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zhengyun21

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PMC-Patients

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6006004-1

comm/PMC006xxxxxx/PMC6006004.xml

Multiple hepatic sclerosing hemangiomas: a case report and review of the literature

A previously healthy 48-year-old male was found to have multiple hepatic tumors and a giant tumor (67 × 53 mm) superior to the inferior vena cave by abdominal ultrasonography during a routine medical examination. He consulted a general physician for further evaluations and had been referred to us because plain CT confirmed multiple tumors, including a giant tumor, in the liver. A laboratory workup on admission showed that total bilirubin (0.6 mg/dL) and albumin (4.2 g/dL) concentrations were within their normal ranges, whereas aspartate aminotransferase (37 IU/L), alanine aminotransferase (70 IU/L), alkaline phosphatase (176 U/L), and gamma-glutamyl transpeptidase (170 IU/L) concentrations were mildly increased. Tumor markers, including alpha-fetoprotein (2.9 ng/ml), protein induced by vitamin K absence or antagonist-II (11 mAU/ml), and carcinoembryonic antigens 19-9 (4.0 IU/L), were within normal limits.\nAbdominal ultrasonography (US) revealed well-defined, hypo echoic masses in segment 1 (S1) (67 × 53 mm in diameter), S6 (13 mm), and S2 (9 mm) in the liver (Fig. –). Abdominal dynamic CT revealed a low-density 65-mm diameter mass with an irregular margin in plain, peripheral early ring enhancement in the arterial phase, and internal heterogeneous enhancement in the delayed phase (Fig. –). Gadolinium-ethoxybenzyl-diethylenetriamine pentaacetic acid-enhanced magnetic response imaging (EOB-MRI) demonstrated a low-signal intensity mass on T1-weighted images. This mass contained several high-signal intensity areas on T2-weighted images. EOB-MRI also revealed a hypointense mass in the hepatobiliary phase (Fig. –). Furthermore, it showed higher intensity compared with the normal liver parenchyma on DWI with a high b value of 1000 (Fig. ). Its ADC value was 2.11 × 10−3 mm2/s (peripheral area) and 2.45 × 10−3 mm2/s (central area), and the ADC mean was 2.33 × 10−3 mm2/s. It was described as a high-intensity mass on the ADC map (Fig. ). There was no accumulation of [18F]-FDG on FDG-PET (Fig. ). Hepatic arteriography revealed hypervascular masses in S1 (65 mm in diameter), S6 (16 mm), and S2 (9 mm) of the liver, with no pooling (Fig. , ). In addition, gastroscopy and colonoscopy showed normal findings.\nOn the basis of these imaging findings, a preoperative diagnosis of multiple scirrhous hepatocellular carcinomas characterized by abundant fibrous stroma was made, and caudate lobectomy of the liver and partial hepatectomy of S2 and S6 were performed. The resected specimen contained a 55 × 56 mm yellowish white solid mass. The cut surfaces of the tumor were elastic and soft with smooth margins (Fig. ).\nHistopathological examination showed that the tumors were composed of various sized irregularly dilated vessels with some blood thrombi, inflammatory cell infiltration, fibrous and hyalinized sclerotic or myxomatous stroma (Fig. , ). On immunohistochemistry staining, these vascular endothelial cells were positive for CD34, suggesting sclerosing hemangioma (Fig. ). The patient tolerated the procedure well and was discharged on postoperative day14. The patient gave consent for publication of details of his case.\nHemangiomas are the commonest benign hepatic tumors, being found in up to 7% of autopsies in one series []. Hemangiomas have a predilection for women in a ratio of 5:1. They are characteristically discovered incidentally during abdominal imaging in individuals aged 40 to 50 years []. Hepatic sclerosing hemangioma, first reported by Shepherd et al. in 1983 [], is a rare type of hepatic hemangioma composed of abundant acellular hyalinized tissue in which small vessels are occasionally seen. Another study reported finding them in only two of 1000 autopsies []. Hepatic sclerosing hemangiomas are caused by degenerative changes such as thrombus formation, necrosis, and scar formation in hepatic cavernous hemangiomas; however, the mechanism(s) for these degenerative changes has not yet been determined []. Makhlouf and Ishak compared the findings in sclerosed hemangioma and sclerosing hemangioma []. They asserted that recent hemorrhages and hemosiderin deposits rich in mast cells are present in sclerosing hemangiomas, whereas fibrosis, increased elastic fibers, and dystrophic or psammomatous calcifications with decreased numbers of mast cells are characteristic of sclerosed hemangiomas [].\nThese pathological changes can result in the radiological features on CT and MRI being atypical of hemangioma; thus, these lesions can be diagnosed as malignant tumors such as intrahepatic cholangiocarcinoma, metastatic liver cancer, and hepatocellular carcinoma. We searched for “hepatic sclerosing hemangioma” in PubMed and ICHUSHI, a bibliographic database established in 1903 and being updated by the Japan Medical Abstracts Society, and identified 41 patients, including our patient, the characteristics of which we here summarize.\nConcerning imaging studies, Miyamoto et al. [] summarized the imaging findings in 41 hepatic sclerosing hemangiomas. Their average diameter was 41.8 mm, ranging from 10 to 145 mm. Abdominal US showed hyperechoic masses in 10 patients and hypoechoic masses in 14. Plain CT generally showed a low-density mass, whereas dynamic CT showed ring enhancement, resembling metastatic liver cancer or intrahepatic cholangiocarcinoma, in 31 of 40 (78%) reported cases. MRI showed low-intensity signals in 28 of 30 (93%) reported cases on T1-weighted images and high-intensity signals in 25 of 30 (83%) reported cases on T2-weighted images. FDG-PET showed no accumulation of [18F]-FDG in six patients who underwent this procedure []. The radiological features revealed by dynamic CT and MRI resembled those of hepatic malignancies. Thus, there were indeterminate imaging features, some imaging findings pointing to intrahepatic cholangiocarcinoma, metastatic liver cancer, or scirrhous hepatocellular carcinoma (ring enhancement, hyperintensity on T2 with hypointensity on T1 imaging). Generally, imaging findings of ring enhancement on dynamic CT is less typical of classical hepatocellular carcinoma than of metastatic liver cancer or intrahepatic cholangiocarcinoma. In recent years, there have been a small number of reports in which DWI and DWI-based ADC were successfully used to differentiate sclerosing hemangioma from liver malignancies. The ADC values for hepatic hemangiomas are reported to be higher than those of malignant liver tumors due to restricted water diffusion from high cellular density [, ]. In our case, the ADC mean was 2.33 × 10−3 mm2/s, and the mass showed higher than the normal liver parenchyma with a high b value of 1000 on DWI. It was described as a high intensity mass on an ADC map, indicating that possibility of the sclerosing hemangioma, used as a diagnostic aid to detect. FDG-PET showed no accumulation of [18F]-FDG, it suggested the possible benign character of this sclerosing hemangioma. However, it should be noticed that negative predictive value of FDG-PET for primary hepatocellular carcinoma is less than 50% with sensitivity over 80% []. Therefore, we selected hepatic resection as a first-line strategy for the management of tumors with unknown potential.\nHistopathologically, the cut surfaces of our patient’s resected tumors corresponded with cross-sectional CT images; the dynamic enhancement pattern is related to the vascular spaces component and the central areas of low density on the arterial and delayed phase to the sclerotic component, which on examination were yellowish-white with many hyalinized areas with poor vessels and fibrous changes [, ]. Additionally, our patient’s serum tumor markers, including AFP and PIVKA-2, were within normal limits. If these multiple tumors had been hepatocellular carcinomas, concentrations of these markers may have been high. Thus, it also suggests that the multiple tumors were not hepatocellular carcinomas.\nConfirmation of our provisional diagnosis would have required obtaining a liver biopsy. Although a fine needle biopsy can differentiate a sclerosing hemangioma from hepatocellular carcinoma, this procedure can potentially lead to rupture or seeding of hepatocellular carcinoma. Fine needle biopsies should only be performed to confirm inoperable hepatocellular carcinoma because seeding of tumor in the needle tract has been reported in 1–3% of cases []. We suspected that our patient’s lesions were scirrhous hepatocellular carcinomas because of the imaging features. In addition, it was considered that the lesion protruding into the intraperitoneal cavity rupture was at risk of rupture. The likelihood of malignancy and risk of rupture resulted in our decision to perform hepatic resection on our patient.

[[48.0, 'year']]

M

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{'4428228-1': 1}

6006242-1

comm/PMC006xxxxxx/PMC6006242.xml

Pushing the limits of immune-related response: a case of “extreme pseudoprogression”

A 52 year-old Chinese man presented with gross hematuria and had a left nephrectomy done in January 2014. Pathology revealed clear cell RCC of Furhman 2 grading, with invasion of the renal vein and peri-renal fat. There were synchronous solitary right lung and right hilar lymph node metastases. Having declined high-dose interleukin-2, he was started on pazopanib in March 2014, achieving partial response. In January 2015, pazopanib was stopped and surgery was attempted for the oligometastatic disease, but the right hilar node was found to be stuck down intra-operatively. External beam radiotherapy was administered post-operatively in February 2015 to the hilar node (55 Gy in 20 fractions). In May 2015, after 4 months off anti-angiogenic therapy, there was global progression of disease with the right hilar node enlarging and new metastases appearing in multiple sites (lungs, muscle, bones). Sunitinib was started (May to September 2015), and the patient went on to receive further lines of drug treatment with everolimus (October 2015 to February 2016) and axitinib (March to October 2016). He also had palliative surgery to the right radius (curettage and fixation in October 2015) and right proximal femur (curettage and bipolar hemiarthroplasty in November 2015). In November 2015, radiotherapy was also given to the right radius and femur post-operatively, to an enlarging and symptomatic scalp metastasis at the vertex, and to 4 brain metastases by gamma knife technique. Further courses of radiotherapy were given to a large lytic sacral metastasis (February to March 2016), several skin and subcutaneous tumors (May to June 2016), and the left knee (July to August 2016). In addition, subcutaneous denosumab was given as adjunctive treatment for bone metastases from October 2015 to September 2016.\nIn October 2016, computed tomography (CT) scan showed widespread metastases with interval progression in the skeletal muscles, liver, spleen, right kidney, right adrenal, pancreas, peritoneum, lungs and right hilar nodal mass. Apart from 3 new small cutaneous metastases, the patient did not have symptoms related to any specific organ site. He required the use of a walking aid after his previous hip surgery. Performance status was 2 by Eastern Cooperative Oncology Group score (ECOG). There were multiple enlarging metastases within the single right kidney, but renal function was appropriate for a post-nephrectomy setting. For example, an upper pole lesion now measured 6.5 × 4.3 cm compared to 2.5 × 2.4 cm in the scan 5 months prior. In view of the florid radiologic progression and previous multiple lines of anti-angiogenic treatment, he was offered immunotherapy. Axitinib was stopped and a single dose of nivolumab at 100 mg was given within the same day.\nThe patient developed acute renal failure 2 weeks later with oliguria, rising creatinine and hyperkalemia. Serum creatinine rose progressively from a pre-nivolumab level of 117 μmol/L to a high of 247 μmol/L in the following 5 weeks (Fig. ). Urine sediment was not active (1 white cell and 1 red cell per high power field each, and no casts). Serum phosphate and serum creatine kinase were normal. Other blood indices showed anemia, mild hypercalcemia (2.81 mmol/L), raised alkaline phosphatase (ALP) and lactate dehydrogenase (LDH). Ultrasound (US) of the remaining kidney showed infiltrative renal tumors. There was no hydronephrosis and renal perfusion was good. Except for renal parenchymal tumor infiltration and inflammatory edema, no other plausible causes of acute renal failure were identified. Renal biopsy was not performed as this was a single kidney. There was clinical deterioration with development of dyspnea, back pain, edema and drop in performance status to ECOG 3. At 4 weeks from nivolumab, the subject of hemodialysis was broached but the patient declined. He also expressed his preference not to have aggressive resuscitation or intensive care unit management, and was referred to the home hospice service. Although the recent 3 new skin metastases had resolved, a non-contrasted CT at 5 weeks post-nivolumab documented worsening tumor in multiple sites: skeletal muscles, liver, spleen, pancreas, peritoneal nodules, lungs, right hilar nodal mass and other soft tissue areas. The right kidney was larger, consistent with increasing intrarenal tumor or inflammation. He was at home on expectant management for the next 4 weeks. Several visits were made by the home hospice team. Palliative medications included oral tramadol and gabapentin, and he was prepared for death.\nAt week 10, the patient unexpectedly walked into the clinic, having felt better a week prior. There was clinical improvement in his general condition and he reported an increase in urine output. Serum creatinine had improved to 131 μmol/L (Fig. ), ALP was normal, and serum calcium had normalized without any anti-resorptive agent. There was severe anemia (hemoglobin 4.4 g/dL) and the LDH was raised at 1019 units/L (range 250–580). Chest radiography showed improvement in the right hilar and lung shadows. Red cell transfusion was administered. At week 11, non-contrasted CT scan showed improvement in tumor status in most of the involved sites including a decreased size of the right kidney. Blood and bone marrow investigations for the anemia were consistent with immune-mediated hemolysis and oral prednisolone was started at week 13. The patient continued to improve and a contrast CT at week 20 showed dramatic improvement in tumor status. In some sites, including the kidney, essentially complete remission was seen. Serum creatinine returned close to baseline (Fig. ). Prednisolone was tapered off to complete a 3 month course with hemoglobin stabilizing at 11.3 g/dL. At 6 months post-nivolumab, the patient was doing well without further immunotherapy.\nSerial CT and US images of the right kidney were analyzed (Fig. ). CT imaging showed marginal increase in kidney size from baseline to the 5 week post-nivolumab scan, and subsequent decrease at the 11 week scan when the renal function had recovered. There was no pre-nivolumab US scan, but the US scans done at 2 and 5 weeks post-nivolumab showed worsening of the renal tumor load (Fig. ). Onset of diffuse renal cortical swelling was also noted in the US at 5 weeks post-nivolumab, as demonstrated by the progressive compression and obscuration of renal medulla and sinus fat. The US changes are commensurate with the progressive worsening of renal function at these time points. A lower pole metastasis shown in the US at 2 weeks post-nivolumab was significantly larger than the corresponding lesion on the baseline contrast CT, despite the differences in imaging modality. A contrast CT at 4 months as well as an US at 6 months post-nivolumab showed decreased renal size and near complete resolution of the renal metastases.

