We are dealing with a severe patient, with target lesions and Nikolsky's sign in > 30% of the body surface area. Together with the history of treatment with carbamazepine, the suspicion will be of toxic epidermal necrolysis (answer 4). If the epidermal detachment is less than 10% then we would be dealing with Stevens-Johnson (and intermediate cases are referred to as overlap, since it is a whole spectrum). Incidentally, we would recommend immediate admission to a major burn unit.

We are dealing with a severe patient, with target lesions and Nikolsky's sign in > 30% of the body surface area. Together with the history of treatment with carbamazepine, the suspicion will be of toxic epidermal necrolysis ([HIDDEN]). If the epidermal detachment is less than 10% then we would be dealing with Stevens-Johnson (and intermediate cases are referred to as overlap, since it is a whole spectrum). Incidentally, we would recommend immediate admission to a major burn unit.

A 60-year-old man on carbamazepine treatment for epilepsy presenting with fever (38.8°C), odynophagia, conjunctivitis, brownish skin lesions spread over a large body surface, target-like in appearance and accompanied by epidermal detachment (greater than 30%) at the slightest friction. It is very likely that she is presenting a clinical picture of:

Acute urticaria of pharmacological cause.

Erythema minor exudative secondary to drugs.

Toxic epidermal necrolysis and Stevens-Johnson syndrome.

Toxic epidermal necrolysis (TEN) and Stevens Johnson Syndrome (SJS) are severe adverse cutaneous drug reactions that predominantly involve the skin and mucous membranes. Both are rare, with TEN and SJS affecting approximately 1or 2/1,000,000 annually, and are considered medical emergencies as they are potentially fatal. They are characterized by mucocutaneous tenderness and typically hemorrhagic erosions, erythema and more or less severe epidermal detachment presenting as blisters and areas of denuded skin. Currently, TEN and SJS are considered to be two ends of a spectrum of severe epidermolytic adverse cutaneous drug reactions, differing only by their extent of skin detachment. Drugs are assumed or identified as the main cause of SJS/TEN in most cases, but Mycoplasma pneumoniae and Herpes simplex virus infections are well documented causes alongside rare cases in which the aetiology remains unknown. Several drugs are at "high" risk of inducing TEN/SJS including: Allopurinol, Trimethoprim-sulfamethoxazole and other sulfonamide-antibiotics, aminopenicillins, cephalosporins, quinolones, carbamazepine, phenytoin, phenobarbital and NSAID's of the oxicam-type. Genetic susceptibility to SJS and TEN is likely as exemplified by the strong association observed in Han Chinese between a genetic marker, the human leukocyte antigen HLA-B*1502, and SJS induced by carbamazepine. Diagnosis relies mainly on clinical signs together with the histological analysis of a skin biopsy showing typical full-thickness epidermal necrolysis due to extensive keratinocyte apoptosis. Differential diagnosis includes linear IgA dermatosis and paraneoplastic pemphigus, pemphigus vulgaris and bullous pemphigoid, acute generalized exanthematous pustulosis (AGEP), disseminated fixed bullous drug eruption and staphyloccocal scalded skin syndrome (SSSS). Due to the high risk of mortality, management of patients with SJS/TEN requires rapid diagnosis, evaluation of the prognosis using SCORTEN, identification and interruption of the culprit drug, specialized supportive care ideally in an intensive care unit, and consideration of immunomodulating agents such as high-dose intravenous immunoglobulin therapy. SJS and TEN are severe and life-threatening. The average reported mortality rate of SJS is 1-5%, and of TEN is 25-35%; it can be even higher in elderly patients and those with a large surface area of epidermal detachment. More than 50% of patients surviving TEN suffer from long-term sequelae of the disease.

Severe carbamazepine-induced cutaneous reaction in the treatment of post-herpetic neuralgia. Case report.

Post-herpetic neuralgia (PHN) is the main complication of herpes zoster. Carbamazepine (CBZ), a well-tolerated anticonvulsant, but frequently associated with severe cutaneous reactions, such as the Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) is used in the treatment of this complication. The objective of this article was to report a case of SJS/TEN secondary to CBZ in a patient with PHN. This is a female patient with continuous severe, burning, chock-like pain in the thoracic region and dorsum associated with reduced strength in the ipsilateral upper limb and diaphoresis. She had crusty and erythematous lesions in the dorsal region of the thorax with allodynia and dysesthesia in the affected dermatome. She was treated with CBZ 300 mg.day(-1), amitriptyline (AMT) 12.5 mg at bedtime, and infiltration with local anesthetic in the affected region. After 15 days, she developed malaise, fever, muscle pain, and arthralgia with a mild non-specific cutaneous rash. Carbamazepine was discontinued immediately. One week later, she was hospitalized with urticaria, generalized exanthema, erythematous cutaneous eruptions, bullae, and purpuric maculae all over her body. The impression was of carbamazepine-induced SJS/TEN. She evolved with progressive worsening of her symptoms, with increase in the number and size of cutaneous lesions, besides generalized erythematous macular rash, areas of necrosis, and erosions with symmetrical loosening of the epidermis in face, neck, thorax, dorsum, and limbs, affecting more that 50% of her body surface, besides involvement of buccal, conjunctival, and genital mucosa with vesicular erosions. She had progressive functional worsening, evolving to septic shock and multiple organ failure followed by death. Stevens-Johnson syndrome and toxic epidermal necrolysis are severe cutaneous reaction with potential for elevated morbidity and mortality that requires immediate intervention and adequate management. In addition, we would like to alert that the use of Carbamazepine should be supervised, especially in the elderly.

Toxic Epidermal Necrolysis: A Case Report on a Drug-Induced Phenomenon.

Toxic epidermal necrolysis (TEN) is a group of severe forms of several life-threatening conditions. As a co-infection of this group, Stevens-Johnson syndrome (SJS) is a rare though severe disease of the skin and mucous membranes. Intake of some drugs could cause reactions such as SJS and TEN. A form of severe connective tissue disorder, TEN is also known as Lyell's syndrome and is a common cause of significant skin and mucous membrane disintegration. Adverse medication reactions are the most prevalent and contribute highly to the incidence rates of the major etiological variables for TEN. Erythema, epidermal detachment that manifests as blisters, and denuded skin patches are the defining features of this pathology. In the majority of cases, the administration of pharmaceutical drugs is thought to be the primary cause of SJS/TEN. In this article, we report a case of a 33-year-old male patient who presented with complaints of lower left facial pain and thus was prescribed carbamazepine. Following this, the patient presented with an adverse reaction to the administration of carbamazepine and was taken off the drug immediately. The treatment included the administration of hydration therapy and appropriate antibiotics for treating the fluid-filled vesicles. The treatment regimen continued for three weeks and was stopped when the skin lesions were scarce and there was an improvement in the overall health of the patient.

Fatal toxic epidermal necrolysis induced by carbamazepine treatment in a patient who previously had carbamazepine-induced Stevens-Johnson syndrome.

Toxic epidermal necrolysis (TEN) is a rare but life-threatening skin disease that is most commonly drug-induced. It has recently been suggested that Stevens-Johnson syndrome (SJS) belongs to the same group of skin disorders, although it has a lower mortality rate than TEN. We report the case of a 26-year-old male schizophrenic patient with a history of carbamazepine-induced SJS 5 years earlier. At the time of his current admission, he was admitted to our psychiatry department with acute agitation due to schizophrenia. However, the patient and his family denied history of drug allergy. After 3 days of carbamazepine treatment, the patient developed TEN (body surface area > 90%). He was transferred to the burn center, but despite appropriate treatment, including intravenous hydrocortisone 200 mg q6h and being covered with sterile biological material, he died. It is important to note that re-administration of a drug that previously caused SJS may lead to TEN, which has a very high mortality rate.

Carbamazepine Induced Stevens-Johnson Syndrome That Developed into Toxic Epidermal Necrolysis: Review of the Literature.

Stevens-Johnson syndrome and toxic epidermal necrolysis are both skin diseases believed to be following the pattern of a type IV hypersensitivity mechanism, which can be triggered by infectious agents or administration of a variety of drugs as part of the spectrum of severe cutaneous adverse reactions (SCARs). Fever and blisters, that peel forming painful raw areas, are early symptoms of this condition, and complications such as dehydration, sepsis, pneumonia, and multiple organ failure are typically seen during the course of the disease. <iCase Presentation</i. We present a case of a 23-year-old female patient referred to our hospital after taking carbamazepine and developing high-grade fever and ulcers that appeared initially in her mouth and face but then progressed despite treatment, extending all over her body and involving about 90% of her BSA. The use of IVIG and plasmapheresis was a good management for our case, helping in our patient's well-being and recovery. Even if there is no stipulated guideline treatment for cases of SJS and TEN, we think that further investigations about IVIG and plasmapheresis should be investigated as a possible way to treat both conditions.

[Drug-induced toxic epidermal necrolysis with secondary aspergillus fumigatus infection: a case report].

