low-dose aspirin 100-150 mg/day (162 mg/day may also be acceptable) starting at 12-16 weeks of gestation to lower the risk of preeclampsia in patients with T1DM or T2DM. B Management of hypertension: consider targeting a BP of 110-135/85 mmHg in pregnant patients with diabetes and chronic hypertension in the interest of reducing the risk for accelerated maternal hypertension. B Titrate therapy from a BP threshold of 140/90 mmHg for better pregnancy outcomes with no increase in the risk of small-for-gestational-age birth weight. B Lessen therapy for BP < 90/60 mmHg. C Medications to avoid: As per ADA 2023 guidelines, avoid using potentially teratogenic medications, such as ACEIs, ARBs, and statins, in sexually active females of childbearing age not using reliable contraception. D As per ES 2013 guidelines, discontinue ACEIs and ARBs in patients with diabetes planning to conceive. B Show 4 more 4. Nonpharmacologic interventions Dietary modifications: As per ADA 2023 guidelines, encourage a balanced intake of macronutrients including nutrient-dense fruits, vegetables, legumes, whole grains, and healthy fats with omega-3 fatty acids (including nuts, seeds, and fish) in the eating pattern. B As per ES 2013 guidelines, offer medical nutrition therapy in all pregnant patients with overt or gestational diabetes, to help achieve and maintain desired glycemic control while providing essential nutrient requirements. B Show 3 more Weight reduction: Advise weight reduction before pregnancy in patients with diabetes and overweight or obesity. B Consider following the IOM revised guidelines for weight gain during pregnancy in patients with overt or gestational diabetes. 5. Therapeutic procedures Considerations for delivery: offer induction between 38-40 weeks of gestation depending on glycemic control and other comorbidity factors in pregnant patients with gestational or with https://web.pathway.md/diseases/rec8f7VCPdwpi3vjw 5/8 6/29/23, 10:10 PM Diabetes during pregnancy Pathway pregestational diabetes mellitus. B Show 2 more 6. Patient education General counseling: As per ADA 2023 guidelines, incorporate preconception counseling into routine diabetes care starting at puberty and continuing in all patients with diabetes and reproductive potential. A Show 2 more As per ES 2013 guidelines, provide preconception counseling to all patients with diabetes planning pregnancy. B Show 2 more 7. Preventative measures Preconception care: As per ADA 2023 guidelines, augment standard preconception care with extra focus on nutrition, diabetes education, and screening for diabetes comorbidities and complications, B in addition to focused attention on achieving glycemic targets. A As per ES 2013 guidelines, initiate folic acid supplementation at a dose of 5 mg/day B in patients with diabetes, beginning 3 months before withdrawing contraceptive measures or otherwise trying to conceive, in order to reduce the risk of neural tube defects. B Show 2 more 8. Follow-up and surveillance Monitoring of glycemic control: As per ADA 2023 guidelines, consider obtaining continuous glucose monitoring in addition to pre- and postprandial blood glucose monitoring to achieve HbA1c targets in diabetes and pregnancy. C Show 3 more As per ES 2013 guidelines, obtain self-monitoring of blood glucose in pregnant patients with overt or gestational diabetes. A Show 2 more Monitoring of fetal health: consider obtaining fetal growth assessment starting at 28 weeks of gestation as a baseline and with subsequent serial assessments every 3-4 weeks to assess the effect of maternal glycemic control on fetal growth rate and amniotic fluid volume in patients with pregestational or gestational diabetes mellitus. C Show 2 more Postpartum care, evaluation of glycemic control: As per ADA 2023 guidelines, evaluate and adjust insulin requirements postpartum as insulin resistance decreases dramatically immediately postpartum and the requirements are often roughly half the prepregnancy requirements for the initial few days postpartum. B https://web.pathway.md/diseases/rec8f7VCPdwpi3vjw 6/8 6/29/23, 10:10 PM Diabetes during pregnancy Pathway Show 4 more As per SOGC 2019 guidelines, obtain a 75-g oral glucose tolerance test between 6 weeks and 6 months postpartum to detect prediabetes and diabetes in patients with gestational diabetes mellitus. B As per ES 2013 guidelines, measure fasting plasma glucose, or advise patients to perform self-monitoring of fasting blood glucose, for 24 to 72 hours after delivery in women who have had gestational diabetes. B Show 2 more Postpartum care (screening for postpartum thyroiditis): consider screening women with T1DM for postpartum thyroiditis with measurement of TSH level at 3 and 6 months postpartum. C Postpartum care, breastfeeding: As per ADA 2023 guidelines, advise breastfeeding to reduce the risk of maternal T2DM. B As per SOGC 2019 guidelines, advise breastfeeding after delivery in all patients with pre- gestational or gestational diabetes mellitus. B As per ES 2013 guidelines: Advise patients with overt or gestational diabetes to breastfeed their infant, whenever possible. A Continue metformin or glyburide therapy during breastfeeding, if necessary, in patients with overt diabetes, if these medications were used successfully during pregnancy. A Postpartum care (contraception): Discuss and implement a contraceptive plan in all patients with diabetes of reproductive potential. A Include psychosocial assessment and support for self-care in postpartum care. B Studies 2023 TOBOGM In women between 4 weeks and 19 weeks 6 days gestation who had a risk factor for hyperglycemia and a diagnosis of gestational diabetes, immediate treatment was superior to no treatment with respect to adverse neonatal outcome. David Simmons et al. N Engl J Med. 2023 Jun 8. References 1. Blumer I, Hadar E, Hadden DR et al. Diabetes and pregnancy: an endocrine society clinical practice guideline. J Clin Endocrinol Metab. 2013 Nov;98 11 4227 49. Open 2. Steven E. Kahn, Cheryl A.M. Anderson, John B. Buse et al. Standards of Care in Diabetes 2023. Diabetes Care. 2023 Jan;46 Supplement_1 S1 S291. Open 3. Howard Berger, Robert Gagnon, Mathew Sermer. Guideline No. 393 Diabetes in Pregnancy. J Obstet Gynaecol Can. 2019 Dec;41 12 1814 1825.e1. Open https://web.pathway.md/diseases/rec8f7VCPdwpi3vjw 7/8 6/29/23, 10:10 PM Diabetes during pregnancy Pathway 4. Moyer VA, U.S. Preventive Services Task Force. Screening for gestational diabetes mellitus: U.S. Preventive Services Task Force recommendation statement. Ann Intern Med. 2014 Mar 18;160 6 414 20. Open 5. Melissa G Rosenstein, Yvonne W Cheng, Jonathan M Snowden et al. The risk of stillbirth and infant death stratified by gestational age in women with gestational diabetes. 2012 Apr;206 4 309.e1 7.2012 Apr;206 4 309.e1 7. Open 6. AbdelHameed Mirghani Dirar and John Doupis. Gestational diabetes from A to Z. World J Diabetes. 2017 Dec 15; 8 12 489 511. Open 7. Fathi I Abourawi. Diabetes mellitus and pregnancy. 2006 Jul 4;1 1 28 41.2006 Jul 4;1 1 28 41. Open 8. American Diabetes Association. 14. Management of Diabetes in Pregnancy: Standards of Medical Care in Diabetes 2019. Diabetes Care. 2019 Jan;42 Suppl 1 S165 S172. Open 9. Melissa G. ROSENSTEIN, Yvonne W. CHENG, Jonathan M. SNOWDEN et al. The Risk of Stillbirth and Infant Death Stratified by Gestational Age in Women with Gestational Diabetes. Am J Obstet Gynecol. 2012 Apr; 206 4 309.e1 309.e7. Open 10. Fathi I Abourawi. Diabetes mellitus and pregnancy. Libyan J Med. 2006; 1 1 28 41. Open https://web.pathway.md/diseases/rec8f7VCPdwpi3vjw 8/8 |
Guideline sources The following summarized guidelines for the evaluation and management of diabetes mellitus type 1 are prepared by our editorial team based on guidelines from the American Diabetes Association (ADA 2023), the Endocrine Society (ES 2022; 2020; 2016), the Kidney Disease: Improving Global Outcomes Foundation (KDIGO 2022; 2020), the Society for Cardiovascular Angiography and Interventions (SCAI/AHA/ACC 2022), the Society for Vascular Medicine (SVM/SVS/APMA 2016), and the American College of Gastroenterology (ACG 2013). 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 Definition T1DM is an endocrine disorder characterized by T-cell mediated autoimmune destruction of insulin-producing beta cells in the pancreas, resulting in insulin deficiency with resultant hyperglycemia and a predisposition to ketoacidosis. 11 Epidemiology https://web.pathway.md/diseases/recJxJJDGtvUMVAC9 1/18 6/29/23, 10:11 PM Diabetes mellitus type 1 Pathway The cause of T1DM is mostly autoimmune destruction of beta cells of the pancreas (70-90%) and idiopathic (10-30%). 13 Pathophysiology The annual incidence of T1DM in the United States is 22.9 per 100,000 person-years. 12 Disease course Environmental triggers in genetically predisposed people lead to the production of autoantibodies against insulin-producing islet beta cells of the pancreas resulting in autoimmune destruction of these cells. This leads to insulin insufficiency and overt hyperglycemia, T1DM and its complications, diabetic ketoacidosis, altered mental status, coma, and death. 14 Prognosis and risk of recurrence On average, the life expectancy of a person with T1DM is approximately 12 years less than the general population. 15 Guidelines 1. Screening and diagnosis Indications for screening (type 1 diabetes): Consider obtaining autoantibodies to insulin, glutamic acid decarboxylase, islet antigen 2, or zinc transporter 8 to screen for presymptomatic T1DM. C Recognize that multiple confirmed islet autoantibodies is a risk factor for clinical diabetes. Consider testing for dysglycemia to further forecast near-term risk. Consider referring patients with multiple islet autoantibodies to a specialized center for further evaluation and/or consideration of a clinical trial or approved therapy to potentially delay the development of clinical diabetes. B Indications for screening (neonatal diabetes): Obtain immediate genetic testing for neonatal diabetes in all patients diagnosed with diabetes in the first 6 months of life, regardless of the current age. A Consult with a center specializing in diabetes genetics to understand the significance of genetic mutations and how best to approach further evaluation, treatment, and genetic counseling in patients with neonatal diabetes mellitus. B Indications for screening (MODY): Obtain genetic testing for MODY in children and young adults without typical characteristics of T1DM or T2DM and often having a family history of diabetes in successive generations (suggestive of an autosomal dominant pattern of inheritance). A Consult with a center specializing in diabetes genetics to understand the significance of genetic mutations and how best to approach further evaluation, treatment, and genetic counseling in patients with MODY. B 2. Diagnostic investigations https://web.pathway.md/diseases/recJxJJDGtvUMVAC9 2/18 6/29/23, 10:11 PM Diabetes mellitus type 1 Pathway General principles: ensure a person-centered communication style using person-centered, culturally sensitive, and strength-based language and active listening, eliciting individual preferences and beliefs, and assessing literacy, numeracy, and potential barriers to care in order to optimize health outcomes and health-related quality of life. B Show 2 more Hemoglobin A1C: obtain HbA1c testing using a method certified by the National GlycoHgb Standardization Program and standardized to the Diabetes Control and Complications Trial assay, in order to avoid misdiagnosis or missed diagnosis. B Show 3 more Glucose tolerance test: ensure adequate carbohydrate intake (at least 150 g/day) for 3 days before oral glucose tolerance testing as a screen for diabetes. A Screening for hypertension: measure BP at every routine clinical visit. Confirm BP using multiple readings, when possible, in patients found to have elevated BP (systolic BP 120-129 mmHg and diastolic < 80 mmHg), including measurements on a separate day, to diagnose hypertension. Diagnose hypertension in case of a systolic BP 130mmHg or a diastolic BP 80 mmHg based on an average of 2 measurements obtained on 2 occasions. A Show 2 more Screening for cardiovascular diseases: Do not obtain routine screening for coronary artery disease in asymptomatic patients, as it does not improve outcomes as long as ASCVD risk factors are treated. D Consider obtaining investigations for coronary artery disease in the presence of any of the following: atypical cardiac symptoms (such as unexplained dyspnea, chest discomfort) signs or symptoms of associated vascular disease (including carotid bruits, TIA, stroke, claudication, or peripheral arterial disease) ECG abnormalities (such as Q waves). C Screening for autoimmune thyroid disease: screen for autoimmune thyroid disease soon after the diagnosis of T1DM and periodically thereafter. B Screening for celiac disease: screen for celiac disease in adult patients with T1DM in the presence of gastrointestinal symptoms, signs, laboratory manifestations, or clinical suspicion suggestive of celiac disease. B Screening for diabetic nephropathy: Assess urinary albumin (such as spot urinary albumin-to-creatinine ratio) and eGFR at least annually in all patients with T2DM regardless of treatment. B Monitor urinary albumin (such as spot urinary albumin-to-creatinine ratio) and eGFR 1-4 times per year depending on the stage of the disease in patients with established diabetic kidney disease. B Screening for diabetic retinopathy: obtain an initial dilated and comprehensive eye examination by an ophthalmologist or optometrist within 5 years after the onset of T1DM. B Show 4 more Screening for diabetic neuropathy: screen for diabetic peripheral neuropathy in all patients with T2DM starting 5 years after the diagnosis and at least annually thereafter. B https://web.pathway.md/diseases/recJxJJDGtvUMVAC9 3/18 6/29/23, 10:11 PM Diabetes mellitus type 1 Pathway Show 2 more Screening for diabetic foot: As per ADA 2023 guidelines, obtain a comprehensive foot evaluation at least annually to identify risk factors for ulcers and amputations. B Show 4 more As per SVS 2016 guidelines, consider obtaining a probe-to-bone test to aid in the diagnosis of diabetic foot osteomyelitis in patients with diabetic foot infection with an open wound. C Show 5 more Screening for anxiety: Consider screening patients with diabetes for symptoms or diabetes-related worries. Consider discussing diabetes-related worries and referring to a qualified mental health professional for further assessment and treatment if anxiety symptoms indicate interference with diabetes self-management behaviors or quality of life. C Refer patients with hypoglycemia unawareness, which can co-occur with fear of hypoglycemia, to a trained professional to receive evidence-based intervention to help re- establish awareness of symptoms of hypoglycemia and reduce fear of hypoglycemia. A Screening for depression: consider obtaining at least annual screening for depressive symptoms in all patients with diabetes, especially in patients with a self-reported history of depression. Use age-appropriate, validated depression screening measures, recognizing that further evaluation will be necessary for patients with a positive screen. C Show 2 more Screening for sleep disorders: consider screening for sleep health in patients with diabetes, including symptoms of sleep disorders, disruptions to sleep due to diabetes symptoms or management needs, and worries about sleep. Refer to sleep medicine and/or a qualified behavioral health professional as indicated. C Screening for cognitive impairment: Monitor cognitive capacity throughout the life span in all patients with diabetes, particularly in patients with documented cognitive disabilities, patients experiencing severe hypoglycemia, very young children, and older adults. B Consider referring for a formal assessment if cognitive capacity changes or appears to be suboptimal for patient decision-making and/or behavioral self-management. C Screening for serious mental illness: provide an increased level of support to patients with diabetes and serious mental illness through enhanced monitoring of and assistance with diabetes self-management behaviors. B Show 2 more Screening for disordered eating behavior: Consider screening for disordered or disrupted eating using validated screening measures when hyperglycemia and weight loss are unexplained based on self-reported behaviors related to medication dosing, meal plan, and physical activity. Review the medical treatment plan to identify potential treatment-related effects on hunger/caloric intake. C Consider reevaluating the treatment plan of patients with diabetes presenting with symptoms of disordered eating behavior, an eating disorder, or disrupted patterns of eating, in consultation with a qualified professional as available. C https://web.pathway.md/diseases/recJxJJDGtvUMVAC9 4/18 6/29/23, 10:11 PM Diabetes mellitus type 1 Pathway Screening for gastroparesis: Measure blood glucose levels in patients with diabetes before gastric emptying testing, and treat hyperglycemia with a test started after blood glucose is < 275 mg/dL. B Measure blood glucose levels in patients with diabetes before gastric emptying testing, and treat hyperglycemia with a test started after blood glucose is < 275 mg/dL. B 3. Medical management Glycemic targets: set a HbA1c goal of < 7% (53 mmol/mol) without significant hypoglycemia in many nonpregnant adult patients with diabetes. A Show 4 more Insulin therapy, indications: As per ADA 2023 guidelines, prescribe multiple daily injections of prandial and basal insulin, or continuous SC insulin infusion in most patients with T1DM. A Show 2 more As per ES 2022 guidelines, consider prescribing long-acting insulin analogs rather than human NPH insulin in adult and pediatric outpatients on basal insulin therapy at high risk for hypoglycemia. C Show 2 more As per ES 2016 guidelines, prescribe continuous subcutaneous insulin infusion over analog- based basal-bolus multiple daily injections in patients with T1DM not achieving the HbA1c goal, as long as the patient and caregivers are willing and able to use the device. B Show 3 more Insulin therapy (delivery systems): prefer insulin pens for most patients with insulin-requiring diabetes on multiple daily injections. Consider prescribing insulin syringes for insulin delivery taking into account individual and caregiver preference, insulin type, dosing therapy, cost, and self-management capabilities. B Show 6 more Monoclonal antibodies: consider administering teplizumab-mzwv infusion in a specialized setting with appropriately trained personnel to delay the onset of symptomatic T1DM in selected 8 years old patients with stage 2 T1DM. C Management of hypoglycemia: As per ADA 2023 guidelines, review occurrence and risk for hypoglycemia at every encounter and investigate as indicated. Consider assessing for awareness of hypoglycemia using validated tools. B Show 5 more As per ES 2022 guidelines, administer glucagon (preparations not requiring reconstitution) in outpatients with severe hypoglycemia. B Management of diabetic nephropathy, general principles: As per ADA 2023 guidelines, optimize glucose control to reduce the risk or slow the progression of CKD. A Show 5 more https://web.pathway.md/diseases/recJxJJDGtvUMVAC9 5/18 6/29/23, 10:11 PM Diabetes mellitus type 1 Pathway As per KDIGO 2022 guidelines, treat patients with diabetes mellitus and CKD with a comprehensive strategy to reduce risks of kidney disease progression and CVD. B Management of diabetic neuropathy: optimize glucose control to prevent or delay the development of neuropathy in patients with T1DM. B Show 2 more Management of diabetic foot: ensure a multidisciplinary approach for patients with foot ulcers and high-risk feet (such as patients on dialysis, patients with Charcot foot, history of ulcers or amputation, or peripheral arterial disease). B Show 4 more Management of hypertension (BP targets): individualize BP targets in patients with diabetes and hypertension through a shared decision-making process addressing cardiovascular risk, potential adverse effects of antihypertensive medications, and patient preferences. B Show 2 more Management of hypertension (lifestyle modifications): advise lifestyle interventions in patients with BP > 120/80 mmHg, consisting of weight loss when indicated, a DASH-style eating pattern including reducing sodium and increasing potassium intake, moderation of alcohol intake, and increased physical activity. A Management of hypertension (pharmacotherapy): initiate and titrated pharmacologic therapy to achieve BP goal of < 130/80 mmHg in patients with confirmed office-based BP 130/80 mmHg. A Show 6 more Management of cardiovascular disease (antihypertensives): Initiate ACEI or ARB therapy to reduce the risk of cardiovascular events in patients with known ASCVD, particularly coronary artery disease. A Initiate -blockers with proven cardiovascular outcomes benefits in patients with HFrEF, unless otherwise contraindicated. A Continue -blockers for 3 years after myocardial infarction. B Management of cardiovascular disease (antiplatelets): consider initiating aspirin therapy (75-162 mg/ day) as a primary prevention strategy in patients with diabetes at increased cardiovascular risk, after a comprehensive discussion with the patient on the benefits versus the comparable increased risk of bleeding. B Show 6 more Management of cardiovascular disease (revascularization): perform CABG surgery (with a left internal mammary artery to the left anterior descending artery) over PCI to reduce mortality and repeat revascularizations in patients with diabetes and multivessel coronary artery disease with the involvement of the left anterior descending artery being appropriate candidates for CABG. A Show 3 more Management of dyslipidemia (lifestyle modifications): Advise lifestyle modifications focusing on weight loss (if indicated), following a Mediterranean style or DASH eating pattern, reducing consumption of saturated fat and trans fat, increasing dietary omega-3 fatty acids, viscous fiber, and plant stanols/sterols intake, and increasing https://web.pathway.md/diseases/recJxJJDGtvUMVAC9 6/18 6/29/23, 10:11 PM Diabetes mellitus type 1 Pathway physical activity to improve the lipid profile and reduce the risk of developing ASCVD in patients with diabetes. A Intensify lifestyle therapy and optimize glycemic control in patients with elevated triglyceride levels ( 150 mg/dL) and/or low HDL cholesterol (< 40 mg/dL for males, < 50 mg/dL for females). B Management of dyslipidemia, statin therapy: As per ADA 2023 guidelines, initiate moderate-intensity statin therapy, in addition to lifestyle therapy, in 40-75 years old patients with diabetes without ASCVD. A Show 5 more As per ES 2020 guidelines, consider initiating statin therapy, irrespective of the cardiovascular risk score, to reduce cardiovascular risks in 40 years old patients with T1DM and/or with duration of diabetes > 20 years and/or microvascular complications. C Show 3 more Management of dyslipidemia (non-statin lipid-lowering therapy): consider adding ezetimibe or a PCSK9 inhibitor to maximum tolerated statin therapy in 40-75 years old patients with diabetes at higher cardiovascular risk, especially patients with multiple ASCVD risk factors and an LDL cholesterol 70 mg/dL. C Show 3 more Management of dyslipidemia (lipid profile monitoring): Consider obtaining a lipid profile at the time of diabetes diagnosis, at an initial medical evaluation, and every 5 years thereafter if under the age of 40 years or more frequently if indicated, in adult patients with diabetes not taking statins or other lipid-lowering therapy. C Obtain lipid profile at the initiation of statins or other lipid-lowering therapy, 4-12 weeks after initiation or a change in dose, and annually thereafter to monitor the response to therapy and inform medication taking. B Management of dyslipidemia (hypertriglyceridemia): evaluate for secondary causes of hypertriglyceridemia and consider initiating medical therapy to reduce the risk of pancreatitis in patients with fasting triglyceride levels 500 mg/dL. B Show 2 more Management of diabetic nephropathy (smoking cessation): Advise patients with diabetes mellitus and CKD to quit using tobacco products. B Counsel patients with diabetes mellitus and CKD to reduce secondhand smoke exposure. B Management of diabetic nephropathy, RAAS blockade: As per ADA 2023 guidelines, initiate either ACEIs or ARBs in nonpregnant patients with diabetes and hypertension with severely increased albuminuria (urinary albumin-to-creatinine ratio 300 mg/g creatinine) and/or eGFR < 60 mL/min/1.73 m . A Show 4 more As per KDIGO 2022 guidelines, initiate an ACEI or an ARB in patients with diabetes, hypertension and albuminuria and titrate the medications to the highest approved dose that is tolerated. B Show 7 more Management of diabetic nephropathy (kidney transplantation): https://web.pathway.md/diseases/recJxJJDGtvUMVAC9 7/18 6/29/23, 10:11 PM Diabetes mellitus type 1 Pathway Consider performing kidney transplantation in kidney transplant candidates with T1DM. C Consider performing simultaneous pancreas-kidney transplantation in kidney transplant candidates with end-stage kidney disease and T1DM in regions where this procedure is available. C Management of diabetic retinopathy: optimize glycemic control to reduce the risk or slow the progression of diabetic retinopathy. A Show 7 more 4. Inpatient care Management of hospitalized patients, general principles: As per ADA 2023 guidelines, consult with a specialized diabetes or glucose management team, when possible, when caring for hospitalized patients with diabetes. B As per ES 2022 guidelines, consider providing inpatient diabetes education as part of a comprehensive diabetes discharge-planning process in adult patients with diabetes hospitalized for a non-critical illness. C Management of hospitalized patients (HbA1C testing): obtain HbA1c testing in all patients with diabetes or hyperglycemia (blood glucose > 140 mg/dL; 7.8 mmol/L) admitted to the hospital, if not performed in the prior 3 months. B Management of hospitalized patients, glucose monitoring: As per ADA 2023 guidelines, support patients to continue using diabetes devices, such as insulin pumps and continuous glucose monitoring systems, in an inpatient setting or during outpatient procedures, once competency in safely using these devices is established and proper supervision is available. B As per ES 2022 guidelines, consider obtaining real-time continuous glucose monitoring with confirmatory bedside point-of-care blood glucose monitoring for adjustments in insulin dosing (in hospital settings where resources and training are available) in adult patients with insulin- treated diabetes at high risk of hypoglycemia hospitalized for a non-critical illness. C As per ES 2022 guidelines, consider initiating continuous glucose monitoring in the inpatient setting for selected patients at high risk for hypoglycemia. C Show 2 more Management of hospitalized patients, insulin therapy: As per ADA 2023 guidelines, use validated written or computerized protocols for insulin administration allowing for predefined adjustments in the insulin dosage based on glycemic fluctuations. B Show 4 more As per ES 2022 guidelines, consider administering scheduled insulin therapy for glycemic control in most adult patients with hyperglycemia hospitalized for a non-critical illness. C Show 7 more As per ES 2016 guidelines, continue continuous subcutaneous insulin infusion in patients admitted to the hospital if the institution has clear protocols for evaluating patients as suitable candidates and appropriate monitoring and safety procedures. B https://web.pathway.md/diseases/recJxJJDGtvUMVAC9 8/18 6/29/23, 10:11 PM Diabetes mellitus type 1 Pathway Management of hospitalized patients, treatment targets: As per ADA 2023 guidelines: Set a target glucose range of 140-180 mg/dL (7.8-10.0 mmol/L) for the majority of critically ill and non-critically ill patients once insulin therapy is started. A Consider setting more stringent goals, such as 110-140 mg/dL (6.1-7.8 mmol/L), in selected patients if those can be achieved without significant hypoglycemia. C As per ES 2022 guidelines: Consider targeting preoperative HbA1c levels of < 8% (63.9 mmol/mol) and blood glucose levels of 100-180 mg/dL (5.6-10 mmol/L) in adult patients with diabetes undergoing elective surgical procedures. C Consider targeting preoperative blood glucose levels of 100-180 mg/dL (5.6-10 mmol/L) in adult patients with diabetes undergoing elective surgical procedures if a HbA1c < 8% (63.9 mmol/mol) target is not feasible. C Management of hospitalized patients (prevention of hypoglycemia): Review treatment regimens and change as necessary to prevent further hypoglycemia when a blood glucose value of < 70 mg/dL (3.9 mmol/L) is documented. B Adopt a hypoglycemia management protocol and implement it in each hospital or hospital system. Establish a plan for the prevention and treatment of hypoglycemia for each patient. Document episodes of hypoglycemia in the hospital in the medical record and track for quality improvement/quality assessment. B Management of hospitalized patients (oral carbohydrate fluids): avoid administering preoperative carbohydrate-containing oral fluids in adult patients with diabetes undergoing surgical procedures. D 5. Nonpharmacologic interventions Smoking cessation: advise all patients to abstain from using cigarettes and other tobacco products or e-cigarettes. A Show 2 more Physical activity: advise most adults with T1DM to engage in 150 minutes of moderate- to vigorous-intensity aerobic activity per week, spread over at least 3 days/week, with no more than 2 consecutive days without activity. Consider advising shorter durations (minimum 75 min/week) of vigorous-intensity or interval training in younger and more physically fit patients. B Show 4 more Dietary modifications (general principles): provide an individualized medical nutrition therapy program as needed to achieve treatment goals, provided by a registered dietitian nutritionist, preferably one who has comprehensive knowledge and experience in diabetes care, in all patients with T1DM. A Show 2 more Dietary modifications (eating patterns): Recognize that there is no ideal macronutrient pattern for patients with diabetes. Individualize meal plans while keeping nutrient quality, total calorie, and metabolic goals in https://web.pathway.md/diseases/recJxJJDGtvUMVAC9 9/18 6/29/23, 10:11 PM Diabetes mellitus type 1 Pathway mind. B Reduce overall carbohydrate intake in patients with diabetes for improving glycemia. Consider applying this to a variety of eating patterns meeting individual needs and preferences. B Dietary modifications (carbohydrates): advise carbohydrate intake emphasizing nutrient- dense carbohydrate sources high in fiber (at least 14 g fiber per 1,000 kcal) and minimally processed. Advise eating plans emphasizing nonstarchy vegetables, fruits, legumes, and whole grains, as well as dairy products, with minimal added sugars. B Show 3 more Dietary modifications (fat): Consider advising an eating plan emphasizing elements of a Mediterranean eating pattern rich in monounsaturated and polyunsaturated fats to improve glucose metabolism and lower CVD risk. C Advise eating foods rich in long-chain omega-3 fatty acids, such as fatty fish (eicosapentaenoic acid and docosahexaenoic acid) and nuts and seeds (alpha-linolenic acid), to prevent or treat CVD. B Dietary modifications (nonnutritive sweeteners): Recognize that the use of nonnutritive sweeteners as a replacement for sugar-sweetened products may reduce overall calorie and carbohydrate intake as long as there is not a compensatory increase in energy intake from other sources. B Encourage patients to decrease both sweetened and nonnutritive-sweetened beverages, with an emphasis on water intake. B Dietary modifications (sodium): limit sodium consumption to < 2,300 mg/day. B Dietary modifications (micronutrients and supplements): insufficient evidence to support the use of dietary supplementation with vitamins, minerals (such as chromium and vitamin D), herbs, or spices (such as cinnamon or aloe vera) to improve outcomes in patients with diabetes not having underlying deficiencies, and for glycemic control. I Dietary modifications (alcohol): Advise adult patients with diabetes drinking alcohol to do so in moderation ( 1 drink/day for adult females and 2 drinks/day for adult males). B Educate patients with diabetes about the signs, symptoms, and self-management of delayed hypoglycemia after drinking alcohol, especially when using insulin or insulin secretagogues. Emphasize the importance of glucose monitoring after drinking alcoholic beverages to reduce hypoglycemia risk. B Psychosocial care: provide psychosocial care, integrated with routine medical care and delivered by trained healthcare professionals using a collaborative, person-centered, culturally informed approach, in all patients with diabetes, with the goal of optimizing health-related quality of life and health outcomes. A Refer to qualified mental health professionals for additional targeted mental health care, when indicated and available. B Show 4 more 6. Specific circumstances https://web.pathway.md/diseases/recJxJJDGtvUMVAC9 10/18 6/29/23, 10:11 PM Diabetes mellitus type 1 Pathway Elderly patients (screening for comorbidities): consider assessing medical, psychological, functional (self-management abilities), and social domains in older adults to provide a framework to determine targets and therapeutic approaches for diabetes management. C Show 2 more Elderly patients (screening for hypoglycemia): ascertain and address episodes of hypoglycemia in older adults with diabetes at routine visits because they have a greater risk of hypoglycemia than younger adults. B Show 3 more Elderly patients (treatment goals): set lower glycemic goals (such as HbA1c < 7.0-7.5%; 53- 58 mmol/mol) in older patients otherwise healthy with few coexisting chronic illnesses and intact cognitive function and functional status. B Show 5 more Elderly patients (lifestyle modifications): Ensure optimal nutrition and protein intake in older patients. B Encourage regular exercise, including aerobic activity, weight-bearing exercise, and/or resistance training, in all older patients able to safely engage in such activities. B Elderly patients (pharmacotherapy): avoid overtreatment of diabetes in older patients. D Show 3 more Elderly patients (residing in nursing facilities): obtain careful assessment in patients with diabetes residing in long-term care facilities to establish individualized glycemic goals and to make appropriate choices of glucose-lowering agents based on their clinical and functional status. B Show 2 more Elderly patients (end-of-life care): Initiate conversations regarding the goals and intensity of care when palliative care is required in older patients with diabetes. Avoid strict glucose and BP control. B Consider simplifying regimens. Consider relaxing the intensity of lipid management or withdrawing lipid-lowering therapy. A Set overall comfort, prevention of distressing symptoms, and preservation of quality of life and dignity as primary goals for diabetes management at the end of life. B Pregnant patients (preconception counseling and care): incorporate preconception counseling into routine diabetes care starting at puberty and continuing in all patients with diabetes and reproductive potential. A Show 6 more Pregnant patients (nutrition): encourage a balanced intake of macronutrients including nutrient-dense fruits, vegetables, legumes, whole grains, and healthy fats with omega-3 fatty acids (including nuts, seeds, and fish) in the eating pattern. B Pregnant patients (glycemic targets): obtain fasting and postprandial blood glucose monitoring in both gestational diabetes mellitus and preexisting diabetes in pregnancy, to achieve optimal glucose levels. Set the following glucose targets: fasting plasma glucose < 95 mg/dL (5.3 mmol/L) and either 1-hour postprandial glucose < 140 mg/dL (7.8 mmol/L) or 2- hour postprandial glucose < 120 mg/dL (6.7 mmol/L). Obtain blood glucose preprandially in some patients with preexisting diabetes. B https://web.pathway.md/diseases/recJxJJDGtvUMVAC9 11/18 6/29/23, 10:11 PM Diabetes mellitus type 1 Pathway Show 5 more Pregnant patients (management of preexisting diabetes): Prescribe insulin for the management of T1DM in pregnancy. A Consider prescribing either multiple daily injections or insulin pump technology in pregnancy complicated by T1DM. C Pregnant patients (management of gestational diabetes): offer lifestyle behavior changes for the management of gestational diabetes mellitus. Add insulin if needed to achieve glycemic targets. A Show 5 more Pregnant patients (BP targets): consider targeting a BP of 110-135/85 mmHg in pregnant patients with diabetes and chronic hypertension in the interest of reducing the risk for accelerated maternal hypertension. B Titrate therapy from a BP threshold of 140/90 mmHg for better pregnancy outcomes with no increase in the risk of small-for-gestational-age birth weight. B Lessen therapy for BP < 90/60 mmHg. C Pregnant patients (prevention of preeclampsia): prescribe low-dose aspirin 100-150 mg/day (162 mg/day may also be acceptable) starting at 12-16 weeks of gestation to lower the risk of preeclampsia in patients with T1DM. B Pregnant patients (postpartum care): evaluate and adjust insulin requirements postpartum as insulin resistance decreases dramatically immediately postpartum and the requirements are often roughly half the prepregnancy requirements for the initial few days postpartum. B Show 7 more Pediatric patients (screening for hypertension): measure BP at every routine visit. Consider obtaining ambulatory BP monitoring in young patients with high BP (BP 90th percentile for age, sex, and height or, in adolescents aged 13 years, BP 120/80 mmHg) on three separate measurements. B Pediatric patients (screening for dyslipidemia): obtain initial lipid profile soon after diagnosis, preferably after glycemia has improved and age is 2 years. Obtain subsequent testing at 9-11 years of age if initial LDL cholesterol is 100 mg/dL (2.6 mmol/L). B Show 2 more Pediatric patients (screening for autoimmune thyroid disease): assess for additional autoimmune conditions soon after the diagnosis of T1DM and if symptoms develop. B Show 2 more Pediatric patients (screening for celiac disease): screen young patients with T1DM for celiac disease by measuring anti-tTG (IgA) with documentation of normal total serum IgA levels, soon after the diagnosis of diabetes, or anti-tTG (IgG) and deamidated gliadin antibodies if IgA is deficient. B Show 2 more Pediatric patients (screening for disordered eating behavior): screen young patients with T1DM for disordered eating starting at 10-12 years of age. Refer to a qualified mental health professional for further assessment and treatment if indicated. B Pediatric patients (screening for diabetic nephropathy): consider obtaining annual screening for albuminuria with a random (morning sample preferred to avoid effects of https://web.pathway.md/diseases/recJxJJDGtvUMVAC9 12/18 6/29/23, 10:11 PM Diabetes mellitus type 1 Pathway exercise) spot urine sample for albumin-to-creatinine ratio at puberty or at age > 10 years, whichever is earlier, once the patient has had diabetes for 5 years. C Pediatric patients (screening for diabetic retinopathy): obtain an initial dilated and comprehensive eye examination once the patient has had T1DM for 3-5 years, provided the patient is aged 11 years or puberty has started, whichever is earlier. B Show 2 more Pediatric patients (screening for diabetic foot): consider obtaining an annual comprehensive foot examination (including inspection, assessment of foot pulses, pinprick, and 10-g monofilament sensation tests, testing of vibration sensation using a 128-Hz tuning fork, and ankle reflex tests) at the start of puberty or at age 10 years, whichever is earlier, once the patient has had T1DM for 5 years. C Pediatric patients (glycemic targets): individualize HbA1c goals and reassess over time, recognizing that an HbA1c level of < 7% (53 mmol/mol) is appropriate for many pediatric and adolescent patients. B Show 3 more Pediatric patients (glucose monitoring): advise all young patients with T1DM to monitor glucose levels multiple times daily (up to 6-10 times/day by blood glucose meter or continuous glucose monitoring), including before meals and snacks, at bedtime, and as needed for safety in specific situations, such as exercise, driving, or the presence of symptoms of hypoglycemia. B Show 3 more Pediatric patients (insulin delivery devices): offer automated insulin delivery systems for diabetes management in young patients with T1DM capable of using the device safely (either by themselves or with caregivers). Decide on the choice of the device based on the patient's and family's circumstances, desires, and needs. A Show 2 more Landmark trials: Insulin-Only Bionic Pancreas In participants 6 years of age with T1DM, bionic pancreas were superior to standard care with respect to glycated Hgb level at week 13. Bionic Pancreas Research Group et al. N Engl J Med. 2022 Sep 29. Pediatric patients (dietary modifications): offer individualized medical nutrition therapy as an essential component of the overall treatment plan in young patients with T1DM. A Show 2 more Pediatric patients (physical activity): advise physical activity in all young patients with T1DM with the goal of 60 minutes of moderate- to vigorous-intensity aerobic activity daily, with vigorous muscle-strengthening and bone-strengthening activities at least 3 days per week. B Show 3 more Pediatric patients (smoking cessation): Elicit a smoking history at initial and follow-up diabetes visits. Discourage smoking in non- smokers and encourage smoking cessation in smokers. A https://web.pathway.md/diseases/recJxJJDGtvUMVAC9 13/18 6/29/23, 10:11 PM Diabetes mellitus type 1 Pathway Discourage the use of electronic cigarettes. A Pediatric patients (psychosocial care): screen for psychosocial issues and family stresses (likely to impact diabetes management) at diagnosis and during routine follow-up care and refer to trained mental health professionals, preferably experienced in childhood diabetes. B Show 6 more Pediatric patients (self-management counseling): provide culturally sensitive and developmentally appropriate individualized diabetes self-management education and support to young patients with T1DM and their parents/caregivers, according to national standards at diagnosis and routinely thereafter. B Pediatric patients (preconception counseling): incorporate preconception counseling starting at puberty into routine diabetes care for all female patients of childbearing potential. A Pediatric patients (management of hypertension): offer lifestyle modification focused on healthy nutrition, physical activity, sleep, and, if appropriate, weight management for the treatment of elevated BP (defined as the 90-95th percentile for age, gender, and height or, in adolescents aged 13 years, 120-129/< 80 mmHg). B Show 2 more Pediatric patients (management of dyslipidemia): optimize glycemia and offer medical nutrition therapy to limit the number of calories from fat to 25-30% and saturated fat to < 7%, limit cholesterol to < 200 mg/day, avoid trans fats and aim for 10% calories from monounsaturated fats, as initial therapy if lipids are abnormal. A Show 3 more Pediatric patients (management of diabetic nephropathy): Consider initiating an ACEI or an ARB, titrated to normalization of albumin excretion, when elevated urinary albumin-to-creatinine ratio (> 30 mg/g) is documented (2 of 3 urine samples obtained over a 6-month interval following efforts to improve glycemic control and normalize BP). C Provide reproductive counseling and avoid using ACEIs and ARBs in individuals of childbearing age not using reliable contraception, due to the potential teratogenic effects. B Pediatric patients (transition to adult care): Begin to prepare young patients for transition to adult healthcare in early adolescence and, at the latest, at least 1 year before the transition. B Provide support and resources for transitioning young adults by both pediatric and adult diabetes care providers. B Patients with COVID-19 infection: support patients with diabetes to achieve individualized targeted glycemic control to reduce the risk of macrovascular and microvascular risk as well as reduce the risk of COVID-19 and its complications. B Show 8 more 7. Patient education Self-management counseling: offer all patients with diabetes participation in self- management education and support to facilitate the knowledge, decision-making, and skills mastery for diabetes self-care. A https://web.pathway.md/diseases/recJxJJDGtvUMVAC9 14/18 6/29/23, 10:11 PM Diabetes mellitus type 1 Pathway Show 9 more 8. Preventative measures Routine immunizations: provide routine immunizations in pediatric and adult patients with diabetes as indicated by age. A 9. Follow-up and surveillance Treatment monitoring: include most components of the initial comprehensive medical evaluation in the follow-up visit. A Show 3 more Continuous glucose monitoring: As per ES 2022 guidelines: Obtain continuous glucose monitoring rather than self-monitoring of blood glucose by fingerstick in patients with T1DM receiving multiple daily injections. B Consider obtaining real-time continuous glucose monitoring and algorithm-driven insulin pumps rather than multiple daily injections with self-monitoring of blood glucose TID in adult and pediatric patients with T1DM. C As per ES 2016 guidelines, obtain real-time continuous glucose monitoring in adult patients with T1DM with HbA1c levels above target and are willing and able to use these devices on a nearly daily basis. A Show 2 more Continous glucose monitoring: as per ADA 2023 guidelines, obtain real-time continuous glucose monitoring A or intermittently scanned continuous glucose monitoring B in adult patients with diabetes on multiple daily injections or continuous subcutaneous insulin infusion capable of using the devices safely, either by themselves or with a caregiver. Decide on the choice of the device based on the patient's circumstances, preferences, and needs. A |
Show 2 more Pediatric patients (screening for disordered eating behavior): screen young patients with T1DM for disordered eating starting at 10-12 years of age. Refer to a qualified mental health professional for further assessment and treatment if indicated. B Pediatric patients (screening for diabetic nephropathy): consider obtaining annual screening for albuminuria with a random (morning sample preferred to avoid effects of https://web.pathway.md/diseases/recJxJJDGtvUMVAC9 12/18 6/29/23, 10:11 PM Diabetes mellitus type 1 Pathway exercise) spot urine sample for albumin-to-creatinine ratio at puberty or at age > 10 years, whichever is earlier, once the patient has had diabetes for 5 years. C Pediatric patients (screening for diabetic retinopathy): obtain an initial dilated and comprehensive eye examination once the patient has had T1DM for 3-5 years, provided the patient is aged 11 years or puberty has started, whichever is earlier. B Show 2 more Pediatric patients (screening for diabetic foot): consider obtaining an annual comprehensive foot examination (including inspection, assessment of foot pulses, pinprick, and 10-g monofilament sensation tests, testing of vibration sensation using a 128-Hz tuning fork, and ankle reflex tests) at the start of puberty or at age 10 years, whichever is earlier, once the patient has had T1DM for 5 years. C Pediatric patients (glycemic targets): individualize HbA1c goals and reassess over time, recognizing that an HbA1c level of < 7% (53 mmol/mol) is appropriate for many pediatric and adolescent patients. B Show 3 more Pediatric patients (glucose monitoring): advise all young patients with T1DM to monitor glucose levels multiple times daily (up to 6-10 times/day by blood glucose meter or continuous glucose monitoring), including before meals and snacks, at bedtime, and as needed for safety in specific situations, such as exercise, driving, or the presence of symptoms of hypoglycemia. B Show 3 more Pediatric patients (insulin delivery devices): offer automated insulin delivery systems for diabetes management in young patients with T1DM capable of using the device safely (either by themselves or with caregivers). Decide on the choice of the device based on the patient's and family's circumstances, desires, and needs. A Show 2 more Landmark trials: Insulin-Only Bionic Pancreas In participants 6 years of age with T1DM, bionic pancreas were superior to standard care with respect to glycated Hgb level at week 13. Bionic Pancreas Research Group et al. N Engl J Med. 2022 Sep 29. Pediatric patients (dietary modifications): offer individualized medical nutrition therapy as an essential component of the overall treatment plan in young patients with T1DM. A Show 2 more Pediatric patients (physical activity): advise physical activity in all young patients with T1DM with the goal of 60 minutes of moderate- to vigorous-intensity aerobic activity daily, with vigorous muscle-strengthening and bone-strengthening activities at least 3 days per week. B Show 3 more Pediatric patients (smoking cessation): Elicit a smoking history at initial and follow-up diabetes visits. Discourage smoking in non- smokers and encourage smoking cessation in smokers. A https://web.pathway.md/diseases/recJxJJDGtvUMVAC9 13/18 6/29/23, 10:11 PM Diabetes mellitus type 1 Pathway Discourage the use of electronic cigarettes. A Pediatric patients (psychosocial care): screen for psychosocial issues and family stresses (likely to impact diabetes management) at diagnosis and during routine follow-up care and refer to trained mental health professionals, preferably experienced in childhood diabetes. B Show 6 more Pediatric patients (self-management counseling): provide culturally sensitive and developmentally appropriate individualized diabetes self-management education and support to young patients with T1DM and their parents/caregivers, according to national standards at diagnosis and routinely thereafter. B Pediatric patients (preconception counseling): incorporate preconception counseling starting at puberty into routine diabetes care for all female patients of childbearing potential. A Pediatric patients (management of hypertension): offer lifestyle modification focused on healthy nutrition, physical activity, sleep, and, if appropriate, weight management for the treatment of elevated BP (defined as the 90-95th percentile for age, gender, and height or, in adolescents aged 13 years, 120-129/< 80 mmHg). B Show 2 more Pediatric patients (management of dyslipidemia): optimize glycemia and offer medical nutrition therapy to limit the number of calories from fat to 25-30% and saturated fat to < 7%, limit cholesterol to < 200 mg/day, avoid trans fats and aim for 10% calories from monounsaturated fats, as initial therapy if lipids are abnormal. A Show 3 more Pediatric patients (management of diabetic nephropathy): Consider initiating an ACEI or an ARB, titrated to normalization of albumin excretion, when elevated urinary albumin-to-creatinine ratio (> 30 mg/g) is documented (2 of 3 urine samples obtained over a 6-month interval following efforts to improve glycemic control and normalize BP). C Provide reproductive counseling and avoid using ACEIs and ARBs in individuals of childbearing age not using reliable contraception, due to the potential teratogenic effects. B Pediatric patients (transition to adult care): Begin to prepare young patients for transition to adult healthcare in early adolescence and, at the latest, at least 1 year before the transition. B Provide support and resources for transitioning young adults by both pediatric and adult diabetes care providers. B Patients with COVID-19 infection: support patients with diabetes to achieve individualized targeted glycemic control to reduce the risk of macrovascular and microvascular risk as well as reduce the risk of COVID-19 and its complications. B Show 8 more 7. Patient education Self-management counseling: offer all patients with diabetes participation in self- management education and support to facilitate the knowledge, decision-making, and skills mastery for diabetes self-care. A https://web.pathway.md/diseases/recJxJJDGtvUMVAC9 14/18 6/29/23, 10:11 PM Diabetes mellitus type 1 Pathway Show 9 more 8. Preventative measures Routine immunizations: provide routine immunizations in pediatric and adult patients with diabetes as indicated by age. A 9. Follow-up and surveillance Treatment monitoring: include most components of the initial comprehensive medical evaluation in the follow-up visit. A Show 3 more Continuous glucose monitoring: As per ES 2022 guidelines: Obtain continuous glucose monitoring rather than self-monitoring of blood glucose by fingerstick in patients with T1DM receiving multiple daily injections. B Consider obtaining real-time continuous glucose monitoring and algorithm-driven insulin pumps rather than multiple daily injections with self-monitoring of blood glucose TID in adult and pediatric patients with T1DM. C As per ES 2016 guidelines, obtain real-time continuous glucose monitoring in adult patients with T1DM with HbA1c levels above target and are willing and able to use these devices on a nearly daily basis. A Show 2 more Continous glucose monitoring: as per ADA 2023 guidelines, obtain real-time continuous glucose monitoring A or intermittently scanned continuous glucose monitoring B in adult patients with diabetes on multiple daily injections or continuous subcutaneous insulin infusion capable of using the devices safely, either by themselves or with a caregiver. Decide on the choice of the device based on the patient's circumstances, preferences, and needs. A Show 12 more Monitoring of glycemic status: assess glycemic status (HbA1c or other glycemic measurements, such as time in range or glucose management indicator) at least two times a year in patients meeting treatment goals (and having stable glycemic control). B Show 3 more Landmark trials: FLASH-UK In adult participants with T1DM and glycated Hgb levels between 7.5-11.0%, flash glucose monitoring was superior to self-monitoring of blood glucose with respect to glycated Hgb level at 24 weeks. Lalantha Leelarathna et al. N Engl J Med. 2022 Oct 20. https://web.pathway.md/diseases/recJxJJDGtvUMVAC9 15/18 6/29/23, 10:11 PM Diabetes mellitus type 1 Pathway 10. Quality improvement Health promotion: ensure that treatment decisions are timely, rely on evidence-based guidelines, include social community support, and are made collaboratively with patients based on individual preferences, prognoses, comorbidities, and informed financial considerations. B Show 5 more Use of diabetes technology: individualize the type and selection of devices based on the patient's specific needs, preferences, and skill level. Include the caregiver's skills and preferences in the decision-making process in the setting of a patient with diabetes partially or wholly managed by someone else (such as a young child or a person with cognitive impairment or dexterity, psychosocial, and/or physical limitations). B Show 5 more Clinical findings Symptoms Past medical history Abdominal pain Addison's disease Confusion CKD Fatigue Celiac disease Headache Diabetic foot Increased appetite Diabetic nephropathy Itching Diabetic neuropathy Nocturia Diabetic retinopathy Polydipsia Dyslipidemia Polyuria Graves' disease Urinary incontinence Hashimoto's disease Vomiting Pernicious anemia Weight loss Vitiligo Neurological exam Integument exam Lethargy Dehydration Skin dryness Lab findings Serological findings Diabetic ketoacidosis Hyperglycemia anti-glutamic acid decarboxylase autoantibodies Metabolic acidosis anti-insulin autoantibodies serum HbA1c anti-islet cell autoantibodies urine glucose anti-zinc transporter 8 autoantibodies C-peptide insulinoma-associated-2 autoantibodies Imaging findings Cerebral edema Diagnostic procedures https://web.pathway.md/diseases/recJxJJDGtvUMVAC9 16/18 6/29/23, 10:11 PM Diabetes mellitus type 1 Pathway Abnormal oral glucose tolerance Studies 2022 FLASH-UK In adult participants with T1DM and glycated Hgb levels between 7.5-11.0%, flash glucose monitoring was superior to self-monitoring of blood glucose with respect to glycated Hgb level at 24 weeks. Lalantha Leelarathna et al. N Engl J Med. 2022 Oct 20. 2022 Insulin-Only Bionic Pancreas In participants 6 years of age with T1DM, bionic pancreas were superior to standard care with respect to glycated Hgb level at week 13. Bionic Pancreas Research Group et al. N Engl J Med. 2022 Sep 29. Show 4 more References 1. Steven E. Kahn, Cheryl A.M. Anderson, John B. Buse et al. Standards of Care in Diabetes 2023. Diabetes Care. 2023 Jan;46 Supplement_1 S1 S291. Open 2. Mary T Korytkowski, Ranganath Muniyappa, Kellie Antinori-Lent et al. Management of Hyperglycemia in Hospitalized Adult Patients in Non-Critical Care Settings: An Endocrine Society Clinical Practice Guideline. J Clin Endocrinol Metab. 2022 Jun 12;dgac278. Open 3. Anthony L McCall, David C Lieb, Roma Gianchandani et al. Management of Individuals With Diabetes at High Risk for Hypoglycemia: An Endocrine Society Clinical Practice Guideline. J Clin Endocrinol Metab. 2022 Dec 7;dgac596. Open 4. Kidney Disease: Improving Global Outcomes KDIGO Diabetes Work Group. KDIGO 2022 Clinical Practice Guideline for Diabetes Management in Chronic Kidney Disease. Kidney Int. 2022 Nov;102 5S S1 S127. Open 5. Anil Hingorani, Glenn M LaMuraglia, Peter Henke et al. The management of diabetic foot: A clinical practice guideline by the Society for Vascular Surgery in collaboration with the American Podiatric Medical Association and the Society for Vascular Medicine. J Vasc Surg. 2016 Feb;63 2 Suppl):3S 21S. Open 6. Jennifer S Lawton, Jacqueline E Tamis-Holland, Sripal Bangalore et al. 2021 ACC/AHA/SCAI Guideline for Coronary Artery Revascularization: A Report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines. Circulation. 2022 Jan 18;145 3):e18-e114. Open 7. Anne L Peters, Andrew J Ahmann, Tadej Battelino et al. Diabetes Technology-Continuous Subcutaneous Insulin Infusion Therapy and Continuous Glucose Monitoring in Adults: An Endocrine Society Clinical Practice Guideline. J Clin Endocrinol Metab. 2016 Nov;101 11 3922 3937. Open 8. Camilleri M, Parkman HP, Shafi MA et al. Clinical guideline: management of gastroparesis. Am J Gastroenterol. 2013 Jan;108 1 18 37. Open https://web.pathway.md/diseases/recJxJJDGtvUMVAC9 17/18 6/29/23, 10:11 PM Diabetes mellitus type 1 Pathway 9. Steven J Chadban, Curie Ahn, David A Axelrod et al. KDIGO Clinical Practice Guideline on the Evaluation and Management of Candidates for Kidney Transplantation. Transplantation. 2020 Apr;104 4S1 Suppl 1 S11 S103. Open 10. Connie B Newman, Michael J Blaha, Jeffrey B Boord et al. Lipid Management in Patients with Endocrine Disorders: An Endocrine Society Clinical Practice Guideline. J Clin Endocrinol Metab. 2020 Dec 1;105 12):dgaa674. Open 11. Kahanovitz L, Sluss PM, Russell SJ. Type 1 Diabetes A Clinical Perspective. Point Care. 2017 Mar;16 1 37 40. Open 12. Rogers MAM, Kim C, Banerjee T et al. Fluctuations in the incidence of type 1 diabetes in the United States from 2001 to 2015 a longitudinal study. BMC Med. 2017 Nov 8;15 1 199. Open 13. Atkinson MA, Eisenbarth GS, Michels AW. Type 1 diabetes. Lancet. 2014 Jan 4;383 9911 69 82. Open 14. Kimber M Simmons, Aaron W Michels. Type 1 diabetes: A predictable disease. World J Diabetes. 2015 Apr 15;6 3 380 90. Open 15. Copenhaver M, Hoffman RP. Type 1 diabetes: where are we in 2017?. Transl Pediatr. 2017 Oct;6 4 359 364. Open 16. Jeff A Simerville, William C Maxted, John J Pahira. Urinalysis: a comprehensive review. Am Fam Physician. 2005 Mar 15;71 6 1153 62. Open 17. ADA. Executive summary: Standards of medical care in diabetes 2013. Diabetes Care. 2013 Jan;36 Suppl 1 S4 10. Open 18. American Diabetes Association. Standards of Medical Care in Diabetes-2017 Abridged for Primary Care Providers. Clin Diabetes. 2017 Jan;35 1 5 26. Open 19. American Diabetes Association. 10. Microvascular Complications and Foot Care: Standards of Medical Care in Diabetes-2018. Diabetes Care. 2018 Jan;41 Suppl 1 S105 S118. Open 20. Diabetes Canada Clinical Practice Guidelines Expert Committee, McFarlane P, Cherney D et al. Chronic Kidney Disease in Diabetes. Can J Diabetes. 2018 Apr;42 Suppl 1 S201 S209. Open 21. Game FL, Attinger C, Hartemann A et al. IWGDF guidance on use of interventions to enhance the healing of chronic ulcers of the foot in diabetes. Diabetes Metab Res Rev. 2016 Jan;32 Suppl 1 75 83. Open 22. American Diabetes Association. Standards of Medical Care in Diabetes-2019 Abridged for Primary Care Providers. Clin Diabetes. 2019 Jan;37 1 11 34. Open 23. American Diabetes Association. 11. Microvascular Complications and Foot Care: Standards of Medical Care in Diabetes 2019. Diabetes Care. 2019 Jan;42 Suppl 1 S124 S138. Open 24. American Diabetes Association Professional Practice Committee, American Diabetes Association Professional Practice Committee:, Draznin B et al. Standards of Medical Care in Diabetes-2022. Diabetes Care. 2022 Jan 1;45 Supplement_1 S1 S264. Open 25. Bionic Pancreas Research Group, Steven J Russell, Roy W Beck et al. Multicenter, Randomized Trial of a Bionic Pancreas in Type 1 Diabetes. N Engl J Med. 2022 Sep 29;387 13 1161 1172. Open 26. Lalantha Leelarathna, Mark L Evans, Sankalpa Neupane et al. Intermittently Scanned Continuous Glucose Monitoring for Type 1 Diabetes. N Engl J Med. 2022 Oct 20;387 16 1477 1487. Open 27. Diabetes Canada Clinical Practice Guidelines Expert Committee, Jean-Fran ois Yale, Breay Paty et al. Hypoglycemia. Can J Diabetes. 2018 Apr;42 Suppl 1 S104 S108. Open https://web.pathway.md/diseases/recJxJJDGtvUMVAC9 18/18 |
Guideline sources The following summarized guidelines for the evaluation and management of diabetes mellitus type 2 are prepared by our editorial team based on guidelines from the American Diabetes Association (ADA 2023), the European Association of Urology (EAU 2023), the American College of Gastroenterology (ACG 2022; 2013), the Endocrine Society (ES 2022; 2020; 2018; 2016), the Canadian Cardiovascular Society (CCS 2022), the Kidney Disease: Improving Global Outcomes Foundation (KDIGO 2022), the Society for Cardiovascular Angiography and Interventions (SCAI/AHA/ACC 2022), the Italian Society of Endocrinology (SIE/SIAMS 2022), the American Heart Association (AHA/ASA 2021), the U.S. Preventive Services Task Force (USPSTF 2021), the European Academy of Andrology (EAA 2020), the Society for Vascular Medicine (SVM/SVS/APMA 2016), and the Community Preventive Services Task Force (CPSTF 2015). 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 Calculator Calculator https://web.pathway.md/diseases/recNW2vC0lM724J79 1/22 6/29/23, 10:11 PM Diabetes mellitus type 2 Pathway American Diabetes Association Finnish Diabetes Risk Score FIN Guidelines 1. Screening and diagnosis Indications for screening: As per ADA 2023 guidelines, screening asymptomatic adults for prediabetes and T2DM with an informal assessment of risk factors or validated risk calculator. B Show 9 more As per USPSTF 2021 guidelines, screen for prediabetes and T2DM in adults aged 35-70 years with overweight or obesity. Offer or refer patients with prediabetes to effective preventive interventions. B 2. Diagnostic investigations General principles: ensure a person-centered communication style using person-centered, culturally sensitive, and strength-based language and active listening, eliciting individual preferences and beliefs, and assessing literacy, numeracy, and potential barriers to care in order to optimize health outcomes and health-related quality of life. B Show 2 more Hemoglobin A1C: obtain HbA1c testing using a method certified by the National GlycoHgb Standardization Program and standardized to the Diabetes Control and Complications Trial assay, in order to avoid misdiagnosis or missed diagnosis. B Show 3 more Glucose tolerance test: ensure adequate carbohydrate intake (at least 150 g/day) for 3 days before oral glucose tolerance testing as a screen for diabetes. A Screening for obesity: use person-centered, nonjudgmental language fostering collaboration between patients and health care professionals, including people-first language (such as "person with obesity" rather than "obese person"). B Show 2 more Screening for hypertension: measure BP at every routine clinical visit. Confirm BP using multiple readings, when possible, in patients found to have elevated BP (systolic BP 120-129 mmHg and diastolic < 80 mmHg), including measurements on a separate day, to diagnose hypertension. Diagnose hypertension in case of a systolic BP 130mmHg or a diastolic BP 80 mmHg based on an average of 2 measurements obtained on 2 occasions. A Show 2 more Screening for cardiovascular diseases: Do not obtain routine screening for coronary artery disease in asymptomatic patients, as it does not improve outcomes as long as ASCVD risk factors are treated. D https://web.pathway.md/diseases/recNW2vC0lM724J79 2/22 6/29/23, 10:11 PM Diabetes mellitus type 2 Pathway Consider obtaining investigations for coronary artery disease in the presence of any of the following: atypical cardiac symptoms (such as unexplained dyspnea, chest discomfort) signs or symptoms of associated vascular disease (including carotid bruits, TIA, stroke, claudication, or peripheral arterial disease) ECG abnormalities (such as Q waves). C Screening for dyslipidemia: Consider obtaining a lipid profile at the time of diabetes diagnosis, at an initial medical evaluation, and every 5 years thereafter if under the age of 40 years or more frequently if indicated, in adult patients with diabetes not taking statins or other lipid-lowering therapy. C Obtain lipid profile at the initiation of statins or other lipid-lowering therapy, 4-12 weeks after initiation or a change in dose, and annually thereafter to monitor the response to therapy and inform medication taking. B Screening for nonalcoholic fatty liver disease: obtain screening/risk stratification for nonalcoholic fatty liver disease with clinically significant fibrosis (defined as moderate fibrosis to cirrhosis) using a calculated fibrosis-4 index (derived from age, ALT, AST, and platelets) in adult patients with T2DM or prediabetes, particularly with obesity or cardiometabolic risk factors/established CVD, even if they have normal liver enzymes. B Show 3 more Screening for diabetic nephropathy: Assess urinary albumin (such as spot urinary albumin-to-creatinine ratio) and eGFR at least annually in all patients with T2DM regardless of treatment. B Monitor urinary albumin (such as spot urinary albumin-to-creatinine ratio) and eGFR 1-4 times per year depending on the stage of the disease in patients with established diabetic kidney disease. B Screening for diabetic retinopathy: obtain an initial dilated and comprehensive eye examination by an ophthalmologist or optometrist at the time of diagnosis in patients with T2DM. B Show 4 more Screening for diabetic neuropathy: screen for diabetic peripheral neuropathy in all patients with T2DM starting at diagnosis and at least annually thereafter. B Show 2 more Screening for diabetic foot: As per ADA 2023 guidelines, obtain a comprehensive foot evaluation at least annually to identify risk factors for ulcers and amputations. B Show 4 more As per SVS 2016 guidelines, consider obtaining a probe-to-bone test to aid in the diagnosis of diabetic foot osteomyelitis in patients with diabetic foot infection with an open wound. C Show 5 more Screening for anxiety: Consider screening patients with diabetes for symptoms or diabetes-related worries. Consider discussing diabetes-related worries and referring to a qualified mental health https://web.pathway.md/diseases/recNW2vC0lM724J79 3/22 6/29/23, 10:11 PM Diabetes mellitus type 2 Pathway professional for further assessment and treatment if anxiety symptoms indicate interference with diabetes self-management behaviors or quality of life. C Refer patients with hypoglycemia unawareness, which can co-occur with fear of hypoglycemia, to a trained professional to receive evidence-based intervention to help re- establish awareness of symptoms of hypoglycemia and reduce fear of hypoglycemia. A Screening for depression: consider obtaining at least annual screening for depressive symptoms in all patients with diabetes, especially in patients with a self-reported history of depression. Use age-appropriate, validated depression screening measures, recognizing that further evaluation will be necessary for patients with a positive screen. C Show 2 more Screening for sleep disorders: consider screening for sleep health in patients with diabetes, including symptoms of sleep disorders, disruptions to sleep due to diabetes symptoms or management needs, and worries about sleep. Refer to sleep medicine and/or a qualified behavioral health professional as indicated. C Screening for cognitive impairment: Monitor cognitive capacity throughout the life span in all patients with diabetes, particularly in patients with documented cognitive disabilities, patients experiencing severe hypoglycemia, very young children, and older adults. B Consider referring for a formal assessment if cognitive capacity changes or appears to be suboptimal for patient decision-making and/or behavioral self-management. C Screening for serious mental illness: provide an increased level of support to patients with diabetes and serious mental illness through enhanced monitoring of and assistance with diabetes self-management behaviors. B Show 2 more Screening for disordered eating behavior: Consider screening for disordered or disrupted eating using validated screening measures when hyperglycemia and weight loss are unexplained based on self-reported behaviors related to medication dosing, meal plan, and physical activity. Review the medical treatment plan to identify potential treatment-related effects on hunger/caloric intake. C Consider reevaluating the treatment plan of patients with diabetes presenting with symptoms of disordered eating behavior, an eating disorder, or disrupted patterns of eating, in consultation with a qualified professional as available. C Screening for gastroparesis (evaluation): evaluate for medication-induced delay in gastric emptying, particularly from GLP-1 agonists and amylin analogs, before assigning an etiological diagnosis of gastroparesis. Discontinue medications delaying gastric emptying to establish the diagnosis with a gastric emptying test. A Show 2 more Screening for gastroparesis (management): consider ensuring optimal glucose control to reduce the future risk of aggravation of gastroparesis in patients with diabetic gastroparesis. C Screening for male hypogonadism: obtain screening for late-onset hypogonadism only in symptomatic male patients. A https://web.pathway.md/diseases/recNW2vC0lM724J79 4/22 6/29/23, 10:11 PM Diabetes mellitus type 2 Pathway 3. Medical management Glycemic targets: set a HbA1c goal of < 7% (53 mmol/mol) without significant hypoglycemia in many nonpregnant adult patients with diabetes. A Show 4 more Initial therapy: include healthy lifestyle behaviors, weight management, diabetes self- management education and support, avoidance of clinical inertia, and social determinants of health in the glucose-lowering management of T2DM. A Show 7 more Landmark trials: Oral GLP-1 (danuglipron 120 mg) In adults with T2DM inadequately controlled by diet and exercise, with or without metformin treatment, danuglipron was superior to placebo with respect to reduction in glycated Hgb at week 16. Aditi R Saxena et al. JAMA Netw Open. 2023 May 1. Insulin therapy (indications): consider introducing insulin therapy early if there is evidence of ongoing catabolism (weight loss), if symptoms of hyperglycemia are present, or when HbA1c levels (> 10%; 86 mmol/mol) or blood glucose levels ( 300 mg/dL; 16.7 mmol/L) are very high. C Show 3 more Insulin therapy (choice of agent): consider prescribing long-acting insulin analogs rather than human NPH insulin in adult and pediatric outpatients on basal insulin therapy at high risk for hypoglycemia. C Show 2 more Insulin therapy, delivery systems: As per ADA 2023 guidelines, prefer insulin pens for most patients with insulin-requiring diabetes on multiple daily injections. Consider prescribing insulin syringes for insulin delivery taking into account individual and caregiver preference, insulin type, dosing therapy, cost, and self-management capabilities. B Show 6 more As per ES 2016 guidelines: Consider prescribing continuous subcutaneous insulin infusion with good adherence to monitoring and dosing in patients with T2DM having poor glycemic control despite intensive insulin therapy, oral agents, other injectable therapy, and lifestyle modifications. C Obtain a structured assessment of the patient's mental and psychological status, prior adherence to diabetes self-care measures, willingness and interest in trying the device, and availability for the required follow-up visits before prescribing continuous subcutaneous insulin infusion. B Management of hypoglycemia: https://web.pathway.md/diseases/recNW2vC0lM724J79 5/22 6/29/23, 10:11 PM Diabetes mellitus type 2 Pathway As per ADA 2023 guidelines, review occurrence and risk for hypoglycemia at every encounter and investigate as indicated. Consider assessing for awareness of hypoglycemia using validated tools. B Show 5 more As per ES 2022 guidelines, administer glucagon (preparations not requiring reconstitution) in outpatients with severe hypoglycemia. B Management of obesity: take into account the medication's effect on weight when choosing glucose-lowering medications in patients with T2DM and overweight/obesity. B Show 3 more Management of hypertension (BP targets): individualize BP targets in patients with diabetes and hypertension through a shared decision-making process addressing cardiovascular risk, potential adverse effects of antihypertensive medications, and patient preferences. B Show 2 more Management of hypertension (lifestyle modifications): advise lifestyle interventions in patients with BP > 120/80 mmHg, consisting of weight loss when indicated, a DASH-style eating pattern including reducing sodium and increasing potassium intake, moderation of alcohol intake, and increased physical activity. A Management of hypertension (pharmacotherapy): initiate and titrated pharmacologic therapy to achieve BP goal of < 130/80 mmHg in patients with confirmed office-based BP 130/80 mmHg. A Show 6 more Management of cardiovascular disease, glucose-lowering medications: As per ADA 2023 guidelines, initiate SGLT-2 inhibitors or GLP-1 receptor agonists with demonstrated CVD benefits as part of the comprehensive cardiovascular risk reduction and/or glucose-lowering regimens in patients with T2DM and established ASCVD or established kidney disease. A Show 5 more As per CCS 2022 guidelines, initiate the following options in adult patients with T2DM and either established ASCVD or multiple risk factors for ASCVD: sodium-glucose cotransporter 2 inhibitors to reduce the risk of all-cause or cardiovascular mortality, major atherosclerotic cardiovascular events, hospitalization for HF or the composite of significant decline in eGFR, progression to end-stage kidney disease or kidney death glucagon-like peptide 1 receptor agonists to reduce the risk of all-cause or cardiovascular mortality, major atherosclerotic cardiovascular events, and nonfatal stroke. B Management of cardiovascular disease (antihypertensives): initiate ACEI or ARB therapy to reduce the risk of cardiovascular events in patients with known ASCVD, particularly coronary artery disease. A Show 2 more Management of cardiovascular disease (antiplatelets): consider initiating aspirin therapy (75-162 mg/ day) as a primary prevention strategy in patients with diabetes at increased cardiovascular risk, after a comprehensive discussion with the patient on the benefits versus the comparable increased risk of bleeding. B https://web.pathway.md/diseases/recNW2vC0lM724J79 6/22 6/29/23, 10:11 PM Diabetes mellitus type 2 Pathway Show 6 more Management of cardiovascular disease (revascularization): perform CABG surgery (with a left internal mammary artery to the left anterior descending artery) over PCI to reduce mortality and repeat revascularizations in patients with diabetes and multivessel coronary artery disease with the involvement of the left anterior descending artery being appropriate candidates for CABG. A Show 3 more Management of dyslipidemia (lifestyle modifications): Advise lifestyle modifications focusing on weight loss (if indicated), following a Mediterranean style or DASH eating pattern, reducing consumption of saturated fat and trans fat, increasing dietary omega-3 fatty acids, viscous fiber, and plant stanols/sterols intake, and increasing physical activity to improve the lipid profile and reduce the risk of developing ASCVD in patients with diabetes. A Intensify lifestyle therapy and optimize glycemic control in patients with elevated triglyceride levels ( 150 mg/dL) and/or low HDL cholesterol (< 40 mg/dL for males, < 50 mg/dL for females). B Management of dyslipidemia, statin therapy: As per ADA 2023 guidelines, initiate moderate-intensity statin therapy, in addition to lifestyle therapy, in 40-75 years old patients with diabetes without ASCVD. A Show 5 more As per ES 2020 guidelines, initiate statin therapy in addition to lifestyle modification to reduce cardiovascular risks in adult patients with T2DM and other cardiovascular risk factors. A Show 2 more Management of dyslipidemia, non-statin lipid-lowering therapy: As per ADA 2023 guidelines, consider adding ezetimibe or a PCSK9 inhibitor to maximum tolerated statin therapy in 40-75 years old patients with diabetes at higher cardiovascular risk, especially patients with multiple ASCVD risk factors and an LDL cholesterol 70 mg/dL. C Show 3 more Landmark trials: PROMINENT In patients with T2DM, mild-to-moderate hypertriglyceridemia, and high-density lipoprotein cholesterol levels 40 mg/dL, pemafibrate was not superior to placebo with respect to the composite of nonfatal myocardial infarction, ischemic stroke, coronary revascularization, or death from cardiovascular causes. Aruna Das Pradhan et al. N Engl J Med. 2022 Nov 24. As per ES 2020 guidelines, consider initiating fibrates in addition to statins to reduce retinopathy progression in adult patients with T2DM and diabetic retinopathy. C Management of dyslipidemia, hypertriglyceridemia: As per ADA 2023 guidelines, evaluate for secondary causes of hypertriglyceridemia and consider initiating medical therapy to reduce the risk of pancreatitis in patients with fasting https://web.pathway.md/diseases/recNW2vC0lM724J79 7/22 6/29/23, 10:11 PM Diabetes mellitus type 2 Pathway triglyceride levels 500 mg/dL. B Show 2 more As per ES 2020 guidelines, consider adding eicosapentaenoic acid ethyl ester to reduce cardiovascular risks in adult patients with T2DM on a statin at low-density lipoprotein goal with residual triglycerides > 150 mg/dL (1.7 mmol/L) and with two additional traditional risk factors or risk-enhancing factors. C Management of dyslipidemia (lipid profile monitoring): Consider obtaining a lipid profile at the time of diabetes diagnosis, at an initial medical evaluation, and every 5 years thereafter if under the age of 40 years or more frequently if indicated, in adult patients with diabetes not taking statins or other lipid-lowering therapy. C Obtain lipid profile at the initiation of statins or other lipid-lowering therapy, 4-12 weeks after initiation or a change in dose, and annually thereafter to monitor the response to therapy and inform medication taking. B Management of nonalcoholic fatty liver disease (lifestyle changes): offer lifestyle changes promoting weight loss, ideally within a structured nutrition plan and physical activity program, for cardiometabolic benefits B and histological improvement in adult patients with T2DM or prediabetes, particularly with overweight or obesity with nonalcoholic fatty liver disease. B Management of diabetic nephropathy, general principles: As per ADA 2023 guidelines, optimize glucose control to reduce the risk or slow the progression of CKD. A Show 5 more As per KDIGO 2022 guidelines, treat patients with diabetes mellitus and CKD with a comprehensive strategy to reduce risks of kidney disease progression and CVD. B Show 3 more Management of diabetic nephropathy (smoking cessation): Advise patients with diabetes mellitus and CKD to quit using tobacco products. B Counsel patients with diabetes mellitus and CKD to reduce secondhand smoke exposure. B Management of diabetic nephropathy (metformin): initiate metformin in patients with T2DM, CKD and an eGFR 30 mL/min/1.73 m . B Show 4 more Management of diabetic nephropathy (GLP-1 receptor agonists): initiate long-acting GLP-1 receptor agonists in patients with T2DM and CKD not achieved individualized glycemic targets despite using metformin and SGLT-2 inhibitor treatment or unable to use these medications. B Show 5 more Management of diabetic nephropathy, SGLT2 inhibitors: As per ADA 2023 guidelines, initiate SGLT-2 inhibitors in patients with an eGFR 25 mL/min/1.73 m and urinary albumin 300 mg/g creatinine to reduce CKD progression and cardiovascular events in patients with T2DM and diabetic kidney disease. A Show 2 more As per KDIGO 2022 guidelines, initiate SGLT-2 inhibitors in patients with T2DM, CKD and an eGFR 20 mL/min/1.73 m . B https://web.pathway.md/diseases/recNW2vC0lM724J79 8/22 6/29/23, 10:11 PM Diabetes mellitus type 2 Pathway Show 7 more Management of diabetic nephropathy, RAAS blockade: As per ADA 2023 guidelines, do not use ACEIs or ARBs for the primary prevention of CKD in patients with diabetes with normal BP, normal urinary albumin-to-creatinine ratio (< 30 mg/g creatinine), and normal eGFR. D Show 5 more As per KDIGO 2022 guidelines, initiate an ACEI or an ARB in patients with diabetes, hypertension and albuminuria and titrate the medications to the highest approved dose that is tolerated. B Show 13 more Management of diabetic retinopathy: optimize glycemic control to reduce the risk or slow the progression of diabetic retinopathy. A Show 7 more Management of diabetic neuropathy: optimize glucose control to slow the progression of neuropathy in patients with T2DM. B Show 2 more Management of diabetic foot: ensure a multidisciplinary approach for patients with foot ulcers and high-risk feet (such as patients on dialysis, patients with Charcot foot, history of ulcers or amputation, or peripheral arterial disease). B Show 4 more 4. Inpatient care Management of hospitalized patients, general principles: As per ADA 2023 guidelines, consult with a specialized diabetes or glucose management team, when possible, when caring for hospitalized patients with diabetes. B Landmark trials: Leuven Surgical Trial In adults admitted to surgical ICU who were receiving mechanical ventilation, intensive insulin therapy was superior to conventional treatment with respect to death. van den Berghe G et al. N Engl J Med. 2001 Nov 8. As per ES 2022 guidelines, consider providing inpatient diabetes education as part of a comprehensive diabetes discharge-planning process in adult patients with diabetes hospitalized for a non-critical illness. C Management of hospitalized patients (HbA1C testing): obtain HbA1c testing in all patients with diabetes or hyperglycemia (blood glucose > 140 mg/dL; 7.8 mmol/L) admitted to the hospital, if not performed in the prior 3 months. B Management of hospitalized patients, glucose monitoring: As per ADA 2023 guidelines, support patients to continue using diabetes devices, such as insulin pumps and continuous glucose monitoring systems, in an inpatient setting or during https://web.pathway.md/diseases/recNW2vC0lM724J79 9/22 6/29/23, 10:11 PM Diabetes mellitus type 2 Pathway outpatient procedures, once competency in safely using these devices is established and proper supervision is available. B As per ES 2022 guidelines, consider obtaining real-time continuous glucose monitoring with confirmatory bedside point-of-care blood glucose monitoring for adjustments in insulin dosing (in hospital settings where resources and training are available) in adult patients with insulin- treated diabetes at high risk of hypoglycemia hospitalized for a non-critical illness. C As per ES 2022 guidelines, consider initiating continuous glucose monitoring in the inpatient setting for selected patients at high risk for hypoglycemia. C Show 2 more Management of hospitalized patients, insulin therapy: As per ADA 2023 guidelines, use validated written or computerized protocols for insulin administration allowing for predefined adjustments in the insulin dosage based on glycemic fluctuations. B Show 4 more As per ES 2022 guidelines, consider administering scheduled insulin therapy for glycemic control in most adult patients with hyperglycemia (with or without known T2DM) hospitalized for a non-critical illness. C Show 9 more As per ES 2016 guidelines, continue continuous subcutaneous insulin infusion in patients admitted to the hospital if the institution has clear protocols for evaluating patients as suitable candidates and appropriate monitoring and safety procedures. B Management of hospitalized patients, treatment targets: As per ADA 2023 guidelines: Set a target glucose range of 140-180 mg/dL (7.8-10.0 mmol/L) for the majority of critically ill and non-critically ill patients once insulin therapy is started. A Consider setting more stringent goals, such as 110-140 mg/dL (6.1-7.8 mmol/L), in selected patients if those can be achieved without significant hypoglycemia. C As per ES 2022 guidelines: Consider targeting preoperative HbA1c levels of < 8% (63.9 mmol/mol) and blood glucose levels of 100-180 mg/dL (5.6-10 mmol/L) in adult patients with diabetes undergoing elective surgical procedures. C Consider targeting preoperative blood glucose levels of 100-180 mg/dL (5.6-10 mmol/L) in adult patients with diabetes undergoing elective surgical procedures if a HbA1c < 8% (63.9 mmol/mol) target is not feasible. C Management of hospitalized patients (prevention of hypoglycemia): Review treatment regimens and change as necessary to prevent further hypoglycemia when a blood glucose value of < 70 mg/dL (3.9 mmol/L) is documented. B Adopt a hypoglycemia management protocol and implement it in each hospital or hospital system. Establish a plan for the prevention and treatment of hypoglycemia for each patient. Document episodes of hypoglycemia in the hospital in the medical record and track for quality improvement/quality assessment. B Management of hospitalized patients (oral carbohydrate fluids): avoid administering preoperative carbohydrate-containing oral fluids in adult patients with diabetes undergoing https://web.pathway.md/diseases/recNW2vC0lM724J79 10/22 6/29/23, 10:11 PM Diabetes mellitus type 2 Pathway surgical procedures. D 5. Nonpharmacologic interventions Smoking cessation: advise all patients to abstain from using cigarettes and other tobacco products or e-cigarettes. A Show 2 more Weight loss: offer nutrition, physical activity, and behavioral therapy to achieve and maintain 5% weight loss in most people with T2DM and overweight or obesity. Offer additional weight loss for further improvements in the management of diabetes and cardiovascular risk. B Include high frequency of counseling with 16 sessions in 6 months in such interventions and focus on nutrition changes, physical activity, and behavioral strategies to achieve a 500-750 kcal/day energy deficit. A Show 6 more Physical activity: advise most adults with T2DM to engage in 150 minutes of moderate- to vigorous-intensity aerobic activity per week, spread over at least 3 days/week, with no more than 2 consecutive days without activity. Consider advising shorter durations (minimum 75 min/week) of vigorous-intensity or interval training in younger and more physically fit patients. B Show 4 more Dietary modifications (general principles): provide an individualized medical nutrition therapy program as needed to achieve treatment goals, provided by a registered dietitian nutritionist, preferably one who has comprehensive knowledge and experience in diabetes care, in all patients with T2DM. A Show 2 more Dietary modifications (eating patterns): recognize that there is no ideal macronutrient pattern for patients with diabetes. Individualize meal plans while keeping nutrient quality, total calorie, and metabolic goals in mind. B Show 2 more Dietary modifications (carbohydrates): advise carbohydrate intake emphasizing nutrient- dense carbohydrate sources high in fiber (at least 14 g fiber per 1,000 kcal) and minimally processed. Advise eating plans emphasizing nonstarchy vegetables, fruits, legumes, and whole grains, as well as dairy products, with minimal added sugars. B Show 3 more Dietary modifications (protein): avoid using carbohydrate sources high in protein for the treatment or prevention of hypoglycemia as ingested protein appears to increase insulin response without increasing plasma glucose concentrations in patients with T2DM. D Dietary modifications (fat): Consider advising an eating plan emphasizing elements of a Mediterranean eating pattern rich in monounsaturated and polyunsaturated fats to improve glucose metabolism and lower CVD risk. C Advise eating foods rich in long-chain omega-3 fatty acids, such as fatty fish (eicosapentaenoic acid and docosahexaenoic acid) and nuts and seeds (alpha-linolenic https://web.pathway.md/diseases/recNW2vC0lM724J79 11/22 6/29/23, 10:11 PM Diabetes mellitus type 2 Pathway acid), to prevent or treat CVD. B Dietary modifications (nonnutritive sweeteners): Recognize that the use of nonnutritive sweeteners as a replacement for sugar-sweetened products may reduce overall calorie and carbohydrate intake as long as there is not a compensatory increase in energy intake from other sources. B Consider advising low- and no-calorie sweetened beverages as alternatives to water. C Dietary modifications (sodium): limit sodium consumption to < 2,300 mg/day. B Dietary modifications (micronutrients and supplements): insufficient evidence to support the use of dietary supplementation with vitamins, minerals (such as chromium and vitamin D), herbs, or spices (such as cinnamon or aloe vera) to improve outcomes in patients with diabetes not having underlying deficiencies, and for glycemic control. I Dietary modifications (alcohol): Advise adult patients with diabetes drinking alcohol to do so in moderation ( 1 drink/day for adult females and 2 drinks/day for adult males). B Educate patients with diabetes about the signs, symptoms, and self-management of delayed hypoglycemia after drinking alcohol, especially when using insulin or insulin secretagogues. Emphasize the importance of glucose monitoring after drinking alcoholic beverages to reduce hypoglycemia risk. B Psychosocial care: provide psychosocial care, integrated with routine medical care and delivered by trained healthcare professionals using a collaborative, person-centered, culturally informed approach, in all patients with diabetes, with the goal of optimizing health-related quality of life and health outcomes. A Refer to qualified mental health professionals for additional targeted mental health care, when indicated and available. B Show 4 more 6. Surgical interventions Bariatric surgery: offer metabolic surgery for the treatment of T2DM in screened surgical candidates with BMI 40 kg/m (BMI 37.5 kg/m in Asian Americans) and in adult patients with BMI 35.0-39.9 kg/m (32.5-37.4 kg/m in Asian Americans) not achieved durable weight loss and improvement in comorbidities (including hyperglycemia) with nonsurgical methods. A Show 6 more 7. Specific circumstances Patients with prediabetes: monitor for the development of T2DM in patients with prediabetes at least annually, modified based on individual risk/benefit assessment. B Show 10 more Elderly patients (screening for comorbidities): consider assessing medical, psychological, functional (self-management abilities), and social domains in older adults to provide a framework to determine targets and therapeutic approaches for diabetes management. C Show 2 more https://web.pathway.md/diseases/recNW2vC0lM724J79 12/22 6/29/23, 10:11 PM Diabetes mellitus type 2 Pathway Elderly patients (screening for hypoglycemia): ascertain and address episodes of hypoglycemia in older adults with diabetes at routine visits because they have a greater risk of hypoglycemia than younger adults. B Show 2 more Elderly patients (treatment goals): set lower glycemic goals (such as HbA1c < 7.0-7.5%; 53- 58 mmol/mol) in older patients otherwise healthy with few coexisting chronic illnesses and intact cognitive function and functional status. B Show 5 more Elderly patients (lifestyle modifications): ensure optimal nutrition and protein intake in older patients. B Show 2 more Elderly patients (pharmacotherapy): prefer medication classes with a low risk of hypoglycemia in older patients with T2DM at increased risk of hypoglycemia. B Show 4 more Elderly patients (residing in nursing facilities): obtain careful assessment in patients with diabetes residing in long-term care facilities to establish individualized glycemic goals and to make appropriate choices of glucose-lowering agents based on their clinical and functional status. B Show 2 more Elderly patients (end-of-life care): Initiate conversations regarding the goals and intensity of care when palliative care is required in older patients with diabetes. Avoid strict glucose and BP control. B Consider simplifying regimens. Consider relaxing the intensity of lipid management or withdrawing lipid-lowering therapy. A Set overall comfort, prevention of distressing symptoms, and preservation of quality of life and dignity as primary goals for diabetes management at the end of life. B Pediatric patients (screening and diagnosis): Consider obtaining risk-based screening for prediabetes and/or T2DM after the onset of puberty or 10 years of age, whichever occurs earlier, in young patients with overweight with BMI 85th percentile or obesity with BMI 95th percentile and having 1 additional risk factors for diabetes: Situation Guidance Diabetes or gestational diabetes during the child's gestation Maternal history T2DM in first- or second-degree relative Family history Native American, African American, Latino, Asian American, Pacific Islander B Race/ethnicity Acanthosis nigricans, hypertension, dyslipidemia, PCOS, or small-for- gestational-age birth weight. C Signs of or conditions associated with insulin resistance Show 3 more https://web.pathway.md/diseases/recNW2vC0lM724J79 13/22 6/29/23, 10:11 PM Diabetes mellitus type 2 Pathway Pediatric patients (screening for hypertension): measure BP in young patients with T2DM at every visit. Consider obtaining ambulatory BP monitoring in young patients with high BP (BP 90th percentile for age, gender, and height or, in adolescents aged 13 years, 120/80 mmHg) on 3 separate measurements. B Pediatric patients (screening for cardiovascular diseases): do not obtain routine screening for heart disease with electrocardiography, echocardiography, or stress testing in asymptomatic young patients with T2DM. D Pediatric patients (screening for dyslipidemia): obtain lipid profile screening initially after optimizing glycemia and annually thereafter. B Pediatric patients (screening for nonalcoholic fatty liver disease): Screen for nonalcoholic fatty liver disease by measuring AST and ALT at diagnosis and annually thereafter in pediatric patients with T1DM. B Consider referring to gastroenterology for persistently elevated or worsening transaminases. C Pediatric patients (screening for nephropathy): advise protein intake at the recommended daily allowance of 0.8 g/kg/day. B Show 5 more Pediatric patients (screening for retinopathy): obtain screening for retinopathy by dilated fundoscopy at or soon after diagnosis and annually thereafter. B Show 3 more Pediatric patients (screening for neuropathy): Screen for diabetic neuropathy by foot examination (including foot inspection, assessment of foot pulses, pinprick and 10-g monofilament sensation tests, testing of vibration sensation using a 128-Hz tuning fork, and ankle reflex tests) at diagnosis and annually thereafter in young patients with T2DM. B Focus on achieving glycemic targets for the prevention of diabetic neuropathy. B Pediatric patients (screening for obstructive sleep apnea): screen for symptoms of sleep apnea at each visit, and refer to a pediatric sleep specialist for evaluation and a polysomnogram, if indicated. Treat obstructive sleep apnea when documented. B Pediatric patients (screening for polycystic ovary syndrome): screen for PCOS in female adolescent patients with T2DM, including laboratory studies when indicated. B Show 2 more Pediatric patients (screening for psychosocial factors): screen for food security, housing stability/homelessness, health literacy, financial barriers, and social/community support and apply that information to treatment decisions. B Show 4 more Pediatric patients (lifestyle modifications): offer intensive lifestyle interventions focusing on weight loss, dyslipidemia, hypertension, and dysglycemia to prevent overt macrovascular disease in early adulthood. B Show 5 more Pediatric patients (glycemic targets): individualize blood glucose monitoring taking into consideration the pharmacologic treatment of the patient. B https://web.pathway.md/diseases/recNW2vC0lM724J79 14/22 6/29/23, 10:11 PM Diabetes mellitus type 2 Pathway Show 5 more Pediatric patients (pharmacotherapy): initiate pharmacotherapy, in addition to behavioral counseling for healthful nutrition and physical activity changes, at diagnosis of T2DM. A Show 8 more Pediatric patients (management of obesity): Consider offering metabolic surgery for the treatment of adolescent patients with T2DM with severe obesity (BMI > 35 kg/m ) and having elevated HbA1c and/or serious comorbidities despite lifestyle and pharmacologic interventions. B Perform metabolic surgery only by an experienced surgeon working as part of a well- organized and engaged multidisciplinary team, including a surgeon, endocrinologist, dietitian nutritionist, behavioral health specialist, and nurse. A Pediatric patients (management of hypertension): offer lifestyle modifications focused on healthy nutrition, physical activity, sleep, and, if appropriate, weight management for the treatment of elevated BP (defined as 90th to < 95th percentile for age, gender, and height or, in adolescents aged 13 years, 120-129/< 80 mmHg) in young patients with T2DM. B Show 2 more Pediatric patients (management of dyslipidemia): Set the following optimal goals: Situation Guidance < 100 mg/dL (2.6 mmol/L) LDL cholesterol > 35 mg/dL (0.91 mmol/L) HDL cholesterol < 150 mg/dL (1.7 mmol/L). B Triglycerides Show 3 more Pediatric patients (transition to adult care): begin to prepare young patients for transition to adult healthcare in early adolescence and, at the latest, at least 1 year before the transition. B Show 2 more Pregnant patients (screening): obtain screening for undiagnosed diabetes in individuals with risk factors planning pregnancy. B Consider testing all individuals of childbearing potential for undiagnosed diabetes. B Show 4 more Pregnant patients (preconception counseling and care): incorporate preconception counseling into routine diabetes care starting at puberty and continuing in all patients with diabetes and reproductive potential. A Show 6 more Pregnant patients (nutrition): encourage a balanced intake of macronutrients including nutrient-dense fruits, vegetables, legumes, whole grains, and healthy fats with omega-3 fatty acids (including nuts, seeds, and fish) in the eating pattern. B Pregnant patients (glycemic targets): obtain fasting and postprandial blood glucose monitoring in both gestational diabetes mellitus and preexisting diabetes in pregnancy, to achieve optimal glucose levels. Set the following glucose targets: fasting plasma glucose < 95 https://web.pathway.md/diseases/recNW2vC0lM724J79 15/22 6/29/23, 10:11 PM Diabetes mellitus type 2 Pathway mg/dL (5.3 mmol/L) and either 1-hour postprandial glucose < 140 mg/dL (7.8 mmol/L) or 2- hour postprandial glucose < 120 mg/dL (6.7 mmol/L). Obtain blood glucose preprandially in some patients with preexisting diabetes. B Show 4 more |