[[52.0, 'year']]

M

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{'8201531-1': 1}

6006564-1

comm/PMC006xxxxxx/PMC6006564.xml

Rare Clinical Entity: Metastatic malignant struma ovarii diagnosed during pregnancy – Lessons for management

A 30-year old woman presented with two weeks of left lower quadrant discomfort during her second month of pregnancy. Ultrasound revealed a mixed cystic and solid left adnexal mass measuring 8.6 × 6.7 × 8.3 cm. Left salpingo-oophorectomy was performed early in the second trimester. Grossly, the left ovary demonstrated an intact 7.5 cm complex cystic-solid mass, with nearly half involved by a friable, tan-yellow tumor with papillary excrescences, some of which were freely floating within the cyst cavity. Histologic sections demonstrated a struma ovarii with over 50% characterized by the classic features of a well-differentiated papillary thyroid carcinoma (Fig. ). The periphery of the tumor impinged on the ovarian capsule and vascular structures; however, no definitive evidence of invasion was seen on the histologic sections reviewed (Fig. -). Typical histology for an ovarian mature cystic teratoma (such as areas of squamous differentiation) were present in other areas of the tumor (Fig. ). The tumor expressed positive immunohistochemical staining for thyroglobulin (Tg) and thyroid transcription factor 1 (TTF1, Fig. ), but was negative for BRAF V600E. Serum Tg was 83.5 ng/mL (reference range for euthyroid status in nonpregnant adults 0–35 ng/mL) seven days after surgical resection of the ovarian mass. The elevated Tg level is reasonably expected given the proximity to surgery and Tg half-life of 65 h [] and the known increase in Tg levels during pregnancy [, ]. Thyroid function tests (TSH 1.66 mU/L) and thyroid ultrasound were normal. Levothyroxine (L-T4) was started to suppress thyroid stimulating hormone (TSH) to 0.1–0.5 mU/L although TSH ranged 0.86–1.41 mU/L during pregnancy. After delivery, Tg was 2.9 ng/mL (TSH 0.57 mU/L). With thyroglobulin levels in a reasonable range as above, the risk of additional studies during pregnancy in terms of radiation or hypothyroid exposure, unknown risk of metastatic disease, and unknown incremental benefit of intervention during pregnancy, the decision was made to delay further testing and therapy until after delivery. Two months after delivery, the patient underwent total thyroidectomy to permit evaluation for metastatic disease and monitoring for recurrence by Tg levels. The thyroid pathology was benign.\nSeveral months after delivery and cessation of lactation, the patient underwent L-T4 withdrawal and her Tg unexpectedly rose from 0.8 ng/mL (TSH 5.8 mU/L) to 113.7 ng/mL (TSH 46 mU/L). A concurrent diagnostic I-131 scan (1 mCi) with planar and SPECT/CT imaging revealed focal central neck activity consistent with thyroid remnant and the presence of iodine-avid skeletal metastatic disease involving the left ninth rib, bilateral proximal femurs, and sacrum (Fig. -). Following 320 mCi I-131 therapy based on blood dosimetry calculations, post-therapy scan revealed the additional finding of miliary pulmonary metastases (Fig. ). Due to high-quality SPECT/CT images, separate dedicated CT and bone scan were not necessary. She was subsequently treated with TSH suppression resulting in an undetectable Tg < 0.1 ng/mL (TSH 0.02 mU/L). In order to assess the interval response to I-131 treatment, follow-up evaluation after L-T4 withdrawal protocol was obtained nine months later, demonstrating a stimulated Tg of 0.2 ng/mL (TSH 36.4 mU/L) and negative diagnostic I-131 scan without focal abnormal radioiodine uptake in the neck or skeleton. As she was planning for a second pregnancy and her pulmonary disease was not visible on the prior diagnostic I-131 scan but became apparent only on the post-therapy scan, a 100 mCi I-131 therapeutic challenge was performed to eliminate any microscopic residual disease and more accurately ascertain the treatment response. The post-therapy I-131 scan demonstrated complete resolution of pulmonary metastatic disease; however, faint residual foci of activity were demonstrated in the left ninth rib, sacrum, and left proximal femur, consistent with a partial therapeutic response in the skeleton (Fig. ). Eight months later, Tg was < 0.1 ng/mL (TSH 0.02 mU/L). The patient continues to do well, being conservatively managed with active clinical and biochemical monitoring during L-T4 suppression therapy.

[[30.0, 'year']]

F

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6006666-1

comm/PMC006xxxxxx/PMC6006666.xml

Very late presentation of anomalous origin of the left coronary artery from the pulmonary artery: case report

A 79-year-old woman was referred for evaluation of abnormal murmur. She presented with a 3-months history of worsening shortness of breath and orthopnea. Physical examination revealed a soft continuous murmur at the left upper chest with basal crackles in both lungs. Chest radiography showed mild cardiomegaly with mild pulmonary congestion. The ECG showed regular sinus rhythm without evidence of ischemia or prior myocardial infarction.\nTransthoracic echocardiography demonstrated a mildly dilated LV with markedly dilated left atrium. The LV ejection fraction was 60% with no wall motion abnormality. There was an unusual, tubular-like structure inside the interventricular septum with a turbulent, predominantly diastolic flow on color Doppler (Fig. , arrows; Additional file ). Transesophageal echocardiography revealed a markedly dilated RCA arising from the right aortic sinus (Fig. , arrow; Additional file ), while the origin of the left coronary artery (LCA) could not be demonstrated. There was a tortuous, abnormal vessel located adjacent to the main pulmonary artery (MPA) emptying into the posteromedial aspect of the MPA. There was an accelerated, continuous flow across the stenotic ostium (asterisk, Fig. , Additional file ). Moreover, there was severe MR from a restricted posterior leaflet of the mitral valve (MV) associated with ventricular remodeling in combination with mitral annular dilatation (Fig. , Additional file ).\nCoronary angiography with a single RCA injection revealed a markedly dilated RCA (Fig. ) providing multiple intercoronary collaterals of various sizes communicating with the left coronary system (Fig. ). The LCA later opacified the MPA through a stenotic ostium (Fig. , asterisk; Additional file ), establishing the diagnostic hallmark of ALCAPA syndrome. The calculated ratio of pulmonary-systemic blood flow was 1.4, confirming a significant left-to-right shunt. Coronary computed tomography angiography clearly identified the ALCAPA with a retropulmonary ostium (Fig. and , asterisks). Volume-rendered image depicted the course of the anomalous coronary arteries and its inter-coronary collateral pathways along the epicardial surface and where the LCA connected to the MPA (Fig. and , open arrow; Additional file ). Stress cardiovascular magnetic resonance perfusion imaging demonstrated no myocardial ischemia, suggesting adequate collateral circulation to the LV.

[[79.0, 'year']]

F

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{'7789775-1': 1, '3554424-1': 1}

6006706-1

comm/PMC006xxxxxx/PMC6006706.xml

Near complete response to Pembrolizumab in microsatellite-stable metastatic sebaceous carcinoma

Herein, we describe the case of a 73 year-old man in good health, who developed widely disseminated sebaceous carcinoma including metastases to brain, visceral organs, lymph nodes, and bone.\nHe initially presented in late October 2016 for removal of a rapidly growing nodule in the anterior abdominal wall. Two days later he developed confusion, urinary incontinence and progressive aphasia. Emergent magnetic resonance imaging (MRI) of the brain showed 4 enhancing gray-white matter junctional lesions, the two largest measured 3.8 × 3.3 cm in the right frontal lobe and 2.3 × 2.5 cm in the left frontal lobe. Two smaller enhancing lesions in the right parietal lobe measured 8 mm and 4 mm in diameter. In November 2016, he underwent craniotomy and resection of bilateral frontal lobe tumors, and he made a full neurologic recovery and went on to receive post-operative gamma knife radiosurgery to the resection cavities and the small parietal brain lesions (Fig. ).\nHis case was reviewed in melanoma tumor boards at the Masonic Cancer Clinic, University of Minnesota. Sections of tumor revealed sheets of epithelial cells with moderate eosinophilic cytoplasm and areas of tumor infiltrating lymphocytes (Fig. ). Cells exhibited nuclear pleomorphism and increased mitotic activity (Fig. ), desmoplastic stromal reaction and necrosis (Fig. ). Immunohistochemical staining was positive for cytokeratin AE1/AE3 and cytokeratin 7, and negative for S100, HMB45, Melan-A, CD45, calretinin, ERG, p40, TTF1, CDX2, and GATA3. The immunoprofile ruled out melanoma, mesothelioma, lymphoma, sarcoma with epithelioid features, and most visceral carcinomas. Microscopic examination revealed intracytoplasmic lipid vesicles (Fig. ), confirmed by diffuse membranous reactivity for adipophilin [, ] (Fig. and ). The findings supported a histopathologic diagnosis of sebaceous carcinoma. Importantly, additional tumor testing confirmed high expression of PD-L1 in 100% of tumor cells (Fig. ). Commercial genomic testing using next-generation sequencing (Foundation Medicine, Massachusetts, USA) confirmed the tumor was microsatellite stable and carried a mutational burden of 17 mutations/Mb. Table also shows various somatic mutations in genes for regulatory transcription factors, DNA repair proteins, growth factor receptors, and targetable MAPK signaling proteins. Several of the affected genes have also been described in cases of sebaceous carcinoma reported in the COSMIC () database [].\nInitial staging positron emission tomography-computed tomography (PET/CT) revealed evidence of widely disseminated disease involving lung and liver, muscle, bone, and multi-compartment bulky lymphadenopathy in chest and abdomen (Fig. ). Standard chemotherapy approaches using platinum-based chemotherapy were reviewed. However, the patient and family strongly favored a less toxic therapy, considering advanced age and quality of life concerns. Given the strong rationale for use of checkpoint inhibitors in several other tumor types, moderately high tumor mutational burden (17 muts/Mb), and strong PD-L1 expression the patient opted for anti-PD1 immunotherapy. He initiated off-label treatment with pembrolizumab (2 mg/kg, every 3 weeks) in December 2016.\nFollow up PET/ CT scan 3 months after the initiation of anti-PD1 therapy revealed remarkable improvement in lymph nodes, lung, and soft tissue, however, there were multiple new and enlarging hepatic and osseous metastases initially worrisome for progression(Fig. ). After multidisciplinary review, the findings were felt consistent with pseudo-progression and immunotherapy was continued. Restaging PET/ CT obtained after 6 months of treatment showed further significant improvements in all previously noted lesions (Fig. ), with residual FDG activity seen in small mediastinal and abdominal lymph nodes. To further characterize the patient’s innate and adaptive immune status at the time of his near complete response, a flow cytometry study of peripheral blood was performed. Lymphocyte subset analysis showed evidence of circulating CD45RA-CD27+ central memory (CM) and effector memory (EM) T cells, and a population of mature CD16 + CD57+ NK cells (Fig. ).\nHe continued on pembrolizumab, however, after 10 months of therapy he developed severe fatigue and orthostatic hypotension requiring hospitalization. Laboratory testing showed him to have secondary adrenal insufficiency with low (< 0.7 mcg/dL) serum cortisol and low (< 11 pg/mL) ACTH levels. He began 1 mg/kg prednisone with a prolonged steroid taper, and during high dose steroid therapy pembrolizumab dosing was interrupted. In December 2017, with 12 months of follow-up, restaging PET/CT was obtained and showed new FDG avid mediastinal and abdominal lymph nodes and a new hepatic and small bowel lesion (Fig. ). Biopsy of the liver lesion in January 2018 confirmed recurrent metastatic sebaceous carcinoma, and repeat immunohistochemical staining showed tumor cells continued to express high levels of PD-L1 (not shown). After discussion, the patient elected to restart immunotherapy, and he was continued on maintenance adrenal replacement therapy with hydrocortisone (10 mg/5 mg). Recent restaging studies demonstrate growth of the mediastinal metastases and a reduction in the size of the hepatic and small bowel metastases, suggestive of pseudoprogression and a durable ongoing response to pembrolizumab (Additional file ).

[[73.0, 'year']]

M

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6006709-1

comm/PMC006xxxxxx/PMC6006709.xml

From debulking to delivery: sequential use of rotational atherectomy and Guidezilla™ for complex saphenous vein grafts intervention

A 70 years-old man with a dilated ischemic cardiomyopathy, triple coronary artery bypass grafting (CABG) in 1990 and chronic renal failure (baseline GFR: 45 ml/min/1.73 m2) underwent a coronary angiography for a Non-ST segment elevation myocardial infarction (NSTEMI). Baseline values of Hs-Troponin T and CK-MB were 497 ng/l and 211 U/l respectively. Native coronary circulation was completely occluded at the proximal segments. Grafts angiography showed a tandem calcified lesions of SVG on distal right coronary artery (RCA) (Fig. ) and an ostial stenosis of the SVG on first obtuse marginal branch (OM1). Left internal mammary artery on the mid left anterior descending artery was patent. Ad Hoc PCI of SVG on RCA was attempted. The proximal calcified stenosis has been crossed with a 1.5 x 12 mm balloon only with the support of Guidezilla™ guide extension catheter (5-in-6 Fr), however the non compliant (NC) balloon 2.5 x 15mm was unable to break the hard and calcified plaque (Fig. ). After several attempts, the procedure was interrupted with a suboptimal result (Fig. and ). An elective PCI of SVG on RCA with rotational atherectomy was performed (left radial approach, 6 French). Two runs with 1.25 mm burr (Fig. ) and 2 runs with 1.5 mm burr (Fig. ) were carried out. Then, the use of distal anchoring balloon warranted support and tracking, made as centring rail for the advance of the tip of the “mother-and-child” catheter into the SVG. During slow deflation of the balloon, the Guidezilla™ catheter (5-in-6 Fr) was advanced distal to the proximal stenosis to be stented, thus allowing a first drug eluting stent (DES) 3.5 × 48 mm to be placed on the mid-distal segment of the graft according to a LSD technique. The same technique was applied to deploy a second DES proximally to the first one (3.5 × 28 mm on proximal segment). During both rotational atherectomy and PCI, as well as at the final angiographic control, we did not observe any sign of embolization and the procedure was successfully completed with a final TIMI III flow without complications. A slight elevations of the cardiac biomarkers the day after the procedure (Hs-Troponin T 564 ng/l and CK-MB 308 U/l) was followed by a gradual reduction of the latters in the next 4 days and the patient was discharged in stable conditions.

[[70.0, 'year']]

M

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6006723-1

comm/PMC006xxxxxx/PMC6006723.xml

Case report: Diffuse hyperplastic perilobar nephroblastomatosis complicated by a unilateral Wilms tumour: diagnosis, treatment and follow-up

A 9-month-old female presented with a 2-month history of abdominal distension and weight loss.\nThe patient underwent an uncomplicated birth following an unremarkable pregnancy at 39-weeks gestational age via elective caesarean section. The toddler attained normal developmental milestones aside from the inability to roll prone from supine. Initially at the 70th percentile at birth for weight, she presented at the 30th percentile with stable height and head circumference (50th percentile). The patient did not exhibit any clinical features of Beckwith Wiedemann Syndrome including omphalocele, macroglossia and macrosomia.\nThere is no consanguinity, no family history of recurrent malignancies, haematological or renal conditions.\nInitial limited ultrasonographic study of the abdomen demonstrated massively enlarged kidneys with loss of corticomedullary differentiation. The right kidney measured 13.1 cm and the left measured 15 cm (normal approximately 6 cm) []. Multiple ill-defined hypoechoic areas were seen randomly interspersed within the renal parenchyma bilaterally suggesting presence of nephrogenic rests and therefore nephroblastomatosis. It was not possible to rule out the presence of Wilms tumour within the nephrogenic rests on ultrasound. No evidence of hydronephrosis, hydroureter or free fluid was seen.\nComputed tomography (CT) of the abdomen and pelvis with IV and oral contrast demonstrated homogenous, diffuse, renal enlargement and loss of normal architecture with renal parenchyma replaced by homogenous low attenuating peripheral masses bilaterally (Fig. ). There were two ill-defined hypodense areas in the medial aspect of the left kidney concerning for malignancy. Residual normal renal parenchyma was present as areas of striate enhancement, hyperdense in comparison to the thick rind of peripheral nodules. Based on clinical presentation and CT findings, the patient was diagnosed with bilateral DHPLNB.\nGadolinium enhanced magnetic resonance imaging (MRI) of the abdomen performed after 6 weeks of chemotherapy demonstrated hypointense peripheral masses on T1/T2 images with thick septations suggestive of bilateral DHPLNB. A small heterogeneously enhancing lesion with multiple small linear and round cysts in the medial left kidney was characterized on T1 imaging, suspicious for a superimposed Wilms tumor (Fig. ). Diffusion weighted imaging was not conducted.\nNo metastases were seen on CT chest with contrast. Pathology after left partial nephrectomy confirmed Wilms tumor.\nCytogenetic analysis was conducted revealing a normal female karyotype (46XX). No other tumour markers or genetic testing was performed.\nBilateral DHPLNB complicated by a left renal mass (likely Wilms Tumour) Renal lymphoma (uncommon in infants and young children).\nThe patient was treated with COG protocol AREN 0534 EE-4A chemotherapy, including vincristine and dactinomycin for 20 weeks. Due to the presence of the superimposed left renal mass, a left partial nephrectomy and perihilar lymph node dissection was performed. Following the surgery, she also received left flank radiotherapy to a dose of 1080 cGy in 6 fractions.\nPathology was consistent with DHPLNB and resection of a Wilms tumor. Resection margins were positive. Nodal dissection was negative. Following surgical resection and chemotherapy, the patient was followed with MRI every 3 months for a year. Thereafter, the patient was followed with MRI every 6 months for 2 years. Currently, the patient is now on surveillance with ultrasounds every 3–4 months.