Among the various drug induced dermatological entities toxic epidermalnecrolysis (TEN) and Stevens-Johnson syndrome (SJS) occupy a primary place in terms of mortality. Toxic epidermal necrolysis also known as Lyell's syndrome was first described by Lyell in 1956. Drugs are by far the most common cause of toxic epidermal necrolysis, in which large sheets of skin are lost from the body surface making redundant the barrier function of the skin, with its resultant complications. Drug-induced toxic epidermal necrolysis are severe adverse cutaneous drug reactions to various precipitating agents that predominantly involve the skin and mucous membranes. Toxic epidermal necrolysis is rare but considered medical emergencies as they are potentially fatal. Drugs are the most common cause accounting for about 65%-80% of the cases. The most common offending agents are sulfonamides, NSAIDs, butazones and hydrantoins. An immune mechanism is implicated in the pathogenesis, but its nature is still unclear. There is a prodormal phase in which there is burning sensation all over the skin and conjunctivae, along with skin tenderness, fever, malaise and arthralgias. Early sites of cutaneous involvement are the presternal region of the trunk and the face, but also the palms and soles, rapidly spread to their maximum extent, the oral mucosa and conjunctiva being affected. Initial lesions are macular, followed by desquamateion, or may be from atypical targets with purpuriccenters that coalesce, from bullae, then slough. The earlier a causative agent is withdrawn the better is the prognosis. Several treatment modalities given in addition to supportive care are reported in the literature, such as systemicsteroids, high-dose intravenous immunoglobulins, ciclosporin, TNF antagonists. Recovery is slow over a period of 14-28 days and relapses are frequent. Mortality is 25%-50% and half the deaths occur due to secondary infection. Here we report a 50-year-old female of drug-induced toxic epidermal necrolysis. She was admitted to the dermatology ward with extensive peeling of skin over the trunk and limbs. She had taken alamotrigine for epilepsy. A week after taking the tablets, the patient developed a severe burning sensation all over the body and followed by a polymorphic erythematous dermatitis and widespread peeling of skin. We treated this patient with high dose corticosteroids, high-dose intravenous immunoglobulins and etanercept, but eventually she died of secondary aspergillus fumigatus infection.

Clinical classification of cases of toxic epidermal necrolysis, Stevens-Johnson syndrome, and erythema multiforme.

To conduct a prospective case-control study about causative factors of severe bullous erythema multiforme, Stevens-Johnson syndrome, and toxic epidermal necrolysis, we needed to define criteria for classifying the cases and standardize the collection of data so that cases could be reliably diagnosed according to this classification. Based on review of case histories and photographs of patients, a group of experts proposed a classification based on the pattern of erythema multiforme-like lesions (categorized as typical targets, raised or flat atypical targets, and purpuric macules) and on the extent of epidermal detachment. An atlas illustrating this classification that included photographs and schematic drawings was developed. We compared the evaluations of 28 cases by four nonphysicians relying on the atlas with the evaluations of the same cases by five experts not using the atlas to determine the usefulness of this atlas for classifying cases according to our nosologic schema. The following consensus classification in five categories was proposed: bullous erythema multiforme, detachment below 10% of the body surface area plus localized "typical targets" or "raised atypical targets"; Stevens-Johnson syndrome, detachment below 10% of the body surface area plus widespread erythematous or purpuric macules or flat atypical targets; overlap Stevens-Johnson syndrome-toxic epidermal necrolysis, detachment between 10% and 30% of the body surface area plus widespread purpuric macules or flat atypical targets; toxic epidermal necrolysis with spots, detachment above 30% of the body surface area plus widespread purpuric macules or flat atypical targets; and toxic epidermal necrolysis without spots, detachment above 10% of the body surface area with large epidermal sheets and without any purpuric macule or target. Using the atlas, the nonexperts showed excellent agreement with the experts. This study suggests that an illustrated atlas is a useful tool for standardizing the diagnosis of acute severe bullous disorders that are attributed to drugs or infectious agents. Whether the five categories proposed represent distinct etiopathologic entities will require further epidemiologic and laboratory investigations.

Stevens-Johnson syndrome-toxic epidermal necrolysis (SJS-TEN) overlap associated with carbamazepine use.

A benign pruritic rash occurs in 10%-15% of persons treated with carbamazepine. A small fraction of them may experience life-threatening dermatological syndromes such as exfoliative dermatitis, erythema multiforme, Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN). The case of an 18-year-old female suffering from bipolar affective disorder (mania) who was being treated with carbamazepine, lithium, chlorpromazine and benzhexol is presented. After 10 days of treatment, she developed high-grade fever and mucocutaneous manifestations of SJS-TEN overlap. She was treated in hospital with systemic corticosteroids, antibiotics, intravenous fluids and other supportive measures, and recovered after 3 weeks.

A 51-Year-Old Woman with Drug-Induced Hypersensitivity Syndrome Associated with Carbamazepine, Reactivation of Human Herpesvirus 6, and Acute Liver Failure: A Case Report.

BACKGROUND Infection with human herpesvirus 6 (HHV-6) is a recognized risk factor for the development of drug-induced hypersensitivity syndrome (DIHS). DIHS is a systemic autoimmune condition that presents with mucocutaneous lesions of varying severity and comprises 3 subtypes: toxic epidermal necrolysis, Stevens-Johnson syndrome, and drug reaction with eosinophilia and systemic symptoms (DRESS). Here, we describe the case of a 51-year-old woman with a diagnosis of DIHS associated with carbamazepine, reactivation of HHV-6, and acute liver failure, which was consistent with DRESS. CASE REPORT We present the case of a 51-year-old Japanese woman who had been taking carbamazepine for epilepsy for the past 3 weeks. She presented with a fever, liver dysfunction, eosinophilia, and the sudden appearance of a skin rash. Steroid therapy was started for suspected drug-induced liver injury. The skin eruption disappeared, and liver dysfunction showed an improving trend. However, after stopping steroid, the pyrexia and eosinophilia reappeared. Therefore, prednisolone was re-administrated. HHV-6 DNA was detected, so HHV-6 reactivation was confirmed. Carbamazepine was stopped, and the clinical manifestations improved. She was ultimately diagnosed with DIHS, consistent with DRESS, associated with carbamazepine and HHV-6 reactivation, and liver dysfunction was assessed histologically. Therefore, the drug-related hepatotoxicity of carbamazepine played a role in causing liver damage rather than HHV-6 infection at that time. CONCLUSIONS We describe a case of DIHS that was also associated with acute liver failure, consistent with DRESS. The case highlights the importance of making the correct diagnosis, as well as the management of mucocutaneous lesions and other systemic conditions (including acute liver failure).

Severe cutaneous reactions caused by barbiturates in seven Iranian children.

The severe adverse cutaneous reactions of erythema multiforme (EM), Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare mucocutaneous diseases associated with significant morbidity and mortality. The most common cause is antiepileptic drugs, particularly carbamazepine and lamotrigine, as well as the barbiturates group (phenobarbital and phenytoin). In this article, we present seven children with severe adverse cutaneous reactions caused by barbiturates. The age of the affected children was between 2 and 11 years and they all had a history of taking barbiturates. Their symptoms started 1-3 weeks after the initiation of barbiturates, including a prodrome characterized by 2-3 days of malaise, fever, cough and anorexia, after which the skin and mucosal lesions appeared and worsened. The skin lesions varied from rash to large bullae, plus different forms of mucous membrane involvement. The offending drugs (barbiturates) were stopped immediately and care was largely supportive. As a result of the morbidity and/or mortality associated with EM, SJS and TEN, physicians should keep in mind their differential diagnosis when cutaneous reactions are observed in patients undergoing barbiturate therapy. Furthermore, although TEN and SJS are life-threatening diseases, early detection and appropriate care can lead to a decrease in the incidence of death. The strategies described here seem to be successful and safe because, despite the serious conditions, our patients responded well. All survived.

Carbamazepine induced toxic epidermal necrolysis and Stevens-Johnson syndrome overlapping during pregnancy in a South-East Asian patient: A case report.

Toxic Epidermal Necrolysis (TEN) and Stevens-Johnson syndrome (SJS) are rare and severe forms of drug-induced skin reaction. Most frequently involved drugs are noted to be non-steroidal anti-inflammatory agents, antibiotics, and anticonvulsants. These have high morbidity and mortality and counts among dermatological emergencies. We report an eventful case of a 22-year-old lady who suffered and recovered from carbamazepine-induced SJS/TEN overlapping during her pregnancy. Our patient had a history of epilepsy for which she was under sodium valproate. Switching to carbamazepine due to its low teratogenicity led our patient to this condition. History of prodromal symptoms and exposure to carbamazepine helped in the diagnosis. Carbamazepine abstinence and a multidisciplinary approach in symptomatic management worked very well for the patient. Carbamazepine-induced TES/SJS manifests multisystem effects and requires a multidisciplinary approach for management. The condition itself is life-threatening and in its addition, their sequelae further threaten the life of the patients. Early intervention is the key. Genetically susceptible are thought to be the ones carrying human leukocyte antigen B*15:02 (HLA-B*15:02) allele and it is most prevalent in South-East Asian populations. Screening of this allele before using carbamazepine prevents the incidence of carbamazepine-induced SJS/TEN. Prodromal symptoms of carbamazepine-induced SJS/TEN constitute flu-like symptoms that should not be missed. Early intervention and multidisciplinary approach prevent secondary infections and complications. Screening for HLA-B*15:02 variant allele and close monitoring of these adverse reactions along with proper counseling to patients goes a long way in preventing the development of this life-threatening condition.