Show 5 more Elderly patients (lifestyle modifications): ensure optimal nutrition and protein intake in older patients. B Show 2 more Elderly patients (pharmacotherapy): prefer medication classes with a low risk of hypoglycemia in older patients with T2DM at increased risk of hypoglycemia. B Show 4 more Elderly patients (residing in nursing facilities): obtain careful assessment in patients with diabetes residing in long-term care facilities to establish individualized glycemic goals and to make appropriate choices of glucose-lowering agents based on their clinical and functional status. B Show 2 more Elderly patients (end-of-life care): Initiate conversations regarding the goals and intensity of care when palliative care is required in older patients with diabetes. Avoid strict glucose and BP control. B Consider simplifying regimens. Consider relaxing the intensity of lipid management or withdrawing lipid-lowering therapy. A Set overall comfort, prevention of distressing symptoms, and preservation of quality of life and dignity as primary goals for diabetes management at the end of life. B Pediatric patients (screening and diagnosis): Consider obtaining risk-based screening for prediabetes and/or T2DM after the onset of puberty or 10 years of age, whichever occurs earlier, in young patients with overweight with BMI 85th percentile or obesity with BMI 95th percentile and having 1 additional risk factors for diabetes: Situation Guidance Diabetes or gestational diabetes during the child's gestation Maternal history T2DM in first- or second-degree relative Family history Native American, African American, Latino, Asian American, Pacific Islander B Race/ethnicity Acanthosis nigricans, hypertension, dyslipidemia, PCOS, or small-for- gestational-age birth weight. C Signs of or conditions associated with insulin resistance Show 3 more https://web.pathway.md/diseases/recNW2vC0lM724J79 13/22 6/29/23, 10:11 PM Diabetes mellitus type 2 Pathway Pediatric patients (screening for hypertension): measure BP in young patients with T2DM at every visit. Consider obtaining ambulatory BP monitoring in young patients with high BP (BP 90th percentile for age, gender, and height or, in adolescents aged 13 years, 120/80 mmHg) on 3 separate measurements. B Pediatric patients (screening for cardiovascular diseases): do not obtain routine screening for heart disease with electrocardiography, echocardiography, or stress testing in asymptomatic young patients with T2DM. D Pediatric patients (screening for dyslipidemia): obtain lipid profile screening initially after optimizing glycemia and annually thereafter. B Pediatric patients (screening for nonalcoholic fatty liver disease): Screen for nonalcoholic fatty liver disease by measuring AST and ALT at diagnosis and annually thereafter in pediatric patients with T1DM. B Consider referring to gastroenterology for persistently elevated or worsening transaminases. C Pediatric patients (screening for nephropathy): advise protein intake at the recommended daily allowance of 0.8 g/kg/day. B Show 5 more Pediatric patients (screening for retinopathy): obtain screening for retinopathy by dilated fundoscopy at or soon after diagnosis and annually thereafter. B Show 3 more Pediatric patients (screening for neuropathy): Screen for diabetic neuropathy by foot examination (including foot inspection, assessment of foot pulses, pinprick and 10-g monofilament sensation tests, testing of vibration sensation using a 128-Hz tuning fork, and ankle reflex tests) at diagnosis and annually thereafter in young patients with T2DM. B Focus on achieving glycemic targets for the prevention of diabetic neuropathy. B Pediatric patients (screening for obstructive sleep apnea): screen for symptoms of sleep apnea at each visit, and refer to a pediatric sleep specialist for evaluation and a polysomnogram, if indicated. Treat obstructive sleep apnea when documented. B Pediatric patients (screening for polycystic ovary syndrome): screen for PCOS in female adolescent patients with T2DM, including laboratory studies when indicated. B Show 2 more Pediatric patients (screening for psychosocial factors): screen for food security, housing stability/homelessness, health literacy, financial barriers, and social/community support and apply that information to treatment decisions. B Show 4 more Pediatric patients (lifestyle modifications): offer intensive lifestyle interventions focusing on weight loss, dyslipidemia, hypertension, and dysglycemia to prevent overt macrovascular disease in early adulthood. B Show 5 more Pediatric patients (glycemic targets): individualize blood glucose monitoring taking into consideration the pharmacologic treatment of the patient. B https://web.pathway.md/diseases/recNW2vC0lM724J79 14/22 6/29/23, 10:11 PM Diabetes mellitus type 2 Pathway Show 5 more Pediatric patients (pharmacotherapy): initiate pharmacotherapy, in addition to behavioral counseling for healthful nutrition and physical activity changes, at diagnosis of T2DM. A Show 8 more Pediatric patients (management of obesity): Consider offering metabolic surgery for the treatment of adolescent patients with T2DM with severe obesity (BMI > 35 kg/m ) and having elevated HbA1c and/or serious comorbidities despite lifestyle and pharmacologic interventions. B Perform metabolic surgery only by an experienced surgeon working as part of a well- organized and engaged multidisciplinary team, including a surgeon, endocrinologist, dietitian nutritionist, behavioral health specialist, and nurse. A Pediatric patients (management of hypertension): offer lifestyle modifications focused on healthy nutrition, physical activity, sleep, and, if appropriate, weight management for the treatment of elevated BP (defined as 90th to < 95th percentile for age, gender, and height or, in adolescents aged 13 years, 120-129/< 80 mmHg) in young patients with T2DM. B Show 2 more Pediatric patients (management of dyslipidemia): Set the following optimal goals: Situation Guidance < 100 mg/dL (2.6 mmol/L) LDL cholesterol > 35 mg/dL (0.91 mmol/L) HDL cholesterol < 150 mg/dL (1.7 mmol/L). B Triglycerides Show 3 more Pediatric patients (transition to adult care): begin to prepare young patients for transition to adult healthcare in early adolescence and, at the latest, at least 1 year before the transition. B Show 2 more Pregnant patients (screening): obtain screening for undiagnosed diabetes in individuals with risk factors planning pregnancy. B Consider testing all individuals of childbearing potential for undiagnosed diabetes. B Show 4 more Pregnant patients (preconception counseling and care): incorporate preconception counseling into routine diabetes care starting at puberty and continuing in all patients with diabetes and reproductive potential. A Show 6 more Pregnant patients (nutrition): encourage a balanced intake of macronutrients including nutrient-dense fruits, vegetables, legumes, whole grains, and healthy fats with omega-3 fatty acids (including nuts, seeds, and fish) in the eating pattern. B Pregnant patients (glycemic targets): obtain fasting and postprandial blood glucose monitoring in both gestational diabetes mellitus and preexisting diabetes in pregnancy, to achieve optimal glucose levels. Set the following glucose targets: fasting plasma glucose < 95 https://web.pathway.md/diseases/recNW2vC0lM724J79 15/22 6/29/23, 10:11 PM Diabetes mellitus type 2 Pathway mg/dL (5.3 mmol/L) and either 1-hour postprandial glucose < 140 mg/dL (7.8 mmol/L) or 2- hour postprandial glucose < 120 mg/dL (6.7 mmol/L). Obtain blood glucose preprandially in some patients with preexisting diabetes. B Show 4 more Pregnant patients (management of gestational diabetes): offer lifestyle behavior changes for the management of gestational diabetes mellitus. Add insulin if needed to achieve glycemic targets. A Show 5 more Pregnant patients (management of preexisting diabetes): prefer insulin for the management of T2DM in pregnancy. B Pregnant patients (prevention of preeclampsia): prescribe low-dose aspirin 100-150 mg/day (162 mg/day may also be acceptable) starting at 12-16 weeks of gestation to lower the risk of preeclampsia in patients with T2DM. B Pregnant patients (BP targets): consider targeting a BP of 110-135/85 mmHg in pregnant patients with diabetes and chronic hypertension in the interest of reducing the risk for accelerated maternal hypertension. B Titrate therapy from a BP threshold of 140/90 mmHg for better pregnancy outcomes with no increase in the risk of small-for-gestational-age birth weight. B Lessen therapy for BP < 90/60 mmHg. C Pregnant patients (postpartum care): evaluate and adjust insulin requirements postpartum as insulin resistance decreases dramatically immediately postpartum and the requirements are often roughly half the prepregnancy requirements for the initial few days postpartum. B Show 7 more Patients with male hypogonadism: As per SIAMS 2022 guidelines, consider initiating testosterone replacement therapy to improve fasting and post-load glycemic control and to reduce the risk of developing T2DM in male patients with hypogonadism and metabolic syndrome or pre-diabetic conditions. C Show 3 more As per EAA 2020 guidelines, do not initiate testosterone replacement therapy to improve glycometabolic control in male patients with T2DM and/or metabolic syndrome. D As per ES 2018 guidelines, do not initiate testosterone replacement therapy to improve glycemic control in patients with T2DM mellitus and low testosterone concentrations. D Patients with stroke: As per ADA 2023 guidelines, consider offering pioglitazone to lower the risk of stroke or myocardial infarction in patients with a history of stroke and evidence of insulin resistance and prediabetes, balancing the benefits with the increased risk of weight gain, edema, and fracture. B Consider offering lower doses to mitigate the risk of adverse effects. C As per AHA 2021 guidelines, consider offering lifestyle optimization (healthy diet, regular physical activity, and smoking cessation) for the prevention of the progression of prediabetes to diabetes in patients with ischemic stroke or TIA and prediabetes. C Show 6 more Patients with COVID-19 infection: support patients with diabetes to achieve individualized targeted glycemic control to reduce the risk of macrovascular and microvascular risk as well as reduce the risk of COVID-19 and its complications. B https://web.pathway.md/diseases/recNW2vC0lM724J79 16/22 6/29/23, 10:11 PM Diabetes mellitus type 2 Pathway Show 8 more 8. Patient education Self-management counseling: offer all patients with diabetes participation in self- management education and support to facilitate the knowledge, decision-making, and skills mastery for diabetes self-care. A Show 9 more 9. Preventative measures Primary prevention, lifestyle modifications: As per ADA 2023 guidelines, refer adult patients with overweight or obesity at high risk of T2DM, as typified by the Diabetes Prevention Program, to an intensive lifestyle behavior change program to achieve and maintain a weight reduction of at least 7% of initial body weight through healthy reduced-calorie diet and 150 minute/week of moderate-intensity physical activity. A Show 4 more As per CPSTF 2015 guidelines, consider offering combined diet and physical activity promotion programs by healthcare systems, communities, and other implementers to provide counseling and support in individuals identified as being at increased risk for T2DM. E Primary prevention (metformin therapy): Consider offering metformin therapy for the prevention of T2DM in adults at high risk of T2DM, as typified by the Diabetes Prevention Program, especially patients aged 25-59 years with BMI 35 kg/m , higher fasting plasma glucose ( 110 mg/dL), and higher HbA1c ( 6.0%), and in patients with a history of gestational diabetes mellitus. B Consider obtaining periodic measurements of vitamin B12 levels in metformin-treated patients, especially in patients with anemia or peripheral neuropathy. C Routine immunizations: provide routine immunizations in pediatric and adult patients with diabetes as indicated by age. A 10. Follow-up and surveillance Treatment monitoring: include most components of the initial comprehensive medical evaluation in the follow-up visit. A Show 3 more Continous glucose monitoring: As per ADA 2023 guidelines, obtain real-time continuous glucose monitoring A or intermittently scanned continuous glucose monitoring B in adult patients with diabetes on multiple daily injections or continuous subcutaneous insulin infusion capable of using the devices safely, either by themselves or with a caregiver. Decide on the choice of the device based on the patient's circumstances, preferences, and needs. A Show 12 more https://web.pathway.md/diseases/recNW2vC0lM724J79 17/22 6/29/23, 10:11 PM Diabetes mellitus type 2 Pathway As per ES 2022 guidelines, consider obtaining real-time continuous glucose monitoring in outpatients with T2DM taking insulin and/or sulfonylureas and being at risk for hypoglycemia. C As per ES 2016 guidelines: Consider obtaining short-term, intermittent continuous glucose monitoring in adult patients with T2DM (not on prandial insulin) with HbA1c levels 7% and willing and able to use the device. C Provide education, training, and ongoing support to help achieve and maintain individualized glycemic goals in adult patients with T2DM using continuous subcutaneous insulin infusion and continuous glucose monitoring. E Monitoring of glycemic status: assess glycemic status (HbA1c or other glycemic measurements, such as time in range or glucose management indicator) at least two times a year in patients meeting treatment goals (and having stable glycemic control). B Show 3 more 11. Quality improvement Health promotion: ensure that treatment decisions are timely, rely on evidence-based guidelines, include social community support, and are made collaboratively with patients based on individual preferences, prognoses, comorbidities, and informed financial considerations. B Show 5 more Use of diabetes technology: individualize the type and selection of devices based on the patient's specific needs, preferences, and skill level. Include the caregiver's skills and preferences in the decision-making process in the setting of a patient with diabetes partially or wholly managed by someone else (such as a young child or a person with cognitive impairment or dexterity, psychosocial, and/or physical limitations). B Show 5 more Clinical findings Patient demographics Symptoms African-american race Asthenia Age Blurry vision Asian ethnicity Chronic lower abdominal pain Native American Fatigue Limb numbness Past medical history Polydipsia Polyuria Diabetic neuropathy Weight loss Hypercholesterolemia Obesity Past obstetric history PCOS Prediabetes Gestational diabetes Repeated infections Recurrent abortion https://web.pathway.md/diseases/recNW2vC0lM724J79 18/22 6/29/23, 10:11 PM Diabetes mellitus type 2 Pathway Family history Social history Diabetes mellitus Physical inactivity Genetic predispositions Lab findings Neurological exam Lactic acidosis Anisocoria urine glucose serum pH Studies 2023 DECLARE-TIMI 58 (post-hoc analysis) In adults 40 years with T2DM and either risk factors for or established ASCVD, dapagliflozin was superior to placebo with respect to first non-elective hospitalization for any cause. Meir Schechter et al. Lancet Diabetes Endocrinol. 2023 Apr. 2023 Oral GLP-1 (danuglipron 40 mg) In adults with T2DM inadequately controlled by diet and exercise, with or without metformin treatment, danuglipron was superior to placebo with respect to reduction in glycated Hgb at week 16. Aditi R Saxena et al. JAMA Netw Open. 2023 May 1. Show 41 more References 1. Steven E. Kahn, Cheryl A.M. Anderson, John B. Buse et al. Standards of Care in Diabetes 2023. Diabetes Care. 2023 Jan;46 Supplement_1 S1 S291. Open 2. Michael Camilleri, Braden Kuo, Linda Nguyen et al. ACG Clinical Guideline: Gastroparesis. Am J Gastroenterol. 2022 Aug 1;117 8 1197 1220. Open 3. Anthony L McCall, David C Lieb, Roma Gianchandani et al. Management of Individuals With Diabetes at High Risk for Hypoglycemia: An Endocrine Society Clinical Practice Guideline. J Clin Endocrinol Metab. 2022 Dec 7;dgac596. Open 4. Connie B Newman, Michael J Blaha, Jeffrey B Boord et al. Lipid Management in Patients with Endocrine Disorders: An Endocrine Society Clinical Practice Guideline. J Clin Endocrinol Metab. 2020 Dec 1;105 12):dgaa674. Open 5. Anne L Peters, Andrew J Ahmann, Tadej Battelino et al. Diabetes Technology-Continuous Subcutaneous Insulin Infusion Therapy and Continuous Glucose Monitoring in Adults: An Endocrine Society Clinical Practice Guideline. J Clin Endocrinol Metab. 2016 Nov;101 11 3922 3937. Open 6. G B John Mancini, Eileen O'Meara, Shelley Zieroth et al. 2022 Canadian Cardiovascular Society Guideline for Use of GLP 1 Receptor Agonists and SGLT2 Inhibitors for Cardiorenal Risk Reduction in Adults. Can J Cardiol. 2022 Aug;38 8 1153 1167. Open https://web.pathway.md/diseases/recNW2vC0lM724J79 19/22 6/29/23, 10:11 PM Diabetes mellitus type 2 Pathway 7. Kidney Disease: Improving Global Outcomes KDIGO Diabetes Work Group. KDIGO 2022 Clinical Practice Guideline for Diabetes Management in Chronic Kidney Disease. Kidney Int. 2022 Nov;102 5S S1 S127. Open 8. Jennifer S Lawton, Jacqueline E Tamis-Holland, Sripal Bangalore et al. 2021 ACC/AHA/SCAI Guideline for Coronary Artery Revascularization: A Report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines. Circulation. 2022 Jan 18;145 3):e18-e114. Open 9. A M Isidori, A Aversa, A Calogero et al. Adult- and late-onset male hypogonadism: the clinical practice guidelines of the Italian Society of Andrology and Sexual Medicine SIAMS and the Italian Society of Endocrinology SIE . J Endocrinol Invest. 2022 Aug 26;1 19. Open 10. Mary T Korytkowski, Ranganath Muniyappa, Kellie Antinori-Lent et al. Management of Hyperglycemia in Hospitalized Adult Patients in Non-Critical Care Settings: An Endocrine Society Clinical Practice Guideline. J Clin Endocrinol Metab. 2022 Jun 12;dgac278. Open 11. Giovanni Corona, Dimitrios G Goulis, Ilpo Huhtaniemi et al. European Academy of Andrology EAA guidelines on investigation, treatment and monitoring of functional hypogonadism in males: Endorsing organization: European Society of Endocrinology. Andrology. 2020 Sep;8 5 970 987. Open 12. Dawn O Kleindorfer, Amytis Towfighi, Seemant Chaturvedi et al. 2021 Guideline for the Prevention of Stroke in Patients With Stroke and Transient Ischemic Attack: A Guideline From the American Heart Association/American Stroke Association. Stroke. 2021 Jul;52 7):e364-e467. Open 13. Pronk NP, Remington PL. Combined Diet and Physical Activity Promotion Programs for Prevention of Diabetes: Community Preventive Services Task Force Recommendation Statement. Ann Intern Med. 2015 Sep 15;163 6 465 8. Open 14. Camilleri M, Parkman HP, Shafi MA et al. Clinical guideline: management of gastroparesis. Am J Gastroenterol. 2013 Jan;108 1 18 37. Open 15. US Preventive Services Task Force, Karina W Davidson, Michael J Barry et al. Screening for Prediabetes and Type 2 Diabetes: US Preventive Services Task Force Recommendation Statement. JAMA. 2021 Aug 24;326 8 736 743. Open 16. A. Salonia, C. Bettocchi, J. Carvalho et al. EAU Guidelines on Sexual and Reproductive Health. EAU. 2023. Open 17. Anil Hingorani, Glenn M LaMuraglia, Peter Henke et al. The management of diabetic foot: A clinical practice guideline by the Society for Vascular Surgery in collaboration with the American Podiatric Medical Association and the Society for Vascular Medicine. J Vasc Surg. 2016 Feb;63 2 Suppl):3S 21S. Open 18. Bhasin S, Brito JP, Cunningham GR et al. Testosterone Therapy in Men With Hypogonadism: An Endocrine Society Clinical Practice Guideline. J Clin Endocrinol Metab. 2018 May 1;103 5 1715 1744. Open 19. Jeff A Simerville, William C Maxted, John J Pahira. Urinalysis: a comprehensive review. Am Fam Physician. 2005 Mar 15;71 6 1153 62. Open 20. ADA. Executive summary: Standards of medical care in diabetes 2013. Diabetes Care. 2013 Jan;36 Suppl 1 S4 10. Open 21. Copeland KC, Silverstein J, Moore KR et al. Management of newly diagnosed type 2 Diabetes Mellitus T2DM in children and adolescents. Pediatrics. 2013 Feb;131 2 364 82. Open 22. Siu AL. Screening for Abnormal Blood Glucose and Type 2 Diabetes Mellitus: U.S. Preventive Services Task Force Recommendation Statement. Ann Intern Med. 2015 Dec 1;163 11 861 8. Open https://web.pathway.md/diseases/recNW2vC0lM724J79 20/22 6/29/23, 10:11 PM Diabetes mellitus type 2 Pathway 23. American Diabetes Association. Standards of Medical Care in Diabetes-2017 Abridged for Primary Care Providers. Clin Diabetes. 2017 Jan;35 1 5 26. Open 24. Diabetes Canada Clinical Practice Guidelines Expert Committee, Bril V, Breiner A et al. Neuropathy. Can J Diabetes. 2018 Apr;42 Suppl 1 S217 S221. Open 25. American Diabetes Association. 10. Microvascular Complications and Foot Care: Standards of Medical Care in Diabetes-2018. Diabetes Care. 2018 Jan;41 Suppl 1 S105 S118. Open 26. Diabetes Canada Clinical Practice Guidelines Expert Committee, McFarlane P, Cherney D et al. Chronic Kidney Disease in Diabetes. Can J Diabetes. 2018 Apr;42 Suppl 1 S201 S209. Open 27. Qaseem A, Barry MJ, Humphrey LL et al. Oral Pharmacologic Treatment of Type 2 Diabetes Mellitus: A Clinical Practice Guideline Update From the American College of Physicians. Ann Intern Med. 2017 Feb 21;166 4 279 290. Open 28. Game FL, Attinger C, Hartemann A et al. IWGDF guidance on use of interventions to enhance the healing of chronic ulcers of the foot in diabetes. Diabetes Metab Res Rev. 2016 Jan;32 Suppl 1 75 83. Open 29. American Diabetes Association. Standards of Medical Care in Diabetes-2019 Abridged for Primary Care Providers. Clin Diabetes. 2019 Jan;37 1 11 34. Open 30. Perkovic V, Jardine MJ, Neal B et al. Canagliflozin and Renal Outcomes in Type 2 Diabetes and Nephropathy. N Engl J Med. 2019 Jun 13;380 24 2295 2306. Open 31. American Diabetes Association. 11. Microvascular Complications and Foot Care: Standards of Medical Care in Diabetes 2019. Diabetes Care. 2019 Jan;42 Suppl 1 S124 S138. Open 32. Holman RR, Bethel MA, Mentz RJ et al. Effects of Once-Weekly Exenatide on Cardiovascular Outcomes in Type 2 Diabetes. N Engl J Med. 2017 Sep 28;377 13 1228 1239. Open 33. Pfeffer MA, Claggett B, Diaz R et al. Lixisenatide in Patients with Type 2 Diabetes and Acute Coronary Syndrome. N Engl J Med. 2015 Dec 3;373 23 2247 57. Open 34. American Diabetes Association. 14. Management of Diabetes in Pregnancy: Standards of Medical Care in Diabetes 2019. Diabetes Care. 2019 Jan;42 Suppl 1 S165 S172. Open 35. George L Bakris, Rajiv Agarwal, Stefan D Anker et al. Effect of Finerenone on Chronic Kidney Disease Outcomes in Type 2 Diabetes. N Engl J Med. 2020 Dec 3;383 23 2219 2229. Open 36. Kacie Doyle-Delgado, James J Chamberlain, Jay H Shubrook et al. Pharmacologic Approaches to Glycemic Treatment of Type 2 Diabetes: Synopsis of the 2020 American Diabetes Association's Standards of Medical Care in Diabetes Clinical Guideline. Ann Intern Med. 2020 Nov 17;173 10 813 821. Open 37. Antonio Bernabe-Ortiz, a, b et al. Diagnostic accuracy of the Finnish Diabetes Risk Score FINDRISC for undiagnosed T2DM in Peruvian population. Prim Care Diabetes. 2018 Dec; 12 6 517 525. Open 38. Jaana Lindstr m, Jaakko Tuomilehto. The diabetes risk score: a practical tool to predict type 2 diabetes risk. Diabetes Care. 2003 Mar;26 3 725 31. Open 39. American Diabetes Association Professional Practice Committee, American Diabetes Association Professional Practice Committee:, Draznin B et al. Standards of Medical Care in Diabetes-2022. Diabetes Care. 2022 Jan 1;45 Supplement_1 S1 S264. Open 40. Ayesha Abid, Shahla Ahmad, Abdul Waheed. Screening for Type II Diabetes Mellitus in the United States: The Present and the Future. Clin Med Insights Endocrinol Diabetes. 2016 Jun 13;9 19 22. Open https://web.pathway.md/diseases/recNW2vC0lM724J79 21/22 6/29/23, 10:11 PM Diabetes mellitus type 2 Pathway 41. Richard F Hamman, Rena R Wing, Sharon L Edelstein et al. Effect of weight loss with lifestyle intervention on risk of diabetes. Diabetes Care. 2006 Sep;29 9 2102 7. Open 42. Kidney Disease: Improving Global Outcomes KDIGO Diabetes Work Group. KDIGO 2020 Clinical Practice Guideline for Diabetes Management in Chronic Kidney Disease. Kidney Int. 2020 Oct;98 4S S1 S115. Open 43. Diabetes Canada Clinical Practice Guidelines Expert Committee, Jean-Fran ois Yale, Breay Paty et al. Hypoglycemia. Can J Diabetes. 2018 Apr;42 Suppl 1 S104 S108. Open 44. Kristi M Crowe-White, Levi W Evans, Gunter G C Kuhnle et al. Flavan-3-ols and Cardiometabolic Health: First Ever Dietary Bioactive Guideline. Adv Nutr. 2022 Dec 22;13 6 2070 2083. Open 45. Meir Schechter, Stephen D Wiviott, Itamar Raz et al. Effects of dapagliflozin on hospitalisations in people with type 2 diabetes: post-hoc analyses of the DECLARE TIMI 58 trial. Lancet Diabetes Endocrinol. 2023 Apr;11 4 233 241. Open 46. Hiddo J L Heerspink, Naveed Sattar, Imre Pavo et al. Effects of tirzepatide versus insulin glargine on kidney outcomes in type 2 diabetes in the SURPASS 4 trial: post-hoc analysis of an open-label, randomised, phase 3 trial. Lancet Diabetes Endocrinol. 2022 Sep 21;S2213 8587 22 00243 1. Open 47. Sandeep R Das, Brendan M Everett, Kim K Birtcher et al. 2020 Expert Consensus Decision Pathway on Novel Therapies for Cardiovascular Risk Reduction in Patients With Type 2 Diabetes: A Report of the American College of Cardiology Solution Set Oversight Committee. J Am Coll Cardiol. 2020 Sep 1;76 9 1117 1145. Open 48. Bertram Pitt, Gerasimos Filippatos, Rajiv Agarwal et al. Cardiovascular Events with Finerenone in Kidney Disease and Type 2 Diabetes. N Engl J Med. 2021 Dec 9;385 24 2252 2263. Open 49. 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Guideline sources The following summarized guidelines for the evaluation and management of diabetic foot infection (DFI) are prepared by our editorial team based on guidelines from the Infectious Diseases Society of America (IDSA/IWGDF 2023), the International Working Group on the Diabetic Foot (IWGDF/NHMRC 2022), the Society for Vascular Medicine (SVM/SVS/APMA 2016), and the Infectious Diseases Society of America (IDSA 2012). 1 2 3 4 5 5 5 6 Definition DFI is a micro-vascular complication characterized by soft tissue or bone infection below the malleoli in a diabetic. 5 Epidemiology DFI is caused by an amalgamation of neuropathy, vasculopathy, immunopathy, and foot ulcer infected with microbes (S. aureus, Proteus species, E. coli, Peptostreptococcus, Veilonella, and Bacteroides species). 5 Disease course https://web.pathway.md/diseases/recJSELILLPoWNtKg 1/10 6/29/23, 10:13 PM Diabetic foot infection Pathway Clinical manifestations include longstanding ulceration of a foot, constitutional symptoms (fever, tachycardia, tachypnea) of infection, wound erythema, hyperkeratosis, anhidrosis, fissures, blisters, nail disorders, and necrosis. Chronic infection may lead to osteomyelitis, gangrene, metabolic instability, and septic shock. 5 Prognosis and risk of recurrence The all-cause mortality associated with lower extremity amputation is 86.80 per 1,000 person- years. 6 Pathway Pathway Diabetic foot infection Diabetic foot infection Diagnosis and investigation Management Guidelines 1. Screening and diagnosis Diagnostic criteria: As per IWGDF 2023 guidelines, diagnose diabetes-related soft tissue infection clinically based on local or systemic signs and symptoms of inflammation. B As per IDSA 2012 guidelines, diagnose DFI in patients with at least 2 classic symptoms or signs of inflammation (erythema, warmth, tenderness, pain, or induration) or purulent secretions. B 2. Classification and risk stratification Severity assessment: As per IWGDF 2023 guidelines, use the IWGDF/IDSA classification scheme to assess the severity of any DFI. B As per IDSA 2012 guidelines, use a validated classification system, such as the IWGDF system, to classify DFIs, help define severity, and predict outcomes. A Show 2 more 3. Diagnostic investigations General principles: assess patients with diabetes presenting with a foot wound at 3 levels: the patient as a whole, the affected foot or limb, and the infected wound. B Physical examination: As per IWGDF 2023 guidelines: Avoid using foot temperature (however measured) for diagnosing DFI. D https://web.pathway.md/diseases/recJSELILLPoWNtKg 2/10 6/29/23, 10:13 PM Diabetic foot infection Pathway Consider obtaining the probe-to-bone test, in combination with plain X-rays and ESR (or CRP or procalcitonin), for the diagnosis of diabetic foot osteomyelitis. C As per SVS 2016 guidelines: Assess for infection on initial presentation of all patients with diabetic foot ulcers. B Consider obtaining the probe-to-bone test to aid in the diagnosis of diabetic foot osteomyelitis in patients with DFI with an open wound. C As per IDSA 2012 guidelines, assess for classic signs of inflammation (redness, warmth, swelling, tenderness, or pain) or purulent secretions, as well as additional or secondary signs (nonpurulent secretions, friable or discolored granulation tissue, undermining of wound edges, foul odor) for the evaluation of DFI. B Show 2 more Inflammatory markers: Obtain inflammatory serum biomarkers, such as CRP, ESR, or procalcitonin, in patients with diabetes and a possible infected foot ulcer if the clinical examination is diagnostically equivocal or uninterpretable. E Consider obtaining ESR, CRP, or procalcitonin, in combination with probe-to-bone testing and plain X-rays, for the diagnosis of diabetic foot osteomyelitis. C Radiography: As per IWGDF 2023 guidelines, consider obtaining plain X-rays for the diagnosis of diabetic foot osteomyelitis. C As per SVS 2016 guidelines, consider obtaining serial plain radiographs of the affected foot to identify bone abnormalities (deformity, destruction), soft tissue gas, and radiopaque foreign bodies in all patients presenting with a new DFI. C As per IDSA 2012 guidelines: Obtain plain radiographs of the affected foot to assess for bony abnormalities (deformity, destruction) and soft tissue gas and radio-opaque foreign bodies. B Consider obtaining serial plain radiographs to diagnose or monitor suspected diabetic foot osteomyelitis. C Magnetic resonance imaging: As per IWGDF 2023 guidelines, obtain MRI when the diagnosis of diabetic foot osteomyelitis remains in doubt despite clinical assessment, radiography, and laboratory tests. B As per SVS 2016 guidelines, obtain MRI in patients requiring additional (more sensitive or specific) imaging, particularly if a soft tissue abscess is suspected or the diagnosis of osteomyelitis remains uncertain. Obtain MRI for the diagnosis of osteomyelitis if the probe-to- bone test is inconclusive or the plain radiography is not useful. B As per IDSA 2012 guidelines: Obtain an MRI of the lower extremities in patients requiring further imaging, particularly when a soft tissue abscess is suspected or the diagnosis of osteomyelitis remains uncertain. B Suspect osteomyelitis as a potential complication of any infected, deep, or large foot ulcer, especially if chronic or overlies a bony prominence. B Tagged WBC and bone scans: https://web.pathway.md/diseases/recJSELILLPoWNtKg 3/10 6/29/23, 10:13 PM Diabetic foot infection Pathway As per IWGDF 2023 guidelines, consider obtaining WBC scintigraphy as an alternative to MRI for the diagnosis of diabetic foot osteomyelitis. C As per SVS 2016 guidelines, consider obtaining WBC or anti-granulocyte scintigraphy, preferably combined with a bone scan, in patients with suspected diabetic foot osteomyelitis if MRI is contraindicated or unavailable. C As per IDSA 2012 guidelines: Consider obtaining labeled WBC scintigraphy combined with a radionuclide bone scan for the diagnosis of diabetic foot osteomyelitis if MRI is unavailable or contraindicated. C Consider obtaining WBC or anti-granulocyte scintigraphy, preferably combined with a bone scan, to evaluate for diabetic foot osteomyelitis if MRI is unavailable or contraindicated. C Other imaging modalities: as per IWGDF 2023 guidelines, consider obtaining positron emission tomography or single photon emission CT as an alternative to MRI for the diagnosis of diabetic foot osteomyelitis. C Wound culture: As per IWGDF 2023 guidelines, consider obtaining microbiological culture, preferably by aseptically collecting a tissue specimen (curettage or biopsy) from the wound, to determine the causative microorganisms in patients with suspected soft tissue DFI. C Show 2 more As per IDSA 2012 guidelines, do not collect specimens for culture for clinically uninfected wounds. D Show 3 more 4. Diagnostic procedures Bone culture and histology: As per IWGDF 2023 guidelines, consider obtaining bone (rather than soft tissue) samples for culture, either intraoperatively or percutaneously, in patients with suspected diabetic foot osteomyelitis (before or after treatment). C As per SVS 2016 guidelines: Obtain bone culture and histology to confirm the diagnosis in patients at high risk for diabetic foot osteomyelitis. Send the samples for culture and histology if the bone is debrided to treat osteomyelitis. B Consider performing a bone biopsy in patients not undergoing bone debridement if faced with diagnostic uncertainty, inadequate culture information, or failure of response to empirical treatment. C 5. Medical management General principles: consider offering either primarily surgical or primarily medical strategies for the treatment of diabetic foot osteomyelitis in properly selected patients. C Setting of care: https://web.pathway.md/diseases/recJSELILLPoWNtKg 4/10 6/29/23, 10:13 PM Diabetic foot infection Pathway As per IDSA 2023 guidelines, consider hospitalizing patients with severe DFI or moderate infection associated with key relevant morbidities. C As per IDSA 2012 guidelines: Hospitalize patients with severe infection, patients with moderate infection and complicating features (such as severe peripheral arterial disease or lack of home support), and patients unable to comply with outpatient treatment for psychological or social reasons. B Consider hospitalizing patients not meeting the above criteria but not improving with outpatient therapy. B Empiric antibiotic therapy, diabetic foot infection: As per IDSA/IWGDF 2023 guidelines, do not initiate systemic antibiotic therapy in patients with uninfected foot ulcers when the goal is to reduce the risk of new infection or to promote ulcer healing. D Show 4 more As per IDSA 2012 guidelines, do not initiate antibiotic therapy in patients with clinically uninfected wounds. D Show 7 more Empiric antibiotic therapy (diabetic foot osteomyelitis): consider initiating antibiotic therapy without surgery in case of forefoot osteomyelitis without an immediate need for incision and drainage to control infection, without PAD, and without exposed bone. C Definitive antibiotic therapy: tailor definitive therapy according to the results of an appropriately obtained culture and sensitivity testing of a wound specimen, as well as the patient's clinical response to the empiric regimen. B Show 2 more Updated evidence: OVIVA In patients being treated for bone or joint infection, oral antibiotic therapy was noninferior to IV antibiotic therapy with respect to definitive treatment failure at 1 year. Li HK et al. N Engl J Med. 2019 Jan 31. Duration of treatment, diabetic foot infection: As per IDSA/IWGDF 2023 guidelines, continue antibiotic therapy for 1-2 weeks in patients with skin or soft tissue DFI. A Show 2 more As per IDSA 2012 guidelines: Consider completing 1-2 weeks of antibiotic therapy for mild infections and 2-3 weeks of antibiotic therapy for moderate-to-severe infections. C Consider continuing antibiotic therapy until, but not beyond, resolution of findings of infection, but not through complete healing of the wound. C Duration of treatment, diabetic foot osteomyelitis: As per IDSA/IWGDF 2023 guidelines: https://web.pathway.md/diseases/recJSELILLPoWNtKg 5/10 6/29/23, 10:13 PM Diabetic foot infection Pathway Consider continuing antibiotic therapy for up to 3 weeks after minor amputation for diabetic foot osteomyelitis and positive bone margin culture and 6 weeks for diabetic foot osteomyelitis without bone resection or amputation. C Use the outcome at a minimum follow-up duration of 6 months after the end of the antibiotic therapy to diagnose remission of diabetic foot osteomyelitis. E As per NHMRC/IWGDF 2022 guidelines, continue antibiotic therapy for no longer than 6 weeks in patients with diabetic foot osteomyelitis. Reconsider the need for collecting a bone specimen for culture, undertaking surgical resection, or selecting an alternative antibiotic regimen if the infection does not clinically improve within the first 2-4 weeks. B Show 3 more As per IDSA 2012 guidelines: Consider continuing antibiotic therapy for 2-5 days in patients with diabetic foot osteomyelitis undergone a radical resection leaving no remaining infected tissue. C Consider continuing treatment for 4 weeks in the presence of persistent infected or necrotic bone. C Topical antimicrobials: As per IDSA 2023 guidelines: Do not use local antibiotics in patients with uninfected foot ulcers when the goal is to reduce the risk of new infection or to promote ulcer healing. D Avoid using topical (sponge, cream, and cement) antibiotics in combination with systemic antibiotics for the treatment of diabetic soft-tissue infection or foot osteomyelitis. D As per IWGDF 2022 guidelines, avoid using any currently available topical antimicrobial agents for the treatment of patients with mild DFIs. D Glycemic control: consider ensuring adequate glycemic control (HbA1c < 7% with strategies to minimize hypoglycemia) to reduce the incidence of diabetic foot ulcers and infections, with subsequent risk of amputation. C 6. Therapeutic procedures Adjunctive therapies, diabetic foot infection: As per IDSA/IWGDF 2023 guidelines: Avoid offering the following adjunctive therapies for the management of DFIs: G-CSFs topical antiseptics or silver preparations honey bacteriophage therapy negative pressure wound therapy (with or without instillation). D Avoid offering hyperbaric oxygen therapy or topical oxygen therapy as an adjunctive treatment for the sole indication of treating a DFI. D As per IDSA 2012 guidelines, consider offering the following adjunctive therapies for selected slow-healing diabetic foot wounds: bioengineered skin equivalents C https://web.pathway.md/diseases/recJSELILLPoWNtKg 6/10 6/29/23, 10:13 PM Diabetic foot infection Pathway growth factors, G-CSFs C hyperbaric oxygen therapy B negative pressure wound therapy. C Adjunctive therapies (diabetic foot osteomyelitis): Avoid offering the following adjunctive therapies for the treatment of diabetic foot osteomyelitis: hyperbaric oxygen therapy growth factors, including G-CSF maggots (larvae) negative pressure therapy (such as vacuum-assisted closure). D 7. Surgical interventions Wound care: provide appropriate wound care in patients with diabetic foot ulcers. B Show 4 more Indications for surgeon consultation: As per IWGDF 2023 guidelines: Obtain urgent surgical consultation in patients with severe DFI or moderate foot infection complicated by extensive gangrene, necrotizing infection, signs suggesting deep (below the fascia) abscess, compartment syndrome, or severe lower limb ischemia. E Obtain an urgent consultation with a surgical specialist and a vascular specialist to determine the indications and timings of a drainage procedure and/or revascularization procedure in patients with diabetes, PAD, and a foot ulcer or gangrene with an infection involving any portion of the foot. E As per IWGDF 2022 guidelines, obtain urgent surgical consultation for patients with osteomyelitis with severe infection or moderate infection complicated by extensive gangrene, necrotizing infection, signs suggesting deep (below the fascia) abscess or compartment syndrome, or severe lower limb ischemia. B As per IDSA 2012 guidelines: Consider requesting an assessment by a surgeon for patients with a moderate or severe DFI. C Consult with a vascular surgeon to evaluate for revascularization whenever ischemia complicates a DFI, especially in patients with a critically ischemic limb. B Wound debridement: As per SVS 2016 guidelines, perform initial sharp debridement of all infected diabetic ulcers and urgent surgical intervention for foot infections involving abscess, gas, or necrotizing fasciitis. B As per IDSA 2012 guidelines, perform debridement of wounds with necrotic tissue or surrounding callus. B Surgical interventions: As per IDSA 2023 guidelines: Consider performing early (within 24-48 hours) surgery combined with antibiotics to remove infected and necrotic tissue in patients with moderate-to-severe DFIs. C https://web.pathway.md/diseases/recJSELILLPoWNtKg 7/10 6/29/23, 10:13 PM Diabetic foot infection Pathway Consider performing surgical resection of infected bone combined with systemic antibiotics in patients with diabetic foot osteomyelitis. C As per IWGDF 2022 guidelines, obtain urgent evaluation for surgery and intensive postoperative medical and surgical follow-up in patients with probable diabetic foot osteomyelitis with concomitant soft tissue infection. B As per IDSA 2012 guidelines, perform urgent surgical intervention for foot infections accompanied by gas in the deeper tissues, an abscess, or necrotizing fasciitis, and less urgent surgery for wounds with substantial nonviable tissue or extensive bone or joint involvement. B 8. Follow-up and surveillance Discharge criteria: Discharge patients with DFI meeting the following criteria: patient is clinically stable, and acceptable glycemic control has been attained patient has undergone any urgently needed surgery patient is able to manage on his/her own or with help at the designated discharge location patient has a well-defined plan that includes an appropriate antibiotic regimen to which he/she will adhere, an off-loading scheme if needed, specific wound care instructions, and appropriate outpatient follow-up. B Clinical findings Symptoms Past medical history Ankle swelling Bacterial infection Changes in foot skin color and temperature Fungal infection of the foot Inflammation Leg pain Peripheral vascular disease Leg swelling Poorly controlled diabetes Wounds or sores Integument exam Neurological exam Ingrowing nail Peripheral neuropathy Skin ulceration Wound infection Lab findings Hyperglycemia Likelihood Ratios Pertinent positives The following findings increase the probability of lower extremity osteomyelitis in adult patients with diabetes. 6 Finding LR+ Value https://web.pathway.md/diseases/recJSELILLPoWNtKg 8/10 6/29/23, 10:13 PM Diabetic foot infection Pathway Increased ESR 11 (1.6-79) Increased ulcer size (> 2 cm ) 7.2 (1.1-49) Positive probe-to-bone test 6.4 (3.6-11) Presence of clinical impression of osteomyelitis 5.5 (1.8-17) bone destruction alone or in combination with soft tissue swelling; localized osteopenia or periosteal reaction in the absence of fracture or neuropathic joint disease Show 2 more Pertinent negatives The following findings decrease the probability of lower extremity osteomyelitis in adult patients with diabetes. 6 Finding LR- Value Absence of findings of osteomyelitis 0.14 (0.08-0.26) Negative probe-to-bone test 0.39 (0.20-0.76) ulcer size not increased ( 2 cm ) 0.48 (0.31-0.76) Absence of clinical impression of osteomyelitis 0.54 (0.30-0.97) bone destruction alone or in combination with soft tissue swelling; localized osteopenia or periosteal reaction in the absence of fracture or neuropathic joint disease Show 1 more References 1. Lipsky BA, Berendt AR, Cornia PB et al. 2012 Infectious Diseases Society of America clinical practice guideline for the diagnosis and treatment of diabetic foot infections. Clin Infect Dis. 2012 Jun;54 12):e132 73. Open 2. ric Senneville, Zaina Albalawi, Suzanne A. van Asten et al. Guidelines on the diagnosis and treatment of foot infection in persons with diabetes. IWGDF Guidelines. 2023. Open 3. Robert J Commons, James Charles, Jane Cheney et al. Australian guideline on management of diabetes-related foot infection: part of the 2021 Australian evidence-based guidelines for diabetes- related foot disease. J Foot Ankle Res. 2022 Jun 9;15 1 47. Open 4. Anil Hingorani, Glenn M LaMuraglia, Peter Henke et al. The management of diabetic foot: A clinical practice guideline by the Society for Vascular Surgery in collaboration with the American Podiatric Medical Association and the Society for Vascular Medicine. J Vasc Surg. 2016 Feb;63 2 Suppl):3S 21S. Open 5. Saba Noor, Rizwan Ullah Khan, Jamal Ahmad. Understanding Diabetic Foot Infection and its Management. Diabetes Metab Syndr. Apr-Jun 2017;11 2 149 156. Open 6. Khalid Al-Rubeaan, Mohammad K Almashouq, Amira M Youssef et al. All-cause mortality among diabetic foot patients and related risk factors in Saudi Arabia. 2017 Nov 27;12 11):e0188097.2017 Nov 27;12 11):e0188097. Open 7. Milne WK, Carpenter CR. Evidence-based emergency medicine/rational clinical examination abstract. Does this patient with diabetes have osteomyelitis of the lower extremity?. Ann Emerg Med. 2009 May;53 5 677 9. Open https://web.pathway.md/diseases/recJSELILLPoWNtKg 9/10 6/29/23, 10:13 PM Diabetic foot infection Pathway 8. Sonia Butalia, MD, Valerie A. Palda et al. Does This Patient With Diabetes Have Osteomyelitis of the Lower Extremity?. JAMA. 2008;299 7 806 813. Open 9. Khalid Al-Rubeaan, Mohammad K. Almashouq, Amira M. Youssef et al. All-cause mortality among diabetic foot patients and related risk factors in Saudi Arabia. PLoS One. 2017; 12 11 e0188097. Open 10. American Diabetes Association Professional Practice Committee, American Diabetes Association Professional Practice Committee:, Draznin B et al. Standards of Medical Care in Diabetes-2022. Diabetes Care. 2022 Jan 1;45 Supplement_1 S1 S264. Open 11. Steven E. Kahn, Cheryl A.M. Anderson, John B. Buse et al. Standards of Care in Diabetes 2023. Diabetes Care. 2023 Jan;46 Supplement_1 S1 S291. Open https://web.pathway.md/diseases/recJSELILLPoWNtKg 10/10 |
Guideline sources The following summarized guidelines for the evaluation and management of diabetic foot are prepared by our editorial team based on guidelines from the American Diabetes Association (ADA 2023), the International Working Group on the Diabetic Foot (IWGDF/NHMRC 2022), and the Society for Vascular Medicine (SVM/SVS/APMA 2016). 1 2 3 4 4 4 5 Definition Diabetic foot is a complication of diabetes characterized by a triad of neuropathy, ischemia, and infection. 4 Epidemiology Diabetic foot is caused due to uncontrolled diabetes leading to the development of peripheral neuropathy (loss of sensation) and peripheral arterial disease (ischemia). 4 Disease course Clinical manifestations due to peripheral neuropathy lead to fissures, bullae, Charcot joint, edema, digital necrosis; and ischemia lead to pain at rest, ulceration on foot margins, digital necrosis, and gangrene. Disease progression may lead to osteomyelitis (chronic discharging https://web.pathway.md/diseases/recoT1Cu8BJ5PvOdO 1/7 6/29/23, 10:12 PM Diabetic foot Pathway sinus and sausage-like appearance of the toe) and gangrene formation that may require amputation. 4 Prognosis and risk of recurrence The all-cause mortality related to diabetic foot ulcer and lower extremity amputation is 42.54 per 1,000 person-years and 86.80 per 1000 person-years. 5 Pathway Pathway Diabetic foot Diabetic foot Management Screening and investigation Guidelines 1. Screening and diagnosis Indications for screening, average-risk patients: As per ADA 2023 guidelines, obtain a comprehensive foot evaluation at least annually to identify risk factors for ulcers and amputations. B As per SVM/APMA/SVS 2016 guidelines, perform foot inspections annually by physicians (MD, DO, DPM) or advanced practice providers with training in foot care. B Indications for screening, high-risk patients: As per ADA 2023 guidelines, obtain foot inspected at every visit in patients with evidence of sensory loss or history of ulceration or amputation. B As per SVM/APMA/SVS 2016 guidelines, consider performing annual vascular examination of the lower extremities and feet including ABI and and toe pressures in patients with diabetes and any of the following: history of diabetic foot ulcer prior abnormal vascular examination prior intervention for peripheral vascular disease known ASCVD (such as coronary, cerebral, or renal). C Indications for screening (pediatric patients): Obtain screening for diabetic neuropathy by foot examination (including foot inspection, assessment of foot pulses, pinprick and 10-g monofilament sensation tests, testing of vibration sensation using a 128-Hz tuning fork, and ankle reflex tests) at diagnosis and annually thereafter in young patients with T2DM. B Consider obtaining an annual comprehensive foot examination at the start of puberty or at age 10 years, whichever is earlier, once the patient has had T1DM for 5 years. C 2. Diagnostic investigations https://web.pathway.md/diseases/recoT1Cu8BJ5PvOdO 2/7 6/29/23, 10:12 PM Diabetic foot Pathway Clinical history: Elicit medical history of ulceration, amputation, Charcot foot, angioplasty or vascular surgery, cigarette smoking, retinopathy, and renal disease. B Assess current symptoms of neuropathy (pain, burning, numbness) and vascular disease (leg fatigue, claudication). B Physical examination: As per ADA 2023 guidelines: Include the following in the foot examination: inspection of skin assessment of foot deformities neurological assessment (10-g monofilament testing with at least one other assessment: pinprick, temperature, vibration) vascular assessment, including pulses in the legs and feet. B Assess lower extremity pulses, capillary refill time, rubor on dependency, pallor on elevation, and venous filling time for initial screening of peripheral arterial disease. B As per SVS 2016 guidelines, include testing for peripheral neuropathy using Semmes- Weinstein test in the foot examination. B Ankle-brachial index: As per ADA 2023 guidelines, obtain ABI testing and further vascular assessment as appropriate in patients with symptoms of claudication or decreased or absent pedal pulses. B As per SVS 2016 guidelines: Consider obtaining ABI measurement in patients with diabetes reached 50 years of age. C Assess pedal perfusion by ABI, ankle and pedal Doppler arterial waveforms, and either toe systolic pressure or transcutaneous oxygen pressure annually in patients with diabetic foot ulcer. B 3. Medical management Multidisciplinary management: ensure a multidisciplinary approach for patients with foot ulcers and high-risk feet (such as patients on dialysis, patients with Charcot foot, history of ulcers or amputation, or peripheral arterial disease). B Glycemic control: consider ensuring adequate glycemic control (HbA1c < 7% with strategies to minimize hypoglycemia) to reduce the incidence of diabetic foot ulcers and infections, with subsequent risk of amputation. C 4. Nonpharmacologic interventions Therapeutic footwear: As per ADA 2023 guidelines, offer specialized therapeutic footwear in high-risk patients with diabetes, including patients with loss of protective sensation, foot deformities, ulcers, callous https://web.pathway.md/diseases/recoT1Cu8BJ5PvOdO 3/7 6/29/23, 10:12 PM Diabetic foot Pathway formation, poor peripheral circulation, and a history of amputation. B As per SVS 2016 guidelines, avoid routine use of specialized therapeutic footwear in average-risk patients with diabetes. D Show 5 more 5. Therapeutic procedures Revascularization: Do not perform prophylactic arterial revascularization to prevent diabetic foot ulcer. D Perform revascularization by either surgical bypass or endovascular therapy in patients with diabetic foot ulcer and PAD. B Negative pressure wound therapy: Consider performing negative pressure wound therapy to reduce wound size, in addition to the best standard of care, in patients with a postoperative foot ulcer. C Avoid performing negative pressure wound therapy in preference to the best standard of care in nonsurgical diabetic foot ulcers. D Hyperbaric oxygen therapy: Consider performing systemic hyperbaric oxygen therapy as an adjunctive treatment in patients with non-healing ischemic diabetic foot ulcers despite the best standard of care. C Avoid performing topical oxygen therapy as a primary or adjunctive intervention in patients with diabetic foot ulcers, including difficult-to-heal ulcers. D Other therapies: As per ADA 2023 guidelines, consider offering adjunctive treatment with RCT-proven advanced agents (such as negative-pressure wound therapy, placental membranes, bioengineered skin substitutes, several acellular matrices, autologous fibrin and leukocyte platelet patches, and topical oxygen therapy) in patients with chronic diabetic foot ulcers failed to heal with optimal standard care alone. B As per IWGDF 2022 guidelines, consider offering placenta-derived products with informed consent as an adjunctive treatment, in addition to the best standard of care, when the latter alone has failed to reduce the size of the wound. C Show 4 more As per SVS 2016 guidelines, offer the following adjunctive wound therapy options in patients with diabetic foot ulcers failing to demonstrate improvement (> 50% wound area reduction) after a minimum of 4 weeks of standard wound therapy, with the choice of adjuvant therapy based on clinical findings, availability of therapy, and cost-effectiveness: negative pressure therapy biologics (platelet-derived growth factor, living cellular therapy, extracellular matrix products, amnionic membrane products) hyperbaric oxygen therapy. B Show 4 more 6. Surgical interventions https://web.pathway.md/diseases/recoT1Cu8BJ5PvOdO 4/7 6/29/23, 10:12 PM Diabetic foot Pathway Wound care: As per IWGDF 2022 guidelines, choose dressings principally on the basis of exudate control, comfort, and cost. B Show 2 more As per SVS 2016 guidelines: Obtain frequent evaluation at 1-4-week intervals with measurement of diabetic foot ulcers to monitor reduction of wound size and healing progress. B Use dressing products maintaining a moist wound bed, controlling exudate, and avoiding maceration of surrounding intact skin in patients with diabetic foot ulcers. B Surgical debridement: As per IWGDF 2022 guidelines, remove slough, necrotic tissue, and surrounding callus of a diabetic foot ulcer with sharp debridement in preference to other methods, taking into account relative contraindications such as pain or severe ischemia. B As per SVS 2016 guidelines, evaluate all patients with diabetic foot ulcer for infection on initial presentation. Perform initial sharp debridement of all infected diabetic ulcers and urgent surgical intervention for foot infections involving abscess, gas, or necrotizing fasciitis. B Show 2 more 7. Patient education Foot self-care education: As per ADA 2023 guidelines, provide general preventive foot self-care education to all patients with diabetes, including patients with loss of protective sensation, on appropriate ways to examine their feet (palpation or visual inspection with an unbreakable mirror) for daily surveillance of early foot problems. B As per SVS 2016 guidelines, educate patients and their families about preventive foot care. B 8. Follow-up and surveillance Indications for specialist referral: refer smoker patients or patients with a history of prior lower extremity complications, loss of protective sensation, structural abnormality, or peripheral arterial disease to foot care specialists for ongoing preventive care and life-long surveillance. B Clinical findings Symptoms Past medical history Ankle swelling Fungal infection of the foot Burning sensation of feet Poorly controlled diabetes https://web.pathway.md/diseases/recoT1Cu8BJ5PvOdO 5/7 6/29/23, 10:12 PM Diabetic foot Pathway Changes in foot skin color and temperature Foot ulcer Leg swelling References 1. Pamela Chen, Keryln Carville, Terry Swanson et al. Australian guideline on wound healing interventions to enhance healing of foot ulcers: part of the 2021 Australian evidence-based guidelines for diabetes-related foot disease. J Foot Ankle Res. 2022 May 25;15 1 40. Open 2. Anil Hingorani, Glenn M LaMuraglia, Peter Henke et al. The management of diabetic foot: A clinical practice guideline by the Society for Vascular Surgery in collaboration with the American Podiatric Medical Association and the Society for Vascular Medicine. J Vasc Surg. 2016 Feb;63 2 Suppl):3S 21S. Open 3. Steven E. Kahn, Cheryl A.M. Anderson, John B. Buse et al. Standards of Care in Diabetes 2023. Diabetes Care. 2023 Jan;46 Supplement_1 S1 S291. Open 4. Sharad P. Pendsey. Understanding diabetic foot. Int J Diabetes Dev Ctries. 2010 Apr-Jun; 30 2 75 79. Open 5. Khalid Al-Rubeaan, Mohammad K Almashouq, Amira M Youssef et al. All-cause mortality among diabetic foot patients and related risk factors in Saudi Arabia. 2017 Nov 27;12 11):e0188097.2017 Nov 27;12 11):e0188097. Open 6. Huang ET, Mansouri J, Murad MH et al. A clinical practice guideline for the use of hyperbaric oxygen therapy in the treatment of diabetic foot ulcers. Undersea Hyperb Med. 2015 May-Jun;42 3 205 47. Open 7. Lipsky BA, Arag n-S nchez J, Diggle M et al. IWGDF guidance on the diagnosis and management of foot infections in persons with diabetes. Diabetes Metab Res Rev. 2016 Jan;32 Suppl 1 45 74. Open 8. Game FL, Attinger C, Hartemann A et al. IWGDF guidance on use of interventions to enhance the healing of chronic ulcers of the foot in diabetes. Diabetes Metab Res Rev. 2016 Jan;32 Suppl 1 75 83. Open 9. American Diabetes Association. Standards of Medical Care in Diabetes-2019 Abridged for Primary Care Providers. Clin Diabetes. 2019 Jan;37 1 11 34. Open 10. American Diabetes Association. 11. Microvascular Complications and Foot Care: Standards of Medical Care in Diabetes 2019. Diabetes Care. 2019 Jan;42 Suppl 1 S124 S138. Open 11. American Podiatric Medical Association. Choosing Wisely APMA recommendations. Choosing Wisely. 2017. Open 12. Satish Chandra Mishra, Kunal C Chhatbar, Aditi Kashikar et al. Diabetic foot. BMJ. 2017; 359 j5064. Open 13. Khalid Al-Rubeaan, Mohammad K. Almashouq, Amira M. Youssef et al. All-cause mortality among diabetic foot patients and related risk factors in Saudi Arabia. PLoS One. 2017; 12 11 e0188097. Open 14. American Diabetes Association Professional Practice Committee, American Diabetes Association Professional Practice Committee:, Draznin B et al. Standards of Medical Care in Diabetes-2022. Diabetes Care. 2022 Jan 1;45 Supplement_1 S1 S264. Open https://web.pathway.md/diseases/recoT1Cu8BJ5PvOdO 6/7 6/29/23, 10:12 PM Diabetic foot Pathway 15. Robert J Commons, James Charles, Jane Cheney et al. Australian guideline on management of diabetes-related foot infection: part of the 2021 Australian evidence-based guidelines for diabetes- related foot disease. J Foot Ankle Res. 2022 Jun 9;15 1 47. Open https://web.pathway.md/diseases/recoT1Cu8BJ5PvOdO 7/7 |
Guideline sources The following summarized guidelines for the evaluation and management of diabetic ketoacidosis (DKA) are prepared by our editorial team based on guidelines from the American Diabetes Association (ADA 2023), the United Kingdom Kidney Association (UKKA 2021), the Kidney Disease: Improving Global Outcomes Foundation (KDIGO 2020), the French Society of Emergency Medicine (SFMU/SRLF 2019), the Diabetes Canada Guidelines (Diabetes Canada 2018), the British Thoracic Society (BTS 2017), and the Guidelines and Audit Implementation Network (GAIN 2014). 1 2 3 4 5 6 7 8 8 8 9 Definition DKA is an acute metabolic complication of diabetes characterized by a triad of hyperglycemia, ketosis, and acidemia. 8 Epidemiology DKA is caused by inadequate insulin therapy in hospital, new diagnosis of T1DM mellitus, poor concordance with insulin treatment, infection (commonly chest, urinary tract, skin), and acute coronary/vascular event. 9 Disease course https://web.pathway.md/diseases/recwQ3Cgp3qeCQ7od 1/6 6/29/23, 10:13 PM Diabetic ketoacidosis Pathway Clinical manifestations include nausea, vomiting, abdominal pain, weakness, polydipsia, polyuria, dehydration, fruity odor of breath, rapid shallow Kussmaul breathing, hypotension, and altered sensorium. Delay in hospitalization, acidosis severity, and peripheral vascular insufficiency are associated with increased mortality. 8 Prognosis and risk of recurrence The overall mortality rate of DKA is around 10%. 8 Calculator Diabetic Ketoacidosis Mortality Guidelines 1. Diagnostic investigations Urine ketones: do not rule out DKA on the basis of negative urine ketones. D Capillary beta-hydroxybutyrate: Consider obtaining point-of-care capillary -hydroxybutyrate testing in the hospital or outpatient setting to screen for DKA in patients with T1DM and capillary blood glucose > 14 mmol/L. C Obtain further testing for DKA in patients with a -hydroxybutyrate > 1.5 mmol/L. B Blood gas analysis: obtain blood gas analysis in patients with breathlessness thought to be at risk of DKA. B Evaluation of patients on SGLT2 inhibitors: assess patients treated with SGLT-2 inhibitors with symptoms of DKA for this condition, even if blood glucose is not elevated. B 2. Medical management IV fluid resuscitation: administer 0.9% sodium chloride (500 mL/hour IV for 4 hours, then 250 mL/hour for 4 hours) in order to correct hypovolemia in adult patients with DKA. B Show 2 more Insulin infusion: As per ADA 2023 guidelines, administer SC or IV insulin to rapidly correct the hyperglycemia and the metabolic derangement in patients with ketosis/ketoacidosis. Re-initiate metformin once acidosis is resolved while continuing SC insulin therapy. A As per Diabetes Canada 2018 guidelines: Administer continuous intravenous infusion of short-acting insulin at a rate of 0.10 units/kg/hour in patients with DKA. B https://web.pathway.md/diseases/recwQ3Cgp3qeCQ7od 2/6 6/29/23, 10:13 PM Diabetic ketoacidosis Pathway Continue IV insulin infusion until resolution of ketosis, B as measured by the normalization of the plasma anion gap. B Insulin infusion (FICS): consider administering insulin IV rather than SC in patients with DKA. C Show 3 more Dextrose infusion: administer IV dextrose once the plasma glucose concentration falls to 14.0 mmol/L, in order to avoid hypoglycemia. B Potassium replacement: Add potassium to IV fluids immediately in patients with presenting with normal potassium levels or hypokalemia. Add potassium in patients initially presenting with hyperkalemia once serum potassium falls to < 5-5.5 mmol/L and the patient is diuresing. Consider administering potassium phosphate with the aim to prevent rhabdomyolysis in patients with DKA and severe hypophosphatemia. Sodium bicarbonate: avoid administering sodium bicarbonate in patients with DKA. D Maintenance fluids: switch from 0.9% sodium chloride to 0.45% sodium chloride (with potassium chloride) as maintenance fluid therapy once the initial fluid deficit has been corrected. Maintain IV fluids at higher osmolality (may need to maintain on normal saline) if plasma osmolality is falling more rapidly than 3 mmol/kg/hour and/or the corrected plasma sodium is reduced. 3. Inpatient care Inpatient monitoring: consider obtaining close monitoring in patients with DKA, ideally in an ICU setting. E Glucose monitoring: recognize that capillary glucose testing with a glucometer may not provide an accurate or reliable measure of blood glucose in DKA. Obtain a venous blood sample for analysis. E 4. Patient education Counseling on SGLT2 inhibitors use: initiate SGLT-2 inhibitors with caution in patients with T2DM at greater risk of DKA, and after discussion with the diabetes team. B Show 6 more 5. Preventative measures Withholding SGLT2 inhibitors: As per UKKA 2021 guidelines, consider withholding SGLT-2 inhibitors for the duration of the fasting period in patients choosing to intermittently fast (such as for Ramadan), particularly in the elderly, patients on diuretics, or with CKD. C As per KDIGO 2020 guidelines, consider withholding SGLT-2 inhibitors during times of prolonged fasting, surgery, or critical medical illness (when patients may be at greater risk for https://web.pathway.md/diseases/recwQ3Cgp3qeCQ7od 3/6 6/29/23, 10:13 PM Diabetic ketoacidosis Pathway ketosis). C Clinical findings Symptoms Past medical history Abdominal pain Diabetes mellitus Confusion Poorly controlled diabetes Fainting Social history Fatigue Increased appetite Alcohol consumption Nausea Neurological exam Polydipsia Polyuria Altered mental status Shortness of breath Coma Somnolence Lethargy Thirst Visual disturbances Integument exam Vomiting Dehydration Weight loss Medication history Corticosteroids SGLT-2 inhibitors Vital signs Hypotension Tachycardia Tachypnea Respiratory exam Fruity breath odor Lab findings Metabolic acidosis blood glucose serum HbA1c serum anion gap serum ketones serum osmolal gap serum phosphate serum potassium serum sodium serum -hydroxybutyrate urine glucose urine ketones https://web.pathway.md/diseases/recwQ3Cgp3qeCQ7od 4/6 6/29/23, 10:13 PM Diabetic ketoacidosis Pathway serum bicarbonate serum magnesium serum pH serum potassium Likelihood Ratios Pertinent positives The following findings increase the probability of diabetic ketoacidosis in diabetic patients with hyperglycemia. 9 10 Finding LR+ Value Decreased serum bicarbonate 84 Increased serum anion gap (> 16 meq/L) 6.13 Increased serum -hydroxybutyrate (> 1.5 mmol/L) 4.67 Increased urine ketones 1.51 Pertinent negatives The following findings decrease the probability of diabetic ketoacidosis in diabetic patients with hyperglycemia. 9 10 Finding LR- Value serum -hydroxybutyrate not increased ( 1.5 mmol/L) 0.03 urine ketones not increased 0.06 serum anion gap not increased ( 16 meq/L) 0.09 serum bicarbonate not decreased 0.16 References 1. Jeannette Goguen, MD, MEd et al. Hyperglycemic Emergencies in Adults. Can J Diabetes. 2018 Apr;42 Suppl 1 S109 S114. Open 2. McVeigh G, Maxwell P, O'Donell S et al. Guidelines for the Treatment of Hyperkalaemia in Adults. GAIN Sub-Group on the Treatment of Hyperkalaemia in Adults. Open 3. Boris Jung, Mika l Martinez, Yann-Erick Claessens et al. Diagnosis and management of metabolic acidosis: guidelines from a French expert panel. Ann Intensive Care. 2019 Aug 15;9 1 92. Open 4. William G. Herrington, Andrew H. Frankel, Alexa Wonnacott et al. UK Kidney Association Clinical Practice Guideline: Sodium-Glucose Co-transporter-2 SGLT 2 Inhibition in Adults with Kidney Disease. UKKA. 2021 Oct. Open 5. Kidney Disease: Improving Global Outcomes KDIGO Diabetes Work Group. KDIGO 2020 Clinical Practice Guideline for Diabetes Management in Chronic Kidney Disease. Kidney Int. 2020 Oct;98 4S S1 S115. Open https://web.pathway.md/diseases/recwQ3Cgp3qeCQ7od 5/6 6/29/23, 10:13 PM Diabetic ketoacidosis Pathway 6. Steven E. Kahn, Cheryl A.M. Anderson, John B. Buse et al. Standards of Care in Diabetes 2023. Diabetes Care. 2023 Jan;46 Supplement_1 S1 S291. Open 7. O'Driscoll BR, Howard LS, Earis J et al. BTS guideline for oxygen use in adults in healthcare and emergency settings. Thorax. 2017 Jun;72 Suppl 1):ii1-ii90. Open 8. Pankaj Seth, Harpreet Kaur, Maneet Kaur. Clinical Profile of Diabetic Ketoacidosis: A Prospective Study in a Tertiary Care Hospital. 2015 Jun;9 6 OC01 4.2015 Jun;9 6 OC01 4. Open 9. Kate Evans. Diabetic ketoacidosis: update on management. 2019 Sep;19 5 396 398.2019 Sep;19 5 396 398. Open 10. Schwab TM, Hendey GW, Soliz TC. Screening for ketonemia in patients with diabetes. Ann Emerg Med. 1999 Sep;34 3 342 6. Open 11. Arora S, Henderson S, Long T et al. Diagnostic Accuracy of Point-of-Care Testing for Diabetic Ketoacidosis at Emergency-Department Triage. Diabetes Care. 2011 Apr; 34 4 852 854. Open 12. Pankaj Seth, Harpreet Kaur, and Maneet Kaur. Clinical Profile of Diabetic Ketoacidosis: A Prospective Study in a Tertiary Care Hospital. J Clin Diagn Res. 2015 Jun; 9 6 OC01 OC04. Open 13. Kate Evans. Diabetic ketoacidosis: update on management. Clin Med Lond). 2019 Sep; 19 5 396 398. Open 14. Stamatis P Efstathiou, Aphrodite G Tsiakou, Dimitrios I Tsioulos et al. A mortality prediction model in diabetic ketoacidosis. Clin Endocrinol (Oxf). 2002 Nov;57 5 595 601. Open https://web.pathway.md/diseases/recwQ3Cgp3qeCQ7od 6/6 |
Guideline sources The following summarized guidelines for the evaluation and management of diabetic nephropathy (DN) are prepared by our editorial team based on guidelines from the American Diabetes Association (ADA 2023), the Kidney Disease: Improving Global Outcomes Foundation (KDIGO 2022), and the Canadian Cardiovascular Society (CCS 2022). 1 2 3 4 4 4 4 Definition DN is a chronic microvascular complication of diabetes characterized by glomerular hyperfiltration, progressive albuminuria, and reduced GFR. 4 Epidemiology DN is caused due to hyperfiltration and reduction of GFR mainly due to hyperglycemia, systemic hypertension, and obesity. 4 Disease course Clinical manifestations of DN include microalbuminuria, macroalbuminuria, hyperglycemia, refractory hypertension, diabetic retinopathy, nephrotic or nephritic syndrome, anemia, bone and mineral metabolism complications. Disease progression may lead to CVD, infections, end-stage renal disease, and death. 4 https://web.pathway.md/diseases/rectlsnLmFenRaCjN 1/6 6/29/23, 10:16 PM Diabetic nephropathy Pathway Prognosis and risk of recurrence 1-year mortality of patients on dialysis is around 21.5% in the United States. 4 Pathway Diabetic nephropathy Diagnosis and management Guidelines 1. Diagnostic investigations Urinary albumin testing: Assess urinary albumin (such as spot urinary albumin-to-creatinine ratio) and eGFR at least annually in all patients with T2DM regardless of treatment. B Monitor urinary albumin (such as spot urinary albumin-to-creatinine ratio) and eGFR 1-4 times per year depending on the stage of the disease in patients with established diabetic kidney disease. B 2. Medical management General principles: treat patients with diabetes mellitus and CKD with a comprehensive strategy to reduce risks of kidney disease progression and CVD. B Show 3 more Goals of treatment: reduce urinary albumin by 30% to slow CKD progression in patients with CKD with 300 mg/g of urinary albumin. B Glycemic control: optimize glucose control to slow the progression of CKD. A Blood pressure control: optimize BP control and reduce BP variability to reduce the risk or slow the progression of CKD. A Metformin: initiate metformin in patients with T2DM, CKD and an eGFR 30 mL/min/1.73 m . B Show 4 more GLP-1 receptor agonists: initiate long-acting GLP-1 receptor agonists in patients with T2DM and CKD not achieved individualized glycemic targets despite use of metformin and SGLT-2 inhibitor treatment or unable to use these medications. B Show 5 more Landmark trials: LEADER In patients with T2DM who had a glycated Hgb level of 7.0% and high cardiovascular risk, liraglutide was noninferior to placebo with respect to death due to cardiovascular causes. https://web.pathway.md/diseases/rectlsnLmFenRaCjN 2/6 6/29/23, 10:16 PM Diabetic nephropathy Pathway Marso SP et al. N Engl J Med. 2016 Jul 28. SGLT2 inhibitors: As per ADA 2023 guidelines, initiate SGLT-2 inhibitors in patients with an eGFR 25 mL/min/1.73 m and urinary albumin 300 mg/g creatinine to reduce CKD progression and cardiovascular events in patients with T2DM and diabetic kidney disease. A Show 2 more Landmark trials: CREDENCE In patients with T2DM and kidney disease, canagliflozin was superior to placebo with respect to the incidence of ESRD, doubling of creatinine, renal death, or CV death. Perkovic V et al. N Engl J Med. 2019 Jun 13. As per CCS 2022 guidelines, initiate SGLT-2 inhibitors to reduce the composite of significant decline in eGFR, progression to end-stage kidney disease, or kidney death, all-cause and cardiovascular mortality, nonfatal myocardial infarction, and hospitalization for HF in adult patients with CKD (urine albumin-to-creatinine ratio > 20 mg/mmol and eGFR 25 mL/min/1.73 m ). B As per KDIGO 2022 guidelines, initiate SGLT-2 inhibitors in patients with T2DM, CKD and an eGFR 20 mL/min/1.73 m . B Show 7 more RAAS blocker therapy: As per ADA 2023 guidelines, do not use ACEIs or ARBs for the primary prevention of CKD in patients with diabetes with normal BP, normal urinary albumin-to-creatinine ratio (< 30 mg/g creatinine), and normal eGFR. D Show 5 more As per KDIGO 2022 guidelines, initiate an ACEI or an ARB in patients with diabetes, hypertension and albuminuria and titrate the medications to the highest approved dose that is tolerated. B Show 13 more 3. Nonpharmacologic interventions Dietary protein intake: aim to a target level of 0.8 g/kg body weight/day of dietary protein intake in patients with nondialysis-dependent stage 3 CKD. A Consider allowing higher levels of dietary protein intake in patients on dialysis since protein energy wasting is a major problem in some patients on dialysis. B Smoking cessation: Advise patients with diabetes mellitus and CKD to quit using tobacco products. B Counsel patients with diabetes mellitus and CKD to reduce secondhand smoke exposure. B https://web.pathway.md/diseases/rectlsnLmFenRaCjN 3/6 6/29/23, 10:16 PM Diabetic nephropathy Pathway 4. Specific circumstances Pediatric patients: consider obtaining annual screening for albuminuria with a random (morning sample preferred to avoid effects of exercise) spot urine sample for albumin-to- creatinine ratio in patients with T1DM at puberty or at age > 10 years, whichever is earlier, once the patient has had diabetes for 5 years. C Show 2 more 5. Preventative measures Primary prevention (glycemic control): optimize glucose control to reduce the risk of CKD. A Primary prevention (blood pressure control): optimize BP control and reduce BP variability to reduce the risk or slow the progression of CKD. A Primary prevention (RAAS blockers): do not use ACEIs or ARBs for the primary prevention of CKD in patients with diabetes with normal BP, normal urinary albumin-to-creatinine ratio (< 30 mg/g creatinine), and normal eGFR. D 6. Follow-up and surveillance Indications for specialist referral: Refer patients for evaluation by a nephrologist if urinary albumin levels are continuously increasing and/or eGFR is continuously decreasing or is < 30 mL/min/1.73 m . A Refer patients promptly to a nephrologist for uncertainty about the etiology of kidney disease, difficult management issues, and rapidly progressing kidney disease. A Serial laboratory monitoring: monitor serum creatinine and potassium levels periodically for the development of increased creatinine or changes in potassium when ACEIs, ARBs, or mineralocorticoid receptor antagonists are used, or hypokalemia when diuretics are used. B Clinical findings Symptoms Past medical history Ankle swelling Hypertension Frothy urine Poorly controlled diabetes Generalized pruritus Ocular exam Polyuria Poor appetite Eyelid swelling Puffy eyelids Vascular exam Leg edema Lab findings https://web.pathway.md/diseases/rectlsnLmFenRaCjN 4/6 6/29/23, 10:16 PM Diabetic nephropathy Pathway Hyperkalemia serum cholesterol serum creatinine urine microalbumin to creatinine urine protein Studies 2021 FIGARO-DKD In patients with T2DM and CKD treated with a renin-angiotensin system inhibitor at the maximum dose, finerenone was superior to placebo with respect to death from cardiovascular causes, nonfatal myocardial infarction, nonfatal stroke, or hospitalization for HF. Bertram Pitt et al. N Engl J Med. 2021 Dec 9. 2013 VA-NEPHRON D In patients with T2DM, a urinary albumin-to-creatinine ratio of at least 300, and an eGFR of 30.0 to 89.9 mL/min/1.73 m of body-surface area, combined angiotensin inhibition was not superior to monotherapy with respect to decline in eGFR (by 30 mL/min if eGFR 60-90 at baseline, or by 50% eGFR 30-60 at baseline), ESRD, or death. Fried LF et al. N Engl J Med. 2013 Nov 14. Show 2 more References 1. Steven E. Kahn, Cheryl A.M. Anderson, John B. Buse et al. Standards of Care in Diabetes 2023. Diabetes Care. 2023 Jan;46 Supplement_1 S1 S291. Open 2. Kidney Disease: Improving Global Outcomes KDIGO Diabetes Work Group. KDIGO 2022 Clinical Practice Guideline for Diabetes Management in Chronic Kidney Disease. Kidney Int. 2022 Nov;102 5S S1 S127. Open 3. G B John Mancini, Eileen O'Meara, Shelley Zieroth et al. 2022 Canadian Cardiovascular Society Guideline for Use of GLP 1 Receptor Agonists and SGLT2 Inhibitors for Cardiorenal Risk Reduction in Adults. Can J Cardiol. 2022 Aug;38 8 1153 1167. Open 4. Radica Z Alicic, Michele T Rooney, Katherine R Tuttle. Diabetic Kidney Disease: Challenges, Progress, and Possibilities. 2017 Dec 7;12 12 2032 2045.2017 Dec 7;12 12 2032 2045. Open 5. American Diabetes Association. Standards of Medical Care in Diabetes-2019 Abridged for Primary Care Providers. Clin Diabetes. 2019 Jan;37 1 11 34. Open 6. Perkovic V, Jardine MJ, Neal B et al. Canagliflozin and Renal Outcomes in Type 2 Diabetes and Nephropathy. N Engl J Med. 2019 Jun 13;380 24 2295 2306. Open 7. American Diabetes Association. 11. Microvascular Complications and Foot Care: Standards of Medical Care in Diabetes 2019. Diabetes Care. 2019 Jan;42 Suppl 1 S124 S138. Open https://web.pathway.md/diseases/rectlsnLmFenRaCjN 5/6 6/29/23, 10:16 PM Diabetic nephropathy Pathway 8. Radica Z. Alicic, Michele T. Rooney, and Katherine R. Tuttle. Diabetic Kidney Disease Challenges, Progress, and Possibilities. Clin J Am Soc Nephrol. 2017 Dec 7; 12 12 2032 2045. Open 9. Bertram Pitt, Gerasimos Filippatos, Rajiv Agarwal et al. Cardiovascular Events with Finerenone in Kidney Disease and Type 2 Diabetes. N Engl J Med. 2021 Dec 9;385 24 2252 2263. Open 10. Kidney Disease: Improving Global Outcomes KDIGO Diabetes Work Group. KDIGO 2020 Clinical Practice Guideline for Diabetes Management in Chronic Kidney Disease. Kidney Int. 2020 Oct;98 4S S1 S115. Open 11. Japanese Society of Nephrology. Essential points from Evidence-based Clinical Practice Guidelines for Chronic Kidney Disease 2018. Clin Exp Nephrol. 2019 Jan;23 1 1 15. Open https://web.pathway.md/diseases/rectlsnLmFenRaCjN 6/6 |
Guideline sources The following summarized guidelines for the evaluation and management of diabetic neuropathy are prepared by our editorial team based on guidelines from the American Diabetes Association (ADA 2023), the American Academy of Neurology (AAN 2022; 2010), the Diabetes Canada Guidelines (Diabetes Canada 2018), and the European Federation of Neurological Societies (EFNS 2010). 1 2 3 4 5 6 7 8 8 8 Definition Diabetic neuropathy is a type of neuropathy that occurs due to microvascular disease and chronic hyperglycemia in diabetic patients. 6 Epidemiology Diabetic neuropathy is caused by persistent hyperglycemia, microvascular insufficiency, oxidative and nitrosative stress, defective neurotropism, and autoimmune-mediated nerve destruction in patients with diabetes mellitus. 8 Pathophysiology The incidence of diabetic neuropathy is estimated at 17.8 per 100,000 person-years in the United Kingdom. 7 Di https://web.pathway.md/diseases/recPepuIukRLfRplP 1/6 6/29/23, 10:16 PM Diabetic neuropathy Pathway Disease course Nerve damage leads to hyperalgesia, dysesthesia, allodynia, weakness, numbness, hypoalgesia, anomalies in thermal sensation and autonomic function, foot ulcerations, gangrene, and amputations. 8 Prognosis and risk of recurrence Diabetic neuropathy is associated with a 1.7-12-fold higher risk of amputation. In addition, autonomic neuropathy is associated with a mortality rate of approximately 25-50% at 5-10 years. 8 Pathway Diabetic neuropathy Diagnosis and management Guidelines 1. Screening and diagnosis Indications for screening: As per ADA 2023 guidelines, screen for diabetic peripheral neuropathy starting at diagnosis of T2DM and 5 years after the diagnosis of T1DM, and at least annually thereafter. B As per AAN 2022 guidelines, assess patients with diabetes for peripheral neuropathic pain and its effect on the patient's function and quality of life. B As per Diabetes Canada 2018 guidelines: Screen for peripheral neuropathy in patients with T2DM at diagnosis and annually thereafter. B Screen for peripheral neuropathy in patients with T1DM annually starting 5 post-pubertal years after diagnosis. B 2. Diagnostic investigations Clinical assessment: As per ADA 2023 guidelines: Elicit a careful history and assess either temperature or pinprick sensation (small fiber function) and vibration sensation using a 128-Hz tuning fork (for large-fiber function) for the assessment of distal symmetric polyneuropathy. Obtain annual 10-g monofilament testing in all patients with diabetes to identify feet at risk for ulceration and amputation. B Assess for symptoms and signs of autonomic neuropathy (orthostatic dizziness, orthostatic hypotension, syncope, resting tachycardia, or dry cracked skin in the extremities) starting at diagnosis and at least annually thereafter in patients with evidence of other https://web.pathway.md/diseases/recPepuIukRLfRplP 2/6 6/29/23, 10:16 PM Diabetic neuropathy Pathway microvascular complications, particularly kidney disease and diabetic peripheral neuropathy. B As per Diabetes Canada 2018 guidelines, screen for peripheral neuropathy by assessing loss of sensitivity to the 10-g monofilament or loss of sensitivity to vibration at the dorsum of the great toe. A Psychosocial assessment: assess patients with painful diabetic neuropathy for the presence of concurrent mood and sleep disorders and treat them as appropriate. B 3. Medical management Glycemic control: As per ADA 2023 guidelines, optimize glucose control to slow the progression of neuropathy in patients with T2DM. B As per Diabetes Canada 2018 guidelines, optimize glycemic control to slow the progression of neuropathy in patients with T1DM A and T2DM. B Systemic therapy: As per ADA 2023 guidelines, offer gabapentinoids, SNRIs, TCAs, and sodium channel blockers as initial treatment for neuropathic pain in patients with diabetes. A Refer to a neurologist or pain specialist if pain control is not achieved within the scope of practice of the treating physician. B As per AAN 2022 guidelines, take into consideration factors other than efficacy, given similar efficacy, including potential adverse effects, patient comorbidities, cost, and patient preferences, when initiating pharmacologic treatment in patients with painful diabetic neuropathy. B Show 4 more As per Diabetes Canada 2018 guidelines: Consider offering the following agents, alone or in combination, for the treatment of patients with painful peripheral neuropathy: gabapentin C or pregabalin B valproate C amitriptyline C duloxetine or venlafaxine. C Consider offering opioid analgesics (tramadol, tapentadol ER, oxycodone ER), taking into account the risks of abuse, dependency, and tolerance, in patients not responding to the above agents. C As per EFNS 2010 guidelines, offer any of the following agents as first-line therapy in patients with painful polyneuropathy: TCAs gabapentin or pregabalin SNRIs (duloxetine, venlafaxine). A Show 2 more Topical therapy: https://web.pathway.md/diseases/recPepuIukRLfRplP 3/6 6/29/23, 10:16 PM Diabetic neuropathy Pathway As per AAN 2022 guidelines, consider offering topical therapy (capsaicin, glyceryl trinitrate spray, Citrullus colocynthis) in patients preferring topical interventions. C As per Diabetes Canada 2018 guidelines, consider offering topical nitrate spray, alone or in combination with systemic therapy, for the treatment of patients with painful peripheral neuropathy. C Management of inadequate response: view an individual intervention as a failure either when the medication has been titrated to a demonstrated efficacious dose for approximately 12 weeks without clinically significant pain reduction or when side effects from the medication outweigh any benefit in reduced neuropathic pain. B Show 2 more 4. Nonpharmacologic interventions Psychosocial interventions: consider offering CBT and exercise in patients preferring nonpharmacologic interventions. C Alternative and complementary medicine: consider offering Ginkgo biloba, Tai Chi, or mindfulness in patients preferring nontraditional interventions. C Footwear: offer specialized therapeutic footwear in high-risk patients with diabetes, including patients with loss of protective sensation, foot deformities, ulcers, callous formation, poor peripheral circulation, and a history of amputation. B 5. Therapeutic procedures Transcutaneous electrical nerve stimulation: consider offering electric nerve stimulation in patients with painful diabetic neuropathy. C 6. Patient education General counseling: counsel patients that the goal of pharmacologic therapy for painful diabetic neuropathy therapy is to reduce, and not necessarily eliminate, pain. B Show 2 more 7. Preventative measures Primary prevention: As per ADA 2023 guidelines, optimize glucose control to prevent or delay the development of neuropathy in patients with T1DM. B As per Diabetes Canada 2018 guidelines, optimize glycemic control to prevent the onset of neuropathy in patients with T1DM A and T2DM. B Clinical findings https://web.pathway.md/diseases/recPepuIukRLfRplP 4/6 6/29/23, 10:16 PM Diabetic neuropathy Pathway Symptoms Past medical history Bloating Diabetes mellitus Burning sensation of feet Diabetic foot Burning sensation of hands Diabetic nephropathy Constipation Diabetic retinopathy Diarrhea Dyslipidemia Difficulty walking Hypertension Early satiety Obesity Erectile dysfunction Orthostatic hypotension Exercise intolerance Poorly controlled diabetes Fecal incontinence Vascular exam Foot pain Hand pain Lower extremity ulcer Loss of appetite Integument exam Nausea Numbness of feet Hypohidrosis Numbness of hands Skin dryness Urinary incontinence Vomiting Social history Tobacco use Neurological exam Hyperesthesia Inability to wALK on heels light touch perception pinprick sensation vibration sensation Lab findings Hyperglycemia References 1. Steven E. Kahn, Cheryl A.M. Anderson, John B. Buse et al. Standards of Care in Diabetes 2023. Diabetes Care. 2023 Jan;46 Supplement_1 S1 S291. Open 2. Raymond Price, Don Smith, Gary Franklin et al. Oral and Topical Treatment of Painful Diabetic Polyneuropathy: Practice Guideline Update Summary: Report of the AAN Guideline Subcommittee. Neurology. 2022 Jan 4;98 1 31 43. Open 3. Diabetes Canada Clinical Practice Guidelines Expert Committee, Bril V, Breiner A et al. Neuropathy. Can J Diabetes. 2018 Apr;42 Suppl 1 S217 S221. Open 4. Dubinsky RM, Miyasaki J. Assessment: efficacy of transcutaneous electric nerve stimulation in the treatment of pain in neurologic disorders (an evidence-based review): report of the Therapeutics and https://web.pathway.md/diseases/recPepuIukRLfRplP 5/6 6/29/23, 10:16 PM Diabetic neuropathy Pathway Technology Assessment Subcommittee of the American Academy of Neurology. Neurology. 2010 Jan 12;74 2 173 6. Open 5. N Attal, G Cruccu, R Baron et al. EFNS guidelines on the pharmacological treatment of neuropathic pain: 2010 revision. Eur J Neurol. 2010 Sep;17 9 1113-e88. Open 6. Juster-Switlyk K, Smith AG. Updates in diabetic peripheral neuropathy. F1000Res. 2016 Apr 25;5. pii: F1000 Faculty Rev-738. Open 7. Reed C, Hong J, Novick D et al. Incidence of diabetic peripheral neuropathic pain in primary care - a retrospective cohort study using the United Kingdom General Practice Research Database. Pragmat Obs Res. 2013 Sep 27;4 27 37. eCollection 2013. Open 8. Vinik AI, Nevoret ML, Casellini C et al. Diabetic neuropathy. Endocrinol Metab Clin North Am. 2013 Dec;42 4 747 87. Open 9. Richardson JK. The clinical identification of peripheral neuropathy among older persons. Arch Phys Med Rehabil. 2002 Nov;83 11 1553 8. Open 10. Kanji JN, Anglin RE, Hunt DL et al. Does this patient with diabetes have large-fiber peripheral neuropathy?. JAMA. 2010 Apr 21;303 15 1526 32. Open 11. American Diabetes Association. Standards of Medical Care in Diabetes-2019 Abridged for Primary Care Providers. Clin Diabetes. 2019 Jan;37 1 11 34. Open 12. American Diabetes Association. 11. Microvascular Complications and Foot Care: Standards of Medical Care in Diabetes 2019. Diabetes Care. 2019 Jan;42 Suppl 1 S124 S138. Open 13. American Diabetes Association Professional Practice Committee, American Diabetes Association Professional Practice Committee:, Draznin B et al. Standards of Medical Care in Diabetes-2022. Diabetes Care. 2022 Jan 1;45 Supplement_1 S1 S264. Open https://web.pathway.md/diseases/recPepuIukRLfRplP 6/6 |
Guideline sources The following summarized guidelines for the evaluation and management of diabetic retinopathy (DR) are prepared by our editorial team based on guidelines from the American Diabetes Association (ADA 2023). 1 2 2 3 Definition Diabetic retinopathy is a chronic microvascular complication of diabetes characterized by visual impairment in one or both the eyes. Epidemiology DR is caused due to uncontrolled longstanding hyperglycemia. 2 Disease course Clinical manifestations of DR include microaneurysms, dot and blot hemorrhages, hard exudates, cotton wool spots, intraretinal microvascular abnormalities, and retinal edema in non- proliferative DR. Severe non-proliferative DR progresses to proliferative DR characterized by neovascularization. Progressive DR results in diabetic macular edema and permanent visual loss. 2 Prognosis and risk of recurrence https://web.pathway.md/diseases/rec4yr6vMjoFc8FDi 1/4 6/29/23, 10:16 PM Diabetic retinopathy Pathway DR is associated with increased all-cause mortality and cardiovascular events risk with an odds ratio of 2.34 (95% CI 1.96-2.80]. 3 Pathway Diabetic retinopathy Diagnosis and management Guidelines 1. Screening and diagnosis Indications for screening: Obtain an initial dilated and comprehensive eye examination by an ophthalmologist or optometrist within 5 years after the onset of T1DM and at the time of diagnosis in patients with T2DM. B Consider employing programs using retinal photography (with remote reading or the use of a validated assessment tool) to improve access to DR screening. Ensure that such programs provide pathways for timely referral for a comprehensive eye examination when indicated. C 2. Medical management Glycemic control: optimize glycemic control to slow the progression of DR. A Cardiovascular risk factor control: optimize BP and control dyslipidemia to slow the progression of DR. A Management of antiplatelet agents: recognize that the presence of retinopathy is not a contraindication to aspirin therapy for cardioprotection, as aspirin does not increase the risk of retinal hemorrhage. A 3. Therapeutic procedures Intravitreous anti-VEGF injections: Consider administering intravitreal injections of anti-VEGF as an alternative to traditional panretinal laser photocoagulation in some patients with proliferative DR and also reduce the risk of vision loss in these patients. B Administer intravitreal injections of anti-VEGF as first-line therapy in most eyes with diabetic macular edema involving the foveal center and impairing vision acuity. A Intravitreous corticosteroid injections: consider administering intravitreal injections of corticosteroids in eyes with persistent diabetic macular edema despite previous anti-VEGF therapy or eyes not being candidates for this first-line approach. B https://web.pathway.md/diseases/rec4yr6vMjoFc8FDi 2/4 6/29/23, 10:16 PM Diabetic retinopathy Pathway Laser photocoagulation: Perform panretinal laser photocoagulation therapy to reduce the risk of vision loss in patients with high-risk proliferative DR and, in some cases, severe nonproliferative DR. A Consider performing macular focal/grid photocoagulation in patients with persistent diabetic macular edema despite previous anti-VEGF therapy or eyes not being candidates for this first-line approach. B 4. Specific circumstances Pediatric patients: obtain an initial dilated and comprehensive eye examination once the patient has had T1DM for 3-5 years, provided the patient is aged 11 years or puberty has started, whichever is earlier. Obtain repeat dilated and comprehensive eye examinations every 2 years after the initial examination. Consider obtaining less frequent examinations, every 4 years, on the advice of an eye care professional and based on risk factor assessment, including a history of HbA1c < 8%. B Show 3 more Pregnant patients: Counsel pregnant patients or patients planning a pregnancy with preexisting diabetes on the risk of development and/or progression of DR. B Obtain an eye examination before pregnancy or in the first trimester in patients with preexisting diabetes. Monitor patients every trimester and for 1 year postpartum as indicated by the degree of retinopathy. B 5. Preventative measures Primary prevention (glycemic control): optimize glycemic control to reduce the risk of DR. A Primary prevention (cardiovascular risk factor control): optimize BP and control dyslipidemia to reduce the risk of developing DR. A 6. Follow-up and surveillance Indications for referral: Refer patients with any of the following to an ophthalmologist knowledgeable and experienced in the management of DR: any level of diabetic macular edema moderate or worse nonproliferative DR (a precursor of proliferative DR) any proliferative DR. A Serial eye examinations (patients without retinopathy): consider repeating screening every 1-2 years if there is no evidence of retinopathy for 1 annual eye exam and glycemia is well controlled. C Serial eye examinations (patients with retinopathy): repeat dilated retinal examinations at least annually if there is any level of DR. Obtain ocular examinations more frequently id https://web.pathway.md/diseases/rec4yr6vMjoFc8FDi 3/4 6/29/23, 10:16 PM Diabetic retinopathy Pathway retinopathy is progressing or sight-threatening. B Clinical findings Symptoms Past medical history Blurred vision Diabetes mellitus Eye pain Dyslipidemia Floaters and flashes Hypertension Vision loss Poorly controlled diabetes Neurological exam Ocular exam Visual field defect Cotton wool spots Macular edema Lab findings Retinal hemorrhage Retinal microaneurysms Hyperglycemia visual acuity References 1. Steven E. Kahn, Cheryl A.M. Anderson, John B. Buse et al. Standards of Care in Diabetes 2023. Diabetes Care. 2023 Jan;46 Supplement_1 S1 S291. Open 2. Ramandeep Singh, Kim Ramasamy, Chandran Abraham et al. Diabetic retinopathy: an update. May- Jun 2008;56 3 178 88.May-Jun 2008;56 3 178 88. Open 3. Caroline K Kramer, Ticiana C Rodrigues, Luis H Canani et al. Diabetic retinopathy predicts all-cause mortality and cardiovascular events in both type 1 and 2 diabetes: meta-analysis of observational studies. 2011 May;34 5 1238 44.2011 May;34 5 1238 44. Open 4. American Diabetes Association. Standards of Medical Care in Diabetes-2019 Abridged for Primary Care Providers. Clin Diabetes. 2019 Jan;37 1 11 34. Open 5. American Diabetes Association. 11. Microvascular Complications and Foot Care: Standards of Medical Care in Diabetes 2019. Diabetes Care. 2019 Jan;42 Suppl 1 S124 S138. Open 6. Ramandeep Singh, Kim Ramasamy, Chandran Abraham et al. Diabetic retinopathy: An update. Indian J Ophthalmol. 2008 May-Jun; 56 3 179 188. Open 7. Caroline K. Kramer, Ticiana C. Rodrigues, Luis H. Canani et al. Diabetic Retinopathy Predicts All- Cause Mortality and Cardiovascular Events in Both Type 1 and 2 Diabetes. Diabetes Care. 2011 May; 34 5 1238 1244. Open 8. American Diabetes Association Professional Practice Committee, American Diabetes Association Professional Practice Committee:, Draznin B et al. Standards of Medical Care in Diabetes-2022. Diabetes Care. 2022 Jan 1;45 Supplement_1 S1 S264. Open https://web.pathway.md/diseases/rec4yr6vMjoFc8FDi 4/4 |
Guideline sources The following summarized guidelines for the evaluation and management of diffuse glioma are prepared by our editorial team based on guidelines from the European Association of Neuro- Oncology (EANO 2021). 1 Calculator Calculator Eastern Cooperative Oncology G Karnofsky performance status s Guidelines 1. Screening and diagnosis https://web.pathway.md/diseases/recBe9Tc1A8oSfRI1 1/3 6/29/23, 10:17 PM Diffuse glioma Pathway Screening: recognize that screening and prevention have no major role for patients with gliomas. B 2. Classification and risk stratification Classification: use the most recent WHO Classification of Tumors of the CNS for glioma classification, complemented by cIMPACT-NOW. B 3. Diagnostic investigations History and physical examination: assess the Karnofsky performance score and neurological function, as well as the patient's age, individual risks, and benefits for clinical decision-making. B Diagnostic imaging: Obtain MRI without and with gadolinium contrast as the first choice diagnostic imaging modality. B Suspect pseudoprogression in patients with an increase of abnormalities on neuroimaging in the first months after local therapeutic interventions (including radiotherapy) and after experimental local treatments. B 4. Diagnostic procedures Tissue diagnosis: perform resection or biopsy in the standard of care of patients with IDH- mutant astrocytomas, WHO grade 2 requiring further treatment, or grade 3. B Show 2 more Analysis of biopsy specimens: obtain routine immunohistochemistry for mutant IDH-1 R132H protein and nuclear expression of ATRX in the diagnostic evaluation of patients with diffuse gliomas. B Show 7 more 5. Medical management Chemotherapy: offer chemotherapy with procarbazine, lomustine and vincristine polychemotherapy after resection or biopsy in the standard of care of patients with IDH-mutant astrocytomas, WHO grade 2 requiring further treatment. B Show 5 more 6. Therapeutic procedures Radiotherapy: perform field radiotherapy after resection or biopsy in the standard of care for patients with IDH-mutant astrocytomas, WHO grade 2 requiring further treatment or grade 3. B Show 2 more https://web.pathway.md/diseases/recBe9Tc1A8oSfRI1 2/3 6/29/23, 10:17 PM Diffuse glioma Pathway 7. Surgical interventions Surgical resection: perform resection as feasible or biopsy followed by involved field radiotherapy and maintenance chemotherapy in the standard of care of patients with IDH- mutant astrocytomas, WHO grade 2 requiring further treatment, or grade 3. B Show 2 more 8. Specific circumstances Elderly patients: offer radiotherapy (such as 2.66 Gy for 15 sessions) or chemotherapy with temozolomide (5 out of 28 days) based on MGMT promoter methylation status in elderly patients with IDH-wild-type glioblastoma ineligible for combined chemoradiotherapy. B 9. Patient education Genetic counseling: refer patients with relevant germline variants or suspected hereditary cancer syndromes for genetic counseling and, subsequently, for possible molecular genetic testing. B 10. Follow-up and surveillance Management of recurrent gliomas: recognize that standards of care of patients with IDH- wild-type glioblastomas are less well defined at recurrence. B Show 3 more Studies 2023 INDIGO In patients with residual or recurrent grade 2 IDH-mutant glioma who had undergone no previous treatment other than surgery, vorasidenib was superior to placebo with respect to median progression-free survival. Ingo K Mellinghoff et al. N Engl J Med. 2023 Jun 4. Online ahead of print. References 1. Michael Weller, Martin van den Bent, Matthias Preusser et al. EANO guidelines on the diagnosis and treatment of diffuse gliomas of adulthood. Nat Rev Clin Oncol. 2021 Mar;18 3 170 186. Open https://web.pathway.md/diseases/recBe9Tc1A8oSfRI1 3/3 |
Guideline sources The following summarized guidelines for the evaluation and management of diffuse large B-cell lymphoma (DLBCL) are prepared by our editorial team based on guidelines from the British Society for Haematology (BSH 2020; 2019), the European Society of Medical Oncology (ESMO 2018; 2016; 2015), and the British HIV Association (BHIVA 2014). 1 3 4 5 6 7 8 Calculator Calculator Calculat Eastern Cooperative Oncology G Karnofsky performance status s Modifie Guidelines 1. Classification and risk stratification Staging: use the Ann Arbor classification system for staging DLBCL. A Prognostic scores: As per ESMO 2018 guidelines: https://web.pathway.md/diseases/recSlLC3Q5JGcLlHv 1/8 6/29/23, 10:17 PM Diffuse large B-cell lymphoma Pathway Recognize that the International Prognostic Index parameters (age > 60 years, high LDH levels, poor performance status, advanced disease stage, and 1 extranodal site) are risk factors for early CNS relapse following first-line treatment of DLBCL, with a direct relationship between the number of unfavorable features and the CNS risk. B Recognize that the involvement of the testes, kidneys, adrenal glands, breast, bone marrow, and bone has also been reported to increase the risk of CNS disease. B As per ESMO 2015 guidelines, use the International Prognostic Index and age-adjusted International Prognostic Index scores for prognostic purposes. A 2. Diagnostic investigations History and physical examination: perform a physical examination and assess performance status and B symptoms in patients with DLBCL. B Imaging for staging: as per ESMO 2015 guidelines, obtain FDG-positron emission tomography/CT for staging DLBCL. B Show 2 more Laboratory evaluation: as per ESMO 2015 guidelines, obtain CBC, routine blood chemistry including LDH and uric acid, and screening tests for human immunodeficiency virus, HBV, and HCV. B Obtain protein electrophoresis. B Cardiac evaluation: assess cardiac function (LVEF) before initiating treatment. B 3. Diagnostic procedures Biopsy and histopathology: as per ESMO 2015 guidelines, perform surgical biopsy as the optimal method of diagnosis. B Show 4 more Ancillary testing: as per ESMO 2015 guidelines, obtain immunophenotypic investigations to confirm the morphological diagnosis of DLBCL. B Show 2 more Lumbar puncture: as per ESMO 2015 guidelines, consider performing a diagnostic lumbar puncture in high-risk patients. B 4. Medical management General principles: as per ESMO 2015 guidelines, stratify treatment strategies according to age, the International Prognostic Index score, and feasibility of dose-intensified approaches. Show 4 more Induction and consolidation therapy: as per ESMO 2015 guidelines, offer 6 cycles of combination chemotherapy with CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone) combined with 6 doses of rituximab every 21 days in young, low-risk patients (age-adjusted International Prognostic Index of 0) with non-bulky disease. Do not use https://web.pathway.md/diseases/recSlLC3Q5JGcLlHv 2/8 6/29/23, 10:17 PM Diffuse large B-cell lymphoma Pathway radiotherapy to initial non-bulky sites or consolidation in patients, whether treated with rituximab or not. A Show 4 more CNS prophylaxis: As per BSH 2020 guidelines, administer CNS prophylaxis in patients with any of the following: high (4-6) CNS-International Prognostic Index involvement of 3 extranodal sites irrespective of CNS-International Prognostic Index anatomical sites: testicular, renal/adrenal, intravascular. B Show 9 more As per ESMO 2018 guidelines: Obtain brain MRI and CSF analysis (by conventional cytology examination and flow cytometry) to assess patients with DLBCL considered as high risk for CNS relapse. B Avoid offering intrathecal chemotherapy in patients with DLBCL deemed at high risk for CNS relapse. Consider administering IV prophylaxis as an option in high-risk patients without evidence of CNS involvement. Offer a combination of anti-lymphoma drugs with good CNS bioavailability aimed at controlling both the CNS and systemic disease, preferably within a clinical trial, in patients with MRI and CSF evidence of CNS involvement at presentation. D As per ESMO 2016 guidelines, administer prophylactic therapy (such as intrathecal chemotherapy with methotrexate or IV systemic methotrexate) to reduce the risk of CNS relapse. B Show 2 more As per ESMO 2015 guidelines: Administer CNS prophylaxis in patients with high-intermediate-risk and high-risk International Prognostic Index, especially in patients with 1 extranodal site or elevated LDH or in patients with testicular, renal, or adrenal involvement. B Consider administering IV high-dose methotrexate for efficient disease control. C 5. Specific circumstances Patients with plasmablastic lymphoma: obtain immunohistochemistry tests (for CD38, CD20, PAX5, CD138, EBER, CD30, MUM-1, Kappa-lambda, HHV8, and ALK) as part of diagnostic assessment in human immunodeficiency virus-negative patients with suspected plasmablastic lymphoma. B Show 3 more Patients with HIV-associated DLBCL: As per ESMO 2015 guidelines, initiate the same treatment in patients with human immunodeficiency virus infection as in patients without human immunodeficiency virus in association with antiviral therapy. B As per BHIVA 2014 guidelines, administer chemotherapy regimens (such as CHOP or EPOCH) used in human immunodeficiency virus-negative patients for first-line treatment of diffuse large-B cell lymphoma in human immunodeficiency virus-positive patients. B https://web.pathway.md/diseases/recSlLC3Q5JGcLlHv 3/8 6/29/23, 10:17 PM Diffuse large B-cell lymphoma Pathway Show 3 more Patients with HBV infection: administer antiviral prophylaxis or obtain periodic HBV deoxyRNA monitoring and initiate antiviral treatment in patients previously exposed to HBV and experiencing reactivation of the virus during treatment. B Patients with central nervous system DLBCL, diagnosis: As per BSH 2019 guidelines, discuss suspected cases of cases primary CNS lymphoma with a primary CNS lymphoma specialist early in their pathway to minimize delays. B Show 5 more As per ESMO 2016 guidelines: Perform stereotactic biopsy as a minimally invasive technique to obtain a histopathological diagnosis. B Do not perform resection of primary CNS lymphoma. B Obtain testicular ultrasound in elderly male patients. B Patients with central nervous system DLBCL (baseline imaging): Obtain contrast-enhanced MRI (including diffusion sequences) as the imaging modality of choice before treatment and for response assessment. Review neuroaxis imaging (brain and entire spinal cord) by a specialist neuroradiologist. B Obtain cross-sectional imaging to exclude systemic disease in all patients: FDG-positron emission tomography-CT as the first choice A contrast-enhanced CT of the neck, chest, abdomen, and pelvis if positron emission tomography-CT is not possible B testicular ultrasound in male patients. B Patients with central nervous system DLBCL (ophthalmological assessment): obtain a thorough ophthalmological assessment, including slit lamp examination, in all patients to exclude intraocular involvement. B Patients with central nervous system DLBCL (cognitive assessment): Assess cognitive and quality of life outcomes in all patients with primary CNS lymphoma before and after treatment, with ongoing long-term monitoring. Assess cognitive domains of attention, processing speed, motor speed, executive function, and memory before and after treatment in all patients receiving treatment for primary CNS lymphoma. Patients with central nervous system DLBCL, induction chemotherapy: As per BSH 2019 guidelines, discuss all confirmed cases of primary CNS lymphoma at a lymphoma multidisciplinary team meeting. Initiate definitive treatment as soon as possible, ideally within 14 days of diagnosis, at an established center with multidisciplinary primary CNS lymphoma expertise. B Show 14 more As per ESMO 2016 guidelines, offer high-dose methotrexate-based regimens in combination with high-dose cytarabine for induction treatment if possible based on the patient's age, performance status, and organ function. A Show 2 more Patients with central nervous system DLBCL (consolidation therapy): recognize that whole- brain radiotherapy as consolidation after high-dose methotrexate-based chemotherapy is still https://web.pathway.md/diseases/recSlLC3Q5JGcLlHv 4/8 6/29/23, 10:17 PM Diffuse large B-cell lymphoma Pathway commonly applied in young patients and generally avoided in patients > 60 years because of the particularly high risk of neurotoxicity (including cognitive deterioration) in elderly patients. B Show 3 more Patients with central nervous system DLBCL (management of intraocular lymphoma): offer systemic high-dose methotrexate-based combination chemotherapy with rituximab for the treatment of primary intraocular lymphoma. Consider offering evidence-based primary CNS lymphoma induction protocols, such as the MATRix regimen, in fit patients. B Show 3 more Patients with central nervous system DLBCL (assessment of treatment response): obtain contrast-enhanced MRI (including diffusion sequences) as the imaging modality of choice for response assessment. Review neuroaxis imaging (brain and entire spinal cord) by a specialist neuroradiologist. B Show 5 more Patients with central nervous system DLBCL, salvage therapy: As per BSH 2019 guidelines, offer enrollment in a clinical trial, wherever possible. B Show 6 more As per ESMO 2016 guidelines: Insufficient evidence to recommend a standard protocol for salvage treatment. Consider offering high-dose chemotherapy followed by ASCT in fit patients. I Offer whole-brain radiotherapy as palliative treatment in patients with primary CNS lymphoma unable to tolerate or relapsing after high-dose, CNS-penetrating chemotherapy and not fit for further chemotherapy. B Patients with primary mediastinal DLBCL (mediastinal biopsy): perform a core or excisional biopsy to establish the histological diagnosis, reviewed by a specialist hematopathologist and discussed in a multidisciplinary team meeting to correlate clinical and pathological findings. A Patients with primary mediastinal DLBCL (bone marrow biopsy): Avoid performing bone marrow biopsy if a positron emission tomography-CT has been obtained. D Consider performing a bone marrow biopsy if it may influence patient risk management (CNS disease risk in patients presenting with extranodal disease or when a positron emission tomography-CT has not been obtained due to clinical urgency to initiate cytotoxic chemotherapy). C Patients with primary mediastinal DLBCL (baseline imaging): obtain baseline positron emission tomography-CT at diagnosis of patients with primary mediastinal DLBCL. A Patients with primary mediastinal DLBCL (fertility counseling): discuss regarding potential fertility preservation techniques before initiating chemotherapy, unless urgent lifesaving treatment is required. B Patients with primary mediastinal DLBCL (pregnant patients): manage pregnant patients presenting with primary mediastinal B-cell lymphoma in liaison with obstetricians and anesthesiologists spelcialized in high-risk pregnancies. B Patients with primary mediastinal DLBCL, induction therapy: https://web.pathway.md/diseases/recSlLC3Q5JGcLlHv 5/8 6/29/23, 10:17 PM Diffuse large B-cell lymphoma Pathway As per BSH 2019 guidelines, offer enrollment in a clinical trial, wherever possible. A Show 2 more As per ESMO 2016 guidelines, consider offering R-CHOP, R-VACOP-B, V-MACOP-B, R- CHOP14, or more intensive chemotherapy regimens, such as DA-EPOCH-R, as the current standard treatment in patients with primary mediastinal large B-cell lymphoma. B Patients with primary mediastinal DLBCL (consolidation therapy): offer consolidative mediastinal radiotherapy in responding patients treated with standard dose chemoimmunotherapy (R-CHOP/R-V/MACOP-B). B Show 3 more Patients with primary mediastinal DLBCL (assessment of treatment response): obtain positron emission tomography-CT at the end of combined modality treatment (2-3 months post-treatment) in patients receiving R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisolone) and involved-site radiotherapy outside a clinical trial. B Show 5 more Patients with primary mediastinal DLBCL, salvage therapy: As per BSH 2019 guidelines, consider offering radiotherapy in patients omitted from initial therapy with localized relapse. B Show 2 more As per ESMO 2016 guidelines: Intensify therapy with high-dose chemotherapy/ASCT in young patients not achieving an adequate response (less than partial response) with an elevated FDG uptake at the post- chemoimmunotherapy positron emission tomography/CT, if residual disease is confirmed by biopsy when feasible. B Attempt reinduction with non-cross-resistant agents followed by consolidation with high- dose chemotherapy/ASCT as a salvage treatment strategy in patients with relapsed/resistant chemosensitive disease. B Patients with testicular DLBCL: perform orchiectomy for diagnostic and therapeutic purposes. B Show 5 more Patients with breast DLBCL: perform biopsy to establish the diagnosis of breast DLBCL. B Show 5 more Patients with bone DLBCL: offer R-CHOP chemotherapy with or without consolidation radiotherapy (30-40 Gy) as the standard approach in patients with any stage of DLBCL with bone involvement. B Show 4 more 6. Follow-up and surveillance Serial clinical assessment: elicit careful history and perform physical examination every 3 months for 1 year, every 6 months for 2 further years, and then once a year with attention to the development of secondary tumors or other long-term side-effects of chemotherapy. B https://web.pathway.md/diseases/recSlLC3Q5JGcLlHv 6/8 6/29/23, 10:17 PM Diffuse large B-cell lymphoma Pathway Serial laboratory assessment: obtain CBC at 3, 6, 12, and 24 months, then only as needed for evaluation of suspicious symptoms or clinical findings in patients suitable for further therapy. B Serial imaging assessment: as per ESMO 2015 guidelines, obtain FDG-positron emission tomography/CT post-treatment assessment in patients with DLBCL. A Show 2 more Repeat biopsy: as per ESMO 2015 guidelines, perform a biopsy in the presence of residual metabolically active tissue if salvage treatment is being considered. B Clinical findings Symptoms Past medical history Fatigue Ataxia-telangiectasia Fever Common variable immunodeficiency Night sweats EBV infection Shortness of breath Gastrointestinal bleeding Testicular lump HCV infection Weight loss Human immunodeficiency virus infection Head and neck exam Rheumatoid arthritis Neck mass SLE Severe combined immunodeficiency Lymphatic exam Sj gren's disease Cervical lymphadenopathy Superior vena cava syndrome Lymphadenopathy Wiskott-aldrich syndrome Imaging findings Abdominal exam Mediastinal enlargement Hepatomegaly Mediastinal lymphadenopathy Palpable abdominal mass Spinal cord compression Splenomegaly Lab findings serum LDH Studies 2020 TRIST In patients with hematologic malignancies requiring hematopoietic cell transplantation, restrictive strategy was noninferior to liberal strategy with respect to a health-related quality of life measured by Functional Assessment of Cancer Therapy-BMT score at day 100. Jason Tay et al. J Clin Oncol. 2020 May 1. https://web.pathway.md/diseases/recSlLC3Q5JGcLlHv 7/8 6/29/23, 10:17 PM Diffuse large B-cell lymphoma Pathway References 1. Christopher P Fox, Elizabeth H Phillips, Jeffery Smith et al. Guidelines for the diagnosis and management of primary central nervous system diffuse large B-cell lymphoma. Br J Haematol. 2019 Feb;184 3 348 363. Open 2. Sridhar Chaganti, Tim Illidge, Sally Barrington et al. Guidelines for the management of diffuse large B- cell lymphoma. Br J Haematol. 2016 Jul;174 1 43 56. Open 3. H Tilly, M Gomes da Silva, U Vitolo et al. Diffuse large B-cell lymphoma DLBCL ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2015 Sep;26 Suppl 5:v116 25. Open 4. Kate Cwynarski, Maria A V Marzolini, Sally F Barrington et al. The management of primary mediastinal B-cell lymphoma: a British Society for Haematology Good Practice Paper. Br J Haematol. 2019 May;185 3 402 409. Open 5. Bower M, Palfreeman A, Alfa-Wali M et al. British HIV Association guidelines for HIV-associated malignancies 2014. HIV Med. 2014 Mar;15 Suppl 2 1 92. Open 6. U Vitolo, J F Seymour, M Martelli et al. Extranodal diffuse large B-cell lymphoma DLBCL and primary mediastinal B-cell lymphoma: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2016 Sep;27(suppl 5):v91-v102. Open 7. Pamela McKay, Matthew R Wilson, Sridhar Chaganti et al. The prevention of central nervous system relapse in diffuse large B-cell lymphoma: a British Society for Haematology good practice paper. Br J Haematol. 2020 Sep;190 5 708 714. Open 8. M Hutchings, M Ladetto, C Buske et al. ESMO Consensus Conference on malignant lymphoma: management of 'ultra-high-risk' patients. Ann Oncol. 2018 Aug 1;29 8 1687 1700. Open 9. Laurie H Sehn, Brian Berry, Mukesh Chhanabhai et al. The revised International Prognostic Index R IPI is a better predictor of outcome than the standard IPI for patients with diffuse large B-cell lymphoma treated with R CHOP. Blood. 2007 Mar 1;109 5 1857 61. Open 10. Abraham S Kanate, Ambuj Kumar, Peter Dreger et al. Maintenance Therapies for Hodgkin and Non- Hodgkin Lymphomas After Autologous Transplantation: A Consensus Project of ASBMT, CIBMTR, and the Lymphoma Working Party of EBMT. JAMA Oncol. 2019 May 1;5 5 715 722. Open 11. Steven H Kroft, Cordelia E Sever, Adam Bagg et al. Laboratory Workup of Lymphoma in Adults: Guideline From the American Society for Clinical Pathology and the College of American Pathologists. Arch Pathol Lab Med. 2021 Mar 1;145 3 269 290. Open 12. C Buske, M Hutchings, M Ladetto et al. ESMO Consensus Conference on malignant lymphoma: general perspectives and recommendations for the clinical management of the elderly patient with malignant lymphoma. Ann Oncol. 2018 Mar 1;29 3 544 562. Open https://web.pathway.md/diseases/recSlLC3Q5JGcLlHv 8/8 |
Guideline sources The following summarized guidelines for the management of digoxin toxicity are prepared by our editorial team based on guidelines from the American Heart Association (AHA 2020), the American Heart Association (AHA/HRS/ACC 2019), and the Extracorporeal Treatments in Poisoning Workgroup (EXTRIP 2016). 1 2 3 Guidelines 1. Medical management Digoxin immune Fab: As per AHA 2020 guidelines, administer antidigoxin Fab antibodies in patients with severe cardiac glycoside toxicity. B As per ACC 2019 guidelines, consider administering digoxin immune Fab to increase HR and improve symptoms in patients with bradycardia associated with symptoms or hemodynamic compromise in the setting of digoxin toxicity. C 2. Therapeutic procedures Extracorporeal treatment: As per ACC 2019 guidelines, do not offer hemodialysis for removal of digoxin in patients with bradycardia associated with symptoms or hemodynamic compromise attributable to digoxin toxicity. D As per EXTRIP 2016 guidelines, do not offer ECTR in any of the following situations: severe digoxin toxicity regardless if Fab is administered https://web.pathway.md/diseases/recGR7F51ejTFagGg 1/2 6/29/23, 10:17 PM Digoxin toxicity Pathway severe digoxin toxicity regardless if Fab is administered suspected digoxin ingestion regardless if Fab is administered elevated digoxin serum concentrations regardless if Fab is administered cardiovascular disturbances if Fab is administered serum potassium > 46.0 mmol/L. D Show 5 more Clinical findings Patient demographics Symptoms Elderly age Abdominal pain Dizziness Vital signs Loss of appetite Nausea Bradycardia Palpitations Tachycardia Shortness of breath Lab findings Visual disturbances Vomiting Hyperkalemia Neurological exam Altered mental status ECG findings AV block Atrial flutter Junctional escape rhythm Multifocal AT St-segment elevation References 1. Fred M Kusumoto, Mark H Schoenfeld, Coletta Barrett et al. 2018 ACC/AHA/HRS Guideline on the Evaluation and Management of Patients With Bradycardia and Cardiac Conduction Delay: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society. Circulation. 2019 Aug 20;140 8):e382-e482. Open 2. Ashish R Panchal, Jason A Bartos, Jos G Caba as et al. Part 3 Adult Basic and Advanced Life Support: 2020 American Heart Association Guidelines for Cardiopulmonary Resuscitation and Emergency Cardiovascular Care. Circulation. 2020 Oct 20;142 16_suppl_2 S366 S468. Open 3. James B Mowry, Emmanuel A Burdmann, Kurt Anseeuw et al. Extracorporeal treatment for digoxin poisoning: systematic review and recommendations from the EXTRIP Workgroup. Clin Toxicol Phila). 2016;54 2 103 14. Open https://web.pathway.md/diseases/recGR7F51ejTFagGg 2/2 |