[[9.0, 'month']]

F

{'18026724': 1, '18597570': 1, '16314292': 1, '9781906': 1, '6607625': 1, '8383276': 1, '15816029': 1, '11112813': 1, '33650837': 1, '22622354': 1, '9672980': 1, '20833859': 1, '29914549': 2}

{}

6006740-1

comm/PMC006xxxxxx/PMC6006740.xml

SLC4A4 compound heterozygous mutations in exon–intron boundary regions presenting with severe proximal renal tubular acidosis and extrarenal symptoms coexisting with Turner’s syndrome: a case report

The patient was a 7-year-old Turkish girl born to non-consanguineous parents. She was being followed up since 3 years of age because of bilateral glaucoma and was prescribed medicines [50 mL of Sholl solution and anti-acidosis capsule (three times a day)] and eye drops (β-blocker and carbonic anhydrase inhibitor). Her mother had oligohydramnios; the patient had intrauterine growth retardation and was born prematurely. There was no family history of any inherited diseases, cataract or pRTA. Both her weight and height were below the 3rd percentile, and she demonstrated intellectual disability. However, the other physical examinations, including neurological signs, were unremarkable.\nThe laboratory tests were as follows: Na, 139 mmol/L; K, 3.1 mmol/L; Cl, 110 mmol/L; blood urea nitrogen, 12 mg/dL and creatinine, 0.9 mg/dL. Blood gas analysis revealed a pH of 7.22, HCO3− concentration of 11 mmol/L and PCO2 of 29 mmHg. In addition, urinalysis revealed a pH of 5, no protein and no glucose. The urinary excretion of amino acids was normal, and the urinary β2-microglobulin level was 110 μg/L (normal: < 240 μg/L). These investigations revealed that the patient had pRTA without Fanconi syndrome – generalized dysfunction of proximal tubule. Considering her short stature, the levels of thyroid hormones, IGF-I and IGFBP3 were normal. Furthermore, whereas the renal ultrasound revealed a 9-mm diameter parenchymal stone in the right kidney, brain MR imaging revealed no intracranial calcification. Neither of the parent showed these symptoms.\nAt the age of 9, our patient presented with micrognathia, fish-mouth, epicanthal folds, ptosis, low-set ears, a short neck with a low hairline, a broad shield-like chest, wide-spaced nipples, hypoplastic areolae, cubitus valgus and short fourth metacarpals, with other symptoms due to NBCe1A absence such as dental abnormalities, suggesting the coincidence of Turner’s syndrome. In addition, her weight and height were still below the 3rd percentile. Hormonal investigation data were as follows: FSH, 69.3 mIU/mL (normal: 4.5–20.0 mIU/mL); LH, 15.9 mIU/mL (3.5–14.0 mIU/mL) and oestradiol, < 5.0 pg/mL, suggesting hypergonadotropic hypogonadism. Her karyotype was 45, XO which confirmed the diagnosis of Turner’s syndrome.\nFrom 200 μL of peripheral blood samples obtained from the patient and her parents, we extracted DNA using the QIAamp DNA Blood Mini Kit (Qiagen Inc.) according to the manufacturer’s instructions. Similarly, we extracted RNA from 1 to 2 mL of the peripheral blood sample using the Isogen (Nippon Gene) or the QIAamp RNA Blood Mini Kit (Qiagen Inc.) according to the manufacturer’s instructions. Then, the complementary DNA (cDNA) of the patient was synthesised from the polyA(+) RNA of the peripheral white blood cells using the cDNA Synthesis Kit (Takara) as previously described [] or the RevertAid First Strand cDNA Synthesis Kit (Thermo Scientific) according to the manufacturer’s instructions.\nThe polymerase chain reaction (PCR) condition used was as follows: denaturation for 9 min at 95 °C, followed by 35 cycles of 95 °C for 1 min, 60 °C for 1 min and 72 °C for 1 min, with a final extension at 72 °C for 7 min. PCRs were performed using a thermal cycler PerkinElmer GeneAmp PCR System 2400 (PerkinElmer Japan, Applied Biosystems Division, Tokyo, Japan). The DNA sequence of each PCR product was determined using the Sanger sequencing method, with the primers shown in Table , in an ABI3100 sequence analyser (Life Technologies, Carlsbad, CA). In addition, AmpliTaq™ (Roche) and attached buffers were used for PCR. The primers in Table were used for the analyses of exons and splicing site sequences of SLC4A4.\nThe sequences of primers used for the detection of β-actin and fragments of SLC4A4 coding sequences were as follows: hACTB748F, 5’-ATTGGCAATGAGCGGTTC-3′, and hACTB979R, 5’-TCTTCATTGTGCTGGGTGC-3′; exon2-3bridgeF, 5’-GTTGGTGGAGATGATTGTTGAC-3′, and exon6-7bridgeR, 5’-GTCATGGAACACCTCATCAGAC-3′; exon5-6bridgeF, 5’-TGCCCACAAGGTTCTTGTTC-3′, and exon8-9bridgeR, 5’-ACCACAGAACCGTCCAGTTC-3′.\nThe quantitative RT-PCR (qRT-PCR) was performed according to its instructional manual, with TaqMan Gene Expression Master Mix (Applied Biosystems, Foster City, CA, USA), TaqMan Gene Expression Assays (Hs00186798_m1 for SLC4A4, Hs01060665_g1 for β-actin; all from Applied Biosystems) and sequence detection system (7500 Fast Real-time PCR System; Applied Biosystems). The expression level was quantified relative to the abundance of β-actin cDNA.\nThe sequencing analysis of the SLC4A4 gene (OMIM 603345, ENST00000340595.3, NM_003759.3) across each exon, including the adjacent intronic sequences of approximately 100 base pairs of the proband, revealed two heterozygous mutations as follows: (a) c.1076 + 3A > C, three bases after the end of exon 7 (Fig. and ) c.1772 − 2A > T, two bases before the beginning of exon 12 (Fig. ). In addition, we analysed the SLC4A4 genes of her parents and confirmed that her mother and father had heterozygous mutations c.1076 + 3A > and c.1772 − 2A > T, respectively. No other mutations in the SLC4A4 gene were detected in the genomes of the patient or her parents. Of note, both mutations are absent from the ExAC database (/).\nOwing to the locations of both mutations on the splice sites, we performed in silico assays to elucidate whether the splicing sites were altered in the proband.\nWe used the webtools ‘Splice Site Score Calculation’ () [, ], ‘NetGene2 Server’ (), ‘Human Splicing Finder Version 3.1’ () and ‘Berkeley Drosophila Genome Project Splice Site Prediction by Neural Network’ () for the in silico evaluation of these mutations. The ‘Splice Site Score Calculation’ demonstrated that the scores of the original sequences were 9.2 and 9.8, whereas the scores of the aberrant sequences were 2.5 and − 1.2, respectively (in order of c.1076 + 3A > C, c.1772 − 2A > T). Because the mean score of the 3′ splice site in constitutive exons was 7.9 and that of the 5′ splice site in constitutive exons was 8.1, the proband’s data suggested that the mutations could cause aberrant splicing (data not shown).\nIn contrast, ‘NetGene 2 Server’ suggested that there may be no splice donor site for the c.1076 + 3A > C mutation and that there may be an aberrant acceptor splice site in c.1772 − 2A > T (data not shown), whereas The ‘Human Splicing Finder Version 3.1’ suggested that in c.1772 − 2A > T the acceptor splice site is broken (data not shown). The ‘Berkeley Drosophila Genome Project Splice Site Prediction by Neural Network’ [] suggested that c.1076 + 3A > C mutation abolishes the original splice donor site and provides an alternative splice donor site (c.1076 + 197_198GT). It also suggested that c.1772 − 2A > T abolishes the original acceptor site and provides alternative acceptor sites (c.1772 − 29_ − 28AG, c.1772 − 37_ − 36AG, c.1772 − 168_ − 167AG).\nFurthermore, the estimated models of aberrant transcription according to previous literature [, ] suggested the appearance of nonsense codons in each allele of the patient’s genome (Fig. and ).\nWe tried to assess the sequence of the SLC4A4 cDNA because we obtained the cDNA of the proband from her mRNA. However, we did not detect the expression of SLC4A4 (Fig. ). Then, owing to the detection of the expression of β-actin in the proband’s cDNA (Fig. ), the absence of the SLC4A4 cDNA sequence suggested that SLC4A4 was either not expressed or expressed at extremely low levels in the proband.\nWe further tried to confirm the expression amount of the SLC4A4 compared to β-actin in the patient by the qRT-PCR. The relative expression ratio of SLC4A4 to β-actin in the proband was extremely low compared to that of the healthy human control (P < 0.01), mother (P < 0.05) and father (P < 0.1) (Fig. ).

[[7.0, 'year']]

F

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{}

6006788-1

comm/PMC006xxxxxx/PMC6006788.xml

Leishmania (Mundinia) orientalis n. sp. (Trypanosomatidae), a parasite from Thailand responsible for localised cutaneous leishmaniasis

The patient was a 57-year-old woman who lives in Chiang Klang District, Nan Province, northern Thailand. She is a gardener and has never been abroad, only travelling to Phitsanulok and Phijit, provinces near Nan in Thailand. The patient presented in May 2014 at Chiang Klang Hospital with a single skin nodule on her left cheek (1.0 × 1.5 cm), and also with crusting at the left angle of the mouth (Fig. ). No skin nodules in other sites of the body were observed. Two pieces of formalin-fixed skin biopsy from the cheek nodule (0.6 × 0.5 × 0.4 cm3 and 0.5 × 0.5 × 0.2 cm3) were sent to the Department of Pathology, Faculty of Medicine, Chiang Mai University for histopathological investigation. Histopathological analyses revealed epidermal ulceration with a heavy, chronic inflammation of the dermis (Fig. ) and numerous intracellular small round or oval-shaped bodies, with the appearance of amastigotes (1–2 μm in width and 2–4 μm in length) of Leishmania spp. (Fig. ). A week later, a fresh skin biopsy from the nodule (0.4 × 0.5 × 0.3 cm3) was collected and sent to Department of Parasitology, Faculty of Medicine, Chiang Mai University for confirmation of diagnosis by parasite culture and species identification. The skin biopsy sample was cultured in Schneider’s insect medium supplemented with 20% foetal bovine serum (FBS) and 50 International Units penicillin/ml, 50 μg/ml streptomycin at 25°C. Motile promastigotes were first observed on day 3 of the culture. Therefore, the patient was confirmed as diagnosed with cutaneous leishmaniasis. She was treated with oral amphotericin B at 1 mg/kg/day for 1 day and fluconazole at 200 mg/day for 45 days. The skin lesion had disappeared completely by six months after treatment (Fig. ). Pre-treatment laboratory investigation showed only mild anaemia with a haemoglobin concentration of 10.9 g/dl, white blood count of 7700 cells/mm3, and platelet count of 483,000/mm3. There was no hepatosplenomegaly or palpable lymph nodes. Liver function was not investigated, renal function was within normal limits and HIV serology was negative. The patient declined a bone marrow biopsy for evaluation of visceral leishmaniasis. She did not report any other underlying disease, routine drug use, or any other symptoms, and in general was in a good state of health.

[[57.0, 'year']]

F

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{'4256172-1': 1}

6006841-1

comm/PMC006xxxxxx/PMC6006841.xml

Combination therapy of ipilimumab and nivolumab induced thyroid storm in a patient with Hashimoto’s disease and diabetes mellitus: a case report

An 85-year-old Japanese man with a history of malignant melanoma of the nasal cavity presented to our department with severe thyrotoxicosis and poor blood glucose control. He had been treated for hypothyroidism secondary to Hashimoto’s disease and type 2 diabetes mellitus with insulin self-injection therapy before undergoing treatment of malignant melanoma. A surgical operation of his nasal cavity was done at first, followed by one of four planned cycles of nivolumab therapy. Subsequently, two courses of ipilimumab were given as standard therapy after nivolumab therapy. Two weeks later after receiving a dual course of ipilimumab as a third treatment, he presented with a fatigue, nausea, and sweating, which progressed to clinical and biochemical thyrotoxicosis. On admission to our hospital, he was febrile with a temperature of 38.0 °C, tachycardic, agitated, and acutely anxious but still conscious (restlessness). His Glasgow Coma Scale score was 14/15. His blood pressure had decreased to 70/50 mmHg.\nHis physical examination revealed a diffuse goiter without exophthalmoses. His abdomen was soft and non-tender and his skin was warm and wet. He was also diaphoretic with jugular venous distension and peripheral edema, and his chest was clinically clear. His medical history included hypothyroidism due to Hashimoto’s disease, diagnosed at 62 years of age and treated with thyroid hormone replacement, as well as type 2 diabetes mellitus treated by self-injection of insulin with a good glycemic control. He had both family histories of thyroid diseases and diabetes. An electrocardiogram showed marked tachycardia with atrial fibrillation, but a chest radiograph was normal.\nThe laboratory data are shown in Table . Laboratory investigation revealed the following thyroid function results: thyroid-stimulating hormone (TSH) below sensitivity, free triiodothyronine (FT3) 31.7 pg/ml, and free thyroxine (FT4) 3.43 ng/dl. Remarkably, his thyroglobulin was elevated to 48,000 IU/ml. TSH receptor antibody was negative, and a 99mTc-labeled thyroid scan revealed a markedly decreased uptake (Tc retention index-uptake ratio 0.0%; normal range 0.4–3.0). His blood glucose was markedly elevated; in which case, we needed to increase the amount of insulin to control his blood glucose. Further immunological investigation revealed normal serum levels of anti-glutamic acid decarboxylase (GAD) antibody, anti-insulinoma antigen 2 (IA-2) antibody, and insulin autoantibody (IAA).\nAccording to the diagnostic criteria of the Japan Thyroid Association for thyroid storm [], he was diagnosed as having thyroid storm 1 (TS1), definite thyroid storm, since he had thyrotoxicosis, a central nervous system symptom (restlessness), fever (38 °C), GI symptoms (nausea, vomiting), and tachycardia (135 beats per minute) in atrial fibrillation. Based on the diagnostic criteria of Burch and Wartofsky for thyroid storm, he scored 60; a score higher than 45 is suggestive of thyroid storm []. Graves’ disease was less likely as thyroid-stimulating immunoglobulin was within the normal range, and 99mTc-scintigraphy revealed a quite low uptake. These results indicated that his diagnosis of thyroid storm was due to destructive thyroiditis.\nHis clinical course is shown in Fig. . He was treated with an intravenously administered insulin infusion and intravenously administered fluid therapy. At first, the thyroid storm was treated with orally administered potassium iodide (50 mg every 6 hours) and a short-acting beta-adrenoreceptor blocker, landiolol hydrochloride, was used at 4–10 μg/kg per minute to control his heart rate. The potassium iodide was aborted when he was diagnosed as having distractive thyroiditis. Prednisolone was given at 0.5–0.7 mg/kg per day as a treatment for irAE and thyroid storm. Although previous reports suggested the optimum dosage of prednisolone to be 1–2 mg/kg per day [, ], we used a lower dose due to the coexisting and uncontrolled diabetes mellitus. By day 5, his tachycardia had resolved, and the landiolol hydrochloride was discontinued. On day 11, his thyroid function was found to have improved, and the amount of total insulin used to control his blood glucose was decreased. On day 25, he was found to have hypothyroidism, and so we restarted the replacement of thyroid hormone. He was discharged from our hospital on day 35 on daily maintenance insulin injection and levothyroxine sodium hydrate.

[[85.0, 'year']]

M

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{'7307285-1': 1, '4737013-1': 1}

6006860-1

comm/PMC006xxxxxx/PMC6006860.xml

Acute myocardial infarction after blunt chest wall trauma with underlying coronary aneurysm: a case report

A 20-year-old female patient presented to our hospital with a history of severe and dull substernal chest pain and breathlessness after being hit by a baseball to her chest 2 h ago. Immediately after being hit, she had an episode of syncope briefly without convulsion. The symptoms lasted for half-an-hour and resolved spontaneously. Her vital signs showed blood pressure of 95/64 mmHg and pulse of 70 beats/minute. There was no sign of chest wall penetrating injury. Electrocardiogram (ECG) revealed sinus rhythm with QS complexes in leads V2 to V3, ST segment elevation and T-wave inversion in leads V2 to V5 (Fig. ). Laboratory tests showed cardiac Troponin I level of 19.03 ng/ml (normal range < 0.01 ng/ml). Chest computer tomography (CT) revealed no traumatic injury but demonstrated coronary artery calcifications (Fig. ). Her parents recalled a history of high fever lasting for several days at the age of 5-year-old. The patient was admitted to our hospital diagnosed of acute traumatic MI. Serum Troponin I was peaked to 20.3 ng/ml, creatine phosphokinase(CK) to 1237 U/L and CK-MB to 101 U/L 12 h after admission. Serum BNP level was normal at 85.9 pg/ml on admission. During hospitalization, serial ECG changes were consistent with an evolving MI. She had frequent ventricular premature beats on the Holter monitor. Trans-thoracic echocardiography revealed normal left ventricular size and mild anterior hypokinesis. Notably, multiple giant left anterior descending (LAD) aneurysms with diameters from 7.5 to 8.5 mm (Fig. ) and slow flow velocity were detected. To further assess CAA, we performed contrast enhanced coronary artery computer tomography angiography (CTA)with three-dimensional (3-D)reconstruction of coronary arteries. A ringed calcification in the proximal portion of LAD artery with multiple aneurysms, thrombi and occlusions were visualized (Fig. ). CTA also demonstrated multiple aneurysms with beads-on-string appearance in the LAD artery (Fig. ). Antinuclear antibody (ANA) was minimally positive (1:10) with unknown significance. Serum levels of C3 (0.864 g/L), C4 (0.912 g/L), CRP (< 1.0 mg/L), ASO (< 25 IU/ml), ESR (15 mm/h), Rheumatoid Factor (< 25 IU/ml) and ANCA were within normal ranges. Those results did not seem to support active vasculitis, rheumatic or immunologic diseases. Based on the typical images and her childhood fever history, we recognized that the patient’s underlying coronary structural abnormality was most likely originated from KD. Considering stable hemodynamic parameters and multiple giant aneurismal dilatations of LAD artery, we managed this young patient with a conservative strategy including dual anti-platelet treatment with aspirin and clopidogrel for at least 1 year, and titrations of captopril and metoprolol to prevent ventricular remodeling. At the 2-month follow-up, the patient remained asymptomatic. ECG showed QS complex only in V2 and V3 leads and other abnormalities were resolved (Fig. ). Echocardiography revealed left ventricular diastolic dimension of 48 mm, normal LAD artery velocity, and no ventricular dilatation or akinesis. She remained asymptomatic two year later.