Stevens–Johnson syndrome

Stevens–Johnson syndrome (SJS) is a type of severe skin reaction. Together with toxic epidermal necrolysis (TEN) and Stevens–Johnson/toxic epidermal necrolysis (SJS/TEN), it forms a spectrum of disease, with SJS being less severe. Erythema multiforme (EM) is generally considered a separate condition. Early symptoms of SJS include fever and flu-like symptoms. A few days later, the skin begins to blister and peel, forming painful raw areas. Mucous membranes, such as the mouth, are also typically involved. Complications include dehydration, sepsis, pneumonia and multiple organ failure. The most common cause is certain medications such as lamotrigine, carbamazepine, allopurinol, sulfonamide antibiotics and nevirapine. Other causes can include infections such as Mycoplasma pneumoniae and cytomegalovirus, or the cause may remain unknown. Risk factors include HIV/AIDS and systemic lupus erythematosus.

[Stevens-Johnson syndrome with transition to toxic epidermal necrolysis after carbamazepine administration, heroin and alcohol abuse].

A 28 year old patient developed a severe bullous exanthem and enanthem combined with hepatitis, fever and blood count abnormalities after taking carbamazepine and consumption of heroin and alcohol. After discontinuing carbamazepine, prednisolone was given over a five day period accompanied by intravenous fluid and electrolyte substitution and local therapy which lead to improvement. Severe bullous skin reactions nowadays are classified into erythema exsudativum multiforme majus (EEMM), Stevens-Johnson syndrome (SJS), overlap Stevens-Johnson syndrome-toxic epidermal necrolysis (SJS/TEN), TEN with maculae and TEN on large erythema, and they are most often caused by antibiotics and anticonvulsant drugs. Heroin and alcohol abuse alters host immunity which subsequently may increase susceptibility to allergic reactions. There is a high (40%) mortality rate for TEN, and patients with organ involvement are at increased risk.

[Severe drug-related skin reaction: toxic epidermal necrolysis caused by carbamazepine].

Drug-related skin reactions often present as macular, maculopapular or urticarial rashes. Severe drug eruptions are rare, with life-threatening events occurring in about 4 per one million persons a year. In pediatric patients the estimated incidence of Stevens-Johnsons-syndrome (SJS) and toxic epidermal necrolysis (TEN) is even lower. They are often caused by antiepileptic drugs like carbamazepine. A 10 (5)/ (12) year old boy of dark complexion suddenly developed a severe bullous skin disease six weeks after starting carbamazepine therapy due to focal epilepsy. Within few hours a life-threatening systemic inflammatory reaction occurred with subsequent respiratory failure. The clinical course was complicated by bacterial sepsis, bilateral thrombosis of the external iliacal veins and bilateral ocular symblephara. The skin healed with considerable pigmentary disturbance. We report the differential diagnostic and therapeutical features of toxic epidermal necrolysis and give a review of the literature.

Drug-induced Stevens-Johnson syndrome: case series from tertiary care centre in Gujarat.

The aims of the present article were to study clinical features and to analyse them in different drug class associated with Stevens-Johnson syndrome (SJS) in a tertiary care hospital in Gujarat, India. A prospective hospital-based study was carried out over a period of 3 years (June 2007 to September 2009) at Sheth Vadilal Hospital, Ahmedabad, India. The diagnosis of SJS was made mainly on the basis of the clinical findings, which included extensive erythema multiforme, purpuric lesions with bullae and detachment of skin involving at least two mucous membranes. Further, in each patient suspected with SJS, various laboratory tests such as complete blood count, liver function tests, metabolic panel, chest X-ray and other serological test were carried out. SJS was confirmed on the basis of most widely accepted Bastuji-Garin definition. Causality assessment was performed using the Naranjo scale. Only 'probable' and 'definite' reactions were included. Antibacterials for systemic use, anti-inflammatory and antirheumatic products and antiepileptics were the drug classes most commonly associated (8 of 29 cases, each) with SJS. Individually, ibuprofen was involved in the highest number of cases (five cases, 17.2%), followed by carbamazepine (four cases, 13.8%) . The mean duration of developing SJS symptoms was 15.9 days (SD = 8.7 days) and improvement after treatment was 14.2 days (SD = 4.6 days). The duration of appearing SJS symptoms varied significantly between different classes of drugs (p < 0.001). The appearance of SJS symptom started within 10 days for anti-inflammatory and antibacterial compared with 24 days of antiepileptic agents. All the patients with antiepileptic agent-induced SJS had 7% to 9% of detached body surface area. In two patients, SJS progressed to toxic epidermal necrolysis and of which one led to death and the other developed long-term complication of conjunctival xerosis. A total of six patients developed long-term complications: four patients had conjunctival synechia, one patient had conjunctival xerosis and one patient had urethral stricture. More than 80% of the SJS events were induced by antibacterial, anti-inflammatory and antiepileptic agents with same frequency. The duration of the appearance of SJS symptoms significantly varied between different drug classes and started within 10 days for anti-inflammatory and antibacterial compared with 24 days of antiepileptic agents.

Erythema multiforme major

Erythema multiforme major is a form of rash with skin loss or epidermal detachment. The term "erythema multiforme majus" is sometimes used to imply a bullous (blistering) presentation. According to some sources, there are two conditions included on a spectrum of this same disease process: Stevens–Johnson syndrome (SJS) Toxic epidermal necrolysis (TEN) which described by Alan Lyell and previously called Lyell syndrome[5]. In this view, EM major, SJS and TEN are considered a single condition, distinguished by degree of epidermal detachment. However, a consensus classification separates erythema multiforme minor, erythema multiforme major, and SJS/TEN as three separate entities. References 5. Orphanet Journal of Rare Diseases 2010, 5:39 doi:10.1186/1750-1172-5-39 External links Drug eruptions

Successful Treatment of Carbamazepine-Induced Toxic Epidermal Necrolysis With Clinical Gastrointestinal Involvement: A Case Report.

Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) is a rare and life-threatening disease of the skin and mucosal surfaces. Although gastrointestinal manifestations in adults are potential prognostic factors for disease severity, there are limited data on such cases and their standard management in the pediatric population. We herein report the case of an 8-year-old girl with a 1-year history of epilepsy, who presented with bilateral conjunctivitis and progressively widespread bullous, and pruritic eruption based on erythematous skin after administration of carbamazepine. A diagnosis of carbamazepine-induced TEN was made, and the drug was immediately discontinued. The result of genetic screening showed that the patient was positive for the HLA-B<sup*</sup15:02 allele. Then, her condition got worse by developing gastrointestinal involvement, including hematemesis and severe watery bloody diarrhea. A combination of the intravenous immunoglobulin and the appropriate dose of systemic steroids have contributed to a favorable outcome in this case. Multidisciplinary care of mucocutaneous involvement, supplemental nutrition, and fluid replacement was also critically warranted. This report aims to contribute to the current literature on TEN-related gastrointestinal manifestations in pediatrics and highlights the need for further investigations in determining the optimal treatment in such cases. In conclusion, we reported the successful treatment of TEN-related gastrointestinal manifestations in a pediatric patient, which should be critically considered in patients with SJS/TEN. Since it may significantly contribute to the poor prognosis of the illness, further investigations in determining standard management in such cases are necessary.

Stevens–Johnson syndrome

Stevens–Johnson syndrome (SJS) is a milder form of toxic epidermal necrolysis (TEN). These conditions were first recognised in 1922. A classification first published in 1993, that has been adopted as a consensus definition, identifies Stevens–Johnson syndrome, toxic epidermal necrolysis, and SJS/TEN overlap. All three are part of a spectrum of severe cutaneous reactions (SCAR) which affect skin and mucous membranes. The distinction between SJS, SJS/TEN overlap, and TEN is based on the type of lesions and the amount of the body surface area with blisters and erosions. It is agreed that the most reliable method to classify EM, SJS, and TEN is based on lesion morphology and extent of epidermal detachment. Blisters and erosions cover between 3% and 10% of the body in SJS, 11–30% in SJS/TEN overlap, and over 30% in TEN. The skin pattern most commonly associated with SJS is widespread, often joined or touching (confluent), papuric spots (macules) or flat small blisters or large blisters

First_Aid_Step2

Patients with erythema nodosum may have a falseVDRL (as in SLE). SJS and TEN constitute two different points on the spectrum of life-threatening exfoliative mucocutaneous diseases that are often caused by a drug-induced immunologic reaction. The epidermal separation of SJS involves < 10% of body surface area (BSA), whereas TEN involves > 30% of BSA. Involvement of 10–30% of BSA is often considered SJS/TEN overlap. May be preceded by erythema multiforme, a ﬂulike prodrome, skin tenderness, a maculopapular drug rash, or painful mouth lesions. Often associated with a history of exposure to new drugs, such as penicillin, sulfonamides, seizure medications (e.g., phenytoin, carbamazepine), quinolones, cephalosporins, allopurinol, corticosteroids, or NSAIDs. Exam reveals severe mucosal erosions with widespread erythematous, cutaneous macules or atypical targetoid lesions. The epidermal lesions often become conﬂuent and show a Nikolsky’s sign and epidermal detachment.

Genital ulcer

Causes of drug induced genital ulcers take the form of Stevens–Johnson syndrome, toxic epidermal necrolysis, erythema multiforme, and fixed drug eruptions in response to a diverse list of medications. Stevens–Johnson syndrome and toxic epidermal necrolysis are more likely than erythema multiforme to have genital manifestations although all present with a toxic appearing patient. A fixed drug eruption in comparison does not present as a toxic appearing patient but rather single or multiple erythematous patches that vary in size and shape which eventually turn dark brown in color and become itchy. The defining characteristic is that these drug eruptions appear in the same location each time the offending medication is used, and will resolve on their own.