Guideline sources The following summarized guidelines for the evaluation and management of dilated cardiomyopathy are prepared by our editorial team based on guidelines from the Heart Failure Society of America (HFSA/AHA/ACC 2022), the European Society of Cardiology (ESC 2022; 2021), the American Heart Association (AHA/ASA 2021), the American Heart Association (AHA/HRS/ACC 2018), the American Heart Association (AHA 2016), the American Heart Association (AHA/ACC 2015), and the European Heart Rhythm Association (EHRA/HRS 2011). 1 2 3 4 5 6 7 8 Calculator Calculator ESC diagnostic criteria for dilate NYHA functional classification f Guidelines 1. Screening and diagnosis f f https://web.pathway.md/diseases/rec8M1y7BZO2OaSC6 S i il l ti 1/10 6/29/23, 10:18 PM Dilated cardiomyopathy Pathway Screening of family relatives: As per ESC 2022 guidelines: Obtain an ECG and echocardiography in a first-degree relative of patients with dilated cardiomyopathy if: the index patient was diagnosed at < 50 years of age or has clinical features suggestive of an inherited cause there is a family history of dilated cardiomyopathy or premature unexpected sudden death. B Consider obtaining an ECG and echocardiography in a first-degree relative of patients with apparently sporadic dilated cardiomyopathy. C As per AHA 2022 guidelines, offer genetic screening and counseling in first-degree relatives of selected patients with genetic or inherited cardiomyopathies to detect cardiac disease and prompt consideration of treatments to decrease HF progression and sudden death. B As per AHA 2016 guidelines, elicit a 3-generational family history to aid in establishing the diagnosis of familial dilated cardiomyopathy in patients with idiopathic dilated cardiomyopathy. B Show 2 more As per HRS 2011 guidelines, obtain mutation-specific genetic testing in family members and appropriate relatives following the identification of a dilated cardiomyopathy-causative mutation in the index case. A 2. Diagnostic investigations Cardiac MRI: As per ESC 2022 guidelines, consider obtaining cardiac MRI with late gadolinium enhancement to assess the etiology and the risk of ventricular arrhythmia/sudden cardiac death in patients with dilated cardiomyopathy. C As per ESC 2021 guidelines, consider obtaining cardiac MRI with late gadolinium enhancement in patients with dilated cardiomyopathy to distinguish between ischemic and non-ischemic myocardial damage. C As per AHA 2018 guidelines: Consider obtaining cardiac MRI with late gadolinium enhancement for diagnosis in patients with suspected non-ischemic cardiomyopathy from myocardial infiltrative processes. B Consider obtaining cardiac MRI with late gadolinium enhancement for assessing the risk of sudden cardiac arrest/sudden cardiac death in patients with suspected non-ischemic cardiomyopathy. C Genetic testing: As per ESC 2022 guidelines: Obtain genetic testing (including at least lamin A/C, PLN, RBM20, and FLNC genes) in patients with dilated cardiomyopathy and atrioventricular conduction delay at < 50 years, or having a family history of dilated cardiomyopathy or sudden cardiac death in a first-degree relative (at age < 50 years). B https://web.pathway.md/diseases/rec8M1y7BZO2OaSC6 2/10 6/29/23, 10:18 PM Dilated cardiomyopathy Pathway Consider obtaining genetic testing (including at least lamin A/C, PLN, RBM20, and FLNC genes) for risk stratification in patients with apparently sporadic dilated cardiomyopathy presenting at a young age or with signs of suspicion for an inherited etiology. C As per AHA 2022 guidelines, consider referring selected patients with non-ischemic cardiomyopathy for genetic counseling and testing to identify conditions likely to guide treatment in patients and family members. C As per AHA 2018 guidelines, consider providing genetic counseling and obtaining genetic testing in patients with non-ischemic cardiomyopathy developing conduction disease or LV dysfunction at < 40 years of age or having a family history of non-ischemic cardiomyopathy or sudden cardiac death in a first-degree relative (< 50 years of age) to detect a heritable disease to clarify prognosis and facilitate cascade screening of relatives. C As per AHA 2016 guidelines: Consider obtaining genetic testing in conjunction with genetic counseling in patients with familial or idiopathic cardiomyopathy. C Consider obtaining genetic testing in patients with familial dilated cardiomyopathy to confirm the diagnosis, to facilitate cascade screening within the family, and to help with family planning. B As per HRS 2011 guidelines: Obtain comprehensive or targeted (LMNA and SCN5A) genetic testing in patients with dilated cardiomyopathy and significant cardiac conduction disease (first-, second-, or third- degree heart block) and/or a family history of premature unexpected sudden death. A Consider obtaining genetic testing in patients with familial dilated cardiomyopathy to confirm the diagnosis, identify patients at the highest risk of arrhythmia and syndromic features, facilitate cascade screening within the family, and help with family planning. C 3. Diagnostic procedures Electrophysiology study: As per ESC 2022 guidelines, consider obtaining electrophysiological evaluation in patients with dilated cardiomyopathy when syncope remains unexplained after noninvasive evaluation. C As per AHA 2018 guidelines: Consider obtaining electrophysiologic testing to evaluate the risk of sustained VT in patients with non-ischemic cardiomyopathy presenting with syncope or other ventricular arrhythmia symptoms and not meeting indications for a primary prevention ICD. C Consider performing ICD placement or obtaining electrophysiologic testing for risk stratification for sudden cardiac death in patients with non-ischemic cardiomyopathy experienced syncope presumed to be due to ventricular arrhythmia and not meeting indications for a primary prevention ICD, if the expected meaningful survival is > 1 year. C 4. Medical management Antiarrhythmic agents: https://web.pathway.md/diseases/rec8M1y7BZO2OaSC6 3/10 6/29/23, 10:18 PM Dilated cardiomyopathy Pathway As per ESC 2022 guidelines, consider adding oral amiodarone or replacing -blockers with sotalol in patients with dilated cardiomyopathy and an ICD experiencing recurrent, symptomatic ventricular arrhythmia despite optimal device programming and -blocker treatment. C As per AHA 2018 guidelines: Consider initiating amiodarone for the prevention of sudden cardiac death in patients with non-ischemic cardiomyopathy survived a cardiac arrest, having sustained VT or symptomatic ventricular arrhythmia, and are ineligible for an ICD (due to a limited life- expectancy and/or functional status or lack of access to an ICD). C Consider initiating amiodarone or sotalol in patients with non-ischemic cardiomyopathy and an ICD experiencing spontaneous ventricular arrhythmia or recurrent appropriate shocks despite optimal device programming and treatment with a -blocker. C 5. Therapeutic procedures Implantable cardioverter-defibrillator, primary prevention of SCD: As per ESC 2022 guidelines, consider performing ICD placement in patients with dilated cardiomyopathy, symptomatic HF (NYHA class II-III), and LVEF 35% after 3 months of optimal medical treatment. B Show 2 more As per HFSA/ACC/AHA 2022 guidelines, perform ICD placement for primary prevention of sudden cardiac death to reduce total mortality in patients with non-ischemic dilated cardiomyopathy or ischemic heart disease at least 40 days post-myocardial infarction with LVEF 35% and NYHA class II-III symptoms on chronic guideline-directed medical therapy, if the expected meaningful survival is > 1 year. A As per HRS/ACC/AHA 2018 guidelines, perform ICD placement in patients with non-ischemic cardiomyopathy, HF with NYHA class II-III symptoms, and LVEF of 35% despite guideline- directed medical therapy, if the expected meaningful survival is > 1 year. A Show 3 more Implantable cardioverter-defibrillator, secondary prevention of SCD: As per ESC 2022 guidelines: Perform ICD placement in patients with dilated cardiomyopathy survived sudden cardiac arrest due to VT/VF or experienced hemodynamically not-tolerated sustained monomorphic VT. B Consider performing ICD placement in patients with dilated cardiomyopathy and hemodynamically tolerated sustained monomorphic VT. C As per HRS/ACC/AHA 2018 guidelines: Perform ICD placement in patients with non-ischemic cardiomyopathy either survived sudden cardiac arrest due to VT/VF or experienced hemodynamically unstable VT B or stable sustained VT B not due to reversible causes, if the expected meaningful survival is > 1 year. B Consider performing ICD placement or obtaining electrophysiologic testing for risk stratification for sudden cardiac death in patients with non-ischemic cardiomyopathy https://web.pathway.md/diseases/rec8M1y7BZO2OaSC6 4/10 6/29/23, 10:18 PM Dilated cardiomyopathy Pathway experienced syncope presumed to be due to ventricular arrhythmia and not meeting indications for a primary prevention ICD, if the expected meaningful survival is > 1 year. C Catheter ablation: As per ESC 2022 guidelines, consider performing catheter ablation in specialized centers in patients with dilated cardiomyopathy and recurrent, symptomatic sustained monomorphic VT or ICD shocks for sustained monomorphic VT, if antiarrhythmic drugs are ineffective, contraindicated, or not tolerated. C As per AHA 2018 guidelines, consider performing catheter ablation for reducing recurrent VT and ICD shocks in patients with non-ischemic cardiomyopathy and recurrent sustained monomorphic VT failed or intolerant to antiarrhythmic medications. C 6. Specific circumstances Athletes: As per ESC 2022 guidelines, discourage participation in high-intensity exercise including competitive sports in patients with dilated cardiomyopathy and a lamin A/C mutation. B As per AHA 2015 guidelines, discourage participation in most competitive sports in symptomatic athletes with dilated cardiomyopathy, with the possible exception of low- intensity (class 1A sports) in selected cases, at least until more information is available. B Pediatric patients (evaluation): exclude underlying causes such as primary arrhythmias, cardiotoxins, congestive heart disease, or other structural defects such as anomalous left coronary artery from the pulmonary artery before making the diagnosis of idiopathic dilated cardiomyopathy in pediatric patients with cardiomyopathy phenotype. B Show 8 more Pediatric patients (management): initiate guideline-directed medical therapy for adult HF (including diuretic agents, -blockers, ACEIs, and other medications as appropriate) in pediatric patients with dilated cardiomyopathy. B Show 4 more Patients with cardiac amyloidosis (evaluation): consider performing an endomyocardial biopsy to identify amyloid protein in cardiac tissue in patients with suspected cardiac amyloidosis, especially if there is no noncardiac tissue evidence of amyloidosis. C Show 9 more Patients with cardiac amyloidosis (management): initiate warfarin therapy (with a goal INR of 2-3) or direct thrombin inhibitors in patients with cardiac amyloidosis and paroxysmal or chronic AF or a history of embolic stroke/TIA. A Show 10 more Patients with alcoholic cardiomyopathy: advise total abstinence in patients with alcoholic cardiomyopathy. B Patients with cocaine-related cardiomyopathy: consider initiating standard therapy for LV dysfunction, including -blockers, in patients with cocaine-related cardiomyopathy with demonstrated abstinence for > 6 months. C Show 2 more https://web.pathway.md/diseases/rec8M1y7BZO2OaSC6 5/10 6/29/23, 10:18 PM Dilated cardiomyopathy Pathway Patients with chemotherapy-related cardiomyopathy: obtain cardiac functional assessment with LVEF measurement at baseline, after completing treatment, and while on treatment at regular intervals, or sooner if HF symptoms develop in patients treated with cardiotoxic chemotherapeutic agents. B Show 6 more Patients with peripartum cardiomyopathy (evaluation): ensure timely diagnosis of peripartum cardiomyopathy to prevent delay of treatment and potential related complications. B Show 5 more Patients with peripartum cardiomyopathy (management): manage patients with peripartum cardiomyopathy by a multidisciplinary team including cardiologists, high-risk obstetricians, and perinatologists, as well as possibly cardiac anesthesiologists, cardiac intensivists, and pediatricians. B Show 13 more Patients with cardiac sarcoidosis: obtain echocardiography to assess LVEF in patients with signs and symptoms of HF. B Show 8 more Patients with acute myocarditis: obtain a 12-lead ECG, TTE, and cardiac troponins in all patients with clinically suspected myocarditis. B Show 7 more Patients with eosinophilic myocarditis: consider performing an endomyocardial biopsy in patients with suspected eosinophilic myocarditis. C Patients with HIV-associated cardiomyopathy: screen for human immunodeficiency virus as a possible cause of dilated cardiomyopathy in patients with dilated cardiomyopathy and risk factors for human immunodeficiency virus. B Show 2 more Patients with Chagas cardiomyopathy: As per ESC 2022 guidelines, consider initiating amiodarone to reduce the arrhythmia burden in patients with Chagas cardiomyopathy presenting with symptomatic premature ventricular complexes or VT. C Show 2 more As per AHA 2016 guidelines, screen for Chagas disease in patients with dilated cardiomyopathy and epidemiological risk factors for Chagas disease, such as origin from Central or South America or exposure to infected vectors. B Patients with autoimmune cardiomyopathy: consider obtaining cardiac biomarkers such as natriuretic peptides (BNP, N-terminal pro-BNP) and cardiac troponin to identify patients with the autoimmune disease with or at risk for HF. C Show 11 more Patients with obesity cardiomyopathy: obtain standard guideline-directed workup for assessment of the pathogenesis and prognosis in patients with HF and obesity. B Show 4 more Patients with growth hormone cardiomyopathy: https://web.pathway.md/diseases/rec8M1y7BZO2OaSC6 6/10 6/29/23, 10:18 PM Dilated cardiomyopathy Pathway Test for acromegaly or GH deficiency in patients with dilated cardiomyopathy with other signs and symptoms of those clinical disorders. B Treat the primary disorder of excess or deficiency of GH in all patients with coexisting dilated cardiomyopathy. B Patients with tachycardia-induced cardiomyopathy: ensure maintenance of sinus rhythm or control of ventricular in treating patients with tachycardia-induced cardiomyopathy. B Patients with LBBB-induced cardiomyopathy: consider offering cardiac resynchronization therapy in patients with suspected cardiomyopathy caused by LBBB. C Patients with PVC-induced cardiomyopathy (evaluation): Suspect premature ventricular complex-induced cardiomyopathy in patients with unexplained reduced ejection fraction and premature ventricular complex burden of at least 10%. B Consider obtaining cardiac MRI in patients with suspected premature ventricular complex- induced cardiomyopathy. C Patients with PVC-induced cardiomyopathy, management: As per ESC 2022 guidelines, perform catheter ablation in patients with cardiomyopathy suspected to be caused by frequent and predominately monomorphic premature ventricular complexes. B Show 3 more As per HRS/ACC/AHA 2018 guidelines, consider performing catheter ablation in patients with recurrent episodes of idiopathic VF initiated by premature ventricular complexes with a consistent QRS morphology. B Show 2 more As per AHA 2016 guidelines, consider offering radiofrequency ablation in patients with premature ventricular contraction-induced cardiomyopathy. C Patients with iron-overload cardiomyopathy: Screen for iron overload including serum ferritin and transferrin saturation when evaluating a new case of cardiomyopathy. B Obtain cardiac MRI in the setting of transferrin saturation > 45% or ferritin > 250 g/L (males) or > 200 g/L (females). B Patients with storage disease cardiomyopathy: initiate enzyme replacement therapy in patients with Pompe or Fabry lysosomal storage disorder. B Patients with thyroid disorders: Obtain thyroid function tests as part of the initial workup in all patients with dilated cardiomyopathy. B Treat hypothyroidism and hyperthyroidism. Consider initiating -blockers in patients with cardiac-related symptoms and signs of hyperthyroidism. B Patients with ischemic stroke: Initiate warfarin for at least 3 months to reduce the risk of recurrent stroke or TIA in patients with ischemic stroke or TIA and LA or LA appendage thrombus in the setting of ischemic or non-ischemic cardiomyopathy and LV dysfunction. B Individualize the decision between anticoagulation and antiplatelet therapy in patients with ischemic stroke or TIA in sinus rhythm with ischemic or non-ischemic cardiomyopathy and https://web.pathway.md/diseases/rec8M1y7BZO2OaSC6 7/10 6/29/23, 10:18 PM Dilated cardiomyopathy Pathway reduced ejection fraction without evidence of LA or LV thrombus. B Clinical findings Symptoms Past medical history Abdominal discomfort AUD Dyspnea Acromegaly Exercise intolerance Amyloidosis Fatigue Becker muscular dystrophy Loss of appetite Chagas disease Nausea Congenital heart disease Orthopnea Cushing's syndrome Palpitations Dermatomyositis Syncope Diabetes mellitus Weight loss Duchenne muscular dystrophy Eosinophilic granulomatosis with polyangiitis Vital signs Exposure to chemotherapy Tachycardia Granulomatosis with polyangiitis systolic BP HF Hyperthyroidism Cardiac exam Hypothyroidism Gallop rhythm Kawasaki disease MR murmur Myocarditis Pulsus alternans Myotonic dystrophy Obesity Integument exam Polyarteritis nodosa Pallor SLE Sarcoidosis ECG findings Scleroderma Substance use disorder AV block VHD LBBB Ventricular arrhythmia Vascular exam Genetic testing Narrow pulse pressure Peripheral edema Lmna gene mutation jugular venous pressure Myh7 gene mutation Scn5a gene mutation Abdominal exam Ttn gene mutation Ascites Hepatomegaly Serological findings anti-heart antibodies https://web.pathway.md/diseases/rec8M1y7BZO2OaSC6 8/10 6/29/23, 10:18 PM Dilated cardiomyopathy Pathway Imaging findings Studies 2022 PAUSE-SCD In patients with cardiomyopathy and monomorphic VT with an indication for ICD implantation, ablation therapy was superior to conventional therapy with respect to the composite outcome of VT recurrence, cardiovascular hospitalization or death. Roderick Tung et al. Circulation. 2022 Jun 21. 2022 PARTITA In patients with dilated cardiomyopathy who received implantable cardioverter defibrillator shock for VT, ablation was superior to standard therapy with respect to the composite outcome of death from any cause or hospitalization for worsening HF. Paolo Della Bella et al. Circulation. 2022 Jun 21. Show 7 more References 1. Biykem Bozkurt, Monica Colvin, Jennifer Cook et al. Current Diagnostic and Treatment Strategies for Specific Dilated Cardiomyopathies: A Scientific Statement From the American Heart Association. Circulation. 2016 Dec 6;134 23):e579-e646. Open 2. Sana M Al-Khatib, William G Stevenson, Michael J Ackerman et al. 2017 AHA/ACC/HRS Guideline for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society. Circulation. 2018 Sep 25;138 13):e272-e391. Open 3. Paul A Heidenreich, Biykem Bozkurt, David Aguilar et al. 2022 AHA/ACC/HFSA Guideline for the Management of Heart Failure: A Report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines. Circulation. 2022 Apr 1;101161CIR0000000000001063. Open 4. Katja Zeppenfeld, Jacob Tfelt-Hansen, Marta de Riva et al. 2022 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death. Eur Heart J. 2022 Aug 26;ehac262. Open 5. Michael J Ackerman, Silvia G Priori, Stephan Willems et al. HRS/EHRA expert consensus statement on the state of genetic testing for the channelopathies and cardiomyopathies this document was developed as a partnership between the Heart Rhythm Society HRS and the European Heart Rhythm Association EHRA . Heart Rhythm. 2011 Aug;8 8 1308 39. Open 6. Theresa A McDonagh, Marco Metra, Marianna Adamo et al. 2021 ESC Guidelines for the diagnosis and treatment of acute and chronic heart failure. Eur Heart J. 2021 Sep 21;42 36 3599 3726. Open https://web.pathway.md/diseases/rec8M1y7BZO2OaSC6 9/10 6/29/23, 10:18 PM Dilated cardiomyopathy Pathway 7. Dawn O Kleindorfer, Amytis Towfighi, Seemant Chaturvedi et al. 2021 Guideline for the Prevention of Stroke in Patients With Stroke and Transient Ischemic Attack: A Guideline From the American Heart Association/American Stroke Association. Stroke. 2021 Jul;52 7):e364-e467. Open 8. Barry J Maron, James E Udelson, Robert O Bonow et al. Eligibility and Disqualification Recommendations for Competitive Athletes With Cardiovascular Abnormalities: Task Force 3 Hypertrophic Cardiomyopathy, Arrhythmogenic Right Ventricular Cardiomyopathy and Other Cardiomyopathies, and Myocarditis: A Scientific Statement From the American Heart Association and American College of Cardiology. Circulation. 2015 Dec 1;132 22):e273 80. Open 9. Anand Padmanabhan, Laura Connelly-Smith, Nicole Aqui et al. Guidelines on the Use of Therapeutic Apheresis in Clinical Practice Evidence-Based Approach from the Writing Committee of the American Society for Apheresis: The Eighth Special Issue. J Clin Apher. 2019 Jun;34 3 171 354. Open 10. Expert Panel on Cardiac Imaging, Prabhakar Rajiah, Jacobo Kirsch et al. ACR Appropriateness Criteria Nonischemic Myocardial Disease with Clinical Manifestations Ischemic Cardiomyopathy Already Excluded). J Am Coll Radiol. 2021 May;18 5S S83 S105. Open 11. Yigal M Pinto, Perry M Elliott, Eloisa Arbustini et al. Proposal for a revised definition of dilated cardiomyopathy, hypokinetic non-dilated cardiomyopathy, and its implications for clinical practice: a position statement of the ESC working group on myocardial and pericardial diseases. Eur Heart J. 2016 Jun 14;37 23 1850 8. Open https://web.pathway.md/diseases/rec8M1y7BZO2OaSC6 10/10 |
Guideline sources The following summarized guidelines for the evaluation and management of disseminated intravascular coagulation are prepared by our editorial team based on guidelines from the International Society on Thrombosis and Haemostasis (ISTH 2015; 2013), the Italian Society for Haemostasis and Thrombosis (SISET 2012), and the British Committee for Standards In Haematology (BCSH 2009). 1 2 3 4 5 5 6 7 8 Definition DIC is a clinicopathologic syndrome characterized by systemic activation of widespread coagulation resulting in severe bleeding and organ failure. 5 Epidemiology DIC is mostly caused by hypercoagulation (infection, particularly sepsis) and hyperfibrinolysis (acute promyelocytic leukemia, obstetric diseases, or aortic aneurysms). 6 Pathophysiology The incidence of DIC in the United States is estimated at 18.6 cases per 100,000 person-years. 5 i https://web.pathway.md/diseases/recGyBhLwbD48Ylbm 1/7 6/29/23, 10:19 PM Disseminated intravascular coagulation Pathway Disease course Clinical manifestations include latent and compensated activation of coagulation (no obvious clinical symptoms) and overt DIC (microvascular thrombosis; multi-organ dysfunction includes renal insufficiency, respiratory failure, hypotension, circulatory failure, or impaired cerebral function; macrovascular thrombi results in venous or arterial obstruction and embolization) Sustained thrombin generation results in severe bleeding and increased risk of death. 7 Prognosis and risk of recurrence The in-hospital mortality of DIC in critically ill patients is 45%. 8 Calculator ISTH criteria criteria for dissemi Guidelines 1. Screening and diagnosis Diagnosis: As per ISTH 2013 guidelines, recognize that there is no gold standard for the diagnosis of DIC and a single test that is, by itself, capable of accurately diagnosing DIC. As per BCSH 2009 guidelines, diagnose DIC based on both clinical and laboratory evaluation. B 2. Classification and risk stratification Scoring systems: As per ISTH 2013 guidelines, use a scoring system for the diagnosis of DIC, recognizing that the ISTH score correlates with key clinical observations and outcomes. B As per SISET 2012 guidelines, consider using any of the following scoring systems to make the diagnosis in patients with suspected DIC: the ISTH score C the JMHW score C the JAAM score. C Show 2 more As per BCSH 2009 guidelines, use the ISTH DIC scoring system to obtain objective measurement of DIC. B 3. Diagnostic investigations https://web.pathway.md/diseases/recGyBhLwbD48Ylbm 2/7 6/29/23, 10:19 PM Disseminated intravascular coagulation Pathway Laboratory tests: Avoid using stand-alone laboratory tests in patients with suspected DIC. D Consider obtaining D-dimer as a fibrin-related marker considering an increase up to 10 times the ULN as moderate and an increase above this threshold as strong. C 4. Medical management General principles: as per ISTH 2013 guidelines, treat the underlying condition as the cornerstone of treatment of DIC. B Prothrombin complex concentrate: As per ISTH 2013 guidelines, consider administering PCC in actively bleeding patients if FFP transfusion is not possible. As per BCSH 2009 guidelines, consider administering factor concentrates, such as PCC, if transfusion of FFP is not possible in patients with bleeding because of fluid overload, recognizing that these will only partially correct the defect because they contain only selected factors, whereas in DIC there is a global deficiency of coagulation factors. C Fibrinogen replacement: As per ISTH 2013 guidelines, consider administering fibrinogen concentrate or cryoprecipitate in actively bleeding patients with persisting severe hypofibrinogenemia (< 1.5 g/L) despite FFP replacement. C As per BCSH 2009 guidelines, consider administering fibrinogen concentrate or cryoprecipitate in patients with severe hypofibrinogenemia (< 1 g/L) persisting despite FFP replacement. C Recombinant factor VIIa: avoid using routine recombinant activated factor VII in bleeding patients with solid tumors or obstetric complications and DIC. D Activated protein C: As per ISTH 2013 guidelines, insufficient evidence to support the use of activated protein C in patients with DIC. Consider using on a case-by-case basis. I As per SISET 2012 guidelines, consider administering recombinant human activated protein C in patients with severe sepsis/septic shock, APACHE II score > 25 (EMEA: at least 2 organs compromised) and DIC. C Show 2 more As per BCSH 2009 guidelines, consider administering recombinant human-activated protein C (continuous infusion, 24 g/kg/hour for 4 days) in patients with severe sepsis and DIC. B Show 2 more Intravenous heparin: As per ISTH 2013 guidelines, consider administering therapeutic doses of heparin in patients with DIC where thrombosis predominates. Prefer LMWH over UFH. C As per BCSH 2009 guidelines: Consider administering therapeutic doses of heparin in patients with DIC with predominating thrombosis, such as arterial or VTE, severe purpura fulminans associated with acral ischemia or vascular skin infarction. https://web.pathway.md/diseases/recGyBhLwbD48Ylbm 3/7 6/29/23, 10:19 PM Disseminated intravascular coagulation Pathway Consider administering continuous infusion UFH in patients with perceived coexisting high risk of bleeding. Consider administering weight adjusted doses (such as 10 g/kg/hour) without the intention of prolonging the activated PTT ratio 1.5-2.5 times the control. Observe clinical signs of bleeding because monitoring the activated PTT in these patients may be complicated. C Antithrombin concentrate: As per SISET 2012 guidelines, avoid using thrombomodulin in patients with hematological cancer or infection and DIC. D As per BCSH 2009 guidelines, do not administer antithrombin in patients with DIC not receiving heparin. D Recombinant thrombomodulin: As per ISTH 2013 guidelines, insufficient evidence to support the use of recombinant human thrombomodulin in patients with DIC. Consider using on a case-by-case basis. I As per SISET 2012 guidelines, avoid using antithrombin in patients with DIC secondary to severe sepsis/septic shock, obstetric complications, burn injury or advanced liver disease. D Dermatan sulfate and gabexate: Avoid using dermatan sulfate in patients with DIC and hematological malignancy. D Avoid using in patients with DIC and sepsis, malignancy, or in patients undergoing surgery. D Antifibrinolytic agents: As per ISTH 2013 guidelines: Avoid using antifibrinolytic agents in patients with DIC. D Consider administering antifibrinolytic agents in patients with DIC presenting with severe bleeding characterized by a marked hyperfibrinolytic state such as leukemia C or trauma. C As per BCSH 2009 guidelines, consider administering lysine analogs, such as tranexamic acid (for example 1 g every 8 hours), in patients with DIC characterized by a primary hyperfibrinolytic state and presenting with severe bleeding. C 5. Inpatient care Thromboprophylaxis: As per ISTH 2013 guidelines, administer prophylaxis for VTE with prophylactic doses of UFH B or LMWH in critically ill, non-bleeding patients with DIC, recognizing that there is no direct evidence of the effects of anticoagulants on DIC. A As per SISET 2012 guidelines, avoid administering UFH or LMWH in patients with DIC except for thromboembolic prophylaxis in patients at high risk not having active bleeding. D 6. Therapeutic procedures Indications for transfusion therapy: https://web.pathway.md/diseases/recGyBhLwbD48Ylbm 4/7 6/29/23, 10:19 PM Disseminated intravascular coagulation Pathway Consider performing transfusion (platelets, plasma, cryoprecipitate) in patients with DIC and active bleeding. C Avoid performing transfusion solely based on laboratory parameters in patients with chronic DIC or without active bleeding. D Platelet transfusion: As per ISTH 2013 guidelines, administer platelets in patients with DIC with active bleeding and a platelet count of < 50 10 /L or in patients at high risk of bleeding and a platelet count of < 20 10 /L. B As per BCSH 2009 guidelines, do not rely primarily on laboratory results to administer platelets or plasma (components) in patients with DIC. Reserve it for patients presenting with bleeding. D Show 2 more Fresh frozen plasma transfusion: As per ISTH 2013 guidelines, consider administering FFP in patients with active bleeding with either prolonged PT/activated PTT (> 1.5 times normal) or decreased fibrinogen (< 1.5 g/dL). Consider administering FFP in patients with DIC requiring an invasive procedure with similar laboratory abnormalities. C As per BCSH 2009 guidelines, consider administering FFP in bleeding patients with DIC and prolonged PT and activated PTT. C Show 2 more Plasmapheresis: avoid performing plasma exchange in patients with sepsis and DIC. D 7. Specific circumstances Patients with malignancy: consider administering platelets to maintain a platelet count > 50 10 /L in patients with DIC and active bleeding. C Show 3 more 8. Follow-up and surveillance Serial laboratory assessment: as per ISTH 2013 guidelines, obtain repeated tests to monitor the dynamic changes based on laboratory results and clinical observations. B Clinical findings Symptoms Past medical history Bleeding Acute pancreatitis Petechia Acute promyelocytic leukemia Liver cirrhosis Past obstetric history Malignancy Pancreatic cancer Pregnancy Sepsis https://web.pathway.md/diseases/recGyBhLwbD48Ylbm 5/7 6/29/23, 10:19 PM Disseminated intravascular coagulation Pathway Traumatic injury Integument exam Vascular exam Erythematous macules Eschar Limb ischemia Gangrene Geographic plaques Lab findings Hemorrhagic bullae ALF Mucosal bleeding Prolonged PTT Purpura Prolonged serum PT Purpura fulminans serum D-dimer Hematological findings serum fibrinogen Schistocytes Imaging findings blood platelet count Intracranial hemorrhage References 1. H Wada, J Thachil, M Di Nisio et al. Guidance for diagnosis and treatment of DIC from harmonization of the recommendations from three guidelines. J Thromb Haemost. 2013 Feb 4. Open 2. Marcello Di Nisio, Francesco Baudo, Benilde Cosmi et al. Diagnosis and treatment of disseminated intravascular coagulation: guidelines of the Italian Society for Haemostasis and Thrombosis SISET . Thromb Res. 2012 May;129 5):e177 84. Open 3. M Levi, C H Toh, J Thachil et al. Guidelines for the diagnosis and management of disseminated intravascular coagulation. British Committee for Standards in Haematology. Br J Haematol. 2009 Apr;145 1 24 33. Open 4. J Thachil, A Falanga, M Levi et al. Management of cancer-associated disseminated intravascular coagulation: guidance from the SSC of the ISTH. J Thromb Haemost. 2015 Apr;13 4 671 5. Open 5. Balwinder Singh, Andrew C Hanson, Rabe Alhurani et al. Trends in the incidence and outcomes of disseminated intravascular coagulation in critically ill patients 2004 2010 a population-based study. 2013 May;143 5 1235 1242.2013 May;143 5 1235 1242. Open 6. Hideo Wada, Takeshi Matsumoto, Yoshiki Yamashita. Diagnosis and treatment of disseminated intravascular coagulation DIC according to four DIC guidelines. 2014 Feb 20;2 1 15.2014 Feb 20;2 1 15. Open 7. Chrysoula Papageorgiou, Georges Jourdi, Eusebe Adjambri et al. Disseminated Intravascular Coagulation: An Update on Pathogenesis, Diagnosis, and Therapeutic Strategies. 2018 Dec;24 9_suppl):8S 28S.2018 Dec;24 9_suppl):8S 28S. Open 8. Yukiko Kimura, Jennifer E Weiss, Kathryn L Haroldson et al. Pulmonary hypertension and other potentially fatal pulmonary complications in systemic juvenile idiopathic arthritis. 2013 May;65 5 745 52.2013 May;65 5 745 52. Open 9. Bick RL. Disseminated intravascular coagulation: objective clinical and laboratory diagnosis, treatment, and assessment of therapeutic response. Semin Thromb Hemost. 1996;22 1 69 88. Open 10. Bakhtiari K, Meijers JC, de Jonge E et al. Prospective validation of the International Society of Thrombosis and Haemostasis scoring system for disseminated intravascular coagulation. Crit Care https://web.pathway.md/diseases/recGyBhLwbD48Ylbm 6/7 6/29/23, 10:19 PM Disseminated intravascular coagulation Pathway Med. 2004 Dec;32 12 2416 21. Open 11. Levi M, de Jonge E, van der Poll T et al. Disseminated intravascular coagulation. Thromb Haemost. 1999 Aug;82 2 695 705. Open 12. Balwinder Singh, Andrew C Hanson, Rabe Alhurani et al. Trends in the incidence and outcomes of disseminated intravascular coagulation in critically ill patients 2004 2010 a population-based study. Chest. 2013 May;143 5 1235 1242. Open 13. Chrysoula Papageorgiou, Georges Jourdi, Eusebe Adjambri et al. Disseminated Intravascular Coagulation: An Update on Pathogenesis, Diagnosis, and Therapeutic Strategies. Clin Appl Thromb Hemost. 2018 Dec;24 9_suppl):8S 28S. Open 14. Hideo Wada, Takeshi Matsumoto, Yoshiki Yamashita. Diagnosis and treatment of disseminated intravascular coagulation DIC according to four DIC guidelines. J Intensive Care. 2014 Feb 20;2 1 15. Open 15. Hideo Wada, Esteban C Gabazza, Hidesaku Asakura et al. Comparison of diagnostic criteria for disseminated intravascular coagulation DIC diagnostic criteria of the International Society of Thrombosis and Hemostasis and of the Japanese Ministry of Health and Welfare for overt DIC. Am J Hematol. 2003 Sep;74 1 17 22. Open https://web.pathway.md/diseases/recGyBhLwbD48Ylbm 7/7 |
Guideline sources The following summarized guidelines for the evaluation and management of distal radius fracture (DRF) are prepared by our editorial team based on guidelines from the Eastern Association for the Surgery of Trauma (EAST/AOTA 2023), the American Academy of Orthopaedic Surgeons (AAOS 2020; 2011), the American College of Radiology (ACR 2019), the British Society for Surgery of the Hand (BSSH/BOA 2018), and the Danish Health Authority (DHA 2016). 1 2 3 4 5 6 Guidelines 1. Diagnostic investigations Diagnostic imaging: As per ACR 2019 guidelines, obtain wrist X-ray as the initial imaging in adult patients with acute blunt or penetrating trauma to the hand or wrist. B Show 6 more Updated evidence: BUCKLED https://web.pathway.md/diseases/recpJq53PXwzoccK2 1/6 6/29/23, 10:19 PM Distal radius fracture Pathway In participants 5-15 years of age who presented to the emergency department with an isolated distal forearm injury, without a clinically visible deformity, in whom further evaluation with imaging was indicated, ultrasonography was noninferior to radiography with respect to PROMIS System score at 4 weeks. Peter J Snelling et al. N Engl J Med. 2023 Jun 1. As per BSSH 2018 guidelines, recognize that there is no demonstrated clear association between any measured radiological parameter and patient-rated outcome. B As per DHA 2016 guidelines: Do not obtain routine preoperative CT. D Consider obtaining supplementary CT if conventional X-ray creates doubt as regards the choice of treatment method. E As per AAOS 2011 guidelines, consider obtaining CT to improve diagnostic accuracy in patients with intra-articular DRFs. C 2. Diagnostic procedures Diagnostic arthroscopy: consider performing arthroscopy to improve diagnostic accuracy for wrist ligament injuries in patients with distal radius intra-articular fractures. C 3. Medical management Pain management: As per EAST 2023 guidelines: Consider administering NSAIDs (such as ketorolac) for pain management in adult patients with a traumatic fracture. C Insufficient evidence to recommend for the preference of selective NSAIDs (COX-2 inhibitors) or nonselective NSAIDs. I As per AAOS 2020 guidelines, consider initiating opioid-sparing and multimodal pain management strategies in patients undergoing treatment for DRFs. E 4. Nonpharmacologic interventions Casting: Insufficient evidence to recommend for or against casting as definitive treatment for unstable fractures that are initially adequately reduced. I Insufficient evidence to recommend for or against immobilization of the elbow in patients treated with cast immobilization. I Splinting: Consider preferring rigid immobilization over removable splints when using a nonoperative treatment for the management of displaced DRFs. C https://web.pathway.md/diseases/recpJq53PXwzoccK2 2/6 6/29/23, 10:19 PM Distal radius fracture Pathway Consider using removable splints in the treatment of patients with minimally displaced DRFs. C Exercise programs: As per AAOS 2020 guidelines, recognize that there is no difference in outcomes between a home exercise program and supervised therapy following treatment of DRFs. B As per BSSH 2018 guidelines, consider advising early mobilization in patients with a stable DRF, with a removable support once pain allows. C As per DHA 2016 guidelines: Do not offer routine rehabilitation supervised by an occupational therapist or a physiotherapist in uncomplicated cases. D Provide guidance and practical instruction concerning self-rehabilitation following distal radial fracture in all patients regardless of the treatment method. E As per AAOS 2011 guidelines, consider offering a home exercise program in patients after DRF. C Show 2 more Adjuvant therapies: As per BSSH 2018 guidelines, do not use vitamin C for the prevention of complex regional pain syndrome in patients with DRFs. D As per AAOS 2011 guidelines: Consider offering ultrasound and/or ice as adjuvant therapy in patients with DRFs. C Consider offering vitamin C as adjuvant therapy for the prevention of disproportionate pain in patients with DRFs. C 5. Therapeutic procedures Closed reduction: Avoid performing manipulation in 65 years old patients with moderately displaced DRFs. D Consider offering nonoperative treatment as a primary treatment for displaced DRF in 65 years old patients. Take other factors, such as pre-injury function, medical comorbidities, and fracture characteristics, into consideration and discuss options with the patient. B 6. Surgical interventions Indications for surgical reduction and fixation: As per AAOS 2020 guidelines: Consider performing surgery to improve radiographic and patient-reported outcomes in non-geriatric patients (< 65 years of age) with fractures with post-reduction radial shortening > 3 mm, dorsal tilt > 10 degrees, or intra-articular displacement or step off > 2 mm. C Recognize that operative treatment compared to nonoperative treatment does not lead to improved long-term patient-reported outcomes in geriatric patients ( 65 years of age). A https://web.pathway.md/diseases/recpJq53PXwzoccK2 3/6 6/29/23, 10:19 PM Distal radius fracture Pathway As per DHA 2016 guidelines, perform surgical treatment of a distal radial fracture in patients of any age if a conventional wrist X-ray examination, following eventual reduction of the fracture, reveals 1 of the following radiological parameters: > 10 degrees of dorsal angulation of the articular surface of the radial in a side view as compared to perpendicular to the longitudinal axis of the radial > 2 mm ulnar variance > 2 mm articular step-off incongruity of the distal radioulnar joint. E Show 3 more As per AAOS 2011 guidelines: Consider performing operative fixation in patients with DRFs with post-reduction radial shortening > 3 mm, dorsal tilt > 10 degrees, or intra-articular displacement or step-off > 2 mm as opposed to cast fixation. C Insufficient evidence to recommend for or against operative management in > 55 years old patients with DRFs. I Anesthesia: consider administering IV regional anesthesia (Bier's block), by a healthcare professional trained in the technique (not necessarily an anesthetist), when reducing dorsally displaced DRFs in adult patients in the emergency department. C Show 2 more Technical considerations for surgery: As per AAOS 2020 guidelines, recognize that there is no significant difference in radiographic or patient-reported outcomes between fixation techniques for complete articular or unstable DRFs, although volar locking plates lead to earlier recovery of function in the short term (3 months). A As per BSSH 2018 guidelines, perform K-wire fixation and casting when surgery is required for dorsally displaced DRFs that can be reduced closed. A Show 2 more Updated evidence: DRAFFT2 In adult patients with a dorsally-displaced distal radius fracture that needed manipulation, K-wire fixation was not superior to cast with respect to Patient Rated Wrist Evaluation score at 12 months. Matthew L Costa et al. BMJ. 2022 Jan 19. As per DHA 2016 guidelines, perform open reduction and internal fixation with a volar angular stable locking plate in patients of any age, if surgery is indicated. Consider performing K-wire osteosynthesis as the primary choice rather than bridging external fixation, if open reduction and internal fixation cannot be performed. E Show 6 more As per AAOS 2011 guidelines, insufficient evidence to recommend for or against: any specific operative method for fixation of DRFs https://web.pathway.md/diseases/recpJq53PXwzoccK2 4/6 6/29/23, 10:19 PM Distal radius fracture Pathway locking plates in > 55 years old patients supplemental bone grafts or substitutes when using locking plates bone graft (autograft or allograft) or bone graft substitutes for the filling of a bone void as an adjunct to other operative treatments concurrent surgical treatment of distal radioulnar joint instability fixation of ulnar styloid fractures associated with DRFs external fixation alone if there is depressed lunate fossa or 4-part fracture (sagittal split). I Show 4 more Arthroscopic assistance: Recognize that there is no difference in outcomes between use of arthroscopic assistance and no arthroscopic assistance in the treatment of patients with DRFs. B Consider obtaining arthroscopic evaluation of the articular surface during operative treatment of patients with intra-articular DRFs. C Nerve decompression: insufficient evidence to recommend for or against performing nerve decompression when nerve dysfunction persists after reduction. I 7. Follow-up and surveillance Follow-up: As per AAOS 2020 guidelines, recognize that there is no difference in outcomes based on frequency of radiographic evaluation in patients treated for DRFs. B As per BSSH 2018 guidelines, consider obtaining careful follow-up after reduction in patients with DRFs displaced on the initial films, as they are at increased risk of subsequent re- displacement. C Show 2 more As per AAOS 2011 guidelines, consider obtaining a post-reduction true lateral X-ray of the carpus to assess distal radioulnar joint alignment in all patients with DRFs. C Show 2 more Clinical findings Symptoms Past medical history Wrist pain Osteoporosis Wrist swelling Social history Past events Sports participation Falls Motor vehicle accident Musculoskeletal exam https://web.pathway.md/diseases/recpJq53PXwzoccK2 5/6 6/29/23, 10:19 PM Distal radius fracture Pathway Wrist deformity Wrist tenderness Studies 2023 BUCKLED In participants 5-15 years of age who presented to the emergency department with an isolated distal forearm injury, without a clinically visible deformity, in whom further evaluation with imaging was indicated, ultrasonography was noninferior to radiography with respect to PROMIS System score at 4 weeks. Peter J Snelling et al. N Engl J Med. 2023 Jun 1. 2022 DRAFFT2 In adult patients with a dorsally-displaced distal radius fracture that needed manipulation, K-wire fixation was not superior to cast with respect to Patient Rated Wrist Evaluation score at 12 months. Matthew L Costa et al. BMJ. 2022 Jan 19. References 1. American Academy of Orthopaedic Surgeons. Management of Distal Radius Fractures: Evidence- Based Clinical Practice Guideline. AAOS. 2020 Dec 5. Open 2. Charles Pailthorpe, Joe Dias, Adam Brooks et al. Best practice for management of Distal Radial Fractures DRFs). BSSH. 2018. Open 3. David M Lichtman, Randipsingh R Bindra, Martin I Boyer et al. American Academy of Orthopaedic Surgeons clinical practice guideline on: the treatment of distal radius fractures. J Bone Joint Surg Am. 2011 Apr 20;93 8 775 8. Open 4. Danish Health Authority. National Clinical Guidelines on the Treatment of Distal Radial Fractures. Sundhedsstyrelsen. 2016 Jan. Open 5. Patrick B Murphy, George Kasotakis, Elliott R Haut et al. Efficacy and safety of non-steroidal anti- inflammatory drugs NSAIDs) for the treatment of acute pain after orthopedic trauma: a practice management guideline from the Eastern Association for the Surgery of Trauma and the Orthopedic Trauma Association. Trauma Surg Acute Care Open. 2023 Feb 21;8 1):e001056. Open 6. Expert Panel on Musculoskeletal Imaging:, Maha Torabi, Leon Lenchik et al. ACR Appropriateness Criteria Acute Hand and Wrist Trauma. J Am Coll Radiol. 2019 May;16 5S S7 S17. Open https://web.pathway.md/diseases/recpJq53PXwzoccK2 6/6 |
Guideline sources The following summarized guidelines for the evaluation of distal symmetric polyneuropathy are prepared by our editorial team based on guidelines from the American Association of Neuromuscular and Electrodiagnostic Medicine (AANEM/AAN 2009). 1 2 2 2 3 Definition Distal symmetric polyneuropathy is diffuse length-dependent peripheral neuropathy characterized by numbness, tingling, pain, and/or weakness that typically starts in the toes and spreads proximally. 2 Epidemiology Distal symmetric polyneuropathy is most commonly caused due to diabetes (32-53%), idiopathic reasons (24-27%), alcohol, vitamin B 12 deficiency, chemotherapy, CKD, paraproteinemia, and inherited conditions (hammertoes, high arches, Charcot Marie Tooth). Other causes include pre- diabetes, infections, inflammation, toxins, autoimmunity, neoplasms, and paraneoplasms. 2 Disease course Clinical manifestations of distal symmetrical polyneuropathy include numbness, tingling, pain, starting in the toes with proximal spread, decreased pinprick and vibration sensation in a stocking-glove distribution, decreased reflexes starting at the ankles, weakness of toe extension, or trouble walking on heels. Progression may lead to weakness, the involvement of fingertips, balance issues resulting in falls and fractures, risk of ulcerations and amputations. 2 Prognosis and risk of recurrence Lower extremity amputations are associated with early postoperative mortality of up to 22%. 3 https://web.pathway.md/diseases/reclc5w1ab3TVTyjg 1/2 6/29/23, 10:20 PM Distal symmetric polyneuropathy Pathway Guidelines 1. Diagnostic investigations Laboratory assessment: Consider screening laboratory tests (e.g., blood glucose, serum B12 and SPEP) in all patients with polyneuropathy. C Consider testing for impaired glucose tolerance in patients with distal symmetric sensory polyneuropathy in whom there is no evidence of diabetes mellitus. C Genetic testing: obtain genetic testing for the accurate diagnosis and classification of hereditary neuropathies. A Show 2 more Clinical findings Symptoms Past medical history Muscle weakness Diabetes mellitus Numbness of soles of feet Poorly controlled diabetes Pain Lab findings Tingling Hyperglycemia Neurological exam Neuropathy References 1. England JD, Gronseth GS, Franklin G et al. Practice Parameter: evaluation of distal symmetric polyneuropathy: role of laboratory and genetic testing (an evidence-based review). Report of the American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, and American Academy of Physical Medicine and Rehabilitation. Neurology. 2009 Jan 13;72 2 185 92. Open 2. Brian C. Callaghan, Raymond S. Price, and Eva L. Feldman. Diagnostic and Therapeutic Advances: Distal Symmetric Polyneuropathy. JAMA. 2015 Nov 24; 314 20 2172 2181. Open 3. Ayeshmanthe Rathnayake, Apoorva Saboo, Usman H Malabu et al. Lower extremity amputations and long-term outcomes in diabetic foot ulcers: A systematic review. 2020 Sep 15;11 9 391 399.2020 Sep 15;11 9 391 399. Open 4. Ayeshmanthe Rathnayake, Apoorva Saboo, Usman H Malabu et al. Lower extremity amputations and long-term outcomes in diabetic foot ulcers: A systematic review. World J Diabetes. 2020 Sep 15; 11 9 391 399. Open https://web.pathway.md/diseases/reclc5w1ab3TVTyjg 2/2 |
Guideline sources The following summarized guidelines for the evaluation and management of dizziness and vertigo are prepared by our editorial team based on guidelines from the Society for Academic Emergency Medicine (SAEM 2023), the European Society for Vascular Surgery (ESVS 2023), the American Physical Therapy Association (APTA 2022), the United States Department of Defense (DoD/VA 2021; 2016), the American College of Radiology (ACR 2018), the Living Concussion Guidelines (LCG 2017), the American Academy of Otolaryngology-Head and Neck Surgery Foundation (AAO-HNSF 2017), and the American Academy of Neurology (AAN 2017). 1 2 3 4 5 6 7 8 9 Calculator Calculator Calculat HINTS examination for stroke in ICVD criteria for M ni re's disea Recogn Guidelines 1. Diagnostic investigations Oculomotor assessment: https://web.pathway.md/diseases/recfF9yX6FwsgXs5p 1/6 6/29/23, 10:20 PM Dizziness and vertigo Pathway Obtain routine three-component head impulse-nystagmus-test of skew examination by clinicians trained in its use to distinguish between central (stroke) and peripheral (inner ear, usually vestibular neuritis) diagnoses in adult patients with acute vestibular syndrome with nystagmus presenting to the emergency department. A Consider eliciting a detailed history and performing a physical examination with emphasis on cranial nerves, including visual fields, eye movements, limb coordination, and gait assessment, to help distinguish between central (TIA) and peripheral (vestibular migraine, M ni re's disease) diagnoses in adult patients with spontaneous episodic vestibular syndrome presenting to the emergency department. C Hearing assessment: consider obtaining hearing assessment at the bedside by finger rub to identify new unilateral hearing loss as an additional criterion to aid in the identification of stroke, even if head impulse-nystagmus-test of skew examination result suggests a peripheral vestibular diagnosis in adult patients with acute vestibular syndrome with nystagmus. C Gait assessment: Consider eliciting a detailed history and performing a physical examination with emphasis on cranial nerves, including visual fields, eye movements, limb coordination, and gait assessment, to distinguish between central (TIA) and peripheral (vestibular migraine, M ni re's disease) diagnoses in adult patients with spontaneous episodic vestibular syndrome presenting to the emergency department. C Consider obtaining a severity assessment of gait unsteadiness to help distinguish between central (stroke) and peripheral (inner ear, usually vestibular neuritis) diagnoses in adult patients with acute vestibular syndrome without nystagmus presenting to the emergency department. C Dix-Hallpike maneuver: As per SAEM 2023 guidelines, perform the Dix-Hallpike maneuver for the diagnosis of posterior canal-benign paroxysmal positional vertigo in adult patients with transient-episodic vestibular syndrome presenting to the emergency department. B As per AAO-HNSF 2017 guidelines, perform the Dix-Hallpike maneuver (by bringing the patient from an upright to supine position with the head turned 45 degrees to one side and the neck extended 20 degrees with the affected ear down) to elicit provoked vertigo associated with torsional, upbeating nystagmus for the diagnosis of posterior semicircular canal benign paroxysmal positional vertigo. Repeat the maneuver with the opposite ear down if the initial maneuver is negative. B Supine roll test: obtain a supine roll test to assess for lateral semicircular canal benign paroxysmal positional vertigo in patients with a history compatible with benign paroxysmal positional vertigo and horizontal or no nystagmus during the Dix-Hallpike maneuver. B Vestibular function testing: As per AAN 2017 guidelines, avoid obtaining cervical vestibular evoked myogenic potential testing for the diagnosis of benign paroxysmal positional vertigo. D As per AAO-HNSF 2017 guidelines, do not obtain routine vestibular testing in patients meeting diagnostic criteria for benign paroxysmal positional vertigo without additional vestibular signs and/or symptoms warranting testing. D https://web.pathway.md/diseases/recfF9yX6FwsgXs5p 2/6 6/29/23, 10:20 PM Dizziness and vertigo Pathway Diagnostic imaging (acute vestibular syndrome): do not obtain routine non-contrast CT of the brain or CT angiography to help distinguish between central (stroke) and peripheral (inner ear, usually vestibular neuritis) diagnoses in adult patients with acute vestibular syndrome (with or without nystagmus) presenting to the emergency department. D Show 2 more Diagnostic imaging, episodic vestibular syndrome: As per ESVS 2023 guidelines, obtain cervical vascular imaging to identify disruption of blood flow during head turning for the diagnosis of vertebrobasilar ischemia (attributed to nipping of the vertebral arteries on head movement) in patients with vertigo or dizziness on head turning. B As per SAEM 2023 guidelines, do not obtain routine CT to distinguish between central (TIA) and peripheral (vestibular migraine, M ni re's disease) diagnoses in adult patients with spontaneous episodic vestibular syndrome presenting to the emergency department. D Show 2 more As per ACR 2018 guidelines, obtain temporal bone CT or head and internal auditory canal MRI in patients with episodic vertigo with or without associated hearing loss or aural fullness (peripheral vertigo). B Diagnostic imaging (persistent vertigo): obtain head and internal auditory canal MRI in patients with persistent vertigo with or without neurological symptoms (central vertigo). B Diagnostic imaging, BPPV: As per SAEM 2023 guidelines, avoid obtaining routine MRI or MRA in adult patients with transient-episodic vestibular syndrome presenting to the emergency department and diagnosed with typical posterior canal-benign paroxysmal positional vertigo by a positive Dix- Hallpike test with the characteristic nystagmus. D As per AAO-HNSF 2017 guidelines, do not obtain radiographic imaging in patients meeting diagnostic criteria for benign paroxysmal positional vertigo without additional signs and/or symptoms warranting imaging. D 2. Medical management Management of benign paroxysmal positional vertigo: As per SAEM 2023 guidelines, perform the Epley canalith repositioning maneuver at the time of diagnosis in adult patients with posterior canal-benign paroxysmal positional vertigo diagnosed by a positive Dix-Hallpike test. B As per AAO-HNSF 2017 guidelines, perform a canalith repositioning procedure for the treatment of patients with posterior canal benign paroxysmal positional vertigo. Do not advise postprocedural postural restrictions after the canalith repositioning procedure. B Show 6 more Management of vestibular neuritis: consider ensuring shared decision-making with patients to weigh the risks and benefits of short-term corticosteroid treatment in adult patients with a clinical diagnosis of vestibular neuritis presenting within 3 days of symptom onset. C https://web.pathway.md/diseases/recfF9yX6FwsgXs5p 3/6 6/29/23, 10:20 PM Dizziness and vertigo Pathway 3. Specific circumstances Patients with post-concussion vestibular dysfunction, acute mTBI: As per LCG 2017 guidelines, perform the Dix-Hallpike maneuver for assessment of patients with symptoms of benign positional vertigo once the cervical spine has been cleared. E Show 3 more As per DoD/VA 2016 guidelines: Consider offering a short-term trial of specific vestibular, visual, and proprioceptive therapeutic exercises to assess the individual patient's responsiveness to treatment in patients with a history of mild TBI presenting with functional impairments due to dizziness, disequilibrium, and spatial disorientation symptoms. Avoid offering a prolonged course of therapy in the absence of patient improvement. C Refer patients to occupational therapy, physical therapy, or other vestibular-trained care providers as appropriate. B Patients with post-concussion vestibular dysfunction (post-acute mTBI): consider offering a trial of specific vestibular rehabilitation and proprioceptive therapeutic exercise in patients with persistent symptoms of dizziness and imbalance attributed to mild TBI. C 4. Follow-up and surveillance Vestibular rehabilitation: As per APTA 2022 guidelines, offer vestibular physical therapy in patients with acute, subacute, or chronic unilateral vestibular hypofunction. A Show 9 more As per AAO-HNSF 2017 guidelines, consider offering vestibular rehabilitation, either self- administered or administered by a clinician, in the treatment of patients with benign paroxysmal positional vertigo. C 5. Quality improvement Health professional training: ensure that emergency department clinicians receive training in bedside physical examination techniques for acute vestibular syndrome (head impulse- nystagmus-test of skew) and diagnostic and therapeutic maneuvers for benign paroxysmal positional vertigo (Dix-Hallpike test and Epley maneuver). B Clinical findings Symptoms Past medical history Dizziness Benign paroxysmal positional vertigo Fainting Brain tumor Gait disturbance Concussion Hearing loss Labyrinthitis Lightheadedness https://web.pathway.md/diseases/recfF9yX6FwsgXs5p 4/6 6/29/23, 10:20 PM Dizziness and vertigo Pathway Loss of balance Migraine Pre-syncope Multiple sclerosis Vertigo M ni re's disease Orthostatic hypotension Past events Otosclerosis Persistent postural-perceptual dizziness Falls Stroke Neurological exam Superior canal dehiscence syndrome Nystagmus Dix-Hallpike test TBI head impulse-nystagmus-test of skew TIA Vertebral artery disease Vestibular neuronitis Vital signs Orthostatic hypotension References 1. No authors listed. Living Concussion Guidelines: Guideline for Concussion & Prolonged Symptoms for Adults 18 years of Age or Older: 2017 April Update. ConcussionsOntario. 2017 Apr. Open 2. Bhattacharyya N, Gubbels SP, Schwartz SR et al. Clinical Practice Guideline: Benign Paroxysmal Positional Vertigo Update). Otolaryngol Head Neck Surg. 2017 Mar;156 3_suppl):S1 S47. Open 3. Jonathan A Edlow, Christopher Carpenter, Murtaza Akhter et al. Guidelines for reasonable and appropriate care in the emergency department 3 GRACE 3 Acute dizziness and vertigo in the emergency department. Acad Emerg Med. 2023 May;30 5 442 486. Open 4. Courtney D Hall, Susan J Herdman, Susan L Whitney et al. Vestibular Rehabilitation for Peripheral Vestibular Hypofunction: An Updated Clinical Practice Guideline From the Academy of Neurologic Physical Therapy of the American Physical Therapy Association. J Neurol Phys Ther. 2022 Apr 1;46 2 118 177. Open 5. Expert Panel on Neurologic Imaging:, Aseem Sharma, Claudia F E Kirsch et al. ACR Appropriateness Criteria Hearing Loss and/or Vertigo. J Am Coll Radiol. 2018 Nov;15 11S S321 S331. Open 6. Terry D Fife, James G Colebatch, Kevin A Kerber et al. Practice guideline: Cervical and ocular vestibular evoked myogenic potential testing: Report of the Guideline Development, Dissemination, and Implementation Subcommittee of the American Academy of Neurology. Neurology. 2017 Nov 28;89 22 2288 2296. Open 7. Ross Naylor, Barbara Rantner, Stefano Ancetti et al. Editor's Choice European Society for Vascular Surgery ESVS 2023 Clinical Practice Guidelines on the Management of Atherosclerotic Carotid and Vertebral Artery Disease. Eur J Vasc Endovasc Surg. 2023 Jan;65 1 7 111. Open 8. Department of Defense/Veterans Affairs. VA/DoD CLINICAL PRACTICE GUIDELINE FOR THE MANAGEMENT AND REHABILITATION OF POST ACUTE MILD TRAUMATIC BRAIN INJURY. VA/DoD. 2021 Jun. Open 9. Department of Defense/Veterans Affairs. VA/DoD CLINICAL PRACTICE GUIDELINE FOR THE MANAGEMENT OF CONCUSSION MILD TRAUMATIC BRAIN INJURY. VA/DoD. 2016 Feb. Open https://web.pathway.md/diseases/recfF9yX6FwsgXs5p 5/6 6/29/23, 10:20 PM Dizziness and vertigo Pathway https://web.pathway.md/diseases/recfF9yX6FwsgXs5p 6/6 |
Guideline sources The following summarized guidelines for the management of dog and cat bites are prepared by our editorial team based on guidelines from the American Association of Family Physicians (AAFP 2014). 1 Guidelines 1. Medical management Antibiotic prophylaxis: Administer antibiotic prophylaxis for high-risk bite wounds. B Consider administering antibiotic prophylaxis for average-risk wounds. C Post-exposure rabies prophylaxis: provide post-exposure rabies prophylaxis to all patients who were bitten by an animal that was possibly exposed to rabies. B 2. Surgical interventions Wound care: consider closing bite wounds if cosmetically desirable. However, leave wounds open if they are at high risk of infection. C 3. Preventative measures Pre-exposure rabies prophylaxis: consider pre-exposure rabies prophylaxis for persons at higher risk of rabies exposure, and for international travelers to at-risk areas who are unlikely https://web.pathway.md/diseases/recKCbLJh9SfPqnXp 1/2 6/29/23, 10:20 PM Dog and cat bites Pathway to get post-exposure prophylaxis within 24 hours of a possible rabies exposure. C References 1. Robert Ellis, Carrie Ellis. Dog and Cat Bites. Am Fam Physician. 2014 Aug 15;90 4 239 43. Open https://web.pathway.md/diseases/recKCbLJh9SfPqnXp 2/2 |
Guideline sources The following summarized guidelines for the evaluation and management of drug allergy are prepared by our editorial team based on guidelines from the American Academy of Allergy, Asthma & Immunology (AAAAI 2022), the British Society for Allergy and Clinical Immunology (BSACI 2015), and the European Academy of Allergy and Clinical Immunology (EAACI/WAO/iCAALL/AAAAI/ACAAI/ICON 2014). 1 2 3 Guidelines 1. Diagnostic investigations Medical history: elicit a history of drug allergy using specific questioning before issuing a prescription from both a medical and legal viewpoint. B Drug challenge testing: As per AAAAI 2022 guidelines, consider obtaining drug challenge testing with a 1- or 2-step drug challenge when the clinical probability of a drug allergy is low in patients without contraindications for a drug challenge. C Show 2 more As per ICON 2014 guidelines, obtain a drug challenge test as the gold standard to identify the drug eliciting a drug hypersensitivity reaction. B Prefer the oral route whenever possible for drug challenge testing. B Show 2 more Evaluation of penicillin allergy: https://web.pathway.md/diseases/reczar7CgdXY7kEyP 1/4 6/29/23, 10:20 PM Drug allergy Pathway As per AAAAI 2022 guidelines, attempt to de-label patients with reported penicillin allergy, if appropriate. B Show 11 more As per BSACI 2015 guidelines, do not obtain an evaluation in patients with a family history but no personal history of penicillin allergy. D Show 7 more As per ICON 2014 guidelines, consider obtaining retesting 2-4 weeks after the initial evaluation in patients experienced severe immediate reactions to -lactams and displayed negative results at the first evaluation which included a drug challenge test. C Evaluation of sulfonamide allergy: consider obtaining a 1-step drug challenge with TMP- SMX when there is a need to delabel a sulfonamide antibiotic allergy in patients with a history of benign cutaneous reactions (such as morbilliform drug eruption, urticaria) to sulfonamide antibiotics occurred > 5 years ago. C Evaluation of fluoroquinolone allergy: consider obtaining a 1- or 2-step drug challenge without preceding skin testing to confirm tolerance in patients with a history of non- anaphylactic reactions to fluoroquinolones. C Evaluation of macrolide allergy: consider obtaining a 1- or 2-step drug challenge without preceding skin testing to confirm tolerance in patients with a history of non-anaphylactic reactions to macrolides. C Evaluation of aspirin allergy: do not obtain oral aspirin challenge to confirm the diagnosis of aspirin-exacerbated respiratory disease in cases of high diagnostic certainty based on clinical history, recognizing that aspirin desensitization remains a therapeutic option when indicated. D Consider obtaining an oral aspirin challenge to confirm the diagnosis of aspirin-exacerbated respiratory disease in cases of diagnostic uncertainty. D Show 2 more Evaluation of abacavir allergy: obtain HLA-B5701 screening before initiating abacavir to reduce the risk of drug hypersensitivity reactions. A Evaluation of excipient allergy: recognize that excipients are a very rare cause of immediate or delayed reactions associated with drugs. Consider suspecting excipient hypersensitivity in patients with a history of anaphylaxis to 2 structurally unrelated drugs or products sharing a common excipient (such as injectable corticosteroids, polyethylene glycol-based laxatives). B Evaluation of contrast media allergy: consider obtaining skin testing for the diagnosis of immediate drug hypersensitivity reactions to iodinated radiocontrast media. C 2. Medical management Drug desensitization (beta-lactams): consider offering desensitization if there is an absolute requirement for a specific -lactam in the presence of positive skin or challenge tests. C Drug desensitization (aspirin): consider offering desensitization to aspirin as a therapeutic intervention in selected asthmatic patients with aspirin-exacerbated respiratory disease or nasal polyps. C https://web.pathway.md/diseases/reczar7CgdXY7kEyP 2/4 6/29/23, 10:20 PM Drug allergy Pathway Choice of an alternative agent (testing): consider obtaining drug challenge testing in a hospital setting to search for safe alternatives when the alternatives belong to the same drug class. C Choice of an alternative agent (antibiotics): consider administering a penicillin without testing or additional precautions in patients with a history of an unverified non-anaphylactic cephalosporin allergy. C Show 2 more Choice of an alternative agent (analgesics): consider administering a selective COX-2 inhibitor as an alternative analgesic in patients with any NSAID hypersensitivity phenotype when an NSAID is needed. C 3. Preventative measures Avoidance of the drug: Advise lifelong avoidance of the drug and cross-reactive drugs in patients with a history of drug-induced anaphylaxis. B Provide an indicative, regularly updated list of drugs to avoid and the list of possible alternatives in patients with a drug hypersensitivity reaction. B Premedication: offer premedication (slow injection, pretreatment with corticosteroids and H1RAs) for non-allergic drug hypersensitivity reactions, although corticosteroids and H1RAs may not reliably prevent IgE-dependent anaphylaxis. B Clinical findings Symptoms Past medical history Abdominal cramps Anaphylaxis Arthralgia Atopy Chest tightness Stevens-johnson syndrome Cough Ocular exam Diarrhea Dizziness Conjunctival injection Dysphagia Eyelid swelling Fainting Dental exam Fever Generalized pruritus Stomatitis Headache Lightheadedness Integument exam Lip swelling Angioedema Malaise Atypical target lesions Nasal congestion Diaphoresis Nausea Maculopapular rash Seizure Morbilliform rash Shortness of breath https://web.pathway.md/diseases/reczar7CgdXY7kEyP 3/4 6/29/23, 10:20 PM Drug allergy Pathway Skin flushing Nikolsky's sign Vomiting Papulovesicular rash Skin bullae Vital signs Skin desquamation Skin edema Hypotension Urticaria Head and neck exam Diagnostic procedures Periorbital edema drug challenge test Swollen uvula skin patch test Tongue swelling skin prick test Respiratory exam Bronchospasm Laryngeal edema Wheezing Serological findings ll ifi I E References 1. P Demoly, N F Adkinson, K Brockow et al. International Consensus on drug allergy. Allergy. 2014 Apr;69 4 420 37. Open 2. David A Khan, Aleena Banerji, Kimberly G Blumenthal et al. Drug Allergy: A 2022 Practice Parameter Update. J Allergy Clin Immunol. 2022 Sep 16;S0091 6749 22 01186 1. Open 3. R Mirakian, S C Leech, M T Krishna et al. Management of allergy to penicillins and other beta- lactams. Clin Exp Allergy. 2015 Feb;45 2 300 27. Open 4. Knut Brockow, Bernhard Przybilla, Werner Aberer et al. Guideline for the diagnosis of drug hypersensitivity reactions. Allergo J Int. 2015; 24 3 94 105. Open https://web.pathway.md/diseases/reczar7CgdXY7kEyP 4/4 |
Guideline sources The following summarized guidelines for the evaluation and management of drug-induced liver injury are prepared by our editorial team based on guidelines from the American Association for the Study of Liver Diseases (AASLD 2022), the American College of Gastroenterology (ACG 2021), the American Gastroenterological Association (AGA 2021), and the European Association for the Study of the Liver (EASL 2019). 1 2 3 4 6 7 7 8 Definition DILI is acute/chronic liver injury characterized by abnormalities in liver function. 6 Epidemiology DILI is mostly caused by antibiotics (amoxicillin-clavulanate; 45.4%), herbal and dietary supplements (16.1%), cardiovascular agents (9.8%), CNS agents (9.1%), anti-neoplastic agents (5.5%), and analgesics (paracetamol overdose; 3.7%). 7 Disease course Clinical manifestations of DILI include jaundice, rashes, acute hepatitis, Stevens-Johnson syndrome, AIH, coagulopathies, hepatic encephalopathy. Progression may lead to chronic liver https://web.pathway.md/diseases/recwmnTv3HE0qONJ3 1/11 6/29/23, 10:21 PM Drug-induced liver injury Pathway disease with fibrosis, cirrhosis, necessitating liver transplantation. 7 Prognosis and risk of recurrence The mortality rate associated with DILI is 7.6%. 8 Calculator Calculator Calculat King's College Criteria for aceta Updated Roussel Uclaf Causality Update Guidelines 1. Screening and diagnosis Indications for monitoring: educate patients to report untoward symptoms to their healthcare providers for early detection of DILI, as well as obtain prospective clinical and laboratory monitoring with certain high-risk drugs (such as immune checkpoint inhibitors, isoniazid, and methotrexate). E Show 7 more Patterns of injury: Recognize that: direct hepatotoxins, such as acetaminophen, can cause liver injury in nearly all exposed patients once a threshold dose or duration of use is exceeded indirect hepatotoxins are generally independent of the dose administered and have a variable latency and manifestations arising from the biological action of the drug on the liver and/or host immune system idiosyncratic DILI is largely independent of the dose and duration of medication use and characterized by a low incidence and variable drug latency and clinical and histological features, and is believed to arise from an aberrant adaptive host immune response to the drug and/or its metabolites. (Guidance statement). E Show 2 more Causality assessment: As per AASLD 2022 guidelines, use structured causality assessment instruments incorporating the dose, duration, and timing of suspect drug and other concomitant drug or herbal and dietary supplement product use, assessment of the laboratory, radiological, and histological features at presentation, and exclusion of competing causes of liver injury. E Show 4 more As per EASL 2019 guidelines, consider using the CIOMS/RUCAM scale to assess causality and guide a systematic and objective evaluation of patients with suspected DILI. C Diagnostic criteria: Define clinically significant DILI as any one of the following: serum AST or ALT > 5 ULN, or ALP > 2 ULN (or pre-treatment baseline if the baseline is abnormal) on 2 separate occasions total serum bilirubin > 2.5 mg/dL along with elevated AST, ALT, or ALP levels https://web.pathway.md/diseases/recwmnTv3HE0qONJ3 2/11 6/29/23, 10:21 PM Drug-induced liver injury Pathway INR > 1.5 with elevated AST, ALT, or ALP levels. E Differential diagnosis: exclude alternative causes of liver injury in all patients with DILI, including testing for viral hepatitis, metabolic liver disease, AIH, and pancreaticobiliary disease. E 2. Classification and risk stratification Classification: As per AASLD 2022 guidelines, classify idiosyncratic DILI by the R-value at presentation (R = ALT:ULN/ALP:ULN; hepatocellular if R 5, mixed if 2 < R < 5, and cholestatic if R 2) to help guide the evaluation of alternative causes of liver injury. E As per EASL 2019 guidelines, classify DILI as hepatocellular, cholestatic or mixed according to the pattern of elevation of liver enzymes based on the first set of laboratory tests available in relation to the clinical event. B Risk factors: As per AASLD 2022 guidelines, recognize that: the daily dose of a medication, its lipophilicity, and the extent of hepatic metabolism influence the risk of causing DILI insufficient evidence to confirm subject age, gender, race, and ethnicity as reliable risk factors for DILI susceptibility some drugs are more likely to cause DILI in older patients (such as amoxicillin-clavulanate, isoniazid), whereas others are more commonly implicated in pediatric patients (such as valproate, minocycline) medical comorbidities, such as obesity and diabetes, are associated with increased incidence and severity of DILI with specific drugs insufficient evidence regarding the role of alcohol, tobacco, and diet in DILI susceptibility patients with preexisting liver disease are at increased risk of developing liver injury with selected drugs (such as methotrexate and anti-tuberculosis therapy) and poor outcomes with a DILI episode PTPN22 gene polymorphism is a risk factor for DILI across multiple drugs and major ethnic groups. E As per EASL 2019 guidelines: Recognize that: age is a contributing factor determining the susceptibility to DILI secondary to particular drugs, and contributing to the phenotype of the disease B female sex is a risk factor for DILI associated with specific drugs B female sex is associated with a greater risk of drug-induced ALF B ethnicity is a risk factor for DILI A chronic hepatitis B and C are risk factors for DILI from anti-human immunodeficiency virus and anti-tuberculosis therapy B regular alcohol intake is a contributing factor to DILI associated with specific drugs such as isoniazid, methotrexate and halothane. B https://web.pathway.md/diseases/recwmnTv3HE0qONJ3 3/11 6/29/23, 10:21 PM Drug-induced liver injury Pathway components of metabolic syndrome are risk factors for the occurrence and the degree of drug-associated fatty liver disease in patients treated with tamoxifen and methotrexate. A Recognize the following properties of drugs conferring a risk for DILI: a daily dose of > 100 mg whatever the drug predominant hepatic metabolism by cytochrome P450 enzymes formation of reactive metabolites dual inhibition of mitochondria and bile salt export pump function. B Risks of rechallenge: As per AASLD 2022 guidelines, avoid offering rechallenge with the suspect drug unless the anticipated benefit is high for a severe or life-threatening condition. D As per ACG 2021 guidelines, do not re-expose patients with DILI to a drug thought likely to have caused hepatotoxicity, especially if the initial liver injury was associated with significant aminotransferase elevation (such as > 5 times the ULN, Hy's law, or jaundice). An exception to this recommendation are life-threatening situations where there is no suitable alternative. D As per EASL 2019 guidelines, recognize that liver injury caused by unintentional rechallenge in clinical practice confers a higher risk of mortality/liver transplantation than the initial DILI episode. B Show 2 more Prognosis: As per AASLD 2022 guidelines, recognize that most adult and pediatric patients with idiosyncratic DILI present with an acute liver injury phenotype (symptomatic or asymptomatic) typically resolves within 6 months of onset without long-term sequelae in 80% of cases. E Show 4 more As per ACG 2021 guidelines, consider using a prognostic model consisting of Model for End- Stage Liver Disease, Charlson comorbidity index, and serum albumin in clinical practice for predicting 6-month mortality in patients presenting with suspected DILI. C As per EASL 2019 guidelines: Recognize that a very small proportion of patients develops chronic liver disease as a potential consequence of idiosyncratic drug-induced liver disease. B Consider using Hy's law to identify patients at risk of progressing severe DILI in the setting of clinical trials. Recognize that thresholds for interrupting or stopping treatment with a study drug, as recommended by the U.S. FDA, are intended as guidelines for studies in drug development. Consider adapting these thresholds depending on individual risk-benefit assessment. C 3. Diagnostic investigations Medication history: elicit a detailed medication and herbal/dietary supplement history within the 180 days before presentation in patients with suspected DILI. E https://web.pathway.md/diseases/recwmnTv3HE0qONJ3 4/11 6/29/23, 10:21 PM Drug-induced liver injury Pathway Abdominal imaging: As per ACG 2021 guidelines, obtain abdominal imaging (ultrasound, CT and MRI) to exclude biliary tract pathology and infiltrative processes in all patients with suspected cholestatic DILI. B As per EASL 2019 guidelines, obtain abdominal ultrasound in all patients with suspected DILI. Consider obtaining additional imaging studies based on the clinical context. B Liver function tests: As per AASLD 2022 guidelines, recognize that serum markers of liver injury, such as serum AST, ALT, and ALP, are not sensitive or specific enough for early detection of DILI. E As per EASL 2019 guidelines, obtain ALT, ALP and TBIL as standard tests to define liver damage and liver dysfunction in patients with DILI. B Show 4 more Viral and serological hepatitis panel: As per ACG 2021 guidelines, obtain standard serologies and HCV-RNA to exclude acute viral hepatitis (A, B, and C) and AIH in patients with suspected hepatocellular or mixed DILI. B Show 3 more As per EASL 2019 guidelines, consider obtaining HCV-RNA and anti-HEV IgM or HEV-RNA in patients with suspected DILI to exclude acute hepatitis C and/or E, particularly in cases not compatible with the drug signature of the suspected causative agent and/or with high aminotransferase levels. C Evaluation for other liver diseases: evaluate patients with suspected hepatocellular or mixed DILI for Wilson disease and Budd-Chiari syndrome if clinically appropriate. B Genetic testing: obtain HLA genotyping in selected clinical scenarios where genetic tests assist the diagnosis and management of patients with DILI. B Show 2 more 4. Diagnostic procedures Liver biopsy: As per AASLD 2022 guidelines: Do not perform a liver biopsy for the diagnosis of idiosyncratic DILI, especially in mild and self-limited cases. Consider performing a liver biopsy in patients with a severe or protracted course of DILI and in case of diagnostic uncertainty. D Recognize that liver biopsy can help to: identify hepatotoxic drugs based on specific histological patterns and exclude concurrent liver diseases determine the mechanism of injury, for example, microvesicular steatosis and necrosis associated with the use of mitochondrial toxin fialuridine predict outcomes, for example, the presence of eosinophils and granulomas is associated with a more favorable outcome, whereas necrosis or fibrosis is associated with poorer outcomes. E https://web.pathway.md/diseases/recwmnTv3HE0qONJ3 5/11 6/29/23, 10:21 PM Drug-induced liver injury Pathway As per ACG 2021 guidelines: Perform liver biopsy if AIH remains a competing etiology and if immunosuppressive therapy is contemplated. B Consider performing liver biopsy if: there is an unrelenting rise in liver biochemistries or signs of worsening liver function despite stopping the suspected offending agent the peak ALT level has not fallen by > 50% at 30-60 days after the onset in cases of hepatocellular DILI despite stopping the suspected offending agent the peak ALP has not fallen by > 50% at 180 days in cases of cholestatic DILI despite stopping the suspected offending agent liver biochemistry abnormalities persist > 180 days, especially if associated with symptoms (such as itching) or signs (such as jaundice and hepatomegaly), to evaluate for the presence of chronic liver diseases and chronic DILI continued use or re-exposure to the implicated agent is expected. C As per EASL 2019 guidelines, consider performing liver biopsy in the evaluation of selected patients with suspected DILI, as liver histology can provide information supporting the diagnosis of DILI or an alternative. C Show 2 more Endoscopic retrograde cholangiography: consider limiting EUS to patients with suspected cholestatic DILI only if routine imaging including MRI or EUS is unable to exclude impacted common bile duct stones, PSC, or pancreaticobiliary malignancy. C 5. Medical management Withdrawal of offending agents: As per AASLD 2022 guidelines, discontinue the suspect drug along with supportive care of antiemetics, antipruritics, and hydration as the mainstay of management of patients with idiosyncratic DILI. E As per ACG 2021 guidelines, discontinue suspected agents promptly in patients with suspected DILI, especially when liver biochemistries are rising rapidly or there is evidence of liver dysfunction. B N-acetylcysteine: As per AASLD 2022 guidelines, consider administering a short course of IV N-acetylcysteine in hospitalized adult patients with DILI-related ALF. E As per ACG 2021 guidelines: Consider offering N-acetylcysteine for the treatment of adult patients with early-stage ALF, given its good safety profile and some evidence for efficacy in early coma stage patients, recognizing that no definitive therapies are available for idiosyncratic DILI with or without ALF. C Avoid using N-acetylcysteine in pediatric patients with severe DILI leading to ALF. D As per EASL 2019 guidelines: https://web.pathway.md/diseases/recwmnTv3HE0qONJ3 6/11 6/29/23, 10:21 PM Drug-induced liver injury Pathway Insufficient evidence to support the use of N-acetylcysteine to reduce the severity of liver injury caused by drugs other than acetaminophen. I Administer N-acetylcysteine in adult patients with idiosyncratic drug-induced ALF early in the course of disease (grade I-II). B Corticosteroids: As per AASLD 2022 guidelines, consider administering corticosteroids for 1-3 months in selected patients with idiosyncratic DILI, including patients with severe hypersensitivity features, drug reaction with eosinophilia and systemic symptoms, and autoimmune features on liver biopsy. E As per ACG 2021 guidelines, insufficient evidence to recommend for or against the use of corticosteroids in patients with DILI. Consider offering corticosteroids in a subset of patients with DILI exhibiting AIH-like features. I As per EASL 2019 guidelines, insufficient evidence to support the routine use of corticosteroids in patients with idiosyncratic DILI. I Ursodeoxycholic acid: As per AASLD 2022 guidelines, insufficient evidence to support the use of UDCA in patients with DILI, although it is presumably safe to administer. I As per EASL 2019 guidelines, insufficient evidence to support the use of UDCA to reduce the severity of liver injury. I Cholestyramine: consider administering a short course of cholestyramine to decrease the course of hepatotoxicity induced by very selected drugs, such as leflunomide and terbinafine. C Carnitine: consider administering carnitine to improve valproate hepatotoxicity. C Defibrotide: administer defibrotide in adult and pediatric patients with moderate-to-severe sinusoidal obstruction syndrome undergoing hematopoietic cell transplantation. E 6. Surgical interventions Liver transplantation: As per AASLD 2022 guidelines, transfer patients with idiosyncratic DILI-related ALF to a liver transplant center because of the low likelihood of spontaneous survival. E As per EASL 2019 guidelines, consider performing liver transplantation as a therapeutic option in patients with drug-induced ALF. C 7. Specific circumstances Patients with HDS-induced liver injury: As per AASLD 2022 guidelines: Recognize that herbal and dietary supplements can cause severe hepatotoxicity with variable clinical, laboratory, and histological phenotypes. E Recognize that: https://web.pathway.md/diseases/recwmnTv3HE0qONJ3 7/11 6/29/23, 10:21 PM Drug-induced liver injury Pathway genetic polymorphisms in the HLA region and the conditions under which a product is consumed may influence the likelihood of a patient developing herbal or dietary supplement-induced hepatotoxicity HLA-B35:01 has been associated with hepatotoxicity attributed to green tea extract in White populations and Polygonatum multiflorum hepatotoxicity in Asian populations. E As per ACG 2021 guidelines, encourage patients to report the use of herbal and dietary supplements to their healthcare providers, acknowledging that supplements are not subjected to the same rigorous testing for safety and efficacy as prescription medications. B Show 5 more As per EASL 2019 guidelines, consider suspecting herbal and dietary supplements as potential causative agents associated with liver injury. C Patients with chronic liver disease: exclude other more common causes of acute liver injury including a flare-up of the underlying liver disease for the diagnosis of DILI in patients with chronic liver disease. B Show 4 more Patients with specific phenotypes (autoimmune hepatitis): Obtain detailed evaluation in patients with suspected drug-induced AIH including causality assessment, serology, genetic tests and liver biopsy whenever possible. B Withdraw corticosteroids once the liver injury has resolved in patients with suspected drug- induced AIH being treated with corticosteroids, and obtain close monitoring. B Patients with specific phenotypes (secondary sclerosing cholangitis): suspect the diagnosis of drug-induced secondary sclerosing cholangitis in patients with a cholestatic pattern of DILI with slow resolution of liver injury and characteristic changes in the biliary system demonstrated on MRCP or ERCP. B Patients with specific phenotypes (granulomatous hepatitis): obtain expert evaluation of liver histology and exclude specific infections, inflammatory and immunological conditions well- recognized as causes of hepatic granulomata for the diagnosis of granulomatous hepatitis. B Patients with specific phenotypes (acute fatty liver): recognize drug-induced fatty liver based on its distinct clinicopathological characteristics in patients exposed to drugs known to interfere with mitochondrial function. B Patients with specific phenotypes (fatty liver disease): Recognize that particular drugs, such as amiodarone, methotrexate, tamoxifen and the chemotherapeutic agents 5-fluorouracil and irinotecan are risk factors for fatty liver disease. B Rely upon the benefits of the treatment against the risk of progressive liver disease when deciding to continue or withdraw these medications. B Patients with specific phenotypes, immune-related hepatotoxicity: As per AGA 2021 guidelines, obtain baseline liver tests (TBIL, ALP, AST, and ALT) and pretreatment screening for HBV (HBsAg, HBcAb, and HBsAb) in all patients undergoing immune checkpoint inhibitor therapy. Repeat liver tests before each immune checkpoint inhibitor treatment cycle, with management based on the CTCAE version 5 grade. B Show 5 more https://web.pathway.md/diseases/recwmnTv3HE0qONJ3 8/11 6/29/23, 10:21 PM Drug-induced liver injury Pathway As per EASL 2019 guidelines: Recognize that immune checkpoint inhibitors can induce immune-related hepatotoxicity in a substantial proportion of patients, with CTLA-4 inhibitors (ipilimumab) being more hepatotoxic than PD-L1 agents (nivolumab) and combination treatments carrying greater risk. A Decide on corticosteroid treatment in patients with immune-mediated hepatitis associated with immune checkpoint inhibitors by a multidisciplinary team involving hepatologists if DILI is sufficiently severe based on clinical and histological assessment. B Patients with specific phenotypes (nodular regenerative hyperplasia): consider withdrawing drugs associated with nodular regenerative hyperplasia, if possible. C Patients with specific phenotypes (liver tumors): Recognize that: oral contraceptives are risk factors for the development of hepatic adenoma. B androgens and androgenic steroids are risk factors for the development of liver tumors, particularly in the context of treating bone marrow failure. B Consider withdrawing medications where possible and obtaining continued monitoring until regression of adenoma or definitive treatment. C Patients with acetaminophen overdose (diagnosis): recognize that acetaminophen is a dose-dependent hepatotoxin causing acute pericentral liver injury when doses of > 4 g are ingested within a 24-hour period or excessive doses over several days. E Show 2 more Patients with acetaminophen overdose (gastrointestinal decontamination): perform gastric lavage and administer activated charcoal in all patients presenting within 4 hours of a single-time-point acetaminophen overdose. E Patients with acetaminophen overdose (N-acetylcysteine): administer IV or PO N- acetylcysteine to prevent liver injury, nearly completely if administered within 12 hours of acetaminophen ingestion. E Patients with acetaminophen overdose (prognosis): recognize that the prognosis in acetaminophen-related ALF is related to the degree of encephalopathy, coagulopathy, and acidosis. E 8. Quality improvement Reporting: report all cases of suspected DILI to specialized national registries. E Clinical findings Patient demographics Symptoms Elderly age Abdominal pain Female sex Dark urine Fatigue https://web.pathway.md/diseases/recwmnTv3HE0qONJ3 9/11 6/29/23, 10:21 PM Drug-induced liver injury Pathway Fever Past medical history Generalized pruritus Antibiotics Loss of appetite Chronic liver disease Pale stools Diabetes mellitus RUQ pain Gastroesophageal varices Skin rash Nonalcoholic fatty liver disease Weight gain Obesity Medication history Social history Acetaminophen Alcohol consumption Amiodarone Amoxicillin-clavulanate Vascular exam Anti-tuberculosis medications Peripheral edema Antifungals Aspirin Integument exam Dapsone Dietary supplements Jaundice Fibrates Hematological findings Fluoroquinolones Halothane WBC count Herbal supplements Isoniazid Macrolides Minocycline NSAIDs Niacin Nitrofurantoin Phenobarbital Phenytoin Rifampin Statins TMP-SMX Vitamin A Anthropomorphic exam Malnutrition Abdominal exam Abdominal distension Ascites Hepatomegaly Splenomegaly Lab findings ALF INR https://web.pathway.md/diseases/recwmnTv3HE0qONJ3 10/11 6/29/23, 10:21 PM Drug-induced liver injury Pathway liver enzymes serum ALP serum ALT serum AST serum TBIL serum creatinine serum gamma- glutamyltransferase serum phosphate serum albumin References 1. Naga P Chalasani, Haripriya Maddur, Mark W Russo et al. ACG Clinical Guideline: Diagnosis and Management of Idiosyncratic Drug-Induced Liver Injury. Am J Gastroenterol. 2021 May 1;116 5 878 898. Open 2. Robert J Fontana, Iris Liou, Adrian Reuben et al. AASLD practice guidance on drug, herbal, and dietary supplement-induced liver injury. Hepatology. 2022 Jul 27. Open 3. European Association for the Study of the Liver, Clinical Practice Guideline Panel: Chair, Panel members et al. EASL Clinical Practice Guidelines: Drug-induced liver injury. J Hepatol. 2019 Jun;70 6 1222 1261. Open 4. Michael Dougan, Yinghong Wang, Alberto Rubio-Tapia et al. AGA Clinical Practice Update on Diagnosis and Management of Immune Checkpoint Inhibitor Colitis and Hepatitis: Expert Review. Gastroenterology. 2021 Mar;160 4 1384 1393. Open 5. J Haanen, M Obeid, L Spain et al. Management of toxicities from immunotherapy: ESMO Clinical Practice Guideline for diagnosis, treatment and follow-up. Ann Oncol. 2022 Dec;33 12 1217 1238. Open 6. Jeong Ill Suh. Drug-induced liver injury. 2020 Jan;37 1 2 12.2020 Jan;37 1 2 12. Open 7. Dev Katarey, Sumita Verma. Drug-induced liver injury. Clin Med Lond). 2016 Dec; 16 Suppl 6 s104 s109. Open 8. Einar S Bjornsson, Helgi K Bjornsson. Mortality associated with drug-induced liver injury DILI . 2017 Dec 19;2 114.2017 Dec 19;2 114. Open 9. Chalasani NP, Hayashi PH, Bonkovsky HL et al. ACG Clinical Guideline: the diagnosis and management of idiosyncratic drug-induced liver injury. Am J Gastroenterol. 2014 Jul;109 7 950 66. Open 10. Einar S. Bj rnsson and Helgi K. Bj rnsson. Mortality associated with drug-induced liver injury DILI . Transl Gastroenterol Hepatol. 2017; 2 114. Open 11. Jeong Ill Suh. Drug-induced liver injury. Yeungnam Univ J Med. 2020 Jan; 37 1 2 12. Open 12. Gaby Danan, Rolf Teschke. RUCAM in Drug and Herb Induced Liver Injury: The Update. Int J Mol Sci. 2015 Dec 24;17 1 14. Open 13. Shirin Ghanavatian, Armen Derian. Tizanidine. StatPearls Internet]. Treasure Island FL StatPearls Publishing. Open https://web.pathway.md/diseases/recwmnTv3HE0qONJ3 11/11 |
Guideline sources The following summarized guidelines for the evaluation and management of duchenne muscular dystrophy (DMD) are prepared by our editorial team based on guidelines from the American College of Chest Physicians (ACCP 2023), the Heart Rhythm Society (HRS 2022), the European Society of Cardiology (ESC 2022), the American Heart Association (AHA/HRS/ACC 2018), the American Academy of Neurology (AAN 2016), and the British Thoracic Society (BTS 2012). 1 2 3 4 5 6 Guidelines 1. Diagnostic investigations Respiratory evaluation: As per ACCP 2023 guidelines, obtain pulmonary function testing to assist with management decisions in patients with neuromuscular disease at risk for respiratory complications. E Show 3 more As per BTS 2012 guidelines, obtain a clinical assessment of respiratory health directed towards identifying progressive muscle weakness, ability to cope with a respiratory infection, https://web.pathway.md/diseases/recYiGrJDFaS04FTU 1/7 6/29/23, 10:21 PM Duchenne muscular dystrophy Pathway aspiration, progression of scoliosis, and sleep-disordered breathing as part of every medical consultation of pediatric patients with neuromuscular disorders. B Show 3 more Sleep evaluation: Obtain assessment for sleep-disordered breathing at least annually in pediatric patients with neuromuscular disorders with any of the following: vital capacity < 60% of predicted B non-ambulance because of progressive muscle weakness B inability to walk B symptoms of obstructive sleep apnea E hypoventilation E clinically apparent diaphragmatic weakness E rigid spine syndrome. E Show 4 more Cardiac evaluation: As per ESC 2022 guidelines, obtain annual follow-up with at least a 12-lead ECG in patients with muscular dystrophies, even in the concealed phase of the disease. B As per HRS 2022 guidelines: Obtain screening cardiac imaging (echocardiography or cardiac MRI) in adult female carriers of a pathogenic or likely pathogenic variant for DMD, even in the absence of cardiac symptoms. B Obtain implantable cardiac monitoring in patients with DMD with symptoms of conduction disorder or arrhythmias without an obvious cause. B As per AHA 2018 guidelines, consider obtaining follow-up for the development of cardiac involvement in patients with muscular dystrophy, even if the patient is asymptomatic at presentation. C Assessment of dysphagia: Obtain a feeding assessment by a speech and language therapist including a videofluoroscopy swallow assessment if the swallow is thought to be unsafe in pediatric patients with neuromuscular disorders with a history of swallowing difficulties. E Obtain videofluoroscopy in pediatric patients with a history of recurrent chest infections or swallowing difficulties. E Assessment of pain: obtain routine pain assessment as part of standard clinical assessment in all pediatric and young patients with neuromuscular disorders. B 2. Respiratory support Airway clearance, manually assisted cough: As per ACCP 2023 guidelines, consider offering manually assisted cough techniques, independently or adding to other modalities (such as lung volume recruitment), in patients with reduced cough effectiveness. C As per BTS 2012 guidelines: https://web.pathway.md/diseases/recYiGrJDFaS04FTU 2/7 6/29/23, 10:21 PM Duchenne muscular dystrophy Pathway Teach augmented cough techniques in pediatric patients with ineffective cough (including 12 years old patients with cough peak flow < 270 L/min), particularly in case of previous episodes of deterioration with respiratory infections. B Offer manual cough assist and air-stacking methods when appropriate to achieve maximum insufflation capacity to improve cough efficiency. B Airway clearance (lung volume recruitment): Consider offering glossopharyngeal breathing for lung volume recruitment and airway clearance in patients with hypoventilation. C Consider offering regular lung volume recruitment (breath stacking) using a handheld resuscitation bag or mouthpiece in patients with reduced lung function or cough effectiveness. C Airway clearance, mechanical insufflation-exsufflation: As per ACCP 2023 guidelines, consider adding regular mechanical insufflation-exsufflation (cough assist device) in patients with reduced cough effectiveness not adequately improved with alternative techniques. C As per BTS 2012 guidelines, consider offering mechanical insufflation-exsufflation in very weak patients, in patients with loss of bulbar function, unable to cooperate with manual- assisted cough or air-stacking, or if these methods are ineffective. C Show 2 more Airway clearance, high-frequency chest wall oscillation: As per ACCP 2023 guidelines, consider offering high-frequency chest wall oscillation for secretion mobilization in patients with difficulties with secretion clearance. Consider combining high-frequency chest wall oscillation with airway clearance therapies, such as cough assistance or lung volume recruitment. C As per BTS 2012 guidelines, consider offering oscillatory techniques, such as high-frequency chest wall oscillation and intrapulmonary percussive ventilation, in pediatric patients with difficulty mobilizing secretions or having persistent atelectasis despite using other airway clearance techniques. C Airway clearance (sputum mobilization): ensure that appropriate emergency equipment (resuscitation bag and suction) is available when using sputum mobilizing techniques, in case of mobilizing large mucus plugs into the central airways where they may result in airway obstruction. E Airway clearance (nebulized saline): consider administering nebulized normal saline in pediatric patients with continued tenacious secretions. E Airway clearance (noninvasive ventilation): Use the patient's ventilator (in patients on regular night-time or diurnal noninvasive ventilation) to support deep breathing during airway clearance treatments and help prevent respiratory muscle fatigue. E Consider using humidification in pediatric patients on noninvasive ventilation and having tenacious airway secretions, with care taken to ensure that this does not result in a troublesome increase in oral secretions. E Airway clearance (acute infection): use airway clearance techniques during respiratory infection when oxygen saturation levels fall < 95% on breathing room air. Use different https://web.pathway.md/diseases/recYiGrJDFaS04FTU 3/7 6/29/23, 10:21 PM Duchenne muscular dystrophy Pathway methods of airway clearance if the techniques being used fail to increase oxygen saturation to 95%. B Noninvasive ventilation: As per ACCP 2023 guidelines, initiate noninvasive ventilation in patients with chronic respiratory failure. B Show 4 more As per BTS 2012 guidelines, initiate noninvasive ventilation in pediatric patients with neuromuscular disorders resulting in symptomatic nocturnal hypoventilation or daytime hypercapnia. B Show 13 more Invasive ventilation: consider offering invasive home mechanical ventilation via tracheostomy as an alternative to noninvasive ventilation in patients failing or intolerant of noninvasive ventilation (including extended daytime noninvasive ventilation use), worsening bulbar function, frequent aspiration, insufficient cough, episodes of chest infection despite adequate secretion management, or declining lung function. C 3. Medical management Corticosteroids: Offer prednisone at a preferred dose of 0.75 mg/kg/day to improve strength and pulmonary function in pediatric patients with DMD. B Consider offering prednisone to improve timed motor function, reduce the need for scoliosis surgery, and delay cardiomyopathy onset by 18 years of age. Recognize that prednisone 0.75 mg/kg/day is associated with a significant risk of weight gain, hirsutism, and cushingoid appearance. B Consider offering deflazacort to improve strength and timed motor function, delay age at loss of ambulation by 1.4-2.5 years, improve pulmonary function, reduce the need for scoliosis surgery, delay cardiomyopathy onset, and increase survival at 5-15 years of follow-up in pediatric patients with DMD. Recognize that deflazacort may be associated with a greater risk of cataracts than prednisone. C Opioids: consider offering nonpharmacological measures in patients with a subjective feeling of dyspnea directing treatment at the cause. E Show 2 more Anticoagulant therapy: initiate anticoagulation in patients with DMD and AF or atrial flutter according to established guidelines taking into consideration the risks of thromboembolism and bleeding on oral anticoagulation. B 4. Nonpharmacologic interventions Nutritional support: Provide feeding advice from a specialist therapist in pediatric patients with swallowing difficulties. E Provide a problem-orientated approach to nutrition aiming to minimize the risk of aspiration, optimize nutritional status, promote comfort, and balance the positive social consequences of https://web.pathway.md/diseases/recYiGrJDFaS04FTU 4/7 6/29/23, 10:21 PM Duchenne muscular dystrophy Pathway continued oral feeding. E 5. Therapeutic procedures Permanent pacemaker implantation: Perform permanent pacemaker implantation in patients with DMD with documented symptomatic bradycardia due to any degree of SND or AV block, if concordant with the patient's goals of care and clinical status. B Perform permanent pacemaker implantation in patients with DMD and third-degree or advanced second-degree AV block at any anatomical level, with or without symptoms, if concordant with the patient's goals of care and clinical status. B Cardiac resynchronization therapy: consider offering cardiac resynchronization therapy in patients with DMD with an LVEF 35% despite guideline-directed medical therapy with a combination of sinus rhythm, LBBB, QRS duration 150 ms, and NYHA class II-IV symptoms, or in patients with suspected RV pacing-induced cardiomyopathy or anticipated RV pacing 40%, if concordant with the patient's goals of care and clinical status. C Implantable cardioverter-defibrillator: As per ESC 2022 guidelines, consider performing ICD placement in patients with DMD and significant late gadolinium enhancement on cardiac MRI. C As per HRS 2022 guidelines: Perform ICD placement in patients with DMD with spontaneously occurring hemodynamically significant sustained VT or VF, if concordant with the patient's goals of care and clinical status. B Consider performing ICD placement in patients with DMD with an LVEF 35% despite guideline-directed medical therapy, if concordant with the patient's goals of care and clinical status. C As per AHA 2018 guidelines, perform ICD placement for primary and secondary prevention in patients with neuromuscular disorders according to the same indications as for patients with non-ischemic cardiomyopathy, if the expected meaningful survival is > 1 year. B 6. Perioperative care Preoperative planning: assess pediatric patients with neuromuscular disorders requiring surgery (including scoliosis surgery) by a multidisciplinary team before any intervention. E Show 3 more 7. Patient education General counseling: engage patients, parents, and carers in a process of mutual participation in decision-making with full disclosure of all information in a sensitive and timely fashion in order to make informed choices consistent with their own values and preferences requires physicians. B Show 2 more https://web.pathway.md/diseases/recYiGrJDFaS04FTU 5/7 6/29/23, 10:21 PM Duchenne muscular dystrophy Pathway 8. Follow-up and surveillance End-of-life care: provide periodic education on function including deactivation in patients (as well as their family members and/or healthcare decision makers) with neuromuscular disorders eligible for or having a pacemaker or ICD. B Show 5 more Clinical findings Symptoms Past medical history Cough Attention deficit hyperactivity disorder Dysphagia Autism spectrum disorder Dyspnea Developmental delay Exercise intolerance HF Fatigue OCD Gait disturbance Hypoventilation Neurological exam Muscle weakness Nausea Cognitive impairment Orthopnea ECG findings Sleeping disorder Vomiting AF Weight loss Ventricular arrhythmia Vital signs Genetic testing Tachycardia Dmd gene mutation O2 saturation Musculoskeletal exam Calf hypertrophy Lordosis Scoliosis ankle ROM muscle strength Imaging findings LVEF References 1. Akram Khan, Lindsy Frazer-Green, Reshma Amin et al. Respiratory Management of Patients with Neuromuscular Weakness: An American College of Chest Physicians Clinical Practice Guideline and Expert Panel Report. Chest. 2023 Mar 13;S0012 3692 23 00353 7. Open https://web.pathway.md/diseases/recYiGrJDFaS04FTU 6/7 6/29/23, 10:21 PM Duchenne muscular dystrophy Pathway 2. William J Groh, Deepak Bhakta, Gordon F Tomaselli et al. 2022 HRS expert consensus statement on evaluation and management of arrhythmic risk in neuromuscular disorders. Heart Rhythm. 2022 Oct;19 10):e61-e120. Open 3. Jeremy Hull, Roona Aniapravan, Elaine Chan et al. British Thoracic Society guideline for respiratory management of children with neuromuscular weakness. Thorax. 2012 Jul;67 Suppl 1:i1 40. Open 4. Sana M Al-Khatib, William G Stevenson, Michael J Ackerman et al. 2017 AHA/ACC/HRS Guideline for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society. Circulation. 2018 Sep 25;138 13):e272-e391. Open 5. Gloss D, Moxley RT rd, Ashwal S et al. Practice guideline update summary: Corticosteroid treatment of Duchenne muscular dystrophy: Report of the Guideline Development Subcommittee of the American Academy of Neurology. Neurology. 2016 Feb 2;86 5 465 72. Open 6. Katja Zeppenfeld, Jacob Tfelt-Hansen, Marta de Riva et al. 2022 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death. Eur Heart J. 2022 Aug 26;ehac262. Open https://web.pathway.md/diseases/recYiGrJDFaS04FTU 7/7 |
Guideline sources The following summarized guidelines for the management of ductal carcinoma in situ are prepared by our editorial team based on guidelines from the American Society for Radiation Oncology (ASTRO/ASCO 2016). 1 4 4 4 5 Definition DCIS of the breast is the proliferation of malignant epithelial cells within the mammary ductal system without involving the basement membrane or invasion into the surrounding stroma. 4 Epidemiology DCIS is caused mainly by chromosomal imbalances including loss of heterozygosity and overexpression of the HER2 (HER2)/neu proto-oncogene and mutation in the p53 tumor suppressor gene. 4 Disease course DCIS is clinically silent in most cases; however, a breast lump or bloody nipple discharge may be present. Progression may lead to invasive breast cancer. 4 Prognosis and risk of recurrence DCIS is not associated with any excess risk of death, except in women aged < 50 years with a standard mortality ratio of 3.44 (95% CI 1.85-6.40). 5 Guidelines https://web.pathway.md/diseases/reclNi2rcyjrE9A7m 1/3 6/29/23, 10:21 PM Ductal carcinoma in situ Pathway 1. Medical management Systemic therapy: as per ASCO 2016 guidelines, rates of ipsilateral breast tumor recurrence are reduced with endocrine therapy, but there is no evidence of an association between endocrine therapy and negative margin width. B 2. Therapeutic procedures Whole-breast radiation therapy: As per ASCO 2016 guidelines: A positive margin, defined as ink on DCIS, is associated with a signicant increase in ipsilateral breast tumor recurrence; the use of whole breast radiotherapy does not nullify this increased risk. A Avoid choosing whole-breast radiotherapy delivery technique, fractionation, and boost dose on the basis of negative margin width. D 3. Surgical interventions Surgical excision: as per ASCO 2016 guidelines, higher rates of ipsilateral breast tumor recurrence are reported with treatment with excision alone, regardless of margin width, than treatment with excision and whole breast radiotherapy. A Clinical findings Patient demographics Symptoms Elderly age Breast lump Breast pain Past medical history Changes to the breast or nipple that are different from the ones present on period Obesity Past obstetric history Medication history Early menarche HRT Late first pregnancy Hormonal contraceptives Late menopause Family history Social history Breast cancer Alcohol consumption Ovarian cancer Physical inactivity Breast exam Genetic testing Inverted nipple Brca1 gene mutation Nipple discharge Brca2 gene mutation https://web.pathway.md/diseases/reclNi2rcyjrE9A7m 2/3 6/29/23, 10:21 PM Ductal carcinoma in situ Pathway References 1. Morrow M, Van Zee KJ, Solin LJ et al. Society of Surgical Oncology-American Society for Radiation Oncology-American Society of Clinical Oncology Consensus Guideline on Margins for Breast- Conserving Surgery With Whole-Breast Irradiation in Ductal Carcinoma In Situ. J Clin Oncol. 2016 Nov 20;34 33 4040 4046. Open 2. F Cardoso, S Kyriakides, S Ohno et al. Early breast cancer: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2019 Aug 1;30 8 1194 1220. Open 3. Smith BD, Bellon JR, Blitzblau R et al. Radiation therapy for the whole breast: Executive summary of an American Society for Radiation Oncology ASTRO evidence-based guideline. Pract Radiat Oncol. 2018 May Jun;8 3 145 152. Open 4. Yash Vaidya, Pradeep Vaidya, Tanvi Vaidya. Ductal Carcinoma In Situ of the Breast. 2015 Apr;77 2 141 6.2015 Apr;77 2 141 6. Open 5. A Roca-Barcelo, G Vinas, H Pla et al. Mortality of women with ductal carcinoma in situ of the breast: a population-based study from the Girona province, Spain 1994 2013 . 2019 Jul;21 7 891 899.2019 Jul;21 7 891 899. Open 6. A Roca-Barcel , G Vi as, H Pla et al. Mortality of women with ductal carcinoma in situ of the breast: a population-based study from the Girona province, Spain 1994 2013 . Clin Transl Oncol. 2019 Jul;21 7 891 899. Open 7. Yash Vaidya, Pradeep Vaidya, and Tanvi Vaidya. Ductal Carcinoma In Situ of the Breast. Indian J Surg. 2015 Apr; 77 2 141 146. Open https://web.pathway.md/diseases/reclNi2rcyjrE9A7m 3/3 |
Guideline sources The following summarized guidelines for the evaluation and management of dumping syndrome (DS) are prepared by our editorial team based on guidelines from the Dumping Syndrome Consensus Group (DS-CG 2020), the American Society of Anesthesiologists (ASA/ACE/OS/AACE/ASMBS/OMA 2020), and the European Association for the Study of Obesity (EASO 2017). 1 2 3 Guidelines 1. Screening and diagnosis Clinical presentation: Recognize that: DS is a complication of esophageal or gastric surgery that can comprise both early and late DS symptoms B early DS is the typical and most frequent manifestation of DS and can occur in isolation or in association with late symptoms B DS affects the quality of life and can be associated with weight loss B symptoms of early DS are driven by rapid delivery of nutrients to the small bowel, which triggers the release of several gastrointestinal hormones, including vasoactive agents, incretins, and glucose modulators B https://web.pathway.md/diseases/recaTzMBAx5lBjH1o 1/4 6/29/23, 10:23 PM Dumping syndrome Pathway hypoglycemia is the main symptom of late DS and is driven by a hyperinsulinemic response and GLP-1 release B DS can contribute to weight loss after bariatric surgery. B Diagnosis: Suspect DS based on clinical history. Recognize that currently available dumping questionnaires have no proven diagnostic value. B Suspect late DS in patients with spontaneous hypoglycemia < 2.8 mmol/L (50 mg/dL). B 2. Diagnostic investigations Oral glucose tolerance test: obtain a modified oral glucose tolerance test for the diagnosis of DS. Regard the test results as positive for early DS in case of an early (30 minutes) increase in hematocrit > 3% or in pulse rate > 10 bpm. Regard the test results as positive for late DS in case of late (60-180 minutes after ingestion) hypoglycemia (< 50 mg/dL). B Mixed meal tolerance test: do not obtain mixed meal tests over modified glucose tolerance test. D Continuous glucose monitoring: insufficient evidence regarding the usefulness of continuous glucose monitoring for diagnosing DS. I Gastric emptying testing: insufficient evidence regarding the usefulness of gastric emptying tests in diagnosing DS. I 3. Medical management Acarbose: offer acarbose for the treatment of patients with DS symptoms, especially symptoms of late DS. B Diazoxide: insufficient evidence regarding the usefulness of diazoxide in the treatment of patients with DS. I Octreotide: As per DS-CG 2020 guidelines, offer somatostatin analogs for the treatment of patients with DS. Recognize that short-acting analogs have greater efficacy but require multiple injections. B As per EASO 2017 guidelines, consider offering octreotide in patients failed to be controlled with dietary modifications. B 4. Nonpharmacologic interventions Dietary modifications: As per AACE 2020 guidelines, eliminate concentrated sweets from the diet after Roux-en-Y gastric bypass to minimize symptoms of DS, as well as after any bariatric procedure to reduce caloric intake. B As per DS-CG 2020 guidelines: https://web.pathway.md/diseases/recaTzMBAx5lBjH1o 2/4 6/29/23, 10:23 PM Dumping syndrome Pathway Initiate dietary interventions with eliminating rapidly absorbable carbohydrates as the first- line treatment approach in patients with DS. B Advise patients to consume high fiber and protein-rich foods, eat slowly, and chew well. B As per EASO 2017 guidelines, initiate nutritional manipulations as the first-line treatment to control DS. B Dietary supplements: insufficient evidence regarding the usefulness of agents increasing meal viscosity in the management of patients with DS. I Continuous enteral feeding: insufficient evidence regarding the usefulness of continuous enteral or gastric feeding in the treatment of patients with DS. I 5. Surgical interventions Indications for reoperation: insufficient evidence to recommend surgical interventions (or re- interventions) in patients with DS. I Clinical findings Symptoms Past medical history Abdominal cramps Orthostatic hypotension Bloating Vital signs Borborygmi Diarrhea Signs of shock Dizziness Tachycardia Early satiety Lab findings Epigastric pain Fatigue Hyperglycemia Headache blood glucose Increased appetite Lightheadedness Diagnostic procedures Malaise modified oral glucose tolerance test Nausea Palpitations Restlessness Shortness of breath Skin flushing Sleepiness Syncope Tremor Trouble concentrating Vomiting Past surgical history Recent bariatric surgery https://web.pathway.md/diseases/recaTzMBAx5lBjH1o 3/4 6/29/23, 10:23 PM Dumping syndrome Pathway Recent gastrectomy Recent gastrointestinal surgery Integument exam Diaphoresis Pallor Hematological findings References 1. Emidio Scarpellini, Joris Arts, George Karamanolis et al. International consensus on the diagnosis and management of dumping syndrome. Nat Rev Endocrinol. 2020 Aug;16 8 448 466. Open 2. Busetto L, Dicker D, Azran C et al. Practical Recommendations of the Obesity Management Task Force of the European Association for the Study of Obesity for the Post-Bariatric Surgery Medical Management. Obes Facts. 2017;10 6 597 632. Open 3. Jeffrey I Mechanick, Caroline Apovian, Stacy Brethauer et al. Clinical Practice Guidelines for the Perioperative Nutrition, Metabolic, and Nonsurgical Support of Patients Undergoing Bariatric Procedures 2019 Update: Cosponsored by American Association of Clinical Endocrinologists/American College of Endocrinology, The Obesity Society, American Society for Metabolic and Bariatric Surgery, Obesity Medicine Association, and American Society of Anesthesiologists. Obesity Silver Spring). 2020 Apr;28 4 O1 O58. Open https://web.pathway.md/diseases/recaTzMBAx5lBjH1o 4/4 |