[[20.0, 'year']]

F

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{'8214881-1': 1}

6006918-1

comm/PMC006xxxxxx/PMC6006918.xml

Case reports of atrial and pericardial rupture from blunt cardiac trauma

We present the case of a 33-year-old female who was an unrestrained driver in a MVC with major front-end damage where airbags were deployed. She arrived to Advocate Christ Medical Center (a high volume, academic, level 1 trauma center) unresponsive with a Glasgow Coma Scale (GCS) of 5 and was intubated for airway protection. Breath sounds were noted to be present bilaterally. She was tachycardic and hypotensive with a heart rate of 143 and blood pressure of 71/46. A focused assessment with sonography for trauma (FAST) demonstrated no evidence of intra-abdominal or pericardial fluid. A left sided hemothorax was present on chest x-ray, and a chest tube was placed with 1200 mL of bloody output initially (See Fig. ). At this point, the decision was made to take patient emergently to the operating room (OR) for exploration.\nA left anterolateral thoracotomy incision was made with the patient in a supine position. There was a significant amount of blood upon entry into the chest cavity, and there was no cardiac tamponade. Despite cross clamping the pulmonary hilum, the bleeding continued. While being resuscitated with the massive transfusion protocol, there was no end tidal CO2 noted. The pericardium was opened, heart delivered, and cardiac massage was started. A separate defect in the superior, right side of the pericardium was found as well as a 3 cm defect in the anterior right atrium (See Fig. ). Despite measures to control hemorrhage and resuscitate the patient, the patient did not survive. The blood loss was greater than 6 l.

[[33.0, 'year']]

F

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{'6006918-2': 2, '5124430-1': 1}

6006918-2

comm/PMC006xxxxxx/PMC6006918.xml

Case reports of atrial and pericardial rupture from blunt cardiac trauma

The second case is a 58-year-old male who was involved in a high-speed MVC. The patient had a prolonged extrication from his vehicle and was intubated after being found unresponsive. On arrival his GCS was 3 T. He was hypotensive with FAST negative for intra-abdominal blood, but a large left hemothorax was identified on the left upper quadrant view. A chest tube was placed with initially 900 mL of blood out followed by 200 mL per hour for 2 h. Based on the output and ongoing resuscitation requirements, the decision was made to take the patient to the OR.\nA left anterolateral thoracotomy incision was made with the patient in supine position. There was approximately 1 L of clotted blood within the chest cavity coming from an anterior pericardial laceration, about 15 cm in length. Through the defect there was brisk bleeding coming from a 1 cm laceration on the left atrial appendage (See Fig. ). The injury was debrided and repaired using a running 3–0 polypropylene suture over a Satinsky clamp. The pericardial defect was closed to prevent cardiac herniation. The patient recovered well and was eventually discharged home.

[[58.0, 'year']]

M

{'14356382': 1, '19680160': 1, '19895542': 1, '32875048': 1, '32190829': 1, '25069684': 1, '34277828': 1, '3314758': 1, '22949784': 1, '27889903': 2, '1548712': 1, '1994075': 1, '10366211': 1, '19540491': 1, '29914563': 2}

{'6006918-1': 2, '5124430-1': 1}

6006941-1

comm/PMC006xxxxxx/PMC6006941.xml

Tonsillar Plasmacytoma: clues on magnetic resonance imaging

A 42-year-old female was seen by a primary care physician, whose examination of the oral cavity revealed smooth bilaterally enlarged tonsils, with the right tonsil being slightly larger than the left. No surface abnormality was evident. She was prescribed two courses of amoxicillin but did not improve. She was then referred to our otorhinolaryngology department. Her medical history was unremarkable. We found no unexplained cervical lymphadenopathy, no significant systemic symptom, no malignancy, and no immunocompromise. All laboratory parameters were within normal limits. Magnetic resonance (MR) images of the palatine tonsils did not reveal any obvious mass lesion. Axial noncontrast T1 (600/8/2 [TR/TE/NEX]) and coronal STIR MR (5700/80/1) images of the palatine tonsils showed that the right tonsil was larger than the left. No mass or abnormal T2 prolongation (suggestive of a tumor) was evident. An axial T2-weighted image (4400/100/2) and a postgadolinium T1-weighted image with fat saturation (550/8/1) also failed to reveal any mass in the right tonsil, but on postcontrast MRI, the left tonsil showed mucosal crypts with linear enhancement, while on the right side the crypts were partly obliterated by a large mass and did not display enhancement (Fig. ).\nThe patient underwent complete surgical resection of the right tonsil because of a possible malignancy. Histological examination of the specimen revealed diffuse sheets of monomorphous plasmacytoid cells with abundant, eosinophilic finely granular cytoplasm, and eccentric nuclei. Occasional binucleated and pleomorphic cells with giant nuclei and prominent nucleoli were also observed, as were mitotic figures. No amyloid deposition was apparent. Immunohistochemically, the plasma cells were diffusely immunoreactive for the λ light chain (DAKO, Glostrup, Denmark), IgG (DAKO), and CD43 (DAKO); partially positive for epithelial membrane antigen (DAKO); but negative for the κ light chain (DAKO), IgA (DAKO), IgM (DAKO), and CD20 (DAKO). Additional immunohistochemical staining showed that the cells were positive for the plasma cell markers CD138, MUM-1, and CD56. Thus, we diagnosed a plasmacytoma (Fig. ).\nA metastatic workup was performed to search for multiple myeloma/light chain disease. Laboratory testing revealed the absence of anemia, and the serum calcium, albumin, total protein, and creatinine levels were normal. The urine lacked Bence-Jones protein. The bone survey was negative. Bone marrow biopsy revealed < 5% plasma cells, ruling out plasmacytoma of the bone. No monoclonal gammopathy was evident, precluding an immunofixation disorder. The kappa-to-lambda ratio was normal. No adjuvant treatment was indicated, and the patient remains clinically free of disease after 6 months of follow-up.

[[42.0, 'year']]

F

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{}

6007005-1

comm/PMC006xxxxxx/PMC6007005.xml

Long standing biliary colic masking chylous ascites in laparoscopic roux-en-Y gastric bypass; a case report

A 29-year-old male, with a known surgical history of LRYGB 9 years ago, presented to the emergency department complaining of sharp, severe abdominal pain in the right upper quadrant (RUQ). The pain began two months ago in an intermittent fashion that would occasionally radiate to his right shoulder and progressively became worse with time. The patient did not report any constipation, distension, or vomiting despite feeling nauseated during these attacks. The pain was not related to any movement or meals and it was moderately relieved by over the counter analgesia.\nTen years ago, the patient was diagnosed with obstructive sleep apnea (OSA), through a sleep study, due to morbid obesity. He then was advised to undergo LRYGB as a treatment. He does have any other medical problems. Family history is notable for diabetes mellitus and hypertension but no history of hyperlipidemia or obesity. His body mass index (BMI) prior to the surgery was 49.3 Kg/m2. He underwent a successful LRYGB with no complications or readmissions. After the surgery for a year and a half, he followed up with his primary surgeon. During that time, he lost 75 Kg, reaching a BMI of 26.9 Kg/m2. He then was able to maintain his weight following the operation, but failed to continue to follow up for the past 2 years. In those 2 years, he gained weight, raising his BMI to 29.9 Kg/m2, after which he modified his diet to reduce his BMI to 23.9 Kg/m2. This weight reduction was achieved prior to his presentation.\nOn physical examination, the patient was afebrile with normal vital signs. Examination of his abdomen revealed moderate tenderness over the epigastrium, central abdomen, and right hypochondrium with a positive Murphy’s sign. His laboratory investigations, including complete blood count, urine analysis, liver and renal function tests, were all within normal limits. An ultrasound examination revealed two gallstones and upper borderline diameter of common bile duct, raising the suspicion of biliary obstruction.\nCorrelating his investigation results with his symptoms, he was admitted and scheduled for a cholecystectomy. Formal laparoscopic cholecystectomy was carried out the next morning. During exploration, twisting of the common channel was observed, indicating an internal hernia, although the patient’s complaint did not suggest internal hernia in the differential diagnosis. The internal hernia was identified but reduction was not feasible through laparoscopy. Mini midline laparotomy was subsequently done for reduction of the hernia and more detailed exploration. The hernia was through the mesenteric defect between the alimentary limb and the biliopancreatic limbs. Both limbs of the anastomosis were identified and complete reduction of the internal hernia was done, in addition to suturing of the defect found in the mesentery. No other defects were found. The small intestine’s mesentery showed prominent whitish colored vessels. Whitish thick fluid was noticed, accumulating in the right paracolic gutter and in Morison’s pouch. All of the fluid was then aspirated, measuring a total of 752 mL. Analysis of the fluid showed a triglyceride level of 534 mg/dL and a Lactate Dehydrogenase (LDH) level of 512 U/L. A classic Blake drain 24 F was inserted into the pelvis. The patient passed an uneventful post-operative period. On Postoperative Day (POD) one, fluid collected from the drain was 440 ml, chylous in appearance, while on POD 2–4 the drainage was serous in appearance. The drain was removed on POD 4. The patient was discharged on POD 4 after being able to ambulate independently on his own and eat and drink with no complications.\nThe patient did not return back for his follow-up visits and when he was called by the team, he confirmed that he has no complains up to this day.

[[29.0, 'year']]

M

{'20947450': 1, '17356530': 1, '21656166': 1, '19249249': 1, '19701808': 1, '25708572': 1, '34594490': 2, '18270773': 1, '17132415': 1, '32375726': 2, '23529939': 1, '27846453': 1, '17312063': 1, '11056439': 1, '18308230': 1, '33196978': 1, '20727835': 1, '15804474': 1, '20401758': 1, '31845081': 1, '29914465': 2}

{'8478472-1': 1, '7204015-1': 1}

6007058-1

comm/PMC006xxxxxx/PMC6007058.xml

Successful treatment of oral Crohn's disease by anti-TNF-alpha dose escalation - a case report

A 34-year-old man with a 15-year history of Crohn’s Disease (CD) was admitted to our hospital due to abdominal pain, non-bloody diarrhea and weight loss. Physical examination demonstrated moderate abdominal tenderness with an abdominal mass in the right lower quadrant. Laboratory findings revealed a significantly elevated C-reactive protein (CRP 7.5 mg/dl). Colonoscopy with ulcerations localized at the Bauhin’s valve and histological examination of obtained mucosal biopsies were suggestive for active CD. As endoscopic intubation of the terminal ileum was not possible, MR enteroclysis was performed and indicative of a predominant inflammatory, short-segment stenosis of the terminal ileum. Given the acute disease flare and the stricturing phenotype, medical treatment was switched from prednisolone and azathioprine to the anti-tumor-necrosis-factor (TNF)-alpha antibody adalimumab. Twelve weeks after induction of adalimumab therapy, clinical remission was achieved and CRP level returned to normal. Another four months later, clinical remission was still maintained and laboratory inflammation markers remained low, but the patient presented in the clinic for Cranio-Maxillo Surgery due to severe pain in the mandibular area. Examination of the oral cavity detected ulcerative lesions of the buccal-side mucosa of the right mandible (Fig. ). To rule out malignancy, a biopsy of the oral lesions was obtained and revealed ulcerative stomatitis with noncaseating granulomas consistent with oral CD (Fig. ). Intensification of immunosuppressive therapy was initiated by shortening the adalimumab administration interval to weekly administration. A follow-up examination after 10 weeks confirmed complete healing of the oral CD lesion (Fig. ). During a follow-up period of 12 months, no signs of active CD became evident under continued therapy.

[[34.0, 'year']]

M

{'22420719': 1, '31889944': 2, '22914295': 1, '28452862': 1, '20808297': 1, '26614685': 1, '27896868': 1, '29914414': 2}

{'6933644-1': 1}

6007063-1

comm/PMC006xxxxxx/PMC6007063.xml

A de novo KMT2D mutation in a girl with Kabuki syndrome associated with endocrine symptoms: a case report