List of skin conditions

Drug-induced nail changes Drug-induced pigmentation Drug-induced pseudolymphoma Drug-induced urticaria Erythema multiforme major (erythema multiforme minor–erythema multiforme von Hebra) Exudative hyponychial dermatitis Fixed drug reaction Halogenoderma Heparin necrosis HIV disease-related drug reaction Hydroxyurea dermopathy Injection site reaction Iododerma Leukotriene receptor antagonist-associated Churg–Strauss syndrome Linear IgA bullous dermatosis (linear IgA dermatosis) Photosensitive drug reaction Red man syndrome Severe cutaneous adverse reactions (includes DRESS syndrome, Steven Johnson syndrome, Toxic epidermal necrolysis, Stevens-Johnson/toxic epidermal necrolysis overlap syndrome, and Acute generalized exanthematous pustulosis) Scleroderma-like reaction to taxanes Serum sickness-like reaction Steroid acne Steroid folliculitis Stevens–Johnson syndrome Sulfonamide hypersensitivity syndrome Texier's disease Toxic epidermal necrolysis (Lyell's syndrome)

Erythema multiforme

Erythema multiforme was first described by von Hebra in 1860. Signs and symptoms The condition varies from a mild, self-limited rash (E. multiforme minor) to a severe, life-threatening form known as erythema multiforme major (or erythema multiforme majus) that also involves mucous membranes. Consensus classification: Erythema multiforme minor—typical targets or raised, edematous papules distributed acrally Erythema multiforme major—typical targets or raised, edematous papules distributed acrally with involvement of one or more mucous membranes; epidermal detachment involves less than 10% of total body surface area Stevens–Johnson syndrome and toxic epidermal necrolysis used to be considered part of the erythema multiforme spectrum, but that is no longer the case.

Stevens-Johnson Syndrome -- Introduction

Stevens-Johnson syndrome/toxic epidermal necrolysis is a rare, acute, serious, and potentially fatal skin reaction in which there are sheet-like skin and mucosal loss accompanied by systemic symptoms. Medications are causative in over 80% of cases. Stevens-Johnson syndrome/toxic epidermal necrolysis is classified by the extent of the detached skin surface area. Stevens-Johnson syndrome: less than 10% body surface area Overlap Stevens-Johnson syndrome/toxic epidermal necrolysis: 10% to 30% body surface area Toxic epidermal necrolysis more than 30% body surface area

Erythema multiforme

Stevens–Johnson syndrome and toxic epidermal necrolysis used to be considered part of the erythema multiforme spectrum, but that is no longer the case. The mild form usually presents with mildly itchy (but itching can be very severe), pink-red blotches, symmetrically arranged and starting on the extremities. It often takes on the classical "target lesion" appearance, with a pink-red ring around a pale center. Resolution within 7–10 days is the norm. Individuals with persistent (chronic) erythema multiforme will often have a lesion form at an injury site, e.g. a minor scratch or abrasion, within a week. Irritation or even pressure from clothing will cause the erythema sore to continue to expand along its margins for weeks or months, long after the original sore at the center heals. Causes

Bullous drug reaction

Bullous drug reaction most commonly refers to a drug reaction in the erythema multiforme group. These are uncommon reactions to medications, with an incidence of 0.4 to 1.2 per million person-years for toxic epidermal necrolysis and 1.2 to 6.0 per million person-years for Stevens–Johnson syndrome. The primary skin lesions are large erythemas (faintly discernible even after confluence), most often irregularly distributed and of a characteristic purplish-livid color, at times with flaccid blisters. See also Skin lesion List of cutaneous conditions References External links Drug eruptions

InternalMed_Harrison

Drug-induced erythroderma (exfoliative dermatitis) may begin as an exanthematous (morbilliform) eruption (Chap. 74) or may arise as diffuse erythema. A number of drugs can produce an erythroderma, including penicillins, sulfonamides, carbamazepine, phenytoin, and allopurinol. Fever and peripheral eosinophilia often accompany the eruption, and there may also be facial swelling, hepatitis, myocarditis, thyroiditis, and allergic interstitial nephritis; this constellation is frequently referred to as drug reaction with eosinophilia and systemic symptoms (DRESS) or drug-induced hypersensitivity reaction (DIHS). In addition, these reactions, especially to aromatic anticonvulsants, can lead to a pseudolymphoma syndrome (with adenopathy and CAuSES of ERyTHRoDERMA 1. Primary cutaneous disorders a. b. c. 2. 3. a. Cutaneous T cell lymphoma (Sézary syndrome, erythrodermic mycosis fungoides) b. 4. Idiopathic (usually older men) a Discussed in detail in Chap. 71.

Stevens-Johnson syndrome due to concomitant use of lamotrigine and valproic acid.

Stevens-Johnson syndrome (SJS) is a rare but life-threatening acute mucocutaneous hypersensitivity reaction, usually related to drugs. Severe cutaneous adverse effects such as SJS and toxic epidermal necrolysis can arise during treatment with antiepileptic drugs (AEDs). A 23-year-old female patient with idiopathic epilepsy was referred to the emergency service with fever, oral and genital mucosal lesions, generalized rash, and weakness. She had been taking carbamazepine, valproic acid, and lamotrigine until 1 week prior to referral. Following consultations with a range of clinicians, the patient was diagnosed with SJS related to lamotrigine on the basis of her history and physical findings, and on consideration of current consensus definitions of this condition. The patient was followed up and treated in the emergency intensive care unit with intravenous fluids, antibacterial therapy, and methylprednisolone. After 18 days, the patient was considered to have made a sufficient recovery and was discharged. Patients who undergo therapy with AEDs, particularly new users of these agents, should be informed of and monitored for possible systemic and cutaneous adverse effects of AEDs. Polytherapy should be avoided for as long as possible as this increases the frequency of adverse effects, and treatment should be discontinued if any rash appears.

InternalMed_Harrison

Induction of SJS is most often due to drugs, especially sulfonamides, phenytoin, barbiturates, lamotrigine, aminopenicillins, nonnucleoside reverse transcriptase inhibitors (e.g., nevirapine), and carbamazepine. Widespread dusky macules and significant mucosal involvement are characteristic of SJS, and the cutaneous lesions may or may not develop epidermal detachment. If the latter occurs, by definition, it is limited to <10% of the body surface area (BSA). Greater involvement leads to the diagnosis of SJS/TEN overlap (10–30% BSA) or TEN (>30% BSA). In addition to primary blistering disorders and hypersensitivity reactions, bacterial and viral infections can lead to vesicles and bullae. The most common infectious agents are HSV (Chap. 216), varicellazoster virus (Chap. 217), and S. aureus (Chap. 172). CHAPTER 72 Skin Manifestations of Internal Disease

Toxic epidermal necrolysis

Signs and symptoms Prodrome TEN ultimately results in extensive skin involvement with redness, necrosis, and detachment of the top (epidermal) layer of the skin and mucosa. Before these severe findings develop, people often have a flu-like prodrome, with a cough, runny nose, fever, decreased appetite and malaise. A history of drug exposure exists on average 14 days (ranging from 1–4 weeks) prior to the onset of symptoms, but may result as early as 48 hours if it is a reexposure. Skin findings Initial skin findings include red-purple, dusky, flat spots known as macules that start on the trunk and spread out from there. These skin lesions then transform into large blisters. The affected skin can then become necrotic or sag from the body and peel off in great swaths.

Adverse Drug Reactions -- Issues of Concern -- Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis

Lesions usually start on the face as an erythematous rash and then spread to all other areas. Lesions typically appear as target-like lesions and flaccid bullae at first.  As the condition progresses, sheet-like detachment of the epidermis and erosions occur. The acute, progressive phase of the illness lasts for approximately 7 to 9 days from initial symptoms. Skin reepithelizes over 7 to 21 days after the acute phase ends. Drugs associated with SJS and TEN include carbamazepine, sulfamethoxazole, phenytoin, lamotrigine, and immune checkpoint inhibitors.

Dual Antiepileptics Induced Stevens-Johnson Syndrome: A Case Report.

Stevens-Johnson syndrome and Toxic Epidermal Necrolysis are acute mucocutaneous reactions hallmark of which is widespread necrosis and detachment of epidermis. SJS/TEN fall under the single disease spectrum with an incidence rate of 1.0 to 6.0 per 1000000 and 0.4 to 1.2 per 1000000 respectively. Here, we present a case of a 46 years female who developed a generalized erythematous rash over her body, 26 days after being exposed to phenytoin and sodium valproate. Given the strong association between SJS and antiepileptic drugs, and the usual presentation being within the first eight weeks of exposure to susceptible medications; we diagnosed her with SJS. Phenytoin and sodium valproate was withdrawn and she was managed with antihistamines and corticosteroids. She improved significantly within 15 days of our intervention. The mortality rates for SJS and TEN are up to 10% and 30-50% respectively. Early identification of SJS, discontinuation of triggering medicines, and prompt initiation of supportive therapy improve the prognosis. Keywords: adverse drug reactions;antiepileptic drugs;case report;stevens-johnson syndrome.

Stevens Johnson syndrome-Toxic Epidermal Necrolysis Overlap induced by sulfasalazine treatment: a case report.