Guideline sources The following summarized guidelines for the evaluation and management of duodenal trauma are prepared by our editorial team based on guidelines from the World Society of Emergency Surgery (WSES/AAST 2019). 1 Guidelines 1. Diagnostic investigations Ultrasound: Obtain extended FAST for detecting free fluid and solid organ injury. A Do not obtain routine ultrasound for the diagnosis of duodeno-pancreatic trauma. Consider obtaining contrast-enhanced ultrasound in stable patients with trauma with suspected pancreatic injury. D Computed tomography: obtain CT with IV contrast for the diagnosis of duodeno-pancreatic injuries in hemodynamically stable or stabilized patients with trauma. A Show 3 more Abdominal radiography: do not obtain abdominal plain films using water-soluble contrast in the early trauma scenario. D https://web.pathway.md/diseases/recl8ELt3MJZBACRX 1/4 6/29/23, 10:23 PM Duodenal trauma Pathway 2. Diagnostic procedures Peritoneal lavage: recognize that diagnostic peritoneal lavage does not improve the specificity of diagnosing duodeno-pancreatic injury, and it is sensitive but not specific for biliary tract injury. B 3. Medical management Non-operative management: use hemodynamic stability as the key factor in determining management strategy in patients with duodenal injury. B Show 4 more 4. Surgical interventions Exploratory laparotomy: perform exploratory laparotomy in hemodynamically unstable (WSES class IV) patients with a positive extended FAST. A Show 2 more Indications for surgery: perform immediate operative intervention in hemodynamically unstable (WSES class IV) patients and in patients with peritonitis, bowel evisceration, or impalement. B Show 4 more 5. Specific circumstances Pediatric patients: Manage pediatric patients with duodenal-pancreatic trauma by specialists with specific skills and only in trauma centers. B Obtain ultrasound with or without contrast enhancement as the diagnostic modality of choice for follow-up imaging in pediatric patients. Prefer MRI if cross-sectional imaging is required. B Pregnant patients: consider obtaining MRCP wherever available as the diagnostic modality of choice for new-onset signs and symptoms in pregnant patients. B Patients with pancreatic injury (evaluation): consider obtaining MRCP as a second-line noninvasive diagnostic modality to definitely rule out pancreatic parenchymal and pancreatic ductal injuries. Consider obtaining MRCP with hepatobiliary contrast for the diagnosis of suspected biliary injuries. B Show 4 more Patients with pancreatic injury (non-operative management): offer nonoperative management in all hemodynamically stable or stabilized patients with minor pancreatic injury (WSES class I, AAST grade I and some grade II) and gallbladder hematomas without perforation (WSES class I, AAST grade I) in the absence of other abdominal injuries requiring surgery. B Show 5 more https://web.pathway.md/diseases/recl8ELt3MJZBACRX 2/4 6/29/23, 10:23 PM Duodenal trauma Pathway Patients with pancreatic injury (operative management): consider performing drainage in patients with WSES class I (AAST grade I and some grade II) pancreatic injury found during exploratory laparotomy. C Show 8 more 6. Follow-up and surveillance Serial clinical assessment: perform serial clinical examination during follow-up after biliary and pancreatic-duodenal trauma. B Serial imaging assessment: Decide on the need and choice of follow-up imaging after discharge based on clinical symptoms (onset of abdominal distention, tenderness, fever, vomiting, jaundice) and in a multidisciplinary setting given the complexity and variability of traumatic injuries. B Obtain CT as the first-line follow-up imaging for new-onset signs and symptoms in adult patients. B Clinical findings Symptoms Past events Abdominal pain Motor vehicle accident Back pain Abdominal exam Epigastric pain Nausea Abdominal distension Vomiting Abdominal tenderness Peritoneal findings Vital signs Hypotension Signs of shock Imaging findings Free intraperitoneal fluid Pneumoperitoneum Studies 2023 Antimicrobial prophylaxis in pancreatoduodenectomy In adults undergoing open pancreatoduodenectomy for any indication, piperacillin-tazobactam was superior to cefoxitin with respect to the rate of postoperative surgical site infection within 30 days. Michael I D'Angelica et al. JAMA. 2023 May 9. https://web.pathway.md/diseases/recl8ELt3MJZBACRX 3/4 6/29/23, 10:23 PM Duodenal trauma Pathway References 1. Federico Coccolini, Leslie Kobayashi, Yoram Kluger et al. Duodeno-pancreatic and extrahepatic biliary tree trauma: WSES AAST guidelines. World J Emerg Surg. 2019 Dec 11;14 56. Open https://web.pathway.md/diseases/recl8ELt3MJZBACRX 4/4 |
Guideline sources The following summarized guidelines for the management of dupuytren's contracture (DC) are prepared by our editorial team based on guidelines from the Dutch Multidisciplinary Guideline (DMG 2022) and the German Society of Radiation Oncology (DEGRO 2015). 1 2 Guidelines 1. Medical management Conservative management: insufficient evidence to recommend noninvasive treatments as primary therapy in patients with primary DC. I 2. Therapeutic procedures Collagenase injections: administer collagenase injections in patients with DC only in clinical trials. E Radiotherapy: As per DMG 2022 guidelines, offer radiotherapy in patients with DC only in clinical trials. E As per DEGRO 2015 guidelines, offer radiotherapy in patients with DC in the earlier nodular stages (N and N/I). B https://web.pathway.md/diseases/receui5VgIY6u1cno 1/3 6/29/23, 10:23 PM Dupuytren's contracture Pathway 3. Perioperative care Preoperative splinting: insufficient evidence to recommend preoperative orthosis fabrication in patients with DC. I Preoperative external fixation: insufficient evidence to recommend external fixation in patients with DC. I Perioperative arthrolysis: insufficient evidence to recommend perioperative arthrolysis in cases where a flexion contracture remains after partial fasciectomy. I 4. Surgical interventions Indications for surgery: Inform patients of the following before offering surgery: surgery cannot result in a completely straight finger or removal of the entire affected fascia wound healing takes time, and scar tissue is to be anticipated intensity and duration of the postoperative period possibility of recurrence. E Reconsider performing surgery in case of the following relative contraindications: immunosuppressive therapy anticoagulant therapy smoking diabetes mellitus vascularly compromised upper extremities. E Partial fasciectomy: Perform partial fasciectomy as first-line treatment in patients with DC. E Perform partial fasciectomy in patients with primary DC with a positive tabletop test. E Dermofasciectomy: Perform dermofasciectomy when the skin above the strand cannot be saved and in cases of persistent recurrence. E Consider performing dermofasciectomy in young patients with a strong diathesis. E Percutaneous needle fasciotomy: consider performing percutaneous needle fasciotomy in elderly patients with a palpable strand, as well as in relatively younger patients with a palpable strand if they wish for a minimally invasive treatment while accepting the higher recurrence rate. E Show 2 more Segmental fasciectomy: insufficient evidence to recommend segmental fasciectomy in patients with DC. I Radical fasciectomy: insufficient evidence to recommend radical fasciectomy in patients with DC. I Salvage techniques: consider offering salvage techniques in patients with severe recurrent contracture when partial fasciectomy does not lead to improvement and in cases with vascular https://web.pathway.md/diseases/receui5VgIY6u1cno 2/3 6/29/23, 10:23 PM Dupuytren's contracture Pathway insufficiency or damaged sensation. E 5. Follow-up and surveillance Postoperative rehabilitation: inform patients about the intensity of the postoperative protocol, the duration of wound healing and scar formation, and the cost coverage of hand therapy. E Show 3 more Clinical findings Past medical history Medication history Frozen shoulder Anticonvulsants Human immunodeficiency virus infection Musculoskeletal exam Ledderhose disease Digital flexion contracture Peyronie's disease Garrod's pads Palmar bands Social history Palmar nodules Alcohol consumption tabletop test Manual labor Occupational exposure to vibration Tobacco use Integument exam Palmar skin thickening References 1. Marius A Kemler, Robert S de Wijn, Annet L van Rijssen et al. Dutch Multidisciplinary Guideline on Dupuytren Disease. J Hand Surg Glob Online. 2022 Dec 24;5 2 178 183. Open 2. M Heinrich Seegenschmiedt, Oliver Micke, Marcus Niewald et al. DEGRO guidelines for the radiotherapy of non-malignant disorders : part III hyperproliferative disorders. Strahlenther Onkol. 2015 Jul;191 7 541 8. Open https://web.pathway.md/diseases/receui5VgIY6u1cno 3/3 |
Guideline sources The following summarized guidelines for the evaluation and management of dyslipidemia are prepared by our editorial team based on guidelines from the American Diabetes Association (ADA 2023), the Canadian Pediatric Cardiology Association (CPCA/CCS 2022), the Heart Failure Society of America (HFSA/AHA/ACC 2022), the Canadian Cardiovascular Society (CCS 2021; 2018; 2016), the European Society of Cardiology (ESC 2021), the Endocrine Society (ES 2020), the European Society of Cardiology (ESC/EAS 2020), the United States Department of Defense (DoD/VA 2020), the American College of Preventive Medicine (ACPM/ADA/PCNA/ABC/ASPC/AAPA/AGS/AHA/ACC/APhA 2019), the U.S. Preventive Services Task Force (USPSTF 2016), and the Society of Cardiovascular Computed Tomography (SCCT 2013). 1 2 3 4 5 6 7 8 9 10 12 13 14 15 15 16 17 18 Definition Dyslipidemia is a disorder of lipid metabolism characterized by elevated LDL cholesterol, decreased HDL cholesterol and/or increased triglycerides, which contributes to the development of atherosclerosis. 15 Epidemiology https://web.pathway.md/diseases/receTcWVpm18Q5oo1 1/19 6/29/23, 10:23 PM Dyslipidemia Pathway p gy Primary dyslipidemia is due to genetic abnormalities, whereas secondary dyslipidemia is multifactorial, and is associated with obesity, physical inactivity, high-carbohydrate and high-fat diets, smoking, alcohol use, uncontrolled diabetes mellitus, hypothyroidism, renal failure, cholestatic liver disease, nephrotic syndrome, and various drugs (corticosteroids, progestogens, androgenic steroids, thiazide diuretics, beta blockers, oral estrogens, retinoic acid derivatives). 15 Pathophysiology In the United States, an estimated 53% of adults have at least one lipid abnormality: 27% have elevated LDL cholesterol, 23% have decreased HDL cholesterol, and 30% have increased triglycerides. 16 Disease course Lipid abnormalities contribute to the formation of atherosclerotic plaque, leading to an increased risk of CVD, stroke, and peripheral arterial disease. 17 Prognosis and risk of recurrence Treatment with statins is associated with a relative reduction in the risk of major adverse vascular events of 22% in men and 16% in women for every 1.0 mmol/L (38.6 mg/dL) reduction in LDL cholesterol. 18 Calculator DLCN criteria for familial hyperc Guidelines 1. Screening and diagnosis Indications for screening, adults: As per CCS 2021 guidelines: Obtain non-HDL cholesterol or ApoB over LDL cholesterol as the preferred lipid parameter for screening in any patient with triglycerides > 1.5 mmol/L. A Measure lipoprotein(a) level once in a person's lifetime as a part of the initial lipid screening. A As per PCNA/ASPC/APhA/AGS/ADA/ACPM/ABC/AAPA/ACC/AHA 2019 guidelines: Measure a fasting or a nonfasting plasma lipid profile to estimate CVD risk and document baseline LDL cholesterol in adult patients 20 years old not being on lipid-lowering therapy. B Consider measuring fasting lipid profile in adult patients 20 years old with a family history of premature atherosclerotic vascular disease or genetic hyperlipidemia, in order to aid in https://web.pathway.md/diseases/receTcWVpm18Q5oo1 2/19 6/29/23, 10:23 PM Dyslipidemia Pathway the understanding and identification of familial lipid disorders. C As per CCS 2016 guidelines: Screen for dyslipidemia with nonfasting lipid and lipoprotein testing in adult patients undergoing cardiovascular risk assessment. A Complete a cardiovascular risk assessment every 5 years in patients 40-75 years old using the modified Framingham Risk Score or Cardiovascular Life Expectancy Model. A Indications for screening (pediatrics): insufficient evidence to assess the risks and benefits of screening for lipid disorders in pediatric and adolescent individuals (< 20 years old). I 2. Classification and risk stratification Risk assessment: as per ESC 2020 guidelines, obtain total risk estimation using a risk estimation system such as SCORE in asymptomatic patients > 40 years old without evidence of CVD, diabetes mellitus, CKD or hypercholesterolemia, or with LDL cholesterol level of > 190 mg/dL (> 4.9 mmoI/L). B Show 4 more 3. Diagnostic investigations Lipid profile, tests: As per EAS/ESC 2020 guidelines, obtain LDL cholesterol as the primary lipid analysis method for screening, diagnosis, and management. B Show 4 more As per PCNA/ASPC/APhA/AGS/ADA/ACPM/ABC/AAPA/ACC/AHA 2019 guidelines: Measure either a fasting or a nonfasting plasma lipid profile in 20 years old patients and not on lipid-lowering therapy to estimate ASCVD risk and to document baseline LDL cholesterol. B Consider measuring direct LDL cholesterol or modified LDL cholesterol estimate to improve accuracy over the Friedewald formula, in adult patients with a LDL cholesterol level < 70 mg/dL (< 1.8 mmol/L). C As per CCS 2016 guidelines, consider using non-HDL cholesterol levels as an alternative to LDL cholesterol for the evaluation of cardiovascular risk in adult patients. B Lipid profile, fasting state: As per PCNA/ASPC/APhA/AGS/ADA/ACPM/ABC/AAPA/ACC/AHA 2019 guidelines, consider obtaining a repeat lipid profile in the fasting state in patients with an initial nonfasting lipid profile showing triglyceride levels 400 mg/dL ( 4.5 mmol/L). B As per CCS 2016 guidelines, consider measuring lipid and lipoprotein levels in the fasting state in patients with a history of triglyceride levels > 400 mg/dL (> 4.5 mmol/L). C Apolipoprotein B: As per ESC 2020 guidelines, obtain ApoB for risk assessment particularly in patients with: high triglycerides levels very low LDL cholesterol levels https://web.pathway.md/diseases/receTcWVpm18Q5oo1 3/19 6/29/23, 10:23 PM Dyslipidemia Pathway metabolic syndrome obesity. B Show 2 more As per CCS 2016 guidelines, consider using ApoB levels as an alternative to LDL cholesterol for the evaluation of cardiovascular risk in adult patients. B Lipoprotein, a: As per EAS/ESC 2020 guidelines: Consider obtaining lipoprotein(a) at least once in each adult person's lifetime to identify patients with very high inherited lipoprotein(a) levels > 180 mg/dL (> 430 mmol/L) having a lifetime risk of ASCVD equivalent to the risk associated with heterozygous familial hypercholesterolemia. C Consider obtaining lipoprotein(a) in selected patients with a family history of premature CVD, and for reclassification in patients at borderline between moderate and high risk. C As per CCS 2016 guidelines, consider using lipoprotein A levels to refine risk assessment in patients with an intermediate Framingham Risk Score, or a family history of premature coronary artery disease. C Screening for hypothyroidism: evaluate for and rule out hypothyroidism as the cause of hyperlipidemia before initiating lipid-lowering therapy in patients with hyperlipidemia. A Screening for anabolic steroid use: obtain clinical or biochemical evaluation for anabolic steroid abuse in patients with low high-density lipoprotein (< 30 mg/dL; 0.8 mmol/L), especially in the absence of hypertriglyceridemia. E Coronary computed tomography: As per CCS 2021 guidelines, consider obtaining CAC screening using CT in asymptomatic adults 40 years of age and at intermediate risk (Framingham Risk Score 10-20%), if treatment decisions are uncertain. B Show 2 more As per ESC 2020 guidelines, consider obtaining coronary CT for the assessment of CAC score as a risk modifier in the cardiovascular risk assessment in asymptomatic patients at low or moderate risk. C As per SCCT 2013 guidelines, avoid obtaining CAC scoring for screening purposes in low- risk asymptomatic individuals, except in those with a family history of premature coronary artery disease. D Arterial ultrasound: consider obtaining arterial ultrasound to assess arterial (carotid and/or femoral) plaque burden as a risk modifier in patients at low or moderate risk. C 4. Medical management Treatment targets: As per ESC 2020 guidelines, consider targeting LDL cholesterol level of < 116 mg/dL (< 3.0 mmol/L) in low risk patients. B Show 3 more As per CCS 2016 guidelines, use a treat-to-target approach to mitigate the risk of CVD. B https://web.pathway.md/diseases/receTcWVpm18Q5oo1 4/19 6/29/23, 10:23 PM Dyslipidemia Pathway Show 2 more Statins: As per CCS 2021 guidelines, initiate high-intensity statin therapy in addition to appropriate health behavior modifications for secondary prevention in patients with CVD. Administer the maximally tolerated statin dose in patients not tolerating a high-intensity statins. A As per ESC 2020 guidelines, initiate a high-intensity statin up to the highest tolerated dose to reach the goals set for the specific level of risk. A As per AHA 2019 guidelines, consider using CAC score in the decision to withhold, postpone or initiate statin therapy in intermediate risk or selected borderline risk adult patients, if the decision about statin use remains uncertain. C Show 4 more As per CCS 2016 guidelines, initiate statin therapy in patients with the following high risk conditions to decrease the risk of CVD events and mortality: clinical atherosclerosis AAA most cases of diabetes mellitus CKD (> 50 years old patients) LDL cholesterol 190 mg/dL ( 5.0 mmol/L). A Show 4 more Ezetimibe: As per CCS 2021 guidelines, intensify lipid-lowering therapy with ezetimibe and/or PCSK9 inhibitor therapy for secondary prevention in all patients with CVD, if LDL cholesterol remains 1.8 mmol/L (or non-HDL cholesterol 2.4 mmol/L or ApoB 0.7 g/L) while receiving the maximally tolerated statin dose. A Landmark trials: IMPROVE-IT In patients who had been hospitalized for an acute coronary syndrome within the preceding 10 days and had LDL cholesterol levels of 50 to 100 mg/dL, simvastatin- ezetimibe was superior to simvastatin monotherapy once daily with respect to death from cardiovascular causes, major coronary event, or nonfatal stroke, Kaplan-Meier event rate at 7 years. Cannon CP et al. N Engl J Med. 2015 Jun 18. As per ESC 2020 guidelines: Add ezetimibe to statins if the goals are not achieved with the maximum tolerated dose of statins. B Consider initiating ezetimibe if statins are not tolerated at any dosage, even after rechallenge. C PCSK9 inhibitors: As per CCS 2021 guidelines: https://web.pathway.md/diseases/receTcWVpm18Q5oo1 5/19 6/29/23, 10:23 PM Dyslipidemia Pathway Intensify lipid-lowering therapy with a PCSK9 inhibitor (evolocumab or alirocumab) with or without the additional use of ezetimibe for secondary prevention in patients with CVD shown to derive the largest benefit from PCSK9 inhibitor therapy, if LDL cholesterol remains 1.8 mmol/L (or non-HDL cholesterol 2.4 mmol/L or ApoB 0.7 g/L) while receiving the maximally tolerated statin dose. B Intensify lipid-lowering therapy with a PCSK9 inhibitor and/or ezetimibe for secondary prevention in all patients with CVD, if LDL cholesterol remains 1.8 mmol/L (or non-HDL cholesterol 2.4 mmol/L or ApoB 0.7 g/L) while receiving the maximally tolerated statin dose. Initiate PCSK9 inhibitor therapy if ezetimibe is used initially and LDL cholesterol remains 1.8 mmol/L (or non-HDL cholesterol 2.4 mmol/L or ApoB 0.7 g/L). A Updated evidence: FOURIER-OLE In patients with a history of established ASCVD and a fasting low-density lipoprotein cholesterol level 70 mg/dL while on statin, evolocumab was superior to placebo in parent study with respect to the composite of cardiovascular death, myocardial infarction, stroke, hospitalization for unstable angina, or coronary revascularization. Michelle L O'Donoghue et al. Circulation. 2022 Oct 11. As per ESC 2020 guidelines, consider adding PCSK9 inhibitor for primary prevention in very high risk patients if the LDL cholesterol goal is not achieved with a maximum tolerated dose of a statin and ezetimibe. C Show 2 more Landmark trials: FOURIER In patients with ASCVD and LDL cholesterol 70 mg/dL (1.8 mmol/L) who were receiving statin therapy, evolocumab was superior to placebo with respect to major cardiovascular events. Sabatine MS et al. N Engl J Med. 2017 May 4. Icosapent ethyl: As per CCS 2021 guidelines, initiate icosapent ethyl to decrease the risk of cardiovascular events in patients with ASCVD or with diabetes and 1 cardiovascular risk factors having an elevated fasting triglyceride level of 1.5-5.6 mmol/L despite treatment with maximally tolerated statin therapy. A As per VA 2020 guidelines: Consider offering icosapent ethyl for secondary prevention to reduce cardiovascular morbidity and mortality in patients on statin therapy with persistently elevated fasting triglycerides > 150 mg/dL. C Insufficient evidence to recommend for or against icosapent ethyl for primary prevention in patients on statin therapy with persistently elevated fasting triglycerides. I Fibrates: https://web.pathway.md/diseases/receTcWVpm18Q5oo1 6/19 6/29/23, 10:23 PM Dyslipidemia Pathway As per VA 2020 guidelines, avoid adding fibrates to statins for primary or secondary prevention. D As per CCS 2016 guidelines, avoid using fibrates to prevent cardiovascular events in patients achieved LDL cholesterol targets on statin therapy. D Bile acid sequestrants: As per ESC 2020 guidelines, consider adding a bile acid sequestrant if the goal is not achieved with statins. C As per CCS 2016 guidelines, consider initiating bile acid sequestrants to lower LDL cholesterol in high-risk patients with LDL cholesterol levels remaining above target despite statin treatment with or without ezetimibe therapy. C Niacin: As per VA 2020 guidelines, avoid using niacin (supplements or prescriptions) for primary or secondary prevention. D As per CCS 2016 guidelines, avoid using niacin to prevent CVD in patients achieved LDL cholesterol targets with statin therapy. D Bempedoic acid: insufficient evidence to recommend for or against using bempedoic acid with or without statins for either primary or secondary prevention. I Updated evidence: CLEAR Outcomes In patients who were unable or unwilling to take statins owing to unacceptable adverse effects and had or were at high risk for CVD, bempedoic acid was superior to placebo with respect to major adverse cardiovascular events. Steven E Nissen et al. N Engl J Med. 2023 Apr 13. 5. Nonpharmacologic interventions Dietary modifications: As per VA 2020 guidelines, advise following a dietitian-led Mediterranean diet for primary and secondary prevention of CVD. B As per CCS 2016 guidelines, advise all patients to follow healthy eating and adopt the Mediterranean dietary pattern in order to reduce cardiovascular risk. A Show 2 more Physical activity: As per VA 2020 guidelines, advise practicing regular aerobic physical activity of any intensity and duration for primary and secondary prevention of CVD. B As per CCS 2016 guidelines, advise engaging in at least 150 minutes of moderate- to vigorous-intensity aerobic physical activity per week, in bouts of 10 minutes or more, in order to reduce the risk of CVD in adult patients. A Smoking cessation: advise abstaining from smoking in order to reduce the risk of CVD. A https://web.pathway.md/diseases/receTcWVpm18Q5oo1 7/19 6/29/23, 10:23 PM Dyslipidemia Pathway Omega-3 fatty acid supplements: As per CCS 2021 guidelines, do not use OTC omega-3 PUFA supplements to reduce CVD risk. D As per VA 2020 guidelines, avoid using omega-3 fatty acids dietary supplements to reduce CVD risk for primary or secondary prevention. D Other supplements: As per VA 2020 guidelines, insufficient evidence to recommend for or against the use of fiber, garlic, ginger, green tea, and red yeast rice supplements to reduce cardiovascular risks. I As per CCS 2016 guidelines, avoid prescribing vitamins, minerals, or supplements to prevent statin-associated myalgias. D 6. Specific circumstances Female patients: As per CCS 2021 guidelines, obtain screening with a complete lipid panel in the late postpartum period in female patients with a pregnancy complication such as the following as they have a higher risk of premature CVD and stroke with onset 10-15 years after index delivery: hypertensive disorders of pregnancy gestational diabetes preterm birth stillbirth low birth weight infant placental abruption. B Show 2 more As per ES 2020 guidelines, initiate statin therapy rather than hormone therapy for the treatment of dyslipidemia in postmenopausal patients. B Show 2 more As per ESC 2020 guidelines, do not use statins in premenopausal patients with diabetes mellitus planning pregnancy or not using adequate contraception. D As per AHA 2019 guidelines, take into account conditions specific to female patients, such as premature menopause and history of pregnancy-associated disorders such as hypertension, preeclampsia, gestational diabetes mellitus, small-for-gestational-age infants, and preterm deliveries, when discussing lifestyle intervention and the potential for benefit of statin therapy. B Show 2 more Elderly patients: As per ESC 2020 guidelines, initiate statin therapy for primary prevention, according to the level of risk, in older patients > 65 and 75 years old. A Show 3 more As per AHA 2019 guidelines, consider initiating a moderate-intensity statin in 75 years old patients with a LDL cholesterol level of 70-189 mg/dL. C https://web.pathway.md/diseases/receTcWVpm18Q5oo1 8/19 6/29/23, 10:23 PM Dyslipidemia Pathway Show 2 more Pediatric patients, screening: As per CPCA/CCS 2022 guidelines, obtain universal lipid screening (fasting or nonfasting, non-HDL cholesterol or LDL cholesterol) within the first decade of life (after 2 years old), coupled with cascade screening for identified cases of probable/definite familial hypercholesterolemia or other monogenic lipid disorders. Consider obtaining selective screening at any time in pediatric patients with identified cardiovascular risk factors or risk conditions or a positive family history of premature CVD or dyslipidemia. E As per PCNA/ASPC/APhA/AGS/ADA/ACPM/ABC/AAPA/ACC/AHA 2019 guidelines, consider obtaining fasting lipid profile or nonfasting non-HDL cholesterol once between the ages of 9 and 11 years, and again between the ages of 17 and 21 years, to detect moderate-to-severe lipid abnormalities in pediatric and adolescent patients without cardiovascular risk factors or family history of early CVD. C Show 3 more As per USPSTF 2016 guidelines, insufficient evidence to assess the risks and benefits of screening for lipid disorders in pediatric and adolescent individuals (< 20 years old). I Pediatric patients (clinical assessment): elicit a thorough history and perform a physical examination, with additional investigations obtained as needed, to exclude secondary causes of pediatric dyslipidemia. E Pediatric patients, genetic testing: As per CPCA/CCS 2022 guidelines, consider obtaining genetic testing, when accessible, to achieve definitive diagnoses of familial hypercholesterolemia or other genetic dyslipidemias. Consider establishing clinical diagnosis independent of genetic testing using available non- genetic criteria. E As per EAS/ESC 2020 guidelines, obtain testing for familial hypercholesterolemia in pediatric patients from the age of 5, or earlier if homozygous familial hypercholesterolemia is suspected. B Pediatric patients, lifestyle modifications: As per CPCA/CCS 2022 guidelines, offer lifestyle and dietary management as the first-line treatment strategy in nearly all patients with pediatric dyslipidemia. Continue lifestyle and dietary management if lipid-lowering medications are started. E As per EAS/ESC 2020 guidelines, educate pediatric patients with familial hypercholesterolemia to adopt a proper diet. B As per PCNA/ASPC/APhA/AGS/ADA/ACPM/ABC/AAPA/ACC/AHA 2019 guidelines: Counsel on lifestyle modifications for lowering LDL cholesterol in pediatric and adolescent patients with lipid abnormalities. B Advise intensifying lifestyle modifications, including moderate caloric restriction and regular aerobic physical activity, in pediatric and adolescent patients with lipid disorders related to obesity. A Pediatric patients, statin therapy: As per CPCA/CCS 2022 guidelines: Decide on the need for pharmacotherapy based on the average of results from at least 2 fasting lipid profiles obtained at least 2 weeks but no more than 3 months apart. E https://web.pathway.md/diseases/receTcWVpm18Q5oo1 9/19 6/29/23, 10:23 PM Dyslipidemia Pathway Consider initiating statin therapy beginning at age 8-12 years when LDL cholesterol remains above specific treatment thresholds despite lifestyle management. E As per EAS/ESC 2020 guidelines, initiate statin therapy in pediatric patients with familial hypercholesterolemia from 8-10 years of age. B As per PCNA/ASPC/APhA/AGS/ADA/ACPM/ABC/AAPA/ACC/AHA 2019 guidelines, consider initiating statin therapy in > 10 years old pediatric and adolescent patients with LDL cholesterol level persistently 190 mg/dL ( 4.9 mmol/L) or 160 mg/dL ( 4.1 mmol/L) with a clinical presentation consistent with familial hypercholesterolemia not responding adequately to 3-6 months of lifestyle therapy. C Pediatric patients (management of hypertriglyceridemia): consider initiating pharmacotherapy in addition to strict dietary management, including omega-3 fatty acids or fibrates, and offering evaluation and management by a lipid specialist in patients with persistent hypertriglyceridemia (2.3-5.5 mmol/L) despite lifestyle interventions or severe (> 5.5 mmol/L) hypertriglyceridemia at diagnosis. E Pediatric patients, treatment targets: As per CPCA/CCS 2022 guidelines, incorporate routine safety monitoring (for hepatic toxicity and myopathy) and LDL cholesterol treatment targets with statin therapy: low-risk conditions: LDL cholesterol < 2.6 mmol/L high-risk conditions: LDL cholesterol < 3.4 mmol/L or 50% reduction. E As per EAS/ESC 2020 guidelines, target LDL cholesterol level of < 135 mg/dL (< 3.5 mmol/L) in > 10 years old pediatric patients with familial hypercholesterolemia. B Pediatric patients (indications for referral): consider referring patients to a pediatric lipid specialist to facilitate lifestyle or pharmacotherapy management or if there is marked dyslipidemia at diagnosis (LDL cholesterol 4.1 mmol/L or triglyceride levels 5.5 mmol/L) or dyslipidemia in the setting of risk factors or at-risk conditions. E Patients with obesity: assess components of the metabolic syndrome and body fat distribution to accurately determine the level of CVD risk in patients with obesity. E Show 4 more Patients with severe hypercholesterolemia: initiate maximally tolerated statin therapy in 20- 75 years old patients with LDL cholesterol level of 190 mg/dL ( 4.9 mmol/L). B Show 5 more Patients with hypertriglyceridemia: As per ES 2020 guidelines, initiate pharmacologic treatment as adjunct to dietary modifications and exercise to prevent pancreatitis in adult patients with fasting triglyceride levels > 500 mg/dL (5.6 mmol/L). B Show 4 more As per ESC 2020 guidelines, initiate statins as first-line therapy to reduce cardiovascular risk in high risk patients with hypertriglyceridemia (triglyceride levels > 200 mg/dL or > 2.3 mmol/L). B Show 3 more As per AHA 2019 guidelines, address and treat lifestyle factors (such as obesity and metabolic syndrome), secondary factors (such as diabetes mellitus, chronic liver or kidney https://web.pathway.md/diseases/receTcWVpm18Q5oo1 10/19 6/29/23, 10:23 PM Dyslipidemia Pathway disease and/or nephrotic syndrome, hypothyroidism), and medications that increase triglycerides in 20 years old adult patients with moderate hypertriglyceridemia (fasting or nonfasting triglycerides 175-499 mg/dL or 2.0-5.6 mmol/L). B Show 3 more Patients with familial hypercholesterolemia, screening: As per EAS/ESC 2020 guidelines, obtain family screening for familial hypercholesterolemia once the index case is diagnosed. B As per CCS 2018 guidelines: Implement cascade screening (lipid profile) protocols at the local, provincial and national level and offer in first-degree relatives of patients with familial hypercholesterolemia. B Consider obtaining universal cholesterol level screening for the detection of familial hypercholesterolemia in children with reverse cascade screening of parents when warranted. C Patients with familial hypercholesterolemia, diagnosis: As per EAS/ESC 2020 guidelines, suspect the diagnosis of familial hypercholesterolemia in patients with: premature coronary artery disease (males < 55 years old; females < 60 years old) severely elevated LDL cholesterol in adults (> 190 mg/dL or > 5 mmol/L) and in pediatrics (> 150 mg/dL or > 4 mmol/L) relatives having premature fatal or non-fatal CVD relatives having tendon xanthomas. B Show 2 more As per CCS 2018 guidelines: Define familial hypercholesterolemia using the Dutch Lipid Clinic Network Criteria, Simon Broome Registry or Familial Hypercholesterolemia Canada definition. A Offer genetic testing, when available, to complement a diagnosis of familial hypercholesterolemia and enable cascade screening. A Patients with familial hypercholesterolemia (CVD risk stratification): do not use current risk calculators (Framingham Risk Score, Pooled Cohort Equation, European SCORE) to determine cardiovascular risk in patients with familial hypercholesterolemia. D Show 2 more Patients with familial hypercholesterolemia, management: As per CCS 2021 guidelines: Initiate PCSK9 inhibitor (alirocumab or evolocumab) to lower LDL cholesterol level in patients with heterozygous familial hypercholesterolemia without clinical ASCVD, if LDL cholesterol remains above the target (LDL cholesterol 2.5 mmol/L or < 50% reduction from baseline or ApoB 0.85 mg/dL or non-HDL cholesterol 3.2 mmol/L) despite maximally tolerated statin therapy with or without ezetimibe therapy. A Initiate PCSK9 inhibitor (alirocumab or evolocumab) in patients with heterozygous familial hypercholesterolemia and ASCVD, if LDL cholesterol remains above the threshold 1.8 mmol/L (or ApoB 0.7 mg/dL or non-HDL cholesterol 2.4 mmol/L) despite maximally tolerated statin therapy with or without ezetimibe. A https://web.pathway.md/diseases/receTcWVpm18Q5oo1 11/19 6/29/23, 10:23 PM Dyslipidemia Pathway Updated evidence: FOURIER-OLE In patients with a history of established ASCVD and a fasting low-density lipoprotein cholesterol level 70 mg/dL while on statin, evolocumab was superior to placebo in parent study with respect to the composite of cardiovascular death, myocardial infarction, stroke, hospitalization for unstable angina, or coronary revascularization. Michelle L O'Donoghue et al. Circulation. 2022 Oct 11. As per EAS/ESC 2020 guidelines, treat patients with familial hypercholesterolemia and ASCVD or another major risk factor as very high risk patients, and patients without ASCVD or other risk factors as high risk patients. B Show 3 more As per PCNA/ASPC/APhA/AGS/ADA/ACPM/ABC/AAPA/ACC/AHA 2019 guidelines: Consider adding a PCSK9 inhibitor in 30-75 years old patients with heterozygous familial hypercholesterolemia and LDL cholesterol level of 100 mg/dL ( 2.6 mmol/L) with maximally tolerated statin and ezetimibe therapy. C Insufficient evidence to support addition of a PCSK9 inhibitor in patients with familial hypercholesterolemia without evidence of clinical ASCVD receiving maximally tolerated statin and ezetimibe therapy. I As per CCS 2018 guidelines, consider advising a healthy lifestyle in patients with familial hypercholesterolemia. C Show 8 more As per CCS 2016 guidelines, consider initiating lomitapide and mipomersen exclusively in patients with homozygous familial hypercholesterolemia. C Patients with familial hypercholesterolemia (homozygous FH): refer patients with homozygous familial hypercholesterolemia to a specialized lipid clinic. Obtain complete evaluation for genetic analysis, presence of ASCVD and initiate aggressive lipid-lowering therapies including consideration for extracorporeal LDL cholesterol removal, lomitapide and PCSK9 inhibitors. B Patients with ASCVD, general indications: As per EAS/ESC 2020 guidelines, consider targeting LDL cholesterol level of < 40 mg/dL (< 1.0 mmol/L) in patients with ASCVD experiencing a second vascular event within 2 years (not necessarily of the same type as the first event) while taking maximally tolerated statin- based therapy. C As per PCNA/ASPC/APhA/AGS/ADA/ACPM/ABC/AAPA/ACC/AHA 2019 guidelines, initiate or continue high-intensity statin therapy with the aim of achieving a 50% reduction in LDL cholesterol levels in 75 years old patients with clinical ASCVD. A Show 8 more Patients with ASCVD: as per ACS 2020 guidelines, initiate or continue high-dose statins as early as possible in all patients with acute coronary syndrome without any contraindication or definite history of intolerance, regardless of initial LDL cholesterol levels. A Show 6 more https://web.pathway.md/diseases/receTcWVpm18Q5oo1 12/19 6/29/23, 10:23 PM Dyslipidemia Pathway Patients with ASCVD (ischemic stroke): initiate intensive LDL cholesterol-lowering therapy in patients with a history of ischemic stroke or TIA, particularly recurrent ischemic stroke, as they are at very high risk of ASCVD. A Patients with ASCVD (PAD): initiate lipid-lowering therapy in patients with peripheral arterial disease, including a maximum tolerated dose of statins, plus ezetimibe or a combination with PCSK9, if needed, to reduce the risk of ASCVD events. A Patients with aortic valvular disease: do not initiate lipid-lowering therapy in patients with aortic valvular stenosis without coronary artery disease to slow progression of aortic valve stenosis in the absence of other indications for its use. D Patients with heart failure: As per ACC 2022 guidelines, initiate statins to prevent symptomatic HF and adverse cardiovascular events in patients with a recent or remote history of myocardial infarction or acute coronary syndrome. A As per ESC 2021 guidelines, initiate statins in patients at high risk of CVD or with CVD to prevent or delay the onset of HF and to prevent HF hospitalizations. A As per ESC 2020 guidelines, do not initiate lipid-lowering therapy in patients with HF in the absence of other indications for its use. D As per AHA 2019 guidelines, consider initiating moderate-intensity statin therapy to reduce the occurrence of ASCVD events in patients with HFrEF attributable to ischemic heart disease having a reasonable life expectancy of 3-5 years and not being already on a statin because of ASCVD. C Patients with diabetes mellitus: As per ADA 2023 guidelines, advise lifestyle modifications focusing on weight loss (if indicated), following a Mediterranean style or DASH eating pattern, reducing consumption of saturated fat and trans fat, increasing dietary omega-3 fatty acids, viscous fiber, and plant stanols/sterols intake, and increasing physical activity to improve the lipid profile and reduce the risk of developing ASCVD in patients with diabetes. A Show 15 more As per ES 2020 guidelines, initiate statin therapy in addition to lifestyle modification to reduce cardiovascular risks in adult patients with T2DM and other cardiovascular risk factors. A Show 8 more As per ESC 2020 guidelines, target LDL cholesterol reduction of 50% from baseline and the following LDL cholesterol levels in patients with with T2DM: < 55 mg/dL (< 1.4 mmol/L) in very high risk patients < 70 mg/dL (< 1.8 mmol/L) in high risk patients. A Show 4 more As per AHA 2019 guidelines, initiate moderate-intensity statin therapy in 40-75 years old patients with diabetes mellitus, regardless of estimated 10-year ASCVD risk. A Show 6 more Patients with chronic kidney disease, management: As per EAS/ESC 2020 guidelines, view patients with KDOQI stage 3-5 CKD as high or very high risk patients for ASCVD. A https://web.pathway.md/diseases/receTcWVpm18Q5oo1 13/19 6/29/23, 10:23 PM Dyslipidemia Pathway Show 2 more As per PCNA/ASPC/APhA/AGS/ADA/ACPM/ABC/AAPA/ACC/AHA 2019 guidelines, consider initiating a moderate-intensity statin or moderate-intensity statins combined with ezetimibe in 40-75 years old patients with LDL cholesterol 70-189 mg/dL, a 10-year ASCVD risk of 7.5% and CKD not treated with dialysis or kidney transplantation. C Landmark trials: SHARP In patients with advanced CKD, simvastatin plus ezetimibe was superior to placebo with respect to major atherosclerotic events. Baigent C et al. Lancet. 2011 Jun 25. As per CCS 2016 guidelines, offer statins or a statin/ezetimibe combination to reduce CVD events in 50 years old patients with CKD not treated with dialysis or a kidney transplant. A Landmark trials: SHARP In patients with advanced CKD, simvastatin plus ezetimibe was superior to placebo with respect to major atherosclerotic events. Baigent C et al. Lancet. 2011 Jun 25. Patients on hemodialysis: As per ESC 2020 guidelines, consider continuing statins, ezetimibe or a statin/ezetimibe combination in patients already using these medications at the time of dialysis initiation, particularly in patients with ASCVD. C As per AHA 2019 guidelines: Consider continuing statin therapy in adult patients with CKD initiating dialysis and already receiving statin therapy. C Avoid initiating statin therapy in adult patients with advanced kidney disease requiring dialysis treatment. D Landmark trials: AURORA In patients, 50 to 80 years of age, who were undergoing maintenance hemodialysis, rosuvastatin was not superior to placebo with respect to death from cardiovascular causes, nonfatal myocardial infarction, or nonfatal stroke (events per 100 patient-years). Fellstrom BC et al. N Engl J Med. 2009 Apr 2. As per CCS 2016 guidelines: Consider continuing lipid-lowering therapy in adult patients with dialysis-dependent CKD already receiving it at the time of dialysis initiation. C https://web.pathway.md/diseases/receTcWVpm18Q5oo1 14/19 6/29/23, 10:23 PM Dyslipidemia Pathway Consider avoiding lipid-lowering therapy in adult patients with dialysis-dependent CKD. D Landmark trials: AURORA In patients, 50 to 80 years of age, who were undergoing maintenance hemodialysis, rosuvastatin was not superior to placebo with respect to death from cardiovascular causes, nonfatal myocardial infarction, or nonfatal stroke (events per 100 patient-years). Fellstrom BC et al. N Engl J Med. 2009 Apr 2. Patients with inflammatory diseases and HIV: initiate moderate-intensity statin therapy or high-intensity statin therapy in 40-75 years old patients with LDL cholesterol levels 70-189 mg/dL, a 10-year ASCVD risk 7.5% and chronic inflammatory disorders or human immunodeficiency virus. B Show 2 more Patients with solid organ transplants: consider initiating statins as first-line therapy in patients with solid organ transplants. Initiate statins at low doses with carefully titrating upwards and with caution regarding potential drug-drug interactions, particularly for patients receiving ciclosporin. C Show 2 more Patients with endocrine disorders (evaluation): obtain a lipid panel for the assessment of triglyceride levels and for calculating LDL cholesterol in adult patients with endocrine disorders. B Show 3 more Patients with endocrine disorders (thyroid disease): avoid treating hyperlipidemia until the patient becomes euthyroid in order to more accurately assess the lipid profile in patients with overt hypothyroidism. D Show 2 more Patients with endocrine disorders (Cushing's syndrome): obtain cardiovascular risk assessment and initiate treatment in adult patients with cured Cushing's syndrome as in the general population. E Show 3 more Patients with endocrine disorders (adult growth hormone deficiency): assess lipid profile at diagnosis to screen for dyslipidemia in adult patients with GH deficiency. B Show 2 more Patients with endocrine disorders (acromegaly): consider obtaining usual lipid profile before and after treatment of GH excess in adult patients with acromegaly. C Patients with endocrine disorders (PCOS): Obtain a fasting lipid panel at diagnosis to assess cardiovascular risk in patients with PCOS. B Avoid using lipid-lowering therapies for the treatment of hyperandrogenism or infertility in patients with PCOS. D https://web.pathway.md/diseases/receTcWVpm18Q5oo1 15/19 6/29/23, 10:23 PM Dyslipidemia Pathway Patients with endocrine disorders (testosterone deficiency): consider initiating testosterone therapy as symptomatically indicated and not as an approach to improve dyslipidemia or CVD risk in patients with low testosterone levels. C 7. Patient education Shared decision-making: As per AHA 2019 guidelines, promote shared decision-making when initiating lipid-lowering therapy, and discuss the potential benefits and adverse effects of treatments, as well as the potential for drug-drug interactions. B Show 2 more As per CCS 2016 guidelines, share the results of cardiovascular risk assessment with the patient to support shared decision-making and improve the likelihood of reaching lipid targets. A 8. Follow-up and surveillance Monitoring for adherence to treatment: As per VA 2020 guidelines, consider offering intensified patient care (such as phone calls, emails, patient education, drug regimen simplification) to improve adherence to lipid-lowering medications. C As per AHA 2019 guidelines, assess adherence to changes in lifestyle and effects of LDL cholesterol-lowering medication by measurement of fasting lipids and appropriate safety indicators 4-12 weeks after statin initiation or dose adjustment and every 3-12 months thereafter based on need to assess adherence or safety. A Monitoring for adverse effects of statins: As per AHA 2019 guidelines, conduct a clinician-patient risk discussion before initiation of statin therapy to review net clinical benefit, weighing the potential for ASCVD risk reduction against the potential for statin-associated side effects, statin-drug interactions, and safety, while emphasizing that side effects can be addressed successfully. A Show 8 more As per CCS 2016 guidelines, evaluate all purported statin-associated symptoms systematically incorporating observation during cessation and reinitiation of statins (same or different statin, same or decreased potency, same or decreased frequency of dosing) to identify a tolerated, statin-based therapy for chronic use. B Clinical findings Symptoms Past medical history Ankle swelling Cushing's syndrome Chest pain Diabetes mellitus Difficulty breathing Genetic abnormality https://web.pathway.md/diseases/receTcWVpm18Q5oo1 16/19 6/29/23, 10:23 PM Dyslipidemia Pathway Fainting Human immunodeficiency virus infection Fatigue Hypercholesterolemia Heartburn Hypothyroidism Leg pain IBD Nausea Metabolic disorders Palpitations Obesity Sweats PCOS Swelling Social history Family history Alcohol consumption Genetic predispositions Lab findings serum LDL serum cholesterol serum triglycerides Studies 2023 RACING In adult patients with diabetes mellitus and ASCVD, moderate-intensity statin plus ezetimibe combination therapy was not superior to high-intensity statin monotherapy with respect to the composite of cardiovascular death, major cardiovascular events, or nonfatal stroke at 3 years. |
< 55 mg/dL (< 1.4 mmol/L) in very high risk patients < 70 mg/dL (< 1.8 mmol/L) in high risk patients. A Show 4 more As per AHA 2019 guidelines, initiate moderate-intensity statin therapy in 40-75 years old patients with diabetes mellitus, regardless of estimated 10-year ASCVD risk. A Show 6 more Patients with chronic kidney disease, management: As per EAS/ESC 2020 guidelines, view patients with KDOQI stage 3-5 CKD as high or very high risk patients for ASCVD. A https://web.pathway.md/diseases/receTcWVpm18Q5oo1 13/19 6/29/23, 10:23 PM Dyslipidemia Pathway Show 2 more As per PCNA/ASPC/APhA/AGS/ADA/ACPM/ABC/AAPA/ACC/AHA 2019 guidelines, consider initiating a moderate-intensity statin or moderate-intensity statins combined with ezetimibe in 40-75 years old patients with LDL cholesterol 70-189 mg/dL, a 10-year ASCVD risk of 7.5% and CKD not treated with dialysis or kidney transplantation. C Landmark trials: SHARP In patients with advanced CKD, simvastatin plus ezetimibe was superior to placebo with respect to major atherosclerotic events. Baigent C et al. Lancet. 2011 Jun 25. As per CCS 2016 guidelines, offer statins or a statin/ezetimibe combination to reduce CVD events in 50 years old patients with CKD not treated with dialysis or a kidney transplant. A Landmark trials: SHARP In patients with advanced CKD, simvastatin plus ezetimibe was superior to placebo with respect to major atherosclerotic events. Baigent C et al. Lancet. 2011 Jun 25. Patients on hemodialysis: As per ESC 2020 guidelines, consider continuing statins, ezetimibe or a statin/ezetimibe combination in patients already using these medications at the time of dialysis initiation, particularly in patients with ASCVD. C As per AHA 2019 guidelines: Consider continuing statin therapy in adult patients with CKD initiating dialysis and already receiving statin therapy. C Avoid initiating statin therapy in adult patients with advanced kidney disease requiring dialysis treatment. D Landmark trials: AURORA In patients, 50 to 80 years of age, who were undergoing maintenance hemodialysis, rosuvastatin was not superior to placebo with respect to death from cardiovascular causes, nonfatal myocardial infarction, or nonfatal stroke (events per 100 patient-years). Fellstrom BC et al. N Engl J Med. 2009 Apr 2. As per CCS 2016 guidelines: Consider continuing lipid-lowering therapy in adult patients with dialysis-dependent CKD already receiving it at the time of dialysis initiation. C https://web.pathway.md/diseases/receTcWVpm18Q5oo1 14/19 6/29/23, 10:23 PM Dyslipidemia Pathway Consider avoiding lipid-lowering therapy in adult patients with dialysis-dependent CKD. D Landmark trials: AURORA In patients, 50 to 80 years of age, who were undergoing maintenance hemodialysis, rosuvastatin was not superior to placebo with respect to death from cardiovascular causes, nonfatal myocardial infarction, or nonfatal stroke (events per 100 patient-years). Fellstrom BC et al. N Engl J Med. 2009 Apr 2. Patients with inflammatory diseases and HIV: initiate moderate-intensity statin therapy or high-intensity statin therapy in 40-75 years old patients with LDL cholesterol levels 70-189 mg/dL, a 10-year ASCVD risk 7.5% and chronic inflammatory disorders or human immunodeficiency virus. B Show 2 more Patients with solid organ transplants: consider initiating statins as first-line therapy in patients with solid organ transplants. Initiate statins at low doses with carefully titrating upwards and with caution regarding potential drug-drug interactions, particularly for patients receiving ciclosporin. C Show 2 more Patients with endocrine disorders (evaluation): obtain a lipid panel for the assessment of triglyceride levels and for calculating LDL cholesterol in adult patients with endocrine disorders. B Show 3 more Patients with endocrine disorders (thyroid disease): avoid treating hyperlipidemia until the patient becomes euthyroid in order to more accurately assess the lipid profile in patients with overt hypothyroidism. D Show 2 more Patients with endocrine disorders (Cushing's syndrome): obtain cardiovascular risk assessment and initiate treatment in adult patients with cured Cushing's syndrome as in the general population. E Show 3 more Patients with endocrine disorders (adult growth hormone deficiency): assess lipid profile at diagnosis to screen for dyslipidemia in adult patients with GH deficiency. B Show 2 more Patients with endocrine disorders (acromegaly): consider obtaining usual lipid profile before and after treatment of GH excess in adult patients with acromegaly. C Patients with endocrine disorders (PCOS): Obtain a fasting lipid panel at diagnosis to assess cardiovascular risk in patients with PCOS. B Avoid using lipid-lowering therapies for the treatment of hyperandrogenism or infertility in patients with PCOS. D https://web.pathway.md/diseases/receTcWVpm18Q5oo1 15/19 6/29/23, 10:23 PM Dyslipidemia Pathway Patients with endocrine disorders (testosterone deficiency): consider initiating testosterone therapy as symptomatically indicated and not as an approach to improve dyslipidemia or CVD risk in patients with low testosterone levels. C 7. Patient education Shared decision-making: As per AHA 2019 guidelines, promote shared decision-making when initiating lipid-lowering therapy, and discuss the potential benefits and adverse effects of treatments, as well as the potential for drug-drug interactions. B Show 2 more As per CCS 2016 guidelines, share the results of cardiovascular risk assessment with the patient to support shared decision-making and improve the likelihood of reaching lipid targets. A 8. Follow-up and surveillance Monitoring for adherence to treatment: As per VA 2020 guidelines, consider offering intensified patient care (such as phone calls, emails, patient education, drug regimen simplification) to improve adherence to lipid-lowering medications. C As per AHA 2019 guidelines, assess adherence to changes in lifestyle and effects of LDL cholesterol-lowering medication by measurement of fasting lipids and appropriate safety indicators 4-12 weeks after statin initiation or dose adjustment and every 3-12 months thereafter based on need to assess adherence or safety. A Monitoring for adverse effects of statins: As per AHA 2019 guidelines, conduct a clinician-patient risk discussion before initiation of statin therapy to review net clinical benefit, weighing the potential for ASCVD risk reduction against the potential for statin-associated side effects, statin-drug interactions, and safety, while emphasizing that side effects can be addressed successfully. A Show 8 more As per CCS 2016 guidelines, evaluate all purported statin-associated symptoms systematically incorporating observation during cessation and reinitiation of statins (same or different statin, same or decreased potency, same or decreased frequency of dosing) to identify a tolerated, statin-based therapy for chronic use. B Clinical findings Symptoms Past medical history Ankle swelling Cushing's syndrome Chest pain Diabetes mellitus Difficulty breathing Genetic abnormality https://web.pathway.md/diseases/receTcWVpm18Q5oo1 16/19 6/29/23, 10:23 PM Dyslipidemia Pathway Fainting Human immunodeficiency virus infection Fatigue Hypercholesterolemia Heartburn Hypothyroidism Leg pain IBD Nausea Metabolic disorders Palpitations Obesity Sweats PCOS Swelling Social history Family history Alcohol consumption Genetic predispositions Lab findings serum LDL serum cholesterol serum triglycerides Studies 2023 RACING In adult patients with diabetes mellitus and ASCVD, moderate-intensity statin plus ezetimibe combination therapy was not superior to high-intensity statin monotherapy with respect to the composite of cardiovascular death, major cardiovascular events, or nonfatal stroke at 3 years. Yong-Joon Lee et al. Eur Heart J. 2023 Mar 14. 2023 LODESTAR In patients with a coronary artery disease diagnosis, treat-to-target strategy was noninferior to high-intensity statin strategy with respect to the composite of death, myocardial infarction, stroke, or coronary revascularization at 3 years. Sung-Jin Hong et al. JAMA. 2023 Mar 6. Show 25 more References 1. Anderson TJ, Gr goire J, Pearson GJ et al. 2016 Canadian Cardiovascular Society Guidelines for the Management of Dyslipidemia for the Prevention of Cardiovascular Disease in the Adult. Can J Cardiol. 2016 Nov;32 11 1263 1282. Open 2. Scott M Grundy, Neil J Stone, Alison L Bailey et al. 2018 AHA/ACC/AACVPR/AAPA/ABC/ACPM/ADA/AGS/APhA/ASPC/NLA/PCNA Guideline on the Management of Blood Cholesterol: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. Circulation. 2019 Jun 18;139 25):e1082-e1143. Open 3. Connie B Newman, Michael J Blaha, Jeffrey B Boord et al. Lipid Management in Patients with Endocrine Disorders: An Endocrine Society Clinical Practice Guideline. J Clin Endocrinol Metab. 2020 https://web.pathway.md/diseases/receTcWVpm18Q5oo1 17/19 6/29/23, 10:23 PM Dyslipidemia Pathway Dec 1;105 12):dgaa674. Open 4. Fran ois Mach, Colin Baigent, Alberico L Catapano et al. 2019 ESC/EAS Guidelines for the management of dyslipidaemias: lipid modification to reduce cardiovascular risk. Eur Heart J. 2020 Jan 1;41 1 111 188. Open 5. Glen J Pearson, George Thanassoulis, Todd J Anderson et al. 2021 Canadian Cardiovascular Society Guidelines for the Management of Dyslipidemia for the Prevention of Cardiovascular Disease in Adults. Can J Cardiol. 2021 Aug;37 8 1129 1150. Open 6. Michael Khoury, Jean-Luc Bigras, Elizabeth A Cummings et al. The Detection, Evaluation, and Management of Dyslipidemia in Children and Adolescents: A Canadian Cardiovascular Society/Canadian Pediatric Cardiology Association Clinical Practice Update. Can J Cardiol. 2022 Aug;38 8 1168 1179. Open 7. Patrick G O'Malley, Michael J Arnold, Cathy Kelley et al. Management of Dyslipidemia for Cardiovascular Disease Risk Reduction: Synopsis of the 2020 Updated U.S. Department of Veterans Affairs and U.S. Department of Defense Clinical Practice Guideline. Ann Intern Med. 2020 Nov 17;173 10 822 829. Open 8. Society of Cardiovascular Computed Tomography. Choosing Wisely SCCT recommendations. Choosing Wisely. 2013. Open 9. Liam R Brunham, Isabelle Ruel, Sumayah Aljenedil et al. Canadian Cardiovascular Society Position Statement on Familial Hypercholesterolemia: Update 2018. Can J Cardiol. 2018 Dec;34 12 1553 1563. Open 10. US Preventive Services Task Force, Bibbins-Domingo K, Grossman DC et al. Screening USPSTF for Lipid Disorders in Children and Adolescents: US Preventive Services Task Force Recommendation Statement. JAMA. 2016 Aug 9;316 6 625 33. Open 11. KDIGO Executive Committee. KDIGO Clinical Practice Guideline for Lipid Management in Chronic Kidney Disease. KDIGO. 2013 Nov;3 3 . Open 12. Theresa A McDonagh, Marco Metra, Marianna Adamo et al. 2021 ESC Guidelines for the diagnosis and treatment of acute and chronic heart failure. Eur Heart J. 2021 Sep 21;42 36 3599 3726. Open 13. Paul A Heidenreich, Biykem Bozkurt, David Aguilar et al. 2022 AHA/ACC/HFSA Guideline for the Management of Heart Failure: A Report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines. Circulation. 2022 Apr 1;101161CIR0000000000001063. Open 14. Steven E. Kahn, Cheryl A.M. Anderson, John B. Buse et al. Standards of Care in Diabetes 2023. Diabetes Care. 2023 Jan;46 Supplement_1 S1 S291. Open 15. Kopin L, Lowenstein C. Dyslipidemia. Ann Intern Med. 2017 Dec 5;167 11 ITC81 ITC96. Open 16. Toth PP, Potter D, Ming EE. Prevalence of lipid abnormalities in the United States: the National Health and Nutrition Examination Survey 2003 2006. J Clin Lipidol. 2012 Jul-Aug;6 4 325 30. Open 17. Helkin A, Stein JJ, Lin S et al. Dyslipidemia Part 1 Review of Lipid Metabolism and Vascular Cell Physiology. Vasc Endovascular Surg. 2016 Feb;50 2 107 18. Open 18. Cholesterol Treatment Trialists' CTT Collaboration, Fulcher J, O'Connell R et al. Efficacy and safety of LDL-lowering therapy among men and women: meta-analysis of individual data from 174,000 participants in 27 randomised trials. Lancet. 2015 Apr 11;385 9976 1397 405. Open https://web.pathway.md/diseases/receTcWVpm18Q5oo1 18/19 6/29/23, 10:23 PM Dyslipidemia Pathway 19. Stone NJ, Robinson JG, Lichtenstein AH et al. 2013 ACC/AHA guideline on the treatment of blood cholesterol to reduce atherosclerotic cardiovascular risk in adults: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines. J Am Coll Cardiol. 2014 Jul 1;63 25 Pt B 2889 934. Open 20. Klug E, Raal FJ, Marais AD et al. South African dyslipidaemia guideline consensus statement: 2018 update A joint statement from the South African Heart Association SA Heart) and the Lipid and Atherosclerosis Society of Southern Africa LASSA . S Afr Med J. 2018 Oct 26;108(11b):973 1000. Open 21. Grundy SM, Stone NJ, Guideline Writing Committee for the Cholesterol Guidelines. 2018 Cholesterol Clinical Practice Guidelines: Synopsis of the 2018 American Heart Association/American College of Cardiology/Multisociety Cholesterol Guideline. Ann Intern Med. 2019 Jun 4;170 11 779 783. Open 22. Society for Post-Acute and Long-Term Care Medicine. Choosing Wisely AMDA recommendations. Choosing Wisely. 2013. Open 23. Mach F, Baigent C, Catapano AL et al. Guidelines for the management of dyslipidaemias: lipid modification to reduce cardiovascular risk. Eur Heart J. 2020 Jan 1;41 1 111 188. Open 24. Ramsey LB, Johnson SG, Caudle KE et al. The clinical pharmacogenetics implementation consortium guideline for SLCO1B1 and simvastatin-induced myopathy: 2014 update. Clin Pharmacol Ther. 2014 Oct;96 4 423 8. Open 25. Kristi M Crowe-White, Levi W Evans, Gunter G C Kuhnle et al. Flavan-3-ols and Cardiometabolic Health: First Ever Dietary Bioactive Guideline. Adv Nutr. 2022 Dec 22;13 6 2070 2083. Open https://web.pathway.md/diseases/receTcWVpm18Q5oo1 19/19 |
Guideline sources The following summarized guidelines for the evaluation and management of dysmetabolic hyperferritinemia are prepared by our editorial team based on guidelines from the Metabolic Hyperferritinemia Consensus Group (MHF-CG 2023) and the American Association for the Study of Liver Diseases (AASLD 2018). 1 2 Guidelines 1. Screening and diagnosis Risk factors: Recognize that insulin resistance and features of metabolic dysfunction are associated with specific alterations of iron metabolism regulation, which are epidemiologically linked with organ damage and clinical outcomes. B Recognize that the pathophysiology of this alteration of iron metabolism regulation seems to be triggered by lipotoxicity in the presence of permissive environmental and genetic determinants. B https://web.pathway.md/diseases/recx1YQ2VZ0pesNbl 1/3 6/29/23, 10:24 PM Dysmetabolic hyperferritinemia Pathway Diagnostic criteria: Consider defining the condition of hyperferritinemia related to metabolic dysfunction as metabolic hyperferritinemia. Consider grading the severity of metabolic hyperferritinemia according to serum levels of ferritin thresholds (grades 1-3). C Consider defining dysmetabolic iron overload syndrome as increased hepatic iron stores in patients with metabolic hyperferritinemia. C 2. Diagnostic investigations Serum ferritin levels: Consider measuring serum ferritin levels for noninvasive diagnosis and assessment of iron metabolism alteration, namely thr metabolic hyperferritinemia, associated with glucose and lipid metabolism dysregulation and with hepatic lipid accumulation. B Consider measuring serum ferritin levels after at least 3 months of lifestyle changes, if possible. C Magnetic resonance imaging: consider obtaining a noninvasive estimation of iron concentration in the liver by MRI in the context of a clinical trial, given the initial evidence that serum levels of ferritin might be associated with iron accumulation in tissue in patients with stable metabolic hyperferritinemia. Consider obtaining it in patients with high serum levels of ferritin (grade 2, but in particular, grade 3) and/or additional clinical risk factors for iron overload. Prefer iron concentration in tissues over serum ferritin levels, when available, for grading metabolic hyperferritinemia. B 3. Diagnostic procedures Liver biopsy: As per MHF-CG 2023 guidelines, do not perform a liver biopsy for the diagnosis of metabolic hyperferritinemia or dysmetabolic iron overload syndrome unless otherwise indicated for the management of an associated liver disease or for specific research purposes. D As per AASLD 2018 guidelines, consider performing a liver biopsy in patients with suspected nonalcoholic fatty liver disease, persistently high serum ferritin, and increased iron saturation, especially in the context of homozygote or heterozygote C282Y HFE mutation. E 4. Medical management Management of cardiovascular risk factors: focus the clinical management on lifestyle factors associated with increased risk of cardiometabolic risk factors (such as caloric intake and dietary patterns, alcohol intake, fructose and salt intake, and sedentary lifestyle) and the pharmacological control of cardiovascular risk factors. B Iron depletion therapy: consider initiating iron depletion therapy as an experimental therapy in patients with metabolic hyperferritinemia and dysmetabolic iron overload syndrome in the context of well-powered controlled trials. C https://web.pathway.md/diseases/recx1YQ2VZ0pesNbl 2/3 6/29/23, 10:24 PM Dysmetabolic hyperferritinemia Pathway 5. Therapeutic procedures Blood donation: recognize that blood donation is not contraindicated in patients with metabolic hyperferritinemia with controlled cardiovascular risk factors, without organ damage or other contraindications to phlebotomy. B References 1. Luca Valenti, Elena Corradini, Leon A Adams et al. Consensus Statement on the definition and classification of metabolic hyperferritinaemia. Nat Rev Endocrinol. 2023 Feb 17;1 12. Open 2. Chalasani N, Younossi Z, Lavine JE et al. The diagnosis and management of nonalcoholic fatty liver disease: Practice guidance from the American Association for the Study of Liver Diseases. Hepatology. 2018 Jan;67 1 328 357. Open https://web.pathway.md/diseases/recx1YQ2VZ0pesNbl 3/3 |
Guideline sources The following summarized guidelines for the evaluation and management of dyspepsia are prepared by our editorial team based on guidelines from the American College of Gastroenterology (ACG/CAG 2017). 1 Guidelines 1. Diagnostic investigations Testing for Helicobacter pylori infection: obtain a noninvasive test for H. pylori, and treat if positive in < 60 years old patients with dyspepsia. A 2. Diagnostic procedures Upper gastrointestinal endoscopy: Perform endoscopy to exclude upper gastrointestinal neoplasia in patients 60 years old with dyspepsia. B Do not perform endoscopy to investigate alarm features in patients < 60 years old with dyspepsia to exclude upper gastrointestinal neoplasia. D 3. Medical management https://web.pathway.md/diseases/rectVh3waGB61Fg37 1/2 6/29/23, 10:25 PM Dyspepsia Pathway Proton pump inhibitors: prescribe empirical PPIs in patients < 60 years old with dyspepsia if they are H. pylori-negative or who remain symptomatic after H. pylori eradication therapy. A Prokinetics: offer prokinetics for < 60 years old patients with dyspepsia not responding to PPIs or H. pylori eradication therapy. B Tricyclic antidepressants: offer TCAs for < 60 years old patients with dyspepsia not responding to PPIs or H. pylori eradication therapy. B 4. Specific circumstances Motility studies in functional dyspepsia: Do not routinely obtain motility studies for patients with functional dyspepsia. D Consider motility studies for selected patients with functional dyspepsia where gastroparesis is strongly suspected. C Management of functional dyspepsia: treat H. pylori infection in patients with functional dyspepsia that are H. pylori-positive. A Show 5 more References 1. Paul Moayyedi, Brian E Lacy, Christopher N Andrews et al. ACG and CAG Clinical Guideline: Management of Dyspepsia. Am J Gastroenterol. 2017 Jul;112 7 988 1013. Open 2. Yang YX, Brill J, Krishnan P et al. American Gastroenterological Association Institute Guideline on the Role of Upper Gastrointestinal Biopsy to Evaluate Dyspepsia in the Adult Patient in the Absence of Visible Mucosal Lesions. Gastroenterology. 2015 Oct;149 4 1082 7. Open https://web.pathway.md/diseases/rectVh3waGB61Fg37 2/2 |
Guideline sources The following summarized guidelines for the evaluation and management of dysphonia are prepared by our editorial team based on guidelines from the American Academy of Otolaryngology-Head and Neck Surgery Foundation (AAO-HNSF 2018). 1 Guidelines 1. Screening and diagnosis Diagnosis: identify dysphonia in patients with altered voice quality, pitch, loudness, or vocal effort impairing communication or reducing the quality of life. B 2. Diagnostic investigations History and physical examination: Elicit medical history and perform a physical examination to assess for underlying causes of dysphonia and factors modifying management, B as well as to identify factors requiring expedited laryngeal evaluation including but not limited to: recent surgical procedures involving the head neck or chest https://web.pathway.md/diseases/recSlH91IPPP5pq8u 1/4 6/29/23, 10:26 PM Dysphonia Pathway recent surgical procedures involving the head, neck, or chest recent endotracheal intubation presence of concomitant neck mass respiratory distress or stridor history of tobacco abuse professional voice use. B Diagnostic imaging: do not obtain CT or MRI before visualizing the larynx in patients with a primary voice complaint. D 3. Diagnostic procedures Laryngoscopy: consider performing diagnostic laryngoscopy at any time in patients with dysphonia. C Show 2 more 4. Medical management Acid-reducing therapy: do not use antireflux medications for the treatment of isolated dysphonia solely based on symptoms attributed to suspected GERD or laryngopharyngeal reflux, without visualization of the larynx. D Corticosteroids: do not use routine corticosteroids in patients with dysphonia before visualizing the larynx. D Antibiotics: do not use antibiotics routinely for the treatment of patients with dysphonia. D 5. Nonpharmacologic interventions Voice therapy: offer voice therapy in patients with dysphonia from a cause amenable to voice therapy. B 6. Therapeutic procedures Botulinum toxin injections: offer botulinum toxin injections, or refer to a clinician who can administer botulinum toxin injections, for the treatment of dysphonia caused by spasmodic dysphonia or other types of laryngeal dystonia. B 7. Surgical interventions Indications for surgery: offer surgery as a therapeutic option in patients with dysphonia with conditions amenable to surgical intervention, such as suspected malignancy, symptomatic benign vocal fold lesions not responding to conservative management, or glottic insufficiency. B https://web.pathway.md/diseases/recSlH91IPPP5pq8u 2/4 6/29/23, 10:26 PM Dysphonia Pathway 8. Patient education General counseling: counsel patients with dysphonia on control/preventive measures. B 9. Follow-up and surveillance Serial clinical assessment: document resolution, improvement, or worsening symptoms of dysphonia or change in the quality of life in patients with dysphonia after treatment or observation. B Clinical findings Symptoms Past medical history Aphonia ALS Change in voice Acromegaly Hoarseness Allergy Amyloidosis Past events Eosinophilic granulomatosis with polyangiitis Recent intubation Gastroesophageal reflux Hypothyroidism Laryngeal cancer Laryngitis Laryngopharyngeal reflux Multiple sclerosis Myasthenia gravis Parkinson's disease Recurrent laryngeal nerve injury Rheumatoid arthritis SLE Sarcoidosis Sj gren's disease Stroke Upper respiratory tract infection Vocal cord nodule Vocal cord paralysis Vocal fold paralysis Vocal fold polyp Social history Voice overuse References https://web.pathway.md/diseases/recSlH91IPPP5pq8u 3/4 6/29/23, 10:26 PM Dysphonia Pathway 1. Stachler RJ, Francis DO, Schwartz SR et al. Clinical Practice Guideline: Hoarseness Dysphonia) (Update). Otolaryngol Head Neck Surg. 2018 Mar;158 1_suppl):S1 S42. Open https://web.pathway.md/diseases/recSlH91IPPP5pq8u 4/4 |
Guideline sources The following summarized guidelines for the evaluation and management of dyspnea in palliative care are prepared by our editorial team based on guidelines from the Global Initiative for Chronic Obstructive Lung Disease (GOLD 2023), the American Society of Clinical Oncology (ASCO 2021), the American Thoracic Society (ATS 2020), the European Respiratory Society (ERS 2017), the British Thoracic Society (BTS 2015), and the Canadian Thoracic Society (CTS 2011). 1 2 3 4 5 6 Calculator Calculator Calculat BODE index for survival of COPD COPD assessment test CAT DECAF Guidelines 1. Diagnostic investigations Clinical assessment: Obtain a systematic assessment of dyspnea using validated patient-reported outcome measures at every inpatient and outpatient encounter in patients with advanced cancer. Use https://web.pathway.md/diseases/reclICyFduWEmymO1 1/5 6/29/23, 10:26 PM Dyspnea in palliative care Pathway a validated observation measure in patients unable to self-report. B Obtain a comprehensive assessment of the severity, chronicity, potential causes, triggers, associated symptoms, and emotional and functional impact in patients with dyspnea, whenever possible. B 2. Respiratory support Airflow interventions: As per GOLD 2023 guidelines, consider offering a fan blowing air onto the face to relieve dyspnea in patients with advanced COPD. C As per ASCO 2021 guidelines, offer airflow interventions such as directing a fan at the cheek (trigeminal nerve distribution) in patients with advanced cancer and dyspnea. B As per BTS 2015 guidelines, offer a fan therapy provided by an appropriately trained healthcare professional in patients with cancer or end-stage cardiorespiratory disease experiencing intractable breathlessness. B Supplemental oxygen: As per GOLD 2023 guidelines, consider administering oxygen supplementation in patients with advanced COPD. C As per ASCO 2021 guidelines: Ensure standard supplemental oxygen is available for patients with hypoxemia experiencing dyspnea (SpO 90% on room air). B Do not administer supplemental oxygen when SpO is > 90%. D As per BTS 2015 guidelines: Do not offer palliative oxygen therapy in patients with cancer or end-stage cardiorespiratory disease experiencing intractable breathlessness if they are non-hypoxemic or having mild levels of hypoxemia above current long-term oxygen therapy thresholds (SpO 92%). D Consider offering palliative oxygen therapy by specialist teams in patients with intractable breathlessness unresponsive to all other modalities of treatment. Obtain an individual formal assessment of the effect of palliative oxygen on reducing breathlessness and improving quality of life. E As per CTS 2011 guidelines: Consider offering continuous oxygen therapy in hypoxemic (at rest) patients with advanced COPD. C Insufficient evidence to support the routine use of supplemental oxygen to reduce dyspnea in non-hypoxemic patients with advanced COPD. I Noninvasive ventilation: As per ASCO 2021 guidelines: Consider offering a time-limited therapeutic trial of high-flow nasal cannula oxygen therapy, if available, in patients with significant dyspnea and hypoxemia despite standard supplemental oxygen. C Consider offering a time-limited therapeutic trial of noninvasive ventilation, if available and not contraindicated, in patients with significant dyspnea despite standard measures. C https://web.pathway.md/diseases/reclICyFduWEmymO1 2/5 6/29/23, 10:26 PM Dyspnea in palliative care Pathway As per ERS 2017 guidelines, consider offering noninvasive ventilation to relieve dyspnea in patients with terminal cancer or other terminal conditions. C 3. Medical management General principles: refer patients with advanced cancer and dyspnea to an interprofessional palliative care team where available. B Management of underlying conditions: provide goal-concordant treatment consistent with the patient's wishes, prognosis, and overall health status in patients with potentially reversible, common etiologies of dyspnea, such as pleural effusion, pneumonia, airway obstruction, anemia, asthma, COPD exacerbation, PE, or treatment-induced pneumonitis. B Show 2 more Opioids: As per GOLD 2023 guidelines, consider offering opioids to relieve dyspnea in patients with advanced COPD. C Consider offering low-dose long-acting oral or parenteral opioids in patients with severe disease. C As per ASCO 2021 guidelines, offer systemic opioids in patients with advanced cancer when nonpharmacologic interventions are insufficient to provide dyspnea relief. B As per ATS 2020 guidelines, consider offering opioid-based therapy, within a personalized shared decision-making approach, in patients with COPD experiencing advanced refractory dyspnea despite otherwise optimal therapy. C As per BTS 2015 guidelines, offer a trial of opioids in patients with cancer or end-stage cardiorespiratory disease experiencing intractable breathlessness. A As per CTS 2011 guidelines, offer oral (but not nebulized) opioids for the treatment of refractory dyspnea in individual patients with advanced COPD. B Benzodiazepines: consider offering short-acting benzodiazepines in patients experiencing dyspnea-related anxiety and continuing to experience dyspnea despite opioids and other nonpharmacologic measures. C Corticosteroids: consider offering systemic corticosteroids in selected patients with airway obstruction or when inflammation is likely a key contributor to dyspnea. C Bronchodilators: offer bronchodilators for palliation of dyspnea in patients with established obstructive pulmonary disorder or evidence of bronchospasm. B Sedation: offer continuous palliative sedation in patients with dyspnea refractory to all standard treatment options and all applicable palliative options, with an expected life expectancy of days. B Therapies with no evidence for benefit: As per ASCO 2021 guidelines, insufficient evidence to recommend for or against the use of antidepressants, neuroleptics, or inhaled furosemide for dyspnea. I As per CTS 2011 guidelines, do not use anxiolytics and antidepressants routinely for the management of dyspnea in patients with advanced COPD. D https://web.pathway.md/diseases/reclICyFduWEmymO1 3/5 6/29/23, 10:26 PM Dyspnea in palliative care Pathway 4. Nonpharmacologic interventions Breathing techniques: As per ASCO 2021 guidelines, consider offering breathing techniques for dyspnea in patients with advanced cancer. C As per CTS 2011 guidelines, advise patients with advanced COPD of the potential benefits of pursed-lip breathing and instruct them in its use. B Walking aids: advise patients with advanced COPD of the potential benefits of walking aids and obtain a professional assessment for choosing a suitable device. B Nutritional support: provide nutritional supplementation to improve respiratory muscle strength and overall health status in malnourished patients. B Alternative and complementary therapies: As per ASCO 2021 guidelines: Consider offering other nonpharmacologic measures for dyspnea, such as posture, relaxation, distraction, meditation, self-management, physical therapy, and music therapy, in patients with advanced cancer. Consider offering acupressure or reflexology, if available. C Insufficient evidence to recommend for or against pulmonary rehabilitation in patients with advanced cancer and dyspnea. I As per CTS 2011 guidelines, insufficient evidence to recommend the routine use of acupuncture, acupressure, distractive auditory stimuli (music), relaxation, handheld fans, counseling and support programs, and psychotherapy in patients with advanced COPD. I 5. Therapeutic procedures Neuromuscular electrical stimulation: As per GOLD 2023 guidelines, consider offering neuromuscular electrical stimulation in patients with advanced COPD. C As per CTS 2011 guidelines, consider offering neuromuscular electrical stimulation and chest wall vibration to reduce dyspnea in patients with advanced COPD. C Studies 2022 BEAMS (morphine 16 mg) In adult patients with COPD and chronic breathlessness, morphine was not superior to placebo with respect to reduction in intensity of worst breathlessness at week 1. Magnus Ekstr m et al. JAMA. 2022 Nov 22. 2022 BEAMS (morphine 8 mg) In adult patients with COPD and chronic breathlessness, morphine was not superior to placebo with respect to reduction in intensity of worst breathlessness at week 1. https://web.pathway.md/diseases/reclICyFduWEmymO1 4/5 6/29/23, 10:26 PM Dyspnea in palliative care Pathway Magnus Ekstr m et al. JAMA. 2022 Nov 22. Show 3 more References 1. Darcy D Marciniuk, Donna Goodridge, Paul Hernandez et al. Managing dyspnea in patients with advanced chronic obstructive pulmonary disease: a Canadian Thoracic Society clinical practice guideline. Can Respir J. 2011 Mar-Apr;18 2 69 78. Open 2. Hardinge M, Annandale J, Bourne S et al. British Thoracic Society guidelines for home oxygen use in adults. Thorax. 2015 Jun;70 Suppl 1:i1 43. Open 3. David Hui, Kari Bohlke, Ting Bao et al. Management of Dyspnea in Advanced Cancer: ASCO Guideline. J Clin Oncol. 2021 Apr 20;39 12 1389 1411. Open 4. Alvar Agusti, Richard Beasley, Bartolome R. Celli et al. Global Strategy for the Diagnosis, Management, and Prevention of Chronic Obstructive Pulmonary Disease: 2023 Report. GOLD. 2023. Open 5. Linda Nici, Manoj J Mammen, Edward Charbek et al. Pharmacologic Management of Chronic Obstructive Pulmonary Disease. An Official American Thoracic Society Clinical Practice Guideline. Am J Respir Crit Care Med. 2020 May 1;201 9):e56-e69. Open 6. Rochwerg B, Brochard L, Elliott MW et al. Official ERS/ATS clinical practice guidelines: noninvasive ventilation for acute respiratory failure. Eur Respir J. 2017 Aug 31;50 2 . pii: 1602426. Open https://web.pathway.md/diseases/reclICyFduWEmymO1 5/5 |
Guideline sources The following summarized guidelines for the evaluation and management of early pregnancy are prepared by our editorial team based on guidelines from the American College of Emergency Physicians (ACEP 2017) and the Society of Obstetricians and Gynaecologists of Canada (SOGC 2014). 1 2 3 3 4 5 Definition Early pregnancy is a normal physiological event for a woman with a mean gestation period < 15 weeks (range of 6 to 24 weeks). 3 Epidemiology Early pregnancy is a result of conception either naturally or following medical intervention. 3 Disease course Clinical manifestations of early pregnancy include nausea, vomiting, back pain, pelvic girdle pain, pelvic cavity pain, vulvar itching, varicose veins, pruritus, leg cramp, exhaustion, frequent coughs and colds, migraines, constipation, hemorrhoids, depression, anxiety, and vaginal bleeding. Vaginal bleeding may lead to spontaneous abortion. 4 Prognosis and risk of recurrence In the United States, mortality associated with legal induced abortion is < 1 per 100,000 procedures. 5 https://web.pathway.md/diseases/recPfjpwxzBnj6tIF 1/3 6/29/23, 4:14 AM Early pregnancy Pathway Guidelines 1. Diagnostic investigations Transabdominal ultrasound: consider first-trimester crown-rump length as the best parameter for determining gestational age and use whenever appropriate. B Dating ultrasound: consider using crown-rump length measurement from either transabdominal or transvaginal ultrasound to determine gestational age. Transvaginal ultrasound is not more accurate in determining gestational age than a transabdominal approach, although it may better visualize early embryonic structures. C 2. Specific circumstances Pregnant patients in the emergency department: Obtain a pelvic ultrasound for symptomatic pregnant patients with any b-hCG level. B Avoid using the b-hCG value to exclude the diagnosis of ectopic pregnancy in patients who have an indeterminate ultrasound result. D 3. Follow-up and surveillance Indications for specialist referral: obtain specialty consultation or arrange close outpatient follow-up for all patients with an indeterminate pelvic ultrasound result. B Serial imaging assessment: consider follow-up of interval growth 2 to 3 weeks following the ultrasound. Confirmation of an accurate due date is difficult when the assignment of gestational age is based on a third-trimester ultrasound. C Clinical findings Symptoms Neurological exam Abdominal pain Mood alteration Bloating Constipation Cramp Fatigue Missed menstrual periods Nausea Swollen breasts Urinary frequency Vomiting Lab findings urine urine -hCG https://web.pathway.md/diseases/recPfjpwxzBnj6tIF 2/3 6/29/23, 4:14 AM Early pregnancy Pathway References 1. Butt K, Lim K. Determination of gestational age by ultrasound. J Obstet Gynaecol Can. 2014 Feb;36 2 171 181. Open 2. Hahn SA, Promes SB, Brown MD. Clinical Policy: Critical Issues in the Initial Evaluation and Management of Patients Presenting to the Emergency Department in Early Pregnancy. Ann Emerg Med. 2017 Feb;69 2 241 250.e20. Open 3. Deirdre Gartland, Stephanie Brown, Susan Donath et al. Women's health in early pregnancy: findings from an Australian nulliparous cohort study. Aust N Z J Obstet Gynaecol. 2010 Oct;50 5 413 8. Open 4. Melissa C Lutterodt, Pernille K hler, Jakob Kragstrup et al. Examining to what extent pregnancy- related physical symptoms worry women in the first trimester of pregnancy: a cross-sectional study in general practice. BJGP Open. 2019 Dec; 3 4 bjgpopen19X101674. Open 5. Suzanne Zane, Andreea A Creanga, Cynthia J Berg et al. Abortion-Related Mortality in the United States: 1998 2010. 2015 Aug;126 2 258 265.2015 Aug;126 2 258 265. Open 6. Suzanne Zane, Andreea A. Creanga, Cynthia J. Berg et al. Abortion-Related Mortality in the United States 1998 2010. Obstet Gynecol. 2015 Aug; 126 2 258 265. Open 7. Liona C Poon, H David McIntyre, Jonathan A Hyett et al. The first-trimester of pregnancy A window of opportunity for prediction and prevention of pregnancy complications and future life. Diabetes Res Clin Pract. 2018 Nov;145 20 30. Open https://web.pathway.md/diseases/recPfjpwxzBnj6tIF 3/3 |
Guideline sources The following summarized guidelines for the management of ebola virus disease are prepared by our editorial team based on guidelines from the World Health Organization (WHO 2022; 2020; 2014), the Center for Disease Control (CDC 2021), and the Ebola Virus Disease Working Group (EBV-WG 2018). 1 2 3 4 5 Guidelines 1. Medical management Monoclonal antobodies: Administer either ansuvimab or atoltivimab/maftivimab/odesivimab for the treatment of patients with RT-PCR confirmed Ebola virus disease and in neonates of unconfirmed Ebola virus disease status, 7 days old, born to mothers with confirmed Ebola virus disease. B Avoid using ZMapp for the treatment of patients with RT-PCR confirmed Ebola virus disease. D Remdesivir: avoid using remdesivir for the treatment of patients with RT-PCR confirmed Ebola virus disease. D Fluid therapy: https://web.pathway.md/diseases/rec5JOeUAIwvNI6UN 1/5 6/29/23, 4:14 AM Ebola virus disease Pathway Administer oral rehydration solution in an adequate amount rather than non-standardized rehydration to reduce mortality in patients with suspected, probable, or confirmed Ebola virus disease. B Administer parenteral rather in patients unable to drink or when volume losses are larger than oral volume intake. B Analgesic therapy: administer analgesic therapy, including parenteral opioids, if necessary to reduce pain in patients with suspected, probable, or confirmed Ebola virus disease experiencing pain. A Antibiotic therapy: administer broad-spectrum antibiotics promptly to reduce mortality in patients with suspected, probable, or confirmed Ebola virus disease and high severity of illness. B 2. Inpatient care Serial clinical assessment: obtain systematic monitoring and charting of vital signs and volume status in all patients with Ebola virus disease. B Serial laboratory assessment: obtain and chart serum biochemistry (such as electrolytes, glucose, and blood gas) in patients with suspected, probable, or confirmed Ebola virus disease, as deemed desirable by the attending clinicians, and manage observed abnormalities according to the clinician's judgment. B 3. Specific circumstances Pregnant patients: provide optimized supportive care in the clinical management of pregnant patients with Ebola virus disease. B Show 7 more Breastfeeding patients: stop breastfeeding if acute Ebola virus disease is suspected or confirmed in a lactating woman or in a breastfeeding child. Separate the child from the breastfeeding woman and provide a breastmilk substitute as needed. B Show 4 more 4. Patient education Communication with relatives: consider facilitating communication with family and friends to reduce psychological distress in patients admitted to the treatment unit with suspected, probable, or confirmed Ebola virus disease. C 5. Preventative measures Personal protective equipment: Ensure that all healthcare workers follow the following precautions while providing clinical care for patients with filovirus disease in order to prevent virus exposure: https://web.pathway.md/diseases/rec5JOeUAIwvNI6UN 2/5 6/29/23, 4:14 AM Ebola virus disease Pathway cover the mucous membranes of their eyes, mouth, and nose completely with personal protective equipment A use either a face shield or goggles B wear a fluid-resistant medical/surgical mask with a structured design not collapsing against the mouth, such as duckbill or cup shape B use a fluid-resistant particulate respirator during procedures generating aerosols of body fluids B wear double gloves, preferably with nitrile material rather than latex B wear protective bodywear in addition to regular on-duty clothing A ; with the choice of bodywear being either a disposable gown and apron, or a disposable coverall and apron, with the gown and the coverall made of fabric tested for resistance to penetration by blood or body fluids or to blood-borne pathogens B ; with the choice of apron being, in order of preference: disposable, waterproof apron, or heavy-duty, reusable waterproof aprons if appropriate cleaning and disinfection between patients are performed, if disposable aprons are not available B wear waterproof boots, such as rubber/gum B wear a head cover covering the head and neck, being separate from the gown or coverall, so that these may be removed separately. B Immunization: Offer pre-exposure vaccination with the rVSV-ZEBOV vaccine in 18 years adults being at highest risk for potential occupational exposure to Ebola virus, such as: responding to an outbreak of Ebola virus disease working as healthcare personnel at designated Ebola treatment centers working as laboratorians or other staff at biosafety level 4 facilities. E 6. Quality improvement Requirements for Ebola treatment unit personnel: ensure a staffing ratio of at least 1 clinician to 4 patients in Ebola treatment units, taking into account the need for patient assessment at least TID and continuous (24 hours/day) monitoring of patients to allow prompt recognition of and reaction to acute changes in condition. B Clinical findings Symptoms Past obstetric history Abdominal pain Miscarriage Arthralgia Vital signs Bleeding tendency Chest pain Hypotension Confusion Signs of shock Cough Tachypnea Diarrhea Ocular exam Dysphagia Fatigue https://web.pathway.md/diseases/rec5JOeUAIwvNI6UN 3/5 6/29/23, 4:14 AM Ebola virus disease Pathway Fever Conjunctival injection Gum bleeding Lab findings Headache Hearing loss Ebola virus RNA Hematemesis Ebola virus antigen Hematochezia Hypocalcemia Hematuria Hypokalemia Hemoptysis Hyponatremia Loss of appetite Melena Myalgia Nausea Nosebleed Odynophagia Throat pain Vaginal bleeding Vomiting Social history Living in or travel to Africa Neurological exam Altered mental status Coma Delirium Neck stiffness Integument exam Ecchymosis Maculopapular rash Petechiae Skin desquamation Serological findings Ebola virus IgG Ebola virus IgM References 1. Richard Kojan, Robert Fowler. Therapeutics for Ebola virus disease. Geneva: World Health Organization; 2022. Open 2. Fran ois Lamontagne, Robert A Fowler, Neill K Adhikari et al. Evidence-based guidelines for supportive care of patients with Ebola virus disease. Lancet. 2018 Feb 17;391 10121 700 708. Open https://web.pathway.md/diseases/rec5JOeUAIwvNI6UN 4/5 6/29/23, 4:14 AM Ebola virus disease Pathway 3. Megan Foeller, Anna Thorson. Guidelines for the management of pregnant and breastfeeding women in the context of Ebola virus disease. Geneva: World Health Organization; 2020. Open 4. Mary J Choi, Caitlin M Cossaboom, Amy N Whitesell et al. Use of Ebola Vaccine: Recommendations of the Advisory Committee on Immunization Practices, United States, 2020. MMWR Recomm Rep. 2021 Jan 8;70 1 1 12. Open 5. No authors listed. Personal protective equipment in the context of filovirus disease outbreak response. Geneva: World Health Organization; 2014. Open https://web.pathway.md/diseases/rec5JOeUAIwvNI6UN 5/5 |
Guideline sources The following summarized guidelines for the evaluation and management of eclampsia are prepared by our editorial team based on guidelines from the Society of Obstetricians and Gynaecologists of Canada (SOGC 2022), the American Association for the Study of Liver Diseases (AASLD 2021), the Myasthenia Gravis Foundation of America (MGFA 2016), and the Italian Association for the Study of the Liver (AISF 2016). 1 2 3 4 5 5 6 6 Definition Eclampsia is the presence of new-onset grand mal seizures in women with preeclampsia. 5 Epidemiology Eclampsia is caused by preeclampsia. 5 Disease course Clinical manifestations of eclampsia include hypertension, proteinuria, generalized edema, and convulsions with headache, RUQ epigastric pain, and visual changes either antepartum, intrapartum, or postpartum. Eclampsia is associated with increased maternal complications, including HELLP (hemolysis, elevated liver enzymes, low platelets) syndrome, abruptio placentae, disseminated intravascular coagulopathy, pulmonary edema, acute renal failure, https://web.pathway.md/diseases/recjnQsCXSa25TDKR 1/4 6/29/23, 4:15 AM Eclampsia Pathway aspiration pneumonia, cardiopulmonary arrest, liver hematoma, and respiratory distress syndrome. 6 Prognosis and risk of recurrence Eclampsia is associated with a mortality rate ranging from 0-1.8% in developed countries and 14% in developing countries. 6 Guidelines 1. Screening and diagnosis Indications for screening: obtain close monitoring for the development of eclampsia in patients with preeclampsia. E 2. Medical management Magnesium sulfate: As per SOGC 2022 guidelines, administer magnesium sulfate as first-line therapy in patients with eclampsia. A As per AISF 2016 guidelines, administer magnesium sulphate for controlling seizures in patients with eclampsia. A 3. Therapeutic procedures Timing for delivery: perform expeditious deliver after maternal stabilization in patients with suspected eclampsia. E 4. Specific circumstances Patients with myasthenia gravis: do not use magnesium sulfate for the management of eclampsia in patients with myasthenia gravis because of its neuromuscular blocking effects. Offer barbiturates or phenytoin to provide adequate treatment. D 5. Preventative measures Magnesium sulfate: As per SOGC 2022 guidelines, administer magnesium sulfate as prophylaxis against eclampsia in patients with preeclampsia and severe hypertension or adverse maternal conditions. A As per AISF 2016 guidelines, administer magnesium sulphate for seizure prophylaxis in patients with severe preeclampsia. A https://web.pathway.md/diseases/recjnQsCXSa25TDKR 2/4 6/29/23, 4:15 AM Eclampsia Pathway Clinical findings Symptoms Past medical history Agitation Diabetes mellitus Blurred vision Hypertension Double vision Vital signs Headache Loss of consciousness Hypertension Photophobia Lab findings Seizure Visual disturbances urine protein Weight gain Past obstetric history Preeclampsia Vascular exam Peripheral edema Studies 2002 MAGPIE In women who had not given birth or were 24 h postpartum with BP 140/90 mmHg and proteinuria 1+, magnesium sulfate was superior to placebo with respect to eclampsia. Altman D et al. Lancet. 2002 Jun 1. References 1. Monika Sarkar, Carla W Brady, Jaquelyn Fleckenstein et al. Reproductive Health and Liver Disease: Practice Guidance by the American Association for the Study of Liver Diseases. Hepatology. 2021 Jan;73 1 318 365. Open 2. Sanders DB, Wolfe GI, Benatar M et al. International consensus guidance for management of myasthenia gravis: Executive summary. Neurology. 2016 Jul 26;87 4 419 25. Open 3. Italian Association for the Study of the Liver AISF , Italian Association for the Study of the Liver AISF. AISF position paper on liver disease and pregnancy. Dig Liver Dis. 2016 Feb;48 2 120 37. Open 4. Laura A Magee, Graeme N Smith, Christine Bloch et al. Guideline No. 426 Hypertensive Disorders of Pregnancy: Diagnosis, Prediction, Prevention, and Management. J Obstet Gynaecol Can. 2022 May;44 5 547 571.e1. Open 5. Mauro H Schenone, Dorothy Miller, Jacques E Samson et al. Eclampsia characteristics and outcomes: a comparison of two eras. 2013;2013 826045.2013;2013 826045. Open 6. Baha M Sibai. Diagnosis, prevention, and management of eclampsia. Obstet Gynecol. 2005 Feb;105 2 402 10. Open https://web.pathway.md/diseases/recjnQsCXSa25TDKR 3/4 6/29/23, 4:15 AM Eclampsia Pathway 7. Douglas KA, Redman CW. Eclampsia in the United Kingdom. BMJ. 1994 Nov 26;309 6966 1395 400. Open 8. Sibai BM. Eclampsia. VI. Maternal-perinatal outcome in 254 consecutive cases. Am J Obstet Gynecol. 1990 Sep;163 3 1049 54; discussion 1054 5. Open 9. Magee LA, Pels A, Helewa M et al. Diagnosis, evaluation, and management of the hypertensive disorders of pregnancy: executive summary. J Obstet Gynaecol Can. 2014 May;36 5 416 41. Open 10. Ahmad Mahran, Hashem Fares, Reham Elkhateeb et al. Risk factors and outcome of patients with eclampsia at a tertiary hospital in Egypt. BMC Pregnancy Childbirth. 2017; 17 435. Open 11. Mauro H. Schenone, Dorothy Miller, Jacques E. Samson et al. Eclampsia Characteristics and Outcomes: A Comparison of Two Eras. J Pregnancy. 2013; 2013 826045. Open 12. Tram T Tran, Joseph Ahn, Nancy S Reau. ACG Clinical Guideline: Liver Disease and Pregnancy. Am J Gastroenterol. 2016 Feb;111 2 176 94; quiz 196. Open https://web.pathway.md/diseases/recjnQsCXSa25TDKR 4/4 |
Guideline sources The following summarized guidelines for the evaluation and management of ecthyma and impetigo are prepared by our editorial team based on guidelines from the Surgical Infection Society Europe (SIS-E/WSES 2018), the Infectious Diseases Society of America (IDSA 2014), and the American Association of Family Physicians (AAFP 2007). 1 1 1 2 2 2 2 3 3 3 4 5 Definition Impetigo is a highly contagious bacterial infection of the superficial layers of the epidermis. Ecthyma is a deeply ulcerated form of impetigo that extends into the dermis. 2 4 Epidemiology Impetigo is nearly always caused by S. aureus and/or -hemolytic Streptococcus species. Community-acquired MRSA is an increasingly frequent etiological agent. 2 Pathophysiology Impetigo predominantly affects children. In patients under 18 years of age, the incidence of impetigo is estimated at 206 per 100,000 person-years. 2 5 Disease course Impetigo begins as erythematous papules that rapidly evolve into vesicles and pustules that rupture, with the dried discharge forming honey-colored crusts on an erythematous base. Ecthyma begin as vesicles that rupture, resulting in circular, erythematous ulcers with adherent crusts, often with surrounding erythematous edema. 1 https://web.pathway.md/diseases/rec49NrZTizWsnZ2l 1/4 6/29/23, 4:15 AM Ecthyma and impetigo Pathway Prognosis and risk of recurrence Acute poststreptococcal glomerulonephritis is a serious complication that affects between 1 and 5 percent of patients with nonbullous impetigo. Rheumatic fever does not appear to be a potential complication of impetigo. Impetigo usually resolves without scarring within two weeks if left untreated. Unlike impetigo, ecthyma heals with scarring. 1 3 3 Guidelines 1. Diagnostic investigations Gram stain and culture: Obtain Gram stain and culture of the pus or exudates from skin lesions in patients with impetigo and ecthyma, in order to help identify whether S. aureus and/or a -hemolytic Streptococcus is the cause. B Consider empiric treatment without obtaining Gram stain and culture in patients with a typical clinical presentation. B 2. Medical management Route of antibiotic administration: As per IDSA 2014 guidelines, use either oral or topical antibiotics for the treatment of most patients with bullous and nonbullous impetigo. Show 2 more As per AAFP 2007 guidelines: Use topical antibiotics for the treatment of patients with impetigo in whom there is a limited involvement of BSA. A Consider using an oral antibiotic for the treatment of patients with impetigo in whom there is extensive disease, and in patients with associated systemic symptoms. C Oral antibiotic therapy: As per WSES 2018 guidelines: Use an antibiotic that is active against Gram-positive bacteria for the treatment of impetigo. B Use an antibiotic that is active against community-acquired MRSA for the treatment of impetigo in patients at risk for MRSA, and in patients who do not respond to first-line therapy. B As per IDSA 2014 guidelines, use an antibiotic active against S. aureus for the systemic treatment of ecthyma or impetigo, and complete 7 days of treatment. A Show 3 more As per AAFP 2007 guidelines: Use one of the following agents as first-line antibiotics for the treatment of impetigo: an antistaphylococcal penicillin, such as dicloxacillin 250-500 mg PO QID for 10 days https://web.pathway.md/diseases/rec49NrZTizWsnZ2l 2/4 6/29/23, 4:15 AM Ecthyma and impetigo Pathway a cephalosporin, such as cephalexin 250-500 mg PO QID for 10 days amoxicillin/clavulanic acid 250-500 mg PO BID for 10 days. A Avoid using oral penicillin V or amoxicillin for the empiric treatment of impetigo. D Topical antibiotic therapy: As per IDSA 2014 guidelines, use one of the following agents as first-line topical agents for the treatment of bullous and nonbullous impetigo in patients in whom oral antibiotics are not indicated: mupirocin ointment to lesions BID for 5 days retapamulin ointment to lesions BID for 5 days. A As per AAFP 2007 guidelines, use muciprocin or fusidic acid as first-line topical agents for the treatment of impetigo in patients in whom oral antibiotics are not indicated. A Show 2 more 3. Specific circumstances Outbreaks of poststreptococcal glomerulonephritis: administer systemic antibiotics to patients with ecthyma during outbreaks of poststreptococcal glomerulonephritis, to help eliminate nephritogenic strains of Streptococcus pyogenes from the community. B Clinical findings Symptoms Past medical history Infection Bacterial infection Pain Inflammation Skin infection Moist environment Head and neck exam Angular cheilitis References 1. Stevens DL, Bisno AL, Chambers HF et al. Practice guidelines for the diagnosis and management of skin and soft tissue infections: 2014 update by the Infectious Diseases Society of America. Clin Infect Dis. 2014 Jul 15;59 2):e10 52. Open 2. Sartelli M, Guirao X, Hardcastle TC et al. 2018 WSES/SIS E consensus conference: recommendations for the management of skin and soft-tissue infections. World J Emerg Surg. 2018 Dec 14;13 58. Open 3. Cole C, Gazewood J. Diagnosis and treatment of impetigo. Am Fam Physician. 2007 Mar 15;75 6 859 64. Open 4. Yee Gyung Kwak, Seong Ho Choi, Tark Kim et al. Clinical Guidelines for the Antibiotic Treatment for Community-Acquired Skin and Soft Tissue Infection. Infect Chemother. 2017 Dec;49 4 301 325. Open https://web.pathway.md/diseases/rec49NrZTizWsnZ2l 3/4 6/29/23, 4:15 AM Ecthyma and impetigo Pathway 5. S Koning, R S A Mohammedamin, J C van der Wouden et al. Impetigo: incidence and treatment in Dutch general practice in 1987 and 2001 results from two national surveys. Br J Dermatol. 2006 Feb;154 2 239 43. Open https://web.pathway.md/diseases/rec49NrZTizWsnZ2l 4/4 |
Guideline sources The following summarized guidelines for the evaluation and management of ectopic pregnancy are prepared by our editorial team based on guidelines from the Society of Obstetricians and Gynaecologists of Canada (SOGC 2021), the Royal College of Obstetricians and Gynaecologists (RCOG 2021; 2016), and the American College of Obstetricians and Gynecologists (ACOG 2018; 2017). 1 2 3 4 5 Guidelines 1. Classification and risk stratification Risk stratification: use risk models (such as M6 model) to stratify pregnancy of unknown location as either high or low risk for ectopic pregnancy to guide treatment decisions. B 2. Diagnostic investigations https://web.pathway.md/diseases/recGWlD9mm9btrJfc 1/9 6/29/23, 4:15 AM Ectopic pregnancy Pathway Serum hCG levels: As per ACOG 2018 guidelines: Do not use serum hCG values alone for the diagnosis of ectopic pregnancy, rather correlate it with the patient's history, symptoms, and ultrasound findings. D Use the conservatively high value (such as high as 3,500 mIU/mL), if the hCG discriminatory level is being used as a diagnostic aid in patients at risk of ectopic pregnancy, to avoid the potential for misdiagnosis and possible interruption of an intrauterine pregnancy that the patient hopes to continue. B As per RCOG 2016 guidelines, consider obtaining serum -human chorionic gonadotrophin measurement for planning the management of an ultrasound visualized tubal pregnancy. C Show 4 more Serum progesterone: do not obtain a measurement of serum progesterone level to predict ectopic pregnancy. D Other laboratory tests: do not obtain routine biochemical investigations for the diagnosis of Cesarean scar pregnancy. D Transvaginal ultrasound, general principles: As per ACOG 2018 guidelines: Obtain transvaginal ultrasound evaluation and confirmation of pregnancy as the minimum diagnostic evaluation in patients with a suspected ectopic pregnancy. Obtain serial evaluation with transvaginal ultrasound or hCG level measurement, or both, to confirm the diagnosis. B Obtain repeated transvaginal ultrasound and serial measurement of hCG, or both, to confirm the diagnosis and guide management in clinically stable patients with a pregnancy of unknown location desiring to continue pregnancy, if intrauterine. B As per RCOG 2016 guidelines, obtain ultrasound as the primary diagnostic modality, using a transvaginal approach supplemented by transabdominal imaging if required. B Show 3 more Transvaginal ultrasound (tubal pregnancy): Obtain transvaginal ultrasound as the diagnostic tool of choice for tubal ectopic pregnancy. B Confirm tubal ectopic pregnancy, if possible, by visualizing an adnexal mass moving separate to the ovary. B Transvaginal ultrasound (cervical pregnancy): Consider using the following ultrasound criteria for the diagnosis of cervical ectopic pregnancy: empty uterus barrel-shaped cervix gestational sac present below the level of the internal cervical os absence of the 'sliding sign' blood flow around the gestational sac using on colort Doppler. C Transvaginal ultrasound (interstitial pregnancy): Consider using the following ultrasound criteria for the diagnosis of interstitial pregnancy: empty uterine cavity https://web.pathway.md/diseases/recGWlD9mm9btrJfc 2/9 6/29/23, 4:15 AM Ectopic pregnancy Pathway products of conception/gestational sac located laterally in the interstitial (intramural) part of the tube and surrounded by < 5 mm of myometrium in all imaging planes presence of the 'interstitial line sign'. C Transvaginal ultrasound (cornual pregnancy): Consider using the following ultrasound criteria for the diagnosis of cornual pregnancy: visualization of a single interstitial portion of the fallopian tube in the main uterine body gestational sac/products of conception seen mobile and separate from the uterus and completely surrounded by myometrium vascular pedicle adjoining the gestational sac to the unicornuate uterus. C Transvaginal ultrasound (ovarian pregnancy): insufficient evidence to recommend specific ultrasound criteria for the diagnosis of ovarian ectopic pregnancy. I Transvaginal ultrasound (abdominal pregnancy): Consider using the following ultrasound criteria for the diagnosis of abdominal pregnancy: absence of an intrauterine gestational sac absence of both an evident dilated tube and a complex adnexal mass gestational cavity surrounded by loops of bowel and separated from them by peritoneum wide mobility similar to the fluctuation of the sac, particularly evident with the pressure of the transvaginal probe toward the posterior cul-de-sac. C Transvaginal ultrasound (heterotopic pregnancy): diagnose heterotopic pregnancy if ultrasound findings demonstrate an intrauterine pregnancy and a coexisting ectopic pregnancy. B Magnetic resonance imaging: consider obtaining MRI as a second-line investigation if the diagnosis is equivocal and there is local expertise in the MRI diagnosis of Cesarean scar pregnancies. C Show 2 more 3. Medical management General principles: As per ACOG 2018 guidelines, decide on the choice of management (methotrexate versus surgery) of ectopic pregnancy guided by the initial clinical, laboratory, and radiologic data as well as patient-informed choice based on a discussion of the benefits and risks of each approach. A As per RCOG 2016 guidelines, take into account the possibility of an intrauterine pregnancy in the management plan of patients with ectopic pregnancy. B Expectant management: As per SOGC 2021 guidelines, consider offering expectant management and very close follow-up in carefully selected patients with early, asymptomatic tubal pregnancy. C As per ACOG 2018 guidelines, consider offering expectant management in patients with ectopic pregnancy in specific circumstances. C https://web.pathway.md/diseases/recGWlD9mm9btrJfc 3/9 6/29/23, 4:15 AM Ectopic pregnancy Pathway As per RCOG 2016 guidelines, consider offering expectant management as an option in clinically stable patients with an ultrasound diagnosis of ectopic pregnancy and decreasing - human chorionic gonadotrophin initially < 1,500 IU/L. C Show 2 more Methotrexate: As per SOGC 2021 guidelines, consider administering single- or double-dose methotrexate in patients with tubal pregnancy meeting criteria for medical management. C Show 5 more As per ACOG 2018 guidelines, recognize that both laparoscopic surgery and IM methotrexate are safe and effective treatment options in clinically stable patients with non- ruptured ectopic pregnancy. Decide between surgical and medical management based on the initial clinical, laboratory, and radiologic data as well as patient-informed choice based on a discussion of the benefits and risks of each approach. A Show 3 more As per RCOG 2016 guidelines, consider administering systemic methotrexate in suitable patients with tubal pregnancy. Do not use methotrexate at the first visit unless the diagnosis of ectopic pregnancy is absolutely clear and a viable intrauterine pregnancy has been excluded. C Show 6 more Anti-D immunoglobulin: As per ACOG 2017 guidelines, administer RhD immune globulin in RhD-negative patients with ectopic pregnancy. B As per RCOG 2016 guidelines, offer anti-D prophylaxis in all RhD-negative patients undergoing surgical removal of an ectopic pregnancy or if bleeding is repeated, heavy or associated with abdominal pain. B 4. Therapeutic procedures Transvaginal targeted injection and aspiration: consider administering a local injection of potassium chloride or hyperosmolar glucose with the aspiration of the sac contents as an option in clinically stable patients with heterotopic pregnancy. C 5. Surgical interventions Surgical management (general indications): Recognize that both laparoscopic surgery and IM methotrexate are safe and effective treatment options in clinically stable patients with non-ruptured ectopic pregnancy. Decide between surgical and medical management based on the initial clinical, laboratory, and radiologic data as well as patient-informed choice based on a discussion of the benefits and risks of each approach. A Perform surgery in patients with ectopic surgery exhibiting any of the following: hemodynamic instability symptoms of an ongoing ruptured ectopic mass (such as pelvic pain) https://web.pathway.md/diseases/recGWlD9mm9btrJfc 4/9 6/29/23, 4:15 AM Ectopic pregnancy Pathway signs of intraperitoneal bleeding. A Surgical management, tubal pregnancy: As per SOGC 2021 guidelines, use a minimally invasive approach, if feasible, for surgical management of patients with tubal pregnancy. A Show 2 more As per ACOG 2018 guidelines, decide on performing a salpingostomy or salpingectomy for the management of patients with tubal pregnancy guided by the patient's clinical status, desire for future fertility and the extent of fallopian tube damage. B As per RCOG 2016 guidelines, prefer laparoscopic approach over open surgery in patients with tubal pregnancy. A Show 2 more Surgical management (cervical pregnancy): reserve surgical methods of management for cervical pregnancy only for patients with life-threatening bleeding, because of the associated high failure rate. B Surgical management (interstitial pregnancy): Consider performing laparoscopic cornual resection or salpingotomy as an effective option in patients with interstitial pregnancy. C Consider performing hysteroscopic resection under laparoscopic or ultrasound guidance as alternative surgical techniques in patients with interstitial pregnancy. C Surgical management, cornual pregnancy: As per SOGC 2021 guidelines, consider performing either laparoscopic cornuotomy or cornual wedge resection if surgery is required for the treatment of patients with interstitial or cornual pregnancy, as both procedures have comparable results. C As per RCOG 2016 guidelines, perform excision of the rudimentary horn via laparoscopy or laparotomy for the management of patients with cornual pregnancy. B Surgical management, Cesarean scar pregnancy: As per SOGC 2021 guidelines: Consider performing hysteroscopy for surgical management of patients with type I Cesarean scar pregnancy. C Consider performing laparoscopy for surgical management of patients with type II Cesarean scar pregnancy. C As per RCOG 2016 guidelines, consider preferring surgical over medical management in patients with Cesarean scar pregnancy, recognizing that insufficient evidence to recommend any one specific intervention over another. C Surgical management, ovarian pregnancy: As per SOGC 2021 guidelines, consider performing laparoscopic ovarian wedge resection rather than oophorectomy in patients with ovarian ectopic pregnancy, if clinically appropriate. C As per RCOG 2016 guidelines, perform definitive surgical treatment if laparoscopy is required to make the diagnosis of ovarian ectopic pregnancy. B Surgical management, abdominal pregnancy: https://web.pathway.md/diseases/recGWlD9mm9btrJfc 5/9 6/29/23, 4:15 AM Ectopic pregnancy Pathway As per SOGC 2021 guidelines, consider performing either laparotomy or laparoscopy to excise an abdominal pregnancy. C As per RCOG 2016 guidelines: Consider performing laparoscopic removal as an option of treatment in patients with early abdominal pregnancy. C Perform laparotomy for the management of patients with advanced abdominal pregnancy. B Surgical management, heterotopic pregnancy: As per SOGC 2021 guidelines: Consider performing surgical excision of the ectopic pregnancy in patients with heterotopic pregnancy. C Consider adding dilatation and curettage to the surgical procedure to evacuate the uterine cavity if the intrauterine pregnancy is not desired. C As per RCOG 2016 guidelines, consider performing surgical removal of ectopic pregnancy as the method of choice in hemodynamically unstable patients with heterotopic pregnancy and as an option in hemodynamically stable patients with heterotopic pregnancy. C 6. Specific circumstances Patients with molar pregnancy: Manage patients with ectopic pregnancy suspected to be molar in nature as any other case of ectopic pregnancy. E Send a tissue specimen to a center with appropriate expertise for pathological review, if there is a local tissue diagnosis of ectopic molar pregnancy. E 7. Patient education General counseling: inform patients with tubal pregnancy undergoing salpingotomy about the risk of persistent trophoblast with the need for serum -human chorionic gonadotrophin level follow-up and that there is a small risk that they may need further treatment in the form of systemic methotrexate or salpingectomy. E Show 3 more Fertility counseling: as per RCOG 2016 guidelines, counsel patients without a history of subfertility or tubal pathology that there is no difference in the rate of fertility, the risk of future tubal ectopic pregnancy or tubal patency rates between the different management methods. B Show 4 more Counseling on methotrexate treatment: As per ACOG 2018 guidelines: Consider counseling patients that methotrexate treatment of ectopic pregnancy does not have an adverse effect on subsequent fertility or on ovarian reserve. C Counsel patients treated with methotrexate about the following: https://web.pathway.md/diseases/recGWlD9mm9btrJfc 6/9 6/29/23, 4:15 AM Ectopic pregnancy Pathway risk of ectopic pregnancy rupture avoidance of certain foods, supplements, or drugs that can decrease efficacy importance of not becoming pregnant again until resolution has been confirmed. B As per RCOG 2016 guidelines: Consider offering muscle relaxation training in patients undergoing treatment for ectopic pregnancy with methotrexate. C Advise patients treated with methotrexate to wait at least 3 months before trying to conceive again. B 8. Follow-up and surveillance Serial hCG monitoring: obtain serial monitoring of hCG levels after administration of methotrexate treatment until a nonpregnancy level (based upon the reference laboratory assay) is reached. B 9. Quality improvement Healthcare professional training: ensure that clinicians undertaking ultrasound for the diagnosis of ectopic pregnancy have received appropriate training. E Show 4 more Equipment and care availability: ensure that providers of early pregnancy care provide a 7- day early pregnancy assessment service with direct access for patients referred by general practitioners and accident and emergency departments. E Show 2 more Clinical findings Symptoms Past medical history Abdominal cramps Chlamydia trachomatis infection Abdominal pain Pelvic inflammatory disease LLQ pain Use of intrauterine device Missed period Social history Pelvic pain RLQ pain Tobacco use Suprapubic pain Abdominal exam Vaginal bleeding Abdominal tenderness Past surgical history Cullen's sign Tubal ligation Grey Turner's sign Tubal surgery Peritoneal findings Rebound tenderness Vital signs https://web.pathway.md/diseases/recGWlD9mm9btrJfc 7/9 6/29/23, 4:15 AM Ectopic pregnancy Pathway Signs of shock Lab findings Gynecologic exam urine pregnancy test Adnexal mass Adnexal tenderness Cervical motion tenderness Imaging findings Absence of intrauterine pregnancy Adnexal mass in the absence of an intrauterine pregnancy Likelihood Ratios Pertinent positives The following findings increase the probability of ectopic pregnancy in adults. 5 6 Finding LR+ Value Presence of adnexal mass in the absence of an intrauterine pregnancy 111 (12-1028) Presence of cervical motion tenderness 4.9 (1.7-14) Presence of peritoneal findings 4.2-4.5 Presence of adnexal mass 2.4 (1.6-3.7) Show 1 more Pertinent negatives The following findings decrease the probability of ectopic pregnancy in adults. 5 6 Finding LR- Value Absence of adnexal mass in the absence of an intrauterine pregnancy 0.12 (0.03-0.55) Absence of adnexal tenderness 0.57 (0.48-0.67) Absence of cervical motion tenderness 0.62 (0.47-0.83) Absence of peritoneal findings 0.78-0.81 Show 1 more References 1. Committee on Practice Bulletins Gynecology. ACOG Practice Bulletin No. 191 Tubal Ectopic Pregnancy. Obstet Gynecol. 2018 Feb;131 2):e65-e77. Open 2. https://obgyn.onlinelibrary.wiley.com/doi/./-. Diagnosis and Management of Ectopic Pregnancy: Green-top Guideline No. 21. BJOG. 2016 Dec;123 13):e15-e55. Open 3. Leslie Po, Jacqueline Thomas, Kelsey Mills et al. Guideline No. 414 Management of Pregnancy of Unknown Location and Tubal and Nontubal Ectopic Pregnancies. J Obstet Gynaecol Can. 2021 https://web.pathway.md/diseases/recGWlD9mm9btrJfc 8/9 6/29/23, 4:15 AM Ectopic pregnancy Pathway May;43 5 614 630.e1. Open 4. American College of Obstetricians and Gynecologists. Practice Bulletin No. 181 Prevention of Rh D Alloimmunization. Obstet Gynecol. 2017 Aug;130 2):e57-e70. Open 5. No authors listed. Management of Gestational Trophoblastic Disease: Green-top Guideline No. 38 June 2020. BJOG. 2021 Feb;128 3):e1-e27. Open 6. Simel D, Rennie D. The Rational Clinical Examination: Evidence-Based Clinical Diagnosis. Mayo Clin Proc. 2009 Nov; 84 11 1045. Open 7. Crochet JR, Bastian LA, Chireau MV. Does this woman have an ectopic pregnancy? the rational clinical examination systematic review. JAMA. 2013 Apr 24;309 16 1722 9. Open https://web.pathway.md/diseases/recGWlD9mm9btrJfc 9/9 |