An 11-year, 7-month-old girl visited the Kyungpook National Children’s Hospital because of a short stature. The patient had been born via vaginal delivery at a gestational age of 38 weeks; she weighed 2.6 kg at birth, and there were no perinatal problems. However, she had an incomplete cleft palate and craniosynostosis at birth. Hearing loss in both ears was detected at the age of 1 month by brainstem-evoked response audiometry during an initial screening test. An ‘inborn errors of metabolism’ workup performed after birth revealed congenital hypothyroidism, for which the patient received levothyroxine at the local hospital until she was 3 years old; normal thyroid function was confirmed after discontinuing the medication.\nWhen she was 1 year old, she was transferred to our hospital because of a developmental delay and for a repair of her incomplete cleft palate and craniosynostosis. Brain computed tomography revealed an auditory defect, and she began wearing hearing aids, as prescribed by an otolaryngologist. The patient underwent skull reconstruction and incomplete cleft palate repair at the Departments of Plastic Surgery and Neurosurgery, respectively. She was scheduled for follow-up at the Department of Pediatric Neurology due to her developmental language delay, but she was lost to follow-up.\nWhen the patient re-visited our hospital at the age of 11 years and 7 months, she had a height of 124.8 cm (standard deviation score [SDS]: − 3.6), weight of 46 kg (SDS: 0.65 kg), and body mass index (BMI) of 29.53 kg/m2 (Z score: 1.89). She had distinctive facial features, including abnormally long openings between the eyelids, arch-shaped eyebrows, a thin upper lip, and large ears (Fig. ). Moreover, she showed postnatal growth retardation and skeletal anomalies, including an incomplete cleft palate, craniosynostosis, and brachydactyly (Fig. ). These dysmorphic features and her developmental delay were considered suggestive of Kabuki syndrome. Her karyotype was 46,XX. Her bone age was 8 years and 10 months, which was considered delayed according to the Greulich and Pyle atlas []. Based on these characteristic clinical features, mutational analyses for the KMT2D and KDM6A genes were performed.\nThe CARE guidelines were followed in reporting this case, and the molecular analyses were approved by the Institutional Review Board of the Kyungpook National University Chilgok Hospital after obtaining informed consent from the patient’s parents. Genomic DNA was extracted from peripheral blood, and library preparations were performed with the TruSight One Sequencing Panel (Illumina, Inc., San Diego, CA, USA), which enriches a 12 Mb region spanning 4813 genes of clinical relevance. Large-scale parallel sequencing was performed with the Illumina NextSeq platform. Sequence reads were aligned with the hg19 reference sequence using the Burrow-Wheeler Aligner (version 0.7.12, MEM algorithm). Duplicate reads were removed using Picard Tools (version 1.96). Local realignment and base quality recalibration were performed with the Genome Analysis Toolkit (GATK, version 3.5), and variant calling was performed using the GATK HaplotypeCaller. Variants were annotated with the Variant Effect Predictor and AnnoVar. Common variants with minor allele frequencies (≥1%) were filtered out using public databases (1000 Genomes Project, Exome Variant Server, and Exome Aggregation Consortium). Population-specific common variants were further filtered using the Korean Reference Genome Database []. The patient had the nonsense mutation of c.8200C > T, p(Arg2734*) in exon 32 in the KMT2D gene. As neither parent had this mutation, as determined by Sanger sequencing, the patient had a de novo heterozygous mutation in the KMT2D gene (Additional file : Figure S1).\nWe then performed additional examinations to detect other anomalies associated with Kabuki syndrome. Two-dimensional echocardiography, abdominal ultrasonography, and brain magnetic resonance imaging that had been performed at 1 year of age, showed no abnormalities. Ophthalmological findings were also normal.\nThe patient exhibited several endocrine problems (transiently congenital hypothyroidism, CDP, obesity and GH deficiency); additional endocrine tests were performed upon admission to our hospital (at the age of 11 years and 7 months).\nThe patient had transiently congenital hypothyroidism. When she was 7 days old, her free thyroxine (T4) was 1.09 ng/dL (reference range [RR], 0.8–2.0 ng/dL), triiodothyronine (T3) was 1.3 ng/mL (RR, 0.2–2.0 ng/mL), and thyroid-stimulating hormone (TSH) was 22.8 mIU/L (RR, 0.7–13.1 mIU/L). She received levothyroxine until she was 3 years old, and normal thyroid function was confirmed after discontinuing the medication. Upon admission to our hospital, she was 11 years and 7 month old her free T4 was 1.6 ng/dL (RR, 0.8–1.8 ng/dL), T3 was 0.8 ng/mL (RR, 0.6–1.9 ng/mL), and TSH was 3.5 mIU/L (RR, 0.3–4.0 mIU/L), indicating normal thyroid function.\nRegarding puberty, the patient showed Tanner stage I–II of breast development; her basal luteinizing hormone level was < 0.07 mIU/mL (RR, 5–20 mIU/mL), basal follicle-stimulating hormone level was 6.19 mIU/mL (RR, 0.3–10 mIU/mL), and estradiol level was < 11.8 pg/mL (RR, 0–16 pg/mL), suggesting a CDP.\nShe was obese and had a buffalo hump. Her cortisol levels while awake at 8 AM, 5 PM, and midnight were 6.93 μg/dL (RR, 3–22 μg/dL), 12.46 μg/dL (RR, 3–22 μg/dL), and 14.87 μg/dL (RR, < 7.5 μg/dL), respectively. Her corresponding adrenocorticotropic hormone levels were 34.2 pg/mL, 40 pg/mL, and 42.8 pg/mL, respectively (RR for all, 0–60 pg/mL). Her urine-free cortisol was 28.9 μg/dL (RR, 4.3–176 μg/dL). Even though the midnight cortisol level was elevated, a low-dose dexamethasone suppression test showed a cortisol level of 0.68 μg/dL (RR, < 1.8 μg/dL). A lipid profile showed a total cholesterol level of 150 mg/dL (RR, 125–200 mg/dL), triglyceride level of 66 mg/dL (RR, < 200 mg/dL), and LDL-cholesterol level of 53 mg/dL (RR, < 130 mg/dL). Her aspartate aminotransferase and alanine aminotransferase levels were 24 U/L (RR, < 97 U/L) and 17 U/L (RR, < 41 U/L), respectively.\nA GH stimulation test (spanning 2 days) was performed because of her short stature and delayed bone age. On the first day, the patient was administered levodopa (500 mg); the samples for the GH assessment were acquired at 0, 30, 60, 90, and 120 min. On the next day, the patient fasted for 8 h in the morning and was intravenously administered 0.1 U/kg of rapid-acting insulin diluted in 5 mL of normal saline for over 1 min, after which the samples for GH assessment were acquired at 0, 15, 30, 60, 90, and 120 min. The maximum GH concentrations in both tests were below 5 μg/L (RR, > 7 μg/L); therefore, the patient was diagnosed with GH deficiency. Her insulin-like growth factor 1 (IGF-1) and insulin-like growth factor-binding protein 3 (IGFBP-3) levels were 130.5 ng/mL (RR, 99–537 ng/mL, SDS: − 1.67) and 3760 ng/mL (RR, 2400–8400 ng/mL, SDS: 0.91), respectively. The patient started GH replacement therapy (Eutropin 0.03 mg/kg/day) when she was 11 years and 7 months old and is currently scheduled for regular follow-ups for growth and puberty changes.

[[7.0, 'month']]

F

{'34824202': 1, '23131014': 1, '3067577': 1, '20236313': 1, '16511588': 1, '3572628': 1, '32953414': 1, '20711175': 1, '22126750': 1, '34012382': 1, '27649541': 1, '34232366': 1, '29482518': 1, '34289049': 1, '7906121': 1, '11095962': 1, '11305802': 1, '21280141': 1, '21683083': 1, '15108197': 1, '7942006': 1, '7277096': 1, '28793284': 1, '31245521': 1, '7607617': 1, '9856564': 1, '10190924': 1, '28669924': 1, '12561059': 1, '27530205': 1, '34055685': 1, '11002345': 1, '34830739': 2, '29914387': 2}

{'8622899-1': 1, '8622899-2': 1, '8622899-3': 1, '8622899-4': 1, '8622899-5': 1, '8622899-6': 1, '8622899-7': 1}

6007364-1

comm/PMC006xxxxxx/PMC6007364.xml

Gastric duplication cyst with elevated CEA level: a case report

A 43-year-old man with a history of hypertension, hyperuricemia, android obesity and bilateral carpal tunnel, presented at his general practitioner with intermittent pain in right lumbar region.\nOn abdominal examination we palpates an epigastric mass, the rest of the physical examination was normal.\nA abdominal CT scan was realized and demonstrates the presence of a voluminous mass of 24 × 15 × 13 cm3, partially cystic, located in the epigastric region, between the left lobe of the liver, the stomach, the pancreas and the transverse colon. The differential diagnoses are: cystic lymphangioma, cystic mesothelioma, non-communicating GD and cystic GIST.\nA biology carried out at the same time an increased CEA level of 281 µg/L, and CA 19-9 being within normal limits.\nMRI gave a diagnosis of a non-communicating GD (Fig. ). The PETCT showed a moderately hypermetabolic uptake in the posterior wall of the mass, which could not differentiate a benign or malignant origin. Gastric endoscopy showed an extrinsic compression of the gastric body, with a normal mucosa.\nA decision for laparotomy surgery was made in view of the possible neoplastic degeneration and the difficulties caused by the volume of the mass. During the surgery, a GD of 30 cm of major axis, located along the entirety of the small curvature and under tension was highlighted (Fig. ). The incision of the cyst allowed 2 L of mucus to be evacueted. The lumen of the cyst did not communicate with that of the stomach. We performed a total gastrectomy with a roux-en-y loop oesophago-jejunostomy and a lymphadenectomy.\nThe histological examination described a normal stomach with a GDC without communication and adjacent to the little curvate. The size of the empty GDC was 21 cm × 10 × 9.5 cm (Fig. ). The sample of the GDC showed a mucus-secreting epithelium with some zones of ulceration. The wall was thick and had a fibrous and muscular aspect.\nNo evidence of dysplasia or metaplasia was found in the stomach and neither in the GDC.\nThe immune marking of pancytokeratine was normal and the KI67 proliferation was little elevated. The PAS coloration showed mucus but there was no signet ring cell. Overall, 18 sampled lymph nodes were free of malignancy.\nThe diagnosis was a total GDC with no malignant transformation.\nThe postoperative course was uneventful and, the patient was discharged on the 10-day postoperatively. At 6 months postoperatively the patient is currently well. The CEA is currently at 3,06 mg/L.

[[43.0, 'year']]

M

{'10710108': 1, '8210590': 1, '31423442': 2, '27221785': 1, '25861524': 2, '15047751': 1, '27289170': 1, '26995580': 1, '1131505': 1, '22456198': 1, '27150283': 2, '29942466': 2}

{'6695537-1': 1, '4858351-1': 1, '4389098-1': 1, '4389098-2': 1, '4389098-3': 1}

6007444-1

comm/PMC006xxxxxx/PMC6007444.xml

Riddle Me This: Acalculous Cholecystitis as an Unusual Complication of Immunoglobulin M Negative Mononucleosis

A 24-year-old Caucasian male presented to our outpatient clinic with fever and pain in the right upper abdominal quadrant. He had a history of a recent upper respiratory tract infection, which was treated with oral amoxicillin. The patient also had a history of left adrenal gland resection, distal pancreatectomy, and splenectomy due to a large pheochromocytoma two years earlier. His past medical history was otherwise unremarkable. A clinical examination revealed a tenderness in the epigastric abdomen and a cervical lymphadenopathy. An ultrasound examination revealed a thickened gallbladder wall as a sign of acute cholecystitis without evidence of gallstones or sludge, as shown in Figure .\nDue to increasing leukocytosis, abdominal pain, and the history of a huge pheochromocytoma, computed tomography (CT) was performed. This confirmed an acalculous cholecystitis and showed a generalized lymphadenopathy. Laboratory values were significant for: white blood cell (WBC) count 23.940/µl (range: 4.500-9.500/µl), thrombocytes 426.000/µl (150.000-400.000), lactate dehydrogenase (LDH) 438 U/l (135-225), aspartate aminotransferase (AST) 116 U/l (10-50), alanine aminotransferase (ALT) 185 U/l (10-50), gamma-glutamyltransferase (GGT) 258 U/l (10-71), alkaline phosphatase (AP) 437 U/l (40-129), c reactive protein (CRP) 10.2 mg/l (< 5mg/l). Table summarizes the laboratory values throughout the hospital stay. Figure presents the course of the patients' liver enzymes.\nThe mononucleosis enzyme-linked immunosorbent assay (ELISA) showed elevated virus capsid antigen (VCA) immunoglobulin G (IgG) levels (39 U/l; range < 0 U/l) and normal VCA immunoglobulin M (IgM) levels. The heterophile antibody test for mononucleosis IgM antibodies was positive. We performed a cytomegalovirus (CMV) ELISA, which showed normal values for IgG and IgM. Epstein-Barr virus (EBV)-specific nuclear antigen (EBNA) antibodies were negative. A VCA IgM control test two weeks later showed elevated VCA IgM antibodies. The peripheral blood smear confirmed atypical lymphocytosis. Under symptomatic therapy, the patient’s state improved rapidly and he was discharged on the 17th day after admission in good general condition.

[[24.0, 'year']]

M

{'15191950': 1, '20195147': 1, '15743745': 1, '32047519': 2, '5007364': 1, '25774295': 1, '34322642': 1, '31501735': 2, '9878041': 1, '12682112': 1, '6895981': 1, '29930883': 2}

{'6721871-1': 1, '7003250-1': 1}

6007447-1

comm/PMC006xxxxxx/PMC6007447.xml

An Unusual Presentation of Acute Abdomen Pain: Splenic and Renal Emboli from Left Ventricular Thrombus

A 47-year-old woman presented to the emergency department with concerns of left upper abdominal pain for one day. She reported that the moderate to severe abdominal pain had a gradual onset, was crampy, constant, and located in the left upper part of her abdomen radiating to the lower chest and back. The pain was associated with diaphoresis, nausea, and several instances of vomiting non-bloody, non-bilious liquid. On admission, her vitals were stable. The results of her complete blood counts, comprehensive metabolic panels, and hepatic function tests were within reference ranges. Her coagulation profile was at a subtherapeutic level. Her past medical history includes nonischemic cardiomyopathy with left ventricular thrombus and four previous cerebrovascular accidents with residual right leg numbness. Her condition was being managed with warfarin. A physical examination showed fine crackles within the left lower lobe and significant tenderness in the left upper abdomen. The patient reported that she had stopped taking warfarin two days prior to admission. Cardiac magnetic resonance imaging (MRI) showed septal and apical predominantly transmural infarct. A transthoracic echocardiogram showed a mildly dilated left ventricle with moderate to severe left ventricular dysfunction, an ejection fraction of 30% to 35% with distal anterior, anteroseptal, and apical akinesis with mild mitral regurgitation. Cardiac catheterization findings were unremarkable. A computed tomography (CT) scan of her abdomen showed mild cardiomegaly with previous infarction on the anterior-inferior wall of the left ventricle. We noted a 37.47 mm calcified thrombus in the left ventricular apex with peripheral calcifications (Figure ).\nWe also noted infarction in the spleen and right kidney with bilateral renal scarring (Figures -).\nThe patient was initially started on a heparin drip and later bridged to warfarin on the third day. She was discharged with complete resolution of her abdominal pain after seven days, and medication compliance was reinforced before discharge.

[[47.0, 'year']]

F

{'9486895': 1, '8190956': 1, '1992865': 1, '11373986': 1, '29930887': 2}

{}

6007448-1

comm/PMC006xxxxxx/PMC6007448.xml

Kikuchi-Fujimoto Disease in a Young African American Male

A 27-year-old African American male with a past medical history of asthma and allergic rhinitis presented with a three-week history fever of unknown origin. Associated symptoms included drenching night sweats, weight loss, fatigue, nausea, and diarrhea. On physical examination, he was found to be hemodynamically stable and febrile at 102°F. He was also found to have extensive left sided, posterior, cervical lymphadenopathy. Laboratory studies indicated a mild anemia with negative hepatitis panel, anti-nuclear antibody (ANA), human immunodeficiency virus (HIV1/HIV2), Lyme antibody, and infectious mononucleosis (IM) serology, non-reactive rapid plasma reagin (RPR), and a negative Babesiosis smear. Computed tomography (CT scan) of the chest and abdomen were unremarkable. Ultrasound (U/S) and CT imaging of the neck demonstrated extensive adenopathy throughout the left posterior cervical carotid chain (Figure ). U/S guided lymph node biopsy showed polymorphous appearance with areas of zonal necrosis and a subpopulation of medium-large atypical lymphoid cells suggesting an atypical lymphoproliferative disorder. Excision biopsy was performed to rule out malignancy and Hodgkin disease. Pathology showed histiocytic necrotizing lymphadenitis (Figure ). The histologic, immunohistochemical and flow cytometric findings were consistent with histiocytic necrotizing lymphadenitis. Immunohistochemical stains were performed to include BCL2, CD2, CD3, CD5, CD7, CD15, CD20, CD30, CD34, CD68, CD117, CD138, Epstein–Barr virus (EBV), herpes simplex virus (HSV), PAX5, Alk1, cytomegalovirus (CMV), Ki67, pankeratin, S100. Proliferating histiocytes marked with CD68 were negative for the listed T cell and B cell markers. Phenotypic analysis by flow cytometry revealed a mixed population of mature T and B lymphocytes with normal T cell:B cell ratio for lymph node. Monoclonal T cell or B cell populations were not detected and there was no immunophenotypic evidence of an acute leukemic process. The patient was managed with ibuprofen and fully recovered in five months’ time.

[[27.0, 'year']]

M

{'9039215': 1, '9781647': 1, '27418858': 2, '9017944': 1, '30847438': 2, '15272543': 1, '18787614': 1, '10874877': 1, '29930886': 2}

{'4935008-1': 1, '6377216-1': 1}

6007497-1

comm/PMC006xxxxxx/PMC6007497.xml

Abatacept as a Therapeutic Option for Rheumatoid Vasculitis

We present a case of a 68-year-old Caucasian female known to have multiple comorbidities: seropositive rheumatoid arthritis, common variable immunodeficiency, hypertension, hypothyroidism, and osteoporosis. The patient was diagnosed with rheumatoid arthritis in 2013. She developed cutaneous vasculitis, confirmed by biopsy performed by her primary care physician in May, 2015. She was initially treated with mycophenolate, hydroxychloroquine, and prednisone with initial improvement. The prednisone was tapered off, but she then developed worsening of joint symptoms and synovitis. For this reason, she was switched to methotrexate. Six months later, leflunomide was substituted due to hair loss and inadequate disease control on methotrexate. It was several months later that her cutaneous lesions worsened again. The decision was made to start the patient on abatacept. Arrangements were made with required laboratory testing and screening prior to starting abatacept. On the following visit, the patient was given abatacept 10 mg/kg intravenous infusion with concurrent therapy of leflunomide, hydroxychloroquine, and prednisone. The patient was reevaluated after two weeks of infusion, with clearing of lesions several days after the first dose of abatacept. On her four-month follow-up visit, the patient continued to demonstrate a satisfactory response to this therapy. Prednisone was discontinued, and the patient was kept on abatacept, hydroxychloroquine, and leflunomide. The patient returned to office a month later without any recurrence of her cutaneous lesions.