Stevens Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) are life-threatening and severe adverse cutaneous drug reactions characterized by epidermal detachment presenting as blisters and areas of denuded skin. SJS, SJS-TEN overlap and TEN differ only by their extent of skin detachment. We report here the case of a young woman (33- year old) admitted to the dermatological unit for epidermal detachment (at 18% of the body surface area), blisters, red macular and papular lesions, developed 15 days after administration of sulfasalazine. Prior to this, she complained of fever and discomfort upon swallowing. Skin biopsy had shown epidermal necrosis compatible with Stevens Johnson Syndrome and Toxic Epidermal Necrolysis. As the epidermal detachment was between 10% and 30%, she was diagnosed as a Stevens Johnson Syndrome/Toxic Epidermal Necrolysis overlap. The course was favorable 17 days after stopping the drug and starting a symptomatic treatment. Practitioners and patients need to be aware of the initial clinical signs of severe cutaneous adverse drug reactions such as fever, influenza-like symptoms, dysphagia or burning eyes. Early discontinuation of medication remains the best way to improve prognosis of patients with Stevens Johnson's Syndrome and Toxic Epidermal Necrolysis.


This is a very simple question and very useful for the management of PTE in the ED. There is no need to worry: a patient with advanced renal failure (stage 5) is contraindicated for any imaging study that requires the administration of IV contrast, such as CT angiography. All other examinations do not require it and therefore would be more than indicated in the presence of a PTE.

A 72-year-old woman with type 2 diabetes and stage 5 chronic renal insufficiency. Given the suspicion of pulmonary thromboembolism, indicate which diagnostic test is contraindicated:

[What may cause diabetes].

The case study describes a case of 49-year-old man with morbid obesity since childhood (BMI 40 kg/m2), arterial hypertension (approx. since aged 15, treated since 2004), dyslipidemia (since 2006), type 2 diabetes mellitus (since 2006, on insulin therapy since 2008) and smoking (until 2011, 20 cigarettes a day). 16 types of medication, 8 for hypertension, statin, therapy for diabetes, aspirin, allopurinol. In 2010 (when aged 45) hospitalized in our clinic with dyspnoea and chest pain with a high pressure reading of 180/110 mm Hg (identified symptoms of heart failure with LV ejection fraction of 33 %, in NYHA II functional class, echocardiographically: left atrium: 46 mm, left ventricular chamber size in diastole: 70 mm, interventricular septum: 12 mm, septal hypokinesis, Doppler ultrasonography of lower limb arteries (calcification, diffuse atherosclerotic changes, absent stenosis), CT coronary angiography (significant stenosis of the left coronary artery). Treatment started with 40 mg oral dose of furosemide daily. In May 2011 he was hospitalized with an acute coronary syndrome: acute NSTEMI of the inferior wall (coronarography: 2-vascular problems, implemented PKI, implanted DES - ramus circumflexus, paroxysmal atrial fibrillation, NYHA III functional class, left ventricular ejection fraction: 30 %, pulmonary hypertension). In 2012 renal denervation for resistant hypertension was carried out, carotid stent implanted for stenosis of the carotid artery, presence of diabetic nephropathy (KDOQI stage 3, GF 40 ml/min). In August 2014 admitted to our clinic with pulmonary oedema, cardiogenic shock, acute ischemia of the right calf with peripheral embolisation, presence of atrial flutter, impairment of renal parameters, echocardiographically: left atrium: 55 mm, left ventricle size: 75 mm, akinesis of the septum and posterior wall, occlusion of the right leg arteries (given the patients serious state angio-surgical intervention was contraindicated, vitally indicated leg amputation considered), the patient died after 4 days of hospitalization in an intensive care unit after unsuccessful treatment. A combination of diabetes, hypertension and ischemic heart disease is frequent and prognostically serious. Diabetes increases cardiovascular morbidity and mortality and therefore we should check for diabetes in all cardiovascular patients.

Demonstration of pulmonary embolism with gadolinium-enhanced spiral CT.

The authors report a case of successful detection of pulmonary embolism using gadolinium-enhanced spiral CT (Gadodiamide, 0.4 mmol/kg, 2 ml/s, delay 18 s) in a 77-year-old woman, with previous allergy to iodinated contrast medium, and renal failure, who presented with pulmonary arterial hypertension. Doppler ultrasound of the lower limbs was first performed and revealed a deep venous thrombosis of the right lower limb. To establish if venous thrombosis was the cause of pulmonary hypertension and to confirm that pulmonary endarterectomy was not indicated in this situation, several imaging modalities were performed. Lung scintigraphy and MRI were non-diagnostic. Gadolinium-enhanced spiral CT demonstrated a large thrombus located proximally and in a segmental artery of the right lower lobe. This case illustrates the potential usefulness of gadolinium as alternative contrast agent with spiral CT to diagnose pulmonary embolism and elucidate the cause of pulmonary arterial hypertension in a patient with some contraindications for iodinated contrast medium injection.

[Thromboembolic events in patients after a negative computed tomography pulmonary angiogram: A retrospective study of 165 patients].

To determine the value of computed tomography (CT) angiography of the chest as a diagnostic test to exclude pulmonary embolism and to assess compliance with diagnostic protocols for thromboembolic disease. We retrospectively studied patients who underwent CT angiography of the chest because of suspected pulmonary embolism in 2004. All the patients were followed for 3 months. The percentage of patients diagnosed with a thromboembolic event based on an objective test during the follow-up period was determined. We analyzed the percentage of patients with a negative CT angiogram on whom additional diagnostic tests (ultrasound of the lower limbs and/or ventilation-perfusion lung scintigraphy) were performed. One hundred sixty-five patients underwent CT angiography of the chest because of suspected pulmonary embolism in 2004. Four of the patients were excluded from the study because they were on chronic anticoagulation therapy and a further 2 were excluded because they had a life expectancy of under 3 months. Of the remaining 159 patients, 60 had CT angiograms that were interpreted as high probability for pulmonary embolism (prevalence of 38%). Thirty-nine of the 99 patients with a negative CT angiogram experienced an objectively confirmed thromboembolic event (63% sensitivity; 95% confidence interval, 53%-73%). Other diagnostic tests were not performed in 46% of the cases. In our setting, a negative single-detector helical CT angiogram was not sensitive enough to exclude the diagnosis of pulmonary embolism. Furthermore, compliance with internationally accepted diagnostic protocols was far from optimal.

Progestins -- Contraindications -- Combined Estrogen-Progestin Contraception

Any factors that increase the risk of cardiovascular disease or thromboembolism are contraindications to COCs due to the procoagulant effects of estrogen and are considered an “unacceptable risk,” as indicated with a Category 4 label. Category 4 denotes unacceptable health risk if the contraceptive is administered to subjects with the existing condition, basically a contraindication. Categories, from 1 to 4, are outlined in detail by the World Health Organization (WHO) and the Centers for Disease Control and Prevention (CDC). These include women ≥35 years old who smoke, 2 or more risk factors for cardiovascular disease (older age, smoking, diabetes, and hypertension), hypertension (≥160 mmHg/≥100 mmHg), venous thromboembolism (VTE) unless on anticoagulation, thrombogenic mutations, ischemic heart disease, history of stroke, complicated valvular heart disease (pulmonary hypertension, risk of atrial fibrillation, history of subacute bacterial endocarditis), diabetes mellitus for longer than 20 years or diabetes with nephropathy, retinopathy, or neuropathy. [10] [20]

First_Aid_Step2

What is metabolic syndrome? Appropriate diagnostic test? A 50-year-old man with angina can exercise to 85% of maximum predicted heart rate. A 65-year-old woman with left bundle branch block and severe osteoarthritis has unstable angina. Target LDL in a patient with diabetes. Signs of active ischemia during stress testing. ECG findings suggesting MI. Coronary territories in MI. A young patient has angina at rest with ST-segment elevation. Cardiac enzymes are normal. Common symptoms associated with silent Mls. The diagnostic test for pulmonary embolism. An agent that reverses the effects of heparin. The coagulation parameter affected by warfarin. A young patient with a family history of sudden death collapses and dies while exercising. Endocarditis prophylaxis regimens. The 6 P’s of ischemia due to peripheral vascular disease. Virchow’s triad. The most common cause of hypertension in young women. The most common cause of hypertension in young men.

Chest pain

Medical tests On the basis of the above, a number of tests may be ordered: An electrocardiogram (ECG) Chest radiograph or chest x rays are frequently performed Echocardiography can be useful in patients with known cardiac disease or aortic dissection CT scanning is used in the diagnosis of aortic dissection V/Q scintigraphy or CT pulmonary angiogram (when a pulmonary embolism is suspected) Blood tests: Troponin I or T (to indicate myocardial damage) Complete blood count Electrolytes and renal function (creatinine) Liver enzymes Creatine kinase (and CK-MB fraction in many hospitals) D-dimer (when suspicion for pulmonary embolism is present but low) serum lipase to exclude acute pancreatitis Management Management of chest pain varies with the underlying cause of the pain and the stage of care.

[Pulmonary thrombendarterectomy in a patient with chronic thromboembolic pulmonary hypertension (CTEPH) and chronic renal failure due to diabetic nephropathy--case report].