Guideline sources The following summarized guidelines for the evaluation and management of encephalitis are prepared by our editorial team based on guidelines from the Association of British Neurologists (ABN/BIANG 2012), the Association of British Neurologists (ABN/BPAIIG 2012), and the Infectious Diseases Society of America (IDSA 2008). 1 2 3 Guidelines 1. Screening and diagnosis Clinical presentation: suspect encephalitis or another CNS infection in patients with current or recent febrile illness with altered behavior, cognition, personality or consciousness, or new seizures or focal neurological signs. B Show 4 more 2. Diagnostic investigations Clinical assessment: assess for epidemiologic clues and risk factors to identify potential etiologic agents in patients with encephalitis. B Show 2 more Evaluation for infectious etiology: https://web.pathway.md/diseases/rec48qDhvhP5jAbgq 1/6 6/29/23, 4:15 AM Encephalitis Pathway Evaluation for infectious etiology: As per ABN 2012 guidelines, determine investigation priority based on the patient's clinical presentation. B Show 15 more As per IDSA 2008 guidelines, obtain specific diagnostic studies in most patients with encephalitis: Situation Guidance Culture of respiratory secretions and nasopharynx, throat, and stool specimens Viruses DFA of sputum for respiratory viruses Blood cultures Bacteria CSF cultures Serologic testing (acute and convalescent phase) for Mycoplasma pneumoniae PCR of respiratory secretions for Mycoplasma pneumoniae Serologic testing (acute and convalescent phase) for Rickettsia rickettsi, Ehrlichia chaffeensis, and Anaplasma phagocytophilum Rickettsiae and ehrlichiae DFA and PCR of skin biopsy specimen (if rash present) for Rickettsia rickettsiae Blood smears for morulae PCR of whole blood and CSF specimens for Ehrlichia and Anaplasma species Serum RPR and FTA-ABS Spirochetes Serologic testing for Borrelia burgdorferi (ELISA and Western blot) CSF Venereal Disease Research Laboratory CSF Borrelia burgdorferi serologic testing (ELISA and Western blot) Calculate IgG antibody index CSF FTA- ABS CXR Mycobacteria PCR and culture of respiratory secretions CSF AFB smear and culture CSF PCR (Gen-Probe amplified Mycobacterium tuberculosis direct test) Blood cultures Fungi CSF cultures https://web.pathway.md/diseases/rec48qDhvhP5jAbgq 2/6 6/29/23, 4:15 AM Encephalitis Pathway Serum and CSF cryptococcal antigen Urine and CSF Histoplama antigen Serum IgG for Toxoplasma gondii in immunocompromised patients. B Protozoa Show 10 more Neuroimaging: As per ABN 2012 guidelines, obtain MRI (including DWI) as the preferred imaging modality as soon as possible (ideally 24 hours of hospital admission, but certainly within 48 hours) in all patients with suspected encephalitis, if the diagnosis is uncertain. B Show 3 more As per IDSA 2008 guidelines, obtain MRI for the evaluation of patients with encephalitis. A Obtain CT, with and without contrast enhancement, if MRI is unavailable, impractical, or cannot be obtained. B Electroencephalography: As per ABN 2012 guidelines, do not obtain EEG routinely in all patients with suspected encephalitis. D Show 2 more As per ABN 2012 guidelines, obtain an EEG in pediatric patients with suspected chronic viral encephalitis, such as subacute sclerosing panencephalitis. B As per IDSA 2008 guidelines, obtain an EEG in patients with encephalitis to identify nonconvulsive seizure activity in confused, obtunded, or comatose patients. B 3. Diagnostic procedures Lumbar puncture: perform lumbar puncture as soon as possible after hospital admission in all patients with suspected encephalitis, unless there is a clinical contraindication. B Consider performing an immediate lumbar puncture after CT on a case-by-case basis, unless the imaging reveals significant brain shift or tight basal cisterns due to or causing raised ICP or an alternative diagnosis or the patient's clinical condition changes. B Show 7 more Brain biopsy: As per ABN 2012 guidelines, do not perform a brain biopsy in the initial assessment of suspected acute viral encephalitis. Consider performing a stereotactic biopsy in patients with suspected encephalitis if no diagnosis has been made after the first week, especially if there are focal abnormalities on imaging. D Show 2 more As per IDSA 2008 guidelines: Do not perform brain biopsy routinely in patients with encephalitis. D Consider performing brain biopsy in patients with encephalitis of unknown etiology with deteriorating condition despite treatment with acyclovir. C https://web.pathway.md/diseases/rec48qDhvhP5jAbgq 3/6 6/29/23, 4:15 AM Encephalitis Pathway 4. Medical management Setting of care: obtain immediate neurological specialist consultation in patients with suspected acute encephalitis and manage them in a setting where a clinical neurological review can be obtained as soon as possible and definitely within 24 hours of referral. B Show 4 more Empirical therapy: As per ABN 2012 guidelines, initiate IV aciclovir (10 mg/kg TID) if the initial CSF and/or imaging findings suggest viral encephalitis, or within 6 hours of admission if these results will not be available, or if the patient is very unwell or deteriorating. B Show 3 more As per IDSA 2008 guidelines, initiate acyclovir in all patients with suspected encephalitis, pending results of diagnostic studies. B Show 2 more Specific therapy, viruses: As per BIANG/ABN 2012 guidelines, continue IV acyclovir treatment for 14-21 days in patients with proven HSV encephalitis, B and perform a repeat lumbar puncture at this time to confirm the CSF is negative for HSV by PCR. Continue IV acyclovir if the CSF is still positive with weekly PCR until it is negative. B Show 8 more As per IDSA 2008 guidelines, administer acyclovir in patients with HSV infection. A Show 12 more Specific therapy (bacteria): administer chloramphenicol, ciprofloxacin, doxycycline, ampicillin, or TMP-SMX in patients with encephalitis caused by Bartonella bacilliformis. B Show 4 more Specific therapy (mycobacteria): complete a 4-drug antituberculous therapy in patients with encephalitis caused by Mycobacterium tuberculosis. B Add adjunctive dexamethasone in patients with meningitis. B Specific therapy (rickettsiae and ehrlichiae): administer doxycycline in patients with encephalitis caused by Anaplasma phagocytophilum. B Show 3 more Specific therapy (spirochetes): Administer ceftriaxone, cefotaxime, or penicillin G in patients with Borrelia burgdorferi encephalitis. B Administer penicillin G, B or ceftriaxone as an alternative, B in patients with Treponema pallidum encephalitis. B Specific therapy (fungi): administer fluconazole as the first-line therapy in patients with encephalitis caused by Coccidoides species. B Consider administering itraconazole B , voriconazole B , or IV or intrathecal amphotericin B B as second-line choices. B Show 2 more Specific therapy (protozoa): consider administering TMP-SMX plus rifampin plus ketoconazole or fluconazole plus sulfadiazine plus pyrimethamine in patients with Acanthamoeba encephalitis. C https://web.pathway.md/diseases/rec48qDhvhP5jAbgq 4/6 6/29/23, 4:15 AM Encephalitis Pathway Show 6 more Specific therapy (helminths): consider administering albendazole plus diethylcarbamazine in patients with encephalitis caused by Baylisascaris procyonis. C Consider administering adjunctive corticosteroids. C Show 2 more Specific therapy (acute disseminated encephalomyelitis): administer high-dose corticosteroids in patients with acute disseminated encephalomyelitis. B Perform plasma exchange B and administer IVIG as an alternative option. B 5. Specific circumstances Patients with autoimmune encephalitis: include antibody-mediated encephalitis in the differential diagnosis of all patients with suspected encephalitis because of the poor outcomes if left untreated. B Show 3 more 6. Follow-up and surveillance Follow-up: Do not discharge patients from the hospital without either a definite or suspected diagnosis. Formulate an arrangement for the outpatient follow-up (to include at least one follow-up appointment) and plans for ongoing therapy and rehabilitation at a discharge meeting. D Ensure that all patients have access to assessment for rehabilitation, irrespective of age. B Clinical findings Symptoms Past medical history Chills Immunocompromising condition Confusion Radiation therapy to the head area Emotional lability Fatigue Lab findings Fever Headache CSF RBCs Myalgia CSF protein Nausea Imaging findings Personality or behavioral changes Cerebral edema Seizure Sleepiness Cytological findings Vomiting CSF WBC count Neurological exam Altered mental status https://web.pathway.md/diseases/rec48qDhvhP5jAbgq 5/6 6/29/23, 4:15 AM Encephalitis Pathway Aphasia Ataxia Coma Cranial nerve palsy Focal neurologic deficits Hemiparesis Meningismus Speech disturbance Hematological findings WBC count blood leukocyte count blood platelet count EEG findings Nonconvulsive status epilepticus Periodic lateralizing epileptiform discharges References 1. T Solomon, B D Michael, P E Smith et al. Management of suspected viral encephalitis in adults Association of British Neurologists and British Infection Association National Guidelines. J Infect. 2012 Apr;64 4 347 73. Open 2. Tunkel AR, Glaser CA, Bloch KC et al. The management of encephalitis: clinical practice guidelines by the Infectious Diseases Society of America. Clin Infect Dis. 2008 Aug 1;47 3 303 27. Open 3. R Kneen, B D Michael, E Menson et al. Management of suspected viral encephalitis in children Association of British Neurologists and British Paediatric Allergy, Immunology and Infection Group national guidelines. J Infect. 2012 May;64 5 449 77. Open https://web.pathway.md/diseases/rec48qDhvhP5jAbgq 6/6 |
Guideline sources The following summarized guidelines for the evaluation and management of endometrial cancer are prepared by our editorial team based on guidelines from the European Society of Medical Oncology (ESMO 2022), the American Society for Radiation Oncology (ASTRO 2022), the European Society for Radiotherapy and Oncology (ESTRO/ESP/ESGO 2021), the Society of Obstetricians and Gynaecologists of Canada (SOGC 2018), the U.S. Preventive Services Task Force (USPSTF 2017), and the Society of Gynecologic Oncology (SGO/ACOG 2015). 1 2 3 4 5 6 7 Calculator Calculator Calculat Eastern Cooperative Oncology G FIGO staging of uterine corpus c Karnof https://web.pathway.md/diseases/recof2ZFRnHotHQ7q 1/11 6/29/23, 4:15 AM Endometrial cancer Pathway Guidelines 1. Screening and diagnosis Indications for screening: insufficient evidence to assess the balance of benefits and harms of performing screening pelvic examinations in asymptomatic, nonpregnant adult females. I Indications for testing: As per SOGC 2018 guidelines, assess for and exclude endometrial cancer in perimenopausal and postmenopausal patients with abnormal vaginal bleeding. B As per SOGC 2018 guidelines, perform endometrial tissue sampling in postmenopausal patients with bleeding and an endometrial thickness of 4-5. B Do not perform routine endometrial sampling in postmenopausal females without bleeding. B Show 3 more 2. Classification and risk stratification Surgical staging: perform comprehensive surgical staging with total hysterectomy, bilateral salpingo-oophorectomy, pelvic and para-aortic lymphadenectomy, and the collection of peritoneal cytology in patients with endometrial cancer. Make exceptions to this approach only after consultation with a practitioner specializing in the treatment of endometrial cancer, such as a gynecologic oncologist. A Show 2 more 3. Diagnostic investigations Initial evaluation: elicit a complete focused history and perform a physical examination in patients with suspected endometrial cancer. Pay attention to predisposing factors for excess estrogen stimulation of the endometrium, such as a long history of anovulation, obesity, menstrual irregularity, or long-term use of unopposed estrogen or tamoxifen. B Show 2 more 4. Diagnostic procedures Hysteroscopy: As per SOGC 2018 guidelines, consider performing a hysteroscopic examination in patients with persistent uterine bleeding with benign endometrial sampling or insufficient endometrial sampling after ultrasound. C As per ACOG 2015 guidelines, perform hysteroscopy with directed dilation and curettage to include any discrete lesions as well as the background endometrium, to confirm the diagnosis of a true premalignant endometrial lesion and exclude an associated endometrial carcinoma. A Biopsy and histopathology: https://web.pathway.md/diseases/recof2ZFRnHotHQ7q 2/11 6/29/23, 4:15 AM Endometrial cancer Pathway As per ESMO 2022 guidelines: Perform endometrial sampling by biopsy or dilation and curettage for histological evaluation of endometrial cancer. B Describe the histological type, the FIGO grade, myometrial invasion, and lymphovascular space invasion (focal/substantial) in all endometrial cancer pathology specimens. B As per ESGO 2021 guidelines, record the histopathologic type, grade, myometrial invasion, and lymphovascular space invasion (no/focal/substantial) in all patients with endometrial carcinoma. B As per SOGC 2018 guidelines: Obtain histologic evaluation of the endometrium in all patients with suspected endometrial cancer. B Consider obtaining a formal review of the histopathology in patients with high-grade tumors or rare histologic types, such as serous, clear cell, or mucinous types. C As per ACOG 2015 guidelines, perform endometrial sampling in an outpatient setting with disposable devices for histologic evaluation of the endometrium. A Show 3 more Molecular testing: As per ASTRO 2022 guidelines, obtain molecular testing in patients with endometrial cancer eligible for adjuvant therapy: obtain immunohistochemistry to assess for mutations in mismatch repair and TP53 genes consider obtaining POLE sequencing to identify hypermutated tumors. B As per ESMO 2022 guidelines, obtain molecular classification through well-established immunohistochemistry staining for p53 and mismatch repair proteins (MLH1, PMS2, MSH2, MSH6) in combination with targeted tumor sequencing (POLE hotspot analysis) in all endometrial cancer pathology specimens regardless of histological type. B As per ESGO 2022 guidelines, obtain molecular classification in all endometrial carcinomas, especially high-grade tumors. B Show 2 more 5. Medical management Setting of care: Plan staging and treatment on a multidisciplinary basis (generally at a tumor board meeting, composed according to local guidelines) and based on the comprehensive and precise knowledge of prognostic and predictive factors for outcomes, morbidity, and quality of life. B Treat patients in a specialized center by a dedicated team of specialists in the diagnosis and management of gynecological cancers, especially in high-risk and/or advanced-stage diseases. B Management of local/locoregional disease, total hysterectomy: As per ESMO 2022 guidelines, perform hysterectomy with bilateral salpingo-oophorectomy as the standard surgical procedure in patients with early-stage endometrial cancer. A Show 2 more https://web.pathway.md/diseases/recof2ZFRnHotHQ7q 3/11 6/29/23, 4:15 AM Endometrial cancer Pathway As per ESP/ESTRO/ESGO 2021 guidelines, perform total hysterectomy with bilateral salpingo-oophorectomy without vaginal cuff resection as the standard surgery in patients with stage I/II endometrial cancer. B Perform total hysterectomy with bilateral salpingo- oophorectomy and lymph node staging as the surgical standard of care in patients with stage II endometrial carcinoma. B Show 4 more As per SGO/ACOG 2015 guidelines: Consider performing vaginal hysterectomy in selected patients with early-stage endometrioid endometrial cancer deemed at high risk of surgical morbidity. C Assess the risk of extrauterine disease and potential for disease recurrence based on age, histologic cell type, and uterine tumor features in patients found to have endometrial cancer incidentally after hysterectomy. Consider individualizing treatment plans based on the findings. B Management of local/locoregional disease, lymph node excision and surgical restaging: As per ASTRO 2022 guidelines, perform bilateral sentinel lymph node mapping over standard pelvic lymphadenectomy to accurately detect subclinical nodal metastases, decrease morbidity, and guide the selection of adjuvant therapy in patients with endometrial cancer. B Show 3 more As per ESMO 2022 guidelines, consider performing sentinel lymphadenectomy for nodal assessment in patients with low-risk/intermediate-risk endometrial cancer, i.e. stage IA G1- G3 and stage IB G1-G2. B Consider omitting it in cases without myometrial invasion. Do not perform systematic lymphadenectomy in these patients. C Show 2 more As per ESP/ESTRO/ESGO 2021 guidelines, perform staging infracolic omentectomy in clinical stage I serous endometrial carcinoma, carcinosarcoma, and undifferentiated carcinoma. Consider omitting it in clear cell and endometrioid carcinoma in stage I disease. B Show 8 more As per SGO/ACOG 2015 guidelines: Consider performing lymphadenectomy to alter or eliminate the need for adjuvant therapy and its associated morbidity. C Avoid performing systematic lymphadenectomy in patients with low-grade disease (grade 1 or grade 2 endometrioid lesions with < 50% myometrial invasion and tumor size of 2 cm) as they are deemed to be at low risk of lymph node metastases. D Management of local/locoregional disease, ovarian preservation: As per ESMO 2022 guidelines, consider performing ovarian preservation in premenopausal patients with stage IA G1 endometrial endometrioid carcinoma. B As per ESP/ESTRO/ESGO 2021 guidelines, consider performing ovarian preservation in < 45 years old premenopausal patients with low-grade endometrioid endometrial carcinoma with myometrial invasion < 50% and no obvious ovarian or other extrauterine diseases. B Show 2 more https://web.pathway.md/diseases/recof2ZFRnHotHQ7q 4/11 6/29/23, 4:15 AM Endometrial cancer Pathway As per SGO/ACOG 2015 guidelines, consider performing ovarian conservation at the time of hysterectomy in premenopausal patients with endometrial cancer wishing to retain ovarian function, but individualizing the decision after evaluation of the risk of extrauterine disease and the potential for disease recurrence based on age, histologic cell type, and uterine tumor features. C Management of local/locoregional disease, adjuvant therapy: As per ASTRO 2022 guidelines, do not offer adjuvant radiotherapy in patients with FIGO stage IA grade 1 or 2 endometrioid carcinoma without intermediate- or high-risk factors. D Show 10 more As per ESMO 2022 guidelines, offer adjuvant vaginal brachytherapy to decrease vaginal recurrence in patients with stage IA G3 or IB G1-G2 endometrioid (mismatch repair deficient and no specific molecular profile) type and no or focal lymphovascular space invasion. A Show 4 more As per ESP/ESTRO/ESGO 2021 guidelines, do not offer adjuvant treatment in patients with low-risk endometrial carcinoma. D Show 6 more As per SGO/ACOG 2015 guidelines: Offer adjuvant radiotherapy in patients with certain stage I and stage II endometrial carcinomas to reduce the local recurrence rate. A Offer vaginal brachytherapy over whole pelvic irradiation as the adjuvant treatment of choice in certain patients with a high-intermediate risk of recurrent endometrial cancer. A Management of local/locoregional disease, systemic therapy: As per ASTRO 2022 guidelines, do not offer systemic therapy in patients with FIGO stage I-II endometroid adenocarcinoma. D Consider offering systemic therapy in patients with myoinvasive FIGO stage I-II endometrial cancer with high-risk histologies. D As per ESMO 2022 guidelines, do not offer adjuvant treatment in patients with stage IA (G1 and G2) with endometrioid (mismatch repair deficient and no specific molecular profile) type and no or focal lymphovascular invasion. D Show 4 more Management of local/locoregional disease (medically unfit patients): consider offering vaginal hysterectomy, with bilateral salpingo-oophorectomy if feasible, in patients unfit for standard surgical therapy. C Show 2 more Management of local/locoregional disease (fertility preservation): refer patients eligible for fertility-preserving treatment to specialized centers. Consider offering fertility-sparing treatment only in patients with atypical hyperplasia/endometrioid intra-epithelial neoplasia or grade 1 endometrioid endometrial carcinoma without myometrial invasion and without genetic risk factors. B Perform endometrial biopsy, preferably through hysteroscopy, in these patients. B Confirm the diagnosis of atypical hyperplasia/endometrioid intra-epithelial neoplasia or grade 1 endometrioid endometrial carcinoma by a pathologist experienced in gynecological pathology B Show 10 more https://web.pathway.md/diseases/recof2ZFRnHotHQ7q 5/11 6/29/23, 4:15 AM Endometrial cancer Pathway Management of local/locoregional disease (estrogen therapy): consider offering estrogen therapy, after thorough counseling about the risks and benefits, for the management of menopausal symptoms in the survivors of early-stage endometrial cancer. B Management of advanced/metastatic disease: As per ASTRO 2022 guidelines, offer adjuvant systemic therapy in patients with FIGO stage III-IVA endometrial cancer of any histology. A Consider offering EBRT with chemotherapy to decrease locoregional recurrence. Consider offering EBRT with concurrent chemotherapy followed by adjuvant chemotherapy in patients receiving radiotherapy. B Consider offering sequential chemotherapy followed by radiotherapy in patients receiving radiotherapy. E As per ESMO 2022 guidelines, consider offering hormone therapy with progestins, i.e. medroxyprogesterone acetate 200 mg or megestrol acetate 160 mg, B or with other options including aromatase inhibitors, tamoxifen, and fulvestrant, C as front-line systemic therapy in patients with low-grade carcinomas endometrioid histology. B Show 5 more As per ESGO 2021 guidelines, consider performing surgical tumor debulking including enlarged lymph nodes in patients with stage III-IV endometrial carcinoma (including carcinosarcoma), if complete macroscopic resection is feasible with acceptable morbidity and quality of life profile, following full preoperative staging and discussion by a multidisciplinary team. C Show 5 more As per ACOG 2015 guidelines, offer chemotherapy for the management of patients with advanced endometrial cancer. A Consider offering a combination of chemotherapy and radiotherapy. B Show 2 more 6. Therapeutic procedures Technical considerations for radiotherapy: deliver intensity-modulated radiotherapy to reduce acute and late toxicity in patients with endometrial carcinoma undergoing adjuvant EBRT. B Show 4 more 7. Perioperative care Preoperative staging: As per ESMO 2022 guidelines: Include the following in the preoperative work-up: clinical and gynecological examination transvaginal ultrasound, pelvic MRI CBC, liver and renal function profiles. B Consider obtaining additional imaging (such as thoracic and abdominal CT and/or FDG- positron emission tomography-CT) in patients at high risk of extrapelvic disease. C As per ESGO 2021 guidelines, include the following in the preoperative work-up: https://web.pathway.md/diseases/recof2ZFRnHotHQ7q 6/11 6/29/23, 4:15 AM Endometrial cancer Pathway family history general assessment and inventory of comorbidities geriatric assessment, if appropriate clinical examination, including pelvic examination expert transvaginal or transrectal ultrasound or pelvic MRI. B Show 2 more As per ACOG 2015 guidelines: Do obtain routine preoperative imaging for the evaluation of metastases. A Consider obtaining a preoperative assessment of metastatic disease with imaging (CT, MRI, or positron emission tomography/CT) or measurement of serum CA-125, or both, under special circumstances, such as when the patient is a poor surgical candidate because of medical comorbidities, when symptoms suggest possible metastasis to unusual sites (such as bones or the CNS), and when preoperative histology demonstrates a high- grade carcinoma (including grade 3 endometrioid, papillary serous, clear cell, and carcinosarcoma). C 8. Specific circumstances Patients with Lynch syndrome: obtain mismatch repair immunohistochemistry (plus analysis of MLH1 promotor methylation status in case of immunohistochemical loss of MLH1/PMS2 expression) or MSI tests in all endometrial carcinomas, irrespective of histologic subtype of the tumor, to identify patients with Lynch syndrome and triage for germline mutational analysis. B Show 3 more 9. Patient education General counseling: counsel patients about the suggested diagnostic and treatment plan and potential alternatives, including risks and benefits of all options. B Genetic counseling: As per ESGO 2021 guidelines, provide genetic counseling in patients with endometrial carcinoma as having an increased risk of Lynch syndrome. B As per SOGC 2018 guidelines, provide genetic counseling and testing to individualize risk- management interventions including screening strategies and treatment options in patients at increased risk of having inherited Lynch syndrome (such as a strong family history of endometrial, ovarian, and colorectal cancers) increasing the lifetime risk of developing endometrial cancer. B 10. Follow-up and surveillance Follow-up: As per ESMO 2022 guidelines, perform physical and gynecological examinations every 6 months for the first 2 years and then yearly until 5 years in patients with low-risk endometrial https://web.pathway.md/diseases/recof2ZFRnHotHQ7q 7/11 6/29/23, 4:15 AM Endometrial cancer Pathway cancer B Consider arranging phone follow-ups as an alternative to hospital-based follow- up consultations in low-risk patients. B Show 2 more As per ACOG 2015 guidelines: Follow up patients after treatment of endometrial cancer every 3-6 months for 2 years, then every 6 months for 3 years, and annually thereafter. Elicit a thorough patient history, assess for new symptoms associated with recurrence (such as vaginal bleeding, pelvic pain, weight loss, or lethargy), and perform a thorough speculum, pelvic, and rectovaginal examination during follow-ups. Do not obtain vaginal cytologic evaluation and annual CXRs because most vaginal recurrences are detected with clinical examination alone and CXR is of low utility in detecting asymptomatic recurrence. B Obtain radiologic evaluation, such as CT or positron emission tomography/CT of the chest, abdomen, and pelvis, only to investigate suspicion of recurrent disease and not for routine surveillance after treatment. B Management of recurrent disease: As per ESMO 2022 guidelines, offer radiotherapy with vaginal brachytherapy as the preferred primary therapy in patients with locoregional recurrence following primary surgery. B Consider adding systemic therapy to salvage radiotherapy. B Show 2 more As per ESGO 2021 guidelines, consider performing surgery in patients with recurrent disease (including peritoneal and lymph node relapse) only if complete removal of macroscopic disease can be achieved with acceptable morbidity. Consider offering systemic treatment and/or radiotherapy postoperatively depending on the extent and pattern of relapse and the amount of residual disease. C Show 16 more Clinical findings Symptoms Past medical history Abdominal pain Cervical cancer Abnormal vaginal bleeding Colorectal cancer Bloating Diabetes mellitus Change in bowel habits Lynch syndrome Early satiety Nulliparity Menstrual irregularity Obesity Pelvic pain PCOS Vaginal bleeding Past obstetric history Medication history Postmenopause Estrogens Abdominal exam Tamoxifen Abdominal distension Family history Imaging findings Lynch syndrome https://web.pathway.md/diseases/recof2ZFRnHotHQ7q 8/11 6/29/23, 4:15 AM Endometrial cancer Pathway Ovarian cancer Endometrial hyperplasia Lab findings HE4 serum cancer antigen 125 Likelihood Ratios Pertinent positives The following findings increase the probability of endometrial cancer in adults. 7 Finding LR+ Value Presence of age 70* 8.2 History of estrogens 3.9 Presence of diabetes mellitus 2.6 History of nulliparity 2.3 in patients with abnormal vaginal bleeding Show 1 more Pertinent negatives The following findings decrease the probability of endometrial cancer in adults. 7 Finding LR- Value No history of postmenopause 0.4 Absence of age 70* 0.8 No history of nulliparity 0.8 No history of estrogens 0.9 in patients with abnormal vaginal bleeding Show 1 more Studies 2019 PORTEC-3 (post-hoc) In women with high-risk endometrial cancer, chemoradiotherapy was superior to radiotherapy alone with respect to overall survival at 5 years. Stephanie M de Boer et al. Lancet Oncol. 2019 Sep. 2018 PORTEC-3 In women with high-risk endometrial cancer, chemoradiotherapy was not superior to radiotherapy with respect to overall survival at 5 years. Stephanie M de Boer et al. Lancet Oncol. 2018 Mar. Show 2 more https://web.pathway.md/diseases/recof2ZFRnHotHQ7q 9/11 6/29/23, 4:15 AM Endometrial cancer Pathway References 1. Nicole Concin, Xavier Matias-Guiu, Ignace Vergote et al. ESGO/ESTRO/ESP guidelines for the management of patients with endometrial carcinoma. Int J Gynecol Cancer. 2021 Jan;31 1 12 39. Open 2. A Oaknin, T J Bosse, C L Creutzberg et al. Endometrial cancer: ESMO Clinical Practice Guideline for diagnosis, treatment and follow-up. Ann Oncol. 2022 Sep;33 9 860 877. Open 3. Matthew M Harkenrider, Nadeem Abu-Rustum, Kevin Albuquerque et al. Radiation Therapy for Endometrial Cancer: An ASTRO Clinical Practice Guideline. Pract Radiat Oncol. 2022 Oct 21;S1879 8500 22 00273 9. Open 4. Marie-Claude Renaud, Tien Le. No. 291 Epidemiology and Investigations for Suspected Endometrial Cancer. J Obstet Gynaecol Can. 2018 Sep;40 9):e703-e711. Open 5. American College of Obstetricians and Gynecologists. Practice Bulletin No. 149 Endometrial cancer. Obstet Gynecol. 2015 Apr;125 4 1006 1026. Open 6. US Preventive Services Task Force, Kirsten Bibbins-Domingo, David C Grossman et al. Screening for Gynecologic Conditions With Pelvic Examination: US Preventive Services Task Force Recommendation Statement. JAMA. 2017 Mar 7;317 9 947 953. Open 7. Wendy Wolfman. No. 249 Asymptomatic Endometrial Thickening. J Obstet Gynaecol Can. 2018 May;40 5):e367-e377. Open 8. Feldman S, Cook EF, Harlow BL et al. Predicting endometrial cancer among older women who present with abnormal vaginal bleeding. Gynecol Oncol. 1995 Mar;56 3 376 81. Open 9. Lambert M. Cancer Screening Recommendations from the ACS A Summary of the 2017 Guidelines. Am Fam Physician. 2018 Feb 1;97 3 208 210. Open 10. American Society for Radiation Oncology. Choosing Wisely ASRO recommendations. Choosing Wisely. 2014. Open 11. Society of Gynecologic Oncology. Choosing Wisely SGO recommendations. Choosing Wisely. 2013. Open 12. Koh WJ, Abu-Rustum NR, Bean S et al. Uterine Neoplasms, Version 1.2018, NCCN Clinical Practice Guidelines in Oncology. J Natl Compr Canc Netw. 2018 Feb;16 2 170 199. Open 13. Carien L Creutzberg, Remi A Nout, Marnix L M Lybeert et al. Fifteen-year radiotherapy outcomes of the randomized PORTEC 1 trial for endometrial carcinoma. Int J Radiat Oncol Biol Phys. 2011 Nov 15;81 4):e631 8. Open 14. B G Wortman, C L Creutzberg, H Putter et al. Ten-year results of the PORTEC 2 trial for high- intermediate risk endometrial carcinoma: improving patient selection for adjuvant therapy. Br J Cancer. 2018 Oct;119 9 1067 1074. Open 15. Stephanie M de Boer, Melanie E Powell, Linda Mileshkin et al. Adjuvant chemoradiotherapy versus radiotherapy alone for women with high-risk endometrial cancer PORTEC 3 final results of an international, open-label, multicentre, randomised, phase 3 trial. Lancet Oncol. 2018 Mar;19 3 295 309. Open 16. Stephanie M de Boer, Melanie E Powell, Linda Mileshkin et al. Adjuvant chemoradiotherapy versus radiotherapy alone in women with high-risk endometrial cancer PORTEC 3 patterns of recurrence https://web.pathway.md/diseases/recof2ZFRnHotHQ7q 10/11 6/29/23, 4:15 AM Endometrial cancer Pathway and post-hoc survival analysis of a randomised phase 3 trial. Lancet Oncol. 2019 Sep;20 9 1273 1285. Open 17. SGO Clinical Practice Endometrial Cancer Working Group, William M Burke, James Orr et al. Endometrial cancer: a review and current management strategies: part I. Gynecol Oncol. 2014 Aug;134 2 385 92. Open 18. SGO Clinical Practice Endometrial Cancer Working Group, William M Burke, James Orr et al. Endometrial cancer: a review and current management strategies: part II. Gynecol Oncol. 2014 Aug;134 2 393 402. Open 19. N Colombo, E Preti, F Landoni et al. Endometrial cancer: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2013 Oct;24 Suppl 6:vi33 8. Open 20. Ann Klopp, Benjamin D Smith, Kaled Alektiar et al. The role of postoperative radiation therapy for endometrial cancer: Executive summary of an American Society for Radiation Oncology evidence- based guideline. Pract Radiat Oncol. 2014 May-Jun;4 3 137 144. Open 21. Martin Koskas, Fr d ric Amant, Mansoor Raza Mirza et al. Cancer of the corpus uteri: 2021 update. Int J Gynaecol Obstet. 2021 Oct;155 Suppl 1 Suppl 1 45 60. Open https://web.pathway.md/diseases/recof2ZFRnHotHQ7q 11/11 |
Guideline sources The following summarized guidelines for the evaluation and management of endometriosis are prepared by our editorial team based on guidelines from the Society of Obstetricians and Gynaecologists of Canada (SOGC 2023; 2010), the European Society of Human Reproduction and Embryology (ESHRE 2022), and the American College of Obstetricians and Gynecologists (ACOG 2018; 2016; 2010). 1 2 3 4 5 6 Guidelines 1. Screening and diagnosis Clinical presentation: Recognize that: https://web.pathway.md/diseases/recjQIQ99LVWEV83Z 1/9 6/29/23, 4:16 AM Endometriosis Pathway symptoms of endometriosis may vary, but certain hallmark symptoms may be more likely to suggest endometriosis; recognize the atypical presentations A endometriosis can be a chronic, relapsing disorder, which may necessitate a long-term follow-up A adolescent patients with endometriosis are more likely than adult patients to present with acyclic pain B physical examination of adolescent patients with endometriosis will rarely reveal abnormalities, as most will have early-stage disease B endometriosis in adolescent patients is often early-stage and atypical. B Diagnosis: Suspect the diagnosis of endometriosis in patients presenting with the following cyclical and non-cyclical signs and symptoms: dysmenorrhea deep dyspareunia dysuria dyschezia painful rectal bleeding or hematuria shoulder tip pain catamenial pneumothorax cyclical cough/hemoptysis/chest pain cyclical scar swelling and pain fatigue infertility. B 2. Diagnostic investigations History and physical examination: As per ESHRE 2022 guidelines, consider performing a clinical examination, including vaginal examination where appropriate, to identify deep nodules or endometriomas in patients with suspected endometriosis, although the diagnostic accuracy is low. B Elicit a history and perform a physical examination for the evaluation of patients with suspected endometriosis. B Perform a pelvic examination, including rectovaginal examination, when deeply infiltrating endometriosis is suspected. B Diagnostic imaging: As per ESHRE 2022 guidelines: Consider obtaining imaging in patients with suspected endometriosis, even if clinical examination is normal. B Obtain ultrasound or MRI in the diagnostic work-up for endometriosis, recognizing that a negative finding does not exclude endometriosis, particularly superficial peritoneal disease. A https://web.pathway.md/diseases/recjQIQ99LVWEV83Z 2/9 6/29/23, 4:16 AM Endometriosis Pathway As per SOGC 2010 guidelines, obtain imaging assessment for the evaluation of patients with suspected endometriosis. B Laboratory studies: As per ESHRE 2022 guidelines: Do not obtain biomarkers in endometrial, blood, menstrual or uterine fluids for the diagnosis of endometriosis. D Obtain genetic testing in patients with suspected or confirmed endometriosis only within a research setting. As per SOGC 2010 guidelines, do not obtain routine CA-125 testing as part of the diagnostic investigation of endometriosis. D 3. Diagnostic procedures Diagnostic laparoscopy: consider performing laparoscopy for the diagnosis and treatment of suspected endometriosis in patients with negative imaging results or if empirical treatment was unsuccessful or inappropriate. C Biopsy: As per ESHRE 2022 guidelines, perform biopsy to confirm laparoscopic identification of endometriotic lesions, although recognize that negative histology does not entirely rule out the disease. B As per SOGC 2010 guidelines: Consider performing biopsy of endometriosis lesions to confirm the diagnosis and rule out underlying malignancy. B Consider performing excision or sampling of suspected endometriosis lesions and endometriomas to help in confirming the diagnosis and excluding underlying malignancy. C 4. Medical management Hormone therapy: As per ESHRE 2022 guidelines, offer hormone treatment (combined hormonal contraceptives, progestogens, gonadotropin releasing hormone agonists or gonadotropin releasing hormone antagonists) as an option to reduce endometriosis-associated pain. A Show 4 more As per SOGC 2010 guidelines, consider using the following agents as first-line therapy in patients with endometriosis: combined hormonal contraceptives (ideally administered continuously) progestin alone (PO, IM, or subcutaneous). B Show 2 more Gonadotropin-releasing hormone agonists/antagonists: As per ESHRE 2022 guidelines, offer GnRH agonists as second-line therapy, for example if hormonal contraceptives or progestogens have been ineffective B , for endometriosis- https://web.pathway.md/diseases/recjQIQ99LVWEV83Z 3/9 6/29/23, 4:16 AM Endometriosis Pathway associated pain, recognizing that insufficient evidence regarding dosage or duration of treatment. A Show 2 more As per SOGC 2010 guidelines: Consider offering GnRH agonist with add-back hormone therapy, or levonorgestrel- releasing intrauterine system, as second-line therapy in patients with endometriosis. B Combine aGnRH agonist with add-back hormone therapy when initiating the therapy, and consider using the combination for longer-term (> 6 months). A Aromatase inhibitors: offer aromatase inhibitors in patients with endometriosis-associated pain refractory to other medical or surgical treatment. Consider initiating aromatase inhibitors in combination with oral contraceptives, progestogens, gonadotropin releasing hormone agonists or GnRH antagonists. A Analgesics: As per ESHRE 2022 guidelines, consider offering NSAIDs or other analgesics (either alone or in combination with other treatments) to reduce endometriosis-associated pain. C As per SOGC 2010 guidelines, prescribe analgesics ranging from NSAIDs to opioids while awaiting resolution of symptoms from directed medical or surgical treatments for endometriosis. B 5. Nonpharmacologic interventions Alternative and complementary therapies: Discuss non-medical strategies to address quality of life and psychological well-being in patients managing symptoms of endometriosis. B Insufficient evidence to recommend any specific non-medical intervention (Chinese medicine, nutrition, electrotherapy, acupuncture, physiotherapy, exercise, and psychological interventions) to reduce pain or improve quality of life measures in patients with endometriosis. I 6. Therapeutic procedures Hormone-releasing intrauterine device: offer a levonorgestrel-releasing intrauterine system or an etonogestrel-releasing subdermal implant to reduce endometriosis-associated pain in patients with endometriosis. A Fertility preservation: Discuss the pros and cons of fertility preservation with patients with extensive ovarian endometriosis. A Insufficient evidence regarding the true benefit of fertility preservation in patients with endometriosis. I 7. Perioperative care https://web.pathway.md/diseases/recjQIQ99LVWEV83Z 4/9 6/29/23, 4:16 AM Endometriosis Pathway Preoperative hormone therapy: do not offer preoperative hormone therapy to improve the immediate outcome of surgery for pain in patients with endometriosis. D Show 2 more 8. Surgical interventions Indications for surgery: As per ESHRE 2022 guidelines, offer surgery as one of the options to reduce endometriosis- associated pain. A Show 3 more As per SOGC 2010 guidelines, reserve surgical management in patients with endometriosis- related pain for those in whom medical treatment has failed. B Show 3 more Excision of endometrioma: As per SOGC 2023 guidelines: Perform laparoscopic resection of endometrioma if there are concerns about access to the follicles for egg retrieval for IVF. B Counsel patients comprehensively about the risk that endometrioma surgery will diminish ovarian reserve. A As per ESHRE 2022 guidelines, perform cystectomy instead of drainage and coagulation when performing surgery in patients with ovarian endometrioma, as cystectomy reduces recurrence of endometrioma and endometriosis-associated pain. A Show 2 more As per SOGC 2010 guidelines, perform excision of ovarian endometriomas > 3 cm in diameter in patients with pelvic pain, if possible. A Hysterectomy: as per ESHRE 2022 guidelines, consider performing hysterectomy (with or without removal of the ovaries) with removal of all visible endometriosis lesions in patients no longer wishing to conceive and failing to respond to more conservative treatments. Inform patients that hysterectomy will not necessarily cure the symptoms or the disease. C Show 2 more Presacral neurectomy: consider presacral neurectomy as an adjunct to the surgical treatment of patients with endometriosis-related pelvic pain. B 9. Specific circumstances Asymptomatic patients: As per ESHRE 2022 guidelines, inform and counsel patients about any incidental finding of endometriosis. B Show 3 more As per SOGC 2010 guidelines, do not offer any medical or surgical intervention for asymptomatic patients with incidental findings of endometriosis at the time of surgery. D Adolescent patients, diagnosis: https://web.pathway.md/diseases/recjQIQ99LVWEV83Z 5/9 6/29/23, 4:16 AM Endometriosis Pathway As per ESHRE 2022 guidelines, consider suspecting the diagnosis of endometriosis in young patients presenting with (cyclical) absenteeism from school or with use of oral contraceptives for treatment of dysmenorrhea. C Show 8 more As per ACOG 2018 guidelines, recognize that endometriosis is the most common cause of secondary dysmenorrhea in adolescents. E Show 4 more As per SOGC 2010 guidelines, consider offering all available therapies for endometriosis in adolescent patients taking into consideration the age of the patient and the side effect profiles of medications. B Adolescent patients, management: As per ESHRE 2022 guidelines, offer hormonal contraceptives or progestogens (systemically or via levonorgestrel intrauterine system) as first-line hormonal therapy in adolescent patients with severe dysmenorrhea and/or endometriosis-associated pain. Recognize that some progestogens may decrease bone mineral density. A Show 6 more As per ACOG 2018 guidelines, offer NSAIDs as the mainstay of pain relief in adolescent patients with endometriosis. E Show 3 more As per SOGC 2010 guidelines, elicit clear vesicles and red intra-abdominal lesions during laparoscopy in adolescent patients, as endometriosis in adolescent patients is often early- stage and atypical. B Adolescent patients (fertility preservation): Inform adolescent patients with endometriosis of the potential detrimental effect of ovarian endometriosis and surgery on ovarian reserve and future fertility. B Inform adolescent patients about the existing fertility preservation options, although recognize that the true benefit, safety and indications in adolescent patients with endometriosis remain unknown. B Pregnant patients: do not advise patients to become pregnant with the sole purpose of treating endometriosis, as pregnancy does not always lead to improvement of symptoms or reduction of disease progression. D Show 3 more Postmenopausal patients: recognize that endometriosis can still be active/symptomatic after menopause. E Show 7 more Patients with infertility (medical management): do not offer ovarian suppression treatment to improve fertility in infertile patients with endometriosis. D Show 3 more Patients with infertility, operative laparoscopy: As per SOGC 2023 guidelines, consider performing operative laparoscopy in patients with endometriosis and a history of infertility if there are other indications for surgery (such as pain), recognizing that it is not a first-line treatment for endometriosis-associated infertility. https://web.pathway.md/diseases/recjQIQ99LVWEV83Z 6/9 6/29/23, 4:16 AM Endometriosis Pathway Take into consideration the patient's age, duration of infertility, and ovarian reserve for counseling. C As per ESHRE 2022 guidelines, consider performing operative laparoscopy as a treatment option for endometriosis-associated infertility to improve the rate of ongoing pregnancy in patients with rASRM stage I/II endometriosis. C Show 3 more Patients with infertility (assisted reproductive technology): consider offering intrauterine insemination with ovarian stimulation instead of expectant management or intrauterine insemination alone, to increase pregnancy rates in infertile patients with rASRM stage I/II endometriosis. C Show 12 more Patients with extrapelvic endometriosis: recognize the symptoms of extrapelvic endometriosis, such as cyclical shoulder pain, cyclical spontaneous pneumothorax, cyclical cough or nodules which enlarge during menses. B Show 3 more 10. Patient education General counseling: inform patients with endometriosis requesting information on their risk of developing cancer that endometriosis is not associated with a significantly higher risk of cancer overall. Inform that although endometriosis is associated with a higher risk of ovarian, breast, and thyroid cancers in particular, the increase in absolute risk compared with women in the general population is low. A Show 2 more 11. Preventative measures Primary prevention: Consider advising females to follow a healthy lifestyle and diet, with reduced alcohol intake and regular physical activity. C Insufficient evidence to support the usefulness of hormonal contraceptives for the primary prevention of endometriosis. I Prevention of recurrence: perform ovarian cystectomy instead of drainage and electrocoagulation for the secondary prevention of endometriosis-associated dysmenorrhea, dyspareunia, and non-menstrual pelvic pain, when surgery is indicated in patients with an endometrioma. Take into account the risk of reduced ovarian reserve. A Show 3 more 12. Follow-up and surveillance Indications for referral: refer patients with deep endometriosis to a center of expertise. B Follow-up: consider offering follow-up and psychological support in patients with confirmed endometriosis, particularly with deep and ovarian endometriosis, although insufficient evidence https://web.pathway.md/diseases/recjQIQ99LVWEV83Z 7/9 6/29/23, 4:16 AM Endometriosis Pathway regarding the benefit of regular long-term monitoring for early detection of recurrence, complications, or malignancy. C Show 4 more Management of recurrence: consider offering any hormone treatment or surgery for the treatment of recurring pain symptoms in patients with endometriosis. C 13. Quality improvement Management outcomes: as per SOGC 2010 guidelines, recognize that: treatment of endometriosis by excision or ablation reduces pain A excision rather than drainage or fulguration provides better pain relief, a reduced recurrence rate, and a histopathological diagnosis in patients with endometriomas A laparoscopic uterine nerve ablation alone does not offer significant relief of endometriosis- related pain A laparoscopic treatment of minimal or mild endometriosis improves pregnancy rates regardless of the treatment modality A the effect on fertility of surgical treatment of deeply infiltrating endometriosis is controversial B laparoscopic excision of ovarian endometriomas > 3 cm in diameter may improve fertility B if a patient with known endometriosis is to undergo IVF, GnRH agonist suppression with add- back hormone therapy for 3 to 6 months before IVF is associated with an improved pregnancy rate. A Clinical findings Symptoms Past obstetric history Chronic lower abdominal pain Female infertility Dyschezia Recurrent abortion Dysuria Gynecologic exam Fatigue LLQ pain Adnexal mass RLQ pain Uterine bleeding Suprapubic pain Vaginal wall nodules Rectal exam Rectal nodules References 1. ESHRE Endometriosis Guideline Development Group. ESHRE guideline: management of women with endometriosis. ESHRE. 2022 Feb 2. Open https://web.pathway.md/diseases/recjQIQ99LVWEV83Z 8/9 6/29/23, 4:16 AM Endometriosis Pathway 2. Nicholas Leyland, Robert Casper, Philippe Laberge et al. Endometriosis: Diagnosis and Management. J Obstet Gynaecol Can. 2010 Jul;32 7 Suppl 2 S1 32. Open 3. No authors listed. Practice bulletin no. 114 management of endometriosis. Obstet Gynecol. 2010 Jul;116 1 223 236. Open 4. No authors listed. ACOG Committee Opinion No. 760 Dysmenorrhea and Endometriosis in the Adolescent. Obstet Gynecol. 2018 Dec;132 6):e249-e258. Open 5. American College of Obstetricians and Gynecologists Committee on Practice Bulletins Gynecology. Practice Bulletin No. 174 Evaluation and Management of Adnexal Masses. Obstet Gynecol. 2016 Nov;128 5):e210-e226. Open 6. Tarek Motan, Roland Antaki, Jinglan Han et al. Guideline No. 435 Minimally Invasive Surgery in Fertility Therapy. J Obstet Gynaecol Can. 2023 Apr;45 4 273 282.e2. Open 7. Hwang H, Chung YJ, Lee SR et al. Clinical evaluation and management of endometriosis: guideline for Korean patients from Korean Society of Endometriosis. Obstet Gynecol Sci. 2018 Sep;61 5 553 564. Open 8. G A J Dunselman, N Vermeulen, C Becker et al. ESHRE guideline: management of women with endometriosis. Hum Reprod. 2014 Mar;29 3 400 12. Open 9. Royal College of Obstetricians and Gynaecologists. The Initial Management of Chronic Pelvic Pain. RCOG. 2012 May. Open https://web.pathway.md/diseases/recjQIQ99LVWEV83Z 9/9 |
Guideline sources The following summarized guidelines for the evaluation and management of endoscopic retrograde cholangiopancreatography are prepared by our editorial team based on guidelines from the American Society for Gastrointestinal Endoscopy (ASGE 2023; 2021; 2019; 2017; 2016; 2015), the American Association for the Study of Liver Diseases (AASLD 2022), the European Society of Gastrointestinal Endoscopy (ESGE 2020; 2019; 2016), the World Society of Emergency Surgery (WSES 2020; 2019), the British Society of Gastroenterology (BSG 2019; 2017), the World Society of Emergency Surgery (WSES/AAST 2019), the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition (NASPGHAN 2018), the American Gastroenterological Association (AGA 2018), the INternational Study Group of Pediatric Pancreatitis: In search for a cuRE (INSPPIRE 2017), the Canadian Best Practice in General Surgery Group (BPIGS 2016), the European Association for the Study of the Liver (EASL 2016), and the American College of Gastroenterology (ACG 2015; 2013). 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 https://web.pathway.md/diseases/reckikcucSpfA7am4 1/13 6/29/23, 4:17 AM Endoscopic retrograde cholangiopancreatography Pathway Calculator Calculator Calculat ASGE/SAGES criteria for stratific Diagnostic criteria for acute pan Glasgo Guidelines 1. Classification and risk stratification Risk factors for complications: Consider regarding patients as being at high risk for post- ERCP pancreatitis in the presence of at least one definite or two likely patient-related or procedure-related risk factors: Situation Guidance Female gender, previous pancreatitis, previous post-ERCP pancreatitis, suspected sphincter of Oddi dysfunction Patient-related definite risk factors Difficult cannulation Procedure-related definite risk factors Pancreatic guidewire passages > 1 Pancreatic injection Younger age Patient-related likely risk factors Nondilated extrahepatic bile duct Absence of chronic pancreatitis Normal serum bilirubin End-stage renal disease Precut sphincterotomy Procedure-related likely risk factors Pancreatic sphincterotomy Biliary balloon sphincter dilation Failure to clear bile duct stones Intraductal ultrasound. C Show 3 more 2. Diagnostic investigations Coagulation studies: avoid obtaining coagulation tests routinely before ERCP in patients not on anticoagulants and without jaundice. D 3. Diagnostic procedures https://web.pathway.md/diseases/reckikcucSpfA7am4 2/13 6/29/23, 4:17 AM Endoscopic retrograde cholangiopancreatography Pathway Diagnostic indications, pancreatobiliary pain: do not perform diagnostic ERCP for the evaluation of pancreaticobiliary-type pain in the absence of objective abnormalities on other pancreaticobiliary imaging or laboratory studies. D Diagnostic indications, acute pancreatitis: As per AGA 2018 guidelines, avoid performing urgent ERCP routinely in patients with acute biliary pancreatitis and no cholangitis. D As per ACG 2013 guidelines, limit the use of endoscopic investigations in patients with acute idiopathic pancreatitis because of unclear risks and benefits. B Diagnostic indications, choledocholithiasis: perform preoperative ERCP, intraoperative cholangiography, or laparoscopic ultrasound, depending on the local expertise and the availability of the technique, in patients at high risk for common bile duct stones. A Diagnostic indications, primary sclerosing cholangitis: As per AASLD 2022 guidelines, avoid performing ERCP for the diagnosis of PSC. D Show 2 more As per BSG 2019 guidelines: Consider performing ERCP in patients with suspected primary sclerosis cholangitis and biliary strictures requiring tissue acquisition (such as cytological brushings) or when therapeutic intervention is indicated. Perform pathological sampling of suspicious strictures in patients undergoing ERCP for dominant strictures. B Avoid performing ERCP in patients with PSC until an expert multidisciplinary assessment is available justifying endoscopic intervention. D As per ACG 2015 guidelines, perform ERCP (with cytology, biopsies, and fluorescence in- situ hybridization) to exclude cholangiocarcinoma in patients with a dominant stricture on imaging. B Diagnostic indications, biliary strictures: consider performing peroral cholangioscopy with directed biopsy as an adjunctive technique for characterizing indeterminate biliary strictures. C Diagnostic indications, pancreatobiliary trauma: consider performing ERCP for both diagnosis and treatment in hemodynamically stable or stabilized adult and pediatric patients with suspected pancreatic duct and extrahepatic biliary tree injuries, even in the early phase after trauma. B Diagnostic indications, pancreatic tumors (cystic lesions): consider performing ERCP for the diagnosis and characterization of suspected main duct intraductal papillary mucinous neoplasms. C Diagnostic indications, pancreatic tumors (solid lesions): do not perform preoperative ERCP in patients with obstructive jaundice due to resectable adenocarcinoma of the pancreas in the absence of cholangitis, unless a substantial delay in operative resection of a symptomatic patient is anticipated. D Diagnostic indications, before laparoscopic cholecystectomy: do not perform routine ERCP before laparoscopic cholecystectomy in the absence of objective signs of biliary obstruction or stone. D https://web.pathway.md/diseases/reckikcucSpfA7am4 3/13 6/29/23, 4:17 AM Endoscopic retrograde cholangiopancreatography Pathway 4. Medical management Management of bleeding: Consider administering local injection of epinephrine (1:10, 000), possibly combined with thermal or mechanical therapy when injection alone fails, for the management of persistent or delayed post-sphincterotomy bleeding. C Consider performing temporary placement of a biliary fully covered self-expandable metal stent for post-sphincterotomy bleeding refractory to standard hemostatic modalities. C Management of perforation: consider providing nonoperative management in patients with suspected periampullary or instrument-related perforations from ERCP without evidence of peritonitis or systemic inflammatory response syndrome. C Management of cholangitis: consider obtaining abdominal ultrasound or CT in patients with post-ERCP cholangitis with no improvement with conservative therapy, to consider repeat ERCP cholangitis. Collect a bile sample for microbiological examination during repeat ERCP. C Management of pancreatitis: Consider testing serum amylase and/or lipase 2-6 hours after ERCP in patients with postprocedural abdominal pain planned to be discharged on the day of ERCP. Consider discharging patients with serum amylase and lipase values < 1.5 and 4 times the ULN, respectively, without concerns about the development of post-ERCP pancreatitis. C Avoid performing salvage pancreatic stenting in patients with post-ERCP pancreatitis. D 5. Therapeutic procedures Therapeutic indications, acute cholangitis: As per ASGE 2021 guidelines: Consider performing ERCP over percutaneous transhepatic biliary drainage in patients with cholangitis. C Consider performing ERCP within 48 hours after admission. C As per ESGE 2019 guidelines, perform endoscopic biliary drainage in patients with acute cholangitis, with timing depending on the severity of cholangitis: Situation Guidance As soon as possible and within 12 hours in patients with septic shock Severe Within 48-72 hours Moderate Elective. B Mild As per EASL 2016 guidelines, perform ERCP with sphincterotomy and stone extraction in patients with biliary pancreatitis with suspected coexistent acute cholangitis, with timing depending on the severity of cholangitis but preferably within 24 hours. A Show 2 more https://web.pathway.md/diseases/reckikcucSpfA7am4 4/13 6/29/23, 4:17 AM Endoscopic retrograde cholangiopancreatography Pathway Therapeutic indications, choledocholithiasis, general indications: As per ASGE 2019 guidelines, perform prompt ERCP in patients with the following high-risk criteria for choledocholithiasis: common bile duct stone on ultrasound or cross-sectional imaging TBIL > 4 mg/dL and dilated common bile duct ascending cholangitis. E Show 2 more As per BSG 2017 guidelines, perform ERCP in patients with common bile duct stones requiring endoscopic therapy. B Therapeutic indications, choledocholithiasis, technical considerations: As per ESGE 2019 guidelines, provide adequate exit for the stones that are to be removed, according to the papilla and common bile duct anatomy and the stone size. B Show 6 more As per BSG 2017 guidelines, recognize that tolerability and likelihood of therapeutic success are higher in selected patients when ERCP is performed with propofol sedation or general anesthesia. Ensure that hospitals managing patients with common bile duct stones have ready and prompt access to anesthesia-supported ERCP, either as an on-site service or provided by another ERCP unit as part of a clinical network. B Therapeutic indications, choledocholithiasis, cholangioscopic interventions: As per ESGE 2019 guidelines, perform cholangioscopy-assisted intraluminal lithotripsy (electrohydraulic or laser) for the treatment of difficult bile duct stones. B As per ASGE 2015 guidelines, consider performing cholangioscopy as an adjunctive technique for the management of difficult bile duct stones not amenable to removal after sphincterotomy with or without balloon dilation or mechanical lithotripsy. C Therapeutic indications, choledocholithiasis (post-ERCP cholecystectomy): perform laparoscopic cholecystectomy within 2 weeks from ERCP in patients treated for choledocholithiasis, to reduce the conversion rate and the risk of recurrent biliary events. B Therapeutic indications, acute pancreatitis: As per WSES 2019 guidelines, do not perform routine ERCP in patients with acute gallstone pancreatitis. D Show 2 more As per BPIGS 2016 guidelines: Perform ERCP within 24-48 hours in patients with acute gallstone pancreatitis associated with bile duct obstruction or cholangitis. B Perform ERCP with sphincterotomy before discharge in patients with acute gallstone pancreatitis, if cholecystectomy cannot be performed during the index admission because of medical comorbidities. B As per ASGE 2015 guidelines, perform ERCP in patients with acute biliary pancreatitis with concomitant cholangitis or biliary obstruction. A As per ACG 2013 guidelines: Perform ERCP within 24 hours of admission in patients with acute pancreatitis and concurrent acute cholangitis. B https://web.pathway.md/diseases/reckikcucSpfA7am4 5/13 6/29/23, 4:17 AM Endoscopic retrograde cholangiopancreatography Pathway Avoid performing ERCP in patients with gallstone pancreatitis in the absence of laboratory or clinical evidence of ongoing biliary obstruction. D Therapeutic indications, sphincter of Oddi dysfunction: perform ERCP with sphincterotomy in patients with type I sphincter of Oddi dysfunction. B Show 2 more Therapeutic indications, primary sclerosing cholangitis: perform ERCP with dilation and stent placement in patients with benign biliary strictures. B Therapeutic indications, postoperative biliary leaks: perform ERCP as first-line therapy for postoperative biliary leaks. A 6. Specific circumstances Pediatric patients: As per NASPGHAN 2018 guidelines, perform ERCP for the management of acute pancreatitis related to choledocholithiasis causing biliary pancreatitis, and for pancreatic duct pathologies, such as ductal stones or ductal leaks. E As per INSPPIRE 2017 guidelines, consider performing ERCP or EUS, depending on the clinical scenario, as an alternative to MRCP in patients with acute recurrent pancreatitis. B Pregnant patients (general principles): recognize that therapeutic ERCP is a safe and effective procedure in pregnant patients, provided that it is performed by experienced endoscopists and the radiation exposure to the fetus is kept as low as possible. B Pregnant patients (prevention of post-ERCP pancreatitis): do not administer NSAIDs for post-ERCP pancreatitis prophylaxis in pregnant patients at 30-week gestation. D Patients with altered anatomy: perform ERCP in patients with Billroth II gastrectomy in referral centers, with the side-viewing endoscope as a first option, and forward-viewing endoscopes (gastroscope, pediatric colonoscope, and balloon enteroscope) as the second choice in cases of failure. B Show 4 more 7. Patient education Informed consent: obtain both oral and written informed consent before ERCP, taking into account individual and procedure-related risks, correct indication, and urgency of ERCP, as well as national practice. B 8. Preventative measures Prevention of pancreatitis, proceduralist expertise: As per ASGE 2017 guidelines, ensure that clinicians performing performing ERCP are facile with procedural techniques reducing the risk of pancreatitis (wire-guided cannulation, prophylactic pancreatic duct stenting). A https://web.pathway.md/diseases/reckikcucSpfA7am4 6/13 6/29/23, 4:17 AM Endoscopic retrograde cholangiopancreatography Pathway As per BSG 2017 guidelines, provide training and subsequent mentorship to ensure competency in access papillotomy in all endoscopists performing ERCP. B Prevention of pancreatitis, rectal NSAIDs: As per ASGE 2023 guidelines, administer periprocedural rectal NSAIDs for the prevention of post-ERCP pancreatitis in unselected and high-risk patients undergoing ERCP. B As per ESGE 2020 guidelines, administer rectal diclofenac or indomethacin at a dose of 100 mg immediately before ERCP in all patients without contraindications to NSAID administration. B Show 2 more As per ASGE 2017 guidelines, administer rectal NSAIDs to reduce the incidence and severity of post-ERCP pancreatitis in high-risk patients without contraindication. B Show 2 more As per BSG 2017 guidelines, administer 100 mg of diclofenac or indomethacin rectally at the time of ERCP to reduce the risk of post-ERCP pancreatitis in all patients without contraindications to NSAIDs. B As per ACG 2013 guidelines, administer postprocedural rectal NSAID suppositories to prevent severe pancreatitis after ERCP in high-risk patients. B Prevention of pancreatitis, IV fluids: As per ASGE 2023 guidelines, consider administering aggressive periprocedural and postprocedural IV hydration to prevent post-ERCP pancreatitis in unselected patients undergoing ERCP. C As per ESGE 2020 guidelines, administer aggressive hydration with lactated Ringer's solution (3 mL/kg/hour during ERCP, 20 mL/kg bolus after ERCP, 3 mL/kg/hour for 8 hours after ERCP) in patients with contraindication to NSAIDs, provided that they are not at risk of fluid overload and that a prophylactic pancreatic duct stent is not placed. B As per ASGE 2017 guidelines, consider administering periprocedural IV hydration with lactated Ringer's solution when feasible to decrease the risk of post-ERCP pancreatitis. C Prevention of pancreatitis (sublingual nitrates): consider administering 5 mg sublingual glyceryl trinitrate before ERCP for the prevention of post-ERCP pancreatitis in patients with contraindications to NSAIDs or to aggressive hydration. C Prevention of pancreatitis (epinephrine): do not administer epinephrine topically onto the papilla for post-ERCP pancreatitis prophylaxis. D Prevention of pancreatitis (protease inhibitors): do not use protease inhibitors for post- ERCP pancreatitis prophylaxis. D Prevention of pancreatitis, pancreatic stenting: As per ASGE 2023 guidelines: Perform pancreatic duct stenting to reduce the risk of post-ERCP pancreatitis in patients undergoing ERCP with repeated or deep pancreatic duct access or performance of ampullectomy. B Consider performing pancreatic duct stenting in high-risk groups, including patients with difficult cannulation, history of post-ERCP pancreatitis, or precut sphincterotomy without fistulotomy technique, as long as pancreatic duct access can be easily achieved. C https://web.pathway.md/diseases/reckikcucSpfA7am4 7/13 6/29/23, 4:17 AM Endoscopic retrograde cholangiopancreatography Pathway As per ESGE 2020 guidelines: Perform prophylactic pancreatic stenting in selected patients at high risk for post-ERCP pancreatitis (inadvertent guide-wire insertion/opacification of the pancreatic duct, double- guidewire cannulation). B Consider using a short 5-Fr pancreatic stent with no internal flange but having a flange or a pigtail on the duodenal side. Assess for the passage of the stent from the pancreatic duct within 5-10 days of placement and remove retained stents endoscopically. C As per ASGE 2017 guidelines, perform pancreatic duct stenting to reduce the incidence and severity of post-ERCP pancreatitis in high-risk patients. A As per BSG 2017 guidelines, consider performing pancreatic stenting, in addition to rectal administration of an NSAID, in patients at high risk of post-ERCP pancreatitis arising from repeated pancreatic duct cannulation. C As per ACG 2013 guidelines, perform pancreatic duct stenting to prevent severe pancreatitis after ERCP in high-risk patients. B Prevention of pancreatitis (primary biliary cannulation): consider performing wire-guided cannulation over contrast-guided cannulation to minimize the risk of post-ERCP pancreatitis in unselected patients undergoing ERCP. C Prevention of pancreatitis (difficult biliary cannulation, definition): Consider defining difficult biliary cannulation by the presence of 1 of the following: > 5 contacts with the papilla whilst attempting to cannulate > 5 minutes spent attempting to cannulate following visualization of the papilla > 1 unintended pancreatic duct cannulation or opacification. C Prevention of pancreatitis (difficult biliary cannulation, contrast- or guidewire-assisted cannulation): Perform guidewire-assisted cannulation for primary biliary cannulation to reduce the risk of post-ERCP pancreatitis. B Consider using a hydrophilic (tipped) guidewire for biliary cannulation to help achieve successful cannulation. C Prevention of pancreatitis (difficult biliary cannulation, pancreatic guidewire-assisted cannulation): perform pancreatic guidewire-assisted biliary cannulation in case of difficult biliary cannulation or repeated unintentional access to the main pancreatic duct. B Show 2 more Prevention of pancreatitis, difficult biliary cannulation, precut sphincterotomy: As per ASGE 2017 guidelines, perform early precut sphincterotomy for difficult biliary cannulation when expertise is available. B As per ESGE 2016 guidelines, perform needle-knife fistulotomy as the preferred technique for precutting. B Show 2 more Prevention of pancreatitis (difficult biliary cannulation, transpancreatic biliary sphincterotomy): Consider performing transpancreatic biliary sphincterotomy in patients with a small, difficult- to-cannulate papilla if unintentional insertion of a guidewire into the pancreatic duct occurs. https://web.pathway.md/diseases/reckikcucSpfA7am4 8/13 6/29/23, 4:17 AM Endoscopic retrograde cholangiopancreatography Pathway B Consider performing prophylactic pancreatic stenting in patients with a history of transpancreatic sphincterotomy. B Prevention of pancreatitis (difficult biliary cannulation, periampullary diverticulum): Consider performing pancreatic duct stenting followed by precut sphincterotomy or needle- knife fistulotomy, depending on the patient's anatomy and operator's experience, to achieve cannulation in patients with periampullary diverticulum and difficult cannulation. C Consider performing a small endoscopic sphincterotomy combined with endoscopic papillary balloon dilation or endoscopic papillary balloon dilation alone to facilitate large stone removal in cases where endoscopic sphincterotomy is technically difficult to complete as a result of a periampullary diverticulum. C Prevention of pancreatitis (difficult biliary cannulation, cannulation and sphincterotomy of the minor papilla): consider administering secretin injection, which can be preceded by methylene blue spray in the duodenum, when cannulation of the minor papilla is difficult. C Show 2 more Prevention of pancreatitis, endoscopic papillary balloon dilation: As per ASGE 2017 guidelines, do not perform endoscopic papillary large balloon dilation of an intact sphincter rather than endoscopic sphincterotomy with or without adjunct balloon sphincteroplasty to facilitate biliary stone extraction in patients without coagulopathy because of the increased risk of pancreatitis. Perform dilation > 1 minute if endoscopic papillary large balloon dilation alone is used. D As per ESGE 2016 guidelines, consider performing endoscopic papillary balloon dilation as an alternative to endoscopic sphincterotomy for extracting common bile duct stones < 8 mm in patients without anatomical or clinical contraindications (such as acute pancreatitis, acute cholangitis, or precut sphincterotomy), especially in the presence of coagulopathy or altered anatomy. B Show 2 more Prevention of pancreatitis, rendezvous ERCP: As per ESGE 2020 guidelines, consider performing intraoperative rendezvous ERCP for common bile duct stone extraction in patients scheduled for cholecystectomy. B As per ESGE 2019 guidelines, consider performing intraoperative rendezvous ERCP in patients with common bile duct stones undergoing cholecystectomy. C As per ESGE 2016 guidelines: Consider performing intraoperative ERCP with laparoendoscopic rendezvous, where local expertise and conditions allow, in patients with choledocholithiasis scheduled for elective cholecystectomy. C Consider performing anterograde guidewire insertion either by a percutaneous or EUS- guided approach, depending on local expertise and facilities, to achieve biliary access when biliary cannulation is unsuccessful with a standard retrograde approach. C Prevention of bleeding, sphincterotomy: As per ESGE 2020 guidelines, avoid performing routine endoscopic biliary sphincterotomy before the insertion of a single plastic or uncovered/partially covered self-expandable metallic stent for relief of biliary obstruction. D https://web.pathway.md/diseases/reckikcucSpfA7am4 9/13 6/29/23, 4:17 AM Endoscopic retrograde cholangiopancreatography Pathway As per ASGE 2017 guidelines, perform sphincterotomy selectively in patients at high-risk for bleeding. B As per ESGE 2016 guidelines, do not perform routine biliary sphincterotomy in patients undergoing pancreatic sphincterotomy, reserving it for patients with evidence of coexisting bile duct obstruction or biliary sphincter of Oddi dysfunction. D Prevention of bleeding, electrosurgical current mode: As per ASGE 2017 guidelines, use a microprocessor-controlled generator with mixed current for sphincterotomy, to reduce the risk of post-sphincterotomy bleeding. B As per ESGE 2016 guidelines: Use mixed current rather than pure cut current alone for sphincterotomy, to decrease the risk of bleeding. B Consider using a current mode providing alternating cutting and coagulation phases instead of conventional blended current, to reduce episodes of uncontrolled cutting ("zippers") and the risk of bleeding at the time of sphincterotomy. C Prevention of infection, antibiotic prophylaxis: As per AASLD 2022 guidelines, administer antimicrobial prophylaxis in the periprocedural period in patients with PSC undergoing ERCP. E As per ESGE 2020 guidelines: Do not administer routine antibiotic prophylaxis before ERCP. D Consider administering antibiotic prophylaxis (with an agent active against Gram-negative bacteria and adapted as much as possible to local epidemiology) before ERCP in case of anticipated incomplete biliary drainage, in severely immunocompromised patients, and when performing cholangioscopy. C As per BSG 2019 guidelines, administer antibiotics prophylaxis in patients with suspected PSC undergoing ERCP. B As per ESGE 2019 guidelines, avoid administering routine antibiotic prophylaxis before ERCP for bile duct stones. D As per ASGE 2017 guidelines, administer antibiotic prophylaxis (with an agent covering biliary flora, such as enteric Gram-negative organisms and enterococci) before ERCP in patients undergone liver transplantation or when there is a possibility of incomplete biliary drainage, and continued after the procedure if biliary drainage is incomplete. B As per ACG 2015 guidelines, administer antibiotic prophylaxis in patients with PSC undergoing ERCP to prevent post-ERCP cholangitis. B Prevention of infection (duodenoscope reprocessing): ensure strict compliance with current manufacturer protocols and U.S. FDA recommendations for duodenoscope reprocessing to limit duodenoscope-related transmission of infections. A Prevention of contrast allergy: avoid administering premedication for the prevention of contrast media allergy during ERCP in patients with a history of food or IV contrast allergies. D 9. Quality improvement https://web.pathway.md/diseases/reckikcucSpfA7am4 10/13 6/29/23, 4:17 AM Endoscopic retrograde cholangiopancreatography Pathway Quality indicators (preprocedural): Record and document the frequencies of the following as preprocedural quality indicators for ERCP: endoscopy is performed for an indication included in a published standard list of appropriate indications B informed consent is obtained B preprocedure history is elicited and directed physical examination is performed B risk for adverse events is assessed before sedation B prophylactic antibiotics are administered only in selected settings where indicated B a sedation plan is documented B management of antithrombotic therapy is formulated in print before the procedure B a team pause is conducted B endoscopy is performed by a specialist fully trained and credentialed to perform that particular procedure. B Quality indicators (intraprocedural): Record and document the frequencies of the following as intraprocedural quality indicators for ERCP: photodocumentation is performed monitoring in patients receiving sedation is obtained the doses and routes of administration of all medications used during the procedure are documented use of reversal agents is documented procedure interruption and premature termination because of oversedation or airway management issues is documented. B Quality indicators (postprocedural): Record and document the frequencies of the following as postprocedural quality indicators for ERCP: discharge from the endoscopy unit according to predetermined discharge criteria is documented patient instructions are provided the plan for pathology follow-up is specified a complete procedure report is created immediate adverse events requiring interventions are documented immediate adverse events requiring interventions including hospitalization occur delayed adverse events leading to hospitalization or additional procedures or medical interventions occur within 14 days patient satisfaction data are obtained communication with referring providers is documented. B References 1. Chapman MH, Thorburn D, Hirschfield GM et al. British Society of Gastroenterology and UK PSC guidelines for the diagnosis and management of primary sclerosing cholangitis. Gut. 2019 Aug;68 8 1356 1378. Open https://web.pathway.md/diseases/reckikcucSpfA7am4 11/13 6/29/23, 4:17 AM Endoscopic retrograde cholangiopancreatography Pathway 2. Jean-Marc Dumonceau, Christine Kapral, Lars Aabakken et al. ERCP-related adverse events: European Society of Gastrointestinal Endoscopy ESGE Guideline. Endoscopy. 2020 Feb;52 2 127 149. Open 3. ASGE Standards of Practice Committee, Vinay Chandrasekhara, Mouen A Khashab et al. Adverse events associated with ERCP. Gastrointest Endosc. 2017 Jan;85 1 32 47. Open 4. Christopher L Bowlus, Lionel Arriv , Annika Bergquist et al. AASLD Practice Guidance on Primary Sclerosing Cholangitis and Cholangiocarcinoma. Hepatology. 2022 Sep 9. Open 5. ASGE Standards of Practice Committee, Krishnavel V Chathadi, Vinay Chandrasekhara et al. The role of ERCP in benign diseases of the biliary tract. Gastrointest Endosc. 2015 Apr;81 4 795 803. Open 6. Lindor KD, Kowdley KV, Harrison ME. ACG Clinical Guideline: Primary Sclerosing Cholangitis. Am J Gastroenterol. 2015 May;110 5 646 59. Open 7. Pier Alberto Testoni, Alberto Mariani, Lars Aabakken et al. Papillary cannulation and sphincterotomy techniques at ERCP European Society of Gastrointestinal Endoscopy ESGE Clinical Guideline. Endoscopy. 2016 Jul;48 7 657 83. Open 8. Douglas G Adler, John G Lieb nd, Jonathan Cohen et al. Quality indicators for ERCP. Gastrointest Endosc. 2015 Jan;81 1 54 66. Open 9. Manes G, Paspatis G, Aabakken L et al. Endoscopic management of common bile duct stones: European Society of Gastrointestinal Endoscopy ESGE guideline. Endoscopy. 2019 May;51 5 472 491. Open 10. Tenner S, Baillie J, DeWitt J et al. American College of Gastroenterology guideline: management of acute pancreatitis. Am J Gastroenterol. 2013 Sep;108 9 1400 15; 1416. Open 11. James L Buxbaum, Carlos Buitrago, Alice Lee et al. ASGE guideline on the management of cholangitis. Gastrointest Endosc. 2021 Aug;94 2 207 221.e14. Open 12. Cheryl E Gariepy, Melvin B Heyman, Mark E Lowe et al. Causal Evaluation of Acute Recurrent and Chronic Pancreatitis in Children: Consensus From the INSPPIRE Group. J Pediatr Gastroenterol Nutr. 2017 Jan;64 1 95 103. Open 13. Earl Williams, Ian Beckingham, Ghassan El Sayed et al. Updated guideline on the management of common bile duct stones CBDS . Gut. 2017 May;66 5 765 782. Open 14. ASGE Standards of Practice Committee, Mohamad A Eloubeidi, G Anton Decker et al. The role of endoscopy in the evaluation and management of patients with solid pancreatic neoplasia. Gastrointest Endosc. 2016 Jan;83 1 17 28. Open 15. James L Buxbaum, Martin Freeman, Stuart K Amateau et al. American Society for Gastrointestinal Endoscopy guideline on post-ERCP pancreatitis prevention strategies: summary and recommendations. Gastrointest Endosc. 2023 Feb;97 2 153 162. Open 16. Greenberg JA, Hsu J, Bawazeer M et al. Clinical practice guideline: management of acute pancreatitis. Can J Surg. 2016 Apr;59 2 128 40. Open 17. Maisam Abu-El-Haija, Soma Kumar, Jose Antonio Quiros et al. Management of Acute Pancreatitis in the Pediatric Population: A Clinical Report From the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition Pancreas Committee. J Pediatr Gastroenterol Nutr. 2018 Jan;66 1 159 176. Open 18. Michele Pisano, Niccol Allievi, Kurinchi Gurusamy et al. 2020 World Society of Emergency Surgery updated guidelines for the diagnosis and treatment of acute calculus cholecystitis. World J Emerg Surg. 2020; 15 61. Open https://web.pathway.md/diseases/reckikcucSpfA7am4 12/13 6/29/23, 4:17 AM Endoscopic retrograde cholangiopancreatography Pathway 19. ASGE Standards of Practice Committee, Buxbaum JL, Abbas Fehmi SM et al. ASGE guideline on the role of endoscopy in the evaluation and management of choledocholithiasis. Gastrointest Endosc. 2019 Jun;89 6 1075 1105.e15. Open 20. ASGE Standards of Practice Committee, V Raman Muthusamy, Vinay Chandrasekhara et al. The role of endoscopy in the diagnosis and treatment of cystic pancreatic neoplasms. Gastrointest Endosc. 2016 Jul;84 1 1 9. Open 21. Crockett SD, Wani S, Gardner TB et al. American Gastroenterological Association Institute Guideline on Initial Management of Acute Pancreatitis. Gastroenterology. 2018 Mar;154 4 1096 1101. Open 22. Federico Coccolini, Leslie Kobayashi, Yoram Kluger et al. Duodeno-pancreatic and extrahepatic biliary tree trauma: WSES AAST guidelines. World J Emerg Surg. 2019 Dec 11;14 56. Open 23. EASL. EASL Clinical Practice Guidelines on the prevention, diagnosis and treatment of gallstones. J Hepatol. 2016 Jul;65 1 146 181. Open 24. Lepp niemi A, Tolonen M, Tarasconi A et al. 2019 WSES guidelines for the management of severe acute pancreatitis. World J Emerg Surg. 2019 Jun 13;14 27. Open 25. Jean-Marc Dumonceau, Andrea Tringali, Ioannis S Papanikolaou et al. Endoscopic biliary stenting: indications, choice of stents, and results: European Society of Gastrointestinal Endoscopy ESGE Clinical Guideline Updated October 2017. Endoscopy. 2018 Sep;50 9 910 930. Open https://web.pathway.md/diseases/reckikcucSpfA7am4 13/13 |
Guideline sources The following summarized guidelines for the evaluation and management of enterocutaneous fistula (ECF) are prepared by our editorial team based on guidelines from the American Society of Colon and Rectal Surgeons (ASCRS 2020), the British Society of Gastroenterology (BSG 2019), the American College of Gastroenterology (ACG 2018), and the Latin American Federation of Nutritional Therapy, Clinical Nutrition and Metabolism (FELANPE/ASPEN 2017). 1 2 3 4 Guidelines 1. Diagnostic investigations Nutritional evaluation: assess patients with ECF for malnutrition at the time of diagnosis. Obtain periodic nutrition assessment if malnutrition is not present at baseline as patients with fistulas have a high likelihood of becoming malnourished due to nutrient malabsorption, fluid and electrolyte losses, and sepsis. B Show 2 more 2. Medical management Immunosuppresive therapy: As per BSG 2019 guidelines, consider initiating immunomodulator and biological therapy for the management of patients with IBD and low volume ECFs. C Show 2 more https://web.pathway.md/diseases/recmi48m0maRYKs5O 1/2 6/29/23, 4:17 AM Enterocutaneous fistula Pathway As per ACG 2018 guidelines, consider initiating infliximab in patients with Crohn's disease and ECF. B Show 2 more Somatostatin analogs: initiate somatostatin analogs as an option to reduce effluent drainage and enhance spontaneous closure in adult patients with high-output (> 500 mL/day) ECF. B 3. Nonpharmacologic interventions Nutritional support: consider initiating oral diet or enteral nutrition after stabilization of fluid and electrolyte balance in adult patients with low-output (< 500 mL/day) ECF (suggesting no distal obstruction). Consider initiating parenteral nutrition to meet fluid, electrolyte, and nutrient requirements to support spontaneous or surgical closure of the ECF in patients with high- output fistula (> 500 mL/day). C Show 5 more 4. Surgical interventions Indications for surgery: As per ASCRS 2020 guidelines, consider performing surgery in patients with Crohn's disease and enteric fistulas persisting despite appropriate medical therapy. B As per BSG 2019 guidelines, offer surgery to achieve symptom control in patients with IBD and high-volume fistulas. B References 1. Vanessa J Kumpf, Jose Eduardo de Aguilar-Nascimento, Jose Ignacio Diaz-Pizarro Graf et al. ASPEN FELANPE Clinical Guidelines. JPEN J Parenter Enteral Nutr. 2017 Jan;41 1 104 112. Open 2. Lamb CA, Kennedy NA, Raine T et al. British Society of Gastroenterology consensus guidelines on the management of inflammatory bowel disease in adults. Gut. 2019 Dec;68 Suppl 3):s1-s106. Open 3. Amy L Lightner, Jon D Vogel, Joseph C Carmichael et al. The American Society of Colon and Rectal Surgeons Clinical Practice Guidelines for the Surgical Management of Crohn's Disease. Dis Colon Rectum. 2020 Aug;63 8 1028 1052. Open 4. Lichtenstein GR, Loftus EV, Isaacs KL et al. ACG Clinical Guideline: Management of Crohn's Disease in Adults. Am J Gastroenterol. 2018 Apr;113 4 481 517. Open https://web.pathway.md/diseases/recmi48m0maRYKs5O 2/2 |
Guideline sources The following summarized guidelines for the evaluation and management of eosinophilia are prepared by our editorial team based on guidelines from the British Society for Haematology (BSH 2017). 1 2 2 2 3 Definition Eosinophilia is a transient or sustained increase in the number of eosinophils in the tissues and/or blood with absolute blood eosinophil counts > 450 to 550 cells/uL. 2 Epidemiology Eosinophilia is caused due to allergies (asthma, atopic dermatitis, sinusitis), drugs (penicillins, ibuprofen, allopurinol, carbamazepine), parasites (ascariasis, cysticercosis, schistosomiasis) and infections (fungi, virus), autoimmune diseases (eosinophilic granulomatosis with polyangiitis, IBD), malignancy (lymphoma, lung cancer), immunodeficiency (Job syndrome, Wiskott-Aldrich syndrome), and idiopathic reasons (hypereosinophilic syndromes). 2 Disease course Clinical manifestations of eosinophilia are heterogeneous that depend on the underlying cause and the affected organs (lungs, heart, skin, blood vessels, kidneys, brain, sinuses) that can range from being asymptomatic to showing symptoms of rash, pruritus, erythema, angioedema, urticaria, hives, fever, fatigue, malaise, tissue swelling, myalgias, weight gain, splenomegaly, Stevens-Johnson syndrome, toxic epidermal necrolysis, thrombosis, and progressive HF. 2 Prognosis and risk of recurrence https://web.pathway.md/diseases/reclOsdLQ2ZMQsbQt 1/3 6/29/23, 4:17 AM Eosinophilia Pathway Prognosis and risk of recurrence Eosinophilia is associated with all-cause mortality with odds ratio of 1.16 (95% CI 1.09-1.24, p < 0.0001) and 1.60 (95% CI 1.35-1.91, p < 0.0001) for mild and severe eosinophilia, respectively. 3 Guidelines 1. Diagnostic investigations Clinical history: evaluate for the underlying cause of eosinophilia and for possible eosinophil- associated end-organ damage. B Blood tests: obtain a full blood count, peripheral blood smear, routine tests of renal and liver function, a bone profile, LDH, ESR and/or CRP, as well as a vitamin B12 assay in all patients. B Evaluation for end-organ damage: assess for end-organ damage using CXR and/or CT of the thorax, echocardiography, serum troponin T, and pulmonary function tests. B Serum tryptase estimation: obtain serum tryptase levels if the differential diagnosis includes chronic eosinophilic leukemia or systemic mastocytosis. B Fluorescence in situ hybridisation (FISH): investigate patients with an eosinophil count of at least 1.5 10 cells/L with no obvious cause for a possible hematological neoplasm associated with clonal eosinophilia, initially by peripheral blood analysis for FIP1L1-PDGFRA by FISH or nested RT-PCR. B 2. Diagnostic procedures Bone marrow biopsy: perform a bone marrow aspirate and biopsy in patients without an identifiable cause for eosinophilia and with negative peripheral blood analysis for FIP1L1- PDGFRA by FISH or nested RT-PCR. Assess for an underlying lymphoma or of the lymphocytic variant of hypereosinophilic syndrome, including consideration of immunophenotyping of peripheral blood and bone marrow lymphocytes and analysis for T-cell receptor gene rearrangement. B 3. Medical management Patients with severe eosinophilia: Initiate high-dose corticosteroids in patients requiring emergency treatment for severe or life- threatening eosinophilia. B Administer concomitant ivermectin in patients at risk for Strongyloides infection, to prevent potentially fatal hyperinfection. B Patients with clonal eosinophilia: administer low-dose imatinib to patients with clonal eosinophilia and FIP1L1-PDGFRA, including patients presenting with acute leukemia. B https://web.pathway.md/diseases/reclOsdLQ2ZMQsbQt 2/3 6/29/23, 4:17 AM Eosinophilia Pathway Patients with idiopathic hypereosinophilic syndrome: administer high-dose corticosteroids in patients with severe or life-threatening eosinophilia in the context of idiopathic hypereosinophilic syndrome. B 4. Surgical interventions Hemopoietic stem cell transplantation: consider hemopoietic stem cell transplantation for patients with clonal eosinophilia with FGFR 1 rearrangement; patients with chronic eosinophilic leukemia, not otherwise specified; and patients hypereosinophilic syndromes patients refractory to or intolerant of both conventional TKI therapy and experimental medical therapy, where available, or who display progressive end-organ damage. C Clinical findings Symptoms Past medical history Diarrhea Allergic reactions Itching Asthma Runny nose Atopy Cancer Integument exam Eosinophilic granulomatosis with polyangiitis Rash Medications Parasite infections Histological findings Hematological findings Charcot-leyden crystals blood eosinophils References 1. Butt NM, Lambert J, Ali S et al. Guideline for the investigation and management of eosinophilia. Br J Haematol. 2017 Feb;176 4 553 572. Open 2. Anna Kovalszki, Peter F. Weller. Eosinophilia. Prim Care. 2016 Dec; 43 4 607 617. Open 3. Christen Lykkegaard Andersen, Volkert Dirk Siersma, Hans Carl Hasselbalch et al. Eosinophilia in routine blood samples and the subsequent risk of hematological malignancies and death. Am J Hematol. 2013 Oct;88 10 843 7. Open https://web.pathway.md/diseases/reclOsdLQ2ZMQsbQt 3/3 |
Guideline sources The following summarized guidelines for the evaluation and management of eosinophilic esophagitis (EoE) are prepared by our editorial team based on guidelines from the British Society of Gastroenterology (BSG/BSPGHAN 2022), the American Gastroenterological Association (AGA 2022; 2020), the European Society of Gastrointestinal Endoscopy (ESGE 2021), the European Society of Eosinophilic Oesophagitis (EUREOS/EAACI/UEG/ESPGHAN 2017), the European Academy of Allergy and Clinical Immunology (EAACI 2014), the American Society for Gastrointestinal Endoscopy (ASGE 2014), and the American College of Gastroenterology (ACG 2013). 1 2 3 4 5 6 7 8 9 9 9 9 10 Definition EoE is chronic, immune/antigen-mediated esophageal disease characterized by eosinophilic infiltration of the esophageal epithelium. 9 Epidemiology In patients with EoE, environmental exposures to food antigens and aeroallergens lead to recruitment of eosinophils to the esophagus. Genetic pleomorphisms (encoding thymic stromal https://web.pathway.md/diseases/rec3GVLRFQcwb3MLy 1/10 6/29/23, 4:18 AM Eosinophilic esophagitis Pathway lymphopoietin, eotaxin-3, and calpain-14 genes) are associated with an increased risk. 9 Pathophysiology The incidence of EoE is estimated at 0.35-12.8 cases per 100,000 person-years, while its prevalence is estimated at 7.3-90.7 persons per 100,000 population. 10 Disease course Eosinophil-mediated inflammation and fibrosis lead to esophageal dysmotility and formation of esophageal strictures that causes clinical manifestations of dysphagia, and food impaction. 9 Prognosis and risk of recurrence In some patients, symptomatic remission occurs spontaneously or following esophageal dilation, without the need for further dietary modification or medical therapy; however, in most patients, the symptoms recur after discontinuation of treatment. 9 Guidelines 1. Screening and diagnosis Clinical presentation: As per BSG 2022 guidelines, recognize that eoE is characterized by symptoms of dysphagia and/or food impaction in adult patients with esophageal histology showing a peak eosinophil count of 15 eosinophils/hpf, or 15 eosinophils/0.3 mm or > 60 eosinophils/mm in the absence of other causes of esophageal eosinophilia A eoE responding clinically and histologically to a PPI is the same disease as EoE not responding to a PPI B eoE and GERD are not mutually exclusive and can coexist in the same patient A eoE is increasing in prevalence in both adults and children A eoE is more common in males than females and in people of white ethnic origin compared with other ethnic groups B having an affected first-degree relative increases the risk of EoE B the incidence rises during adolescence and peaks in early adulthood A there is seasonal variation in the symptoms of EoE in many patients, which seems to be associated with higher pollen counts B food bolus obstruction and dysphagia are strongly associated with a diagnosis of EoE in adult patients B eosinophilic oesophagitis is the most common cause of spontaneous perforation of the esophagus and this can occur at any age. A As per UEG 2017 guidelines, recognize that: eoE is a chronic, local immune-mediated esophageal disease, characterized clinically by symptoms related to esophageal dysfunction and histologically by eosinophil-predominant inflammation; do not interpret clinical manifestations or pathologic data in isolation https://web.pathway.md/diseases/rec3GVLRFQcwb3MLy 2/10 6/29/23, 4:18 AM Eosinophilic esophagitis Pathway solid food dysphagia, food impaction, and non-swallowing associated chest pain are the most commonly reported symptoms in adult patients with EoE A untreated EoE is usually associated with persistent symptoms and inflammation, leading to esophageal remodeling resulting in stricture formation and functional abnormalities B eoE significantly impacts the health-related quality of life impairing the social and psychological functioning B there is no evidence that EoE is a premalignant condition B eoE appears to have no causal or temporal relationship with hypereosinophilic syndromes, IBD, esophageal atresia, and connective tissue disorders B eoE is a distinct form of food allergy and IgE-mediated food allergies are common in patients with EoE A rhinitis, asthma, and eczema are significantly more common in patients with EoE compared to the general population, however, insufficient evidence to support that atopy predisposes to EoE B eoE and GERD are different entities and may coexist, either unrelated or interacting bidirectionally. B Diagnostic criteria: As per UEG 2017 guidelines, set a 15 eosinophils/hpf (standard size of ~ 0.3 mm ) in esophageal mucosa, taken as the peak concentration in the specimens examined, as the threshold for eosinophil density for the diagnosis of EoE. B Show 3 more As per ACG 2013 guidelines, diagnose esophageal eosinophilia in patients in whom eosinophils are identified in the squamous epithelium of the esophagus, and assess for an underlying cause. A Show 2 more Differential diagnosis: As per UEG 2017 guidelines, exclude other systemic and local causes of esophageal eosinophilia. As per ACG 2013 guidelines, diagnose PPI-responsive esophageal eosinophilia when patients have esophageal symptoms and histologic findings of esophageal eosinophilia, but demonstrate symptomatic and histologic response to proton-pump inhibition. B 2. Diagnostic investigations Allergy tests: As per BSG 2022 guidelines, do not obtain allergy testing to foods (such as skin prick test, specific IgE, or patch testing) for choosing the type of dietary restriction therapy for EoE. D As per UEG 2017 guidelines, insufficient evidence to recommend obtaining allergy tests to identify food triggers of EoE. I As per EAACI 2014 guidelines, insufficient evidence to recommend obtaining serum food allergen-specific IgE or a skin prick test to generate a successful elimination diet. I https://web.pathway.md/diseases/rec3GVLRFQcwb3MLy 3/10 6/29/23, 4:18 AM Eosinophilic esophagitis Pathway Esophageal physiologic testing: consider obtaining esophageal physiological testing in patients with EoE with ongoing dysphagia, despite histological remission and the absence of fibrostenotic disease at endoscopy. B 3. Diagnostic procedures Esophageal biopsy: As per BSG 2022 guidelines, perform esophageal biopsies in all adult patients undergoing endoscopy in the presence of endoscopic signs associated with EoE, or symptoms of dysphagia or food bolus obstruction, with a normal-looking esophagus. A Show 9 more As per ESGE 2021 guidelines, take at least 6 biopsies, 2-4 biopsies from the distal esophagus and 2-4 biopsies from the proximal esophagus, targeting areas with endoscopic mucosal abnormalities in patients with suspected EoE. Place distal and proximal biopsies in separate containers. B Show 2 more As per UEG 2017 guidelines: Take at least 6 biopsies from different locations, focusing on areas with endoscopic mucosal abnormalities. B Do not rely on endoscopic findings only to establish a diagnosis of EoE. D As per EAACI 2014 guidelines, perform upper endoscopy with 2-4 biopsies from both the proximal and distal esophagus for the diagnosis of EoE. Perform biopsy after double-dose PPI therapy of at least 6 weeks to rule out esophageal eosinophilia caused by GERD and to exclude PPI-responsive esophageal eosinophilia. B As per ACG 2013 guidelines, obtain esophageal biopsies to diagnose EoE; 2-4 biopsies should be obtained from both the proximal and distal esophagus to maximize the likelihood of detecting esophageal eosinophilia. B 4. Medical management General principles: As per BSG 2022 guidelines, attempt early diagnosis and treatment of EoE effectively to prevent long-term complications of fibrosis and strictures requiring subsequent endoscopic intervention. B As per UEG 2017 guidelines, decide on the choice of initial therapy (PPIs, diet, or topical corticosteroids) individually discussed with the patient and might be potentially interchangeable over time. B Swallowed corticosteroids: As per BSG 2022 guidelines: Administer topical corticosteroids to induce clinical and histological remission A and to reduce the development of strictures in patients with EoE. B https://web.pathway.md/diseases/rec3GVLRFQcwb3MLy 4/10 6/29/23, 4:18 AM Eosinophilic esophagitis Pathway Recognize that systemic side effects of topical corticosteroids have not been documented during the long-term treatment of patients with eosinophilic oesophagitis. A As per AGA 2020 guidelines: Administer topical corticosteroids in patients with EoE. B Consider topical rather than oral corticosteroids in patients with EoE. C As per UEG 2017 guidelines, administer topical corticosteroids for induction of histological remission in patients with EoE. A As per ACG 2013 guidelines: Administer topical corticosteroids (fluticasone or budesonide, swallowed rather than inhaled) for an initial duration of 8 weeks as first-line pharmacotherapy in patients with EoE. A Consider offering a longer course or a higher dose of topical corticosteroids in patients without symptomatic and histologic improvement after an initial course of topical corticosteroids. C Systemic corticosteroids: As per BSG 2022 guidelines, do not use systemic corticosteroids in patients with EoE. D As per UEG 2017 guidelines, do not use systemic cortiocosteroids in patients with EoE. D As per ACG 2013 guidelines: Consider initiating prednisone for the treatment of EoE if topical corticosteroids are not effective or in patients requiring rapid improvement in symptoms. C Consider initiating systemic corticosteroids in patients without symptomatic and histologic improvement after an initial course of topical corticosteroids. C Proton pump inhibitors: As per BSG 2022 guidelines: Initiate PPI therapy to induce clinical and histological remission in patients with eosinophilic oesophagitis. B Continue PPIs BID for at least 8-12 weeks before assessment of histological response. B Consider switching to alternative treatments, such as diet or topical corticosteroids, if PPI therapy causes unwanted side effects (diarrhea, gastrointestinal infections, or magnesium deficiency). B As per AGA 2020 guidelines, consider PPIs in patients with symptomatic EoE. C As per UEG 2017 guidelines, initiate PPI therapy to induce clinical and histological remission in patients with EoE. B Anti-immunoglobulin E therapy: do not offer anti-IgE therapy for the treatment of patients with EoE. D Other pharmacologic therapies: As per BSG 2022 guidelines: Do not use sodium cromoglycate, montelukast, and anti-histamines for the treatment of EoE, but consider offering for concomitant atopic disease. D Do not use immunomodulators (such as azathioprine and 6-mercaptopurine) and monoclonal antibody therapies (such as anti-TNF and anti-integrin therapies) in patients https://web.pathway.md/diseases/rec3GVLRFQcwb3MLy 5/10 6/29/23, 4:18 AM Eosinophilic esophagitis Pathway with EoE. D As per AGA 2020 guidelines, consider the following treatment options in patients with EoE only in the context of a clinical trial: anti-IL-5 therapy anti-IL-13 therapy anti-interleukin-4 receptor-alpha therapy anti-TNF therapy immunomodulators montelukast cromolyn sodium. C As per ACG 2013 guidelines, insufficient evidence to support the use of mast cell stabilizers or leukotriene inhibitors, and biological therapies in patients with EoE. I Management of anxiety/depression: offer effective therapy to alleviate anxiety and depression in patients with EoE as it affects patients due to persistent symptoms and social restrictions. B Maintenance therapy (indications): aim maintenance therapy at minimizing symptoms and preventing complications of EoE, while preserving quality of life and minimizing long-term adverse effects of treatments. B Maintenance therapy, corticosteroids: As per BSPGHAN/BSG 2022 guidelines, offer maintenance therapy with topical corticosteroids to reduce the risk of recurrent food bolus obstruction. B As per BSPGHAN/BSG 2022 guidelines, initiate maintenance treatment following clinical review as clinical and histological relapse is high after withdrawal of topical corticosteroid treatment. B As per EUREOS/EAACI/ESPGHAN/UEG 2017 guidelines, offer long-term topical corticosteroids for maintaining remission in corticosteroid-responsive patients. B As per ACG 2013 guidelines, consider offering maintenance therapy with topical corticosteroids and/or dietary restriction in all patients, particularly in patients with severe dysphagia or food impaction, high-grade esophageal stricture, and rapid symptomatic/histologic relapse following initial therapy. C Maintenance therapy, proton pump inhibitors: As per AGA 2022 guidelines, avoid offering a trial of deprescribing PPI therapy in patients with EoE. D As per BSPGHAN/BSG 2022 guidelines, continue PPIs to maintain remission in patients achieved histological response. B As per EUREOS/EAACI/ESPGHAN/UEG 2017 guidelines, offer long-term PPI therapy for maintaining remission in PPI-responsive patients. B 5. Nonpharmacologic interventions Dietary modifications: https://web.pathway.md/diseases/rec3GVLRFQcwb3MLy 6/10 6/29/23, 4:18 AM Eosinophilic esophagitis Pathway As per BSG 2022 guidelines, offer elimination diets to achieve clinical and histological remission in adult patients with EoE. B Show 5 more As per AGA 2020 guidelines, consider advising the following dietary interventions in patients with EoE: elemental diet C empirical six-food elimination diet C allergy testing-based elimination diet. C As per UEG 2017 guidelines, consider offering an elemental diet only after the failure of proper medical treatment and/or elimination diet. C As per ACG 2013 guidelines: Advise dietary elimination as initial therapy in the treatment of eosinophilic eosinophilia in both pediatric and adult patients with EoE. B Consider offering an elimination diet in patients without symptomatic and histologic improvement after an initial course of topical corticosteroids. C 6. Therapeutic procedures Esophageal dilation: As per BSG 2022 guidelines, perform endoscopic dilatation to improve symptoms in patients with fibrostenotic disease due to eosinophilic oesophagitis. B Show 2 more As per AGA 2020 guidelines, consider performing endoscopic dilation in adult patients with dysphagia from a stricture associated with EoE. C As per UEG 2017 guidelines, consider performing endoscopic dilation in patients with dysphagia/food impaction unresponsive to anti-inflammatory treatment. B As per ASGE 2014 guidelines, reserve esophageal dilation for adult patients with a dominant esophageal stricture or ring and if remaining symptomatic despite medical therapy. B As per ACG 2013 guidelines, consider performing esophageal dilation, approached conservatively, as an effective therapy in symptomatic patients with strictures that persist in spite of medical or dietary therapy. C 7. Specific circumstances Pediatric patients, diagnosis: As per BSPGHAN/BSG 2022 guidelines, recognize that symptoms of EoE in pediatric patients may be nonspecific and vary with the age of the patient. B Show 2 more As per EUREOS/EAACI/ESPGHAN/UEG 2017 guidelines: Recognize that the most common symptoms in younger pediatric and infant patients with EoE are reflux-like symptoms, vomiting, abdominal pain, food refusal, and failure to thrive. A https://web.pathway.md/diseases/rec3GVLRFQcwb3MLy 7/10 6/29/23, 4:18 AM Eosinophilic esophagitis Pathway Recognize that solid food dysphagia, food impaction, and non-swallowing associated chest pain are the most commonly reported symptoms in older pediatric patients with EoE. A Pediatric patients (management): Offer elimination diets to achieve clinical and histological remission in pediatric patients with EoE. B Consider ensuring a formal transition of care from pediatric to adult services to improve symptom control, concordance with therapy, and reduce emergency presentations in EoE. C Patients with esophageal perforation: Obtain contrast-enhanced CT to assess the degree of extravasation in patients with EoE perforation. B Offer conservative management with multidisciplinary input from gastroenterology, surgery, and radiology specialists in patients with EoE perforation if there is limited extravasation. B Patients with Candida infection: administer topical antifungals while continuing topical corticosteroids in patients with Candida infection secondary to topical corticosteroid treatment. B 8. Patient education General counseling: counsel patients about the chronic nature of the disease and the high likelihood of symptom recurrence after discontinuation of treatment. B 9. Follow-up and surveillance Indications for referral: As per BSG 2022 guidelines, manage patients with EoE refractory to treatment and/or with significant concomitant atopic disease jointly by a gastroenterologist and specialist allergist to optimize treatment. B As per EAACI 2014 guidelines, refer patients with EoE to an allergist/immunologist for diagnostic evaluation. B As per ACG 2013 guidelines, consider consultation with an allergist to identify and treat extraesophageal atopic conditions, assist with treatment of EoE, and help guide elemental and elimination diets. C Assessment of treatment response: As per BSG 2022 guidelines, perform endoscopy with biopsy while on treatment (dietary or pharmacological treatment) to assess response to treatment as symptoms may not always correlate with histological activity. B Show 2 more As per UEG 2017 guidelines, assess the efficacy of any therapy by a follow-up endoscopy after a 6- to 12-week initial course. B Perform a follow-up histological examination to monitor the disease because symptoms do not correlate accurately with histologic disease activity. B https://web.pathway.md/diseases/rec3GVLRFQcwb3MLy 8/10 6/29/23, 4:18 AM Eosinophilic esophagitis Pathway As per ACG 2013 guidelines, aim treatment at complete resolution of symptoms and pathology in patients with EoE, acknowledging that a range of reductions in symptoms and histology is a more realistic and practical goal in clinical practice. B Clinical findings Symptoms Past medical history Abdominal pain Allergic reactions Chest discomfort Allergic rhinitis Chest pain Asthma Dysphagia Atopic dermatitis Food impaction Atopy Food regurgitation Ehlers-danlos syndrome Heartburn GERD Loss of appetite Loeys-dietz syndrome Nausea Marfan syndrome Vomiting Hematological findings Weight loss blood eosinophils Anthropomorphic exam Malnutrition Histological findings Tissue eosinophilia References 1. Anjan Dhar, Hasan N Haboubi, Stephen E Attwood et al. British Society of Gastroenterology BSG and British Society of Paediatric Gastroenterology, Hepatology and Nutrition BSPGHAN joint consensus guidelines on the diagnosis and management of eosinophilic oesophagitis in children and adults. Gut. 2022 May 23;gutjnl-2022 327326. Open 2. Ikuo Hirano, Edmond S Chan, Matthew A Rank et al. AGA Institute and the Joint Task Force on Allergy-Immunology Practice Parameters Clinical Guidelines for the Management of Eosinophilic Esophagitis. Ann Allergy Asthma Immunol. 2020 May;124 5 416 423. Open 3. Roos E Pouw, Maximilien Barret, Katharina Biermann et al. Endoscopic tissue sampling Part 1 Upper gastrointestinal and hepatopancreatobiliary tracts. European Society of Gastrointestinal Endoscopy ESGE Guideline. Endoscopy. 2021 Nov;53 11 1174 1188. Open 4. Lucendo AJ, Molina-Infante J, Arias et al. Guidelines on eosinophilic esophagitis: evidence-based statements and recommendations for diagnosis and management in children and adults. United European Gastroenterol J. 2017 Apr;5 3 335 358. Open 5. Dellon ES, Gonsalves N, Hirano I et al. ACG clinical guideline: Evidenced based approach to the diagnosis and management of esophageal eosinophilia and eosinophilic esophagitis EoE . Am J Gastroenterol. 2013 May;108 5 679 92. Open https://web.pathway.md/diseases/rec3GVLRFQcwb3MLy 9/10 6/29/23, 4:18 AM Eosinophilic esophagitis Pathway 6. Muraro A, Werfel T, Hoffmann-Sommergruber K et al. EAACI food allergy and anaphylaxis guidelines: diagnosis and management of food allergy. Allergy. 2014 Aug;69 8 1008 25. Open 7. Pasha SF, Acosta RD, Chandrasekhara V et al. The role of endoscopy in the evaluation and management of dysphagia. Gastrointest Endosc. 2014 Feb;79 2 191 201. Open 8. Laura E Targownik, Deborah A Fisher, Sameer D Saini. AGA Clinical Practice Update on De- Prescribing of Proton Pump Inhibitors: Expert Review. Gastroenterology. 2022 Feb 16;S0016 5085 21 04083 X. Open 9. Furuta GT, Katzka DA. Eosinophilic Esophagitis. N Engl J Med. 2015 Oct 22;373 17 1640 8. Open 10. Dellon ES, Hirano I. Epidemiology and Natural History of Eosinophilic Esophagitis. Gastroenterology. 2018 Jan;154 2 319 332.e3. Open 11. Jean-Marc Dumonceau, Pierre H Deprez, Christian Jenssen et al. Indications, results, and clinical impact of endoscopic ultrasound EUS)-guided sampling in gastroenterology: European Society of Gastrointestinal Endoscopy ESGE Clinical Guideline Updated January 2017. Endoscopy. 2017 Jul;49 7 695 714. Open https://web.pathway.md/diseases/rec3GVLRFQcwb3MLy 10/10 |