[[68.0, 'year']]

F

{'8882028': 1, '12860726': 1, '29401118': 1, '12410699': 1, '25183241': 1, '20412708': 1, '22124545': 1, '27052429': 1, '7242322': 1, '22076726': 1, '29930884': 2}

{}

6007498-1

comm/PMC006xxxxxx/PMC6007498.xml

Reduction of bilateral dislocation of TMJ and Rendu Osler Weber syndrome: case report and physiopathological model

The case of a 46-year-old male patient with a background of HHT and aortic valve replacement due to aortic insufficiency is presented. He denies having had psychiatrict treatment, consumed antipsychotic drugs, had recent molar extractions or a background of TMJ dislocation.\nOn the fifth day after a lung transplant, in the intensive therapy unit, the patient begins to experience symptoms of mandibular pain, trismus and extrapyramidalism symptoms in the form of oromandibular dystonia. In physical exam he presented signs of bilateral jaw subluxation with reuptake inhibition of the articular condyle–disc complex, contraction at the masticatory muscles level, fascies dolorosa, dystonia of the facial third with articular blocking and incapacity for bucal closure or lateral excursion and slight dysphagia. As background of the hospitalization, he had received, an hour before said event, 2.5 mg of haloperidol and 0.25 mg of risperidone (two doses) due to hyperactive delirium. It was decided to take a computed tomography (CT) of the craniofacial complex and brain without IV contrast (Fig. ), where the following was seen: dislocation of both mandibular condyles with anterior position to the temporal’s glenoid cavity, without evidence of associated bone injuries.\nAnalgesia and muscular relaxation of the patient was carried out. The Nelaton reduction maneuver and later placing of Barton bandaging was done following the technique, using an antiseptic. Posterior clinical and radiological reduction is verified through CT, evidencing a normal condylar position. The patient progresses without further subluxations during his hospitalization.

[[46.0, 'year']]

M

{'22722545': 1, '25773451': 1, '28521822': 1, '24262159': 1, '24139071': 2, '17900209': 1, '7013948': 1, '10751092': 1, '26668447': 1, '29977502': 2}

{'4016029-1': 1}

6007794-1

comm/PMC006xxxxxx/PMC6007794.xml

Management of donor site infections in split-thickness skin graft\nwith water-filtered infrared-A (wIRA)

A 61-year-old male was referred to our burn center with burn injuries. The burn\npercentage was 20% Total Burn Surface Area (TBSA) and IIb-III degree, involving\nthorax, abdomen, and right arm. Patient was treated with split-thickness skin graft.\nWe discharged the patient after successful management with healed graft and nearly\nhealed donor wounds. 8 months later, the patient was referred to us from his primary\ncare physician for management of infected open wounds in donor sites. Physical\nexamination revealed infected area with hypergranulated tissue in donor sites, left\nupper limb and left lower limb (Figure 1 ). Wound cultures showed Staphylococcus\naureus, Escherichia coli and\nStaphylococcus epidermidis (Methicillin-resistent, MRSE)\nbacteria. Patient was a heavy smoker and suffered from multiple sclerosis. Up to our\nknowledge, neglect and inadequate wound care were responsible for the opening and\nsecondary infection of donor sites. One day after admission we performed a surgical\ndebridement and removed the hypertrophic granulation tissue. After the operation we\nstarted daily wound dressing with a non-adherent antimicrobial alginate dressing\n(Silvercell®) and irradiation with water-filtered infrared-A\n(wIRA). Water-filtered infrared-A (wIRA) protocols used at our department were\nradiations all wound areas three times daily with a 60 cm distance for 30 minutes\nwith a “Hydrosun 505®” model (Hydrosun Medizintechnik,\nMüllheim, Germany). The patient tolerated this application well without any\ncomplaints. After 5 weeks the patient was discharged with healed donor sites left\nthigh and left lower leg and nearly totally healed sites left forearm and left upper\narm (Figure 2 ).

[[61.0, 'year']]

M

{'31134043': 1, '25385134': 1, '34307094': 1, '27408610': 1, '33288367': 1, '20204090': 1, '29977732': 2}

{}

6008297-1

comm/PMC006xxxxxx/PMC6008297.xml

Weaning from long-term mechanical ventilation utilizing closed-loop ventilation mode (IntelliVent®-ASV®) in a patient with spinal cord injury

An 80-year-old man with known cervical canal stenosis due to ossification of the cervical posterior longitudinal ligament was transferred to an emergency room soon after a falling accident. The patient was alert and had complete tetraplegia, sensory deficits below the C4 cervical sensory level, and progressive restrictive respiratory failure that required assisted ventilation, and, eventually, a tracheostomy. Cervical spine magnetic resonance imaging showed cervical cord compression that was worse at the C3–C4 intervertebral level, and an intramedullary high signal at C3 and C4 vertebral levels, and the patient was managed conservatively. During the conventional weaning process of trials of intermittent spontaneous breathing in the ICU, an unexpected cardiac arrest made both the patient and medical staff reluctant to proceed with further weaning. Fifty-eight days after cervical cord injury, the patient was transferred to our hospital for further rehabilitation. He was fully conscious and received pressure-support mechanical ventilation with back-up rates of 16 breaths. He had total tetraplegia below shoulder girdle muscles with a preserved sensation from C2 to C3 on both sides, and a urinary obstruction. The limbs and trunk were stiff with contracture, and muscle stretch reflexes of the limbs were abolished. Neurological level of injury was C3 according to the ISNCSCI, with a total sensory score of four. There was no sensory sacral sparing, while the deep anal pressure was preserved, and the American Spinal Injury Association scale graded B. Chest roentgenogram and computerized tomography showed bilateral, widespread atelectasis. The patient occasionally showed signs of pulmonary infection. Intensive pulmonary care and rehabilitation, including physical support for expectoration, mechanically assisted removal of tracheal secretions (CoughAssist E70®, Philips Japan, Tokyo), ventilator muscle training, and management of the spasticity of the abdominal wall, resulted in the improvement of the roentgenological findings. His expiratory tidal volume (VT) measured 150 mL.\nAlthough the neurological status according to the ISNCSCI was unchanged, the patient was willing to be weaned from mechanical ventilation as his general condition improved. After informed consent was given, we attempted automated weaning from mechanical ventilation using IntelliVent®-ASV on and after 131 days of injury. IntelliVent®-ASV (Hamilton Medical AG, Switzerland) is a closed-loop ventilation mode that adjusts the pressure support in terms of the percentage of the ideal minute volume (%MV). The ideal MV (100%MV) is calculated from the patient’s height and gender. Based on Otis’ equation [], adaptive support ventilation will select the best VT–respiratory rate (RR) coupling for the optimal work of breathing. IntelliVent®-ASV has interrelated functions: an auto-adjustment for carbon dioxide (CO2) elimination, an auto-adjustment for oxygenation, an auto-weaning tool named Quick Wean (QW), and a spontaneous breathing trial (SBT). The setting of the target %MV is automated based on either the monitored end-tidal CO2 or monitored spontaneous breathing rate. The auto adjustment of fraction of inspiratory oxygen or positive end-expiratory pressure is based on the monitored oxygen saturation fraction of hemoglobin with a pulse oximeter (SpO2) []. The QW mode is an optional automated weaning, and progressively reduces the pressure support, monitors for readiness-to-wean criteria, and provides the option to automatically conduct a fully controlled SBT. In the QW mode, when the spontaneous rate is less than the upper limit of the predicted target range, %MV is automatically decreased gradually to the selected level. To facilitate the recovery of VT by means of the loading work on respiratory muscles, we set the level of support to 70%MV for the QW mode. When spontaneous breathing satisfied the pre-determined conditions of oxygenation and ventilation for 1 min, the SBT mode was used. We operated the SBT mode during the day from 9:00 a.m. to 6:00 p.m.\nIn the QW mode, the duration of 70%MV and SBT time (25%MV) increased gradually (Figs. and ). Twenty-two days after the introduction of the QW and SBT modes in association with intensive pulmonary rehabilitation, SBT was running almost fully during the daytime. At this time, the expiratory VT was increased to 350 mL from 150 mL upon admission to our hospital. Thereafter, we withdrew the pressure support ventilation during the nighttime 39 days after the introduction of the auto-weaning mode, and the patient’s weaning process was completed.

[[80.0, 'year']]

M

{'25777333': 1, '15074456': 1, '27339104': 1, '26957117': 1, '15436363': 1, '22404065': 1, '29951276': 2}

{}

6008505-1

comm/PMC006xxxxxx/PMC6008505.xml

Eight-and-a-half syndrome: a rare potentially life-threatening disease

A fifty-six-year-old hypertensive male came in for consult due to an inability to abduct the right eye. History of the present illness started about thirty-four years prior when he first noted a limited abduction of the right eye. During the interim, he noted a gradually progressive limitation in all movements of the right eye, and a limitation in adduction of the left eye, associated with occasional diplopia. No consults were sought until a month prior when he noticed a sudden-onset right-sided facial weakness and dysarthria, with no hemiparesis or hemiplegia.\nThe patient came into the clinic with good visual acuity, good color vision, and no relative afferent pupillary defect, but with a right horizontal conjugate gaze palsy, a limitation of adduction of the right eye with associated nystagmus of the left eye on abduction, and a right peripheral facial nerve palsy demonstrated by a four-millimeter lagophthalmos, a shallow nasolabial fold, and an inability to wrinkle the forehead. The rest of the neurologic examination was unremarkable. Slit lamp examination was also essentially unremarkable except for immature cataracts. Indirect ophthalmoscopy showed evidence of hypertensive retinopathy on both eyes.\nFigure 1 shows the patient’s extraocular muscle movements in a composite diagram. There is a right horizontal gaze palsy and a limited adduction of the right eye. The sole extraocular muscle movement remaining is an abduction of the left eye.\nFigure 2 highlights the peripheral facial nerve palsy. The patient has a shallow nasolabial fold on the right, a four-millimeter lagophthalmos on the right, and weakness of the occipitofrontalis, corrugator supercilli and procerus muscles.\nOn magnetic resonance imaging, there is a hemorrhage with a complete hemosiderin ring at the level of the pons/ponto-medullary junction, associated with a well-defined lesion with a “popcorn ball” appearance resembling that of a cavernoma (Figure 3 , Figure 4 , and Figure 5 ).\nThe patient was co-managed with neurosurgery and neurology services for possible resection.

[[56.0, 'year']]

M

{'25629087': 1, '33133620': 1, '16529962': 1, '22011629': 1, '22835716': 1, '33907534': 1, '25962103': 1, '22393168': 1, '9621267': 1, '25266840': 1, '18482369': 1, '16543522': 1, '24674953': 1, '29977730': 2}

{}

6008506-1

comm/PMC006xxxxxx/PMC6008506.xml

Treatment of choroidal neovascularization in a case of factor VIII deficiency: Ten-year follow-up

A 21-year-old female presented with complaints of seeing a black spot in front of the right eye associated with distortion of the central vision of one week duration. There was no history of trauma. Best-corrected visual acuity (BCVA) in both eyes was 20/20. Anterior segment examination was essentially normal in both eyes. Fundus examination of the right eye revealed a yellowish-gray subretinal lesion with subretinal fluid supero-temporal to the fovea and pigment epithelial detachment (Figure 1 ). The lesion spared the fovea. Optic disc and retinal vasculature appeared normal and no vitreous cells were noted. Fundus examination of the left eye was normal.\nThe patient was a diagnosed case of factor VIII deficiency about a month back. Her coagulation profile and hemogram were normal. However, factor VIII – C level was 37.5% (Normal range 50–150%). Fundus fluorescein angiography (FFA) revealed a classic extrafoveal choroidal neovascular membrane (CNVM) (Figure 1 ). Focal laser photocoagulation to the CNVM was done after discussing the treatment options with the patient. The patient reported after 6 weeks with visual acuity in the right eye 20/30, N6. Clinical examination revealed a regressing extrafoveal neovascular component and an active juxtafoveal neovascular component. Clinical findings were confirmed on FFA and optical coherence tomography (OCT) (Figure 2 ). The patient was treated with photodynamic therapy (PDT) followed by intravitreal triamcinolone acetonide (IVTA) injection (4 mg/0.1 ml) 2 days later. After 6 weeks, visual acuity in the right eye was 20/30 and the intraocular pressure (IOP) was 32 mmHg. Topical Timolol maleate 0.5% twice a day was started. Since there was persistent hyperfluorescence related to the extrafoveal CNVM, thermal laser photocoagulation was done.\nWith treatment, IOP came down to 12 mmHg in 2 weeks and the same treatment was continued. On the next visit eight weeks later, visual acuity was 20/60, N10. Clinical examination revealed recurrence of the juxtafoveal choroidal neovascular membrane, which was confirmed on FFA and OCT. The patient was treated with four intravitreal injections of bevacizumab (1.25 mg/0.05 ml) at an interval of 4–6 weeks. The IOP was found to have normalized and hence topical antiglaucoma treatment was stopped.\nSix weeks after the fourth injection, visual acuity in the right eye improved to 20/30, N6. CNVM appeared scarred (Figure 3 ). However, there was persistent leakage on FFA. The patient was treated with a combination of PDT and intravitreal bevacizumab. After 7 weeks, visual acuity improved to 20/20. Clinically, CNVM appeared scarred. The patient was followed up at intervals of 2 weeks. Fourteen weeks later, the patient reported with an increase in distortion in the right eye. BCVA was 20/20 in the right eye. OCT showed subretinal fluid and FFA showed recurrent extrafoveal CNVM. The patient was treated with intravitreal bevacizumab (1.25 mg/0.05 ml). The patient was seen six weeks later when she reported resolution of symptoms; her visual acuity was 20/20. Clinical findings were confirmed on OCT and FFA (Figure 4 ). When seen last (i.e. 10 years post bevacizumab injection), BCVA was maintained at 20/20 and the fundus remained stable.

[[21.0, 'year']]

F

{'15652853': 1, '1521819': 1, '5945175': 1, '29977729': 2}

{}

6008534-1

comm/PMC006xxxxxx/PMC6008534.xml

Phantom Sensations Following Brachial Plexus Nerve Block: A Case Report

A 54-year-old woman with all limbs intact received BPA in advance of right thumb carpometacarpal arthroplasty with ligament reconstruction. Immediately after BPA onset, she felt her right forearm and hand resting across her chest when it was hanging over the side of the gurney. After surgery, her right hand felt “heavy” with the fingers stacked vertically on top of each other, as shown in Figure . She began experiencing right thumb pain 14–16 h after the operation had been completed. However, the sensation of immobility of her 2nd through 5th digits in the stacked position lasted for ~3.5 days after surgery and 69 h after the anesthesia wore off. During this time, although the patient described the phantom sensations as being uncomfortable, she experienced no pain in the fingers. No nerve conduction studies were performed.\nThis study was carried out in accordance with the recommendations of the University of Tennessee Health Science Center. The procedure discussed in this report was not part of a research study but rather routine clinical care. The subject gave written informed consent for publication of her clinical details in accordance with the Declaration of Helsinki.

[[54.0, 'year']]

F

{'9204932': 1, '18032777': 1, '25935556': 1, '23959836': 1, '30717476': 1, '3991231': 1, '24308369': 1, '17500095': 1, '2293143': 1, '30488117': 1, '25596440': 1, '27547774': 1, '4702820': 1, '25900792': 1, '11114177': 1, '19927067': 1, '32631395': 1, '28850360': 1, '27863697': 1, '27551416': 1, '19318298': 1, '12849487': 1, '8637922': 1, '19556069': 1, '29497188': 1, '10714655': 1, '23608362': 1, '25529640': 1, '9762952': 1, '7777055': 1, '28163694': 1, '32982914': 1, '29951033': 2}

{}

6008614-1

comm/PMC006xxxxxx/PMC6008614.xml

Iatrogenic Splenic Laceration Presenting as Syncope

A 70-year-old man presented to the emergency department (ED) after falling twice at home. While standing after eating a light meal, he had two separate episodes of brief loss of consciousness. On the second fall, the patient had hit his right shoulder, cheek, and foot, prompting the visit. The patient had received a colonoscopy earlier in the day for routine cancer screening. He had followed proper protocol regarding his bowel prep and had not had any immediate complications related to the procedure or anesthesia. Since the colonoscopy, he had continuous bouts of cramping abdominal pain. He had also felt dizzy throughout this period but attributed it to dehydration related to his bowel prep. The patient denied striking his head, chest pain, shortness of breath, nausea, vomiting, or blood in his stools. His past medical history was significant for coronary artery disease, hyperlipidemia, and hypertension. The patient took his benazepril, aspirin, and atorvastatin on the day prior to colonoscopy.\nOn physical exam, the patient was afebrile with a heart rate of 87 and blood pressure of 130/78. The patient's head, neck, chest, and neurological exams were entirely normal. His abdominal exam was remarkable for tenderness in the right and left lower quadrant with some voluntary guarding, but no masses or rebound was appreciated. His orthopedic exam demonstrated tenderness to his right acromioclavicular joint and ecchymosis of his right 5th metatarsal with no deformity and normal range of motion at all joints.\nOn laboratory evaluation, the patient's hemoglobin was 12.4 g/dL, with normal platelets and chemistry studies. Head computed tomography (CT) and shoulder and chest radiography were normal. Foot radiography demonstrated a nondisplaced 5th metatarsal fracture. CT of the abdomen and pelvis demonstrated moderate hemoperitoneum with splenic laceration (Figures , , and ).\nThe patient was admitted to the intensive care unit and underwent serial abdominal exams and every four-hour hemoglobin measurements. His hemoglobin fell to 8 g/dL at 48 hours after admission but subsequently stabilized. The patient was observed in the hospital and ultimately discharged home on hospital day 3 in good condition.