A case of 63-years old man suffering from CTEPH and chronic renal failure due to diabetic nephropathy is presented. First diagnosis of pulmonary hypertension was made at the beginning of 2000. Then the secondary anticoagulant prophylaxis were introduced. Despite such treatment there was no improvement and level of pulmonary hypertension increased. Finally, the pulmonary thrombectomy was considered because of proximal location of thromboembolic obstruction in pulmonary artery. Before surgery, the hemodialysis was started. Pulmonary thrombendarterectomy along with right coronary artery bypass grafting were performed in November 2001. The procedure was conducted successfully. The patient's condition improved and pulmonary pressure dropped substantially. During follow-up anticoagulant prophylaxis and hemodialysis are continued. It might be conducted that pulmonary thrombendarterectomy can be successfully performed also in patients with complex coexisting conditions.

Pulmonary hypertension

Exclude other diseases If the echocardiogram is compatible with a diagnosis of pulmonary hypertension, common causes of pulmonary hypertension (left heart disease and lung disease) are considered and further tests are performed accordingly. These tests generally include electrocardiography (ECG), pulmonary function tests including lung diffusion capacity for carbon monoxide and arterial blood gas measurements, X-rays of the chest and high-resolution computed tomography (CT) scanning. Ventilation/perfusion scintigraphy If heart disease and lung disease have been excluded, a ventilation/perfusion scan is performed to rule out CTEPH. If unmatched perfusion defects are found, further evaluation by CT pulmonary angiography, right heart catheterization, and selective pulmonary angiography is performed. CT scan

Case Study: 60-Year-Old Female Presenting With Shortness of Breath -- Diagnosis

Myxedema coma or severe hypothyroidism Pericardial effusion secondary to myxedema coma COPD exacerbation Acute on chronic hypoxic respiratory failure Acute respiratory alkalosis Bilateral community-acquired pneumonia Small bilateral pleural effusions Acute mild rhabdomyolysis Acute chronic, stage IV, renal failure Elevated troponin I levels, likely secondary to Renal failure Diabetes mellitus type 2, non-insulin-dependent Extreme obesity Hepatic dysfunction

Pneumonitis

Difficulty breathing Food aversion Lethargy End-stage fibrosis and respiratory failure eventually lead to death in cases without proper management of chronic pneumonitis. Diagnosis A chest X-ray or CT is necessary to differentiate between pneumonitis and pneumonia of an infectious etiology. Some degree of pulmonary fibrosis may be evident in a CT which is indicative of chronic pulmonary inflammatory processes. Diagnosis of Pneumonitis is often difficult as it depends on a high degree of clinical suspicion when evaluating a patient with a recent onset of a possible interstitial lung disease. In addition, interpreting pathologic and radiographic test results remains a challenge to clinicians. Pneumonitis is often difficult to recognize and discern from other interstitial lung diseases. Diagnostic procedures currently available include:

A Rare Case of MRSA Pericarditis with Expanding, Purulent Pericardial Effusion Leading to Uremic Kidney Failure from a Right, Necrotic Toe.

Purulent pericarditis is an extremely rare entity with only a few reported cases so far. This condition deserves prompt diagnosis because of its significant mortality rate if left untreated. A 76-year-old man with a past medical history of coronary artery disease (CAD) with percutaneous coronary intervention (PCI) to the left anterior descending artery (LAD) and right circumflex artery (RCA), ischemic cardiomyopathy with moderately reduced ejection fraction (EF 45-50%), peripheral artery disease (PAD), COVID-19 pneumonia complicated by fibrotic lung disease (on 3 liters of home oxygen), type-2 diabetes mellitus (T2DM), hypertension (HTN), hyperlipidemia (HLD), and chronic kidney disease (CKD) stage III presented with complaints of pleuritic chest pain and shortness of breath. On hospital day 1, he was afebrile and hemodynamically stable with physical exam remarkable for bibasilar crackles and dry gangrene of his right first toe. He developed progressive altered mental status, hypotension, oliguric renal failure, and respiratory distress on hospital day 6. On exam at this time, he had an elevated jugular venous distension (JVD) of 12-14 cm water, pericardial friction rub with decreased heart sounds, and orthopnea; all were consistent with cardiac tamponade clinically. An electrocardiogram (EKG) showed new ST elevations in leads I, II, and aVL with ST depression in aVR and V1 with only mild elevation in troponin I to 0.07 ng/mL. A transthoracic echocardiogram (TTE) was done on hospital day 7 and showed a moderate sized pericardial effusion with inferior vena cava (IVC) enlargement but no atrial collapse, ventricular collapse, IVC collapse, or respiratory variation in the mitral and tricuspid inflow velocities. Blood cultures grew methicillin-resistant <iStaphylococcus aureus</i (MRSA) on hospital day 6, and he was started on intravenous (IV) vancomycin. The differential diagnosis for his enlarging pericardial effusion included purulent pericarditis, uremic pericarditis, or hemorrhagic effusion. He had urgent diagnostic and therapeutic pericardiocentesis with removal of 350 milliliters of fluid. The pericardial fluid was cloudy, tan-brown with a gram stain showing gram-positive cocci in clusters and cultures growing MRSA, which confirmed the diagnosis of purulent pericarditis secondary to MRSA infection. After the pericardiocentesis, his blood pressure, respiratory distress, and renal failure improved. The source of the bacteremia was from osteomyelitis of his gangrenous, right toe with bone biopsy growing both MRSA and <iStreptococcus anginosus</i. He underwent toe amputation for definitive source control. He was discharged on hospital day 24 with a plan to complete 6 weeks of IV vancomycin.

Diagnosis Early diagnosis still remains a challenge in CTEPH, with a median time of 14 months between symptom onset and diagnosis in expert centres. A suspicion of PH is often raised by echocardiography, but an invasive right heart catheterisation is required to confirm it. Once PH is diagnosed, the presence of thromboembolic disease requires imaging. The recommended diagnostic algorithm stresses the importance of initial investigation using an echocardiogram and V/Q scan and confirmation with right heart catheter and pulmonary angiography (PA).

[Prevalence and difficulties in chronic obstructive pulmonary disease diagnosis in patients suffering from severe peripheral arterial disease].

Cigarette smoking is the most prominent risk factor for chronic obstructive pulmonary disease (COPD) and peripheral arterial disease (PAD). Prevalence of COPD among cigarette smokers is about 23% in Poland. Effort dyspnea which develops in advanced stage of COPD is the leading cause of seeking medical advice. Physical activity among patients with PAD is reduced due to intermittent claudication. It may cause delayed COPD diagnosis in this group of patients. To estimate the prevalence of COPD among patients suffering from severe PAD who were hospitalized in surgery department due to critical limb ischemia and to evaluate difficulties during COPD diagnosis in this group of patients. We examined 64 patients suffering from severe PAD (at least IIb stage according to Fontaine's scale). Patients were asked about the typical symptoms of COPD: chronic cough, regular sputum production and dyspnea. To evaluate the intensity of dyspnea we used British Medical Research Council scale. Spirometry was performed to every patient. Patients with airways obstruction underwent spirometry after the administration of inhaled bronchodilator. Every patient who had the post-bronchodilator value of FEV,/FVC ratio below the lower limit of normal values was diagnosed with COPD. It was not possible to evaluate the dyspnea intensity using MRC scale in 44% of 34 patients who reported dyspnea. During the study we diagnosed 16 patients with COPD (9 pts had 1st stage of COPD according to GOLD classification, 7 pts--2nd). 9 patients had been formerly diagnosed with COPD (2 pts--1st stage, 5 pts--2nd, and 2 pts--3rd). Spirometry-defined COPD was present in 39% of study group. The analysis of data from patients with PAD coexistent COPD revealed that 60% of them were current cigarette smokers and 28% of them declared passive smoking exposure. The other known negative prognostic factors in patients with COPD such as BMI lower than 21 kg/mr2, FEV1% of predicted value below 65% and X-rays photograph visible emphysema were observed in 40%, 32% and 12% of patients with PAD and coexistent COPD respectively. Hypertension was present in 52% of patients with PAD and coexistent COPD, cardiac arterial disease in 52% and chronic heart failure due to cardiac arterial disease in 36%. Further analysis of laboratory tests revealed dyslipidemia and chronic kidney disease in 52% and 60% of these patients respectively. COPD diagnosis among patients suffering from severe PAD involves several difficulties such as uselessness of MRC dyspnea scale and frequent heart diseases which delay the COPD diagnosis being considered as primary dyspnea reason. Many factors which have been proven to be responsible for worse prognosis due to COPD were observed in patients with PAD and coexistent COPD. High prevalence of COPD among patients suffering from PAD suggests the spirometry as a COPD screening would be justified in that group.

Pulmonary artery

Clinical significance The pulmonary artery is relevant in a number of clinical states. Pulmonary hypertension is used to describe an increase in the pressure of the pulmonary artery, and may be defined as a mean pulmonary artery pressure of greater than 25mmHg. As can be measured on a CT scan, a diameter of more than 29 mm diameter is often used as a cut-off to indicate pulmonary hypertension. In Chest X-rays, diameter of descending pulmonary artery more than 16 mm indicates pulmonary hypertension. This may occur as a result of heart problems such as heart failure, lung or airway disease such as COPD or scleroderma, or thromboembolic disease such as pulmonary embolism or emboli seen in sickle cell anaemia. Most recently, computational fluid based tools (non-invasive) have been proposed to be at par with the current clinical tests (invasive) of pulmonary hypertension.

A clinical analysis of an elderly patient with medium-high-risk pulmonary thromboembolism and chronic kidney disease is presented. Recommendations on modern principles of diagnosis, choice of treatment tactics in patients with this pathology are given. Much attention is paid to the safety of anticoagulant treatment.