Guideline sources The following summarized guidelines for the evaluation and management of eosinophilic granulomatosis with polyangiitis (EGPA) are prepared by our editorial team based on guidelines from the European Expert Group on Eosinophilic Granulomatosis with Polyangiitis (EGPA-EEG 2023), the European League Against Rheumatism (EULAR 2023), the Vasculitis Foundation (VF/ACR 2021), the Kidney Disease: Improving Global Outcomes Foundation (KDIGO 2021), and the Single Hub and Access Point for Pediatric Rheumatology in Europe (SHARE initiative 2019). 1 2 3 4 5 Calculator Calculator Calculat ACR/EULAR diagnostic criteria f FFS score for polyarteritis nodos Revised https://web.pathway.md/diseases/reccICvzn3ijJLw0V 1/8 6/29/23, 4:18 AM Eosinophilic granulomatosis with polyangiitis Pathway Guidelines 1. Screening and diagnosis Diagnosis: suspect EGPA in patients with asthma, chronic rhinosinusitis, and eosinophilia developing end-organ involvement, particularly peripheral neuropathy, lung infiltrates, cardiomyopathy, or other complications (such as skin, gastrointestinal, or kidney involvement). B Show 2 more Differential diagnosis: rule out other eosinophilic and vasculitic disorders and investigate the main disease complications, particularly heart, respiratory, skin, renal, and nervous system involvement, in the evaluation of patients with suspected EGPA. B 2. Diagnostic investigations Antineutrophil cytoplasmic antibodies: As per EGPA-EEG 2023 guidelines, obtain ANCA testing in all patients with suspected EGPA. B As per EULAR 2023 guidelines, obtain proteinase 3-ANCA and myeloperoxidase-ANCA testing, with a high-quality antigen-specific assay as the primary method of testing, in patients with signs and/or symptoms raising suspicion of a diagnosis of ANCA-associated vasculitis. A Serum immunoglobulins: measure serum immunoglobulin concentrations before each course of rituximab to detect secondary immunodeficiency in patients with ANCA-associated vasculitis receiving rituximab. B Echocardiography: consider obtaining echocardiography at diagnosis in patients with EGPA. C 3. Diagnostic procedures Biopsy: As per EGPA-EEG 2023 guidelines, perform a biopsy when feasible in the evaluation of patients with suspected EGPA, although it is not essential to make the diagnosis of EGPA. B As per EULAR 2023 guidelines, perform a biopsy to establish a new diagnosis of ANCA- associated vasculitis and for further evaluation of patients suspected of having relapsing vasculitis. B 4. Medical management General principles: https://web.pathway.md/diseases/reccICvzn3ijJLw0V 2/8 6/29/23, 4:18 AM Eosinophilic granulomatosis with polyangiitis Pathway As per EULAR 2023 guidelines: Offer multidisciplinary management in centers with, or with ready access to, expertise in vasculitis in patients with ANCA-associated vasculitis. B Offer the best care based on shared decision-making between the patient and the physician, taking efficacy, safety, and costs into account, in patients with ANCA-associated vasculitis. B As per KDIGO 2021 guidelines: Manage patients with ANCA-associated vasculitis at centers with experience in ANCA- associated vasculitis. B Do not delay immunosuppressive therapy while waiting for a kidney biopsy to be performed or reported in patients with a clinical presentation compatible with small-vessel vasculitis and positive or proteinase 3-ANCA and myeloperoxidase-ANCA serology, especially in patients rapidly deteriorating. D As per ACR 2021 guidelines, consider using the Five-Factor Score to guide the management in patients with EGPA. C Induction of remission: As per EGPA-EEG 2023 guidelines, define EGPA remission as the absence of clinical signs or symptoms attributable to active disease, including asthma and ear-nose-throat manifestations. Consider using the daily dose of corticosteroids for the definition of remission and choosing a maximum daily dose of 7.5 mg of prednisone as the cut-off. B Show 2 more As per EULAR 2023 guidelines: Initiate a combination of high-dose corticosteroids and cyclophosphamide for induction of remission in patients with new-onset EGPA with organ-threatening or life-threatening manifestations. Consider initiating a combination of high-dose corticosteroids and rituximab as an alternative. B Initiate corticosteroids for induction of remission in patients with new-onset EGPA without organ-threatening or life-threatening manifestations. B As per ACR 2021 guidelines, consider initiating the following medications for remission induction in patients with active, non-severe EGPA: mepolizumab and corticosteroids over methotrexate, azathioprine, or mycophenolate mofetil and corticosteroids C methotrexate, azathioprine, or mycophenolate mofetil and corticosteroids over corticosteroids alone C methotrexate, azathioprine, or mycophenolate mofetil and corticosteroids over cyclophosphamide or rituximab and corticosteroids. C Show 3 more Maintenance of remission: As per EGPA-EEG 2023 guidelines: Initiate corticosteroids alone or in combination with mepolizumab in patients with non- severe EGPA. Taper corticosteroids to the minimum effective dose to reduce toxicity. B Initiate rituximab, mepolizumab, or traditional DMARDs in combination with corticosteroids for maintenance of remission in patients with severe EGPA. B https://web.pathway.md/diseases/reccICvzn3ijJLw0V 3/8 6/29/23, 4:18 AM Eosinophilic granulomatosis with polyangiitis Pathway As per EULAR 2023 guidelines: Consider initiating methotrexate, C azathioprine, mepolizumab, or rituximab for maintenance of remission in patients with EGPA after induction of remission for organ- threatening or life-threatening disease. C Initiate mepolizumab for maintenance of remission in patients with relapsing EGPA after induction of remission for non-organ-threatening or life-threatening manifestations at the time of relapse. B As per ACR 2021 guidelines, consider initiating methotrexate, azathioprine, or mycophenolate mofetil over rituximab for remission maintenance in patients with severe EGPA entered remission with cyclophosphamide therapy. C Show 2 more Management of refractory disease: As per EGPA-EEG 2023 guidelines, define refractory EGPA as unchanged or increased disease activity after 4 weeks of appropriate remission-induction therapy. Distinguish the persistence or worsening of systemic manifestations from respiratory manifestations. B Show 2 more As per EULAR 2023 guidelines, initiate mepolizumab for induction of remission in patients with refractory EGPA without active organ-threatening or life-threatening disease. B As per KDIGO 2021 guidelines: Consider increasing the dose of corticosteroids (IV or PO), or adding rituximab if cyclophosphamide induction had been used previously, or vice versa, in patients with refractory disease. Consider performing plasma exchange. C Consider performing plasma exchange, in addition to corticosteroids with either cyclophosphamide or rituximab, in patients with diffuse alveolar bleeding with hypoxemia. C 5. Therapeutic procedures Plasma exchange: Consider performing plasma exchange in patients with serum creatinine > 5.7 mg/dL (500 mmol/l) requiring dialysis or with rapidly increasing serum creatinine, and in patients with diffuse alveolar hemorrhage having hypoxemia. C Perform plasma exchange in patients with an overlap syndrome of ANCA-associated vasculitis and anti-GBM. B Landmark trials: PEXIVAS (plasmapheresis) In patients with severe ANCA-associated vasculitis, plasma exchange was not superior to no plasma exchange with respect to the composite outcome of death from any cause or end-stage kidney disease. Michael Walsh et al. N Engl J Med. 2020 Feb 13. https://web.pathway.md/diseases/reccICvzn3ijJLw0V 4/8 6/29/23, 4:18 AM Eosinophilic granulomatosis with polyangiitis Pathway 6. Surgical interventions Kidney transplantation: delay kidney transplantation until patients are in complete clinical remission for 6 months. Do not delay transplantation because of the persistence of ANCA. B 7. Specific circumstances Pediatric patients: use the Chapel Hill definition (2012) when referring to EGPA in the pediatric population, recognizing that there are no pediatric diagnostic or classification criteria for EGPA. B Show 4 more Patients with renal involvement: insufficient evidence to support the use of rituximab and corticosteroids in patients presenting with markedly reduced or rapidly declining GFR (serum creatinine > 4 mg/dL; > 354 mmol/L). Prefer cyclophosphamide and corticosteroids for induction therapy, or a combination of rituximab and cyclophosphamide as an alternative. I Show 3 more Patients with sinonasal involvement: As per EGPA-EEG 2023 guidelines, optimize topical therapy in patients with EGPA with ear- nose-throat involvement. Involve otolaryngologists in the management of these patients. B As per ACR 2021 guidelines, consider offering nasal rinses and topical therapies (such as antibiotics, lubricants, and corticosteroids) in patients with sinonasal involvement in EGPA. C Patients with respiratory tract involvement: As per EGPA-EEG 2023 guidelines, optimize inhaled therapy in patients with EGPA having active asthma. Involve pulmonologists in the management of these patients. B As per KDIGO 2021 guidelines: Consider performing plasma exchange in patients with diffuse alveolar hemorrhage having hypoxemia. C Perform plasma exchange in patients with an overlap syndrome of ANCA-associated vasculitis and anti-GBM. B Patients on leukotriene inhibitors: Consider continuing leukotriene inhibitors in patients with newly diagnosed EGPA receiving leukotriene inhibitors. C Recognize that leukotriene inhibitors are not contraindicated in patients with EGPA with active asthma and/or sinonasal disease. B 8. Patient education General counseling: provide patients with ANCA-associated vasculitis education focusing on the impact of ANCA-associated vasculitis and its prognosis, key warning symptoms, and treatment (including treatment-related complications). B https://web.pathway.md/diseases/reccICvzn3ijJLw0V 5/8 6/29/23, 4:18 AM Eosinophilic granulomatosis with polyangiitis Pathway 9. Preventative measures Prophylaxis for Pneumocystis jirovecii pneumonia: As per EULAR 2023 guidelines, administer TMP-SMX for prophylaxis against P. jirovecii pneumonia and other infections in patients with ANCA-associated vasculitis receiving rituximab, cyclophosphamide, and/or high doses of corticosteroids. B As per ACR 2021 guidelines, consider administering prophylaxis for the prevention of P. jirovecii pneumonia in patients with EGPA receiving cyclophosphamide or rituximab. C 10. Follow-up and surveillance Serial clinical assessment: As per EGPA-EEG 2023 guidelines: Use validated clinical tools in the assessment of disease activity on follow-up. B Obtain routine monitoring of EGPA-related manifestations, with particular reference to lung function, cardiovascular events, and neurological complications. Obtain long-term monitoring of comorbidities, such as cancer, infections, and osteoporosis. B As per EULAR 2023 guidelines: Obtain periodic assessment for treatment-related adverse effects and comorbidities in patients with ANCA-associated vasculitis. Offer prophylaxis and lifestyle advice to reduce treatment-related complications and other comorbidities. B Obtain structured clinical assessment, rather than ANCA and/or CD19-positive B cell testing alone, to inform decisions on changes in treatment in patients with ANCA- associated vasculitis. B Serial laboratory assessment: do not use the persistence of ANCA positivity, an increase in ANCA levels, or a change in ANCA from negative to positive to guide treatment decisions, as those are only modestly predictive of future disease relapse. D Management of relapse: As per EGPA-EEG 2023 guidelines: Define EGPA relapse as the recurrence of clinical signs or symptoms attributable to active disease following a period of remission. View the need for an increase in the corticosteroid dose or the initiation of or an increase in an immunosuppressant as a relapse. Differentiate the relapse or new onset of systemic vasculitis (systemic relapse) from the isolated exacerbation of asthma and ear-nose-throat manifestations (respiratory relapse). B Treat relapses according to the type (systemic versus respiratory) and severity. Initiate rituximab or cyclophosphamide with corticosteroids in patients with severe systemic relapses. Increase the dose of corticosteroids and/or add mepolizumab in patients with non-severe systemic and respiratory relapses. B As per EULAR 2023 guidelines, initiate a combination of high-dose corticosteroids and cyclophosphamide for induction of remission in patients with relapsing EGPA with organ- threatening or life-threatening manifestations. Consider initiating a combination of high-dose corticosteroids and rituximab as an alternative. B Show 2 more https://web.pathway.md/diseases/reccICvzn3ijJLw0V 6/8 6/29/23, 4:18 AM Eosinophilic granulomatosis with polyangiitis Pathway As per KDIGO 2021 guidelines, reintroduce corticosteroids in combination with cyclophosphamide or rituximab (preferably rituximab) in patients with relapsing disease (life- or organ-threatening). B As per ACR 2021 guidelines, consider initiating rituximab over cyclophosphamide for remission re-induction in patients with EGPA experienced relapse with severe disease manifestations after prior successful remission induction with cyclophosphamide. C Show 4 more Clinical findings Symptoms Past medical history Abdominal pain Allergic rhinitis Arthralgia Asthma Diarrhea Head and neck exam Fever Myalgia Nasal polyps Nausea Lab findings Vomiting Weight loss CRP ESR Neurological exam serum creatinine Mononeuritis multiplex Serological findings Peripheral neuropathy serum IgE Integument exam c-ANCA Livedo reticularis p-ANCA Maculopapular rash Palpable purpura Petechiae Subcutaneous nodules Hematological findings Anemia WBC count blood eosinophils Imaging findings Lung nodule Pulmonary infiltrates Studies 2021 ADVOCATE https://web.pathway.md/diseases/reccICvzn3ijJLw0V 7/8 6/29/23, 4:18 AM Eosinophilic granulomatosis with polyangiitis Pathway In patients with ANCA-associated vasculitis, avacopan was noninferior to prednisone with respect to remission at week 26. David R W Jayne et al. N Engl J Med. 2021 Feb 18. 2020 PEXIVAS (corticosteroids) In patients with severe ANCA-associated vasculitis, a reduced-dose corticosteroid regimen was noninferior to a standard-dose corticosteroid regimen with respect to the composite outcome of death from any cause or end-stage kidney disease. Michael Walsh et al. N Engl J Med. 2020 Feb 13. Show 3 more References 1. Giacomo Emmi, Alessandra Bettiol, Elena Gelain et al. Evidence-Based Guideline for the diagnosis and management of eosinophilic granulomatosis with polyangiitis. Nat Rev Rheumatol. 2023 May 9. Open 2. Sharon A Chung, Carol A Langford, Mehrdad Maz et al. 2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Antineutrophil Cytoplasmic Antibody-Associated Vasculitis. Arthritis Rheumatol. 2021 Aug;73 8 1366 1383. Open 3. Kidney Disease: Improving Global Outcomes KDIGO Glomerular Diseases Work Group. KDIGO 2021 Clinical Practice Guideline for the Management of Glomerular Diseases. Kidney Int. 2021 Oct;100 4S S1 S276. Open 4. Nienke de Graeff, Noortje Groot, Paul Brogan et al. European consensus-based recommendations for the diagnosis and treatment of rare paediatric vasculitides - the SHARE initiative. Rheumatology Oxford). 2019 Apr 1;58 4 656 671. Open 5. Bernhard Hellmich, Beatriz Sanchez-Alamo, Jan H Schirmer et al. EULAR recommendations for the management of ANCA-associated vasculitis: 2022 update. Ann Rheum Dis. 2023 Mar 16;ard-2022 223764. Open 6. L Guillevin, F Lhote, M Gayraud et al. Prognostic factors in polyarteritis nodosa and Churg-Strauss syndrome. A prospective study in 342 patients. Medicine Baltimore). 1996 Jan;75 1 17 28. Open 7. Lo c Guillevin, Christian Pagnoux, Raphaele Seror et al. The Five-Factor Score revisited: assessment of prognoses of systemic necrotizing vasculitides based on the French Vasculitis Study Group FVSG cohort. Medicine Baltimore). 2011 Jan;90 1 19 27. Open 8. Anand Padmanabhan, Laura Connelly-Smith, Nicole Aqui et al. Guidelines on the Use of Therapeutic Apheresis in Clinical Practice Evidence-Based Approach from the Writing Committee of the American Society for Apheresis: The Eighth Special Issue. J Clin Apher. 2019 Jun;34 3 171 354. Open 9. Peter C Grayson, Cristina Ponte, Ravi Suppiah et al. 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology Classification Criteria for Eosinophilic Granulomatosis with Polyangiitis. Ann Rheum Dis. 2022 Mar;81 3 309 314. Open 10. Eleana Ntatsaki, David Carruthers, Kuntal Chakravarty et al. BSR and BHPR guideline for the management of adults with ANCA-associated vasculitis. Rheumatology Oxford). 2014 Dec;53 12 2306 9. Open https://web.pathway.md/diseases/reccICvzn3ijJLw0V 8/8 |
Guideline sources The following summarized guidelines for the evaluation and management of epistaxis are prepared by our editorial team based on guidelines from the Hereditary Haemorrhagic Telangiectasia Working Group (HHT-WG 2020) and the American Academy of Otolaryngology- Head and Neck Surgery Foundation (AAO-HNSF 2020). 1 2 Guidelines 1. Diagnostic investigations Initial assessment: Distinguish patients with nosebleed requiring prompt management from patients who do not, at the time of initial contact. B Document factors increasing the frequency or severity of bleeding in patients with nosebleeds, including personal or family history of bleeding disorders, use of anticoagulant or antiplatelet medications, or intranasal drug use. B Anterior rhinoscopy: obtain anterior rhinoscopy to identify the source of bleeding after removal of any blood clot, if present, in patients with nosebleeds. B https://web.pathway.md/diseases/rec37bjWmUrl4yPpa 1/4 6/29/23, 4:18 AM Epistaxis Pathway 2. Diagnostic procedures Nasal endoscopy: Perform, or refer to a clinician who can perform, nasal endoscopy to identify the site of bleeding and guide further management in patients with recurrent nasal bleeding, despite prior treatment with packing or cautery, or with recurrent unilateral nasal bleeding. B Consider performing, or referring to a clinician who can perform, nasal endoscopy to examine the nasal cavity and nasopharynx in patients with epistaxis that is difficult to control or when there is concern for unrecognized pathology contributing to epistaxis. C 3. Medical management General principles: Treat patients with an identified site of bleeding with an appropriate intervention, which may include 1 of the following: moisturizing or lubricating agents topical vasoconstrictors nasal cautery. B 4. Nonpharmacologic interventions Nasal compression: apply firm sustained compression to the lower third of the nose, with or without the assistance of the patient or caregiver, for 5 minutes in patients with active nasal bleeding requiring prompt management. B Nasal packing: apply nasal packing in patients with ongoing active nasal bleeding when bleeding precludes identification of a bleeding site despite nasal compression. B Show 2 more 5. Therapeutic procedures Nasal cautery: anesthetize the bleeding site and restrict application of cautery only to the active or suspected site of bleeding when nasal cautery is chosen for treatment. B 6. Surgical interventions Ligation and embolization: evaluate, or refer to a clinician who can evaluate, candidacy for surgical arterial ligation or endovascular embolization in patients with persistent or recurrent bleeding not controlled by packing or nasal cauterization. B 7. Specific circumstances Patients on anticoagulant and antiplatelet therapy: Initiate first-line treatments before transfusion, reversal of anticoagulation, or withdrawal of anticoagulation/antiplatelet medications in patients using these medications, in the absence https://web.pathway.md/diseases/rec37bjWmUrl4yPpa 2/4 6/29/23, 4:18 AM Epistaxis Pathway of life-threatening bleeding. B Use resorbable packing for nasal packaging in patients using anticoagulation or antiplatelet medications. B Hereditary hemorrhagic telangiectasia (evaluation): assess, or refer to a specialist who can assess, the presence of nasal telangiectasias and/or oral mucosal telangiectasias in patients with a history of recurrent bilateral nosebleeds or a family history of recurrent nosebleeds for the diagnosis of HHT syndrome. B Patients with hereditary hemorrhagic telangiectasia (topical therapy): advise using moisturizing topical therapies humidifying the nasal mucosa to reduce epistaxis in patients with HHT-related epistaxis. B Patients with hereditary hemorrhagic telangiectasia (medical therapy): Consider administering oral tranexamic acid for the management of epistaxis not responding to moisturizing topical therapies in patients with HHT. B Consider administering systemic antiangiogenic agents for the management of epistaxis in patients with HHT failed to respond to moisturizing topical therapies, ablative therapies, and/or tranexamic acid. B Patients with hereditary hemorrhagic telangiectasia (ablative therapy): consider performing ablative therapies for nasal telangiectasias, including laser treatment, radiofrequency ablation, electrosurgery, and sclerotherapy, in patients with HHT failed to respond to moisturizing topical therapies. C Patients with hereditary hemorrhagic telangiectasia (surgery): Consider performing septodermoplasty for the management of epistaxis in patients with HHT failed to sufficiently respond to moisturizing topical therapies, ablative therapies, and/or tranexamic acid. C Consider performing nasal closure for the management of epistaxis in patients with HHT failed to sufficiently respond to moisturizing topical therapies, ablative therapies, and/or tranexamic acid. B 8. Patient education Patient education: educate patients with nosebleeds and their caregivers about preventive measures for nosebleeds, home treatment for nosebleeds, and indications to seek additional medical care. B 9. Follow-up and surveillance Follow-up: document the outcomes of intervention within 30 days or document transition of care in patients treated with non-resorbable packing, surgery, or arterial ligation/embolization. B References https://web.pathway.md/diseases/rec37bjWmUrl4yPpa 3/4 6/29/23, 4:18 AM Epistaxis Pathway 1. Marie E Faughnan, Johannes J Mager, Steven W Hetts et al. Second International Guidelines for the Diagnosis and Management of Hereditary Hemorrhagic Telangiectasia. Ann Intern Med. 2020 Dec 15;173 12 989 1001. Open 2. David E Tunkel, Samantha Anne, Spencer C Payne et al. Clinical Practice Guideline: Nosebleed Epistaxis). Otolaryngol Head Neck Surg. 2020 Jan;162 1_suppl):S1 S38. Open https://web.pathway.md/diseases/rec37bjWmUrl4yPpa 4/4 |
Guideline sources The following summarized guidelines for the evaluation and management of erectile dysfunction (ED) are prepared by our editorial team based on guidelines from the Italian Society of Andrology and Sexual Medicine (SIAMS 2023), the European Association of Urology (EAU 2023; 2022), the European Society for Sexual Medicine (ESSM 2022), the American Urological Association (AUA/SMSNA 2022), the Italian Society of Endocrinology (SIE/SIAMS 2022), the Canadian Urological Association (CUA 2022), the American Urological Association (AUA 2021; 2018; 2015; 2014), the American Society of Anesthesiologists (ASA/ACE/OS/AACE/ASMBS/OMA 2020), the European Academy of Andrology (EAA 2020), the European Huntington's Disease Network (EHDN 2019), and the Canadian Neurological Sciences Federation (CNSF 2019). 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 17 17 17 Definition https://web.pathway.md/diseases/recsRPAiXiNov4aap 1/12 6/29/23, 4:18 AM Erectile dysfunction Pathway ED is a male sexual dysfunction characterized by an inability to achieve or maintain a penile erection sufficient for sexual satisfaction. 17 Epidemiology ED is caused due to endocrine (reduced serum testosterone levels), psychogenic (stress, depression, anxiety), nonendocrine (spinal cord injury, multiple sclerosis, TBI, diabetes, hypertension, dyslipidemia, radical pelvic surgery, medications) factors. 17 Disease course Clinical manifestations of ED may include gynecomastia, a decrease in beard and body hair due to hypogonadism, tachycardia, arterial bruit, bradycardia, arrhythmia, Peyronie disease, phimosis, frenulum breve, in addition to comorbidities (hypertension, diabetes, obesity). 17 Prognosis and risk of recurrence ED is not associated with increased mortality. 17 Guidelines 1. Screening and diagnosis Indications for screening: Assess sexual function and screen for ED in all patients with any of the following: obesity A diabetes mellitus A arterial hypertension, A including taking certain antihypertensive agents B dyslipidemia A low testosterone A taking antidepressants or antipsychotics A history of pelvic surgery for malignancies A gout B systemic diseases, especially in patients with organ failure. B Consider assessing sexual functuon and screening for ED in all patients with any of the following: COPD and obstructive sleep apnea C central and peripheral neurological diseases potentially affecting male sexual response C endocrine conditions, such as thyroid, adrenal, and pituitary diseases C chronic prostatitis/chronic pelvic pain syndrome C Peyronie's disease C young patients with a history of previous treatment with drugs affecting the serotoninergic pathway or the conversion of testosterone to dihydrotestosterone C couple infertility, particularly when undergoing assisted reproduction techniques. C https://web.pathway.md/diseases/recsRPAiXiNov4aap 2/12 6/29/23, 4:18 AM Erectile dysfunction Pathway 2. Classification and risk stratification Severity assessment: use validated questionnaires to assess the severity of ED. E 3. Diagnostic investigations History and physical examination: As per EAU 2023 guidelines, elicit a comprehensive medical and sexual history in all patients with ED. Take into consideration psychosexual development, including life stressors, cultural aspects, and cognitive/thinking style of the patient regarding their sexual performance. A Show 2 more As per SIAMS 2023 guidelines, consider obtaining educational, psychological, psycho- sexological, and marital assessments in all patients with ED. C Show 6 more As per AUA 2018 guidelines, elicit a thorough medical, sexual, and psychosocial history and perform a physical examination in patients with symptoms of ED. B Doppler ultrasound: consider obtaining penile color Doppler ultrasound, at least in flaccid condition, in all patients with ED. C Nocturnal penile tumescence and rigidity monitoring: consider obtaining nocturnal penile tumescence and rigidity test or other instrumental examinations only in selected patients. C Laboratory tests: As per EAU 2023 guidelines: Obtain routine laboratory tests, including glucose and lipid profile and total testosterone, to identify and treat any reversible risk factors and modifiable lifestyle factors. A Obtain specific diagnostic tests in the initial evaluation of ED in the presence of the following conditions: Situation Guidance Asymptomatic, < 3 risk factors for coronary artery disease (excluding sex) Low-risk category Mild, stable angina (evaluated and/or being treated) Uncomplicated previous myocardial infarction LV dysfunction/congestive HF (NYHA class I or II) Post-successful coronary revascularization Controlled hypertension Mild valvular disease > 3 risk factors for coronary artery disease (excluding sex) Intermediate-risk category https://web.pathway.md/diseases/recsRPAiXiNov4aap 3/12 6/29/23, 4:18 AM Erectile dysfunction Pathway Moderate, stable angina Recent myocardial infarction (> 2, < 6 weeks) LV dysfunction/congestive HF (NYHA class III) Non-cardiac sequelae of atherosclerotic disease (such as stroke, peripheral vascular disease) High-risk arrhythmias High-risk category Unstable or refractory angina Recent myocardial infarction (< 2 weeks) LV dysfunction/congestive HF (NYHA class IV) Hypertrophic obstructive and other cardiomyopathies Uncontrolled hypertension Moderate-to-severe valvular disease. A As per SIAMS 2023 guidelines, obtain routine laboratory tests, including fasting blood glucose, HbA1c, total and high-density lipoprotein cholesterol, and triglycerides, in all patients with ED. A Show 2 more As per AUA 2018 guidelines, obtain selective laboratory testing in patients with symptoms of ED. B Show 2 more Screening for cardiovascular diseases: As per SIAMS 2023 guidelines: Elicit symptoms of coronary artery disease in all patients with ED at each visit and assess the cardiovascular risk profile using cardiovascular algorithms, such as SCORE2 and SCORE2-Older Persons. A Consider assessing CAC score (if permitted by local expertise and availability) as a further diagnostic test in patients with calculated risks around decision thresholds (low-to- intermediate cardiovascular risk profile) to relocate them to different risk groups. C As per AUA 2018 guidelines, counsel patients that ED is a risk marker for underlying CVD and other health conditions that may warrant evaluation and treatment. B Screening for benign prostatic hyperplasia: screen patients with ED with the IPSS and patients with benign prostate obstruction with the IEF. B 4. Medical management Avoidance of drugs affecting erectile function: Consider prescribing drugs with the lowest impact on sexual function. C https://web.pathway.md/diseases/recsRPAiXiNov4aap 4/12 6/29/23, 4:18 AM Erectile dysfunction Pathway Avoid using -blockers as first-line therapy in patients with newly diagnosed arterial hypertension in the absence of specific cardiological indications. D Phosphodiesterase-5 inhibitors: As per EAU 2023 guidelines, offer PDE5 inhibitors as first-line therapy for ED. A As per SIAMS 2023 guidelines: Initiate short- or long-acting PDE5 inhibitors as first-line therapy in patients with ED. A Consider preferring short-acting PDE5 inhibitors in patients with high cardiovascular risk B and long-acting PDE5 inhibitors in patients with LUTS. A Consider combining chronic and on-demand PDE5 inhibitors in patients not responding to conventional therapy. E As per AUA 2018 guidelines, offer approved oral PDE5 inhibitors, unless contraindicated, as a treatment option in patients with ED. B Titrate the dose of PDE5 inhibitors to provide optimal efficacy. B Provide instructions to patients prescribed oral PDE5 inhibitors, to maximize benefit/efficacy. B Testosterone replacement therapy: As per AUA 2018 guidelines, inform patients with ED and testosterone deficiency that treatment with a PDE5 inhibitor may be more effective if combined with testosterone therapy. B As per AUA 2018 guidelines, avoid prescribing testosterone to men with ED who have normal testosterone levels. D 5. Nonpharmacologic interventions Lifestyle modifications: As per EAU 2023 guidelines: Offer lifestyle changes and risk factor modification before or at the same time as initiating ED treatments. A Treat a curable cause of ED first, when found. B As per SIAMS 2023 guidelines, advise adopting healthy diets to reduce the risk of ED. B Advise a Mediterranean dietary pattern to prevent the development or reduce the progression of ED in patients with diabetes, obesity, or metabolic syndrome. B Show 3 more As per AUA 2018 guidelines, offer lifestyle modifications, including changes in diet and increased physical activity to improve overall health and erectile function in patients with ED having comorbidities known to negatively affect erectile function. B Dietary supplements: insufficient evidence to support the use of nutritional supplements in patients with ED. I Erection rehabilitation: Start pro-erectile treatments at the earliest opportunity after radical prostatectomy/pelvic surgery and other curative treatments for prostate cancer. B Insufficient evidence to recommend any specific regimen for penile rehabilitation after radical prostatectomy. I https://web.pathway.md/diseases/recsRPAiXiNov4aap 5/12 6/29/23, 4:18 AM Erectile dysfunction Pathway Vacuum erection devices: As per EAU 2023 guidelines, offer vacuum erection devices as an alternative first-line therapy in well-informed patients with infrequent sexual intercourse and in patients with comorbidities requiring noninvasive, drug-free management of ED. B As per SIAMS 2023 guidelines, consider offering vacuum erection devices alone or combined with other therapies in patients with ED if PDE5 inhibitors and intracavernosal/transurethral therapies are contraindicated, ineffective, or not tolerated. C As per AUA 2018 guidelines, offer vacuum erection devices as a treatment option in patients with ED. B Psychosocial interventions: As per EAU 2023 guidelines, offer CBT (including the partner) combined with medical treatment to maximize treatment outcomes. A As per SIAMS 2023 guidelines, consider integrating psycho-sexological therapies with lifestyle changes and medical, physical, and surgical therapies. C Show 2 more As per AUA 2018 guidelines, consider referring patients being treated for ED to a mental health professional, in order to promote treatment adherence, reduce performance anxiety, and integrate treatments into a sexual relationship. C 6. Therapeutic procedures Intraurethral alprostadil: As per EAU 2023 guidelines, offer topical/intraurethral alprostadil as an alternative first-line therapy in well-informed patients not wishing or not suitable for oral vasoactive therapy, not wishing to have intracavernous injections, or in patients preferring a less invasive therapy. B As per SIAMS 2023 guidelines, consider offering topical or intraurethral alprostadil in patients with ED if PDE5 inhibitors are contraindicated, ineffective, or not tolerated, as well as in patients preferring a less-invasive treatment. C As per AUA 2018 guidelines: Offer intraurethral alprostadil as a treatment option in patients with ED. B Perform an in-office test in patients considering the use of intraurethral alprostadil. B Intracavernosal injections: As per EAU 2023 guidelines: Administer intracavernous injections as an alternative first-line therapy in well-informed patients or as second-line therapy. A Do not use platelet-rich plasma for the treatment of ED outside of a clinical trial. D As per SIAMS 2023 guidelines: Administer intracavernosal alprostadil in patients with ED if PDE5 inhibitors are contraindicated, ineffective for organic reasons, or not tolerated. A Insufficient evidence to recommend stem cells or platelet-rich plasma and penile mechanical hemodynamic revascularization procedures. I https://web.pathway.md/diseases/recsRPAiXiNov4aap 6/12 6/29/23, 4:18 AM Erectile dysfunction Pathway As per AUA 2018 guidelines, offer intracavernosal injections as a treatment option in patients with ED. B Show 2 more Extracorporeal shock wave therapy: As per EAU 2023 guidelines, offer low-intensity shock wave therapy (with or without PDE5 inhibitors) in patients with mild vasculogenic ED or poor responders to PDE5 inhibitors, or as an alternative first-line therapy in well-informed patients not wishing or not suitable for oral vasoactive therapy or desiring a curable option. B As per SIAMS 2023 guidelines, consider offering low-intensity shockwave therapy in patients with mild vasculogenic ED not responding to PDE5 inhibitors. C As per AUA 2018 guidelines, insufficient evidence to recommend low-intensity extracorporeal shock wave therapy in patients with ED, except in an investigational setting. I 7. Surgical interventions Penile vascular surgery: Consider performing penile arterial reconstruction in young patients with ED and focal pelvic/penile arterial occlusion in the absence of documented generalized vascular disease or veno-occlusive dysfunction. C Do not perform penile venous surgery for the treatment of ED. D Penile prosthesis implantation: As per EAU 2023 guidelines, offer penile prosthesis implantation when other treatments fail or depending upon patient preference. A As per SIAMS 2023 guidelines, offer penile prosthesis implantation in patients with ED if all other therapies are ineffective or contraindicated. A As per AUA 2018 guidelines, inform patients with ED regarding the treatment option of penile prosthesis implantation, including a discussion of benefits and risks/burdens. B Show 2 more 8. Specific circumstances Patients with obesity (indications for bariatric surgery): consider offering bariatric surgery to decrease ED in patients with morbid obesity. C Patients with obesity (after bariatric surgery): assess for zinc deficiency in patients with unexplained hypogonadism or ED after a bariatric procedure. B Patients with hypogonadism: As per EAU 2023 guidelines, initiate testosterone replacement therapy as first-line treatment in patients with symptomatic hypogonadism and mild ED. A Initiate a combination of PDE5 inhibitors and testosterone therapy in more severe forms of ED. B As per SIAMS 2023 guidelines, initiate testosterone in patients with hypogonadal ED, recognizing that the best results are obtained in patients with overt hypogonadism (total testosterone < 8 nmol/L). A https://web.pathway.md/diseases/recsRPAiXiNov4aap 7/12 6/29/23, 4:18 AM Erectile dysfunction Pathway As per SIAMS 2022 guidelines, initiate testosterone replacement therapy in patients with hypogonadism with low sexual desire and/or ED. A As per EAA 2020 guidelines, initiate testosterone replacement therapy to improve libido, erectile function, and sexual satisfaction in patients with hypogonadism with sexual/ED. A Patients with hyperprolactinemia: treat ED in patients with severe hyperprolactinemia to improve sexual desire, testosterone levels, and erectile function. B Patients with Peyronie's disease (surgical management): Perform surgical treatment in patients with Peyronie's disease when the inadequate quality of erections does not allow satisfactory sexual intercourse. B Perform revision surgery, if necessary, at least 6 months after the initial procedure to allow for complete healing and stabilization of the deformity and for adequate assessment of postoperative erectile function. B Patients with other endocrine disorders: consider treating patients with hypothyroidism, hyperthyroidism, C and hypocortisolism with specific therapies to also improve erectile function. C Patients with Peyronie's disease (PDE-5 inhibitors): consider offering PDE5 inhibitors to treat concomitant ED or to optimize penetration when the deformity results in difficulty in penetrative intercourse in patients with Peyronie's disease. C Patients with Peyronie's disease, penile prosthesis: As per EAU 2023 guidelines, perform penile prosthesis implantation, with or without any additional procedure (modeling, plication, incision or excision with or without grafting) in patients with Peyronie's disease with ED not responding to pharmacotherapy. A As per ESSM 2022 guidelines, consider performing penile prosthesis implantation, alone or in combination with straightening maneuvers, during revision surgery, in order to minimize further penile length loss or to avoid worsening of erectile function. C As per AUA 2015 guidelines, consider performing penile prosthesis implantation in patients with Peyronie's disease with ED and/or penile deformity sufficient to prevent coitus despite pharmacotherapy and/or vacuum device therapy. C Patients with benign prostatic hyperplasia: As per CUA 2022 guidelines, offer long-acting PDE5 inhibitors as monotherapy in patients with LUTS/benign prostatic hyperplasia, particularly in patients with both LUTS and ED. B As per EAU 2022 guidelines, offer PDE5 inhibitors in patients with moderate-to-severe LUTS, with or without ED. A As per AUA 2021 guidelines, consider offering tadalafil 5 mg daily as a treatment option in patients with LUTS secondary/attributed to benign prostatic hyperplasia, irrespective of comorbid ED. C Patients with premature ejaculation: consider addressing the erectile function before any therapeutical attempt to improve ejaculatory control in patients with loss of control of erection and ejaculation. C Patients with urethral injury: monitor patients for ED for at least one year after urethral injury. B https://web.pathway.md/diseases/recsRPAiXiNov4aap 8/12 6/29/23, 4:18 AM Erectile dysfunction Pathway Patients with Parkinson's disease: Offer the following in patients with Parkinson's disease and ED: discontinue drugs associated with ED such as alpha-blockers, or anorgasmia such as SSRIs; recognize that dopaminergic therapy can have both negative and positive effects on this symptom B consider offering sildenafil 50-100 mg 1 hour before sex in patients with Parkinson's disease with ED B consider offering other drugs, such as tadalafil 10 mg 30 min-12 hours before sex, or vardenafil 10 mg 1 hour before sex as alternative choices B consider administering apomorphine injections 5-10 min before sex as an alternative option in some patients B consider administering intracavernous injections of papaverine or alprostadil in selected patients. B Patients with Huntington's disease: attempt to identify sexual disorders and determine their triggers and their impact on relationships in patients with Huntington's disease. Consider offering psychological support and/or referral to a specialist in psychosexual disorders. Show 2 more 9. Patient education Counseling before arterial embolization for priapism: inform patients with non-ischemic priapism that embolization carries a risk of ED. B Counseling before radical prostatectomy: As per EAU 2023 guidelines, discuss with patients undergoing radical prostatectomy (any technique) about the risk of sexual changes other than ED, including libido reduction, changes in orgasm, anejaculation, Peyronie's-like disease, and penile size changes. A As per AUA 2018 guidelines, inform patients desiring preservation of erectile function after treatment of prostate cancer by radical prostatectomy or radiotherapy that early use of PDE5 inhibitors post-treatment may not improve spontaneous, unassisted erectile function. B Counseling before intracavernosal injections: instruct patients receiving intracavernosal teaching or an in-office pharmacologically-induced erection to return to the office or emergency department if they have an erection lasting > 4 hours. E 10. Preventative measures Counseling before anti-androgenic therapy: inform all patients treated with antiandrogenic agents about possible negative effects on erectile function. B 11. Follow-up and surveillance Assessment of treatment response: https://web.pathway.md/diseases/recsRPAiXiNov4aap 9/12 6/29/23, 4:18 AM Erectile dysfunction Pathway As per EAU 2023 guidelines, assess for inadequate/incorrect information about the mechanism of action and the ways the drugs are taken as they are the main causes of a lack of response to PDE5 inhibitors. B As per AUA 2018 guidelines, use validated questionnaires to assess treatment effectiveness and to guide future management. E Management of iatrogenic priapism: administer intracavernous phenylephrine as the initial treatment in patients with prolonged erection of 4 hours following intracavernous injection pharmacotherapy for ED. E Clinical findings Patient demographics Symptoms Hispanic Anorgasmia Anxiety Past medical history Decreased libido Decreased sexual response Adrenal insufficiency Depression Alzheimer's disease Difficulty attaining an erection Ankylosing spondylitis Difficulty maintaining an erection Atherosclerotic disease Sleeping disorder CVD Chronic prostatitis/chronic pelvic pain syndrome Past surgical history Coronary artery disease Pelvic surgery Cushing's syndrome Prostatectomy Diabetes mellitus Lab findings Dyslipidemia Gout serum testosterone Huntington's disease Hyperprolactinemia Hypertension Hypogonadism Hypospadias Hypothyroidism Metabolic syndrome Multiple sclerosis Nonalcoholic fatty liver disease Obesity Parkinson's disease Pelvic radiation therapy Penile cancer Penile injury Peyronie's disease Phimosis Prostate cancer Psoriasis https://web.pathway.md/diseases/recsRPAiXiNov4aap 10/12 6/29/23, 4:18 AM Erectile dysfunction Pathway Spinal cord injury Stroke Urethral injury Social history Tobacco use References 1. Daniar Osmonov, Ahmed Ragheb, Sam Ward et al. ESSM Position Statement on Surgical Treatment of Peyronie's Disease. Sex Med. 2022 Feb 1;10 1 100459. Open 2. G Corona, D Cucinotta, G Di Lorenzo et al. The Italian Society of Andrology and Sexual Medicine SIAMS , along with ten other Italian Scientific Societies, guidelines on the diagnosis and management of erectile dysfunction. J Endocrinol Invest. 2023 Jan 25;1 34. Open 3. Trinity J. Bivalacqua, MD PhD, Bryant K. Allen et al. The Diagnosis and Management of Priapism: an AUA/SMSNA Guideline 2022 . J Urol. 2022. Open 4. A M Isidori, A Aversa, A Calogero et al. Adult- and late-onset male hypogonadism: the clinical practice guidelines of the Italian Society of Andrology and Sexual Medicine SIAMS and the Italian Society of Endocrinology SIE . J Endocrinol Invest. 2022 Aug 26;1 19. Open 5. A. Salonia, C. Bettocchi, J. Carvalho et al. EAU Guidelines on Sexual and Reproductive Health. EAU. 2023. Open 6. Burnett AL, Nehra A, Breau RH et al. Erectile Dysfunction: AUA Guideline. J Urol. 2018 Sep;200 3 633 641. Open 7. Anne-Catherine Bachoud-L vi, Joaquim Ferreira, Renaud Massart et al. International Guidelines for the Treatment of Huntington's Disease. Front Neurol. 2019 Jul 3;10 710. Open 8. Morey AF, Brandes S, Dugi DD rd et al. Urotrauma: AUA guideline. J Urol. 2014 Aug;192 2 327 35. Open 9. Jeffrey I Mechanick, Caroline Apovian, Stacy Brethauer et al. Clinical Practice Guidelines for the Perioperative Nutrition, Metabolic, and Nonsurgical Support of Patients Undergoing Bariatric Procedures 2019 Update: Cosponsored by American Association of Clinical Endocrinologists/American College of Endocrinology, The Obesity Society, American Society for Metabolic and Bariatric Surgery, Obesity Medicine Association, and American Society of Anesthesiologists. Obesity Silver Spring). 2020 Apr;28 4 O1 O58. Open 10. Ajay Nehra, Ralph Alterowitz, Daniel J Culkin et al. Peyronie's Disease: AUA Guideline. J Urol. 2015 Sep;194 3 745 53. Open 11. David Grimes, Megan Fitzpatrick, Joyce Gordon et al. Canadian guideline for Parkinson disease. CMAJ. 2019 Sep 9;191 36 E989 E1004. Open 12. S. Gravas, J.N. Cornu, M. Gacci et al. EAU Guidelines on Management of Non-Neurogenic Male Lower Urinary Tract Symptoms LUTS , incl. Benign Prostatic Obstruction BPO . EAU. 2022 Mar. Open 13. Dean Elterman, M lanie Aub -Peterkin, Howard Evans et al. UPDATE Canadian Urological Association guideline: Male lower urinary tract symptoms/benign prostatic hyperplasia. Can Urol Assoc J. 2022 Aug;16 8 245 256. Open https://web.pathway.md/diseases/recsRPAiXiNov4aap 11/12 6/29/23, 4:18 AM Erectile dysfunction Pathway 14. American Urological Association. Choosing Wisely AUA recommendations. Choosing Wisely. 2018. Open 15. Giovanni Corona, Dimitrios G Goulis, Ilpo Huhtaniemi et al. European Academy of Andrology EAA guidelines on investigation, treatment and monitoring of functional hypogonadism in males: Endorsing organization: European Society of Endocrinology. Andrology. 2020 Sep;8 5 970 987. Open 16. Lori B Lerner, Kevin T McVary, Michael J Barry et al. Management of Lower Urinary Tract Symptoms Attributed to Benign Prostatic Hyperplasia: AUA GUIDELINE PART I Initial Work-up and Medical Management. J Urol. 2021 Oct;206 4 806 817. Open 17. Faysal A. Yafi, Lawrence Jenkins, Maarten Albersen et al. Erectile dysfunction. Nat Rev Dis Primers. 2016; 2 16003. Open 18. Gretchen M Irwin. Erectile Dysfunction. Prim Care. 2019 Jun;46 2 249 255. Open 19. Bobby B Najari, James A Kashanian. Erectile Dysfunction. JAMA. 2016 Nov 1;316 17 1838. Open 20. A Kaminsky, H Sperling, G Popken. Primary and secondary prevention of erectile dysfunction]. Urologe A. 2011 Oct;50 10 1265 8, 1270. Open 21. Paolo Capogrosso, Christian Fuglesang S Jensen, Giulia Rastrelli et al. Male Sexual Dysfunctions in the Infertile Couple-Recommendations From the European Society of Sexual Medicine ESSM . Sex Med. 2021 Jun;9 3 100377. Open 22. A Michael Lincoff, Shalender Bhasin, Panagiotis Flevaris et al. Cardiovascular Safety of Testosterone-Replacement Therapy. N Engl J Med. 2023 Jun 16. Open https://web.pathway.md/diseases/recsRPAiXiNov4aap 12/12 |
Guideline sources The following summarized guidelines for the evaluation and management of esophageal cancer are prepared by our editorial team based on guidelines from the Society for Immunotherapy of Cancer (SITC 2023), the Japan Endocrine Society (JES 2023), the American Society of Clinical Oncology (ASCO 2023; 2021; 2020), the Perioperative Quality Initiative (POQI/AUGIS 2022), the European Society of Medical Oncology (ESMO 2022), the American College of Radiology (ACR 2022), the American Association for Thoracic Surgery (AATS/ESTS 2022), the College of American Pathologists (CAP/AMP/FCC 2022), the American College of Gastroenterology (ACG 2022; 2016), the European Society of Gastrointestinal Endoscopy (ESGE 2021; 2017), the Canadian Task Force on Preventive Health Care (CTFPHC 2020), the American Society for Gastrointestinal Endoscopy (ASGE 2019; 2018; 2013), the British Society of Gastroenterology (BSG 2018), the American Society for Clinical Pathology (ASCP/CAP/ASCO 2017), the Society of Thoracic Surgeons (STS 2013), and the American Gastroenterological Association (AGA 2012). 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 25 26 27 27 28 Definition https://web.pathway.md/diseases/recGMUdC2zPetYflS 1/12 6/29/23, 4:19 AM t o e Esophageal cancer Pathway Esophageal cancer refers to malignant disease arising from the esophageal epithelium, and principally comprises SCC and adenocarcinoma. 25 Epidemiology Exposure to various carcinogens, genetic susceptibility, and repeated insults to the esophageal mucosa increase the risk of malignant transformation of esophageal epithelial cells, with the subsequent development of esophageal cancer. 27 Pathophysiology The overall annual incidence of esophageal cancer in the United States is 4.7 cases per 100,000 person-years. 26 Disease course Predominant changes in the squamous mucosa of the proximal esophagus predispose to esophageal SCC, while intestinal metaplasia of the mucosa of the distal esophagus predisposes to esophageal adenocarcinoma. 27 Prognosis and risk of recurrence The overall 5-year survival rate of patients with esophageal cancer is estimated at 15-20%. 28 Calculator Calculator Calculat Assess Respiratory Risk in Surgi Eastern Cooperative Oncology G Karnof Guidelines 1. Screening and diagnosis Indications for screening (GERD): do not screen adult patients with chronic GERD for esophageal adenocarcinoma or precursor conditions (Barrett's esophagus or dysplasia). D Indications for screening, Barrett's esophagus, general principles: As per ACG 2022 guidelines, perform both white light endoscopy and chromoendoscopy in patients undergoing endoscopic surveillance of Barrett's esophagus. B Show 6 more As per ASGE 2019 guidelines, perform chromoendoscopy, including virtual chromoendoscopy and Seattle protocol biopsy sampling, in patients with Barrett's esophagus undergoing surveillance. B Show 3 more As per ESGE 2017 guidelines, obtain endoscopic surveillance in patients with Barrett's esophagus. B Show 4 more https://web.pathway.md/diseases/recGMUdC2zPetYflS 2/12 6/29/23, 4:19 AM Esophageal cancer Pathway As per AGA 2012 guidelines, do not obtain surveillance examination within 3 years in patients with Barrett's esophagus with a second endoscopy confirming the absence of dysplasia on biopsy. D Indications for screening, Barrett's esophagus, without dysplasia: As per ASGE 2019 guidelines, consider performing surveillance endoscopy in patients with nondysplastic Barrett's esophagus. C As per ACG 2016 guidelines: Consider performing repeat endoscopy at 1-2 years to rule out Barrett's esophagus in patients with suspected Barrett's esophagus and lack of intestinal metaplasia on histology. C Do not perform repeat endoscopy if the initial endoscopy is negative for Barrett's esophagus. Perform repeat endoscopy after PPI therapy for 8-12 weeks if endoscopy reveals esophagitis (Los Angeles Classification B, C, or D), to ensure healing of esophagitis and exclude the presence of underlying Barrett's esophagus. D Indications for screening, Barrett's esophagus, with dysplasia: As per ASGE 2019 guidelines, do not obtain routine EUS to differentiate mucosal versus submucosal disease in patients with Barrett's esophagus with high-grade dysplasia/intramucosal cancer or nodules. D As per ASGE 2018 guidelines, consider obtaining surveillance over endoscopic eradication therapy in patients with low-grade dysplasia placing a high value on avoiding adverse events related to endoscopic eradication therapy. C As per BSG 2018 guidelines: Perform a repeat endoscopy at 6 months in patients with low-grade dysplasia. B Offer endoscopic ablation therapy, preferably with radiofrequency ablation and after a review by a specialist multidisciplinary team, in patients with low-grade dysplasia in any of the follow-up endoscopy (confirmed by an expert gastrointestinal pathologist in at least 2 sets of biopsies). Offer 6-monthly surveillance if ablation is not performed. A As per ESGE 2017 guidelines, optimize antireflux therapy and perform repeated endoscopy at 6 months in patients with a diagnosis of "indefinite for dysplasia" confirmed by a second expert gastrointestinal pathologist. Obtain surveillance as in patients with nondysplastic Barrett's esophagus if no definite dysplasia is found in subsequent biopsy samples (including if the biopsies are again classified as "indefinite for dysplasia"). B Show 2 more As per ACG 2016 guidelines, obtain endoscopic surveillance at intervals of 3-5 years in patients with Barrett's esophagus without dysplasia. B Show 2 more Indications for screening, Barrett's esophagus, post-treatment: As per ACG 2022 guidelines, obtain endoscopic surveillance in patients with Barrett's esophagus successfully completed endoscopic eradication therapy. B As per ASGE 2018 guidelines, consider performing surveillance endoscopy in patients with dysplasia and intramural cancer achieved complete eradication of intestinal metaplasia after endoscopic eradication therapy. C https://web.pathway.md/diseases/recGMUdC2zPetYflS 3/12 6/29/23, 4:19 AM Esophageal cancer Pathway As per ACG 2016 guidelines, continue endoscopic surveillance to detect recurrent intestinal metaplasia and/or dysplasia following successful endoscopic therapy and complete eradication of intestinal metaplasia. B Show 3 more 2. Classification and risk stratification Staging: As per ACR 2022 guidelines, obtain CT of the chest and abdomen with IV contrast or FDG- positron emission tomography/CT of the skull base to mid-thigh for initial staging of patients with newly diagnosed esophageal cancer. B As per ESMO 2022 guidelines, perform a physical examination, gastrointestinal endoscopy, and obtain contrast-enhanced CT or FDG-positron emission tomography-CT of the chest, abdomen pelvis during initial staging and risk assessment. Consider obtaining EUS for tumor and nodal staging. B Show 2 more As per ESGE 2017 guidelines: Consider performing EUS-guided sampling for the assessment of regional lymph nodes in T1 (and, depending on local treatment policy, T2) adenocarcinoma and of lesions suspicious for metastasis, such as distant lymph nodes, left liver lobe lesions, and suspected peritoneal carcinomatosis. C Obtain integrated FDG-positron emission tomography-CT over EUS for lymph node restaging and for predicting complete pathological response after neoadjuvant therapy. Consider performing EUS-guided sampling only in highly selected cases. B As per ASGE 2013 guidelines: Perform EUS and FNA (when indicated), in conjunction with cross-sectional imaging, for accurate staging of esophageal carcinoma. B Consider performing endoscopic mucosal resection or endoscopic submucosal dissection for the staging and treatment of nodular Barrett's esophagus and suspected intramucosal SCC and adenocarcinoma. C As per STS 2013 guidelines, consider obtaining CT of the chest and abdomen B and positron emission tomography for the staging of early-stage esophageal cancer. C Show 4 more 3. Diagnostic procedures Upper gastrointestinal endoscopy: As per ESMO 2022 guidelines, perform upper gastrointestinal endoscopy in patients with new dysphagia, gastrointestinal bleeding, recurrent aspiration or emesis and weight loss and/or loss of appetite. B As per STS 2013 guidelines, perform flexible endoscopy with biopsy as the primary method for the diagnosis of esophageal carcinoma. B Biopsy and histopathology: https://web.pathway.md/diseases/recGMUdC2zPetYflS 4/12 6/29/23, 4:19 AM Esophageal cancer Pathway As per ESMO 2022 guidelines, establish the diagnosis of esophageal cancer by histopathological assessment of multiple ( 6) endoscopic biopsies to guarantee an adequate representation of the tumor and sufficient tissue for molecular analysis. B Show 2 more As per ESGE 2021 guidelines: Take at least 6 biopsies in patients with suspected advanced esophageal cancer. B Take only 1-2 targeted biopsies for esophageal lesions potentially amenable to endoscopic resection (Paris classification 0-I, 0-II) to confirm the diagnosis and not compromise subsequent endoscopic resection. B As per ESGE 2017 guidelines, consider performing EUS-guided sampling aiming at a core biopsy after the failure of standard biopsy techniques in patients with diffuse esophageal wall thickening. Consider using newly developed biopsy techniques under optical endoscopic guidance as an alternative. C Show 3 more Ancillary testing (flow cytometry): obtain flow cytometry in patients with suspected gastrointestinal lymphoma, such as diffuse esophageal wall thickening. B Ancillary testing, PD-L1 testing: As per SITC 2023 guidelines: Obtain tumor testing for PD-L1 expression (using a validated antibody) in all patients with gastroesophageal cancer. B Use the CPS to quantify PD-L1 expression in patients with gastroesophageal adenocarcinoma. Use the CPS or the TPS to determine eligibility for immune checkpoint inhibitor therapy in patients with esophageal SCC. B As per ESMO 2022 guidelines, assess the PD-L1 expression by immunohistochemistry according to the TPS or CPS as a validated predictive biomarker for immune checkpoint inhibitor therapy for esophageal SCC. B Ancillary testing, HER2 testing: As per SITC 2023 guidelines, obtain HER2 testing on tumor tissue in patients with gastroesophageal adenocarcinoma. B As per ASCO/ASCP/CAP 2017 guidelines: Obtain HER2 testing on tumor tissue in patients with advanced esophageal adenocarcinoma being potential candidates for HER2-targeted therapy. A Obtain request HER2 testing on tumor tissue of the biopsy or resection specimens (primary or metastasis), if such specimens are available and adequate, or on FNA specimens (cell blocks) as an acceptable alternative, preferably before initiating trastuzumab therapy. B Ancillary testing, MMR/MSI: As per SITC 2023 guidelines, assess high MSI/mismatch repair deficiency status in all patients with gastroesophageal cancers. B Consider obtaining MSI status testing by next- generation sequencing. Consider obtaining mismatch repair status testing by immunohistochemistry. As per FCC/AMP/CAP 2022 guidelines, obtain mismatch repair-immunohistochemistry and/or MSI testing by PCR over MSI testing by next-generation sequencing to detect https://web.pathway.md/diseases/recGMUdC2zPetYflS 5/12 6/29/23, 4:19 AM Esophageal cancer Pathway deoxyRNA mismatch repair defects in patients with gastroesophageal cancer suitable for immune checkpoint inhibitor therapy. A Ancillary testing (tumor mutational burden): consider obtaining tumor mutational burden testing in patients with gastroesophageal cancer. C Consider obtaining tumor mutational burden testing by next-generation sequencing. Ancillary testing (genomic testing): insufficient evidence to recommend for or against genomic testing in patients with gastroesophageal adenocarcinoma. I Ancillary testing (EBV testing): do not obtain routine EBV testing for immune checkpoint inhibitor treatment determination in patients with gastroesophageal cancer. 4. Medical management General principles: As per ESMO 2022 guidelines, ensure multidisciplinary assessment and planning before commencing any treatment. B As per ASCO 2020 guidelines, offer multimodality therapy in patients with locally advanced esophageal carcinoma. B Management of locally advanced disease, surgery: As per JES 2023 guidelines, consider performing esophagectomy in patients with clinical stage I (T1bN0M0) esophageal cancer. C Show 3 more As per ESMO 2022 guidelines, perform surgery in patients with resectable esophageal adenocarcinoma, even after complete clinical tumor response to preoperative chemoradiotherapy or chemotherapy. B As per ASCO 2020 guidelines, decide on surgery in patients with esophageal SCC in the context of shared decision-making, taking into account age, comorbidities, patient preference, caregiver support, and other factors. A Management of locally advanced disease, chemoradiotherapy: As per JES 2023 guidelines, consider offering definitive chemoradiotherapy with adequate follow-up and salvage therapy in patients with clinical stage I (T1bN0M0) esophageal cancer desiring esophageal preservation. C Show 3 more As per ESMO 2022 guidelines, consider offering preoperative/perioperative chemotherapy or chemoradiotherapy in all patients with locally advanced resectable disease. B Show 5 more As per ASCO 2020 guidelines: Offer preoperative chemoradiotherapy or chemoradiotherapy without surgery (definitive chemoradiotherapy) in patients with locally advanced esophageal SCC. B Offer preoperative chemoradiotherapy or perioperative chemotherapy in patients with locally advanced esophageal adenocarcinoma. B Management of locally advanced disease, immunotherapy: As per JES 2023 guidelines: https://web.pathway.md/diseases/recGMUdC2zPetYflS 6/12 6/29/23, 4:19 AM Esophageal cancer Pathway Offer postoperative nivolumab therapy, regardless of the histologic type or tumor expression level of PD-L1, in patients with clinical stage II-III esophageal cancer failed to show a pathologic complete response after preoperative chemoradiotherapy plus surgery with radical resection. A Insufficient evidence to recommend postoperative nivolumab therapy in patients with clinical stage II-III esophageal failed to show a pathologic complete response after preoperative chemotherapy plus surgery with radical resection. I As per SITC 2023 guidelines, offer adjuvant nivolumab as the standard of care in patients with resected (R0) stage II or III esophageal or GEJ cancer with residual pathological disease following neoadjuvant chemoradiotherapy. B As per ESMO 2022 guidelines, offer adjuvant nivolumab following neoadjuvant chemoradiotherapy in patients with SCC or adenocarcinoma of the esophagus or GEJ showing evidence of residual pathological disease in the resection specimen ( ypT1 and/or ypN1). A As per ESMO 2022 guidelines, offer trastuzumab-chemotherapy in patients with HER2- positive tumors. A Show 2 more As per ASCO 2021 guidelines, offer nivolumab following neoadjuvant chemoradiotherapy and surgery in patients with locally advanced esophageal carcinoma with ECOG status 0-1 failed to experience a pathological complete response (with a residual disease of at least ypT1 or ypN1 in resected specimens). B Management of advanced/metastatic disease, immunotherapy/targeted therapy: As per ASCO 2023 guidelines, offer pembrolizumab plus fluoropyrimidine- and platinum- based chemotherapy in patients with HER2-negative esophageal SCC and PD-L1 CPS 10. A Show 5 more As per JES 2023 guidelines, offer pembrolizumab, cisplatin, and 5-fluorouracil regimen as first-line therapy in patients with unresectable, advanced/recurrent esophageal cancer. A Show 6 more As per SITC 2023 guidelines, do not offer single-agent immune checkpoint inhibitor therapy in patients with untreated, advanced, microsatellite stable/mismatch repair-proficient gastroesophageal cancer. Consider offering single-agent anti-PD-1 therapy as an option in patients with untreated, advanced, high MSI/mismatch repair-deficient gastroesophageal cancer ineligible for chemotherapy. D Show 6 more As per ESMO 2022 guidelines, offer platinum and fluoropyrimidine as first-line chemotherapy in patients with advanced, untreated esophageal SCC. B Offer dose-reduced oxaliplatin- capecitabine as an alternative option in patients unsuitable for full-dose chemotherapy. A Show 5 more As per ASCO/ASCP/CAP 2017 guidelines, offer combination chemotherapy and HER2- targeted therapy as initial treatment in appropriate patients with HER2-positive advanced esophageal adenocarcinoma. B Management of advanced/metastatic disease (chemoradiotherapy): https://web.pathway.md/diseases/recGMUdC2zPetYflS 7/12 6/29/23, 4:19 AM Esophageal cancer Pathway Consider offering definitive chemoradiotherapy in patients with unresectable clinical stage IVA esophageal cancer. C Consider offering additional chemotherapy in patients with clinical stage IVA esophageal carcinoma showing complete response to definitive chemoradiotherapy. C Management of advanced/metastatic disease (surgery): consider performing surgical resection in patients with unresectable, locally advanced esophageal cancer (cT4 N0-3M0) becoming resectable after definitive chemoradiotherapy or induction chemotherapy. C Palliative care: As per JES 2023 guidelines: Offer oral prednisolone, submucosal triamcinolone injection, or concurrent oral prednisolone and submucosal triamcinolone injection to prevent stenosis after endoscopic resection. B Consider offering palliative radiotherapy in patients with clinical stage IVB esophageal cancer presenting with obstruction. C As per ESMO 2022 guidelines, offer early palliative care referral and nutritional support in patients with advanced esophageal cancer. A As per ASGE 2013 guidelines, perform esophageal stent placement as the preferred method for palliation of dysphagia and fistulae secondary to esophageal cancer in the majority of patients, to provide immediate and durable symptom relief. B 5. Nonpharmacologic interventions Nutritional support: assess nutritional status and history of weight loss B and provide nutritional support as appropriate. B 6. Perioperative care Analgesia: perform either thoracic epidural or paravertebral blockade as the primary method of analgesia for esophagectomy to reduce pulmonary complications. B Perioperative thromboprophylaxis: consider administering parenteral anticoagulation with subcutaneous LMWH or UFH for VTE prevention in patients undergoing esophagectomy. C Consider preferring LMWH over UFH. C Show 6 more Perioperative supportive care: Target normovolemia to reduce pulmonary complications during esophagectomies. B Use a lung protective ventilation strategy throughout the operation comprising minimization of peak pressure, limiting tidal volume, optimizing PEEP, and using recruitment maneuvers to reduce pulmonary complications. B Perioperative nutrition: Do not place nasogastric tubes routinely during esophagectomies to reduce the risk of pulmonary complications. D Initiate clear oral fluids in the immediate postoperative phase after esophagectomy. B https://web.pathway.md/diseases/recGMUdC2zPetYflS 8/12 6/29/23, 4:19 AM Esophageal cancer Pathway Postoperative rehabilitation: offer chest physiotherapy and early mobilization to reduce pulmonary complications after esophagectomy. B 7. Surgical interventions Technical considerations for surgery: As per ESMO 2022 guidelines, perform laparoscopy in patients with locally advanced (T3/T4) adenocarcinoma of the GEJ crossing the diaphragm to infiltrate the anatomical cardia. B Show 3 more As per AUGIS 2022 guidelines: Perform either minimally invasive esophagectomy B or robot-assisted minimally invasive esophagectomy over open esophagectomy to reduce the risk of pulmonary complications. B Do not perform routine pyloric drainage procedures to reduce pulmonary complications. D As per ASGE 2013 guidelines, consider performing endoscopic mucosal resection or endoscopic submucosal dissection for the treatment and staging of nodular Barrett's esophagus and suspected intramucosal SCC and adenocarcinoma. C Show 2 more 8. Patient education General counseling: consider a variety of factors, including patient preferences, quality of life, and prognosis with the patient and family before initiating endoscopic palliation for esophageal malignancy. C 9. Follow-up and surveillance Follow-up: As per ACR 2022 guidelines, obtain FDG-positron emission tomography/CT of the skull base to mid-thigh for the evaluation of patients with esophageal cancer undergoing treatment. B Show 2 more As per ESMO 2022 guidelines: Recognize that the majority ( 90%) of relapses occur within the first 2 years after completion of local therapy. Focus on symptoms, nutrition, and psychosocial support during follow-up visits. B Consider obtaining a 3-month follow-up based on endoscopy, biopsies, and CT to detect early recurrence in case of complete response to definitive chemoradiotherapy. C Clinical findings Symptoms Past medical history Acid reflux Aspiration pneumonia https://web.pathway.md/diseases/recGMUdC2zPetYflS 9/12 6/29/23, 4:19 AM Esophageal cancer Pathway Chest pain Barrett's esophagus Cough Bloom syndrome Dysphagia Bone metastasis Epigastric pain Brain metastasis Fatigue Exposure to radiation therapy Heartburn Fanconi anemia Hematemesis GERD Hoarseness HPV infection Melena Howel-evans syndrome Nausea Liver metastasis Odynophagia Lung metastasis Vomiting Obesity Weight loss Peritoneal metastasis Upper gastrointestinal bleeding Social history Abdominal exam Alcohol consumption Consumption of hot food and beverage Epigastric mass Integument exam High red meat consumption Tobacco use Digital clubbing Lymphatic exam Hematological findings Supraclavicular lymphadenopathy Anemia Lab findings Iron deficiency Studies 2023 ANTICIPUSC In patients at high risk of postoperative pulmonary complications after elective or semi-urgent surgery with an Assess Respiratory Risk in Surgical Patients in Catalonia score 45, prophylactic noninvasive ventilation was not superior to usual postoperative care with respect to the rate of in-hospital acute respiratory failure within 7 days after surgery. Stanislas Abrard et al. Br J Anaesth. 2023 Jan. 2006 MAGIC In patients with resectable adenocarcinoma of the stomach, esophagogastric junction, or lower esophagus, perioperative chemotherapy plus surgery was superior to surgery alone with respect to overall survival. David Cunningham et al. N Engl J Med. 2006 Jul 6. References https://web.pathway.md/diseases/recGMUdC2zPetYflS 10/12 6/29/23, 4:19 AM Esophageal cancer Pathway 1. Ronan J Kelly, Katherine Bever, Joseph Chao et al. Society for Immunotherapy of Cancer SITC clinical practice guideline on immunotherapy for the treatment of gastrointestinal cancer. J Immunother Cancer. 2023 Jun;11 6):e006658. Open 2. Pritam Singh, James Gossage, Sheraz Markar et al. Association of Upper Gastrointestinal Surgery of Great Britain and Ireland AUGIS /Perioperative Quality Initiative POQI consensus statement on intraoperative and postoperative interventions to reduce pulmonary complications after oesophagectomy. Br J Surg. 2022 Oct 14;109 11 1096 1106. Open 3. Yuko Kitagawa, Ryu Ishihara, Hitoshi Ishikawa et al. Esophageal cancer practice guidelines 2022 edited by the Japan esophageal society: part 1. Esophagus. 2023 Mar 18. Open 4. Evans JA, Early DS, Chandraskhara V et al. The role of endoscopy in the assessment and treatment of esophageal cancer. Gastrointest Endosc. 2013 Mar;77 3 328 34. Open 5. Thomas K Varghese Jr, Wayne L Hofstetter, Nabil P Rizk et al. The Society of Thoracic Surgeons guidelines on the diagnosis and staging of patients with esophageal cancer. Ann Thorac Surg. 2013 Jul;96 1 346 56. Open 6. Bas Weusten, Raf Bisschops, Emanuel Coron et al. Endoscopic management of Barrett's esophagus: European Society of Gastrointestinal Endoscopy ESGE Position Statement. Endoscopy. 2017 Feb;49 2 191 198. Open 7. Manish A Shah, Erin B Kennedy, Daniel V Catenacci et al. Treatment of Locally Advanced Esophageal Carcinoma: ASCO Guideline. J Clin Oncol. 2020 Aug 10;38 23 2677 2694. Open 8. F Lordick, F Carneiro, S Cascinu et al. Gastric cancer: ESMO Clinical Practice Guideline for diagnosis, treatment and follow-up. Ann Oncol. 2022 Oct;33 10 1005 1020. Open 9. Constantine A. Raptis, MD, Alan Goldstein et al. ACR Appropriateness Criteria Staging and Follow- up of Esophageal Cancer. ACR. 2022. Open 10. R Obermannov , M Alsina, A Cervantes et al. Oesophageal cancer: ESMO Clinical Practice Guideline for diagnosis, treatment and follow-up. Ann Oncol. 2022 Jul 29;S0923 7534 22 01850 6. Open 11. ASGE STANDARDS OF PRACTICE COMMITTEE, Bashar Qumseya, Shahnaz Sultan et al. ASGE guideline on screening and surveillance of Barrett's esophagus. Gastrointest Endosc. 2019 Sep;90 3 335 359.e2. Open 12. St phane Groulx, Heather Limburg, Marion Doull et al. Guideline on screening for esophageal adenocarcinoma in patients with chronic gastroesophageal reflux disease. CMAJ. 2020 Jul 6;192 27 E768 E777. Open 13. Jean-Marc Dumonceau, Pierre H Deprez, Christian Jenssen et al. Indications, results, and clinical impact of endoscopic ultrasound EUS)-guided sampling in gastroenterology: European Society of Gastrointestinal Endoscopy ESGE Clinical Guideline Updated January 2017. Endoscopy. 2017 Jul;49 7 695 714. Open 14. Manish A Shah, Wayne L Hofstetter, Erin B Kennedy et al. Immunotherapy in Patients With Locally Advanced Esophageal Carcinoma: ASCO Treatment of Locally Advanced Esophageal Carcinoma Guideline Rapid Recommendation Update. J Clin Oncol. 2021 Oct 1;39 28 3182 3184. Open 15. Angela N Bartley, Mary Kay Washington, Carol Colasacco et al. HER2 Testing and Clinical Decision Making in Gastroesophageal Adenocarcinoma: Guideline From the College of American Pathologists, American Society for Clinical Pathology, and the American Society of Clinical Oncology. J Clin Oncol. 2017 Feb;35 4 446 464. Open 16. Standards of Practice Committee, Sachin Wani, Bashar Qumseya et al. Endoscopic eradication therapy for patients with Barrett's esophagus-associated dysplasia and intramucosal cancer. https://web.pathway.md/diseases/recGMUdC2zPetYflS 11/12 6/29/23, 4:19 AM Esophageal cancer Pathway Gastrointest Endosc. 2018 Apr;87 4 907 931.e9. Open 17. Yaron Shargall, Wojtek Wiercioch, Alessandro Brunelli et al. Joint 2022 European Society of Thoracic Surgeons and The American Association for Thoracic Surgery guidelines for the prevention of cancer- associated venous thromboembolism in thoracic surgery. Eur J Cardiothorac Surg. 2022 Dec 2;63 1):ezac488. Open 18. Massimiliano di Pietro, Rebecca C Fitzgerald, BSG Barrett's guidelines working group. Revised British Society of Gastroenterology recommendation on the diagnosis and management of Barrett's oesophagus with low-grade dysplasia. Gut. 2018 Feb;67 2 392 393. Open 19. Shaheen NJ, Falk GW, Iyer PG et al. ACG Clinical Guideline: Diagnosis and Management of Barrett's Esophagus. Am J Gastroenterol. 2016 Jan;111 1 30 50. Open 20. Angela N Bartley, Anne M Mills, Eric Konnick et al. Mismatch Repair and Microsatellite Instability Testing for Immune Checkpoint Inhibitor Therapy: Guideline From the College of American Pathologists in Collaboration With the Association for Molecular Pathology and Fight Colorectal Cancer. Arch Pathol Lab Med. 2022 Oct 1;146 10 1194 1210. Open 21. Roos E Pouw, Maximilien Barret, Katharina Biermann et al. Endoscopic tissue sampling Part 1 Upper gastrointestinal and hepatopancreatobiliary tracts. European Society of Gastrointestinal Endoscopy ESGE Guideline. Endoscopy. 2021 Nov;53 11 1174 1188. Open 22. American Gastroenterological Association. Choosing Wisely: Recommendations of the American Gastroenterological Association. Choosing Wisely. 2012 Apr. Open 23. Nicholas J Shaheen, Gary W Falk, Prasad G Iyer et al. Diagnosis and Management of Barrett's Esophagus: An Updated ACG Guideline. Am J Gastroenterol. 2022 Apr 1;117 4 559 587. Open 24. Manish A Shah, Erin B Kennedy, Ashley E Alarcon-Rozas et al. Immunotherapy and Targeted Therapy for Advanced Gastroesophageal Cancer: ASCO Guideline. J Clin Oncol. 2023 Jan 5;JCO2202331. Open 25. Lagergren J, Smyth E, Cunningham D et al. Oesophageal cancer. Lancet. 2017 Nov 25;390 10110 2383 2396. Open 26. Patel N, Benipal B. Incidence of Esophageal Cancer in the United States from 2001 2015 A United States Cancer Statistics Analysis of 50 States. Cureus. 2018 Dec 10;10 12):e3709. Open 27. Smyth EC, Lagergren J, Fitzgerald RC et al. Oesophageal cancer. Nat Rev Dis Primers. 2017 Jul 27;3 17048. Open 28. Abbas G, Krasna M. Overview of esophageal cancer. Ann Cardiothorac Surg. 2017 Mar;6 2 131 136. Open 29. Ajani JA, D'Amico TA, Bentrem DJ et al. Esophageal and Esophagogastric Junction Cancers, Version 2.2019, NCCN Clinical Practice Guidelines in Oncology. J Natl Compr Canc Netw. 2019 Jul 1;17 7 855 883. Open 30. Ajani JA, D'Amico TA, Bentrem DJ et al. Esophageal and Esophagogastric Junction Cancers, Version 2.2019, NCCN Clinical Practice Guidelines in Oncology. J Natl Compr Canc Netw. 2019 Jul 1;17 7 855 883. Open 31. ASGE Standards of Practice Committee, Early DS, Acosta RD et al. Adverse events associated with EUS and EUS with FNA. Gastrointest Endosc. 2013 Jun;77 6 839 43. Open https://web.pathway.md/diseases/recGMUdC2zPetYflS 12/12 |
Guideline sources The following summarized guidelines for the evaluation and management of esophageal dysphagia are prepared by our editorial team based on guidelines from the Canadian Association of Gastroenterologists (CAG 2018). 1 2 2 3 4 Definition Esophageal dysphagia is characterized by difficulty transporting food down the esophagus due to involvement of the esophageal body and esophagogastric junction. 2 Epidemiology Esophageal dysphagia is caused by structural or inflammatory abnormalities (strictures, rings, webs, malignancy, reflux esophagitis, eosinophilic esophagitis) and motility disorders (achalasia, ineffective esophageal motility, esophageal spasm, esophagogastric junction outflow obstruction). 2 Disease course Clinical manifestations of esophageal dysphagia include difficulty swallowing solids and liquids, intermittent or progressive dysphagia, food bolus impaction, food regurgitation, heartburn, weight loss, anorexia, respiratory distress, hypotension, and atypical chest pain. 3 Prognosis and risk of recurrence Annual mortality associated with GERD is 0.46 per 100,000 individuals. 4 https://web.pathway.md/diseases/rec9Ot9rr5kLFbhao 1/3 6/29/23, 4:19 AM Esophageal dysphagia Pathway Guidelines 1. Diagnostic investigations Clinical assessment: identify patients with oropharyngeal dysphagia according to presenting symptoms and physical examination. B Show 2 more Barium esophagram: consider obtaining a barium esophagram in some patients with esophageal dysphagia when there is limited local access to endoscopy, in order to assess for significant structural lesions and facilitate timely referral to urgent endoscopy and specialist consultation. B 2. Diagnostic procedures Upper GI endoscopy: Obtain upper gastrointestinal endoscopy as the initial test to maximize diagnostic yield in patients with persistent esophageal dysphagia. B Perform esophageal biopsies to detect mucosal pathology in all patients undergoing endoscopy for esophageal dysphagia, unless there are clear features of erosive reflux esophagitis. B Esophageal manometry: obtain esophageal manometry to evaluate for esophageal motility disorders in patients with persistent esophageal dysphagia in whom structural and inflammatory causes have been ruled out. B 3. Medical management Proton pump inhibitors: Provide a diagnostic and therapeutic trial of an oral PPI (given BID) in patients < 50 years of age presenting with esophageal dysphagia and reflux symptoms, in whom there are no alarm features to suggest underlying malignancy (bleeding, odynophagia, weight loss, or vomiting). B Obtain further testing if dysphagia does not resolve completely after a 4-week trial acid suppression therapy in patients with esophageal dysphagia in whom a trial of a PPI was initiated. B Clinical findings Patient demographics Symptoms Elderly age Bringing back up undigested food Past medical history Cough Dysphagia Esophageal tumors Food regurgitation Exposure to radiation therapy https://web.pathway.md/diseases/rec9Ot9rr5kLFbhao 2/3 6/29/23, 4:19 AM Esophageal dysphagia Pathway Heartburn Gastroesophageal reflux Hoarseness Scleroderma Muscle spasms Endoscopic findings Weight loss Esophageal foreign body Head and neck exam Drooling References 1. Louis WC. Liu, Christopher N. Andrews, David Armstrong et al. Clinical Practice Guidelines for the Assessment of Uninvestigated Esophageal Dysphagia. Journal of the Canadian Association of Gastroenterology, 2018, 1 1 , 5 19. Open 2. Louis W C Liu, Christopher N Andrews, David Armstrong et al. Clinical Practice Guidelines for the Assessment of Uninvestigated Esophageal Dysphagia. 2018 Feb 9;1 1 5 19.2018 Feb 9;1 1 5 19. Open 3. Prianka Chilukuri, Florence Odufalu, Christine Hachem. Dysphagia. May-Jun 2018;115 3 206 210.May-Jun 2018;115 3 206 210. Open 4. Tuomo K Rantanen, Eero I T Sihvo, Jari V Rasanen et al. Gastroesophageal reflux disease as a cause of death is increasing: analysis of fatal cases after medical and surgical treatment. 2007 Feb;102 2 246 53.2007 Feb;102 2 246 53. Open 5. Louis W C Liu, Christopher N Andrews, David Armstrong et al. Clinical Practice Guidelines for the Assessment of Uninvestigated Esophageal Dysphagia. J Can Assoc Gastroenterol. 2018 Apr; 1 1 5 19. Open 6. Prianka Chilukuri, Florence Odufalu, Christine Hachem. Dysphagia. Mo Med. 2018 May-Jun; 115 3 206 210. Open 7. Tuomo K Rantanen, Eero I T Sihvo, Jari V R s nen et al. Gastroesophageal reflux disease as a cause of death is increasing: analysis of fatal cases after medical and surgical treatment. Am J Gastroenterol. 2007 Feb;102 2 246 53. Open https://web.pathway.md/diseases/rec9Ot9rr5kLFbhao 3/3 |
Guideline sources The following summarized guidelines for the evaluation and management of esophageal perforation are prepared by our editorial team based on guidelines from the World Society of Emergency Surgery (WSES 2019). 1 Guidelines 1. Diagnostic investigations Laboratory studies: Obtain routine blood tests in patients with suspected esophageal perforation: CBC liver tests (bilirubin, ALT, AST) urea creatinine electrolytes (sodium, potassium, chlorine, magnesium, calcium) pH serum lactate. B Computed tomography: obtain contrast-enhanced CT and CT esophagography as the imaging modality of choice in patients with suspected esophageal perforation. B https://web.pathway.md/diseases/recxZg9dX8xSZrM1l 1/3 6/29/23, 4:19 AM Esophageal perforation Pathway 2. Diagnostic procedures Upper gastrointestinal endoscopy: perform diagnostic endoscopy in patients with suspected esophageal perforation and doubtful CT findings. B 3. Medical management Antibiotics: administer broad-spectrum antibiotics (aerobic and anaerobic bacteria) in patients eligible for non-surgical management. B Proton pump inhibitors: administer PPIs in patients eligible for non-surgical management. B 4. Nonpharmacologic interventions Indications for non-surgical management: consider non-surgical management of esophageal perforation in stable patients with early presentation, contained esophageal disruption, and minimal contamination of surrounding spaces if highly specialized surveillance is available. B Nutritional support: keep patients eligible for non-surgical management NPO. B Show 2 more 5. Therapeutic procedures Therapeutic endoscopy: Perform endoscopic treatment for closing esophageal perforation that occur and are recognized during an endoscopic procedure. B Consider endoscopy as first-line therapy in patients with late presentation and in patients with non-endoscopic esophageal perforation. C 6. Surgical interventions Surgery: Proceed with surgery in all patients who do not meet the criteria for non-surgical management: delay in management of < 24 h absence of symptoms and signs of sepsis cervical or thoracic location of the esophageal perforation contained perforation by surrounding tissues (intramural; minimal peri-esophageal extravasation of contrast material with intra-esophageal drainage; absence of massive pleural contamination) no preexistent esophageal disease possibility of close surveillance by expert esophageal team availability of surgical and radiological skills all day and all night. B Show 7 more https://web.pathway.md/diseases/recxZg9dX8xSZrM1l 2/3 6/29/23, 4:19 AM Esophageal perforation Pathway References 1. Mircea Chirica, Michael D Kelly, Stefano Siboni et al. Esophageal emergencies: WSES guidelines. World J Emerg Surg. 2019 May 31;14 26. Open 2. Jeffrey H Lee, Prashant Kedia, Stavros N Stavropoulos et al. AGA Clinical Practice Update on Endoscopic Management of Perforations in Gastrointestinal Tract: Expert Review. Clin Gastroenterol Hepatol. 2021 Nov;19 11 2252 2261.e2. Open https://web.pathway.md/diseases/recxZg9dX8xSZrM1l 3/3 |
Guideline sources The following summarized guidelines for the management of esophageal stricture are prepared by our editorial team based on guidelines from the American Society for Gastrointestinal Endoscopy (ASGE 2014). 1 2 2 3 4 Definition Esophageal stricture is a chronic condition characterized by dysphagia due to esophageal inflammation, collagen deposition and scar tissue. 2 Epidemiology Esophageal stricture is caused mainly by GERD, esophagitis, tumors (adenocarcinoma, SCC), corrosive injury to the esophagus, intrinsic/extrinsic compressions, Plummer-Vinson syndrome, postsurgical complications, and congenital defects. 3 Disease course Clinical manifestations of an esophageal stricture include dysphagia, gastroesophageal reflux, vomiting, chest pain, cough, hoarseness, gastrointestinal bleeding, anemia, and weight loss. There is an increased risk of recurrence after initial dilatation. 2 Prognosis and risk of recurrence In-hospital mortality in benign and malignant esophageal strictures is 1.4% and 3.1%, respectively. 4 Guidelines https://web.pathway.md/diseases/rechN2bT9lWPOjuu1 1/3 6/29/23, 4:19 AM Esophageal stricture Pathway 1. Medical management Antisecretory treatment: initiate antisecretory treatment in patients undergoing endoscopic dilation for a peptic esophageal stricture, in order to reduce the risk of recurrent strictures. A Adjunctive corticosteroid injections: consider performing intralesional corticosteroid injection as adjunctive treatment for patients with recurrent or refractory benign esophageal peptic strictures. C 2. Therapeutic procedures Endoscopic dilation: perform endoscopic dilation for patients with dysphagia secondary to benign intrinsic strictures of the esophagus. A Show 2 more Esophageal stent placement: consider performing esophageal stent placement only in patients with refractory esophageal strictures that do not respond to sequential dilation and/or intralesional corticosteroid injection. C Clinical findings Symptoms Past medical history Burning sensation Cancer Choking and coughing fits Esophageal tumors Dysphagia Exposure to radiation therapy Epigastric abdominal pain Gastroesophageal reflux Food impaction Hiatal hernia Medications Past surgical history Peptic ulcer disease Recent surgery Social history Alcohol consumption References 1. Pasha SF, Acosta RD, Chandrasekhara V et al. The role of endoscopy in the evaluation and management of dysphagia. Gastrointest Endosc. 2014 Feb;79 2 191 201. Open 2. Ana Ruig mez, Luis Alberto Garc a Rodr guez, Mari-Ann Wallander et al. Esophageal stricture: incidence, treatment patterns, and recurrence rate. Am J Gastroenterol. 2006 Dec;101 12 2685 92. Open 3. D W McBride Jr, S D Roper. Ca(2+)-dependent chloride conductance in Necturus taste cells. 1991 Oct;124 1 85 93.1991 Oct;124 1 85 93. Open 4. Abhinav Goyal, Kshitij Chatterjee, Sujani Yadlapati et al. Health-Care Utilization and Complications of Endoscopic Esophageal Dilation in a National Population. 2017 Jul;50 4 366 371.2017 Jul;50 4 366 371. Open https://web.pathway.md/diseases/rechN2bT9lWPOjuu1 2/3 6/29/23, 4:19 AM Esophageal stricture Pathway 5. Abhinav Goyal, Kshitij Chatterjee, Sujani Yadlapati et al. Health-Care Utilization and Complications of Endoscopic Esophageal Dilation in a National Population. Clin Endosc. 2017 Jul; 50 4 366 371. Open https://web.pathway.md/diseases/rechN2bT9lWPOjuu1 3/3 |
Guideline sources The following summarized guidelines for the evaluation and management of essential thrombocythemia (ET) are prepared by our editorial team based on guidelines from the European Leukemia Net (ELN 2018), the European Society of Medical Oncology (ESMO 2015), the British Committee for Standards In Haematology (BCSH 2012), and the British Society for Haematology (BSH 2010). 1 2 3 4 Calculator Calculator Revised IPSET score for essenti WHO criteria for essential throm Guidelines 1. Screening and diagnosis https://web.pathway.md/diseases/recmSleoxzC8TqF9W 1/6 6/29/23, 4:19 AM Essential thrombocythemia Pathway Indications for testing: evaluate all patients with unexplained splanchnic vein thrombosis for the presence of myeloproliferative neoplasms even if the blood count is normal. B Diagnostic criteria: As per ELN 2018 guidelines, use the 2016 revised WHO criteria for diagnosis of ET. E As per ESMO 2015 guidelines, ensure accurate differentiation among the three unique myeloproliferative neoplasm subtypes as well as the exclusion of reactive conditions (in mutation-negative patients only) and disorders, such as myelodysplasia and chronic myeloid leukemia, for appropriate prognosis and treatment decision-making. Do not use the generic diagnostic label 'myeloproliferative neoplasm' alone. A 2. Classification and risk stratification Risk of thrombosis: As per ELN 2018 guidelines: Use the IPSET-thrombosis system (including age, previous thrombosis, cardiovascular risk factors, and JAK2V617F mutation) for prognostic purposes in all patients at diagnosis of ET. E Consider assessing general risk factors for thrombosis, including smoking history, diabetes mellitus, hypertension, and hyperlipidemia. E As per ESMO 2015 guidelines, categorize patients with newly diagnosed myeloproliferative neoplasm at baseline according to the risks associated with the disease. B As per BSH 2010 guidelines, stratify patients according to their risk of thrombotic complications as follows: Situation Guidance Age > 60 years, or High risk History of ET-related thrombotic or hemorrhagic event, or Platelet count of > 1, 500 10 /L Age 40-60 years with no high-risk features Intermediate risk Age < 40 years with no high-risk features. B Low risk Show 3 more 3. Diagnostic investigations Genetic testing: As per ELN 2018 guidelines, obtain mutational analysis for JAK2V617F, CALR, and MPL on peripheral blood or bone marrow samples in patients with suspected ET. E https://web.pathway.md/diseases/recmSleoxzC8TqF9W 2/6 6/29/23, 4:19 AM Essential thrombocythemia Pathway As per ESMO 2015 guidelines, obtain testing for 3 driver mutations in all patients with suspected myeloproliferative neoplasms. B 4. Diagnostic procedures Bone marrow biopsy: perform bone marrow biopsy in all patients with suspected Philadelphia chromosome-negative myeloproliferative neoplasms, except for patients with polycythemia vera with Hgb > 18.5 g/dL in males and > 16.5 g/dL in females. E 5. Medical management Treatment target: As per ESMO 2015 guidelines, set an optimal platelet target of < 400 10 /L (although unvalidated) in patients with ET. B As per BSH 2010 guidelines, return the platelet count into the normal range as the target of treatment. B Cytoreductive therapy: As per ELN 2018 guidelines, administer hydroxyurea, recombinant interferon-alpha, or anagrelide to produce a hematological response in high-risk patients with ET. Administer hydroxyurea or recombinant interferon-alpha as first-line therapy agents. E Show 2 more Landmark trials: ANAHYDRET In patients with essential thrombocythemia who have high-risk profile, anagrelide was noninferior to hydroxyurea with respect to a ET-related major and minor thrombohemorrhagic events at 6 months. Gisslinger H et al. Blood. 2013 Mar 7. As per ESMO 2015 guidelines, administer hydroxyurea as first-line cytoreductive therapy in patients with high-risk ET. Consider initiating interferon, or sometimes anagrelide, as first-line therapy in specific groups of patients. B Show 2 more Landmark trials: Hydroxyurea versus anagrelide in high-risk ET In patients with essential thrombocythemia who were at high-risk for vascular events and already receiving low-dose aspirin, hydroxyurea was superior to anagrelide with respect to arterial or venous thrombosis, serious hemorrhage, or death from vascular causes. Harrison CN et al. N Engl J Med. 2005 Jul 7. Antiplatelet therapy: https://web.pathway.md/diseases/recmSleoxzC8TqF9W 3/6 6/29/23, 4:19 AM Essential thrombocythemia Pathway As per ELN 2018 guidelines, initiate low-dose aspirin in all patients with IPSET-thrombosis high-risk disease as well as in patients with low- or intermediate-risk ET when reaching age 60 years, or when uncontrolled cardiovascular risk factors or JAK2V617F mutation are present. E As per ESMO 2015 guidelines, initiate low-dose aspirin in all patients with ET without clear contraindications to aspirin. B As per BSH 2010 guidelines, initiate aspirin in all patients with ET unless contraindicated. B 6. Perioperative care Preoperative management: consider controlling blood counts preoperatively to standard targets in high-risk patients undergoing surgery, when bleeding is a risk or thromboprophylaxis would normally be prescribed. C 7. Specific circumstances Pediatric patients: Exclude reactive thrombocytosis rigorously and diagnose ET only in the presence of definitive diagnostic features. B Insufficient evidence to guide the management of ET in pediatric patients, however, offer a conservative approach where possible. I Pregnant patients: manage pregnant patients with ET by a multidisciplinary team. E Show 3 more Breastfeeding patients: recognize that breastfeeding is contraindicated with cytoreductive therapy. Decide about breastfeeding while taking interferon on an individual basis after discussion regarding possible risks and benefits. B Patients on hormonal therapy: As per ESMO 2015 guidelines: Obtain an individualized risk-benefit assessment to decide on hormonal therapy. B Advise using progesterone-only preparations for oral contraception and avoid combined oral contraceptives. B As per BSH 2010 guidelines, discourage the use of combined oral contraceptives in female patients with ET. E Show 2 more Patients undergoing surgery: obtain individual risk assessment (including disease-, patient-, and procedure-specific risks) for VTE in patients undergoing surgery, and offer appropriate graded elastic compression stockings and pharmacotherapy. E Show 4 more Patients with leukemic transformation: As per ESMO 2015 guidelines, consider administering acute leukemia-like regimens in patients with leukemic transformation deemed potential candidates for allo-SCT. Consider offering azacitidine. C https://web.pathway.md/diseases/recmSleoxzC8TqF9W 4/6 6/29/23, 4:19 AM Essential thrombocythemia Pathway As per BSH 2010 guidelines: Diagnose leukemic transformation when 20% blasts are consistently present either in blood or bone marrow. E Recognize that most patients with leukemic transformation have poor overall survival. Consider offering stem cell transplantation in patients with leukemic transformation. B Patients with post-ET myelofibrosis: As per BCSH 2012 guidelines, consider using the International Prognostic Scoring System, DIPSS, and DIPSS-plus prognostic scores in patients with post-ET myelofibrosis. C As per BSH 2010 guidelines, obtain regular monitoring (3-yearly) for early signs of progression because of the risks of myelofibrotic transformation in patients receiving anagrelide treatment. B Show 3 more Clinical findings Patient demographics Symptoms Elderly Abdominal discomfort Bone pain Past medical history Chest pain Easy bruising Acquired vWD Fainting Acute coronary syndrome Fatigue DVT Generalized pruritus PE Gingival bleeding Splanchnic vein thrombosis Headache Stroke Lightheadedness TIA Night sweats Integument exam Nosebleed Paresthesia Ecchymosis Tinnitus Erythromelalgia Visual disturbances Skin erythema Weight loss Hematological findings Abdominal exam blood platelet count Splenomegaly Lab findings ESR serum CRP serum LDH Genetic testing Calr gene mutation https://web.pathway.md/diseases/recmSleoxzC8TqF9W 5/6 6/29/23, 4:19 AM Essential thrombocythemia Pathway Jak2 gene mutation Studies 2013 ANAHYDRET In patients with essential thrombocythemia who have high-risk profile, anagrelide was noninferior to hydroxyurea with respect to a ET-related major and minor thrombohemorrhagic events at 6 months. Gisslinger H et al. Blood. 2013 Mar 7. 2005 Hydroxyurea versus anagrelide in high-risk ET In patients with essential thrombocythemia who were at high-risk for vascular events and already receiving low-dose aspirin, hydroxyurea was superior to anagrelide with respect to arterial or venous thrombosis, serious hemorrhage, or death from vascular causes. Harrison CN et al. N Engl J Med. 2005 Jul 7. References 1. Claire N Harrison, David Bareford, Nauman Butt et al. Guideline for investigation and management of adults and children presenting with a thrombocytosis. Br J Haematol. 2010 May;149 3 352 75. Open 2. Tiziano Barbui, Ayalew Tefferi, Alessandro M Vannucchi et al. Philadelphia chromosome-negative classical myeloproliferative neoplasms: revised management recommendations from European LeukemiaNet. Leukemia. 2018 May;32 5 1057 1069. Open 3. A M Vannucchi, T Barbui, F Cervantes et al. Philadelphia chromosome-negative chronic myeloproliferative neoplasms: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2015 Sep;26 Suppl 5:v85 99. Open 4. John T Reilly, Mary Frances McMullin, Philip A Beer et al. Guideline for the diagnosis and management of myelofibrosis. Br J Haematol. 2012 Aug;158 4 453 71. Open 5. Mahnur Haider, Naseema Gangat, Terra Lasho et al. Validation of the revised International Prognostic Score of Thrombosis for Essential Thrombocythemia IPSET-thrombosis) in 585 Mayo Clinic patients. Am J Hematol. 2016 Jun;91 4 390 4. Open https://web.pathway.md/diseases/recmSleoxzC8TqF9W 6/6 |
Guideline sources The following summarized guidelines for the evaluation and management of Ewing's sarcoma (ES) are prepared by our editorial team based on guidelines from the European Reference Network on Genetic Tumour Risk Syndromes (GENTURIS/ERN PaedCan/EURACAN/ESMO 2021) and the National Comprehensive Cancer Network (NCCN 2021). 1 2 Calculator Calculator Calculat Eastern Cooperative Oncology G Karnofsky performance status s TNM cl Guidelines 1. Diagnostic investigations History and physical examination: As per ESMO 2021 guidelines, elicit medical history and perform physical examination in the initial work-up of patients with suspected primary bone sarcoma. B https://web.pathway.md/diseases/recK5pKn04x5UXoyE 1/4 6/29/23, 4:20 AM Ewing's sarcoma Pathway As per NCCN 2021 guidelines, elicit history and perform physical examination in patients presenting with suspected ES. B Imaging for staging: As per ESMO 2021 guidelines, obtain radiological assessment in the initial work-up of patients with suspected primary bone sarcoma. B Show 2 more As per NCCN 2021 guidelines: Obtain the following imaging modalities in the initial work-up of patients with ES: MRI +/- CT (both with contrast) of primary site chest CT positron emission tomography/CT (head-to-toe) and/or bone scan. B Consider obtaining screening MRI of spine and pelvis in the initial work-up of patients with ES. C Laboratory tests: As per ESMO 2021 guidelines, obtain baseline serum ALP and LDH in patients with ES. B As per NCCN 2021 guidelines, obtain LDH at presentation of patients with ES. B 2. Diagnostic procedures Biopsy and pathology: As per ESMO 2021 guidelines, perform biopsy in the initial work-op of patients with suspected primary bone sarcoma. B Show 6 more As per NCCN 2021 guidelines: Obtain cytogenetics and/or molecular testing (may require re-biopsy) in patients with ES. B Consider performing bone marrow biopsy in patients with ES. C 3. Medical management Setting of care: manage patients with bone sarcomas at reference centers and/or within reference networks able to provide access to the full spectrum of care and age-specific expertise. B Management of local/locoregional disease: As per ESMO 2021 guidelines, offer interval-compressed vincristine, doxorubicin, cyclophosphamide alternating with ifosfamide, etoposide as first-line therapy in patients with ES. B Show 6 more As per NCCN 2021 guidelines, offer the following local control options in patients with stable/improved disease following primary treatment: wide excision https://web.pathway.md/diseases/recK5pKn04x5UXoyE 2/4 6/29/23, 4:20 AM Ewing's sarcoma Pathway definitive radiotherapy and chemotherapy amputation in selected cases. B Show 7 more Management of advanced/metastatic disease: As per ESMO 2021 guidelines, offer chemotherapy in patients with metastatic disease at diagnosis similar to that used for localized disease. B Show 4 more As per NCCN 2021 guidelines, offer wide excision or definitive radiotherapy and chemotherapy for local control to primary site in patients with metastatic ES. B Show 7 more 4. Specific circumstances Patients with extraskeletal ES: Treat patients with extraskeletal ES following the same principles as for bone ES, incorporating chemotherapy in all patients and radiotherapy in most patients. B Decide on the number of chemotherapy cycles in patients with cutaneous/subcutaneous ES on an individual case basis, at a multidisciplinary level and with the patient. B 5. Patient education Fertility consultation: consider offering fertility consultation at initial presentation of patients with ES. C 6. Follow-up and surveillance Surveillance: As per ESMO 2021 guidelines, consider including the following in the follow-up of patients with high-grade bone sarcomas: physical examination cross-sectional imaging plain radiograph of the primary site CXR/chest CT. C Show 3 more As per NCCN 2021 guidelines, include the following in the surveillance of patients with stable/improved ES following primary treatment: physical examination MRI +/- CT (both with contrast of primary site) chest imaging (X-ray or CT) every 2-3 months radiographs of primary site CBC and other laboratory studies as indicated. B Show 2 more https://web.pathway.md/diseases/recK5pKn04x5UXoyE 3/4 6/29/23, 4:20 AM Ewing's sarcoma Pathway Management of relapse: As per ESMO 2021 guidelines, offer alkylating agents in combination with topoisomerase inhibitors, irinotecan with temozolomide, gemcitabine and docetaxel, high-dose ifosfamide or carboplatin with etoposide in patients with relapsed disease. B As per NCCN 2021 guidelines, offer the following options in patients with relapsed/refractory disease: initially stable/improved disease following primary treatment: chemotherapy +/- radiotherapy +/- without surgery progressive disease following primary and additional treatment: chemotherapy or best supportive care. B Show 2 more Clinical findings Patient demographics Symptoms Children Back pain White race Chest pain Cough Past medical history Dyspnea Fatigue Bone marrow metastasis Fever Lung metastasis Localized bone pain Lab findings Localized bone swelling Muscle weakness serum ALP Urinary retention serum LDH Weight loss Imaging findings Neurological exam Codman's triangle Sciatica Hair-on-end appearance Mottled bone lesion Hematological findings Multilayered periosteal reaction Anemia Osteolytic lesion References 1. S J Strauss, A M Frezza, N Abecassis et al. Bone sarcomas: ESMO EURACAN GENTURIS ERN PaedCan Clinical Practice Guideline for diagnosis, treatment and follow-up. Ann Oncol. 2021 Dec;32 12 1520 1536. Open 2. J Sybil Biermann, Angela Hirbe, Warren Chow et al. Bone Cancer, Version 2.2022, NCCN Clinical Practice Guidelines in Oncology. NCCN. 2021 Oct 8. Open https://web.pathway.md/diseases/recK5pKn04x5UXoyE 4/4 |
Guideline sources The following summarized guidelines for the evaluation and management of exercise-associated hyponatremia are prepared by our editorial team based on guidelines from the Wilderness Medical Society (WMS 2020). 1 Guidelines 1. Diagnostic investigations Assessment: obtain point-of-care testing in at-risk symptomatic patients when available. Integrate all available clinical and historical information into an assessment of the patient's hydration status (history of fluid intake, food intake, presenting signs and symptoms, body weight if available, and urine output), if testing is unavailable. B 2. Medical management Intravenous fluids: do not administer IV hypotonic fluids in patients with suspected fluid overload exercise-associated hyponatremia. D Show 2 more 3. Inpatient care https://web.pathway.md/diseases/recApOgw6COUOM4Yo 1/2 6/29/23, 4:20 AM Exercise-associated hyponatremia Pathway Transfer of care: inform receiving caregivers about the potential diagnosis of exercise- associated hyponatremia and appropriate fluid management (withhold hypotonic fluids) when transferring care. B Acute inpatient care: avoid administering oral and IV hypotonic or isotonic fluids early in the management of patients with exercise-associated hyponatremia, although, consider using in certain clinical contexts once sodium correction has been initiated or hypovolemia is confirmed. D Show 3 more 4. Nonpharmacologic interventions Oral fluid restriction: advise restricting oral fluids if exercise-associated hyponatremia from fluid overload is associated with mild symptoms. Do not administer hypotonic fluids in patients with suspected exercise-associated hyponatremia. B Oral sodium: consider adding oral sodium in hypertonic solutions or advising foods with high sodium content (salty snacks) for increasing serum sodium levels and enhancing symptom relief (over fluid restriction) in patients with mild exercise-associated hyponatremia, if tolerated. C 5. Preventative measures Proper hydration: advise avoiding sustained overhydration during exercise, as it is the primary risk factor for development of all variants of exercise-associated hyponatremia. A Show 2 more Salt supplementation: advise ensuring freely available sodium and/or salty snacks for consumption along with the appropriate fluid intake, particularly in long, hot events in non-heat acclimatized persons, but recognize that this strategy will not prevent exercise-associated hyponatremia when combined with overdrinking. B 6. Follow-up and surveillance Observation: observe patients for at least 60 minutes after exercise to ensure no decompensation from delayed symptomatic exercise-associated hyponatremia occurs after cessation of exercise. B References 1. Brad L Bennett, Tamara Hew-Butler, Mitchell H Rosner et al. Wilderness Medical Society Clinical Practice Guidelines for the Management of Exercise-Associated Hyponatremia: 2019 Update. Wilderness Environ Med. 2020 Mar;31 1 50 62. Open https://web.pathway.md/diseases/recApOgw6COUOM4Yo 2/2 |