[[70.0, 'year']]

M

{'24686342': 1, '26679172': 1, '29332407': 1, '17697197': 1, '27200481': 1, '23382625': 1, '26905938': 1, '4524804': 1, '24782929': 2, '33083066': 2, '21960762': 1, '21951473': 1, '29974002': 2}

{'3978400-1': 1, '7563073-1': 1}

6008617-1

comm/PMC006xxxxxx/PMC6008617.xml

Hemodynamic Response to Massive Bleeding in a Patient with Congenital Insensitivity to Pain with Anhidrosis

A 36-year-old woman (height, 147 cm; weight, 50 kg) with CIPA was scheduled for revision of left total hip arthroplasty. She was diagnosed as having CIPA because of recurrent episodes of unexplained fever, anhidrosis, burns, and bone fractures after birth. She had previously undergone 7 operations for spinal deformity and 1 operation of total hip arthroplasty in both the left and right sides. Although lack of general diaphoresis and thermal nociception were observed, the patient performed body surface cooling at her own discretion when she felt she was at a risk of hyperthermia, and her body temperature was kept approximately 36°C. No signs of mental retardation or orthostatic hypotension were observed. No abnormality was detected on chest radiographs and electrocardiograms. Blood biochemistry revealed no abnormality except mild anemia indicated by a hemoglobin level of 10.6 g/dl.\nNo premedication was administered. After the patient was brought into the operating room, routine monitoring and measurement of the bispectral index (BIS) were started. Body temperature was measured at 3 different sites (urinary bladder, esophagus, and precordial skin) and controlled by a hot-air-type heater. Propofol was administered at an effect-site concentration of 4 μg/ml by target-controlled infusion. After muscle relaxation had been achieved by administration of 50 mg of rocuronium, the trachea was intubated. Immediately after endotracheal intubation, systolic blood pressure increased from 130 to 145 mmHg, and heart rate increased from 60 to 95 beats per minute (bpm). Two minutes later, systolic blood pressure had decreased to 125 mmHg. Propofol was continuously infused intravenously at a target concentration of 2 to 4 μg/ml () and BIS levels were maintained between 40 and 60. After an arterial catheter had been placed, her position was changed from the supine to right lateral position. Surgery was then started.\nSince no circulatory change associated with pain occurred during surgery, opioids were not administered. Regarding hemodynamics, when 600 ml of blood was rapidly lost within 20 minutes, blood pressure decreased from 113/66 to 93/55 mmHg and heart rate increased from 55 to 70 bpm ( a). Similarly, when 850 ml of blood was lost within 30 minutes, systolic blood pressure decreased from 108/65 to 95/60 mmHg and heart rate increased from 66 to 74 bpm ( b). Administration of 0.1 mg of phenylephrine increased blood pressure from 87/55 to 117/76 mmHg and decreased heart rate from 70 to 65 bpm ( c).\nThe operative time was 6 hours and 49 minutes, and the duration of anesthesia was 8 hours and 41 minutes. The volume of blood loss was 3350 ml. Blood transfusion was performed with 1600 ml of preoperatively donated autologous blood, 900 ml of salvaged blood, and 720 ml of fresh frozen plasma. Intraoperative body temperature was controlled and kept between 36.0°C and 36.9°C at all 3 measurement points. After surgery had been completed, the patient was returned to the supine position and she was extubated. Since she did not complain of any pain after the surgery, no analgesic was administered. She was discharged at 6 weeks after the operation.\nBlood samples were collected 3 times: before anesthesia induction, after the start of surgery, and at the end of surgery. The levels of catecholamine fractions and cortisol were measured. Norepinephrine levels were below the normal range at all time points, and the levels of epinephrine and cortisol were within the normal ranges at all time points ().

[[36.0, 'year']]

F

{'3031124': 1, '11812683': 1, '16618305': 1, '8390330': 1, '25627679': 1, '8366567': 1, '16427527': 1, '28838318': 2, '26484462': 1, '15078381': 1, '15695606': 1, '22882139': 1, '8696348': 1, '11792664': 1, '29971168': 2}

{'5571499-1': 1}

6008645-1

comm/PMC006xxxxxx/PMC6008645.xml

Two Surgical Cases of Combined Hepatocellular-Cholangiocarcinoma, Intermediate-Cell Subtype: Potentially Characteristic Gross Features

The first patient, who was a man in his late seventies with an unremarkable previous medical history, presented with isovascular nodule accompanied by slow venous wash-out on abdominal dynamic CT in the left lobe of liver. The laboratory data, including the blood cell count, chemistry, and tumor marker levels, were within the normal limits, with the exception of mildly elevated CRP (0.39 mg/dL) and decreased hemoglobin (8.9 g/dL) levels. Neither infection of HBV nor infection of HCV was noted. Based on the clinical findings, the initial diagnosis by the clinicians was most likely HCC, and, thus, left partial hepatectomy was performed. On gross examination, the cut surface of hepatic nodule () showed a poorly demarcated peripheral nodule in lobulated margins, measuring 32 x 21 mm in diameter, which appeared clearly whitish in color. The background of this liver showed no remarkable change (). A microscopic examination of the tumor demonstrated an unencapsulated, ill-defined, and expansive nodule (). This cancerous nodule showed a solid proliferation of atypical epithelial cells, arranged predominantly in solid nests (), trabeculae, and/or ill-defined, fused tubule-like structures (). On a high-power view, these atypical cells were small-to-medium-sized and relatively uniform, having enlarged hyperchromatic nuclei and scant cytoplasm without any evidence of intracytoplasmic mucin (). Intriguingly, prominent fibrous stroma was not evident in this tumor. Immunohistochemistry revealed that the abovementioned carcinoma cells were specifically positive for not only CK7 (cholangiocytes marker) but also CK8/CK18 (markers for both hepatocytes and cholangiocytes) and CK19/CD56 (potential stem cells markers), whereas they were negative for Hepatocyte (hepatocytes marker) [, ]. Based on all of these features, the final diagnosis was primary CHC-INT. To date, this patient has been followed up for 1 year since surgery, and he remains well without any sign of recurrence.

[[77.5, 'year']]

M

{'8872774': 1, '27634656': 1, '18152860': 1, '24712771': 1, '26969740': 1, '23388123': 1, '29967709': 2}

{'6008645-2': 2}

6008645-2

comm/PMC006xxxxxx/PMC6008645.xml

Two Surgical Cases of Combined Hepatocellular-Cholangiocarcinoma, Intermediate-Cell Subtype: Potentially Characteristic Gross Features

The second patient, who was also a man in his early seventies with more than 25-year follow-up for HCV-positive chronic hepatitis and recurrent HCC, presented with mildly hypervascular and ring-enhanced nodule accompanied by venous wash-out on abdominal dynamic CT in the S5 of the remnant liver. The laboratory data were mostly within the normal limits, with the exception of mildly elevated AST (37 IU/L), total bilirubin (1.8 mg/dL), and CEA (5.55 ng/mL) levels. No infection of HBV was observed. Based on the clinical findings, the initial diagnosis by the clinicians was recurrent HCC, and, thus, partial S5 hepatectomy was performed. On gross examination, the cut surface of hepatic nodule () showed a poorly demarcated portal nodule in lobulated margins with central necrosis, measuring 35 x 26 mm in diameter, which appeared clearly whitish in color, accompanied by not only gross but also histopathological portal vein permeation (). A microscopic examination of the tumor showed an unencapsulated, ill-defined, and expansive/infiltrative nodule, displaying a solid proliferation of atypical epithelial cells, arranged predominantly in solid nests, trabeculae, and/or irregular and fused tubule-like structures, aggressively involving the portal vein with focal perineural invasion (). On a high-power view, these atypical cells were very similar to those of the abovementioned first case. Prominent fibrous stroma was not seen either. The background of this liver showed mild chronic hepatitis (F1/A1) and steatosis. Immunohistochemistry showed that those carcinoma cells were specifically positive for not only CK7 () but also CK18 () and c-kit (potential stem cells marker) [, ], whereas they were negative for CK8, Hepatocyte, and CD56. Based on all of these features, the final diagnosis was primary CHC-INT as well. The recurrence of CHC-INT in the remnant liver occurred 1 year and 3 months after this surgery, but he remains not worse with follow-up for the postoperative 2 years.

[[72.5, 'year']]

M

{'8872774': 1, '27634656': 1, '18152860': 1, '24712771': 1, '26969740': 1, '23388123': 1, '29967709': 2}

{'6008645-1': 2}

6008661-1

comm/PMC006xxxxxx/PMC6008661.xml

Seemingly Harmless Differentiated Thyroid Carcinoma Presenting as Bone Metastasis

A 62-year-old male presented with refractory sacral coccygeal pain. The patient had past medical history of type 2 diabetes mellitus (treated with linagliptin/metformin), nontreated high blood pressure, right-sided hemiparesis following meningitis in childhood, nephrolithiasis, and smoking history. The pelvic computed tomography (CT) revealed a 9x7.5x9 cm bulky mass in the sacrum with locally increased soft tissue density, causing extensive lytic lesions of the sacred vertebrae and extending to the left iliac bone, suggestive of chordoma. The patient underwent total sacrectomy with partial excision and reconstruction of the left iliac bone. The anatomopathological examination revealed sacrococcygeal involvement by a thyroid carcinoma, as verified by immunohistochemical staining for thyroglobulin and TTF-1, predominantly papillary (follicular variant), however with foci of nondifferentiated (insular) carcinoma (Figures and ). Thyroid ultrasonography showed a solid nodule of 20 mm in the right lobe and two solid hypoechogenic nodules of 11 and 9 mm in the left lobe, the smallest with coarse calcifications. No lymphadenopathies were found. 18F-fluorodeoxyglucose positron-emission tomography (18F-FDG-PET) revealed a hypermetabolic focus in the left lobe of the thyroid, consistent with the suspected malignant neoplasia, and uptake of the radiopharmaceutical drug in the fifth lumbar vertebra and pelvic bones, consistent with secondary involvement (). Consequently, the patient underwent total thyroidectomy. Histological examination revealed only follicular and oxyphilic variants of multifocal papillary carcinoma (at least six foci) and none of insular carcinoma, with dimensions ranging from 2 to 15 mm, without signs of hematogenic, lymphatic, or perineural permeation, as well as no signs of invasion of the capsule or extrathyroidal extension, with resection margins uninvolved by tumour (pT1b[m]NxM1R0) (). Radioactive iodine therapy (RAI) was then performed. Posttherapy scintigraphy showed hyperfixation in the remnants of the sacrum and lower lumbar spine, bilateral iliac bone, and anterior cervical region (). TSH-stimulated thyroglobulin was 24490 ng/mL. Follow-up magnetic resonance imaging (MRI) revealed persistence and progression of the pelvic lesion. At this time the patient was unable to walk, had a chronic indwelling bladder catheter, suffered from fecal incontinence, and presented with uncontrolled refractory chronic pain.

[[62.0, 'year']]

M

{'25804379': 1, '26576627': 1, '24399786': 1, '28278295': 1, '32984526': 1, '21816796': 1, '28554832': 1, '23476646': 1, '26452365': 2, '25726410': 1, '25417114': 1, '17667551': 1, '21804527': 1, '27272238': 1, '29967702': 2}

{'6008661-2': 2, '4600274-1': 1}

6008661-2

comm/PMC006xxxxxx/PMC6008661.xml

Seemingly Harmless Differentiated Thyroid Carcinoma Presenting as Bone Metastasis

A 64-year-old male presented with pain in the left iliac region for 6 months. His past medical history was significant for gastric peptic ulcer disease (submitted to partial gastrectomy and chronically treated with lansoprazole), nephrolithiasis, and hyperuricemia. At admission a poorly defined mass on the left posterior iliac crest was palpable. The patient underwent a pelvic CT, which revealed a 9 cm lytic lesion of left iliac bone with soft tissue involvement (). A biopsy was performed and the histological examination and immunohistochemical staining for thyroglobulin and TTF-1 showed iliac involvement of a well-differentiated thyroid carcinoma (Figures and ). Thyroid ultrasonography disclosed a poorly defined 8 mm nodule in the left inferior lobe, heterogeneous and partially calcified, and a 4 mm hypoechoic nodule in the right lobe, without associated lymphadenopathies. The patient was submitted to total thyroidectomy and anatomopathological examination revealed a 1.1 cm papillary carcinoma, of follicular variant, with capsular invasion and limited extrathyroidal extension (ETE). Consequently, RAI therapy was performed. Postablative 131 iodine scintigraphy and 18F-FDG-PET () did not reveal further lesions. TSH-stimulated thyroglobulin was 185051 ng/mL. The patient is currently waiting for a hemipelvectomy.

[[64.0, 'year']]

M

{'25804379': 1, '26576627': 1, '24399786': 1, '28278295': 1, '32984526': 1, '21816796': 1, '28554832': 1, '23476646': 1, '26452365': 2, '25726410': 1, '25417114': 1, '17667551': 1, '21804527': 1, '27272238': 1, '29967702': 2}

{'6008661-1': 2, '4600274-1': 1}

6008675-1

comm/PMC006xxxxxx/PMC6008675.xml

Aortic Aneurysm as a Complication of Granulomatosis with Polyangiitis Successfully Treated with Prednisolone and Cyclophosphamide: A Case Report and Review of the Literature

A 57-year-old Japanese man was admitted to our hospital with a chief complaint of back pain and fever for one month. A computed tomography (CT) scan showed an aneurysm of the infrarenal aorta, with a diameter of 34 mm, and inflammation of the surrounding adipose tissue, nodular lesions of the bilateral lungs, and left maxillary sinusitis (). He was referred to our hospital for further evaluation and treatment.\nAt the time of admission, the patient was 168 cm tall and weighed 56.6 kg. His blood pressure was 98/69 mmHg, pulse was 84 beats per minute, and body temperature was 39.6°C. Serum creatinine was 0.66 mg/mL, and urinalysis showed 1+ occult blood; urinary sediment contained 20 red blood cells per high-power field. Serum analysis showed leukocytosis (10,000/μL) and an elevated C-reactive protein level (29.5 mg/dL). The anti-PR3-ANCA level was 187 IU/mL, and the anti-MPO-ANCA level was normal. The abdominal aortic aneurysm was suspected to be infected, and we began administering antibiotics. The patient's general medical condition failed to improve.\nThe clinical findings of left maxillary sinusitis, multiple nodular lesions in the lungs, fever, and positive anti-PR3-ANCA were clinically suspicious for GPA. Video-associated thoracoscopic lung biopsy was performed. The biopsy specimens demonstrated granulation tissue with necrosis and multinucleated giant cells (). Most infiltrating cells were neutrophils. The ratio (%) of IgG4 to total IgG-positive cells was 33%, and there were 60 IgG4+ plasma cells per HPF in the lung.\nThe patient was diagnosed with GPA. He was treated with an intravenous semipulse dose of methylprednisolone, followed by oral prednisolone 1 mg/kg (55 mg) per day and intravenous administration of cyclophosphamide (700 mg/body once per month). All of the patient's symptoms and CT findings rapidly improved (), and the PR3-ANCA level promptly decreased to the normal range. The diameter of the aneurysm changed from 34 mm to 21 mm after treatment. On tapering doses of steroids, the patient is currently in remission, and the inflammation and PR3-ANCA elevation have completely resolved. There has been no disease recurrence for 4 years after initiation of therapy.