Rheumatoid lung disease

Prognosis is related to the underlying disorder and the type and severity of lung disease. In severe cases, lung transplantation can be considered. This is more common in cases of bronchiolitis obliterans, pulmonary fibrosis, or pulmonary hypertension. Most complications are not fatal, but does reduce life expectancy to an estimated 5 to 10 years. Epidemiology The prevalence of RA is around 0.3–1.2% (0.92% of Americans). Women are 2–3 times more susceptible than men. The prevalence of rheumatoid lung disease in patients with RA depends on the method used for diagnosis: chest X rays (5%), high resolution CT scans (10–40%).

Diagnostic yield and renal complications after computed tomography pulmonary angiograms performed in a community-based academic hospital.

Venous thromboembolism and pulmonary embolism (VTE/PE) remain a diagnostic challenge. The computed tomography pulmonary angiogram (CTPA) has emerged as a popular diagnostic test for PE. However, there is limited data on diagnostic yield and complications in actual clinical settings. Our goal was to determine the diagnostic yield for PE and rate of renal complications following CTPA in a large community hospital setting. A retrospective chart review of 1,514 patients who underwent CTPA in the emergency department or during the initial 24 hours of admission to a community-based academic hospital. Of 1,514 CTPAs, 125 were positive for VTE/PE yielding a positive diagnosis in 8.2%. Dyspnea was the most common symptom in patients and a normal physical exam was the most common finding. Among the 925 patients with adequate data to calculate the rate of contrast-induced nephropathy (CIN), 25.8% had an increase of at least 25% in serum creatinine following the CTPA. Pre-existing diabetes and age were the most important predictors of CIN. CTPA has a low diagnostic yield for PE in a community setting, and in some patient populations, the rate of contrast-induced nephropathy may be higher than previously reported in the literature. Due to the retrospective nature of this study we were limited in using pre-test scoring systems and in measuring the impact of alternative CT diagnoses on patient management.

Tuberous sclerosis

Scan the abdomen for tumours in various organs, but most importantly angiomyolipomata in the kidneys. MRI is superior to CT or ultrasound. Take blood pressure and test renal function. In adult women, test pulmonary function and perform a high-resolution computed tomography (HRCT) of the chest. Examine the skin under a Wood's lamp (hypomelanotic macules), the fingers and toes (ungual fibroma), the face (angiofibromas), and the mouth (dental pits and gingival fibromas). In infants under three, perform an echocardiogram to spot rhabdomyomas, and electrocardiogram (ECG) for any arrhythmia. Use a fundoscope to spot retinal hamartomas or achromic patches.

ECG of the month. Electrocardiogram in a man with chronic kidney failure. ECG is abnormal and indicative of heart disease.

A 70-year-old man came to the Access to Primary Care Clinic at the Interim LSU Public Hospital because he had been told at another hospital that he needed a kidney doctor. The patient had a history of high blood pressure, type 2 diabetes mellitus, long-standing kidney disease, an above the knee amputation on the left, gout, a possible coronary stent procedure five years ago, and recently poor appetite and inability to care for himself. He had a long history of medical noncompliance and was taking no medications when he came to the hospital. He denied all cardiac symptoms, including chest discomfort. He was admitted to hospital because of a blood pressure of 240/110 mmHg, a serum creatinine of 6.0 mg/dL, and an estimated glomerular filtration rate of 11 mL/min - i.e., chronic kidney disease, stage V. His electrocardiogram was read by the computer as normal (Figure 1).

Situs ambiguus

In pulmonary valve stenosis, there is a reduction in blood flow to the lungs due to an obstruction of the heart at the pulmonic valve. This contributes to cyanosis and pulmonary hypertension. Diagnosis For proper diagnosis of situs ambiguus, cardiac and non-cardiac features must be evaluated. Diagnostic criteria for atrial isomerism includes observation of symmetry of thoracic visceral organs upon echocardiogram, arrhythmia upon electrocardiogram, and chest x-ray for confirmation of the heart's location across the left-right axis. Additional radiographic and cross-sectional imaging may be obtained to evaluate both cardiac and non-cardiac manifestations of situs ambiguus. In addition, a series of gastrointestinal tests can be conducted for observation of intestinal malrotation, as well as a scan of the liver and spleen for biliary function.

Case Study: 60-Year-Old Female Presenting With Shortness of Breath -- Differential Diagnosis

Acute on chronic COPD exacerbation Acute on chronic renal failure Bacterial pneumonia Congestive heart failure NSTEMI Pericardial effusion Hypothyroidism Influenza pneumonia Pulmonary edema Pulmonary embolism

Heart transplantation

Absolute contraindications: Irreversible kidney, lung, or liver disease Active cancer if it is likely to impact the survival of the patient Life-threatening diseases unrelated to the cause of heart failure, including acute infection or systemic disease such as systemic lupus erythematosus, sarcoidosis, or amyloidosis Vascular disease of the neck and leg arteries. High pulmonary vascular resistance – over 5 or 6 Wood units. Relative contraindications: Insulin-dependent diabetes with severe organ dysfunction Recent thromboembolism such as stroke Severe obesity Age over 65 years (some variation between centers) – older patients are usually evaluated on an individual basis. Active substance use disorder, such as alcohol, recreational drugs or tobacco smoking (which increases the chance of lung disease) Patients who are in need of a heart transplant but do not qualify may be candidates for an artificial heart or a left ventricular assist device (LVAD).

Stuart W. Jamieson

Journal articles "Cardiac transplantation in 150 patients at Stanford University", British Medical Journal, No. 1 (1979), pp. 93–95. (With Edward B. Stinson and Norman Shumway) "Primary Pulmonary Hypertension and Pregnancy", Chest Journal, Vol. 89, No. 3 (March 1986), pp. 383–388. "Pulmonary endarterectomy: Experience and lessons learned in 1,500 cases" The Annals of Thoracic Surgery, Vol. 76, No. 5 (November 2003), pp. 1457–1464. "Pulmonary endarterectomy", US Cardiology, Vol 1, No. 1 (2004), pp. 1–3. "Technical advances of pulmonary endarterectomy for chronic thromboembolic pulmonary hypertension", Seminars in Thoracic and Cardiovascular Surgery, Vol. 18, Issue 3 (2006), pp. 243–249. (With M. Madani) “Pulmonary endarterectomy: recent changes in a single institution's experience of more than 2,700 patients”, The Annals of Thoracic Surgery, Vol. 94, Issue 1 (July 2012), References

[Indications for aortocoronary bypass operation in elderly patients: medical point of view].

Aorto-coronary (A-C) bypass operations were performed in 20 patients aged 68 to 78 years, and the indications for this operation were discussed retrospectively. The subjects consisted of 14 patients successfully operated and six patients unsuccessfully operated (death 3, graft occlusion 2, perioperative infarction 1). The results were as follows: Average age: There was no difference between the successful group (71.1 +/- 3.3 years; mean +/- SD) and the unsuccessful group (70.3 +/- 2.9 years). Coronary arteriographic findings: The average number of narrowed branches (greater than or equal to 75% stenosis) was 4.3 +/- 3.0 in the successful group (triple vessel disease in 12 cases and double vessel disease in four) and 2.7 +/- 0.5 in the unsuccessful group (triple vessel disease in four and double vessel disease in two), showing no significant difference between the two groups. Nine patients (64.3%) in the former group and one (16.7%) in the latter group had significant left main coronary artery disease (greater than or equal to 50% stenosis). Hemodynamic findings: Ejection fractions of less than 40% existed in three patients (50%) solely in the unsuccessful group; two of whom died. Left ventricular end-diastolic pressures of 30 mmHg or more existed in two cases (33.3%) only in the latter group. Other cardiac findings: Left ventricular end-diastolic dimension of 55 mm or more was found in one (7.1%) successful case and four (66.7%) unsuccessful cases. 201Tl-scintigrams showed a dilated left ventricular cavity in one case (7.1%) in the former group and two (33.3%) in the latter. Functions of other organs: Forced expiratory volume 1.0% or % vital capacity less than 70% was recognized in two cases (33.3%) only in the latter group. A level of serum creatinine more than 1.5 mg/dl was seen in only one case in the latter group. A case with advanced diabetes mellitus was present in the unsuccessful group, and this patient died of diabetic coma. A-C bypass operations are indicated for elderly patients when they have good cardiac function and have no complications of the kidneys, lungs and other organs. It is presumed that grafting the main coronary branches, thereby shortening the time of cardiopulmonary arrest prevents postoperative complications.

[Chronic thromboembolic pulmonary hypertension in local hospital].