Guideline sources The following summarized guidelines for the evaluation and management of exercise-induced bronchoconstriction (EIB) are prepared by our editorial team based on guidelines from the American Academy of Allergy, Asthma & Immunology (AAAAI/ACAAI 2016) and the American Thoracic Society (ATS 2013). 1 2 Guidelines 1. Screening and diagnosis Diagnosis: confirm the diagnosis of EIB by demonstrating airways reversibility or challenge in association with a history consistent with EIB because self-reported symptoms are not adequate. B Differential diagnosis: Suspect the diagnosis of exercise-induced anaphylaxis instead of EIB based on a history of shortness of breath or other respiratory tract symptoms accompanied by systemic symptoms (such as pruritus, urticaria, and hypotension). B Refer patients for psychological evaluation if the symptoms (such as hyperventilation and anxiety disorders) are in the differential diagnosis of EIB. B 2. Diagnostic investigations https://web.pathway.md/diseases/rectXVq5lHLidOgxS 1/4 6/29/23, 4:21 AM Exercise-induced bronchoconstriction Pathway Bronchoprovocation testing: obtain a standardized bronchoprovocation (exercise or a surrogate) challenge for the diagnosis of EIB, as the prevalence of EIB varies with the type of challenge and the conditions under which the challenge is performed. A Show 12 more 3. Medical management General principles: recognize that EIB in patients with asthma can indicate lack of control of underlying asthma. Treat uncontrolled asthma to get control of EIB. B Show 2 more Inhaled beta-2 agonists: As per AAAAI 2016 guidelines, administer inhaled short-acting -agonists for protection against EIB and for accelerating recovery of pulmonary function when given after a decrease in pulmonary function after exercise. A Show 2 more As per ATS 2013 guidelines: Administer an inhaled short-acting -agonist before exercise (typically 15 minutes before, less than daily) in patients with EIB. A Do not use daily inhaled long-acting -agonist as single therapy in patients with EIB experiencing continuous symptoms despite using an inhaled short-acting -agonist before exercise or in patients requiring an inhaled short-acting -agonist daily or more frequently. D Inhaled corticosteroids: As per AAAAI 2016 guidelines, consider administering ICSs in combination with other therapies to decrease the frequency and severity of EIB. B Show 2 more As per ATS 2013 guidelines, administer daily ICS in patients with EIB having continuous symptoms despite using an inhaled short-acting -agonist before exercise or in patients requiring an inhaled short-acting -agonist daily or more frequently. Recognize that it may take 2-4 weeks after the initiation of therapy to see maximal improvement. Do not use ICSs only before exercise. B Inhaled mast cell stabilizers: As per AAAAI 2016 guidelines: Consider administering inhaled cromolyn sodium and nedocromil sodium shortly before exercise to attenuate EIB, recognizing that it has a short duration of action and does not have bronchodilator activity. B Consider administering cromolyn sodium and nedocromil sodium alone or as added therapy with other drugs in patients with EIB. B As per ATS 2013 guidelines, administer a mast cell stabilizer before exercise in patients with EIB experiencing continuous symptoms despite using an inhaled short-acting -agonist before exercise or in patients requiring an inhaled short-acting -agonist daily or more frequently. A Inhaled anticholinergics: https://web.pathway.md/diseases/rectXVq5lHLidOgxS 2/4 6/29/23, 4:21 AM Exercise-induced bronchoconstriction Pathway As per AAAAI 2016 guidelines, consider administering inhaled ipratropium bromide in patients not responding to other agents, recognizing that its ability to attenuate EIB is inconsistent. B As per ATS 2013 guidelines, consider administering an inhaled anticholinergic before exercise in patients with EIB experiencing continuous symptoms despite using an inhaled short-acting -agonist before exercise or in patients requiring an inhaled short-acting - agonist daily or more frequently. C Leukotriene receptor antagonists: As per AAAAI 2016 guidelines: Consider administering daily leukotriene inhibitors to attenuate EIB, recognizing that they do not lead to tolerance. B Consider administering leukotriene inhibitors for intermittent or maintenance prophylaxis, recognizing that they provide incomplete protection and are not effective for reversing airway obstruction. B As per ATS 2013 guidelines, administer daily leukotriene receptor antagonist in patients with EIB experiencing continuous symptoms despite using an short-acting -agonist before exercise or in patients requiring an inhaled short-acting -agonist daily or more frequently. B Antihistamines: Consider offering antihistamines to prevent EIB in patients with EIB and allergies experiencing continuous symptoms despite using an inhaled short-acting -agonist before exercise or requiring an inhaled short-acting -agonist daily or more frequently. C Do not offer antihistamines in non-allergic patients with EIB experiencing continuous symptoms despite using an inhaled short-acting -agonist before exercise or requiring an inhaled short-acting -agonist daily or more frequently. D 4. Nonpharmacologic interventions Warm-up exercises: As per AAAAI 2016 guidelines, advise practicing pre-exercise warm-up in patients with EIB to reduce the severity of EIB. A As per ATS 2013 guidelines, advise practicing interval or combination warm-up exercise before planned exercise in all patients with EIB. B Face masks: consider advising routine use of an air warming and humidifying device (mask) during exercise in patients with EIB exercising in cold weather. C Low-salt diet: As per AAAAI 2016 guidelines, consider advising reduction of sodium intake with caution in patients with EIB. C As per ATS 2013 guidelines, consider offering a low-salt diet in patients with EIB having an interest in dietary modifications to for symptom control. C Supplements: As per AAAAI 2016 guidelines, consider offering fish oil and ascorbic acid supplementation with caution in patients with EIB. C https://web.pathway.md/diseases/rectXVq5lHLidOgxS 3/4 6/29/23, 4:21 AM Exercise-induced bronchoconstriction Pathway As per ATS 2013 guidelines: Consider offering fish oil C and ascorbic acid in patients with EIB having an interest in dietary modifications for symptom control. C Avoid offering lycopene in patients with EIB having an interest in dietary modifications for symptom control. D Clinical findings Symptoms Past medical history Chest discomfort Asthma Chest pain Respiratory exam Cough Exercise intolerance Wheezing Fatigue Shortness of breath Sputum production Social history Sports participation References 1. John M Weiler, John D Brannan, Christopher C Randolph et al. Exercise-induced bronchoconstriction update-2016. J Allergy Clin Immunol. 2016 Nov;138 5 1292 1295.e36. Open 2. Jonathan P Parsons, Teal S Hallstrand, John G Mastronarde et al. An official American Thoracic Society clinical practice guideline: exercise-induced bronchoconstriction. Am J Respir Crit Care Med. 2013 May 1;187 9 1016 27. Open https://web.pathway.md/diseases/rectXVq5lHLidOgxS 4/4 |
Guideline sources The following summarized guidelines for the evaluation and management of extrahepatic portal vein obstruction are prepared by our editorial team based on guidelines from the European Association for the Study of the Liver (EASL 2016). 1 2 3 3 3 Definition Extrahepatic portal vein obstruction is a vascular liver disorder characterized by variceal dilation, ascites, and portal hypertension. 2 Epidemiology Extrahepatic portal vein obstruction is caused by portal vein thrombosis and portal cavernoma. 3 Disease course Acute manifestations include abdominal pain, fever, nausea, postprandial fullness, anorexia, general malaise, splenomegaly, and ascites. Progression may lead to variceal bleeding, bowel infarction, perforation, peritonitis, sepsis, metabolic acidosis, renal and respiratory failure, shock, and death due to multiorgan failure. Chronic manifestations range from asymptomatic presentation to complications of portal hypertension such as variceal bleeding, thrombocytopenia, splenomegaly, jaundice. Other symptoms include transient ascites, early satiety, abdominal discomfort, intestinal ischemia, hepatic encephalopathy, and portal biliopathy (jaundice, coagulation disturbances, cholangitis, gall stones, hemobilia, and secondary biliary cirrhosis). 3 Prognosis and risk of recurrence https://web.pathway.md/diseases/recp0fBHlBM0I2wwd 1/3 6/29/23, 4:21 AM Extrahepatic portal vein obstruction Pathway Prognosis and risk of recurrence The 1-year mortality rate for liver transplantation patients with portal vein thrombosis is 15%. 3 Guidelines 1. Screening and diagnosis Clinical presentation: assess for extrahepatic portal vein obstruction in any patient presenting with features of portal hypertension, hypersplenism or abdominal pain, or biliary tract disease. A Indications for screening: consider screening for extrahepatic portal vein obstruction in patients with myeloproliferative disease and in patients with antiphospholipid syndrome. C 2. Diagnostic investigations Diagnostic imaging: obtain Doppler ultrasound as first line investigation for the diagnosis of extrahepatic portal vein obstruction. Obtain CT for diagnostic confirmation and extension assessment. A Evaluation for liver disease: exclude underlying cirrhosis or obliterative portal venopathy in patients with abnormal liver tests, dysmorphic appearance of the liver on imaging, or abnormal liver elastometry results. A 3. Medical management Anticoagulant therapy: consider permanent anticoagulation in patients with a strong prothrombotic condition, past history suggesting intestinal ischemia, or recurrent thrombosis on follow-up. C Management of prothrombotic conditions: manage any underlying prothrombotic condition(s) according to established guidelines. B Management of portal hypertension: treat portal hypertension according to established guidelines for cirrhosis. B Clinical findings Symptoms Past medical history Abdominal pain Antiphospholipid syndrome Diarrhea Gastroesophageal varices Fever Hyperhomocysteinemia Liver cirrhosis Medication history Myeloproliferative disease Hormonal contraceptives Past obstetric history https://web.pathway.md/diseases/recp0fBHlBM0I2wwd 2/3 6/29/23, 4:21 AM Extrahepatic portal vein obstruction Pathway Pregnancy Abdominal exam Rectal exam Abdominal distension Ascites Rectal blood Hepatomegaly Splenomegaly Hematological findings Lab findings Pancytopenia Lactic acidosis liver enzymes Imaging findings Intestinal congestion References 1. European Association for the Study of the Liver. EASL Clinical Practice Guidelines: Vascular diseases of the liver. J Hepatol. 2016 Jan;64 1 179 202. Open 2. Zeeshan A. Wani, Riyaz A. Bhat, Ajeet S. Bhadoria et al. Extrahepatic Portal Vein Obstruction and Portal Vein Thrombosis in Special Situations: Need for a New Classification. Saudi J Gastroenterol. 2015 May-Jun; 21 3 129 138. Open 3. A Kumar, P Sharma, A Arora. Review article: portal vein obstruction epidemiology, pathogenesis, natural history, prognosis and treatment. Aliment Pharmacol Ther. 2015 Feb;41 3 276 92. Open 4. Italian Association for the Study of the Liver AISF , Italian Association for the Study of the Liver AISF. AISF position paper on liver disease and pregnancy. Dig Liver Dis. 2016 Feb;48 2 120 37. Open https://web.pathway.md/diseases/recp0fBHlBM0I2wwd 3/3 |
Guideline sources The following summarized guidelines for the evaluation and management of extrapulmonary tuberculosis are prepared by our editorial team based on guidelines from the European Bone and Joint Infection Society (EBJIS 2023), the U.S. Preventive Services Task Force (USPSTF 2023), the French Society of Infectious Diseases (SPLIF 2023), the Canadian Thoracic Society (CTS 2022), the World Health Organization (WHO 2022; 2021; 2020; 2017), the Neurocritical Care Society (NCS 2020), the European Association of Nuclear Medicine (EANM/ESNR/ESCMID 2019), the Infectious Diseases Society of America (IDSA/ATS/CDC 2017; 2016), the European Society of Cardiology (ESC/EACTS 2015), the Infectious Diseases Society of America (IDSA 2015; 2008), and the British Thoracic Society (BTS 2010). 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 Guidelines 1. Screening and diagnosis Screening for latent tuberculosis: As per USPSTF 2023 guidelines, obtain screening for latent tuberculosis infection in populations at increased risk. B https://web.pathway.md/diseases/recSbGzA8a48XKtDL 1/11 6/29/23, 4:21 AM Extrapulmonary tuberculosis Pathway As per WHO 2020 guidelines, obtain systematic testing for latent tuberculosis infection in patients initiating anti-TNF treatment, receiving dialysis, preparing for an organ or hematological transplant, or having silicosis. E Show 3 more As per ATS 2017 guidelines, obtain an IGRA rather than a tuberculin skin test in 5 years old patients meeting the following criteria: likely to be infected with Mycobacterium tuberculosis low or intermediate risk of disease progression it has been decided that testing for latent tuberculosis infection is warranted history of BCG vaccination or it is expected that the individual is unlikely to return to have the TST read. A Show 4 more 2. Diagnostic investigations Specimen collection (general principles): make every effort to collect clinical samples for both mycobacteriologic (AFB smear, culture, NAAT) and histopathologic tests. E Specimen collection, bone and joint: As per EBJIS 2023 guidelines, collect synovial fluid for mycobacteriologic analysis (AFB stain, mycobacterial culture, and NAAT) in patients with suspected tuberculous arthritis, such as a subacute or chronic course of arthritis (weeks to months or even years), especially in patients living in or previously living in endemic areas or with a prior history of tuberculosis. B As per SPLIF 2023 guidelines, collect less invasive samples (respiratory or lymph node) preliminary before considering disco-vertebral biopsy in patients with suspected spinal tuberculosis. E Fluid specimen analysis (general principles): consider obtaining an analysis for counts and chemistries on amenable fluid specimens collected from sites of suspected extrapulmonary tuberculosis. C Fluid specimen analysis (pleural fluid): obtain an analysis for cell count and differential, protein, glucose, pH, LDH, and cytology on pleural effusion/pleural fluid in patients with suspected tuberculosis. E Fluid biomarkers: As per ATS 2017 guidelines: Consider obtaining adenosine deaminase level measurement on fluid collected from patients with suspected pleural, meningeal, peritoneal, or pericardial tuberculosis. C Consider obtaining free interferon-gamma level measurement on fluid collected from patients with suspected pleural or peritoneal tuberculosis. C As per CTS 2010 guidelines, consider obtaining surrogate markers (such as adenosine deaminase) as rule-out tests for pleural tuberculosis in low-incidence countries. C Acid-fast bacilli smear (general principles): consider obtaining AFB smear microscopy on specimens collected from sites of suspected extrapulmonary tuberculosis. C https://web.pathway.md/diseases/recSbGzA8a48XKtDL 2/11 6/29/23, 4:21 AM Extrapulmonary tuberculosis Pathway Acid-fast bacilli smear (pleural fluid): obtain AFB smear on pleural effusion/pleural fluid in patients with suspected pleural tuberculosis. E Acid-fast bacilli smear (synovial fluid): obtain AFB smear on synovial fluid specimens in patients with suspected tuberculous arthritis. B Mycobacterial culture, general principles: As per CTS 2022 guidelines, prioritize obtaining mycobacterial culture if the specimen is insufficient for all testing, given it has the highest diagnostic yield and allows for gold- standard phenotypic drug testing. E As per CDC/IDSA/ATS 2017 guidelines: Obtain mycobacterial cultures on specimens collected from sites of suspected extrapulmonary tuberculosis. B Submit one culture isolate from each mycobacterial culture-positive patient to a regional genotyping laboratory for genotyping. B Mycobacterial culture (pleural fluid): obtain Gram stain and culture on pleural effusion/pleural fluid in patients with suspected pleural tuberculous. E Mycobacterial culture (synovial fluid): obtain mycobacterial culture on synovial fluid specimens in patients with suspected tuberculous arthritis. B Nucleic acid amplification testing: As per EBJIS 2023 guidelines, obtain an NAAT on synovial fluid specimens in patients with suspected tuberculous arthritis. B As per WHO 2021 guidelines, consider obtaining Xpert MTB/RIF in lymph node aspirate, C lymph node biopsy, C pleural fluid, C peritoneal fluid, C pericardial fluid, C synovial fluid, C or urine specimens C rather than smear microscopy/culture as an initial diagnostic test in adult and pediatric patients with signs and symptoms of extrapulmonary tuberculosis. C Show 3 more As per ATS 2017 guidelines, consider obtaining an NAAT on specimens collected from sites of suspected extrapulmonary tuberculosis. C Blood/skin tests: As per EANM 2019 guidelines, obtain a purified protein derivative skin test and an IGRA in patients with suspected spondylodiscitis and risk factors for Mycobacterium tuberculosis. B As per IDSA 2015 guidelines, consider obtaining a purified protein derivative skin test or an IGRA in patients with subacute native vertebral osteomyelitis at risk for Mycobacterium tuberculosis (originating or residing in endemic regions or having risk factors). C Testing for pulmonary tuberculosis: test for pulmonary tuberculosis to assess infectiousness and potentially assist with diagnosis in patients with presumed extrapulmonary tuberculosis. E 3. Diagnostic procedures https://web.pathway.md/diseases/recSbGzA8a48XKtDL 3/11 6/29/23, 4:21 AM Extrapulmonary tuberculosis Pathway Tissue biopsy (general principles): perform a histological examination of specimens collected from sites of suspected extrapulmonary tuberculosis. B Tissue biopsy (pleural biopsy): Send pleural biopsies for both histological examination and culture to improve the diagnostic sensitivity for tuberculosis. B Prefer thoracoscopic pleural biopsies to yield positive mycobacterial culture (and therefore drug sensitivity) results. B Tissue biopsy (synovial biopsy): perform a synovial biopsy for culture and histopathological analysis in patients with suspected tuberculous arthritis. B 4. Medical management Setting of care: As per WHO 2017 guidelines, consider offering the following treatment administration options in patients on tuberculosis treatment: community- or home-based directly observed treatment over health facility-based directly observed treatment or unsupervised treatment C directly observed treatment administered by trained lay providers or health-care workers over directly observed treatment administered by family members or unsupervised treatment C video-observed treatment over directly observed treatment when the video communication technology is available and can be appropriately organized and operated by healthcare providers and patients. C As per ATS 2016 guidelines, consider delivering directly observed treatment rather than self- administered therapy for routine treatment of patients with all forms of tuberculosis. C Management of tuberculous lymphadenitis (antimycobacterial therapy): consider administering standard anti-tuberculosis therapy for 6 months in patients with treating drug- susceptible tuberculous lymphadenitis. C Management of tuberculous lymphadenitis (surgery/drainage): perform surgical/drainage procedures for the relief of discomfort caused by enlarged nodes or tense, fluctuant nodes in patients with tuberculous lymphadenitis. E Management of pleural tuberculosis (antimycobacterial therapy): consider administering standard anti-tuberculosis therapy for 6 months in patients with drug-susceptible pleural tuberculosis. C Management of pleural tuberculosis (adjunctive corticosteroids): avoid administering routine adjunctive corticosteroids in patients with pleural tuberculosis. D Management of pleural tuberculosis (thoracentesis): Avoid performing routine therapeutic thoracentesis/chest tube drainage for pleural tuberculosis-associated effusions. D Consider performing therapeutic thoracentesis on a case-by-case basis to relieve symptoms of significant dyspnea. E https://web.pathway.md/diseases/recSbGzA8a48XKtDL 4/11 6/29/23, 4:21 AM Extrapulmonary tuberculosis Pathway Management of tuberculous pericarditis, antimycobacterial therapy: As per CTS 2022 guidelines, consider administering standard anti-tuberculosis therapy for 6 months in patients with drug-susceptible tuberculosis pericarditis. C As per EACTS/ESC 2015 guidelines, initiate empiric anti-tuberculosis therapy for exudative pericardial effusion in patients living in endemic areas, after excluding other causes. B Show 2 more Management of tuberculous pericarditis, adjunctive corticosteroids: As per CTS 2022 guidelines, consider administering initial adjunctive corticosteroids in all human immunodeficiency virus-negative patients with tuberculosis pericarditis. C Show 2 more As per WHO 2017 guidelines, consider administering initial adjuvant corticosteroids in patients with tuberculous pericarditis. C As per CDC/IDSA/ATS 2016 guidelines, do not administer routine initial adjunctive corticosteroids in patients with tuberculous pericarditis. D As per EACTS/ESC 2015 guidelines: Consider administering adjunctive corticosteroids in human immunodeficiency virus- negative patients with tuberculous pericarditis. C Avoid administering corticosteroids in human immunodeficiency virus-positive patients with tuberculous pericarditis. D Management of tuberculous pericarditis (intrapericardial urokinase): consider administering intrapericardial urokinase to reduce the risk of constriction in patients with effusive tuberculous pericarditis. C Management of tuberculous pericarditis, pericardiectomy: As per CTS 2022 guidelines, consider performing early pericardiectomy in patients with recurrent pericardial effusions or persistently elevated central venous pressures despite the removal of pericardial fluid and the use of anti-tuberculosis drugs. E As per EACTS/ESC 2015 guidelines, perform pericardiectomy if the patient is not improving or is deteriorating after 4-8 weeks of anti-tuberculosis therapy. B Management of tuberculous meningitis, antimycobacterial therapy: As per CTS 2022 guidelines, consider initiating empiric therapy immediately in patients with high suspicion of tuberculosis meningitis while awaiting diagnostic results. E Show 3 more As per IDSA 2008 guidelines, initiate 4-drug antituberculous therapy in patients with tuberculous meningitis. B Management of tuberculous meningitis, adjunctive corticosteroids: As per CTS 2022 guidelines, administer a course of corticosteroids guided by disease severity in all patients presenting with tuberculosis meningitis. B As per NCS 2020 guidelines, administer corticosteroids to reduce mortality in patients with tuberculosis meningitis. B Consider continuing corticosteroids for 2 two weeks. B As per WHO 2017 guidelines, administer initial adjunctive corticosteroids with dexamethasone or prednisolone tapered over 6-8 weeks in patients with tuberculous https://web.pathway.md/diseases/recSbGzA8a48XKtDL 5/11 6/29/23, 4:21 AM Extrapulmonary tuberculosis Pathway meningitis. B As per IDSA 2008 guidelines, administer adjunctive dexamethasone in patients with tuberculous. meningitis. B Management of abdominal tuberculosis: consider administering standard anti-tuberculosis therapy for 6 months in patients with drug-susceptible abdominal tuberculosis. C Management of genitourinary tuberculosis: Consider administering standard anti-tuberculosis therapy for 6 months in patients with drug- susceptible genitourinary tuberculosis. C Consider administering isoniazid and rifampin for a minimum of 9 months in patients with BCG disease, given inherent pyrazinamide resistance. C Management of bone and joint tuberculosis, antimycobacterial therapy: As per EBJIS 2023 guidelines, initiate anti-tuberculosis therapy alone in patients with early tuberculosis arthritis. B Show 3 more As per CTS 2022 guidelines, consider administering standard anti-tuberculosis therapy for 6 months in patients with drug-susceptible bone and joint tuberculosis with extension to 9-12 months in patients with markers of increased risk of failure/relapse; a diagnostic biopsy sample smear positive for AFB and/or elevated ESR/CRP at the planned end of treatment. C Management of bone and joint tuberculosis, surgery: As per EBJIS 2023 guidelines: Avoid performing surgical intervention in the active phase of tuberculosis arthritis. Consider performing debridement and synovectomy only in exceptional cases with large abscesses, significantly devitalized bone, or showing inadequate response to medical management. D Consider performing surgical management with excisional arthroplasty or arthrodesis in patients with substantial joint destruction, ankylosis, deformity, significant loss of function, or chronic pain after tuberculosis arthritis. B As per CTS 2022 guidelines: Avoid performing routine surgical intervention as part of treatment for spinal tuberculosis. Consider performing surgery for spinal tuberculosis in patients with progressive neurologic deterioration and in < 15 years old patients with significant kyphosis. D Avoid performing surgical intervention as part of the treatment of joint tuberculosis. Consider performing surgery to prevent the extension of disease and to provide relief of pain and immobility after control of infection is established. D Management of ocular tuberculosis: consider administering standard anti-tuberculosis therapy for 6 months in patients with drug-susceptible (suspected or confirmed) intraocular tuberculosis. C Management of skin tuberculosis: consider administering standard anti-tuberculosis therapy for 6 months in patients with drug-susceptible tuberculosis of the skin, including tuberculids. C Management of disseminated tuberculosis: https://web.pathway.md/diseases/recSbGzA8a48XKtDL 6/11 6/29/23, 4:21 AM Extrapulmonary tuberculosis Pathway Consider initiating empiric anti-tuberculosis treatment in severely ill or significant immunocompromised patients while waiting for mycobacterial culture confirmation. E Consider administering standard anti-tuberculosis therapy for 6 months in patients with drug- susceptible disseminated tuberculosis without CNS involvement. Consider extending treatment to 9-12 months in immunocompromised patients if the predisposing condition is not modified (such as patients with human immunodeficiency virus not receiving antiretroviral therapy or continuation of immunosuppressive therapy). C Management of tuberculoma (antimycobacterial therapy): consider administering standard anti-tuberculosis therapy for 9-12 months in patients with drug-susceptible tuberculoma and spinal arachnoiditis because of the severity of the disease and the risk of morbidity with inadequate treatment. E Management of tuberculoma (adjunctive corticosteroids): avoid administering adjunctive corticosteroids routinely in patients with CNS tuberculoma without meningitis. D 5. Specific circumstances Patients with HIV infection: As per WHO 2022 guidelines, initiate antiretroviral therapy as early as possible (within the first 8 weeks) following the initiation of antituberculosis treatment in all patients with human immunodeficiency virus infection and drug-resistant tuberculosis requiring second-line antituberculosis drugs, irrespective of the CD4 cell count. B As per WHO 2017 guidelines, initiate antiretroviral therapy in all patients with tuberculosis and human immunodeficiency virus infection, regardless of the CD4 cell count. Initiate tuberculosis treatment first, followed by antiretroviral therapy as soon as possible within the first 8 weeks of treatment. A . Initiate antiretroviral therapy within the first 2 weeks of initiating tuberculosis treatment in human immunodeficiency virus infection-positive patients with profound immunosuppression, such as CD4 counts < 50 cells/mm . As per ATS 2016 guidelines, initiate antiretroviral therapy during tuberculosis treatment in patients coinfected with human immunodeficiency virus, ideally starting within the first 2 weeks of tuberculosis treatment in patients with CD4 counts < 50 cells/ L and by 8-12 weeks of tuberculosis treatment in patients with CD4 counts 50 cells/ L. A 6. Patient education Patient support: As per WHO 2017 guidelines, provide health education and counseling on the disease and treatment adherence in patients receiving tuberculosis treatment. B Show 2 more As per ATS 2016 guidelines, consider offering case management interventions (such as patient education/counseling, field/home visits, integration/coordination of care with specialists and medical home, patient reminders, and incentives/enablers) in addition to curative interventions in patients with tuberculosis. C https://web.pathway.md/diseases/recSbGzA8a48XKtDL 7/11 6/29/23, 4:21 AM Extrapulmonary tuberculosis Pathway Clinical findings Symptoms Past medical history Abdominal pain BCG therapy Agitation Human immunodeficiency virus infection Amenorrhea Immunocompromising condition Back pain Pulmonary tuberculosis Back stiffness Blurred vision Cardiac exam Bone pain Chest pain Pericardial friction rub Constipation Abdominal exam Cough Diarrhea Abdominal distension Dysphagia Ascites Dysuria Hepatomegaly Epigastric pain Palpable abdominal mass Fever Splenomegaly Flank pain Floaters and flashes Genitourinary exam Headache Scrotal mass Hematuria Hoarseness Lymphatic exam Limb weakness Axillary lymphadenopathy Loss of appetite Cervical lymphadenopathy Malaise Inguinal lymphadenopathy Menstrual irregularity Submandibular lymphadenopathy Muscle spasms Nausea Supraclavicular lymphadenopathy Night sweats Nocturia Odynophagia Imaging findings Personality or behavioral changes Abdominal lymphadenopathy Photophobia Cardiomegaly Pleuritic chest pain Pericardial effusion RUQ pain Pericardial thickening Seizure Pleural effusion Shortness of breath Suprapubic pain Urinary frequency Urinary urgency Vaginal bleeding Vaginal discharge Vision loss Vomiting https://web.pathway.md/diseases/recSbGzA8a48XKtDL 8/11 6/29/23, 4:21 AM Extrapulmonary tuberculosis Pathway Weight loss Neurological exam Altered mental status Cauda equina syndrome Focal neurologic deficits Limb paresis Meningismus Respiratory exam Dullness to percussion Pleural friction rub Musculoskeletal exam Kyphosis Muscle atrophy Skeletal deformity spinal ROM Gynecologic exam Adnexal mass Uterine enlargement Integument exam Draining sinus tract Jaundice Studies 2022 SHINE In children with non-severe, symptomatic, presumed drug-susceptible tuberculosis, 4 months treatment was noninferior to 6 months treatment with respect to treatment failure or disease recurrence at 72 weeks. Anna Turkova et al. N Engl J Med. 2022 Mar 10. 2018 Rifampin in latent TB In patients with latent tuberculosis infection, 4-month regimen of rifampin, as compared with 9- month regimen of isoniazid, was not inferior for the prevention of active tuberculosis. In rifampin group, among 3443 patients, confirmed active tuberculosis and clinically diagnosed active tuberculosis developed in 4 and 4 patients, respectively, during 7732 person-years of follow-up, as compared with 4 and 5 patients, respectively, among 3416 patients in the isoniazid group during 7652 person-years of follow-up. The rate differences (rifampin minus isoniazid) for confirmed active tuberculosis and for confirmed or clinically diagnosed tuberculosis were < 0.01 cases per 100 person-years (95% CI -0.14 to 0.16) and < 0.01 cases per 100 person-years https://web.pathway.md/diseases/recSbGzA8a48XKtDL 9/11 6/29/23, 4:21 AM Extrapulmonary tuberculosis Pathway (95% CI -0.23 to 0.22), respectively. The upper boundaries of the 95% CI for the rate differences of the confirmed cases and for the confirmed or clinically diagnosed cases of tuberculosis were less than the prespecified noninferiority margin of 0.75% in cumulative incidence. The difference in the treatment-completion rates was 15.1% (95% CI 12.7-17.4). The rate differences for adverse events of grade 3-5 occurring within 146 days (120% of the 4-month planned duration of the rifampin regimen) for all events and hepatotoxic events were -1.1% (95% CI -1.9 to -0.4) and -1.2% (95% CI -1.7 to -0.7), respectively. Menzies D et al. N Engl J Med. 2018 Aug 2. Show 1 more References 1. Tunkel AR, Glaser CA, Bloch KC et al. The management of encephalitis: clinical practice guidelines by the Infectious Diseases Society of America. Clin Infect Dis. 2008 Aug 1;47 3 303 27. Open 2. Christen Ravn, Jeroen Neyt, Natividad Benito et al. Guideline for management of septic arthritis in native joints SANJO . J Bone Jt Infect. 2023 Jan 12;8 1 29 37. Open 3. Leila Barss, William J. A. Connors & Dina Fisher. Chapter 7 Extra-pulmonary tuberculosis. CTS. 2022 Mar 25;. Open 4. Elena Lazzeri, Alessandro Bozzao, Maria Adriana Cataldo et al. Joint EANM/ESNR and ESCMID- endorsed consensus document for the diagnosis of spine infection (spondylodiscitis) in adults. Eur J Nucl Med Mol Imaging. 2019 Nov;46 12 2464 2487. Open 5. Payam Nahid, Susan E Dorman, Narges Alipanah et al. Official American Thoracic Society/Centers for Disease Control and Prevention/Infectious Diseases Society of America Clinical Practice Guidelines: Treatment of Drug-Susceptible Tuberculosis. Clin Infect Dis. 2016 Oct 1;63 7):e147-e195. Open 6. Adler Y, Charron P, Imazio M et al. 2015 ESC Guidelines for the diagnosis and management of pericardial diseases: The Task Force for the Diagnosis and Management of Pericardial Diseases of the European Society of Cardiology ESC Endorsed by: The European Association for Cardio-Thoracic Surgery EACTS . Eur Heart J. 2015 Nov 7;36 42 2921 2964. Open 7. No authors listed. Guidelines for treatment of drug-susceptible tuberculosis and patient care. Geneva: World Health Organization; 2017. Open 8. David M Lewinsohn, Michael K Leonard, Philip A LoBue et al. Official American Thoracic Society/Infectious Diseases Society of America/Centers for Disease Control and Prevention Clinical Practice Guidelines: Diagnosis of Tuberculosis in Adults and Children. Clin Infect Dis. 2017 Jan 15;64 2 111 115. Open 9. US Preventive Services Task Force, Carol M Mangione, Michael J Barry et al. Screening for Latent Tuberculosis Infection in Adults: US Preventive Services Task Force Recommendation Statement. JAMA. 2023 May 2;329 17 1487 1494. Open 10. No authors listed. WHO consolidated guidelines on tuberculosis: module 1 prevention: tuberculosis preventive treatment. Geneva: World Health Organization; 2020. Open 11. Berbari EF, Kanj SS, Kowalski TJ et al. 2015 Infectious Diseases Society of America IDSA Clinical Practice Guidelines for the Diagnosis and Treatment of Native Vertebral Osteomyelitis in Adults. Clin Infect Dis. 2015 Sep 15;61 6):e26 46. Open https://web.pathway.md/diseases/recSbGzA8a48XKtDL 10/11 6/29/23, 4:21 AM Extrapulmonary tuberculosis Pathway 12. Hooper C, Lee YC, Maskell N et al. Investigation of a unilateral pleural effusion in adults: British Thoracic Society Pleural Disease Guideline 2010. Thorax. 2010 Aug;65 Suppl 2:ii4 17. Open 13. No authors listed. WHO consolidated guidelines on tuberculosis: module 3 diagnosis: rapid diagnostics for tuberculosis detection, 2021 update. Geneva: World Health Organization; 2021. Open 14. Aaron M Cook, G Morgan Jones, Gregory W J Hawryluk et al. Guidelines for the Acute Treatment of Cerebral Edema in Neurocritical Care Patients. Neurocrit Care. 2020 Jun;32 3 647 666. Open 15. Mantelas M, Katsiki N, Antonitsis P et al. 2022 SPILF Clinical Practice Guidelines for the Diagnosis and Treatment of Disco-Vertebral Infection in Adults. Infect Dis Now. 2023 Jan 20;S2666 9919 23 00009 X. Open 16. No authors listed. WHO consolidated guidelines on tuberculosis. Module 4 treatment - drug- resistant tuberculosis treatment, 2022 update. Geneva: World Health Organization; 2022. Open https://web.pathway.md/diseases/recSbGzA8a48XKtDL 11/11 |
Guideline sources The following summarized guidelines for the evaluation and management of facioscapulohumeral muscular dystrophy are prepared by our editorial team based on guidelines from the American College of Chest Physicians (ACCP 2023), the Heart Rhythm Society (HRS 2022), the American Association of Neuromuscular and Electrodiagnostic Medicine (AANEM/AAN 2015), and the European Federation of Neurological Societies (EFNS 2011). 1 2 3 4 5 5 5 5 Definition Facioscapulohumeral muscular dystrophy is an autosomal dominant inherited disorder characterized by asymmetric and progressive weakness of the muscles of the face, scapular, and humeral muscles, followed by weakness of distal lower extremities and pelvic girdle. 5 Epidemiology Facioscapulohumeral muscular dystrophy is caused by a genetic abnormality with contraction of a part of a repeated sequence in the D4Z4 region on chromosome 4q35 and mutations in gene SMCHD1. 5 Disease course Clinical manifestations of facioscapulohumeral muscular dystrophy include progressive weakness of the facial, shoulder arm muscles, foot drop, asymmetrical abdominal weakness https://web.pathway.md/diseases/recgxQZ5z8X0KSiPX 1/4 6/29/23, 4:00 AM Facioscapulohumeral muscular dystrophy Pathway (Beevor's sign), paraspinal muscle weakness, bent-spine, limb-girdle patterns, asymptomatic atrial arrhythmias, hearing loss, retinal vascular changes, and respiratory involvement that leads to patients wheelchair-bound. 5 Prognosis and risk of recurrence Facioscapulohumeral muscular dystrophy is not associated with increased mortality. 5 Guidelines 1. Classification and risk stratification Severity prediction: recognize that patients with large D4Z4 deletion sizes (contracted D4Z4 allele of 10-20 kb) are more likely to develop a more significant disability, and at an earlier age, and symptomatic extramuscular manifestations. B 2. Diagnostic investigations Genetic testing: As per AAN 2015 guidelines, obtain genetic confirmation of facioscapulohumeral muscular dystrophy type 1 in patients with atypical presentations and no first-degree relatives with genetic confirmation of the disease. B As per EFNS 2011 guidelines, diagnose facioscapulohumeral dystrophy with molecular testing of respective genes without additional investigations in patients with the typical presentation and suggestive family history (a male patient with muscular dystrophy with an uncle having a similar phenotype). B Respiratory evaluation: As per ACCP 2023 guidelines, obtain pulmonary function testing to assist with management decisions in patients with neuromuscular disease at risk for respiratory complications. E Show 3 more As per AAN 2015 guidelines, obtain baseline pulmonary function testing in all patients with facioscapulohumeral muscular dystrophy. Monitor patients regularly if they have abnormal baseline pulmonary function test results or any combination of severe proximal weakness, kyphoscoliosis, wheelchair dependence, or comorbid conditions affecting ventilation. B Cardiac evaluation: As per HRS 2022 guidelines, consider obtaining cardiac evaluation, including physical examination, ECG, ambulatory ECG, and cardiac imaging (echocardiography or cardiac MRI), at diagnosis with periodic retesting in patients with facioscapulohumeral muscular dystrophy, even in the absence of cardiac symptoms. C As per AAN 2015 guidelines, avoid obtaining routine screening cardiac tests without signs or symptoms of cardiac disease. Refer patients for cardiac evaluation if they develop overt signs or symptoms of cardiac disease. D Hearing evaluation: obtain screening for hearing loss in all young pediatric patients with facioscapulohumeral muscular dystrophy at diagnosis, and yearly thereafter until patients start https://web.pathway.md/diseases/recgxQZ5z8X0KSiPX 2/4 6/29/23, 4:00 AM Facioscapulohumeral muscular dystrophy Pathway school, as hearing loss may not be present at diagnosis and can be progressive. B Retinal evaluation: refer patients with facioscapulohumeral muscular dystrophy and large deletions (contracted D4Z4 allele of 10-20 kb) to an experienced ophthalmologist for dilated indirect fundoscopy. Use the presence and severity of retinal vascular disease at the initial screening to determine the frequency of subsequent monitoring. B 3. Respiratory support Airway clearance: consider offering glossopharyngeal breathing for lung volume recruitment and airway clearance in patients with hypoventilation. C Show 4 more Noninvasive ventilation: initiate noninvasive ventilation in patients with chronic respiratory failure. B Show 4 more Invasive ventilation: consider offering invasive home mechanical ventilation via tracheostomy as an alternative to noninvasive ventilation in patients failing or intolerant of noninvasive ventilation (including extended daytime noninvasive ventilation use), worsening bulbar function, frequent aspiration, insufficient cough, episodes of chest infection despite adequate secretion management, or declining lung function. C 4. Medical management Management of strength: avoid using albuterol, corticosteroids, or diltiazem to improve strength in patients with facioscapulohumeral muscular dystrophy. D Management of pain: inquire about pain routinely in patients with facioscapulohumeral muscular dystrophy. B Show 2 more 5. Nonpharmacologic interventions Aerobic exercises: consider encouraging patients with facioscapulohumeral muscular dystrophy to engage in low-intensity aerobic exercise programs, guided by experienced physical therapists. C 6. Surgical interventions Scapular fixation: consider performing surgical scapular fixation cautiously in selected patients after careful consideration of the overall muscle impairment in the involved arm, assessment of potential gain in the ROM by manual fixation of the scapula, the patient's rate of disease progression, and the potential adverse consequences of surgery and prolonged post- surgical bracing. C https://web.pathway.md/diseases/recgxQZ5z8X0KSiPX 3/4 6/29/23, 4:00 AM Facioscapulohumeral muscular dystrophy Pathway Clinical findings Symptoms Past medical history Facial weakness Abdominal muscle weakness Loss of balance Ocular exam Lower leg weakness Myalgia Retinal abnormalities Neurological exam Musculoskeletal exam Beevor's sign Asymmetrical limb weakness Impaired balance on one foot Foot drop Inability to wALK on heels Hip weakness Lordosis Head and neck exam Quadriceps atrophy Hearing loss Shoulder weakness Winging of the scapula Genetic testing Shortening of the repeated DNA elements within the 4q35 region of chromosome 4 References 1. J M Burgunder, L Sch ls, J Baets et al. EFNS guidelines for the molecular diagnosis of neurogenetic disorders: motoneuron, peripheral nerve and muscle disorders. Eur J Neurol. 2011 Feb;18 2 207 217. Open 2. Tawil R, Kissel JT, Heatwole C et al. Evidence-based guideline summary: Evaluation, diagnosis, and management of facioscapulohumeral muscular dystrophy: Report of the Guideline Development, Dissemination, and Implementation Subcommittee of the American Academy of Neurology and the Practice Issues Review Panel of the American Association of Neuromuscular & Electrodiagnostic Medicine. Neurology. 2015 Jul 28;85 4 357 64. Open 3. Akram Khan, Lindsy Frazer-Green, Reshma Amin et al. Respiratory Management of Patients with Neuromuscular Weakness: An American College of Chest Physicians Clinical Practice Guideline and Expert Panel Report. Chest. 2023 Mar 13;S0012 3692 23 00353 7. Open 4. William J Groh, Deepak Bhakta, Gordon F Tomaselli et al. 2022 HRS expert consensus statement on evaluation and management of arrhythmic risk in neuromuscular disorders. Heart Rhythm. 2022 Oct;19 10):e61-e120. Open 5. Jeffrey Statland, Rabi Tawil. Facioscapulohumeral muscular dystrophy. 2014 Aug;32 3 721 8, ix.2014 Aug;32 3 721 8, ix. Open 6. Jeffrey M. Statland, Rabi Tawil. Facioscapulohumeral Muscular Dystrophy. Continuum Minneap Minn). 2016 Dec 5; 22 6 1916 1931. Open 7. Jeffrey M. Statland, Rabi Tawil. Facioscapulohumeral Muscular Dystrophy. Neurol Clin. 2014 Aug; 32 3 721 ix. Open https://web.pathway.md/diseases/recgxQZ5z8X0KSiPX 4/4 |
Guideline sources The following summarized guidelines for the evaluation and management of factor V Leiden are prepared by our editorial team based on guidelines from the American Society of Hematology (ASH 2023; 2018), the Pathway (Pathway 2023), the British Society for Haematology (BSH 2022), the International Federation of Gynecology and Obstetrics (FIGO 2021), the European Society for Vascular Surgery (ESVS 2021; 2017), the American Association for the Study of Liver Diseases (AASLD 2021), the American College of Gastroenterology (ACG 2020), the European Stroke Organisation (ESO 2017), the Anticoagulation Forum (AF 2016), the European Society for Trauma and Emergency Surgery (ESTES 2016), the European Association for the Study of the Liver (EASL 2016), the Society of Obstetricians and Gynaecologists of Canada (SOGC 2014), the Society for Vascular Surgery (SVS 2014), the Central European Vascular Forum (CEVF/IUA/IUP 2012), the British Committee for Standards In Haematology (BCSH 2012), the American College of Chest Physicians (ACCP 2012), and the American Heart Association (AHA/ASA 2011). 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 Calculator Calculator Calculat Caprini Score for venous thromb Geneva Risk Score for venous th IMPROV Guidelines https://web.pathway.md/diseases/rec908OQ43qB7Tngk 1/7 6/29/23, 4:01 AM Factor V Leiden Pathway 1. Screening and diagnosis Indications for screening, general principles: As per BSH 2022 guidelines, do not obtain routine thrombophilia testing in first-degree relatives of patients with a history of VTE. D As per AF 2016 guidelines, do not obtain testing for thrombophilia in asymptomatic family members of patients with VTE or hereditary thrombophilia, including when contemplating pregnancy or estrogen therapy. D Indications for screening (minor provoking risk factors): avoid obtaining testing for factor V Leiden to guide thromboprophylaxis in patients with a family history of factor V Leiden (with or without VTE) and having a minor provoking risk factor for VTE, such as immobility or minor injury, illness, or infection. D Indications for screening (before combined oral contraceptives): do not obtain thrombophilia testing to guide the use of combined oral contraceptives in females in the general population. D Show 2 more Indications for screening (before hormonal therapy): avoid obtaining thrombophilia testing to guide the use of hormone-replacement therapy in patients from the general population. D Show 2 more Indications for screening (pregnancy): consider obtaining testing for the known familial thrombophilia in females with a family history of VTE and known homozygous factor V Leiden or a combination of factor V Leiden and prothrombin G20210A in the family. Consider administering antepartum thromboprophylaxis in patients with the same familial thrombophilia. C Show 2 more Indications for testing, first episode of thrombosis: As per BSH 2022 guidelines: Do not obtain genetic testing for FVL mutation to predict a first episode of venous thrombosis. D Do not obtain routine testing for heritable thrombophilic traits after a venous thrombotic event to guide management decisions. D As per AF 2016 guidelines, do not obtain thrombophilia testing following an episode of provoked or unprovoked VTE. D Show 2 more Indications for testing (deep vein thrombosis): do not obtain thrombophilia testing in patients with provoked deep vein thrombosis. D Show 2 more Indications for testing, cerebral vein thrombosis: As per ESO 2017 guidelines: Avoid obtaining thrombophilia testing in patients with cerebral venous thrombosis to reduce death, improve functional outcomes, or prevent recurrent venous thrombosis. D Consider obtaining thrombophilia testing in patients with a high pre-test probability of carrying severe thrombophilia (a personal and/or family history of venous thrombosis, https://web.pathway.md/diseases/rec908OQ43qB7Tngk 2/7 6/29/23, 4:01 AM Factor V Leiden Pathway young age at cerebral venous thrombosis, cerebral venous thrombosis without a transient or permanent risk factor) to prevent recurrent venous thrombotic events. E As per AHA/ASA 2011 guidelines: Consider obtaining testing for prothrombotic conditions, including FVL mutation, in patients with cerebral venous thrombosis. C Consider obtaining thrombophilia testing to identify underlying coagulation defects in pediatric patients with cerebral venous thrombosis. C Indications for testing, mesenteric vein thrombosis: As per ESVS 2017 guidelines, consider obtaining genetic testing for thrombophilia in patients with mesenteric vein thrombosis. C As per ESTES 2016 guidelines, obtain testing for thrombophilia in patients with mesenteric vein thrombosis. B Indications for testing (portal vein thrombosis): obtain testing for inherited thrombophilias, including FVL mutation, in patients with portal vein thrombosis. A Indications for testing, Budd-Chiari syndrome: As per AASLD 2021 guidelines, obtain a full thrombophilia workup at the time of hepatic vein thrombosis/Budd-Chiari syndrome diagnosis. Evaluate additional factors even when one causal factor is identified, and consult with a hematologist. E As per EASL 2016 guidelines, obtain testing for inherited thrombophilias, including FVL mutation, in patients with Budd-Chari syndrome. A Indications for testing, superficial vein thrombosis: As per BCSH 2012 guidelines, do not obtain routine testing for underlying thrombophilia in patients with superficial venous thrombosis. D As per CEVF/IUP/IUA 2012 guidelines, obtain testing for thrombophilia in patients with spontaneous nonvaricose superficial venous thrombosis or recurrent varicose superficial venous thrombosis. E Indications for testing (unusual site venous thrombosis): do not obtain testing for inherited thrombophilias in patients with thrombosis at an unusual site. D Indications for testing (recurrent thrombosis): consider obtaining thrombophilia testing in patients with a history of recurrent venous thrombosis and chronic recurrent venous leg ulcers. C Indications for testing (arterial thrombosis): do not obtain testing for inherited thrombophilias in patients with arterial thrombosis D or acute ischemic stroke, D including neonatal stroke. D 2. Medical management Management of venous thromboembolism (general principles): manage VTE in FVL mutation carriers according to current published guidelines on VTE (including deep vein thrombosis, PE, cerebral venous thrombosis, as well as thrombosis in unusual sites). E https://web.pathway.md/diseases/rec908OQ43qB7Tngk 3/7 6/29/23, 4:01 AM Factor V Leiden Pathway Management of venous thromboembolism (deep vein thrombosis): initiate full-dose extended anticoagulant therapy with periodic reevaluations in patients with deep vein thrombosis and high risk thrombophilia, including homozygous FVL mutation. B Management of venous thromboembolism (cerebral vein thrombosis): consider initiating indefinite anticoagulation with a target INR of 2.0-3.0 in patients with cerebral venous thrombosis with severe thrombophilia, including homozygous FVL mutation. C Management of venous thromboembolism, mesenteric vein thrombosis: As per ACG 2020 guidelines, initiate indefinite anticoagulation in patients with mesenteric vein thrombosis and thrombophilia. B As per ESVS 2017 guidelines, initiate lifelong anticoagulation in patients with mesenteric vein thrombosis with proven thrombophilia. B As per ESTES 2016 guidelines, initiate anticoagulation for a minimum of 6 months in patients with mesenteric vein thrombosis and thrombophilia. B Management of venous thromboembolism (portal vein thrombosis): consider initiating anticoagulation in patients with chronic portal vein thrombosis with evidence of inherited thrombophilia. Consider continuing anticoagulation indefinitely in patients with thrombophilia. C 3. Specific circumstances Pregnant patients, screening: As per BSH 2022 guidelines, do not obtain screening for heritable thrombophilias in patients with pregnancy complications, such as recurrent miscarriage or adverse pregnancy outcomes. D As per FIGO 2021 guidelines, do not obtain screening for hereditary thrombophilias in patients with a history of fetal growth restriction. D As per SOGC 2014 guidelines: Do not obtain universal screening for thrombophilias in patients experiencing adverse pregnancy outcomes (severe preeclampsia, IUGR, stillbirth). D Do not obtain routine screening for all inherited thrombophilias in patients with a first episode of VTE during in pregnancy. D Pregnant patients, antepartum: As per ASH 2018 guidelines: Avoid initiating antepartum thromboprophylaxis to prevent a first venous thromboembolic event in patients with heterozygous FVL mutation, regardless of family history of VTE. D Consider initiating antepartum thromboprophylaxis to prevent a first venous thromboembolic event in patients with homozygous FVL mutation or combined thrombophilias, regardless of family history of VTE. C As per SOGC 2014 guidelines, initiate prophylactic-dose thromboprophylaxis during pregnancy in patients with any of the following: personal history of provoked VTE and low-risk thrombophilia A https://web.pathway.md/diseases/rec908OQ43qB7Tngk 4/7 6/29/23, 4:01 AM Factor V Leiden Pathway asymptomatic homozygous FVL mutation B asymptomatic combined thrombophilia. B As per ACCP 2012 guidelines: Consider initiating antepartum thromboprophylaxis with prophylactic- or intermediate-dose LMWH in patients with homozygous FVL mutation, no personal history of VTE, and a positive family history of VTE. C Consider obtaining antepartum clinical vigilance in patients with homozygous FVL mutation, no personal history of VTE, and no family history of VTE. C Pregnant patients, postpartum: As per ASH 2018 guidelines: Avoid initiating postpartum thromboprophylaxis to prevent a first venous thromboembolic event in patients with heterozygous FVL mutation, regardless of family history of VTE. D Consider initiating postpartum thromboprophylaxis to prevent a first venous thromboembolic event in patients with homozygous FVL mutation or combined thrombophilias, regardless of family history. C As per SOGC 2014 guidelines: Initiate postpartum thromboprophylaxis in patients with homozygous FVL mutation or combined thrombophilia. B Consider initiating postpartum thromboprophylaxis in patients with heterozygous FVL mutation with any of the following clinical or pregnancy-related risk factors: Situation Guidance BMI 30 kg/m at first antepartum visit Maternal risk factors Smoking > 10 cigarettes/day antepartum Cardiac disease Maternal comorbidity Systemic lupus erythematous Sickle cell disease IBD Varicose veins Gestational diabetes Gestational comorbidity Preeclampsia IUGR Placenta previa Preterm delivery Delivery-related factors Emergency C-section Peripartum or postpartum blood loss of > 1 L or blood product replacement Stillbirth C https://web.pathway.md/diseases/rec908OQ43qB7Tngk 5/7 6/29/23, 4:01 AM Factor V Leiden Pathway As per ACCP 2012 guidelines, consider initiating postpartum thromboprophylaxis for 6 weeks with prophylactic- or intermediate-dose LMWH or vitamin K antagonists targeted at INR of 2.0-3.0 in patients with homozygous FVL mutation and no personal history of VTE, regardless of family history of VTE. C Clinical findings Past medical history Past obstetric history Acute ischemic stroke IUGR Budd-chiari syndrome Miscarriage Cerebral venous thrombosis Placental abruption DVT Preeclampsia Mesenteric vein thrombosis Stillbirth Portal vein thrombosis Lab findings Prothrombin G20210A mutation Splanchnic vein thrombosis Activated protein C resistance Superficial vein thrombosis Prolonged PTT Family history VTE Genetic testing F5 gene mutation References 1. Nir Melamed, Ahmet Baschat, Yoav Yinon et al. FIGO International Federation of Gynecology and Obstetrics) initiative on fetal growth: best practice advice for screening, diagnosis, and management of fetal growth restriction. Int J Gynaecol Obstet. 2021 Mar;152 Suppl 1 Suppl 1 3 57. Open 2. Scott M Stevens, Scott C Woller, Kenneth A Bauer et al. Guidance for the evaluation and treatment of hereditary and acquired thrombophilia. J Thromb Thrombolysis. 2016 Jan;41 1 154 64. Open 3. Saskia Middeldorp, Robby Nieuwlaat, Lisa Baumann Kreuziger et al. American Society of Hematology 2023 Guidelines for Management of Venous Thromboembolism: Thrombophilia Testing. Blood Adv. 2023 May 17;bloodadvances.2023010177. Open 4. Tilsed JV, Casamassima A, Kurihara H et al. ESTES guidelines: acute mesenteric ischaemia. Eur J Trauma Emerg Surg. 2016 Apr;42 2 253 70. Open 5. Deepa J Arachchillage, Lucy Mackillop, Arvind Chandratheva et al. Guidelines for thrombophilia testing: A British Society for Haematology guideline. Br J Haematol. 2022 May 29. Open 6. E Kalodiki, V Stvrtinova, C Allegra et al. Superficial vein thrombosis: a consensus statement. Int Angiol. 2012 Jun;31 3 203 16. Open 7. Karapetyan H, Grigoryan A, Mullie L. Recommendations & Algorithms. Pathway Editors. Open https://web.pathway.md/diseases/rec908OQ43qB7Tngk 6/7 6/29/23, 4:01 AM Factor V Leiden Pathway 8. Stavros K Kakkos, Manjit Gohel, Niels Baekgaard et al. Editor's Choice European Society for Vascular Surgery ESVS 2021 Clinical Practice Guidelines on the Management of Venous Thrombosis. Eur J Vasc Endovasc Surg. 2021 Jan;61 1 9 82. Open 9. Chan WS, Rey E, Kent NE et al. Venous thromboembolism and antithrombotic therapy in pregnancy. J Obstet Gynaecol Can. 2014 Jun;36 6 527 53. Open 10. Patrick G Northup, Juan Carlos Garcia-Pagan, Guadalupe Garcia-Tsao et al. Vascular Liver Disorders, Portal Vein Thrombosis, and Procedural Bleeding in Patients With Liver Disease: 2020 Practice Guidance by the American Association for the Study of Liver Diseases. Hepatology. 2021 Jan;73 1 366 413. Open 11. Bates SM, Rajasekhar A, Middeldorp S et al. American Society of Hematology 2018 guidelines for management of venous thromboembolism: venous thromboembolism in the context of pregnancy. Blood Adv. 2018 Nov 27;2 22 3317 3359. Open 12. Gustavo Saposnik, Fernando Barinagarrementeria, Robert D Brown Jr et al. Diagnosis and management of cerebral venous thrombosis: a statement for healthcare professionals from the American Heart Association/American Stroke Association. Stroke. 2011 Apr;42 4 1158 92. Open 13. Douglas A Simonetto, Ashwani K Singal, Guadalupe Garcia-Tsao et al. ACG Clinical Guideline: Disorders of the Hepatic and Mesenteric Circulation. Am J Gastroenterol. 2020 Jan;115 1 18 40. Open 14. M Bj rck, M Koelemay, S Acosta et al. Editor's Choice Management of the Diseases of Mesenteric Arteries and Veins: Clinical Practice Guidelines of the European Society of Vascular Surgery ESVS . Eur J Vasc Endovasc Surg. 2017 Apr;53 4 460 510. Open 15. Campbell Tait, Trevor Baglin, Henry Watson et al. Guidelines on the investigation and management of venous thrombosis at unusual sites. Br J Haematol. 2012 Oct;159 1 28 38. Open 16. Shannon M Bates, Ian A Greer, Saskia Middeldorp et al. VTE, thrombophilia, antithrombotic therapy, and pregnancy: Antithrombotic Therapy and Prevention of Thrombosis, 9th ed: American College of Chest Physicians Evidence-Based Clinical Practice Guidelines. Chest. 2012 Feb;141 2 Suppl):e691S- e736S. Open 17. European Association for the Study of the Liver. EASL Clinical Practice Guidelines: Vascular diseases of the liver. J Hepatol. 2016 Jan;64 1 179 202. Open 18. J M Ferro, M G Bousser, P Canh o et al. European Stroke Organization guideline for the diagnosis and treatment of cerebral venous thrombosis - endorsed by the European Academy of Neurology. Eur J Neurol. 2017 Oct;24 10 1203 1213. Open 19. O'Donnell TF Jr, Passman MA, Marston WA et al. Management of venous leg ulcers: clinical practice guidelines of the Society for Vascular Surgery and the American Venous Forum. J Vasc Surg. 2014 Aug;60 2 Suppl):3S 59S. Open https://web.pathway.md/diseases/rec908OQ43qB7Tngk 7/7 |
Guideline sources The following summarized guidelines for the evaluation and management of falls in the elderly are prepared by our editorial team based on guidelines from the World Falls Guidelines (WFG 2022), the American College of Obstetricians and Gynecologists (ACOG 2021), the U.S. Preventive Services Task Force (USPSTF 2018), the Danish Health Authority (DHA 2018), the Eastern Association for the Surgery of Trauma (EAST 2016), and the Osteoporosis Canada Foundation (OC 2010). 1 2 3 4 5 6 7 7 8 8 Definition Falls in the elderly is an accidental event due to loss of center of gravity and imbalance that results in an increased risk of traumatic injuries and hospitalizations. 7 Epidemiology Falls in the elderly can be caused due to intrinsic (sight disorders, hearing disorders, balance deficit, alterations in CNS, musculoskeletal alterations; neurological, cardiovascular, endocrine, psychiatric, and iatrogenic pathologies) and extrinsic (environmental factors such as obstacles, inadequate footwear) predisposing factors. 8 https://web.pathway.md/diseases/recCny4SfSOLQBKqV 1/8 6/29/23, 4:01 AM Falls in the elderly Pathway Disease course Clinical manifestations of falls in the elderly include pain, bruising, lacerations, fractures (mostly upper extremity and hip fractures), and intracranial bleeding in severe cases. Falls in the elderly increase the risk of hospitalizations and morbidity. 7 Prognosis and risk of recurrence Falls-related mortality in adults with 65, 75, and 85 years of age is 50 per 100,000, 150 per 100,000 and 5,252 per 100,000 individuals, respectively. 8 Guidelines 1. Screening and diagnosis Indications for screening: As per WFG 2022 guidelines, ask about falls when interacting with older adults, as they often will not be spontaneously reported. B Show 2 more As per ACOG 2021 guidelines, assess the risk of falls in postmenopausal patients with low bone mineral density or osteoporosis. E 2. Classification and risk stratification Risk prediction, general principles: As per WFG 2022 guidelines, obtain multiprofessional, multifactorial assessment in community-dwelling older adults identified to be at high risk of falling, to guide tailored interventions. A Show 5 more As per EAST 2016 guidelines, perform risk stratifaction with targeted, comprehensive fall risk-reduction strategies tailored to high risk groups. A Risk prediction (screening for environmental hazards): identify individual's environmental hazards where they live and assess their capacities and behaviors in relation to them as part of a multifactorial falls risk assessment. A Risk prediction (cardiovascular assessment): obtain a cardiovascular assessment, initially including cardiac history, auscultation, lying and standing orthostatic BP, and surface 12-lead ECG, as part of a multifactorial falls risk assessment. A Show 2 more Risk prediction (assessment of gait and balance): assess gait and balance as part of the risk assessment of falls. A Risk prediction (assessment of dizziness): assess for dizziness symptoms and obtain follow- up assessment as necessary to identify cardiovascular, neurological and/or vestibular causes. E Risk prediction (assessment of vision and hearing): https://web.pathway.md/diseases/recCny4SfSOLQBKqV 2/8 6/29/23, 4:01 AM Falls in the elderly Pathway Assess for vision impairment as part of a multifactorial falls risk assessment, measure visual acuity and examine for other visual impairments, such as hemianopia and neglect where appropriate. E Assess for hearing impairment as part of a multifactorial falls risk assessment, measure and examine for hearing impairments and refer to a specialist where appropriate. E Risk prediction (assessment of urinary symptoms): assess for urinary symptoms as part of a multifactorial falls risk assessment. E Risk prediction (assessment of pain): assess for pain as part of a multifactorial falls risk assessment, followed as indicated by a comprehensive pain assessment. E Risk prediction (assessment of mental health): Obtain assessment of cognition as part of a multifactorial falls risk assessment in older adults. A Assess for depressive symptoms as part of a multifactorial falls risk assessment, followed by further mental state assessment if necessary and referral to a specialist where appropriate. E Risk prediction (assessment of nutritional status): assess nutritional status, including vitamin D intake, as part of a multifactorial falls risk assessment, followed by supplementation where appropriate. E 3. Diagnostic investigations Clinical history: As per WFG 2022 guidelines, ask older adults presenting with a fall or related injury about the details of the event and its consequences, previous falls, transient loss of consciousness, or dizziness, and any preexisting impairment of mobility or concerns about falling causing limitation of usual activities. E As per EAST 2016 guidelines, consider obtaining screening for frailty in elderly patients with falls. C 4. Medical management Management of cardiovascular issues: Manage orthostatic hypotension as a component of a multidomain intervention. B Offer the same intervention for cardiovascular disorders identified during assessment for risk of falls as for similar conditions associated with syncope, in addition to other interventions based on the multifactorial falls risk assessment. A Management of vestibular issues: consider managing vestibular issues as part of the multifactorial approach. E Management of impaired vision: consider managing impaired vision as part of the multifactorial approach. E Management of pain: consider providing adequate pain control as part of the multifactorial approach. E https://web.pathway.md/diseases/recCny4SfSOLQBKqV 3/8 6/29/23, 4:01 AM Falls in the elderly Pathway Management of patient concerns: offer exercise, CBT, and/or occupational therapy as part of a multidisciplinary approach to reduce concerns about falling in community-dwelling older adults. A 5. Inpatient care Inpatient assessment: obtain a multifactorial falls risk assessment in all hospitalized patients aged > 65 years. Do not use scored falls risk screening tools in hospitals for multifactorial falls risk assessment in older adults. B Show 2 more Inpatient prevention strategies: Deliver a tailored education on falls prevention in all hospitalized older adults ( 65 years of age) and other high-risk groups. B Implement personalized single or multidomain falls prevention strategies in acute B or subacute care A based on identified risk factors, behaviors, or situations in all hospitalized older adults or younger persons at risk of falls. A 6. Specific circumstances Patients in care homes: do not obtain falls risk screening to identify care home residents at risk for falls since all residents should be deemed at high risk of falls. D Show 7 more Patients with Parkinson's disease: consider obtaining a falls risk assessment, including a self- report 3-risk factor assessment tool including a history of falls in the previous year, freezing of gait in the past month, and slow gait speed, in older patients with Parkinson's disease. B Show 3 more Patients with hip fracture: offer an individualized and progressive exercise program to improve mobility, including standing up, balance, walking, and climbing stairs, as a fall prevention strategy in older adults after sustaining a hip fracture. A Commence such programs in the hospital B and continue in the community. B 7. Patient education General counseling: advise on how to maintain safe mobility and optimize physical functioning in older patients at low risk of falls, taking into consideration the individual's circumstances, priorities, preferences, and resources. Reinforce health promotion/prevention messaging relevant to falls and fracture risks, including physical activity, lifestyle habits, and nutrition, including vitamin D intake. E 8. Preventative measures General principles: incorporate the individual's values and preferences in the care plan developed to prevent falls and related injuries. A Include both the individual's and their https://web.pathway.md/diseases/recCny4SfSOLQBKqV 4/8 6/29/23, 4:01 AM Falls in the elderly Pathway caregivers' perspectives when creating fall prevention care plans for older adults with cognitive impairment. B Environmental modifications: As per WFG 2022 guidelines, provide counseling for modifications of the physical home environment for fall hazards as part of a multidomain falls prevention intervention. A As per ACOG 2021 guidelines, offer fall prevention strategies, including environmental assessment and modification, in patients at risk of falls. E As per DHA 2018 guidelines, consider offering changes to improve the safety in the home environment of elderly adults at risk of falls living at home. C As per EAST 2016 guidelines, consider offering physical environment modification to prevent falls in frail elderly people. C Exercise interventions: As per WFG 2022 guidelines, offer an exercise program based on an individual assessment. E Show 6 more As per ACOG 2021 guidelines, offer fall prevention strategies, including weight-bearing and muscle-strengthening exercises, in patients at risk of falls. E As per DHA 2018 guidelines: Consider offering balance training in elderly adults at risk of falls living at home or in institutional care. C Consider offering movement concepts, such as Tai Chi, yoga, dance, or dance-like training in elderly adults at risk of falls living at home. C Updated evidence: Treadmill balance training in older adults In community-dwelling adults 65 years of age and were able to walk without a walking aid, perturbation-based balance training was not superior to treadmill walking training with respect to a daily-life fall rates in the past 12 months. Jens Eg N rgaard et al. JAMA Netw Open. 2023 Apr 3. As per USPSTF 2018 guidelines, offer exercise interventions to prevent falls in community- dwelling 65 years old adults at increased risk for falls. B As per EAST 2016 guidelines, consider offering evidence-based exercise programs for frail elderly individuals. C As per OC 2010 guidelines, consider offering exercises focusing on balance, such as Tai Chi, or on balance and gait training in individuals at risk of falls. B Multifactorial interventions: As per WFG 2022 guidelines, offer multidomain interventions, informed by a multiprofessional, multifactorial falls risk assessment, in community-dwelling older adults at high risk of falls. A https://web.pathway.md/diseases/recCny4SfSOLQBKqV 5/8 6/29/23, 4:01 AM Falls in the elderly Pathway As per ACOG 2021 guidelines, offer fall prevention strategies, including individualized multifactorial interventions such as vision assessment and treatment, balance training, and environmental assessment and modification, in patients at risk of falls. E As per USPSTF 2018 guidelines, offer multifactorial interventions to prevent falls in selective groups of 65 years old community-dwelling individuals at increased risk for falls. B Hip protectors: As per EAST 2016 guidelines, consider offering hip protectors in frail elderly individuals in the appropriate environment. C As per OC 2010 guidelines, consider offering hip protectors in older adults at high risk for fracture residing in long-term care facilities. C Vestibular rehabilitation: consider offering vestibular rehabilitation in elderly patients with diagnosed vestibular dysfunction. C Vitamin D/calcium supplementation: As per USPSTF 2018 guidelines, do not use vitamin D supplements for the prevention of falls in community-dwelling 65 years old adults. D As per EAST 2016 guidelines, consider offering vitamin D and calcium supplementation for the prevention of fall-related injuries in frail elderly individuals. C Medication review: As per WFG 2022 guidelines: Assess for fall history and the risk of falls before prescribing potential fall risk-increasing drugs in older adults. A Obtain a medication review and deprescribe fall risk-increasing drugs as part of multidomain fall prevention interventions. A Use a validated, structured screening and assessment tool to identify fall risk-increasing drugs when conducting a general medication review or medication review targeted to fall prevention. B As per DHA 2018 guidelines: Discontinue benzodiazepines in elderly adults in case of > 4 weeks of use, since benzodiazepines increase the risk of falls. B Keep selective serotonin receptor inhibitors only upon due consideration and only if non- pharmacological treatment has been ineffective, in > 65 years old adults with moderate depression, since selective serotonin receptor inhibitors may increase the risk of falling and the effect on depression and quality of life is likely to be negligible. B Clinical findings Symptoms Past medical history Dizziness Dementia Gait disturbance Depression Lightheadedness Diabetes mellitus Loss of balance Diabetic neuropathy Loss of consciousness Epilepsy https://web.pathway.md/diseases/recCny4SfSOLQBKqV 6/8 6/29/23, 4:01 AM Falls in the elderly Pathway Loss of sensation HF Muscle weakness Orthostatic hypotension Palpitations Osteoporosis Urinary incontinence Parkinson's disease Vasovagal syncope Rheumatoid arthritis Stroke Medication history Visual disorder Vitamin D deficiency Polypharmacy Psychotropic medications Vital signs Anthropomorphic exam Bradycardia Hypotension Frailty Neurological exam Musculoskeletal exam Cognitive impairment Abnormal timed Up and Go test Peripheral neuropathy ECG findings Integument exam Cardiac arrhythmias Studies 2023 Treadmill balance training in older adults In community-dwelling adults 65 years of age and were able to walk without a walking aid, perturbation-based balance training was not superior to treadmill walking training with respect to a daily-life fall rates in the past 12 months. Jens Eg N rgaard et al. JAMA Netw Open. 2023 Apr 3. References 1. US Preventive Services Task Force, Grossman DC, Curry SJ et al. Interventions to Prevent Falls in Community-Dwelling Older Adults: US Preventive Services Task Force Recommendation Statement. JAMA. 2018 Apr 24;319 16 1696 1704. Open 2. Manuel Montero-Odasso, Nathalie van der Velde, Finbarr C Martin et al. World guidelines for falls prevention and management for older adults: a global initiative. Age Ageing. 2022 Sep 2;51 9):afac205. Open 3. Papaioannou A, Morin S, Cheung AM et al. 2010 clinical practice guidelines for the diagnosis and management of osteoporosis in Canada: summary. CMAJ. 2010 Nov 23;182 17 1864 73. Open 4. Crandall M, Duncan T, Mallat A et al. Prevention of fall-related injuries in the elderly: An Eastern Association for the Surgery of Trauma practice management guideline. J Trauma Acute Care Surg. 2016 Jul;81 1 196 206. Open 5. Danish Health Authority. National clinical guideline for the prevention of falls in elderly people: Quick guide. Sundhedsstyrelsen. 2018 Apr. Open https://web.pathway.md/diseases/recCny4SfSOLQBKqV 7/8 6/29/23, 4:01 AM Falls in the elderly Pathway 6. American College of Obstetricians and Gynecologists Committee on Clinical Practice Guidelines Gynecology. Osteoporosis Prevention, Screening, and Diagnosis: ACOG Clinical Practice Guideline No. 1. Obstet Gynecol. 2021 Sep 1;138 3 494 506. Open 7. Suleiman I. Sharif, Alaa B. Al-Harbi, Alaa M. Al-Shihabi et al. Falls in the elderly: assessment of prevalence and risk factors. Pharm Pract Granada). 2018 Jul-Sep; 16 3 1206. Open 8. Pietro Pasquetti, Lorenzo Apicella, Giuseppe Mangone. Pathogenesis and treatment of falls in elderly. 2014 Sep;11 3 222 5.2014 Sep;11 3 222 5. Open 9. Carpenter CR. Evidence-based emergency medicine/rational clinical examination abstract. Will my patient fall?. Ann Emerg Med. 2009 Mar;53 3 398 400. Open 10. American Academy of Neurology. Choosing Wisely AAN recommendations. Choosing Wisely. 2014. Open 11. American Physical Therapy Association. Choosing Wisely APTA recommendations. Choosing Wisely. 2014. Open 12. Society for Post-Acute and Long-Term Care Medicine. Choosing Wisely AMDA recommendations. Choosing Wisely. 2015. Open 13. Pietro Pasquetti, Lorenzo Apicella, and Giuseppe Mangone. Pathogenesis and treatment of falls in elderly. Clin Cases Miner Bone Metab. 2014 Sep-Dec; 11 3 222 225. Open 14. Jens Eg N rgaard, Stig Andersen, Jesper Ryg et al. Effect of Treadmill Perturbation-Based Balance Training on Fall Rates in Community-Dwelling Older Adults: A Randomized Clinical Trial. JAMA Netw Open. 2023 Apr 3;6 4):e238422. Open https://web.pathway.md/diseases/recCny4SfSOLQBKqV 8/8 |
Guideline sources The following summarized guidelines for the evaluation and management of familial adenomatous polyposis (FAP) are prepared by our editorial team based on guidelines from the European Society of Gastrointestinal Endoscopy (ESGE 2023), the American Society for Gastrointestinal Endoscopy (ASGE 2020), and the American College of Gastroenterology (ACG 2015). 1 2 3 Guidelines 1. Screening and diagnosis Indications for screening: perform screening sigmoidoscopy or colonoscopy in pediatric patients with definite or suspected FAP at ages 10-12 years. B Show 3 more 2. Diagnostic investigations Genetic testing: obtain genetic counseling and testing in patients with clinical polyposis defined as 10 adenomas found on a single endoscopy and 20 adenomas during their https://web.pathway.md/diseases/recSheLhax8rSQTnp 1/4 6/29/23, 4:02 AM Familial adenomatous polyposis Pathway lifetime. B Show 2 more 3. Diagnostic procedures Upper gastrointestinal endoscopy: perform careful examination of the ampulla and periampullary region using a duodenoscope or cap-assisted gastroscope given the predilection for cancer in this area. B Biopsy: perform biopsy sampling of the ampulla to assess for villous histology or dysplasia only in patients with an identifiable mucosal abnormality, while avoiding the pancreatic orifice because of the risk for pancreatitis. B 4. Medical management Chemoprevention: offer chemopreventive agents within the confines of a tertiary hereditary cancer center and/or as part of clinical trials. B 5. Therapeutic procedures Endoscopic resection: Perform endoscopic resection of: gastric and duodenal polyps > 1 cm, given the risk of developing dysplasia all antral polyps, given the predominance of gastric adenomas in this location. B 6. Surgical interventions Indications for colectomy: Perform immediate colectomy in patients with FAP, attenuated FAP or MUTYH-associated polyposis in the presence of documented or suspected cancer or significant symptoms. B Consider performing colectomy in patients with FAP, attenuated FAP or MUTYH-associated polyposis in the presence of multiple adenomas > 6 mm, a significant increase in adenoma number, and inability to adequately survey the colon because of multiple diminutive polyps. B 7. Follow-up and surveillance Surveillance endoscopy, colon: As per ASGE 2020 guidelines: Perform follow-up colonoscopy in patients with rectosigmoid polyps if sigmoidoscopy was the initial screening test. B Perform surveillance colonoscopy at 1-2 year intervals in patients with FAP B , attenuated FAP or MUTYH-associated polyposis. B As per ACG 2015 guidelines: https://web.pathway.md/diseases/recSheLhax8rSQTnp 2/4 6/29/23, 4:02 AM Familial adenomatous polyposis Pathway Obtain screening for colorectal cancer by annual colonoscopy or flexible sigmoidoscopy beginning at puberty in patients at risk for or affected with the classic adenomatous polyposis syndromes. B Perform surveillance colonoscopy in families with attenuated FAP or MUTYH-associated polyposis. B Surveillance endoscopy (small bowels): obtain surveillance of the proximal small bowel in patients with FAP using conventional forward-viewing and side-viewing endoscopes. B Show 2 more Post-operative surveillance: As per ASGE 2020 guidelines: Perform pouch endoscopy or ileoscopy at 1-2 year intervals in patients with ileal pouch anal anastomosis or ileostomy. B Perform sigmoidoscopy at 6-months to 1-year intervals indefinitely in patients with ileorectal anastomosis. B As per ACG 2015 guidelines, obtain postsurgical surveillance by yearly endoscopy of rectum or ileal pouch, and examination of an ileostomy every 2 years. B Surveillance for extracolonic malignancies: As per ASGE 2020 guidelines: Perform upper gastrointestinal surveillance endoscopy based on the interval advised for the most severely affected organ, whether stomach or duodenum. B Perform random sampling biopsy and targeted biopsy sampling of any suspicious lesions during surveillance examination to assess for dysplasia and accurate duodenal Spigelman stage. Recognize that baseline Spigelman score 7 is associated with development of duodenal high-grade dysplasia. B As per ACG 2015 guidelines, perform upper gastrointestinal endoscopy including duodenoscopy starting at age 25-30 years to screen for gastric and proximal small bowel tumors. Obtain repeated surveillance every 6 months to 4 years depending on Spigelman stage of duodenal polyposis: Situation Guidance 4 years 0 2-3 years I 1-3 years II 6-12 months III Surgical evaluation. B IV Show 3 more Clinical findings https://web.pathway.md/diseases/recSheLhax8rSQTnp 3/4 6/29/23, 4:02 AM Familial adenomatous polyposis Pathway Symptoms Past medical history Abdominal pain Colorectal cancer Bloating Colorectal polyps Constipation Hepatoblastoma Diarrhea Pancreatic cancer Fatigue Thyroid cancer Hematochezia Mucus in stools Pain with bowel movements Rectal bleeding Weight loss Endoscopic findings References 1. Sapna Syngal, Randall E Brand, James M Church et al. ACG clinical guideline: Genetic testing and management of hereditary gastrointestinal cancer syndromes. Am J Gastroenterol. 2015 Feb;110 2 223 62; quiz 263. Open 2. Julie Yang, Suryakanth R Gurudu, Cathryn Koptiuch et al. American Society for Gastrointestinal Endoscopy guideline on the role of endoscopy in familial adenomatous polyposis syndromes. Gastrointest Endosc. 2020 May;91 5 963 982.e2. Open 3. Marco Pennazio, Emanuele Rondonotti, Edward J Despott et al. Small-bowel capsule endoscopy and device-assisted enteroscopy for diagnosis and treatment of small-bowel disorders: European Society of Gastrointestinal Endoscopy ESGE Guideline Update 2022. Endoscopy. 2023 Jan;55 1 58 95. Open 4. Monique E van Leerdam, Victorine H Roos, Jeanin E van Hooft et al. Endoscopic management of polyposis syndromes: European Society of Gastrointestinal Endoscopy ESGE Guideline. Endoscopy. 2019 Sep;51 9 877 895. Open https://web.pathway.md/diseases/recSheLhax8rSQTnp 4/4 |
Guideline sources The following summarized guidelines for the evaluation and management of familial hypercholesterolemia are prepared by our editorial team based on guidelines from the Canadian Pediatric Cardiology Association (CPCA/CCS 2022), the Canadian Cardiovascular Society (CCS 2021; 2018), the European Society of Cardiology (ESC/EAS 2020), and the American College of Preventive Medicine (ACPM/ADA/PCNA/ABC/ASPC/AAPA/AGS/AHA/ACC/APhA 2019). 1 2 3 4 5 Calculator DLCN criteria for familial hyperc Guidelines 1. Screening and diagnosis f https://web.pathway.md/diseases/rec3NR9IfcU05ASZA I di ti i 1/7 6/29/23, 4:02 AM Familial hypercholesterolemia Pathway Indications for screening: As per CCS 2022 guidelines, obtain universal lipid screening (fasting or nonfasting, non-HDL cholesterol or LDL cholesterol) within the first decade of life (after 2 years old), coupled with cascade screening for identified cases of probable/definite familial hypercholesterolemia or other monogenic lipid disorders. Consider obtaining selective screening at any time in pediatric patients with identified cardiovascular risk factors or risk conditions or a positive family history of premature CVD or dyslipidemia. E As per AHA 2019 guidelines, consider obtaining fasting or nonfasting lipoprotein profile as early as age 2 years to detect familial hypercholesterolemia or rare forms of hypercholesterolemia in pediatric and adolescent patients with a family history of either early CVD or significant hypercholesterolemia. C Screening of family relatives: As per ESC 2020 guidelines, obtain family screening for familial hypercholesterolemia once the index case is diagnosed. B As per AHA 2019 guidelines, consider obtaining reverse-cascade screening of family members of pediatric and adolescent patients found to have moderate-to-severe hypercholesterolemia, including cholesterol testing for first-, second-, and when possible, third-degree biological relatives, for detection of familial forms of hypercholesterolemia. C As per CCS 2018 guidelines: Implement cascade screening (lipid profile) protocols at the local, provincial and national level and offer in first-degree relatives of patients with familial hypercholesterolemia. B Consider obtaining universal cholesterol level screening for the detection of familial hypercholesterolemia in children with reverse cascade screening of parents when warranted. C Diagnosis: As per CCS 2022 guidelines, consider obtaining genetic testing, when accessible, to achieve definitive diagnoses of familial hypercholesterolemia or other genetic dyslipidemias. Consider establishing clinical diagnosis independent of genetic testing using available non-genetic criteria. E As per ESC 2020 guidelines, suspect the diagnosis of familial hypercholesterolemia in patients with: premature coronary artery disease (males < 55 years old; females < 60 years old) severely elevated LDL cholesterol in adults (> 5 mmol/L; > 190 mg/dL) and in pediatrics (> 4 mmol/L; > 150 mg/dL) relatives having premature fatal or non-fatal CVD relatives having tendon xanthomas. B Show 2 more As per CCS 2018 guidelines, define familial hypercholesterolemia using the Dutch Lipid Clinic Network Criteria, Simon Broome Registry or Familial Hypercholesterolemia Canada definition. A 2. Classification and risk stratification https://web.pathway.md/diseases/rec3NR9IfcU05ASZA 2/7 6/29/23, 4:02 AM Familial hypercholesterolemia Pathway CVD risk stratification: do not use current risk calculators (Framingham Risk Score, Pooled Cohort Equation, European SCORE) to determine cardiovascular risk in patients with familial hypercholesterolemia. D Show 2 more 3. Diagnostic investigations Genetic testing: As per CCS 2022 guidelines, consider obtaining genetic testing, when accessible, to achieve definitive diagnoses of familial hypercholesterolemia or other genetic dyslipidemias. E As per ESC 2020 guidelines, obtain testing for familial hypercholesterolemia in pediatric patients from the age of 5, or earlier if homozygous familial hypercholesterolemia is suspected. B As per CCS 2018 guidelines, offer genetic testing, when available, to complement a diagnosis of familial hypercholesterolemia and enable cascade screening. A 4. Medical management General principles: Institute a personalized treatment plan taking into account at a minimum age, additional cardiovascular risk factors, psychosocial and socioeconomic factors, and personal and family preferences, developed as a shared decision process as the diagnosis of familial hypercholesterolemia using validated clinical criteria and/or genotyping may occur at any age and imparts a high, lifelong risk of ASCVD. B Include statins as the primary therapy and secondary agents as required including ezetimibe and PCSK9 inhibitors in a personalized treatment plan in patients with familial hypercholesterolemia requiring medications. B Treatment targets: As per ESC 2020 guidelines, treat patients with familial hypercholesterolemia and ASCVD or another major risk factor as very high risk patients, and patients without ASCVD or other risk factors as high risk patients. B Show 3 more As per CCS 2018 guidelines, consider targeting LDL cholesterol < 2.0 mmol/L and non-HDL cholesterol < 2.6 mmol/L in patients with familial hypercholesterolemia and ASCVD. C Statins: As per ESC 2020 guidelines: Initiate high-intensity statin therapy up to the highest tolerated dose to reach the goals set for the specific level of risk. A Initiate statins in pediatric patients with familial hypercholesterolemia from 8-10 years of age. B As per AHA 2019 guidelines, consider initiating statin therapy in > 10 years old pediatric and adolescent patients with LDL cholesterol level persistently 190 mg/dL ( 4.9 mmol/L) or https://web.pathway.md/diseases/rec3NR9IfcU05ASZA 3/7 6/29/23, 4:02 AM Familial hypercholesterolemia Pathway 160 mg/dL ( 4.1 mmol/L) with a clinical presentation consistent with familial hypercholesterolemia not responding adequately to 3-6 months of lifestyle therapy. C As per CCS 2018 guidelines: Initiate statins as first-line therapy in patients with familial hypercholesterolemia. A Do not use statins during pregnancy. D Ezetimibe: As per ESC 2020 guidelines: Add ezetimibe to statins if the goals are not achieved with the maximum tolerated dose of statins. B Consider initiating ezetimibe if statins are not tolerated at any dosage, even after rechallenge. C As per CCS 2018 guidelines, consider initiating ezetimibe as second-line agent to achieve unmet LDL cholesterol goals. C PCSK9 inhibitors: As per CCS 2021 guidelines: Initiate PCSK9 inhibitor (alirocumab or evolocumab) to lower LDL cholesterol level in patients with heterozygous familial hypercholesterolemia without clinical ASCVD, if LDL cholesterol remains above the target (LDL cholesterol 2.5 mmol/L or < 50% reduction from baseline or ApoB 0.85 mg/dL or non-HDL cholesterol 3.2 mmol/L) despite maximally tolerated statin therapy with or without ezetimibe therapy. A Initiate PCSK9 inhibitor (alirocumab or evolocumab) in patients with heterozygous familial hypercholesterolemia and ASCVD, if LDL cholesterol remains above the threshold 1.8 mmol/L (or ApoB 0.7 mg/dL or non-HDL cholesterol 2.4 mmol/L) despite maximally tolerated statin therapy with or without ezetimibe. A Updated evidence: FOURIER-OLE In patients with a history of established ASCVD and a fasting low-density lipoprotein cholesterol level 70 mg/dL while on statin, evolocumab was superior to placebo in parent study with respect to the composite of cardiovascular death, myocardial infarction, stroke, hospitalization for unstable angina, or coronary revascularization. Michelle L O'Donoghue et al. Circulation. 2022 Oct 11. As per ESC 2020 guidelines, initiate or add a PCSK9 inhibitor in very high risk patients with familial hypercholesterolemia if the goal is not achieved with a maximum tolerated dose of a statin and ezetimibe. B As per AHA 2019 guidelines: Consider adding a PCSK9 inhibitor in 30-75 years old patients with heterozygous familial hypercholesterolemia and LDL cholesterol level of 100 mg/dL ( 2.6 mmol/L) with maximally tolerated statin and ezetimibe therapy. C Insufficient evidence to support addition of a PCSK9 inhibitor in patients with familial hypercholesterolemia without evidence of clinical ASCVD receiving maximally tolerated https://web.pathway.md/diseases/rec3NR9IfcU05ASZA 4/7 6/29/23, 4:02 AM Familial hypercholesterolemia Pathway statin and ezetimibe therapy. I As per CCS 2018 guidelines, consider initiating monoclonal antibody inhibitors of PCSK9 in adult patients with familial hypercholesterolemia without ASCVD if they have not achieved a 50% reduction in LDL cholesterol from baseline level and reached an LDL cholesterol level of at least < 3.5 mmol/L or lower (as determined by the shared decision process between physician and patient) on maximally tolerated statin therapy with or without ezetimibe. B Bile acid sequestrants: consider adding a bile acid sequestrant if the goal is not achieved with statins. C MTP inhibitors: consider initiating lomitapide and mipomersen exclusively in patients with homozygous familial hypercholesterolemia. C 5. Nonpharmacologic interventions Lifestyle modifications: As per ESC 2020 guidelines, educate pediatric patients with familial hypercholesterolemia to adopt a proper diet. B As per CCS 2018 guidelines, consider advising a healthy lifestyle in patients with familial hypercholesterolemia. C 6. Specific circumstances Pediatric patients: as per CCS 2018 guidelines, consider initiating statin therapy usually between 8-10 years of age if LDL cholesterol remains 4.9 mmol/L or 4.1 mmol/L with a family history of premature ASCVD or other cardiovascular risk factors or risk conditions. C 7. Follow-up and surveillance Indications for referral: refer patients with homozygous familial hypercholesterolemia to a specialized lipid clinic. Obtain complete evaluation for genetic analysis, presence of ASCVD and initiate aggressive lipid-lowering therapies including consideration for extracorporeal LDL cholesterol removal, lomitapide and PCSK9 inhibitors. B Clinical findings Symptoms Past medical history Ankle pain Cerebrovascular disease Arthralgia Ischemic heart disease Peripheral vascular disease Family history Ocular exam Hypercholesterolemia Corneal arcus Musculoskeletal exam Xanthelasma palpebrarum https://web.pathway.md/diseases/rec3NR9IfcU05ASZA 5/7 6/29/23, 4:02 AM Familial hypercholesterolemia Pathway Tendon xanthomas Integument exam Lab findings Planar xanthomas Tuberous xanthomas serum LDL serum cholesterol Imaging findings Aortic stenosis Studies 2023 CLEAR Outcomes In patients who were unable or unwilling to take statins owing to unacceptable adverse effects and had or were at high risk for CVD, bempedoic acid was superior to placebo with respect to major adverse cardiovascular events. Steven E Nissen et al. N Engl J Med. 2023 Apr 13. 2019 CLEAR Harmony In patients taking the maximum tolerated dose of statin therapy with ASCVD, bempedoic acid was not superior to placebo with respect to adverse events. Kausik K Ray et al. N Engl J Med. 2019 Mar 14. References 1. Liam R Brunham, Isabelle Ruel, Sumayah Aljenedil et al. Canadian Cardiovascular Society Position Statement on Familial Hypercholesterolemia: Update 2018. Can J Cardiol. 2018 Dec;34 12 1553 1563. Open 2. Fran ois Mach, Colin Baigent, Alberico L Catapano et al. 2019 ESC/EAS Guidelines for the management of dyslipidaemias: lipid modification to reduce cardiovascular risk. Eur Heart J. 2020 Jan 1;41 1 111 188. Open 3. Glen J Pearson, George Thanassoulis, Todd J Anderson et al. 2021 Canadian Cardiovascular Society Guidelines for the Management of Dyslipidemia for the Prevention of Cardiovascular Disease in Adults. Can J Cardiol. 2021 Aug;37 8 1129 1150. Open 4. Scott M Grundy, Neil J Stone, Alison L Bailey et al. 2018 AHA/ACC/AACVPR/AAPA/ABC/ACPM/ADA/AGS/APhA/ASPC/NLA/PCNA Guideline on the Management of Blood Cholesterol: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. Circulation. 2019 Jun 18;139 25):e1082-e1143. Open 5. Michael Khoury, Jean-Luc Bigras, Elizabeth A Cummings et al. The Detection, Evaluation, and Management of Dyslipidemia in Children and Adolescents: A Canadian Cardiovascular Society/Canadian Pediatric Cardiology Association Clinical Practice Update. Can J Cardiol. 2022 Aug;38 8 1168 1179. Open 6. Anand Padmanabhan, Laura Connelly-Smith, Nicole Aqui et al. Guidelines on the Use of Therapeutic Apheresis in Clinical Practice Evidence-Based Approach from the Writing Committee of the American Society for Apheresis: The Eighth Special Issue. J Clin Apher. 2019 Jun;34 3 171 354. Open https://web.pathway.md/diseases/rec3NR9IfcU05ASZA 6/7 6/29/23, 4:02 AM Familial hypercholesterolemia Pathway https://web.pathway.md/diseases/rec3NR9IfcU05ASZA 7/7 |
Guideline sources The following summarized guidelines for the evaluation and management of familial Mediterranean fever (FMF) are prepared by our editorial team based on guidelines from the Egyptian Expert Group on Familial Mediterranean Fever (FMF-EEG 2022), the European League Against Rheumatism (EULAR 2016), the Brazilian Society of Rheumatology (BSR 2016), the Single Hub and Access Point for Pediatric Rheumatology in Europe (SHARE initiative 2015), and the American Academy of Allergy, Asthma & Immunology (AAAAI/ACAAI 2015). 1 2 3 4 5 Calculator Calculator Calculat Simplified Livneh diagnostic crit Tel-Hashomer diagnostic criteri Yalcink Guidelines 1. Screening and diagnosis Diagnosis: as per AAAAI 2015 guidelines, suspect FMF in patients presenting with recurrent and often prolonged fever attacks associated with serosal, cutaneous, and synovial https://web.pathway.md/diseases/recam5wEC5aGsxnfs 1/6 6/29/23, 4:01 AM Familial Mediterranean fever Pathway manifestations. B Diagnosis (RBR): Recognize that FMF is a genetic disease presenting an autosomal recessive trait and caused by a mutation in the MEFV gene. E Diagnose FMF based on clinical manifestations, characterized by recurrent febrile episodes associated with abdominal and chest pain, or arthritis of large joints. E 2. Diagnostic investigations Clinical assessment: assess for persistent systemic signs of inflammation in patients with suspected FMF in the absence of demonstrable infection or autoimmune disease. B Laboratory tests: Recognize that laboratory tests are not specific, with high serum levels of inflammatory proteins in the acute phase, but often, high levels are found even between attacks. E Consider assessing serum amyloid A levels for the monitoring of treatment effectiveness. E Genetic testing: as per SHARE initiative 2015 guidelines, consider supporting, but not excluding, the clinical diagnosis of FMF by genetic testing. C Show 7 more Genetic testing (RBR): Regard patients with p.Met694V mutation as being at risk of more severe disease with a high risk for amyloidosis. E Recognize that mutations and polymorphisms in genes other than MEFV may have an impact on the development of FMF or even on the severity of the disease. E 3. Medical management General principles (EEG-FMF): consider identifying possible triggers to help in preventing possible attacks by temporarily increasing the colchicine dose. C General principles: As per EULAR 2016 guidelines: Diagnose and treat FMF initially by a physician with experience in FMF. B Set a goal to reach complete control of unprovoked attacks and minimize subclinical inflammation in between attacks. B Colchicine: As per EULAR 2016 guidelines: Initiate colchicine therapy, in single or divided doses depending on tolerance and compliance, B as soon as a clinical diagnosis is made. A Take other possible causes into consideration when suspecting an attack. Continue the usual dose of colchicine during the attacks. B As per AAAAI 2015 guidelines, initiate colchicine as the mainstay of treatment for FMF. B https://web.pathway.md/diseases/recam5wEC5aGsxnfs 2/6 6/29/23, 4:01 AM Familial Mediterranean fever Pathway Colchicine (RBR): initiate colchicine to prevent acute inflammatory episodes and progression toward amyloidosis in patients with FMF. E Colchicine (EEG-FMF): initiate treatment of FMF at the time of clinical diagnosis, without waiting for genetic testing. Offer a period of 3-month observation if the attacks are not severe or frequent to confirm the pattern of the attacks before initiating treatment. A Show 3 more Nonsteroidal anti-inflammatory drugs: as per EULAR 2016 guidelines, adminisyer NSAIDs during FMF attacks. B Nonsteroidal anti-inflammatory drugs (EEG-FMF): Administer NSAIDs during acute attacks. B Consider administering NSAIDs for the treatment of leg pain. C Management of protracted febrile myalgia: As per EULAR 2016 guidelines: Administer corticosteroids for symptom relief in patients with protracted febrile myalgia. Consider administering NSAIDs and IL-1 inhibitors as a treatment option. B Consider administering NSAIDs for the management of exertional leg pain. C Management of protracted febrile myalgia (EEG-FMF): Administer low-dose corticosteroids for the management of symptoms in patients with protracted febrile myalgia. Consider administering NSAIDs and IL-1 inhibitors as another treatment option. B Consider administering NSAIDs for the management of leg pain. C Management of resistant disease: as per EULAR 2016 guidelines, initiate alternative biological treatments in compliant patients not responding to the maximum tolerated dose of colchicine. B Management of resistant disease (RBR): consider administering biologics as an alternative option in patients with FMF not responding or intolerant to colchicine. E Management of resistant disease (EEG-FMF): ensure optimal patient compliance and adherence to therapy in patients with colchicine resistance and failure. Increase the dose of colchicine up to a maximum tolerated (up to 2 mg in pediatric and 3 mg in adult patients). B Show 3 more Management of chronic arthritis: as per EULAR 2016 guidelines, consider initiating additional medications, such as DMARDs, intra-articular corticosteroid injections, or biologics, for chronic arthritis in patients with FMF. C Management of chronic arthritis (EEG-FMF): consider initiating DMARDs (such as methotrexate), biologics (anti-TNF), or administering intra-articular corticosteroid injections for the management of chronic arthritis in patients with FMF. C Management of AA amyloidosis: ensure good control of FMF and maintain normal serum amyloid A protein concentration between attacks in order to prevent amyloidosis. B Show 2 more https://web.pathway.md/diseases/recam5wEC5aGsxnfs 3/6 6/29/23, 4:01 AM Familial Mediterranean fever Pathway 4. Specific circumstances Pregnant patients: As per EULAR 2016 guidelines: Do not discontinue colchicine during conception, pregnancy, or lactation. Do not perform amniocentesis. D Do not discontinue colchicine before conception in male patients. Consider reducing the dose or discontinuing temporarily in the rare case of azoospermia or oligospermia proven to be related to colchicine. D Pregnant patients (EEG-FMF): Do not discontinue colchicine before conception, during pregnancy, or during lactation. D Do not discontinue colchicine in males before conception, except if conception is delayed. D Pediatric patients: Initiate colchicine with the following doses (not exceeding 2 mg/day, in single or divided doses depending on tolerance - divide if diarrhea or related abdominal pain - and patient compliance) in pediatric patients with FMF: Situation Guidance 0.5 mg/day < 5 years old 0.5-1.0 mg/day 5-10 years old 1.0-1.5 mg/day. B > 10 years old 5. Follow-up and surveillance Assessment of treatment response: Monitor patients for 3-6 months to assess for the therapeutic effect on attack frequency and severity (clinical diary and acute phase reactants). B Consider extending the follow-up frequency up to 1-year in controlled compliant patients. C Assessment of treatment compliance: recognize that there is a high rate of poor compliance with colchicine therapy because of concerns about lifelong use of the drug, adverse effects (such as bloating and diarrhea), fertility, embarrassment, and laziness. B Show 3 more Dose adjustments (EEG-FMF): increase the colchicine dose in patients with persistent attacks and/or of subclinical inflammation. B Show 2 more Dose adjustments: as per EULAR 2016 guidelines, increase the colchicine dose in case of the persistence of attacks or subclinical inflammation. B Show 3 more Monitoring for treatment-related adverse effects: as per EULAR 2016 guidelines, monitor response, toxicity, and compliance every 6 months. B Show 3 more https://web.pathway.md/diseases/recam5wEC5aGsxnfs 4/6 6/29/23, 4:01 AM Familial Mediterranean fever Pathway Monitoring for treatment-related adverse effects (EEG-FMF): recognize that diarrhea and vomiting are the most common side effects of treatment, and these effects are dose-dependent and more common at higher doses. B Show 3 more Clinical findings Patient demographics Symptoms Mediterranean countries Abdominal pain Anxiety Past medical history Arthralgia Chest pain CKD Fatigue Glomerulonephritis Fever Cardiac exam Hematuria Increased appetite Pericarditis Irritability Myalgia Abdominal exam Restlessness Peritoneal findings Scrotal pain Splenomegaly Ocular exam Genitourinary exam Scleritis Scrotal erythema Uveitis Scrotal swelling Respiratory exam Integument exam Pleuritis Palmar erythema Musculoskeletal exam Raynaud's phenomenon Subcutaneous nodules Arthritis Urticaria Polyarticular synovitis Imaging findings Lymphatic exam Atelectasis Lymphadenopathy Pericardial effusion Pericardial thickening Lab findings ESR serum CRP serum amyloid A serum fibrinogen urine protein Genetic testing Mefv gene mutation https://web.pathway.md/diseases/recam5wEC5aGsxnfs 5/6 6/29/23, 4:01 AM Familial Mediterranean fever Pathway References 1. Seza Ozen, Erkan Demirkaya, Burak Erer et al. EULAR recommendations for the management of familial Mediterranean fever. Ann Rheum Dis. 2016 Apr;75 4 644 51. Open 2. S. Salah, H. Lotfy, M. H. Abu-Zaid et al. Egyptian evidence-based consensus on clinical practice recommendations for the management of familial Mediterranean fever. Egypt Rheumatol Rehabil. 2022 Jun 21;49 39 . Open 3. Gabriella Giancane, Nienke M Ter Haar, Nico Wulffraat et al. Evidence-based recommendations for genetic diagnosis of familial Mediterranean fever. Ann Rheum Dis. 2015 Apr;74 4 635 41. Open 4. Maria Teresa R A Terreri, Wanderley Marques Bernardo, Claudio Arnaldo Len et al. Guidelines for the management and treatment of periodic fever syndromes familial Mediterranean fever. Rev Bras Reumatol Engl Ed. 2016 Jan-Feb;56 1 37 43. Open 5. Francisco A Bonilla, David A Khan, Zuhair K Ballas et al. Practice parameter for the diagnosis and management of primary immunodeficiency. J Allergy Clin Immunol. 2015 Nov;136 5 1186 205.e1 78. Open 6. V ronique Hentgen, Gilles Grateau, Isabelle Kone-Paut et al. Evidence-based recommendations for the practical management of Familial Mediterranean Fever. Semin Arthritis Rheum. 2013 Dec;43 3 387 91. Open 7. M Pras. Familial Mediterranean fever: from the clinical syndrome to the cloning of the pyrin gene. Scand J Rheumatol. 1998;27 2 92 7. Open 8. A Livneh, P Langevitz, D Zemer et al. Criteria for the diagnosis of familial Mediterranean fever. Arthritis Rheum. 1997 Oct;40 10 1879 85. Open 9. Fatos Yal inkaya, Seza Ozen, Zeynep Birsin Oz akar et al. A new set of criteria for the diagnosis of familial Mediterranean fever in childhood. Rheumatology Oxford). 2009 Apr;48 4 395 8. Open https://web.pathway.md/diseases/recam5wEC5aGsxnfs 6/6 |
Guideline sources The following summarized guidelines for the evaluation and management of febrile infant are prepared by our editorial team based on guidelines from the American Academy of Pediatrics (AAP 2021) and the American College of Emergency Physicians (ACEP 2016). 1 2 Guidelines 1. Diagnostic investigations Blood tests: consider obtaining inflammatory markers in well-appearing 8-21 days old infants. C Show 2 more Urine tests: As per AAP 2021 guidelines, obtain urinalysis with a specimen collected by catheterization or suprapubic aspiration of bladder in well-appearing febrile 8-21 days old infants. Obtain urine culture if urinalysis is positive. B Show 2 more As per ACEP 2016 guidelines, consider obtaining urinalysis and urine culture to identify UTI in well-appearing infants and pediatric patients aged 2 months to 2 years with a fever ( 38.0 C or 100.4 F), especially with a higher risk for UTI (such as females aged < 12 months, uncircumcised males, nonblack race, fever lasting > 24 hours, higher fever of 39 C, negative test result for respiratory pathogens, and no obvious source of infection). C Show 3 more Chest radiography: https://web.pathway.md/diseases/recABlXGA3XMLcZg5 1/3 6/29/23, 4:02 AM Febrile infant Pathway Consider obtaining a CXR in well-appearing immunocompetent febrile infants aged 2 months to 2 years presenting with cough, hypoxia, rales, high fever, fever duration > 48 hours, or tachycardia and tachypnea out of proportion to fever, in the absence of an obvious source of infection. C Do not obtain a CXR in well-appearing immunocompetent febrile infants aged 2 months to 2 years presenting with wheezing or a high likelihood of bronchiolitis. D 2. Diagnostic procedures Cerebrospinal fluid analysis: As per AAP 2021 guidelines, obtain CSF analysis (WBC, protein, glucose, Gram stain) and bacterial culture in well-appearing febrile 8-21 days old infants. B Show 4 more As per ACEP 2016 guidelines: Consider performing a lumbar puncture in full-term well-appearing febrile 29-90 days old infants. C Avoid performing lumbar puncture in full-term well-appearing febrile 29-90 days old infants diagnosed with a viral illness, given the lower risk for meningitis. Withhold antibiotics if lumbar puncture is deferred unless another bacterial source is identified. D 3. Medical management Setting of care: hospitalize well-appearing febrile 22-28 days old infants in a facility with nurses and staff experienced in the care of neonates/young infants when CSF is not obtained or is uninterpretable. B Show 6 more Antibiotic therapy (general principles): obtain active monitoring in well-appearing febrile 8- 21 days old infants while awaiting results of bacterial cultures in a hospital setting with nurses and staff experienced in the care of neonates/young infants. B Show 2 more Antibiotic therapy (parenteral): administer parenteral antibiotics in well-appearing febrile 8- 21 days old infants. B Show 6 more Antibiotic therapy (oral): Administer oral antibiotics in well-appearing febrile 29-60 days old infants if all of the following are met: CSF analysis, if obtained, is normal urinalysis is positive all obtained inflammatory markers are normal. B Discontinue parenteral antibiotics, if started, and initiate or continue oral antibiotic therapy in well-appearing febrile 29-60 days old infants with UTI managed at home if all of the following are met: urine culture is positive https://web.pathway.md/diseases/recABlXGA3XMLcZg5 2/3 6/29/23, 4:02 AM Febrile infant Pathway all other bacterial cultures are negative at 24-36 hours the infant is clinically well or improving (such as fever, feeding). B Antibiotic therapy (discontinuation): Discontinue antibiotics in hospitalized 8-21 days old infants if all of the following are met: all culture results are negative at 24-36 hours or only positive for contaminants the infant continues to appear clinically well or is improving (such as fever, feeding) no other reasons for hospitalization. B Show 2 more 4. Follow-up and surveillance Hospital discharge criteria: Discharge hospitalized 8-21 days old infants if all of the following are met: culture results are negative for 24-36 hours or only positive for contaminants the infant continues to appear clinically well or is improving (such as fever, feeding) no other reasons for hospitalization. B Show 2 more References 1. Robert H Pantell, Kenneth B Roberts, William G Adams et al. Evaluation and Management of Well- Appearing Febrile Infants 8 to 60 Days Old. Pediatrics. 2021 Aug;148 2):e2021052228. Open 2. Mace SE, Gemme SR, Valente JH et al. Clinical Policy for Well-Appearing Infants and Children Younger Than 2 Years of Age Presenting to the Emergency Department With Fever. Ann Emerg Med. 2016 May;67 5 625 639.e13. Open 3. Borja Gomez, Santiago Mintegi, Silvia Bressan et al. Validation of the "Step-by-Step" Approach in the Management of Young Febrile Infants. Pediatrics. 2016 Aug;138 2):e20154381. Open https://web.pathway.md/diseases/recABlXGA3XMLcZg5 3/3 |
Guideline sources The following summarized guidelines for the evaluation and management of febrile neutropenia (FN) are prepared by our editorial team based on guidelines from the American Society of Clinical Oncology (ASCO 2023; 2018), the Infectious Diseases Society of America (IDSA/ASCO 2018), the European Society of Medical Oncology (ESMO 2016), and the Infectious Diseases Society of America (IDSA 2011). 1 2 3 4 5 6 6 6 6 Definition FN is a common consequence of cancer chemotherapy characterized by fever and underlying infection. 6 Epidemiology FN is caused by antineoplastic therapy (side-effect leading to myelosuppression or mucositis) in cancer patients. 6 Disease course Clinical manifestations of FN include fever, underlying infection associated with bacteremia, sepsis, hemodynamic instability, and death. 6 Prognosis and risk of recurrence https://web.pathway.md/diseases/recf47ynJ22uvcc9p 1/10 6/29/23, 4:02 AM Febrile neutropenia Pathway The 30-day mortality associated with FN ranges from 6% to 10%. 6 Calculator Calculator Calculat Clinical Index of Stable Febrile N Diagnostic criteria for febrile ne MASCC Guidelines 1. Screening and diagnosis Indications for screening: Assess the risk of FN (in consultation with infectious disease specialists as needed) systematically, taking into account the following factors: Situation Guidance Advanced age Patient-related factors Performance status Nutritional status Prior episode of FN Comorbidities Cancer diagnosis Cancer-related factors Cancer stage Remission status Cancer treatment response Cytotoxic regimen Treatment-related factors Dose intensity Degree and duration of gastrointestinal and/or oral mucositis Degree and duration of cytopenia. B Diagnostic criteria: As per ASCO 2018 guidelines, define fever in patients with neutropenia as a single oral temperature of 38.3 C (101 F) or a temperature of 38.0 C (100.4 F) sustained over a 1-hour period. Show 2 more As per IDSA 2011 guidelines: Define fever as a single oral temperature measurement of 38.3 C (101 F) or a temperature of 38.0 C (100.4 F) sustained over a 1-hour period. Define neutropenia as an absolute neutrophil count of < 500/ L or an absolute neutrophil count expected to decrease to < 500/ L during the next 48 hours. https://web.pathway.md/diseases/recf47ynJ22uvcc9p 2/10 6/29/23, 4:02 AM Febrile neutropenia Pathway 2. Classification and risk stratification Risk assessment (general principles): assess the risk of severe infectious complications as part of the initial evaluation of patients with FN. B Show 2 more Risk assessment, scores: As per ASCO 2018 guidelines: Use a validated predictive tool, such as the MASCC score or the Talcott's rules, to identify patients with FN eligible for outpatient management. B Consider using the CISNE as an additional tool to determine the risk of major complications in clinically stable patients with solid tumors after mild-to-moderate-intensity chemotherapy, assuming proximity to an appropriate medical facility able to provide 24- hour access. C As per ESMO 2016 guidelines, use a validated predictive tool, such as the MASCC score, to assess the risk of complications in patients with FN. A As per IDSA 2011 guidelines, consider obtaining formal risk stratification using the MASCC scoring system in patients with FN. B 3. Diagnostic investigations Clinical assessment: assume that fever in a patient with neutropenia from cancer therapy is the result of an infection in the absence of an alternative explanation. Attempt to establish clinical and microbiologic diagnoses that may affect antibacterial choice and prognosis. B Show 2 more Routine laboratory tests: Obtain the following laboratory tests as part of the initial evaluation of patients with FN: CBC with differential leukocyte and platelet count serum electrolytes, creatinine, BUN, and lactate liver biochemical tests, including TBIL, ALP, and transaminases. B Microbiological cultures: Obtain at least 2 sets of blood cultures from different anatomic sites (including a peripheral site and the lumen of a central venous catheter, if present) in patients with FN. B Obtain cultures from other sites (such as urine, lower respiratory tract, CSF, stool, or wounds) as clinically indicated. B Respiratory virus testing: Obtain a nasopharyngeal swab for the detection of influenza virus in patients with influenza- like illness, as evidenced by sudden onset of a respiratory illness characterized by fever, cough, and 1 of the following: malaise, sore throat, coryza, arthralgias, or myalgias. B Consider obtaining an expanded viral panel for the detection of additional respiratory viruses (such as influenza virus, parainfluenza virus, adenovirus, coronavirus, RSV, human metapneumovirus, enteroviruses, rhinovirus) in patients with symptoms of a respiratory viral illness in the setting of hematologic malignancy or hematopoietic stem cell transplant. C https://web.pathway.md/diseases/recf47ynJ22uvcc9p 3/10 6/29/23, 4:02 AM Febrile neutropenia Pathway Chest imaging: obtain chest imaging in patients with signs and/or symptoms of lower respiratory tract infection. B 4. Medical management Setting of care, outpatient: As per ASCO 2018 guidelines, use clinical judgment when selecting candidates for outpatient management. B As per ESMO 2016 guidelines, offer outpatient management in patients with FN at low risk of complications, if adequate follow-up is available. A As per IDSA 2011 guidelines, consider offering outpatient management in carefully selected patients with FN at low risk of complications. B Setting of care, inpatient: As per ASCO 2018 guidelines: Assess patients for admission to the hospital if any of the following occur: fever not resolving after 2-3 days of an initial, empirical, broad-spectrum antibiotic regimen fever recurring after a period of defervescence new signs or symptoms of infection use of oral medications is no longer possible or tolerable change in the empirical regimen or an additional antimicrobial drug becomes necessary microbiologic tests identify species not susceptible to the initial regimen. B Consider assessing low-risk outpatients with FN not defervescing after 2-3 days of an initial, empirical, broad-spectrum antibiotic regimen for admission to the hospital. C As per ESMO 2016 guidelines, hospitalize all patients with FN at high risk of complications. A As per IDSA 2011 guidelines, hospitalize all patients with FN at high risk of complications, if they are not already inpatients. B Show 3 more Setting of care (discharge): Ensure that patients with FN eligible for discharge and outpatient management also meet the following psychosocial and logistic requirements: residence 1 hour or 48 km ( 30 miles) from clinic or hospital primary care physician or oncologist agrees to outpatient management ability to comply with logistic requirements, including frequent clinic visits presence of family member or caregiver at home 24 hours/day access to a telephone and transportation 24 hours/day no history of noncompliance with treatment protocols. B Obtain monitoring as follows in outpatients with FN: frequent evaluation for at least 3 days in a clinic or at home daily or frequent telephone contact to verify by home thermometry that the fever resolves https://web.pathway.md/diseases/recf47ynJ22uvcc9p 4/10 6/29/23, 4:02 AM Febrile neutropenia Pathway monitoring of absolute neutrophil count and platelet count for myeloid reconstitution frequent return visits to the clinic. B Antibiotic therapy, low-risk patients: As per ASCO 2018 guidelines, administer the first dose of empiric antibiotic therapy in the clinic, emergency department, or hospital department, after fever has been documented and pretreatment blood samples have been collected, in patients with FN eligible for outpatient management. B Show 2 more As per ESMO 2016 guidelines: Initiate oral empirical therapy in low-risk patients, including patients with anticipated brief ( 7 days duration) neutropenic periods or no or few comorbidities. B Consider initiating oral and/or outpatient empirical antibiotic therapy in carefully selected low-risk patients. B As per IDSA 2011 guidelines, administer initial oral or IV empirical antibiotic doses in low-risk patients in a clinic or hospital setting. Consider transitioning to outpatient oral or IV treatment if they meet specific clinical criteria. A Show 2 more Antibiotic therapy (high-risk patients): initiate monotherapy with an anti-pseudomonal - lactam agent (such as cefepime), a carbapenem (meropenem or imipenem-cilastatin), or piperacillin-tazobactam for IV empirical antibiotic therapy in high-risk patients with FN. A Show 5 more Antibiotic therapy (modifications): guide modifications to the initial antibiotic regimen based on clinical and microbiologic data. B Show 7 more Antibiotic therapy (duration): continue appropriate antibiotics for at least the duration of neutropenia, until the absolute neutrophil count is 500/ L, or longer if indicated according to the particular organism and site of infection. B Show 2 more Antifungal therapy (low-risk patients): do not administer empirical antifungal therapy routinely in low-risk patients, as the risk of invasive fungal infection is low. D Antifungal therapy (high-risk patients): initiate empirical antifungal therapy and investigate for invasive fungal infections in patients with persistent or recurrent fever after 4-7 days of antibiotic therapy, if the duration of neutropenia is expected to be > 7 days. A Show 3 more Antiviral therapy: administer neuraminidase inhibitors in patients with influenza virus infection, if the infecting strain is susceptible. B Show 3 more Granulocyte colony-stimulating factors: do not use G-CSFs for the treatment of established FN. D 5. Specific circumstances https://web.pathway.md/diseases/recf47ynJ22uvcc9p 5/10 6/29/23, 4:02 AM Febrile neutropenia Pathway Pediatric patients (risk assessment): adopt a validated risk stratification strategy and incorporate it into routine clinical management. B Show 2 more Pediatric patients (diagnostic evaluation): obtain blood cultures at the onset of FN from all lumens of central venous catheters. B Consider obtaining peripheral blood cultures concurrent with central venous catheter cultures. B Show 5 more Pediatric patients (antibiotic therapy, low-risk patients): Consider offering initial or step-down outpatient management if the infrastructure is in place to ensure careful monitoring and follow-up. C Consider initiating oral antibacterial therapy if the patient is able to reliably tolerate this route of administration. C Pediatric patients (antibiotic therapy, high-risk patients): Initiate monotherapy with an anti-pseudomonal -lactam, a fourth-generation cephalosporin, or a carbapenem as empiric antibacterial therapy in pediatric high-risk FN. A Reserve the addition of a second anti-Gram-negative agent or a glycopeptide for clinically unstable patients with suspected resistant infection, or for centers with a high rate of resistant pathogens. B Pediatric patients (antibiotic therapy, modification and discontinuation): discontinue double coverage for Gram-negative infection or empiric glycopeptide (if initiated) after 24-72 hours in patients responding to initial empiric antibacterial therapy, if there is no specific microbiologic indication to continue combination therapy. B Show 4 more Pediatric patients (antifungal therapy, biomarkers): Avoid obtaining serum galactomannan to guide empiric antifungal management for prolonged FN ( 96 hours) in patients at high risk for invasive fungal disease. D Do not obtain -D-glucan or fungal PCR testing in blood to guide empiric antifungal management for prolonged FN ( 96 hours) in patients at high risk for invasive fungal disease. D Pediatric patients (antifungal therapy, low-risk patients): consider withholding empiric antifungal therapy in patients at low risk for invasive fungal disease with prolonged ( 96 hours) fever and neutropenia. C Pediatric patients (antifungal therapy, high-risk patients): Administer caspofungin or liposomal amphotericin B for empiric antifungal therapy, unless a preemptive antifungal therapy approach is chosen, in patients at high risk for invasive fungal disease with prolonged ( 96 hours) FN unresponsive to broad-spectrum antibacterial therapy. A Consider implementing a preemptive antifungal therapy approach by deferring empiric antifungal therapy and initiating antifungal therapy only if evaluation suggests or indicates invasive fungal disease in non-HSCT patients at high risk for invasive fungal disease not receiving anti-mold prophylaxis with prolonged ( 96 hours) FN unresponsive to broad- spectrum antibacterial therapy. C https://web.pathway.md/diseases/recf47ynJ22uvcc9p 6/10 6/29/23, 4:02 AM Febrile neutropenia Pathway Patients with central venous catheters: exercise hand hygiene, maximal sterile barrier precautions, and cutaneous antisepsis with chlorhexidine during central venous catheter insertion for all central venous catheter insertions. A Show 5 more 6. Preventative measures Infection prevention: As per ASCO 2018 guidelines: Ensure that all healthcare workers comply with hand hygiene and respiratory hygiene/cough etiquette guidelines to reduce the risk for aerosol- and direct or indirect contact-based transmission of pathogenic microorganisms in the healthcare setting. B Advise outpatients with neutropenia from cancer therapy to avoid prolonged contact with environments that have high concentrations of airborne fungal spores (such as construction and demolition sites, intensive exposure to soil through gardening or digging, or household renovation). B As per IDSA 2011 guidelines, follow proper hand hygiene to prevent transmission of infection in the hospital. B Show 4 more Influenza vaccination: As per ASCO 2018 guidelines, provide yearly influenza vaccination with an inactivated vaccine in all patients receiving chemotherapy for malignancy and all family and household contacts and healthcare providers. B As per IDSA 2011 guidelines: Provide yearly influenza immunization with an inactivated influenza vaccine in all patients being treated for cancer. B Consider immunizing patients between chemotherapy cycles, > 7 days after the last treatment, or > 2 weeks before chemotherapy starts, in order to maximize the serologic response to immunization. C Antibiotic prophylaxis: As per ASCO 2018 guidelines: Administer antibiotic prophylaxis with a fluoroquinolone in patients at high risk for FN or profound, protracted neutropenia (most patients with acute myeloid leukemia/myelodysplastic syndromes or HSCT recipients treated with myeloablative conditioning regimens). Do not administer antibiotic prophylaxis routinely in patients with solid tumors. B Administer antimicrobial prophylaxis wutg TMP-SMX in patients receiving chemotherapy regimens associated with > 3.5% risk for pneumonia from P. jirovecii (such as containing 20 mg prednisone equivalents daily for 1 month or purine analogs). A As per IDSA 2011 guidelines, consider administering fluoroquinolone (levofloxacin or ciprofloxacin) prophylaxis in high-risk patients with expected prolonged and profound neutropenia (absolute neutrophil count 100/ L for > 7 days). B Show 3 more https://web.pathway.md/diseases/recf47ynJ22uvcc9p 7/10 6/29/23, 4:02 AM Febrile neutropenia Pathway Antifungal prophylaxis: As per ASCO 2018 guidelines, administer antifungal prophylaxis with an oral triazole or parenteral echinocandin in patients at risk for profound, protracted neutropenia (most patients with acute myeloid leukemia/myelodysplastic syndrome or HSCT recipients). Do not administer antifungal prophylaxis routinely in patients with solid tumors. B As per IDSA 2011 guidelines, administer antifungal prophylaxis with fluconazole, itraconazole, voriconazole, posaconazole, micafungin, or caspofungin against Candida infection in patients with a substantial risk of invasive candidal infection (such as allogeneic HSCT recipients or patients undergoing intensive remission-induction or salvage-induction chemotherapy for acute leukemia). A Show 3 more Antiviral prophylaxis: As per ASCO 2018 guidelines: Administer antiviral prophylaxis with a nucleoside analog (such as acyclovir) in HSV- seropositive patients undergoing allogeneic HSCT or leukemia induction therapy. A Administer treatment with a nucleoside reverse transcription inhibitor (such as entecavir or tenofovir) in patients at high risk of HBV reactivation. B As per IDSA 2011 guidelines, administer antiviral prophylaxis with acyclovir against HSV in HSV-seropositive patients undergoing allogeneic HSCT or leukemia induction therapy. A Granulocyte colony-stimulating factors: As per ESMO 2016 guidelines, administer G-CSF to prevent FN in patients > 60 years of age, patients with serious comorbidities, or receiving chemotherapy regimens with a high risk (> 20%) of FN. A As per IDSA 2011 guidelines, consider administering G-CSFs to prevent FN in patients with an anticipated risk of FN 20%. B Clinical findings Symptoms Past medical history Abdominal pain BMT Anal pain Exposure to chemotherapy Burning on urination Exposure to radiation therapy Chills Human immunodeficiency virus infection Cough Immunocompromising condition Diarrhea Leukemia Dysuria Lymphoma Fever Multiple myeloma Mouth sores Sore throat Respiratory exam Sweating Toothache Dyspnea Vaginal itching Hematological findings https://web.pathway.md/diseases/recf47ynJ22uvcc9p 8/10 6/29/23, 4:02 AM Febrile neutropenia Pathway blood leukocyte count Vital signs blood neutrophil count HR body temperature systolic BP Gynecologic exam Studies 2022 SHORT In adult patients who had fever of unknown origin during high-risk neutropenia and were treated for hematological malignancy, short treatment was noninferior to extended treatment with respect to carbapenem -sensitive infection or recurrent fever. Nick A de Jonge et al. Lancet Haematol. 2022 Jun 9. References 1. Randy A Taplitz, Erin B Kennedy, Eric J Bow et al. Outpatient Management of Fever and Neutropenia in Adults Treated for Malignancy: American Society of Clinical Oncology and Infectious Diseases Society of America Clinical Practice Guideline Update. J Clin Oncol. 2018 May 10;36 14 1443 1453. Open 2. Klastersky J, de Naurois J, Rolston K et al. Management of febrile neutropaenia: ESMO Clinical Practice Guidelines. Ann Oncol. 2016 Sep;27(suppl 5):v111-v118. Open 3. Freifeld AG, Bow EJ, Sepkowitz KA et al. Clinical practice guideline for the use of antimicrobial agents in neutropenic patients with cancer: 2010 update by the Infectious Diseases Society of America. Clin Infect Dis. 2011 Feb 15;52 4):e56 93. Open 4. Thomas Lehrnbecher, Paula D Robinson, Roland A Ammann et al. Guideline for the Management of Fever and Neutropenia in Pediatric Patients With Cancer and Hematopoietic Cell Transplantation Recipients: 2023 Update. J Clin Oncol. 2023 Jan 23;JCO2202224. Open 5. Randy A Taplitz, Erin B Kennedy, Eric J Bow et al. Antimicrobial Prophylaxis for Adult Patients With Cancer-Related Immunosuppression: ASCO and IDSA Clinical Practice Guideline Update. J Clin Oncol. 2018 Oct 20;36 30 3043 3054. Open 6. Fatemeh Karimi, Farzaneh Ashrafi, Azadeh Moghaddas et al. Management of Febrile Neutropenia: A Description of Clinical and Microbiological Findings by Focusing on Risk Factors and Pitfalls. J Res Pharm Pract. 2018 Jul-Sep; 7 3 147 156. Open 7. American Society of Clinical Oncology. Choosing Wisely ASCO recommendations. Choosing Wisely. 2012. Open 8. Crawford J, Becker PS, Armitage JO et al. Myeloid Growth Factors, Version 2.2017, NCCN Clinical Practice Guidelines in Oncology. J Natl Compr Canc Netw. 2017 Dec;15 12 1520 1541. Open 9. Almarie Uys, Bernardo L Rapoport, Ronald Anderson. Febrile neutropenia: a prospective study to validate the Multinational Association of Supportive Care of Cancer MASCC risk-index score. Support Care Cancer. 2004 Aug;12 8 555 60. Open https://web.pathway.md/diseases/recf47ynJ22uvcc9p 9/10 6/29/23, 4:02 AM Febrile neutropenia Pathway 10. Nick A de Jonge, Jonne J Sikkens, Sonja Zweegman et al. Short versus extended treatment with a carbapenem in patients with high-risk fever of unknown origin during neutropenia: a non-inferiority, open-label, multicentre, randomised trial. Lancet Haematol. 2022 Jun 9;S2352 3026 22 00145 4. Open 11. Kochanek M, Schalk E, von Bergwelt-Baildon M et al. Management of sepsis in neutropenic cancer patients: 2018 guidelines from the Infectious Diseases Working Party AGIHO and Intensive Care Working Party (iCHOP of the German Society of Hematology and Medical Oncology DGHO . Ann Hematol. 2019 May;98 5 1051 1069. Open 12. Thomas Lehrnbecher, Paula Robinson, Brian Fisher et al. Guideline for the Management of Fever and Neutropenia in Children With Cancer and Hematopoietic Stem-Cell Transplantation Recipients: 2017 Update. J Clin Oncol. 2017 Jun 20;35 18 2082 2094. Open 13. Thomas J Smith, Kari Bohlke, Gary H Lyman et al. Recommendations for the Use of WBC Growth Factors: American Society of Clinical Oncology Clinical Practice Guideline Update. J Clin Oncol. 2015 Oct 1;33 28 3199 212. Open https://web.pathway.md/diseases/recf47ynJ22uvcc9p 10/10 |
Guideline sources The following summarized guidelines for the evaluation and management of fecal incontinence are prepared by our editorial team based on guidelines from the American Society of Colon and Rectal Surgeons (ASCRS 2023), the American College of Gastroenterology (ACG 2021), the American College of Obstetricians and Gynecologists (ACOG 2019), and the National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK 2015). 1 2 3 4 Calculator Wexner/Cleveland Clinic Fecal In Guidelines 1. Screening and diagnosis Indications for screening: Obtain screen for fecal incontinence in female patients with risk factors, such as: age > 50 years residence in long-term care facilities https://web.pathway.md/diseases/recfTssZTNoahWkPe 1/5 6/29/23, 4:03 AM Fecal incontinence Pathway residence in long term care facilities pelvic floor disorders history of obstetric anal sphincter injuries history of pelvic irradiation engagement in anal intercourse urinary incontinence chronic diarrhea rectal urgency diabetes obesity. B 2. Diagnostic investigations History and physical examination: As per ASCRS 2023 guidelines, elicit a history to help determine the etiology of incontinence, including specific risk factors for incontinence, and characterize the duration and severity of symptoms. E Show 2 more As per ACOG 2019 guidelines, elicit a complete medical history, assess symptoms and perform physical examination of the rectal, vaginal and perineal areas in female patients reporting fecal incontinence symptoms. B Anorectal physiology testing: As per ASCRS 2023 guidelines, consider obtaining anorectal physiology testing (manometry, anorectal sensation, volume tolerance, and compliance) to help define the elements of dysfunction and guide management. C As per ACOG 2019 guidelines, do not obtain anorectal manometry during routine evaluation of female patients with fecal incontinence. D Pudendal nerve conduction studies: As per ASCRS 2023 guidelines, do not obtain pudendal nerve terminal motor latency testing routinely in patients with fecal incontinence. D As per ACOG 2019 guidelines, do not obtain pudendal nerve terminal motor latency testing during routine evaluation of female patients with fecal incontinence. D Diagnostic imaging: As per ASCRS 2023 guidelines, consider obtaining endoanal ultrasound to evaluate sphincter anatomy when planning a sphincter repair. C As per ACOG 2019 guidelines, do not obtain anal sphincter imaging or defecography during routine evaluation of female patients with fecal incontinence. D Laboratory tests: do not obtain specific laboratory tests during initial evaluation of fecal incontinence in female patients unless diarrheal infectious processes are suspected. D 3. Diagnostic procedures https://web.pathway.md/diseases/recfTssZTNoahWkPe 2/5 6/29/23, 4:03 AM Fecal incontinence Pathway Lower gastrointestinal endoscopy: As per ASCRS 2023 guidelines, perform endoscopy according to established screening guidelines and in patients presenting with symptoms warranting further evaluation (changes in bowel habits, bleeding). B As per ACOG 2019 guidelines, consider performing colonoscopy in female patients presenting with fecal incontinence and a change in bowel habits, especially if accompanied by any "red flag" symptoms, such as unexplained weight loss, abdominal pain, rectal bleeding, melena, or anemia. C 4. Medical management Conservative management: As per ASCRS 2023 guidelines, offer dietary and medical management as first-line therapy in patients with fecal incontinence. B As per ACG 2021 guidelines, offer antidiarrheal drugs (such as loperamide, diphenoxylate with atropine, bile salt binding agents, anticholinergic agents, and clonidine) when fecal incontinence is accompanied by diarrhea. B As per ACOG 2019 guidelines: Consider offering conservative measures, such as dietary modifications, bowel scheduling, fiber supplementation, and stool-modifying agents (including antimotility agents and laxatives) in female patients with fecal incontinence. C Offer non-surgical treatments for initial management of female patients with fecal incontinence due to the modest short-term efficacy and a low risk of adverse events, except for patients with fistulae or rectal prolapse. B 5. Nonpharmacologic interventions Dietary modifications: Consider advising fiber supplementation in female patients with fecal incontinence. C Offer dietary manipulation (food diaries and dietary changes) and bowel schedules (regular toileting) in conjunction with other treatment in female patients with fecal incontinence. B Pelvic floor exercises: consider offering pelvic floor muscle exercises, with or without biofeedback, to strengthen the anal sphincter and levator ani muscles in female patients with fecal incontinence. C Bowel training: consider offering bowel training programs to improve rectal evacuation in selected patients with fecal incontinence. C Anal and vaginal mechanical inserts: As per ASCRS 2023 guidelines, do not offer vaginal or anal mechanical insert devices routinely for fecal incontinence. D As per ACG 2021 guidelines, consider offering anal plugs, vaginal balloons, and other devices to impede defecation in selected patients not responding to conservative measures and biofeedback. C https://web.pathway.md/diseases/recfTssZTNoahWkPe 3/5 6/29/23, 4:03 AM Fecal incontinence Pathway 6. Therapeutic procedures Biofeedback therapy: As per ASCRS 2023 guidelines, consider offering biofeedback therapy in patients with fecal incontinence. C As per ACG 2021 guidelines, offer biofeedback (pelvic floor rehabilitative techniques with visual or auditory feedback) in patients with fecal incontinence not responding to education and conservative measures. B Perianal injection of bulking agents: As per ASCRS 2023 guidelines, do not offer injections of biocompatible bulking agents into the anal canal routinely for the treatment of fecal Incontinence. D As per ACG 2021 guidelines, consider offering injection of bulking agents such as dextranomer sodium in selected patients with fecal incontinence not responding to conservative measures or biofeedback. C As per ACOG 2019 guidelines, consider offering injection of anal spicter bulking agents as a short-term treatment option to decrease fecal inconinence episodes in female patients with fecal incontinence failed more conservative treatments. C Sacral nerve stimulation: As per ASCRS 2023 guidelines, consider performing sacral neuromodulation as a first-line surgical option in patients with fecal incontinence with or without sphincter defects. C As per ACG 2021 guidelines, offer sacral nerve stimulation in patients with moderate-to- severe fecal incontinence failed conservative measures, biofeedback, and other low-cost, low-risk techniques. B As per ACOG 2019 guidelines, consider offering sacral nerve stimulation as a surgical treatment option in female patients with fecal incontinence with or without anal sphincter disruption failed conservative treatments. C Transanal radiofrequency: do not offer temperature-controlled radiofrequency energy applied to the sphincter complex for the treatment of fecal incontinence. D 7. Surgical interventions Indications for surgery: As per ASCRS 2023 guidelines, consider performing anal sphincteroplasty in patients with a defect in the external anal sphincter, recognizing that clinical results often deteriorate over time. C Show 3 more As per ACG 2021 guidelines: Consider performing anal sphincteroplasty in patients with acute injuries to the anal sphincters. C Consider offering an end stoma surgery in patients with severe fecal incontinence not responding to other treatments. C https://web.pathway.md/diseases/recfTssZTNoahWkPe 4/5 6/29/23, 4:03 AM Fecal incontinence Pathway As per ACOG 2019 guidelines, offer further evaluation and treatment by a healthcare provider with expertise in pelvic surgery in female patients being candidates for surgical therapy (such as patients with rectovaginal fistulas or rectal prolapse) or if not responding to conservative treatments. B Show 2 more References 1. Arnold Wald, Adil E Bharucha, Berkeley Limketkai et al. ACG Clinical Guidelines: Management of Benign Anorectal Disorders. Am J Gastroenterol. 2021 Oct 1;116 10 1987 2008. Open 2. Bordeianou, Liliana G M.D., M.P.H. et al. The American Society of Colon and Rectal Surgeons Clinical Practice Guideline for the Treatment of Fecal Incontinence. Dis Colon Rectum. 2023 Feb 14. Open 3. No authors listed. ACOG Practice Bulletin No. 210 Fecal Incontinence. Obstet Gynecol. 2019 Apr;133 4):e260-e273. Open 4. William E Whitehead, Satish S C Rao, Ann Lowry et al. Treatment of fecal incontinence: state of the science summary for the National Institute of Diabetes and Digestive and Kidney Diseases workshop. Am J Gastroenterol. 2015 Jan;110 1 138 46; quiz 147. Open 5. Expert Panel on GYN and OB Imaging: Gaurav Khatri, MD, Priyadarshani R. Bhosale et al. American College of Radiology ACR Appropriateness Criteria Pelvic Floor Dysfunction in Females. ACR. 2021. Open 6. Ian M Paquette, Madhulika G Varma, Andreas M Kaiser et al. The American Society of Colon and Rectal Surgeons' Clinical Practice Guideline for the Treatment of Fecal Incontinence. Dis Colon Rectum. 2015 Jul;58 7 623 36. Open 7. Raimund Stein, Guy Bogaert, Hasan S Dogan et al. EAU/ESPU guidelines on the management of neurogenic bladder in children and adolescent part II operative management. Neurourol Urodyn. 2020 Feb;39 2 498 506. Open 8. J M Jorge, S D Wexner. Etiology and management of fecal incontinence. Dis Colon Rectum. 1993 Jan;36 1 77 97. Open https://web.pathway.md/diseases/recfTssZTNoahWkPe 5/5 |
Guideline sources The following summarized guidelines for the evaluation and management of female genital cosmetic surgery are prepared by our editorial team based on guidelines from the Society of Obstetricians and Gynaecologists of Canada (SOGC 2022), the Japanese Society for Neonatal Screening (JSNS/JSPU/JES/JSPE 2022), and the American College of Obstetricians and Gynecologists (ACOG 2020; 2017). 1 2 3 4 Guidelines 1. Diagnostic investigations Clinical assessment: As per SOGC 2022 guidelines, elicit a complete medical, sexual, and gynecological history, and confirm the absence of any major sexual or psychological dysfunction, including body dysmorphic disorder, in females requesting vaginal cosmetic procedures. Rule out any possibility of coercion or exploitation. A As per ACOG 2020 guidelines, be aware to recognize sexual function disorders, depression, anxiety, and other psychiatric conditions. Assess females, if indicated, for body dysmorphic disorder. Refer for evaluation before surgery in case of suspected psychological concerns. E https://web.pathway.md/diseases/rec1Jt8ljLRNR2nWA 1/3 6/29/23, 4:03 AM Female genital cosmetic surgery Pathway 2. Therapeutic procedures Vaginal laser therapy: do not offer laser therapy for genitourinary syndrome of menopause or cosmetic genital indications. D 3. Surgical interventions Indications for surgery: insufficient evidence to recommend any female genital cosmetic surgery or procedure to improve sexual satisfaction and/or self-image. Do not promote these surgeries for the enhancement of sexual function or self-image when choosing to proceed with these cosmetic procedures. I Show 2 more 4. Specific circumstances Adolescent patients: As per SOGC 2022 guidelines, ensure that physicians seeing adolescents requesting female genital cosmetic surgery have additional expertise in counseling adolescents. B As per ACOG 2017 guidelines, educate and reassure regarding normal variation in anatomy, growth, and development as the first step in adolescents seeking medical treatment. E Show 3 more Patients with congenital adrenal hyperplasia: perform clitoroplasty in patients with cosmetic problems and clitoromegaly. Prefer a neurovascular bundle-preserving procedure as standard surgery. B 5. Patient education General counseling: As per SOGC 2022 guidelines, educate females about their anatomy and help them appreciate individual variations, including transitions through the reproductive life cycle. B Show 2 more As per ACOG 2020 guidelines, inform females that surgery or procedures altering sexual appearance or function (excluding procedures performed for clinical indications, such as clinically diagnosed female sexual dysfunction, pain with intercourse, interference in athletic activities, previous obstetric or straddle injury, reversing female genital cutting, vaginal prolapse, incontinence, or gender affirmation surgery) are not medically indicated, pose a substantial risk, and their safety and effectiveness have not been established. E Show 2 more 6. Quality improvement Advertising: https://web.pathway.md/diseases/rec1Jt8ljLRNR2nWA 2/3 6/29/23, 4:03 AM Female genital cosmetic surgery Pathway As per SOGC 2022 guidelines, be cautious in advertising female genital cosmetic surgeries, to ensure such advertising is factual and not misleading. A As per ACOG 2020 guidelines, ensure that advertisements in any media are accurate and not misleading or deceptive. Recognize that rebranding existing surgical procedures (many of which are similar to, if not the same as, the traditional anterior and posterior colporrhaphy) and marketing them as new cosmetic vaginal procedures is misleading. E References 1. Dorothy Shaw, Lisa Allen, Cynthia Chan et al. Guideline No. 423 Female Genital Cosmetic Surgery and Procedures. J Obstet Gynaecol Can. 2022 Feb;44 2 204 214.e1. Open 2. No authors listed. Elective Female Genital Cosmetic Surgery: ACOG Committee Opinion, Number 795. Obstet Gynecol. 2020 Jan;135 1):e36-e42. Open 3. Tomohiro Ishii, Kenichi Kashimada, Naoko Amano et al. Clinical guidelines for the diagnosis and treatment of 21-hydroxylase deficiency 2021 revision). Clin Pediatr Endocrinol. 2022;31 3 116 143. Open 4. No authors listed. Committee Opinion No. 686 Breast and Labial Surgery in Adolescents. Obstet Gynecol. 2017 Jan;129 1):e17-e19. Open https://web.pathway.md/diseases/rec1Jt8ljLRNR2nWA 3/3 |
Guideline sources The following summarized guidelines for the evaluation and management of fertility preservation in patients with cancer are prepared by our editorial team based on guidelines from the Society of Obstetricians and Gynaecologists of Canada (SOGC 2023), the European Society of Human Reproduction and Embryology (ESHRE 2020), the European Society of Medical Oncology (ESMO 2020), the American Society of Clinical Oncology (ASCO 2018), and the Spanish Association of Medical Oncology (SEOM 2016). 1 2 3 4 5 6 6 7 8 Definition Fertility preservation for patients with cancer is a therapeutic option designed to maximize the reproductive future of oncological patients, including oocyte/embryo cryopreservation for female patients and sperm cryopreservation for male patients. 6 Epidemiology Fertility preservation for patients with cancer is for female and male cancer patients at risk for infertility due to anticancer treatment. 7 Disease course Fertility preservation techniques for females include embryo cryopreservation, oocyte cryopreservation, ovarian tissue cryopreservation (for pediatric and patients with hormone- dependent diseases), ovarian transposition (oophoropexy), fertoprotective adjuvant agents. Emerging techniques include activation of ovarian follicles, in vitro follicle culture, artificial https://web.pathway.md/diseases/recmUDrzahn15zOfw 1/7 6/29/23, 4:03 AM Fertility preservation in patients with cancer Pathway ovaries, and specific target tissue drugs (nanoparticles, novel fertoprotective agents). Fertility preservation techniques for males include cryopreservation of spermatozoa and cryopreservation of SSC in prepubertal children. 6 Prognosis and risk of recurrence Fertility preservation for patients with cancer is not associated with increased mortality. 8 Guidelines 1. Classification and risk stratification Risk assessment: As per ESHRE 2020 guidelines: Obtain an individual assessment of the indications and risks before fertility preservation interventions. Ensure a multidisciplinary team approach for an accurate assessment of risks. B Assess the risk of gonadotoxicity in all patients undergoing gonadotoxic treatments, taking into consideration the characteristics of the proposed treatment, the patient, and the disease. B As per ESMO 2020 guidelines, view all patients as being at potential risk of developing treatment-related gonadotoxicity, as there is no absolute threshold of exposure to anticancer therapies determining gonadal failure and infertility. B 2. Diagnostic investigations Ovarian reserve testing: obtain antral follicle count or anti-M llerian hormone level measurement for predicting high and low responses to ovarian stimulation. B Show 4 more 3. Medical management Setting of care: As per ESMO 2020 guidelines: Refer all patients with a potential interest in fertility preservation to an appropriate fertility specialist/unit. B Offer fertility preservation strategies preferably at the time of diagnosis before treatment initiation. B As per ASCO 2018 guidelines: Refer patients expressing interest in fertility preservation, as well as ambivalent patients, to a reproductive specialist. E Refer patients distressed about potential infertility to a psychosocial provider. E Fertility preservation in females, oocyte/embryo cryopreservation: https://web.pathway.md/diseases/recmUDrzahn15zOfw 2/7 6/29/23, 4:03 AM Fertility preservation in patients with cancer Pathway As per ESHRE 2020 guidelines: Offer oocyte and embryo cryopreservation as options for fertility preservation. B Offer unfertilized oocyte cryopreservation or both embryo and oocyte cryopreservation in patients having a partner. B Inform patients about the following: accurate, center-specific expertise and live birth rates with oocyte and embryo cryopreservation success rates after oocyte and embryo cryopreservation at the time of cancer diagnosis may be lower than in patients without cancer risk of losing reproductive autonomy and possible issues with ownership of stored embryos. B As per ESMO 2020 guidelines, consider offering cryopreservation of oocytes or embryos before initiating anticancer therapies if a 2-week treatment delay is feasible. B As per ASCO 2018 guidelines, consider offering cryopreservation of unfertilized oocytes as an option, particularly in patients not having a male partner, not wishing to use donor sperm, or having religious or ethical objections to embryo cryopreservation. E Show 2 more As per SEOM 2016 guidelines: Recognize that the major limitation of the embryo and mature oocyte cryopreservation is the time to complete ovarian stimulation. Recognize that immature oocyte cryopreservation is not as successful as cryopreserving oocytes or embryos that have matured in vivo and is considered experimental. Offer oocyte cryopreservation as the preferred option when embryo cryopreservation is not possible, particularly in patients without a male partner or having religious or ethical objections to embryo freezing. B Perform oocyte cryopreservation either by conventional slow freezing or by vitrification. Fertility preservation in females, ovarian tissue cryopreservation: As per ESHRE 2020 guidelines, offer ovarian tissue cryopreservation in patients undergoing moderate/high-risk gonadotoxic treatment where oocyte/embryo cryopreservation is not feasible or per patient preference. B Show 7 more As per ESMO 2020 guidelines, consider offering ovarian tissue cryopreservation as an alternative procedure when oocyte/embryo cryopreservation is not feasible. B Show 3 more As per ASCO 2018 guidelines, consider offering ovarian tissue cryopreservation for future transplantation, recognizing that this method does not require ovarian stimulation or sexual maturity and restores global ovarian function. E As per SEOM 2016 guidelines, harvest and freeze ovarian tissue for ovarian tissue cryopreservation, allowing the preservation of oocytes within primordial follicles, recognizing that this procedure allows immediate initiation of cancer treatment as it does not require prior ovarian stimulation or sperm donation. B Fertility preservation in females (ovarian tissue retransplantation): ensure a multidisciplinary approach for the decision to perform ovarian tissue transplantation in patients https://web.pathway.md/diseases/recmUDrzahn15zOfw 3/7 6/29/23, 4:03 AM Fertility preservation in patients with cancer Pathway with cancer. B Show 8 more Fertility preservation in females, ovarian suppression: As per ESHRE 2020 guidelines, offer GnRH agonists during chemotherapy as an option for ovarian function protection in premenopausal patients with breast cancer. A Do not offer GnRH agonists during chemotherapy instead of cryopreservation techniques. B Show 2 more As per ESMO 2020 guidelines, offer temporary ovarian suppression with a GnRH agonist for ovarian function preservation, irrespective of tumor subtype, in premenopausal patients with breast cancer undergoing (neo)adjuvant chemotherapy. A Show 2 more As per ASCO 2018 guidelines, consider offering GnRH agonists to reduce the likelihood of chemotherapy-induced ovarian insufficiency when proven fertility preservation methods (such as oocyte, embryo, or ovarian tissue cryopreservation) are not feasible, and in young patients with breast cancer. E As per SEOM 2016 guidelines, consider offering GnRH agonists as an option in patients with early-stage receptor-negative breast cancer if embryo or oocyte cryopreservation is not feasible. Do not offer GnRH agonists to preserve fertility in patients with other types of cancer. C Fertility preservation in females (aromatase inhibitors): consider offering aromatase inhibitors to prevent supraphysiological estrogen concentrations during ovarian stimulation in female patients with estrogen-sensitive tumors. C Fertility preservation in females, ovarian transposition: As per SOGC 2023 guidelines: Consider performing ovarian transposition to improve post-treatment ovarian function in patients undergoing pelvic radiotherapy. C Consider referring patients to a reproductive endocrinologist and possible egg/embryo cryopreservation before performing ovarian transposition pelvic radiation. Recognize that transvaginal ovarian access for IVF is difficult even though unassisted pregnancies and live births are possible after ovarian transposition. Recognize that there is a high probability that pelvic radiation will result in a uterine environment incapable of carrying a pregnancy, necessitating a gestational carrier. C As per ESHRE 2020 guidelines: Consider performing ovarian transposition to prevent premature ovarian insufficiency when pelvic radiotherapy without chemotherapy is planned. C Do not perform ovarian transposition in patients with a reduced ovarian reserve and patients at risk of having ovarian metastases. D As per ESMO 2020 guidelines: Consider performing ovarian transposition to preserve ovarian function in 40 years old patients with an indication for pelvic radiotherapy. B Perform ovarian transposition by an experienced laparoscopist to minimize complications and maximize the chances of ovarian function preservation. B https://web.pathway.md/diseases/recmUDrzahn15zOfw 4/7 6/29/23, 4:03 AM Fertility preservation in patients with cancer Pathway As per ASCO 2018 guidelines, consider performing ovarian transposition (oophoropexy) when pelvic irradiation is performed as a cancer treatment. E Show 2 more Fertility preservation in females (in vitro oocyte maturation): Recognize that in vitro oocyte maturation is an innovative fertility preservation procedure B and requires specific expertise. Offer in vitro oocyte maturation only when oocyte cryopreservation is required but ovarian stimulation is not feasible. B Consider offering in vitro oocyte maturation after ex vivo extraction as an experimental procedure. C Fertility preservation in females (gonadal shielding): consider using gonadal shielding as an alternative strategy to ovarian transposition, not requiring surgical intervention. C Fertility preservation in males, sperm cryopreservation: As per ESMO 2020 guidelines, offer sperm cryopreservation before initiating anticancer treatments (chemotherapy, radiotherapy, or surgery) as a standard of care in any male patient with cancer at risk of infertility. B As per ASCO 2018 guidelines, offer sperm banking in postpubertal male patients receiving cancer treatment. E Show 2 more As per SEOM 2016 guidelines, offer sperm cryopreservation with 1-3 samples, B collected before initiating treatment. Show 2 more Fertility preservation in males, testicular tissue cryopreservation: As per ASCO 2018 guidelines, consider offering testicular tissue cryopreservation and reimplantation or grafting of human testicular tissue only as part of clinical trials or approved experimental protocols. E As per SEOM 2016 guidelines, consider offering testicular tissue or spermatogonial cryopreservation and reimplantation or grafting of human testicular tissue in prepubertal patients. Fertility preservation in males, hormonal gonadoprotection: As per ESMO 2020 guidelines, do not offer medical gonadoprotection (GnRH agonist with or without androgens, antiandrogens, or progestins) for fertility preservation in male patients with cancer. D As per ASCO 2018 guidelines, do not offer hormonal therapy as a means to preserve fertility in male patients with cancer. D As per SEOM 2016 guidelines, do not offer hormonal therapy for fertility preservation in male patients with cancer. D Fertility preservation in males (gonadal shielding): shield or remove the testes from the radiation field whenever possible to reduce radiotherapy exposure and reduce the risk of infertility. B 4. Specific circumstances https://web.pathway.md/diseases/recmUDrzahn15zOfw 5/7 6/29/23, 4:03 AM Fertility preservation in patients with cancer Pathway Pediatric patients: As per ASCO 2018 guidelines: Consider offering established methods of fertility preservation, such as sperm or oocyte cryopreservation, in postpubertal minor pediatric patients, with appropriate patient assent and parent or guardian consent. E Recognize that the only available fertility preservation options for prepubertal minor pediatric patients are ovarian and testicular tissue cryopreservation, which are considered experimental. E As per SEOM 2016 guidelines, recognize that there are no reports of live births after ovarian cortical tissue cryopreserved prepubertally and reimplanted at a later date, primarily because of the young age of the study participants. B Show 2 more Patients with gynecologic malignancies: consider performing ovarian cystectomy as a fertility-sparing treatment option in patients with early-stage ovarian cancer. E Show 2 more Patients with hereditary cancer syndromes: offer sperm cryopreservation and oocyte or embryo cryopreservation as the preferred options in newly diagnosed patients with hereditary cancer syndromes interested in fertility preservation. B Show 3 more Patients with premature ovarian insufficiency: do not offer fertility preservation in patients with overt premature ovarian insufficiency. D 5. Patient education General counseling: As per ESHRE 2020 guidelines, counsel patients with cancer eligible for fertility preservation regarding the following: impact of cancer, other diseases and their treatments on reproductive function impact of cancer, other diseases and their treatment on fertility fertility preservation options issues related to cryopreservation storage after fertility preservation infertility and fertility treatments pregnancy after gonadotoxic treatment or underlying condition other childbearing and parenting options. B Show 3 more As per ESMO 2020 guidelines, provide complete oncofertility counseling as early as possible in the treatment planning process in all patients of reproductive age with cancer, irrespective of the type and stage of the disease. B Show 2 more As per ASCO 2018 guidelines: Address the possibility of infertility in adult and pediatric patients with cancer as early as possible before treatment initiation. E https://web.pathway.md/diseases/recmUDrzahn15zOfw 6/7 6/29/23, 4:03 AM Fertility preservation in patients with cancer Pathway Encourage patients to participate in registries and clinical studies, if available, to help define the safety and efficacy of interventions and strategies to preserve fertility in patients with cancer. E As per SEOM 2016 guidelines, discuss the risk of infertility before initiating cancer treatment in patients of reproductive age. A 6. Follow-up and surveillance Pregnancy after cancer treatment: As per ESHRE 2020 guidelines, assess fitness for pregnancy, taking into account treatment late effects, the age of the patient, and the interval since treatment before the use of stored material. B Show 10 more As per ESMO 2020 guidelines, take into consideration patient/couple- and disease/treatment-related factors when counseling adult cancer survivors regarding the feasibility and safety of post-treatment pregnancies. B Show 4 more References 1. ESHRE Guideline Group on Female Fertility Preservation, Richard A Anderson, Fr d ric Amant et al. ESHRE guideline: female fertility preservation. Hum Reprod Open. 2020 Nov 14;2020 4):hoaa052. Open 2. M Mu oz, A Santaballa, M A Segu et al. SEOM Clinical Guideline of fertility preservation and reproduction in cancer patients 2016 . Clin Transl Oncol. 2016 Dec;18 12 1229 1236. Open 3. Kutluk Oktay, Brittany E Harvey, Ann H Partridge et al. Fertility Preservation in Patients With Cancer: ASCO Clinical Practice Guideline Update. J Clin Oncol. 2018 Jul 1;36 19 1994 2001. Open 4. M Lambertini, F A Peccatori, I Demeestere et al. Fertility preservation and post-treatment pregnancies in post-pubertal cancer patients: ESMO Clinical Practice Guidelines. Ann Oncol. 2020 Dec;31 12 1664 1678. Open 5. Tarek Motan, Roland Antaki, Jinglan Han et al. Guideline No. 435 Minimally Invasive Surgery in Fertility Therapy. J Obstet Gynaecol Can. 2023 Apr;45 4 273 282.e2. Open 6. Sofia Del-Pozo-Lerida, Cristina Salvador, Fina Martinez-Soler et al. Preservation of fertility in patients with cancer Review). 2019 May;41 5 2607 2614.2019 May;41 5 2607 2614. Open 7. Kutluk Oktay, Brittany E Harvey, Ann H Partridge et al. Fertility Preservation in Patients With Cancer: ASCO Clinical Practice Guideline Update. J Clin Oncol. 2018 Jul 1;36 19 1994 2001. Open 8. Matteo Lambertini, Lucia Del Mastro, Maria C Pescio et al. Cancer and fertility preservation: international recommendations from an expert meeting. 2016 Jan 4;14 1.2016 Jan 4;14 1. Open 9. Matteo Lambertini, Lucia Del Mastro, Maria C. Pescio et al. Cancer and fertility preservation: international recommendations from an expert meeting. BMC Med. 2016; 14 1. Open 10. Sof a Del-Pozo-L rida, Cristina Salvador, Fina Mart nez-Soler et al. Preservation of fertility in patients with cancer Review). Oncol Rep. 2019 May;41 5 2607 2614. Open https://web.pathway.md/diseases/recmUDrzahn15zOfw 7/7 |
Guideline sources The following summarized guidelines for the evaluation of fetal central nervous system ultrasound are prepared by our editorial team based on guidelines from the World Association of Perinatal Medicine (WAPM 2021). 1 Guidelines 1. Diagnostic investigations Skull ossification: assess the normal shape of the fetal head/skull and the cranial bone ossification at the anatomy scan by axial scans (trans-thalamic or trans-ventricular planes). E Show 2 more Cerebral hemispheres: Assess the symmetry of hemispheres at the anatomy scan by axial scans (trans-thalamic or trans-ventricular planes). E Assess the presence of a central interhemispheric fissure and a falx dividing equally the hemispheres at the anatomy scan by axial scans (trans-thalamic or trans-ventricular planes). E Lateral ventricles (occipital horns): assess the occipital horn of the lateral ventricle distal to the transducer at the anatomy scan by axial scans (trans-ventricular plane). E Show 2 more Lateral ventricles (frontal horns): assess the presence and orientation of two frontal horns of the lateral ventricles medially separated by cavum septi pellucidi at the anatomy scan by axial scans (trans-thalamic or trans-ventricular planes). E Cavum septi pellucidi: assess the presence of the cavum septi pellucidi at the anatomy scan by axial scans (trans-thalamic or trans-ventricular planes). E https://web.pathway.md/diseases/recrbvB7fkk24RMXx 1/2 6/29/23, 4:05 AM Fetal central nervous system ultrasound Pathway Corpus callosum: obtain median/midsagittal view to directly demonstrate the corpus callosum in terms of presence or absence (complete-partial). E Thalami: assess the presence of two thalami separated from each other in the midline at the anatomy scan by axial scans (trans-thalamic plane). E Insula: consider assessing the presence of a normal developed Sylvian fissure for its shape at the mid-trimester anatomy scan by axial scans (trans-ventricular plane as well as trans- thalamic plane), recognizing that it does not rule out every abnormality. E Cerebellum: assess the presence of normal cerebellar hemispheres joined in the middle by the cerebellar vermis at the anatomy scan by axial scan (trans-cerebellar plane). E Show 2 more Cisterna magna: Assess the presence of a normal cisterna magna at the anatomy scan by axial scan (trans- cerebellar plane). E Measure the cisterna magna at the anatomy scan by axial scan (trans-cerebellar plane). E Spine: assess the presence and regularity of the whole spine (including the sacrum) and integrity of the skin at the anatomy scan by a sagittal scan. E References 1. Valentina De Robertis, Cihat Sen, Ilan Timor-Tritsch et al. WAPM World Association of Perinatal Medicine Practice Guidelines: Fetal central nervous system examination. J Perinat Med. 2021 Jun 4;49 9 1033 1041. Open https://web.pathway.md/diseases/recrbvB7fkk24RMXx 2/2 |
Guideline sources The following summarized guidelines for the evaluation and management of fibromuscular dysplasia are prepared by our editorial team based on guidelines from the American Heart Association (AHA/ASA 2021), the Hypertension Canada Guidelines (Hypertension Canada 2020), the Society for Vascular Surgery (SVS 2020), the American College of Radiology (ACR 2019), the Society for Vascular Medicine (SVM/ESH 2019), the European Society of Cardiology (ESC/ESVS 2018), the Kidney Disease: Improving Global Outcomes Foundation (KDIGO 2017), and the Society for Cardiovascular Angiography and Interventions (SCAI/SNIS/SVM/AANS/ASNR/CNS/AANN/SVS/AHA/ACR/SAIP/ACC/ASA/SIR 2011). 1 2 3 4 5 6 7 8 9 9 9 10 Definition FMD is a noninflammatory, nonatherosclerotic arterial disease of medium-sized arteries characterized by arterial stenosis, occlusion, aneurysm, and dissection. 9 Epidemiology The exact cause of FMD is unknown. 9 Disease course https://web.pathway.md/diseases/rec0uu1MUwNH7NOwo 1/7 6/29/23, 4:05 AM Fibromuscular dysplasia Pathway Clinical manifestations of FMD depend on the artery involved (commonly carotid, renal, vertebral) resulting in stenosis, occlusion, aneurysm, and dissection. Renal involvement presented with renovascular hypertension; carotid artery involvement symptoms included headache, pulsatile tinnitus, and dizziness; and mesenteric (celiac and mesenteric) artery involvement presented with abdominal pain, hypertension, diarrhea, nausea, vomiting, headache, hemoperitoneum, and shock. 9 Prognosis and risk of recurrence The in-hospital mortality associated with FMD is 0.74%. 10 Guidelines 1. Screening and diagnosis Indications for testing (symptomatic patients): Evaluate for FMD-related renal artery stenosis in patients with hypertension presenting with 1 of the following clinical clues: significant (> 1.5 cm), unexplained asymmetry in kidney sizes abdominal bruit without apparent atherosclerosis FMD in another vascular territory family history of FMD. B Indications for testing (patients with RAA): consider screening female patients of childbearing age with renal artery aneurysm for FMD with a focused history and one-time axial imaging study (CT angiography or MRA) to assess for cerebrovascular, mesenteric, and iliac artery dysplasia. C Indications for testing (family relatives): Do not obtain genetic testing in asymptomatic relatives of patients with FMD as there are currently no genetic tests that are specific to FMD. D Perform clinical examination and obtain imaging-based evaluation of potentially affected arterial beds in relatives of patients with FMD upon presentation with suggestive symptoms or signs of FMD: Situation Guidance < 30 years old patients with hypertension, especially females Clinical signs of renal artery FMD Accelerated, malignant, or grade 3 (> 180/110 mmHg) hypertension Drug-resistant hypertension (BP target not achieved despite 3 drug-therapy at optimal doses including a diuretic) Unilateral small kidney without a causative urological abnormality Abdominal bruit in the absence of atherosclerotic disease or risk factors for https://web.pathway.md/diseases/rec0uu1MUwNH7NOwo 2/7 6/29/23, 4:05 AM Fibromuscular dysplasia Pathway atherosclerosis Suspected renal artery dissection/infarction Presence of FMD in at least one other vascular territory Severe and/or chronic migraine headaches, especially in the presence of other suggestive symptoms or signs Cardinal symptoms or signs of cerebrovascular FMD Pulsatile tinnitus ('whooshing' or 'swooshing' sound in the ears timed to heart beat) Cervical bruit on exam Stroke, TIA, or amaurosis fugax Unilateral head/neck pain or focal neurologic findings (such as partial Horner's syndrome with ipsilateral ptosis and miosis) suggestive of a cervical artery dissection Headaches (not chronic migraine or not migraine-type) Possible symptoms of cerebrovascular FMD Tinnitus (not pulsatile) Dizziness/lightheadedness. E Diagnosis: Establish the diagnosis of FMD in the presence of at least one focal or multifocal arterial lesion. Do not make the diagnosis solely in the presence of aneurysm, dissection, or tortuosity. E View the presence of aneurysm, dissection, or tortuosity in another/other vascular beds as multivessel involvement of all affected vascular beds if the patient has a focal or multifocal lesion in one vascular bed to establish the diagnosis of FMD. E 2. Classification and risk stratification Classification: classify arterial lesions of FMD according to angiographic appearance as focal or multifocal FMD. E 3. Diagnostic investigations Renal artery imaging: As per CHEP 2020 guidelines, obtain MRA or CT angiography to evaluate for FMD of the renal arteries. B https://web.pathway.md/diseases/rec0uu1MUwNH7NOwo 3/7 6/29/23, 4:05 AM Fibromuscular dysplasia Pathway As per SVM 2019 guidelines, consider obtaining imaging-based evaluation for renal artery FMD in the presence of suggestive symptoms and signs. E Show 2 more Cervicocephalic vascular imaging: As per CHEP 2020 guidelines, screen for cervicocephalic lesions and intracranial aneurysm in patients with confirmed FMD of the renal arteries. B As per SVM 2019 guidelines, consider obtaining imaging-based evaluation for cerebrovascular FMD in the presence of suggestive symptoms and signs. E Show 5 more Peripheral artery imaging: obtain contrast-enhanced CT angiography, contrast-enhanced MRA, or arteriography of lower extremities for the initial imaging in patients with suspected or known FMD. B Imaging of other arteries: screen for FMD in other vascular beds in the presence of suggestive symptoms in patients with confirmed FMD of the renal arteries. B 4. Medical management Thromboprophylaxis: As per SVM 2019 guidelines, consider initiating antiplatelet therapy (aspirin 75-100 mg daily) in patients with FMD, in the absence of contraindication, to prevent thrombotic and thromboembolic complications. E As per ASA 2011 guidelines, consider platelet-inhibitor medication in patients with FMD of the carotid arteries to prevent thromboembolism. Optimal drug and dosing regimens have not been established. C 5. Therapeutic procedures Renal artery revascularization: As per CHEP 2020 guidelines, perform renal artery angioplasty without stenting for the treatment of patients with FMD-related renal artery stenosis. Do not perform stenting unless required because of a periprocedural dissection. B As per ESC 2018 guidelines, consider performing balloon angioplasty with bailout stenting in patients with hypertension and/or signs of renal impairment related to renal arterial FMD. C Carotid artery revascularization: consider carotid angioplasty with or without stenting for patients with retinal or hemispheric cerebral ischemic symptoms related to FMD of the ipsilateral carotid artery, but comparative data addressing these methods of revascularization are not available. C 6. Surgical interventions Surgical revascularization: consider performing surgical revascularization in patients with complex lesions of FMD in the renal arteries less amendable to angioplasty, stenosis https://web.pathway.md/diseases/rec0uu1MUwNH7NOwo 4/7 6/29/23, 4:05 AM Fibromuscular dysplasia Pathway associated with complex aneurysm, and restenosis despite two unsuccessful attempts of angioplasty. C 7. Specific circumstances Patients with ischemic stroke: initiate antiplatelet therapy, control BP control, and offer lifestyle modifications for the prevention of future ischemic events in patients with a history of ischemic stroke or TIA and FMD without other attributable causes. B Show 2 more Kidney donation: regard donor candidates with FMD involving the orifices of both renal arteries as ineligible for kidney donation. 8. Follow-up and surveillance Indications for referral: refer patients with confirmed renal FMD to a hypertension specialist. B Follow-up: As per ESH 2019 guidelines: Follow-up patients with FMD at least annually, including clinical assessment, assessment of renal function, and imaging. E Insufficient evidence to recommend specific algorithms for modality and frequency of imaging studies in the follow-up. Customize the timing of follow-up imaging to each patient's pattern and severity of disease, including the need for monitoring of aneurysms or dissections and local imaging resources and experience. I As per ASA 2011 guidelines, consider annual noninvasive imaging of the carotid arteries in patients with FMD, to detect changes in the extent or severity of disease, although the effect on outcomes is unclear. C Clinical findings Symptoms Past medical history Abdominal pain worsening after eating Acute coronary syndrome CKD Chest pain Carotid-cavernous fistula Claudication Cervical artery dissection Cold fingers or toes Ehlers-danlos syndrome Difficulty speaking Intracranial aneurysm Dizziness Marfan syndrome Facial weakness Mesenteric ischemia Headache Migraine Nausea Renal artery aneurysm Neck pain Renal artery dissection Numbness Stroke https://web.pathway.md/diseases/rec0uu1MUwNH7NOwo 5/7 6/29/23, 4:05 AM Fibromuscular dysplasia Pathway Sweating TIA Temporary loss of vision Vertebral arteriovenous fistula Tinnitus Visceral artery aneurysm Weakness Social history Weakness in the legs Weight loss Cigarette smoking Family history Vascular exam Early-onset hypertension Abdominal bruit Cervical bruit Vital signs Cold limb Hypertension Femoral bruit Pulse differences between arms Respiratory exam Integument exam Dyspnea Digital ischemia Imaging findings Arterial aneurysm Arterial dissection Arterial tortuosity Renal atrophy String of beads sign References 1. Brott TG, Halperin JL, Abbara S et al. 2011 ASA/ACCF/AHA/AANN/AANS/ACR/ASNR/CNS/SAIP/SCAI/SIR/SNIS/SVM/SVS guideline on the management of patients with extracranial carotid and vertebral artery disease: executive summary. A report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines, and the American Stroke Association, American Association of Neuroscience Nurses, American Association of Neurological Surgeons, American College of Radiology, American Society of Neuroradiology, Congress of Neurological Surgeons, Society of Atherosclerosis Imaging and Prevention, Society for Cardiovascular Angiography and Interventions, Society of Interventional Radiology, Society of NeuroInterventional Surgery, Society for Vascular Medicine, and Society for Vascular Surgery. Circulation. 2011 Jul 26;124 4 489 532. Open 2. Expert Panels on Vascular Imaging:, Christopher J Francois, Erik P Skulborstad et al. ACR Appropriateness Criteria Nonatherosclerotic Peripheral Arterial Disease. J Am Coll Radiol. 2019 May;16 5S S174 S183. Open 3. Doreen M Rabi, Kerry A McBrien, Ruth Sapir-Pichhadze et al. Hypertension Canada's 2020 Comprehensive Guidelines for the Prevention, Diagnosis, Risk Assessment, and Treatment of Hypertension in Adults and Children. Can J Cardiol. 2020 May;36 5 596 624. Open 4. Gornik HL, Persu A, Adlam D et al. First International Consensus on the diagnosis and management of fibromuscular dysplasia. Vasc Med. 2019 Apr;24 2 164 189. Open 5. Dawn O Kleindorfer, Amytis Towfighi, Seemant Chaturvedi et al. 2021 Guideline for the Prevention of Stroke in Patients With Stroke and Transient Ischemic Attack: A Guideline From the American Heart https://web.pathway.md/diseases/rec0uu1MUwNH7NOwo 6/7 6/29/23, 4:05 AM Fibromuscular dysplasia Pathway Association/American Stroke Association. Stroke. 2021 Jul;52 7):e364-e467. Open 6. Victor Aboyans, Jean-Baptiste Ricco, Marie-Louise E L Bartelink et al. 2017 ESC Guidelines on the Diagnosis and Treatment of Peripheral Arterial Diseases, in collaboration with the European Society for Vascular Surgery ESVS Document covering atherosclerotic disease of extracranial carotid and vertebral, mesenteric, renal, upper and lower extremity arteriesEndorsed by: the European Stroke Organization ESO The Task Force for the Diagnosis and Treatment of Peripheral Arterial Diseases of the European Society of Cardiology ESC and of the European Society for Vascular Surgery ESVS . Eur Heart J. 2018 Mar 1;39 9 763 816. Open 7. Rabih A Chaer, Christopher J Abularrage, Dawn M Coleman et al. The Society for Vascular Surgery clinical practice guidelines on the management of visceral aneurysms. J Vasc Surg. 2020 Jul;72 1S 3S 39S. Open 8. Krista L Lentine, Bertram L Kasiske, Andrew S Levey et al. KDIGO Clinical Practice Guideline on the Evaluation and Care of Living Kidney Donors. Transplantation. 2017 Aug;101 8S Suppl 1 S1 S109. Open 9. Masayoshi Ko, Kenya Kamimura, Kohei Ogawa et al. Diagnosis and management of fibromuscular dysplasia and segmental arterial mediolysis in gastroenterology field: A mini-review. 2018 Aug 28;24 32 3637 3649.2018 Aug 28;24 32 3637 3649. Open 10. Chayakrit Krittanawong, Anirudh Kumar, Kipp W Johnson et al. Prevalence, Presentation, and Associated Conditions of Patients With Fibromuscular Dysplasia. Am J Cardiol. 2019 Apr 1;123 7 1169 1172. Open 11. Persu A, Giavarini A, Touz E et al. European consensus on the diagnosis and management of fibromuscular dysplasia. J Hypertens. 2014 Jul;32 7 1367 78. Open 12. Masayoshi Ko, Kenya Kamimura, Kohei Ogawa et al. Diagnosis and management of fibromuscular dysplasia and segmental arterial mediolysis in gastroenterology field: A mini-review. World J Gastroenterol. 2018 Aug 28; 24 32 3637 3649. Open https://web.pathway.md/diseases/rec0uu1MUwNH7NOwo 7/7 |
Guideline sources The following summarized guidelines for the evaluation and management of fibromyalgia (FM) are prepared by our editorial team based on guidelines from the Canadian Expert Group on Cannabinoids Use in Chronic Pain (CCP-CEG 2023), the Egyptian Consensus Group on Fibromyalgia (ECG-FM 2022), the Italian Society of Rheumatology (ISR 2021), the European League Against Rheumatism (EULAR 2017), the National Fibromyalgia Guideline Advisory Panel (NFGAP 2013), the Spanish Interdisciplinary Consensus Group on Fibromyalgia (SICG- FM 2010), and the Brazilian Society of Rheumatology (BSR 2010). 1 2 3 4 5 6 7 8 8 8 9 Definition FM is a syndrome characterized by chronic widespread pain, fatigue, and sleep disruption. 8 Epidemiology The exact cause of FM is unknown; however, central sensitization involving genetic, immunological, and hormonal factors have been implicated. 8 Disease course Clinical manifestations of FM include chronic widespread pain (diffuse, multifocal, deep, gnawing, burning, waxing and waning, and migratory), tender joints, fatigue, sleep disturbances, weight fluctuations, morning stiffness, irritable bowel disease, cognitive disturbance, depression, https://web.pathway.md/diseases/recB8e4Lh5NhNbbfY 1/9 6/29/23, 4:05 AM Fibromyalgia Pathway anxiety, headaches, heat and cold intolerance, irritable bladder syndrome, restless legs, and Raynaud's phenomenon. 8 Prognosis and risk of recurrence FM is not associated with increased mortality. 9 Calculator Diagnostic criteria for fibromyal Guidelines 1. Screening and diagnosis Diagnostic criteria: As per ECG-FM 2022 guidelines, use the 2010 ACR diagnostic criteria for FM. A Show 2 more As per ISR 2021 guidelines: Establish the clinical diagnosis of FM based on peculiar symptoms lasting at least 3 months, excluding symptoms related to other diseases. B Consider using the 2016 review of the ACR diagnostic criteria in the initial assessment to support a clinical diagnosis, recognizing that symptoms vary over time. B Recognize that physical examination should be normal except for hypersensitivity to soft tissue pressure. B Do not confirm the diagnosis of FM syndrome based on the assessment of tender points according to the 1990 ACR diagnostic criteria. B Diagnose FM syndrome as a clinical construct without any confirmatory laboratory tests. Avoid obtaining repeated evaluations after diagnosis unless guided by the onset of new symptoms or semeiotic findings. Obtain additional laboratory and radiographic tests depending on the patient's clinical evaluation suggesting another medical condition. B As per NFGAP 2013 guidelines, diagnose FM in patients with diffuse body pain persisting for at least 3 months, possibly also having symptoms of fatigue, sleep disturbance, cognitive changes, mood disorder, and other somatic symptoms to variable degrees, unexplained by other illnesses. B Show 3 more As per BSR 2010 guidelines, recognize that FM is a complex and heterogeneous health condition with a disturbance in the processing of pain associated with other secondary characteristics. B Show 2 more 2. Diagnostic investigations https://web.pathway.md/diseases/recB8e4Lh5NhNbbfY 2/9 6/29/23, 4:05 AM Fibromyalgia Pathway Physical examination: As per EULAR 2017 guidelines, obtain a comprehensive assessment of pain, function, and psychosocial context in patients with FM. B As per NFGAP 2013 guidelines: Perform a physical examination in all patients with a symptom suggesting a diagnosis of FM. Recognize that physical examination should be within normal limits except for tenderness on pressure of soft tissues (hyperalgesia or increased pain following a painful stimulus). B Perform an examination of soft tissues for generalized tenderness by manual palpation, recognizing that the specific tender point examination according to the 1990 ACR diagnostic criteria is not required to confirm a clinical diagnosis of FM. B Laboratory and imaging tests: As per ECG-FM 2022 guidelines: Limit laboratory testing to a CBC, ESR, CRP, TSH, HCV antibodies, vitamin D, and anti- tissue transglutaminase IgA antibodies, and CK to rule out conditions that can present similarly to FM, such as endocrine diseases (hypothyroidism), rheumatic conditions (early inflammatory arthritis or polymyalgia rheumatica), celiac disease, or neurological disease (myopathy or multiple sclerosis), depending upon the clinical evaluation. B Consider obtaining musculoskeletal ultrasound to rule out inflammatory arthritic conditions wherever applicable. C As per NFGAP 2013 guidelines: Obtain limited initial laboratory testing, including CBC, ESR, CRP, CK, and TSH. B Obtain additional laboratory and radiographic tests depending on the patient's clinical evaluation suggesting another medical condition. B Mental health assessment: As per ECG-FM 2022 guidelines, consider obtaining psychological evaluation in selected patients. C As per NFGAP 2013 guidelines, identify and address other comorbid conditions, including depression, to reduce healthcare costs. B As per BSR 2010 guidelines, assess the seriousness of other symptoms, including fatigue, sleep disturbances, mood disturbances, and cognition disturbances, and the impact of these on the quality of life of the patient. B As per SICG-FM 2010 guidelines, assess the levels of depression and catastrophism in patients with FM. B 3. Medical management General principles: As per ECG-FM 2022 guidelines, recognize that there is no cure for FM. Direct treat-to-target to the reduction of symptoms, healthy lifestyle practices, and maintenance of optimal function, with patient outcome goals, clearly defined at the first visit. B Show 4 more https://web.pathway.md/diseases/recB8e4Lh5NhNbbfY 3/9 6/29/23, 4:05 AM Fibromyalgia Pathway As per EULAR 2017 guidelines, provide a graduated treatment approach in patients with FM. B Show 2 more As per NFGAP 2013 guidelines, manage patients with FM in the primary care setting. Augment this care by providing access to a multidisciplinary team where feasible. A Show 5 more Antidepressants: As per ECG-FM 2022 guidelines, consider offering duloxetine or milnacipran in patients with severe pain. B As per ISR 2021 guidelines, consider offering serotonin reuptake inhibitors (fluoxetine or paroxetine), norepinephrine (duloxetine), or TCA drugs (amitriptyline) in patients with FM syndrome. B As per EULAR 2017 guidelines, consider offering low-dose amitriptyline, duloxetine, or milnacipran in patients with FM. B As per NFGAP 2013 guidelines, consider offering TCAs, SSRIs, or SNRIs for pain and other symptoms in patients with FM. B Decide on the choice of antidepressants according to available evidence for efficacy, physician knowledge, patient characteristics, and side effect profile. C Anticonvulsants: As per ISR 2021 guidelines, consider offering anticonvulsants, particularly pregabalin, for pain-modulating properties. Initiate treatment with the lowest possible dose followed by a progressive increase in dosage with attention to adverse events. B As per EULAR 2017 guidelines, consider offering pregabalin in patients with FM. B As per NFGAP 2013 guidelines, offer anticonvulsants at the lowest possible dose, followed by up-titration with attention to adverse events, in patients with FM. A Skeletal muscle relaxants: As per ISR 2021 guidelines, consider offering cyclobenzaprine in patients with FM syndrome, especially in the context of significant sleep disturbances. B As per EULAR 2017 guidelines, consider offering cyclobenzaprine in patients with FM. B Non-opioid analgesics: As per ECG-FM 2022 guidelines, reserve NSAIDs for associated conditions, such as OA. A As per ISR 2021 guidelines, consider offering acetaminophen in selected patients. C As per NFGAP 2013 guidelines: Consider offering acetaminophen as analgesic therapy in selected patients, paying close attention to safe dosing. C Administer NSAIDs, if prescribed (particularly for associated conditions, such as OA), at the lowest dose and for the shortest possible period, given potentially serious adverse events. B Opioids: As per ECG-FM 2022 guidelines, consider offering tramadol in selected patients. Do not use strong opioids. B https://web.pathway.md/diseases/recB8e4Lh5NhNbbfY 4/9 6/29/23, 4:05 AM Fibromyalgia Pathway As per ISR 2021 guidelines, reserve a therapeutic trial with weak opioids, especially tramadol, B in patients with moderate-to-severe pain not responding to other treatment modalities. B Monitor closely pharmacological action, with particular reference to drug- dependent side effects or behavioral disorders. B Discontinue opioids in the absence of symptom improvement. B As per EULAR 2017 guidelines, consider offering tramadol in patients with FM. B As per NFGAP 2013 guidelines: Reserve a trial of opioids, beginning with a weak opioid (such as tramadol), for patients with moderate-to-severe pain unresponsive to other treatment modalities. B Avoid using strong opioids, and ensure that patients continuing opioids show improved pain and function. Monitor for continued efficacy and side effects or evidence of aberrant drug behaviors. D Cannabinoids: As per ISR 2021 guidelines, consider offering cannabinoids in patients with FM syndrome, especially in the context of significant sleep disturbances. C As per NFGAP 2013 guidelines, consider offering a trial of a prescribed pharmacological cannabinoid in patients with FM, particularly in the setting of important sleep disturbance. C Cannabinoids (CEG-CCP): offer cannabinoid-based medicines as an adjunct treatment for the management of back pain, FM pain, or other chronic pain in patients with FM not achieving an adequate response with standard analgesics. B Dopamine agonists: As per BSR 2010 guidelines, offer pramipexole to reduce pain in patients with FM, B especially in the presence of sleep disturbances and restless legs syndrome. A As per SICG-FM 2010 guidelines, offer pramipexole as a treatment option in patients with group 1 (Giesecke subgrouping) FM. B Serotonin antagonists: offer tropisetron for the management of pain in patients with FM. B Sedatives: offer zoplicone and zolpidem for the management of sleep disturbances in patients with FM. B Corticosteroids: as per ECG-FM 2022 guidelines, do not use corticosteroids in patients with FM. D 4. Nonpharmacologic interventions General principles: As per ECG-FM 2022 guidelines, offer non-pharmacological therapies as initial management, based on availability, cost, and patient preferences. B As per NFGAP 2013 guidelines, offer non-pharmacological strategies with active patient participation as an integral component of the therapeutic plan in patients with FM. A Work limitations: As per NFGAP 2013 guidelines: https://web.pathway.md/diseases/recB8e4Lh5NhNbbfY 5/9 6/29/23, 4:05 AM Fibromyalgia Pathway Encourage patients to remain in the workforce and, if necessary, provide recommendations that could help maintain optimal productivity, as outcome is generally more favorable for employed patients. B Encourage patients with FM on a prolonged sick leave to participate in an appropriate rehabilitation program with focus on improving function, including return to work if possible. B As per BSR 2010 guidelines, recognize that FM does not justify work exclusion. B Exercise: As per ECG-FM 2022 guidelines, advise patients with FM to engage in an activity, either land-based or water, that is enjoyable, easy to follow, convenient, and within budget to improve adherence, as subjective muscle pain may be a barrier to optimal exercise activity. A As per ISR 2021 guidelines: Encourage patients to pursue a regular lifestyle, gradually increasing physical activity through stimuli and/or maintaining/improving function and promoting self-management by multimodal therapy. B Consider offering the following physical therapies, adapted to the patient individual performance level, in patients with FM syndrome: aerobic resistance training strengthening exercise water activity/water jogging thermal therapy (bath in thermal springs). B As per EULAR 2017 guidelines, advise practicing aerobic and strengthening exercises in patients with FM. A As per NFGAP 2013 guidelines, advise patients with FM to participate in a graduated exercise program of their choosing for global health benefits and probable effects on FM symptoms. A Psychological interventions: As per ISR 2021 guidelines: Inform patients of the negative impact that the psychological distress associated with FM syndrome can generate. B Promote psychological assessment and counseling. B Consider offering the following psychological therapies: CBT and occupational therapy, including patient education, even for a short period B hypnosis, guided imagination, or therapeutic writing. C As per EULAR 2017 guidelines, consider offering CBT in patients with FM. B As per NFGAP 2013 guidelines, offer interventions improving self-efficacy to help patients cope with symptoms of FM. A Show 3 more Multicomponent therapies: As per EULAR 2017 guidelines, consider offering multicomponent therapies in patients with FM. B https://web.pathway.md/diseases/recB8e4Lh5NhNbbfY 6/9 6/29/23, 4:05 AM Fibromyalgia Pathway As per NFGAP 2013 guidelines, consider offering multicomponent therapy to facilitate the attainment of effective coping skills and the promotion of self-management. C Alternative and complementary therapies: As per ISR 2021 guidelines, consider offering the following non-conventional therapies: meditative movement therapies, such as qigong, yoga, and Tai chi B mindfulness-based stress reduction programs B relaxation training combined with exercise B acupuncture B hydrotherapy. B As per EULAR 2017 guidelines, consider offering the following complementary therapies in patients with FM: acupuncture hydrotherapy qigong yoga tai chi mindfulness-based stress reduction. B As per NFGAP 2013 guidelines, insufficient evidence to recommend complimentary and alternative medicine therapies for the management of FM symptoms. I As per SICG-FM 2010 guidelines, do not offer experimental therapies or alternatives of any type. D 5. Patient education General counseling: As per ECG-FM 2022 guidelines, offer education and active participation with reassurance regarding "no harm" caused by physical activity, especially if the patient is passive. Encourage self-efficacy and social support to facilitate the practice of health-promoting lifestyles. Use a graded incremental activity to maintain or improve function. B As per ISR 2021 guidelines, inform patients about the recommended and non-recommended treatment measures after FM syndrome diagnosis. B Show 4 more As per NFGAP 2013 guidelines, inform patients that the outcome in many cases is favorable, even if symptoms of FM tend to increase and decrease over time. B Show 2 more 6. Follow-up and surveillance Indications for specialist referral: consider obtaining specialist consultation, including referral to a sleep specialist or psychologist, in selected cases. Reserve continued care by a specialist in patients failed management in primary care or having more complex comorbidities. C https://web.pathway.md/diseases/recB8e4Lh5NhNbbfY 7/9 6/29/23, 4:05 AM Fibromyalgia Pathway Assessment of treatment response: As per ECG-FM 2022 guidelines, recognize that FM symptoms persist and fluctuate over time, even with treatment. Consider using early treatment response to a specific medication as a treatment effect indicator. Recognize that factors such as passivity, poor internal locus of control, cognitive dysfunction, prominent mood disorder, perfectionist, meticulous and obsessive personalities, and uncontrolled underlying disease (if any) may have a negative influence on the outcome. B As per NFGAP 2013 guidelines, consider measuring outcomes by a narrative report of symptom status or patient global impression of change, without requiring more complex questionnaires. C Show 2 more Follow-up: As per NFGAP 2013 guidelines: Schedule clinical follow-up according to the judgment of the physician or healthcare team, with more frequent visits during the initial phase of management or until symptoms are stabilized. B Monitor patients for the development of new symptoms during the continued care of FM to ensure that symptoms are not due to some other medical illness. B Clinical findings Patient demographics Symptoms Female predominance Abdominal pain Anxiety Past medical history Bloating Constipation IBS Depression Migraine Difficulty sleeping Obesity Fatigue Rheumatoid arthritis Headache SLE Joint pain TMJ syndrome Limb numbness Memory impairment Numbness of soles of feet Pain Provoked by stress Stiffness Trouble concentrating Family history Genetic predispositions References https://web.pathway.md/diseases/recB8e4Lh5NhNbbfY 8/9 6/29/23, 4:05 AM Fibromyalgia Pathway 1. C Alegre de Miquel, J Garc a Campayo, M Tom s Fl rez et al. Interdisciplinary consensus document for the treatment of fibromyalgia. Actas Esp Psiquiatr. 2010 Mar-Apr;38 2 108 20. Open 2. Fitzcharles MA, Ste-Marie PA, Goldenberg DL et al. 2012 Canadian Guidelines for the diagnosis and management of fibromyalgia syndrome: executive summary. Pain Res Manag. 2013 May-Jun;18 3 119 26. Open 3. Yasser El Miedany, Naglaa Gadallah, Diaa Mohasseb et al. Consensus evidence-based clinical practice recommendations for the management of fibromyalgia. Egypt Rheumatol Rehabil. 2022 May 19;49 30 . Open 4. Roberto Ezequiel Heymann, Eduardo dos Santos Paiva, Milton Helfenstein Jr et al. Brazilian consensus on the treatment of fibromyalgia. Rev Bras Reumatol. 2010 Jan-Feb;50 1 56 66. Open 5. A Ariani, L Bazzichi, P Sarzi-Puttini et al. The Italian Society for Rheumatology clinical practice guidelines for the diagnosis and management of fibromyalgia Best practices based on current scientific evidence. Reumatismo. 2021 Aug 3;73 2 89 105. Open 6. Macfarlane GJ, Kronisch C, Dean LE et al. EULAR revised recommendations for the management of fibromyalgia. Ann Rheum Dis. 2017 Feb;76 2 318 328. Open 7. Alan D Bell, Caroline MacCallum, Shari Margolese et al. Clinical Practice Guidelines for Cannabis and Cannabinoid-Based Medicines in the Management of Chronic Pain and Co-Occurring Conditions. Cannabis Cannabinoid Res. 2023 Mar 27. Open 8. Enrico Bellato, Eleonora Marini, Filippo Castoldi et al. Fibromyalgia syndrome: etiology, pathogenesis, diagnosis, and treatment. 2012;2012 426130.2012;2012 426130. Open 9. Frederick Wolfe, Afton L Hassett, Brian Walitt et al. Mortality in fibromyalgia: a study of 8,186 patients over thirty-five years. 2011 Jan;63 1 94 101.2011 Jan;63 1 94 101. Open 10. Aidan C. Tan, Tiina Jaaniste, and David Champion. Chronic Widespread Pain and Fibromyalgia Syndrome: Life-Course Risk Markers in Young People. Pain Res Manag. 2019; 2019 6584753. Open 11. Frederick Wolfe, Afton L Hassett, Brian Walitt et al. Mortality in fibromyalgia: a study of 8,186 patients over thirty-five years. Arthritis Care Res Hoboken). 2011 Jan;63 1 94 101. Open 12. Enrico Bellato, Eleonora Marini, Filippo Castoldi et al. Fibromyalgia Syndrome: Etiology, Pathogenesis, Diagnosis, and Treatment. Pain Res Treat. 2012; 2012 426130. Open 13. Daniel J Clauw. Fibromyalgia: a clinical review. JAMA. 2014 Apr 16;311 15 1547 55. Open 14. Andrea T Borchers, M Eric Gershwin. Fibromyalgia: A Critical and Comprehensive Review. Clin Rev Allergy Immunol. 2015 Oct;49 2 100 51. Open 15. Seth Lederman, Lesley M Arnold, Ben Vaughn et al. Efficacy and Safety of TNX 102 SL Sublingual Cyclobenzaprine) for the Treatment of Fibromyalgia: Results From the Randomized, Placebo-Controlled RELIEF Trial. Arthritis Care Res Hoboken). 2023 May 11. Open https://web.pathway.md/diseases/recB8e4Lh5NhNbbfY 9/9 |
Guideline sources The following summarized guidelines for the management of focal atrial tachycardia (FAT) are prepared by our editorial team based on guidelines from the European Society of Cardiology (ESC 2020) and the American Heart Association (AHA/HRS/ACC 2016). 1 2 Calculator NYHA functional classification f Guidelines 1. Medical management Acute management: Consider administering intravenous adenosine (6-18 mg bolus) for acute management of hemodynamically stable patients with focal AT. C Show 2 more Administer IV formulations of the following medications for acute management of hemodynamically stable patients with focal AT: -blockers diltiazem verapamil. B Show 3 more Ongoing management: https://web.pathway.md/diseases/recwf1PcPFxHl3j7a 1/2 6/29/23, 4:05 AM Focal atrial tachycardia Pathway Ongoing management: Consider initiating the following medications for the management of patients with focal AT if ablation is not desirable or feasible: -blockers nondihydropyridine CCBs (verapamil or diltiazem) in the absence of HFrEF propafenone or flecainide in the absence of structural or ischemic heart disease. C Show 2 more Consider initiating oral formulations of the following medications for ongoing management of patients with symptomatic focal AT: -blockers diltiazem verapamil. C Show 2 more 2. Therapeutic procedures Synchronized electrical cardioversion: Perform synchronized direct current cardioversion for acute management of hemodynamically unstable patients with focal AT. B Perform synchronized direct current cardioversion for acute management of hemodynamically stable patients with focal AT if drug therapy fails to convert or control the tachycardia. B Perform synchronized cardioversion for acute management of hemodynamically unstable patients with focal AT. B Catheter ablation: perform catheter ablation as an alternative to pharmacological therapy in symptomatic patients with focal AT. B References 1. Richard L Page, Jos A Joglar, Mary A Caldwell et al. 2015 ACC/AHA/HRS Guideline for the Management of Adult Patients With Supraventricular Tachycardia: Executive Summary: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society. Circulation. 2016 Apr 5;133 14):e471 505. Open 2. Josep Brugada, Demosthenes G Katritsis, Elena Arbelo et al. 2019 ESC Guidelines for the management of patients with supraventricular tachycardia The Task Force for the management of patients with supraventricular tachycardia of the European Society of Cardiology ESC . Eur Heart J. 2020 Feb 1;41 5 655 720. Open https://web.pathway.md/diseases/recwf1PcPFxHl3j7a 2/2 |
Guideline sources The following summarized guidelines for the evaluation and management of focal liver lesions are prepared by our editorial team based on guidelines from the European Association for the Study of the Liver (EASL 2022; 2016), the American College of Radiology (ACR 2020), the European Federation of Societies for Ultrasound (EFSU 2020), the American College of Gastroenterology (ACG 2014), and the Spanish Working Group on Inherited Kidney Diseases (SWG-IKD 2014). 1 2 3 4 5 6 Guidelines 1. Diagnostic investigations Contrast-enhanced ultrasound: As per ACR 2020 guidelines: Obtain contrast-enhanced abdominal ultrasound of an indeterminate > 1 cm liver lesion on initial ultrasound in patients with a normal liver with no suspicion or evidence of extrahepatic malignancy or underlying liver disease. B Obtain contrast-enhanced abdominal ultrasound of an incidental liver lesion > 1 cm on initial ultrasound, non-contrast or single-phase CT, or non-contrast MRI in patients with known chronic liver disease. B As per EFSU 2020 guidelines, obtain liver ultrasound using B-mode and Doppler mode before obtaining contrast-enhanced ultrasound to characterize focal liver lesions. B Show 14 more Cross-sectional imaging: https://web.pathway.md/diseases/recKkO8VoEkTQ5cHC 1/5 6/29/23, 4:06 AM Focal liver lesions Pathway As per ACR 2020 guidelines, obtain contrast-enhanced multiphase CT, contrast-enhanced MRI, or contrast-enhanced ultrasound of an indeterminate > 1 cm liver lesion on initial ultrasound in patients with a normal liver with no suspicion or evidence of extrahepatic malignancy or underlying liver disease. B Show 7 more As per ACG 2014 guidelines, obtain MRI or triple-phase CT in patients with cirrhosis with a lesion of > 1 cm on ultrasound. B 2. Specific circumstances Patients with simple hepatic cysts, evaluation: As per EASL 2022 guidelines, obtain ultrasound as the first-line imaging for the diagnosis of hepatic cysts. B Show 2 more As per ACG 2014 guidelines, obtain CT or MRI for further evaluation of a hepatic cyst identified on ultrasound with septations, fenestrations, calcifications, irregular walls, or daughter cysts. B Patients with simple hepatic cysts, management: As per EASL 2022 guidelines, offer the best locally available volume-reducing therapy for the treatment of symptomatic simple hepatic cysts without biliary communication. B As per ACG 2014 guidelines, observe asymptomatic patients with simple hepatic cysts with expectant management. B Show 2 more Patients with simple hepatic cysts (surveillance): do not monitor asymptomatic patients with simple hepatic cysts or peribiliary cysts. D Show 2 more Patients with polycystic liver disease (evaluation): obtain abdominal ultrasound to screen for polycystic liver disease in all patients diagnosed with autosomal dominant polycystic kidney disease. B Do not obtain genetic testing for the screening of polycystic liver disease. B Show 4 more Patients with polycystic liver disease, management: As per EASL 2022 guidelines, decide on the choice of treatment based on symptoms and complications related to the presence of cysts in combination with liver phenotypes: Situation Guidance Abdominal fullness Symptoms Lack of appetite or early satiety Acid reflux Nausea and vomiting Pain in rib cage, sides, abdomen or back Shortness of breath Limited mobility, fatigue https://web.pathway.md/diseases/recKkO8VoEkTQ5cHC 2/5 6/29/23, 4:06 AM Focal liver lesions Pathway Anxiety about the future Concern or dissatisfaction with abdomen size Problems with intercourse Involuntary weight loss Jaundice Complications Hepatic venous outflow obstruction Portal hypertension Recurrent cyst infection Recurrent cyst hemorrhage Numerous small-to-medium-sized cysts throughout the liver Phenotypes Large or strategically located cyst percutaneously accessible Single or multiple large superficial cysts Small-to-medium-sized cysts clustered in a few segments in the presence of some less affected liver segments Massive polycystic liver disease Severely affecting the quality of life or accompanied by recurrent complications. B Show 3 more As per ACG 2014 guidelines, do not use mTOR inhibitors or somatostatin analogs in patients with polycystic liver disease. D Show 2 more As per SWG-IKD 2014 guidelines, perform volume-reducing procedures (aspiration and sclerotherapy, fenestration, or segmental liver resection), performed only by a surgeon with expertise in polycystic liver disease owing to the abnormal anatomy of the liver and the high morbidity of these surgical procedures, only in highly symptomatic patients with polycystic liver disease. B Patients with hepatic hemangioma: As per EASL 2016 guidelines, suspect hepatic hemangioma in the presence of a hyperechoic lesion in patients with a normal and healthy liver. Obtain ultrasound to confirm the diagnosis with typical imaging features (homogeneous hyperechoic, sharp margin, posterior enhancement, and absence of halo sign) in a lesion < 3 cm. obtain contrast-enhanced imaging (such as contrast-enhanced ultrasound, contrast- enhanced CT, or MRI) in patients with an oncological condition or an underlying liver disease. (evidence level II-2, grade of recommendation 1). B Show 5 more As per ACG 2014 guidelines, obtain MRI or CT for the diagnosis of hepatic hemangioma. B https://web.pathway.md/diseases/recKkO8VoEkTQ5cHC 3/5 6/29/23, 4:06 AM Focal liver lesions Pathway Show 4 more Patients with focal nodular hyperplasia: As per EASL 2016 guidelines, obtain contrast-enhanced ultrasound, CT, or MRI for the diagnosis of focal nodular hyperplasia (~ 100% specificity with typical imaging features observed in combination). Recognize that MRI has the highest diagnostic performance overall, and the highest diagnostic accuracy by contrast-enhanced ultrasound is achieved in focal nodular hyperplasia < 3 cm. B Show 3 more As per ACG 2014 guidelines, obtain MRI or CT for the diagnosis of focal nodular hyperplasia. Do not perform a routine liver biopsy to confirm the diagnosis. B Show 4 more Patients with nodular regenerative hyperplasia: perform liver biopsy for the diagnosis of nodular regenerative hyperplasia. B Show 3 more Patients with hepatic mucinous cystic neoplasms: View a combination of 1 major and 1 minor feature as worrisome features for mucinous cystic neoplasms of the liver: major features: thick septation, nodularity minor features: upstream biliary dilatation, thin septations, internal hemorrhage, perfusional change, < 3 coexistent hepatic cysts. B Show 4 more Patients with biliary mucinous cystic neoplasms: Do not perform routine fluid aspiration in patients with suspected biliary cystadenoma because of the limited sensitivity and the risk of malignant dissemination. D Perform complete surgical excision, by an experienced team, in patients with imaging characteristics suggestive of biliary cystadenoma or biliary cystadenocarcinoma, such as internal septations, fenestrations, calcifications, or irregular walls. B Patients with hepatocellular adenoma: As per EASL 2016 guidelines, prefer MRI as it is superior to all other imaging modalities and due to its intrinsic properties to detect fat and vascular spaces it offers an opportunity to subtype hepatocellular adenoma up to 80%. B Show 11 more As per ACG 2014 guidelines, avoid the use of oral contraceptives, hormone-containing intrauterine devices, and anabolic steroids in patients with hepatocellular adenoma. D Show 4 more Patients with hepatocellular carcinoma: suspect HCC, until otherwise proven, in patients with chronic liver disease, especially with cirrhosis, presenting with a solid focal liver lesion, as they are at very high risk for having HCC. B Show 2 more Patients with cholangiocarcinoma: Obtain MRI or CT in patients with clinically or sonographically suspected cholangiocarcinoma. B Perform liver biopsy to establish the diagnosis of cholangiocarcinoma if the patient is non- operable. B https://web.pathway.md/diseases/recKkO8VoEkTQ5cHC 4/5 6/29/23, 4:06 AM Focal liver lesions Pathway Patients with hydatid cysts: prefer MRI over CT for concomitant evaluation of the biliary tree and cystic contents in patients with suspected hydatid cysts. B Show 4 more References 1. Expert Panel on Gastrointestinal Imaging, Victoria Chernyak, Jeanne M Horowitz et al. ACR Appropriateness Criteria Liver Lesion-Initial Characterization. J Am Coll Radiol. 2020 Nov;17 11S S429 S446. Open 2. Marrero JA, Ahn J, Rajender Reddy K et al. ACG clinical guideline: the diagnosis and management of focal liver lesions. Am J Gastroenterol. 2014 Sep;109 9 1328 47. Open 3. European Association for the Study of the Live. EASL Clinical Practice Guidelines on the management of cystic liver diseases. EASL. 2022 Jun. Open 4. European Association for the Study of the Liver EASL . EASL Clinical Practice Guidelines on the management of benign liver tumours. J Hepatol. 2016 Aug;65 2 386 98. Open 5. Elisabet Ars, Carmen Bernis, Gloria Fraga et al. Spanish guidelines for the management of autosomal dominant polycystic kidney disease. Nephrol Dial Transplant. 2014 Sep;29 Suppl 4:iv95 105. Open 6. Christoph F Dietrich, Christian P llson Nols e, Richard G Barr et al. Guidelines and Good Clinical Practice Recommendations for Contrast-Enhanced Ultrasound CEUS in the Liver-Update 2020 WFUMB in Cooperation with EFSUMB, AFSUMB, AIUM, and FLAUS. Ultrasound Med Biol. 2020 Oct;46 10 2579 2604. Open 7. Tram T Tran, Joseph Ahn, Nancy S Reau. ACG Clinical Guideline: Liver Disease and Pregnancy. Am J Gastroenterol. 2016 Feb;111 2 176 94; quiz 196. Open https://web.pathway.md/diseases/recKkO8VoEkTQ5cHC 5/5 |
Guideline sources The following summarized guidelines for the evaluation and management of focal nodular hyperplasia (FNH) are prepared by our editorial team based on guidelines from the American College of Radiology (ACR 2020), the European Federation of Societies for Ultrasound (EFSU 2020), the American College of Gastroenterology (ACG 2016; 2014), and the European Association for the Study of the Liver (EASL 2016). 1 2 3 4 5 6 Guidelines 1. Diagnostic investigations Contrast-enhanced ultrasound: As per ACR 2020 guidelines: Obtain contrast-enhanced abdominal ultrasound of an indeterminate > 1 cm liver lesion on initial ultrasound in patients with a normal liver with no suspicion or evidence of extrahepatic malignancy or underlying liver disease. B Obtain contrast-enhanced abdominal ultrasound of an incidental liver lesion > 1 cm on initial ultrasound, non-contrast or single-phase CT, or non-contrast MRI in patients with known chronic liver disease. B https://web.pathway.md/diseases/recJJ43FNQFht1T7R 1/4 6/29/23, 4:06 AM Focal nodular hyperplasia Pathway As per EFSU 2020 guidelines, obtain liver ultrasound using B-mode and Doppler mode before obtaining contrast-enhanced ultrasound to characterize focal liver lesions. B Show 14 more As per EASL 2016 guidelines, obtain contrast-enhanced ultrasound for the diagnosis of FNH (~ 100% specificity with typical imaging features observed in combination). Recognize that the highest diagnostic accuracy by contrast-enhanced ultrasound is achieved in FNH < 3 cm. B Cross-sectional imaging: As per ACR 2020 guidelines, obtain contrast-enhanced multiphase CT, contrast-enhanced MRI, or contrast-enhanced ultrasound of an indeterminate > 1 cm liver lesion on initial ultrasound in patients with a normal liver with no suspicion or evidence of extrahepatic malignancy or underlying liver disease. B Show 7 more As per EASL 2016 guidelines, obtain CT or MRI for the diagnosis of FNH (~ 100% specificity with typical imaging features observed in combination). Recognize that MRI has the highest diagnostic performance overall. B As per ACG 2014 guidelines, obtain MRI or CT for the diagnosis of FNH. B 2. Diagnostic procedures Liver biopsy: do not perform a routine liver biopsy to confirm the diagnosis of FNH. D 3. Medical management Indications for treatment: As per EASL 2016 guidelines, do not offer treatment in patients with FNH. D As per ACG 2014 guidelines, do not offer any therapeutic intervention in patients with asymptomatic FNH. D 4. Specific circumstances Pregnant patients: do not obtain routine imaging or surveillance for asymptomatic FNH during pregnancy. D Patients with hereditary hemorrhagic telangiectasia: suspect FNH in patients with HHT with liver masses, and obtain noninvasive, contrast-enhanced imaging for diagnostic confirmation. B Patients on oral contraceptives: Counsel patients that the use of oral contraceptives is not contraindicated in FNH. B Obtain annual ultrasound for 2-3 years in female patients with FNH wishing to continue the use of oral contraceptives. B https://web.pathway.md/diseases/recJJ43FNQFht1T7R 2/4 6/29/23, 4:06 AM Focal nodular hyperplasia Pathway 5. Patient education General counseling: counsel patients that pregnancy and the use of oral contraceptives or anabolic steroids are not contraindicated in FNH. B 6. Follow-up and surveillance Indications for referral: refer patients with atypical imaging findings or in the presence of symptoms to a benign liver tumor multidisciplinary team. B Serial imaging assessment: As per EASL 2016 guidelines, do not obtain follow-up imaging in patients with a typical lesion of FNH unless there is underlying vascular liver disease. D As per ACG 2014 guidelines: Do not obtain follow-up imaging in patients with a firm diagnosis of FNH unless oral contraceptives are used. D Obtain annual ultrasound for 2-3 years in female patients with FNH wishing to continue the use of oral contraceptives. B Clinical findings Symptoms Past medical history Abdominal pain Budd-chiari syndrome HHT Abdominal exam Hepatic AVM Hepatic adenoma Hepatomegaly Hepatic hemangioma Palpable abdominal mass Imaging findings Hepatic mass with central scar Spoke wheel sign References 1. Marrero JA, Ahn J, Rajender Reddy K et al. ACG clinical guideline: the diagnosis and management of focal liver lesions. Am J Gastroenterol. 2014 Sep;109 9 1328 47. Open 2. Expert Panel on Gastrointestinal Imaging, Victoria Chernyak, Jeanne M Horowitz et al. ACR Appropriateness Criteria Liver Lesion-Initial Characterization. J Am Coll Radiol. 2020 Nov;17 11S S429 S446. Open 3. Tram T Tran, Joseph Ahn, Nancy S Reau. ACG Clinical Guideline: Liver Disease and Pregnancy. Am J Gastroenterol. 2016 Feb;111 2 176 94; quiz 196. Open 4. European Association for the Study of the Liver EASL . EASL Clinical Practice Guidelines on the management of benign liver tumours. J Hepatol. 2016 Aug;65 2 386 98. Open https://web.pathway.md/diseases/recJJ43FNQFht1T7R 3/4 6/29/23, 4:06 AM Focal nodular hyperplasia Pathway 5. European Association for the Study of the Liver. EASL Clinical Practice Guidelines: Vascular diseases of the liver. J Hepatol. 2016 Jan;64 1 179 202. Open 6. Christoph F Dietrich, Christian P llson Nols e, Richard G Barr et al. Guidelines and Good Clinical Practice Recommendations for Contrast-Enhanced Ultrasound CEUS in the Liver-Update 2020 WFUMB in Cooperation with EFSUMB, AFSUMB, AIUM, and FLAUS. Ultrasound Med Biol. 2020 Oct;46 10 2579 2604. Open 7. Monika Sarkar, Carla W Brady, Jaquelyn Fleckenstein et al. Reproductive Health and Liver Disease: Practice Guidance by the American Association for the Study of Liver Diseases. Hepatology. 2021 Jan;73 1 318 365. Open https://web.pathway.md/diseases/recJJ43FNQFht1T7R 4/4 |
Guideline sources The following summarized guidelines for the evaluation and management of focal segmental glomerulosclerosis are prepared by our editorial team based on guidelines from the Kidney Disease: Improving Global Outcomes Foundation (KDIGO 2021) and the Japanese Society of Nephrology (JSN 2016). 1 2 Guidelines 1. Diagnostic investigations Initial evaluation: Evaluate for secondary causes in adult patients with FSGS without nephrotic syndrome. B Consider referring selected patients with FSGS to specialized centers for genetic testing. C 2. Medical management Corticosteroids: As per KDIGO 2021 guidelines, initiate high-dose oral corticosteroids as first-line immunosuppressive treatment in patients with primary FSGS. B Show 2 more As per JSN 2016 guidelines: Initiate corticosteroids to reduce the urinary protein level and to prevent the decline of renal function in patients with FSGS. B https://web.pathway.md/diseases/recfXCIpSQxTJPNle 1/3 6/29/23, 4:06 AM Focal segmental glomerulosclerosis Pathway Consider administering corticosteroid pulse therapy when absorption of oral corticosteroids seems difficult. Immunosuppressants: As per KDIGO 2021 guidelines, do not initiate immunosuppression in adult patients with FSGS of undetermined cause or in patients with secondary FSGS. D Show 4 more As per JSN 2016 guidelines, initiate a combination of cyclosporine and corticosteroids to reduce the urinary protein level in patients with corticosteroid-resistant FSGS. B Show 3 more 3. Follow-up and surveillance Management of relapse: consider managing adult patients with previous corticosteroid- sensitive primary FSGS experiencing a relapse as adult patients with relapsing minimal change disease. C Clinical findings Patient demographics Symptoms African-american race Ankle swelling Fatigue Past medical history Headache Malaise Nephrotic syndrome Puffy eyelids Vascular exam Weight gain Peripheral edema Vital signs Abdominal exam Hypertension Abdominal distension Neurological exam Ascites Lethargy Gynecologic exam Genitourinary exam Vulvar swelling Scrotal edema Hematological findings Lab findings Anemia Hyperlipidemia serum LDL serum urea urine protein serum HDL serum albumin https://web.pathway.md/diseases/recfXCIpSQxTJPNle 2/3 6/29/23, 4:06 AM Focal segmental glomerulosclerosis Pathway References 1. Kidney Disease: Improving Global Outcomes KDIGO Glomerular Diseases Work Group. KDIGO 2021 Clinical Practice Guideline for the Management of Glomerular Diseases. Kidney Int. 2021 Oct;100 4S S1 S276. Open 2. Nishi S, Ubara Y, Utsunomiya Y et al. Evidence-based clinical practice guidelines for nephrotic syndrome 2014. Clin Exp Nephrol. 2016 Jun;20 3 342 70. Open 3. No authors listed. Chapter 6 Idiopathic focal segmental glomerulosclerosis in adults. Kidney Int Suppl 2011 . 2012 Jun;2 2 181 185. Open 4. Anand Padmanabhan, Laura Connelly-Smith, Nicole Aqui et al. Guidelines on the Use of Therapeutic Apheresis in Clinical Practice Evidence-Based Approach from the Writing Committee of the American Society for Apheresis: The Eighth Special Issue. J Clin Apher. 2019 Jun;34 3 171 354. Open https://web.pathway.md/diseases/recfXCIpSQxTJPNle 3/3 |
Guideline sources The following summarized guidelines for the evaluation and management of folate deficiency are prepared by our editorial team based on guidelines from the British Society for Haematology (BSH 2014). 1 2 2 3 4 Definition Folate deficiency is dietary folate equivalents < 400 ug/day in adults and < 600 ug/day in pregnant women characterized by megaloblastic anemia, and risk of neural tube defects and congenital anomalies. 2 Epidemiology Folate deficiency is mostly caused by inadequate folate intake, pregnancy and lactation, alcoholism, gene polymorphism (C1561T), and certain medications (methotrexate, anticonvulsants, sufasalazine, pyrimethamine). 3 Pathophysiology The prevalence of folate deficiency anemia in the United States adults is estimated at < 0.1%. Disease course Clinical manifestations include megaloblastic anemia and increased incidence of various cancers (colorectal, prostate, and breast cancer). Pregnancy-related complications include neural tube defects )spina bifida, anencephaly), and other congenital anomalies (congenital heart defects, https://web.pathway.md/diseases/recxI4tG9TotECfWJ 1/4 6/29/23, 4:06 AM Folate deficiency Pathway oral cleft lip and palate) in neonates, maternal megaloblastic anemia, low infant birth weight, stillbirth, and premature delivery. 2 Prognosis and risk of recurrence Folate deficiency is associated with increased all-cause mortality with a hazard ratio of 1.33 (95% CI 1.01-1.76). 4 Pathway Folate deficiency Diagnosis and management Guidelines 1. Screening and diagnosis Indications for testing: test for folate deficiency in clinical situations similar to those in which testing for cobalamin deficiency is obtained. A Diagnostic criteria: recognize that a serum folate level < 7 nmol/L (< 3 g/L) is indicative of folate deficiency. B 2. Diagnostic investigations Serum folate and cobalamin: obtain cobalamin and folate assays concurrently, given the close relationship in their metabolism. A CBC and peripheral blood smear: consider suspecting cobalamin or folate deficiency in patients in whom blood film shows oval macrocytes and hypersegmented neutrophils in the presence of an elevated MCV. C Red blood cell folate: Avoid routine testing of RBC folate, since serum folate alone is sufficient in most cases. D Consider RBC folate assays in patients in whom serum folate is normal yet folate deficiency is strongly suspected, after having excluded cobalamin deficiency. C Serum homocysteine: consider plasma total homocysteine levels to confirm suspected folate deficiency only in special circumstances; consider a level above 15 mol/L as indicative of folate deficiency, but assess in relation to local reference ranges. C Evaluation for medication-associated folate deficiency: consult with pharmacy references to clarify any suspicion of low serum folate levels associated with prescribed medications. 3. Medical management Folate supplementation: https://web.pathway.md/diseases/recxI4tG9TotECfWJ 2/4 6/29/23, 4:06 AM Folate deficiency Pathway Treat folate deficiency according to the following schedules : due to dietary insufficiency, pregnancy, or antiepileptics: folic acid 5 mg PO daily for 4 months due to malabsorptive states: consider folic acid 15 mg PO daily for 4 months. A Continue folic acid supplementation until term in pregnant patients. 4. Specific circumstances Patients with pernicious anemia: avoid initial treatment with oral cobalamin in pernicious anemia, but consider in maintenance or correction of suboptimal levels in asymptomatic patients. D Clinical findings Symptoms Past medical history Confusion Lack of folic acid in diet Depression Medications Fatigue Visual disorder Memory impairment Social history Mouth ulcers Muscle weakness Alcohol consumption Paresthesia Hematological findings Past obstetric history Anemia Pregnancy Macrocytic anemia Pancytopenia Lab findings Hgb MCV reticulocyte count serum MMA serum homocysteine serum folate Histological findings Findings of celiac disease References 1. Devalia V, Hamilton MS, Molloy AM et al. Guidelines for the diagnosis and treatment of cobalamin and folate disorders. Br J Haematol. 2014 Aug;166 4 496 513. Open 2. Yen-Ming Chan, Regan Bailey, Deborah L O'Connor. Folate. Adv Nutr. 2013 Jan 1;4 1 123 5. Open 3. Lindsay H Allen. Causes of vitamin B12 and folate deficiency. 2008 Jun;29 2 Suppl):S20 34; discussion S35 7.2008 Jun;29 2 Suppl):S20 34; discussion S35 7. Open https://web.pathway.md/diseases/recxI4tG9TotECfWJ 3/4 6/29/23, 4:06 AM Folate deficiency Pathway 4. Yang Peng, Bin Dong, Zhiqiang Wang. Serum folate concentrations and all-cause, cardiovascular disease and cancer mortality: A cohort study based on 1999 2010 National Health and Nutrition Examination Survey NHANES . 2016 Sep 15;219 136 42.2016 Sep 15;219 136 42. Open 5. Snow CF. Laboratory diagnosis of vitamin B12 and folate deficiency: a guide for the primary care physician. Arch Intern Med. 1999 Jun 28;159 12 1289 98. Open 6. Oluwaseun A Odewole, Rebecca S Williamson, Neil A Zakai et al. Near-elimination of folate- deficiency anemia by mandatory folic acid fortification in older US adults: Reasons for Geographic and Racial Differences in Stroke study 2003 2007. Am J Clin Nutr. 2013 Oct;98 4 1042 7. Open 7. Lindsay H Allen. Causes of vitamin B12 and folate deficiency. Food Nutr Bull. 2008 Jun;29 2 Suppl):S20 34; discussion S35 7. Open 8. Yang Peng, Bin Dong, Zhiqiang Wang. Serum folate concentrations and all-cause, cardiovascular disease and cancer mortality: A cohort study based on 1999 2010 National Health and Nutrition Examination Survey NHANES . Int J Cardiol. 2016 Sep 15;219 136 42. Open 9. Julie Parrott, Laura Frank, Rebecca Rabena et al. American Society for Metabolic and Bariatric Surgery Integrated Health Nutritional Guidelines for the Surgical Weight Loss Patient 2016 Update: Micronutrients. Surg Obes Relat Dis. 2017 May;13 5 727 741. Open https://web.pathway.md/diseases/recxI4tG9TotECfWJ 4/4 |