Chronic thromboembolic pulmonary hypertension is a serious consequence of pulmonary embolism associated with considerable morbidity and mortality. It develops in about 0.1-3.8% among patients who survive pulmonary embolism despite adecvate treatment. Retrospectively evaluate incidence, clinical picture and difficulties in diagnostics and treatment of patients, in which the diagnosis was confirmed. In years 1996-2007 there were 33,108 patients hospitalized at 1. Internal Clinic of University hospital in Martin, 267 patients (134 women) had pulmonary embolism, 5 (2 women) of them were diagnosed chronic thromboembolic pulmonary hypertension. 3 of them overcame acute pulmonary embolism and one of them had deep venous thrombosis at the same time. These patients, during acute pulmonary embolism, were administrated trombolytic treatment. Two patients were hospitalized because of progressive dyspnoe, 1 patient as acute myocardial infarction without ST segment elevation, 1 patient as heart failure and 1 patient because of susceptive recommitement of pulmonary embolism with hemoptysis. ECG of right heart owerload were found in 4 patients, in all 5 patients in transthoracic echocardiographic examination a pulmonary hypertension were present, in 3 patients there were CT angiography negative and all 5 patients were positive in perfuse scintigraphy of lungs. This time, 2 of them are after successful operation, pulmonary endarterectomy and 1 patient (woman) died as right heart failure. Rest of the patients (2) are followed up by cardiologist in stadium NYHA II. Time till making definitive diagnosis, in spite of progressive discomfort and approved pulmonary embolism, was relatively long (1-5 years), ECG changes was most of the time diagnosed as ischaemic ones. Negative CT angiography of lungs was another step to long term diagnostics. Exactly right and early diagnosis is conclusive, because pulmonary endarterectomy, if its well indicated, is a treatment method, which makes better quality of life and survivance, as we can see in our 2 patients. On the other hand not indicated operation caused the death in our 1 patient.

Pulmonology

Procedures Clinical procedures Pulmonary clinical procedures include the following pulmonary tests and procedures: Medical laboratory investigation of blood (blood tests). Sometimes arterial blood gas tests are also required. Spirometry the determination of maximum airflow at a given lung volume as measured by breathing into a dedicated machine; this is the key test to diagnose airflow obstruction. Pulmonary function testing including spirometry, as above, plus response to bronchodilators, lung volumes, and diffusion capacity, the latter being a measure of lung oxygen absorptive area Bronchoscopy with bronchoalveolar lavage (BAL), endobronchial and transbronchial biopsy and epithelial brushing Chest X-rays CT scan Scintigraphy and other methods of nuclear medicine Positron emission tomography (especially in lung cancer) Polysomnography (sleep studies) commonly used for the diagnosis of sleep apnea

[Renal artery stenosis and chronic renal failure in NIDDM].

The NIDDM patient, willingly with high blood pressure and atheroma, has frequently an abnormal renal function. Must a renal artery stenosis (RAS) be searched as a determining or favorising cause? We have searched RAS by color duplex scan, in 60 consecutive NIDDM patients with altered renal function (creatinine clearance < or = 60 mL/min). Metabolic blood pressure (ABPM), cardiovascular and renal investigations have been realised. The population was composed of 22F/38M with middle age: 70.7 +/- 6.2 yrs, diabetic duration: 11.6 +/- 8 yrs, the plasma creatinine was: 161 +/- 78 mumol/L and clearance: 40 +/- 13 mL/min. Thirty eight had albuminuria, 28 had plasma creatinine > or = 150 mumol/L. All patients had high blood pressure. Significative RAS (> or = 70%) was detected in 15 patients (25%) by color duplex scan and proved with arteriography (n = 10) or angio NMR (n = 5). Twelve (80%) had unilateral stenosis (4 thrombosis), 3 (20%) bilateral stenosis. Renal US lead the diagnosis in 10 patients (66%): unilateral or bilateral hypotrophy. Those 15 patients had these following characteristics: 4F/11M (sex R : 0.36), middle age: 70.8 +/- 7.2 yrs, diabetic duration: 14.3 +/- 7.5 yrs, HbA1c was at 8.4 +/- 2%, 8 (53%) patients require insuline and 5 have retinopathy, plasma creatinine was at 169 +/- 6 mumol/L; 32% of patients with plasma creatinine > or = 150 mumol/L had RAS (n = 9/60%), creatinine clearance was at 38 +/- 12 mL/min (7/47% < or = 30 mL/min), 9 (60%) had macroalbuminuria and 5 (33%) microalbuminuria. All hypertensive patients were treated (mean SBP: 148 +/- 16, mean DBP: 82 +/- 7 mmHg) and had 62 +/- 28% SBP escape and 33 +/- 19% DBP escape. Ten had severe hypertension (at least 3 hypotensive drugs), 12 received CEI; 8 (53%) were smokers; 14 (93%) had one or more macroangiopathies (10/66% coronary heart diseases, 7/46% lower limbs arteritis, 6/40% carotid atheroma); 13 of these macroangiopathies are severe. In conclusion, renal failure (especially evolutive and/or treated with CEI) in NIDDM must call up a RAS (25%) specially in elderly males with a long diabetes duration, severe hypertension and macroangiopathies. This patient profile must lead to a color duplex scan to confirm the diagnosis already suspected by the renal echography.

Pulmonary rehabilitation

Guidelines Clinical practice guidelines have been issued by various regulatory authorities. American College of Chest Physicians (ACCP) and the American Association of Cardiovascular and Pulmonary Rehabilitation has provided evidence-based guidelines in 1997 and has updated it. British Thoracic Society Standards of Care (BTS) Subcommittee on Pulmonary Rehabilitation has published its guidelines in 2001. Canadian Thoracic Society (CTS) 2010 Guideline: Optimizing pulmonary rehabilitation in chronic obstructive pulmonary disease. National Institute for Health and Care Excellence (NICE) Guidelines Contraindications The exclusion criteria for pulmonary rehabilitation consists of the following: Unstable cardiovascular disease Orthopaedic contraindications Neurological contraindication Unstable pulmonary disease

A 44-Year-Old Man With Dyspnea and a Pulmonary Artery Filling Defect.

A 44-year-old man with a history of coronary artery disease, type 2 diabetes mellitus, and OSA reported progressively worsening dyspnea on exertion over a 6-week period. Outpatient CT angiogram revealed a pulmonary artery filling defect. He was sent to the ED where he was started on a heparin drip for unprovoked pulmonary embolism (PE). Echocardiogram revealed normal cardiac function without evidence of right heart strain. Lower extremity ultrasound was negative for DVT. He improved symptomatically, and no risk factors for PE were identified. He was discharged on apixaban. Five weeks later, the patient returned to the ED with hemoptysis. He reported compliance with anticoagulation and improvement of his dyspnea on exertion. History remained negative for recent travel, trauma, surgery, clotting disorders, thromboembolic disease, and alcohol or drug use. He had a 60 pack-year cigarette smoking history and quit 3 months prior.

Tetralogy of Fallot

Diagnosis There are three different useful diagnostic tests used for the diagnosis of Tetralogy of Fallot. These include a chest radiograph, electrocardiogram, and echocardiogram. The echocardiography determines the final diagnosis and typically offers enough information for surgical treatment planning. About half of all patients are now diagnosed before they are born. Differential diagnosis is when physicians diagnose between two or more conditions for a person's symptoms and this can include primary pulmonary causes of cyanosis, cyanotic heart lesions, pulmonary stenosis and transposed arterial trunks.

[Early results of aortic valve replacement in patients 65 year and older].

30 patients aged 65 years or more, who underwent aortic valve replacement in the Department of Cardiac Surgery, Medical University of Łódź in 2000, were assessed. This group consisted of 19 women (63.3%) and 11 men (36.7%). The mean age of the patients was 71.83 years +/- 3.76, age range from 66 to 78 years. The analysis of preoperative clinical state of the patients revealed prevalence of diabetes mellitus in 23 pts (76.7%), chronic bronchitis in 4 pts (13.3%), arterial hypertension in 8 pts (26.7%), pulmonary hypertension in 8 pts (26.7%), atrial fibrillation in 4 pts (13.3%), ventricular arrhythmia in 13 pts (43.3%) and cigarette smoking in 8 pts (26.7%). The mean left ventricular ejection fraction was 50.8% (+/- 8.12%, from 31% to 64%). All patients before aortic valve replacement procedure underwent coronary angiography. All patients were operated on cardiopulmonary by-pass and myocardial protection was obtained by the use of cold crystalloid cardioplegy solution based on St. Thomas Hospital formula. 7 patients (23.3%) underwent myocardial revascularization procedure simultaneously. In early postoperative period the following complications were observed: death--2 (6.67%), low cardiac output syndrome--4 (13.33%), need of use of intraaortic contrapulsation--3 (10%), need of use of inotropic agents--13 (43.33%), respiratory failure with the need of prolonged intubation--8 (26.67%), acute renal failure--2 (6.67%), stroke--1 (3.33%), need of temporary cardiac pacing--4 (13.33%), need of rethoracotomy--2 (6.67%). The results of aortic valve replacement procedures in patients over 65 years age good, in spite of prevalence of number risk factors in these patients. The high rate (23.3%) of patients requiring simultaneous myocardial revascularization is remarkable. The age of a patient should not be treated as a contradiction to the cardiac surgery.

2009 swine flu pandemic in Malaysia

On 18 August, three more death cases were confirmed and brought the number of death cases in Malaysia to 67. A 33-year-old woman was transferred to ICU after her lungs disease began to deteriorate after 4 days of treatment in a private hospital. She showed symptoms on 31 July and antiviral drugs were started on 7 August. On 12 August, she died due to bronchopneumonia. Her result later came back and was positive for the flu. A 10-year-old school girl, who was diagnosed with Systemic lupus erythematosus was sent to ICU after she had breathing difficulty, chest pain and stomach ache. Antiviral drugs were given since 8 July. Despite on medicine, she died on 13 August due to Systemic lupus erythematous with severe pneumonia with renal impairment. A 71-year-old woman with Chronic obstructive pulmonary disease, hypertension, Ischaemic heart disease and Diabetes mellitus type 2, showed symptoms of the flu and was hospitalised on 12 August. However, on 14 August, she died due to Ischaemic