[[57.0, 'year']]

M

{'14605297': 1, '31286925': 2, '645752': 1, '22218969': 1, '28780079': 1, '31799479': 1, '20066062': 2, '18541754': 1, '21124083': 1, '12368751': 1, '15210387': 1, '23889996': 2, '16216089': 1, '22325479': 1, '1975175': 1, '19748223': 1, '15045631': 1, '15480908': 1, '15565392': 1, '23412710': 1, '24492165': 1, '2252417': 1, '8563123': 1, '10791632': 1, '15370727': 1, '9112274': 1, '33082919': 1, '6847264': 1, '21757317': 1, '1739240': 1, '7828392': 1, '31867470': 1, '17888497': 1, '16859602': 1, '26986706': 1, '29971178': 2}

{'6615146-1': 1, '3750224-1': 1, '2804726-1': 1}

6008686-1

comm/PMC006xxxxxx/PMC6008686.xml

Elevated Intracranial Pressure as a Cause of Sick Sinus Syndrome

A 55-year-old male presented to the emergency department 3 weeks after right frontal nodule resection of his multifocal glioblastoma multiforme. The tumor was also in the corpus callosum and the right pontine area. The patient was known to have high ICP (confirmed by magnetic resonance imaging) due to the mass effect of the tumor for which a debulking surgery was done. Recovery from surgery went well, and the patient had a residual left-sided weakness and was able to ambulate by using a walker at discharge. At presentation to the emergency department, the patient had 2 episodes of unprovoked syncope with nausea, somnolence, and worsening of his left-sided weakness. The patient's past medical history was insignificant except for controlled hypertension and epilepsy. The patient's home medications were dexamethasone, levetiracetam, hydromorphone, perindopril, amlodipine, and pantoprazole. While in the emergency room, the patient was noted to have a temporary pause on the cardiac monitor which was symptomatic. The patient's vital signs on admission were a Glasgow Coma Scale of 15, blood pressure of 132/86, heart rate of 61, and respiratory rate of 18 (regular), afebrile, and an oxygen saturation at 94% on 2 L/min of oxygen. When the oxygen was removed, the patient's respiratory rate decreased to 12, oxygen saturation dropped to 89%, and the patient became nonalert. An EKG was done which showed multiple intermittent sinus pauses of durations of 4–6 seconds. The blood work and urine analysis were all within the normal range. A diagnosis of SSS was made, and the patient was admitted to the CCU for pacemaker implantation. While admitted to the CCU, his EKG showed intermittent sinus pauses with the longest having a duration of around 3.6 seconds. A pacemaker was inserted. A 24-hour monitoring post procedure was uneventful, and the patient's telemetry showed a normal sinus rhythm in the 80s to 90s with no paced beats. The patient was then discharged home to be followed up at the pacemaker clinic. During the follow-up, the patient's hemiparesis did not recover and was still using the walker to ambulate. A computed tomography (CT) scan done later showed rapid progression of all lesions, except the one in the pons, with vasogenic edema. There was a significant mass effect with midline shift and uncal herniation.

[[55.0, 'year']]

M

{'10454059': 1, '8519515': 1, '10752323': 1, '12939550': 1, '9191793': 1, '8351439': 1, '23939447': 1, '17285416': 1, '26485000': 1, '1927916': 1, '12725451': 1, '8595618': 1, '6744068': 1, '16995599': 1, '17420362': 1, '18182296': 1, '8419012': 1, '19826756': 1, '16104550': 1, '21572755': 2, '29967700': 2}

{'3085225-1': 1}

6008689-1

comm/PMC006xxxxxx/PMC6008689.xml

A Rare Extraskeletal Osteosarcoma Appearing After 55 Years on a Large Stage 3 Burn Scar

A 66-year-old phototype 6 woman from North-Africa was admitted to our department due to an indolent ulcerovegetative mass that developed on the anterior face of the right thigh. The lesion spontaneously exhibited greyish discharge. The patient anamnesis revealed that she had sustained a hot-water burn to her leg at the age of 11 years. She stated no definitive treatment by skin grafting and had only been treated conservatively following injury. The burned right thigh healed progressively, leaving a large scar on the skin surface. There had been no particular issues concerning the lesion until 2 months previously, at which point the patient noticed a progressive mass in the scar area but had not taken any steps to have it cured. Upon physical examination, the mass was 4 x 2 cm and suggested at diagnosis of Marjolin's ulcer (Figures and ). Histopathologic features of the surgical specimen were characterized by predominant chondroid matrix with marked cellularity, high grade atypia, and high mitotic activity. Foci of bone and osteoid formation were also seen (Figures and ). A diagnosis of cutaneous extraosseous osteosarcoma was suggested after excluding an origin in bone or other primary tumor sites by computed tomography. Indeed, clinical examination and extensive total body radiologic workup revealed absence of bone lesions in any body site. Unfortunately, the patient rejected the therapeutic proposal of definitive large surgical excision and we failed to obtain any notice regarding her further evolution.

[[66.0, 'year']]

F

{'21143461': 1, '26475059': 1, '1062407': 1, '9613404': 1, '18380719': 1, '22148040': 2, '1900726': 1, '31625124': 1, '11039378': 1, '25716761': 1, '10876262': 1, '1290267': 1, '31850101': 1, '22137442': 1, '24341890': 1, '17028504': 1, '25160098': 1, '15896501': 1, '28105506': 1, '19349873': 1, '29971170': 2}

{'3229055-1': 1}

6008691-1

comm/PMC006xxxxxx/PMC6008691.xml

Epithelioid Angiosarcoma Arising from a Huge Leiomyoma: A Case Report and a Literature Review

A 48-year-old woman, gravida 1, para 1, visited the internal medicine department at another hospital with a complaint of abdominal fullness and weight loss of 10kg during the last year. A huge abdominal mass was palpated, and she was referred to the gynecology department to search for a tumor of uterine origin. She was premenopausal and had no significant past medical history. Physical findings revealed a large elastic hard mass extending from the xiphoid to the pubic bone. The magnetic resonance imaging (MRI) examination revealed a huge tumor on the uterine corpus, and a number of dilated vessels were observed between the tumor and the myometrium. Therefore, the tumor was suspected to derive from the uterus. The tumor showed an uneven signal on T2-weighted sagittal section (), and the enhanced MRI study showed that the tumor edge but not the center was enhanced (). As such, necrosis was suspected to have occurred in the center of the tumor. Uterine sarcoma was primarily suspected due to the large size, degeneration, and necrosis on MRI imaging. Computed tomography (CT) examination showed no lymph node swelling or distant metastasis. Preoperative laboratory testing revealed anemia (hemoglobin level, 5.6g/dl). We transfused 18 units of RCC before surgery. CT examination and ultrasonography on lower extremities indicated an absence of thrombosis. Preoperative serum levels of CEA, CA 19-9, CA 125, and LDH were within normal limits. A biopsy of the endometrium was not collected as the sounding examination of the endometrium was unsuccessful due to a deviated uterine cervix. At this point, preoperatively, we suspected the tumor was a leiomyosarcoma or leiomyoma with degeneration.\nThe patient underwent laparotomy, where we identified a huge tumor occupying a space from the pelvis to the diaphragm. The tumor surface was smooth and hard with many dilated veins (). A massive tumor with a diameter of 30 cm was observed arising from the posterior uterine wall with a smooth contour and invaded the retroperitoneal cavity under the mesentery. The tumor was firmly adhered to both the mesentery and right ovary. There were no findings of extra-uterine dissemination. The intraoperative frozen section report for the uterine tumor was of degenerated myoma with no findings indicating malignancy. A total abdominal hysterectomy (TAH) and right salpingo-oophorectomy (RSO) were performed. The operation duration and blood loss were approximately 216 minutes and 1000 ml, respectively. The excised specimen weighed 7600 g.\nMacroscopic findings of the tumor revealed a well-circumscribed tumor showing extensive continuity with the posterior wall of the uterus, measuring 28 × 23 cm (). On the sliced surface of the tumor, an obvious heterogeneous pattern was recognized within the mixture of a whitish homogeneous area, suggesting benign uterine fibroids, and a vulnerable area, due to bleeding and necrosis ().\nFor the intraoperative frozen section, we examined three areas, namely, a white homogenous part, a necrotic part, and a cystic part, of which all were findings of a leiomyoma. In the permanent histological examination, 10 additional sections were collected from the tumor. The basic histological findings of all the sections were the same. The tumor was comprised of spindle-shaped cells, homologous to smooth muscle cells, which were arranged in bundles with areas of hyalinization, consistent with a degenerated leiomyoma. The tumor was mostly comprised of degenerated uterine leiomyoma. However, enlarged blood vessels were observed within an area of approximately 2 cm × several mm, and proliferation of atypical cells showing a fine meshwork microvascular structure was observed in the blood vessel cavity (). These atypical cells consisted of various contours, such as cubic, polygonal, and short spindle shape. The nucleus was circular with a high degree of vacuolar enlargement and pleomorphism. Abnormal mitotic figures were also interspersed (). A tumor derived from a blood vessel was thus considered, and malignancy was suggested by the presence of nuclear atypia and abnormal mitosis.\nImmunohistochemical analysis revealed the atypical tumor cells to be positive for ERG, CD31, and AE1/3 (Figures and ), partially positive for Factor VIII, and negative for α-SMA, desmin, H-caldesmon, EMA, CD34, and D2-40. From the above, the atypical tumor cells were of epithelial origin and the final diagnosis was epithelioid angiosarcoma arising in a degenerated uterine leiomyoma.\nThe efficacy of postoperative adjuvant therapy for angiosarcoma has not been demonstrated and there is currently no established chemotherapy regimen. In this case, because the atypical tumor was observed in the blood vessel cavity, we thought it could have been spread hematogenously throughout the body. Hence, we selected adjuvant chemotherapy rather than adjuvant radiotherapy. Six courses of combination adjuvant chemotherapy with paclitaxel (150mg/m2), epirubicin (50mg/m2), and carboplatin (area under the curve = 4) were administered in the present case, following referral to previous reported cases. No recurrence has been observed 10 months after the primary surgery.

[[48.0, 'year']]

F

{'8085563': 1, '17356953': 1, '9500227': 1, '1660853': 1, '17291268': 1, '28540360': 1, '24900095': 1, '20485141': 1, '29338993': 1, '32158582': 2, '7197190': 1, '10525385': 1, '17858281': 1, '7199374': 1, '28055103': 1, '26352549': 1, '18642010': 1, '8249890': 1, '3121527': 1, '16053668': 1, '24125656': 1, '511004': 1, '18486900': 1, '1702585': 1, '2323746': 1, '6030215': 1, '20537949': 1, '10570428': 1, '29967705': 2}

{'7060455-1': 1}

6008761-1

comm/PMC006xxxxxx/PMC6008761.xml

Unexpected Liver Embryonal Sarcoma in the Adult: Diagnosis and Treatment

A 60-year-old woman came to our attention for abdominal pain, distension, and weight loss (about 6 kg in two months). Abdominal examination revealed a bulky mass occupying the right abdominal quadrants. She was submitted to laboratory routine tests without evidence of chronic liver disease and no alterations of serum tumor markers. Computed tomography (CT) scan confirmed the presence of an expansive mass (15 × 12 × 23 cm), poorly separable from the surrounding liver parenchyma, with heterogeneous contrast enhancement. The lesion was partially cystic with thickened walls and an intralesional solid component ().\nAfter multidisciplinary discussion with radiologist and oncologist, indication to surgery was given and the patient underwent laparotomy. At exploration, a well-defined, partially cystic tumor was found originating from the fifth and sixth hepatic segments and adherent to—but not infiltrating—the right colon and omentum. En bloc resection of the mass and a S5-S6 liver bisegmentectomy were performed. The postoperative period was uneventful, and the patient was discharged on sixth postoperative day.\nThe surgical specimen consisted of a lobulated yellow-reddish neoplasm sized 33 × 19 × 11 cm, with gelatinous cystic and hemorrhagic areas on cut surface. A fibrous discontinuous pseudocapsule separated the tumor from the adjacent compressed liver parenchyma. Microscopically, the tumor was composed of stellate or spindle shaped cells with bizarre morphology and ill-defined outlines, loosely arranged in an abundant myxoid matrix (). Scattered tumor cells with marked nuclear abnormalities and hyperchromasia, as well as multinucleated giant cells, were present. Atypical mitotic figures were easily found. Characteristically, tumor cells showed multiple, different-sized, eosinophilic, PAS-positive globules in the cytoplasm. Immunostainings revealed CD10, CD68, and vimentin expression in tumor cells, with focal and weak expression of wide spectrum cytokeratins (AE1/AE3) (). These features were sufficient for the diagnosis of UESL.\nAfter the definitive histological diagnosis and multidisciplinary evaluation, the oncologist proposed an adjuvant therapy of six cycles with vincristine, actinomycin D, and cyclophosphamide, which was accepted by the patient. 30 months after surgery, she is alive without signs of recurrence.

[[60.0, 'year']]

F

{'17390190': 1, '17573940': 1, '1727675': 1, '20615364': 1, '34979950': 1, '26925712': 1, '25214865': 2, '15063896': 1, '23138115': 1, '18361435': 1, '8780739': 1, '22540346': 2, '10591560': 1, '25674229': 1, '208754': 1, '25611111': 1, '12673559': 1, '15710230': 1, '21937079': 1, '19906110': 1, '1702267': 1, '18836810': 1, '34815717': 2, '32831921': 1, '9331300': 1, '34208030': 1, '16785789': 1, '11815984': 1, '6303939': 1, '1551077': 1, '18926232': 1, '29971180': 2}

{'4160596-1': 1, '8605883-1': 1, '3407002-1': 1}

6008769-1

comm/PMC006xxxxxx/PMC6008769.xml

Fat Embolism Syndrome in Duchenne Muscular Dystrophy Patients: Early Recognition and Aggressive Therapy

A 14-year-old male with DMD has been on daily oral steroid since 9 years of age. He weighed 53 kg (47th percentile) and was 147 cm tall (less than 3rd percentile). He became nonambulatory at 12 years of age. Forced vital capacity (FVC) was 2.37 L or 80% predicted. A polysomnogram was completed and revealed moderate obstructive sleep apnea. His echocardiogram (ECHO) was normal with cardiac magnetic resonance imaging (MRI) demonstrating normal left ventricular ejection fraction (LVEF). He participated in many school activities including wheelchair soccer.\nHe presented to the emergency department with confusion, tachycardia, tachypnea, and fever up to 39.2 degrees Celsius a few hours following soccer practice. During practice, he had been transferred from his wheelchair and his right leg had brushed the ground causing him to have to be placed on the ground until more help was obtained to put him back in his wheelchair. Brain computed tomography (CT) was negative for any acute intracranial process contributing to his current state. Chest computed tomography (CT) scan was negative for pulmonary embolism but showed patchy diffuse nodular airspace opacities seen scattered throughout both lung fields (). He had progressive acute hypoxic respiratory failure requiring mechanical ventilation. Shortly after intubation, he suffered cardiac arrest requiring 3 minutes of cardiopulmonary resuscitation (CPR) before return of spontaneous circulation. ECHOs were consistent with increased pulmonary vascular resistance including moderate right ventricle dysfunction and elevated pulmonary arterial pressure. Duplex ultrasound evaluation of lower extremities was without evidence of deep venous thrombosis bilaterally. X-rays of lower extremities revealed cortical step off at the proximal left femoral neck and nondisplaced fracture at the distal right tibial metaphysis and fibular metaphysis (). Ophthalmologic exam revealed Purtscher-like retinopathy. The patient was treated with 48 hours of antibiotics that were discontinued with negative blood, urine, and mini bronchoalveolar lavage (BAL) culture. The patient was treated with mechanical ventilation and right ventricular afterload reduction with milrinone and inhaled nitric oxide for pulmonary hypertension. These therapies were able to be weaned off, and the patient was able to be extubated on day five of admission. He was later transferred to the pediatric floor and later discharged home without any residual effects from the entire event.

[[14.0, 'year']]

M

{'4547466': 1, '19945913': 1, '23741098': 2, '29066903': 1, '11740000': 1, '19561953': 1, '5487573': 1, '34511509': 1, '19825491': 1, '25180103': 1, '22920089': 1, '30788612': 1, '23951421': 1, '20220408': 1, '30009059': 2}

{'6008769-2': 2, '3669557-1': 1}

6008769-2

comm/PMC006xxxxxx/PMC6008769.xml

Fat Embolism Syndrome in Duchenne Muscular Dystrophy Patients: Early Recognition and Aggressive Therapy

An 11-year-old male with DMD has been on daily oral steroid since 9 years of age. He weighed 30 kg (5.6th percentile) and he was 120 cm tall (less than 3rd percentile). He is able to ambulate without assistance. FVC was 1.5 L or 96% predicted. His ECHO was normal with cardiac MRI demonstrating normal LVEF.\nHe sustained a nondisplaced Salter-Harris type II fracture of the distal left femur () from a fall that required surgical fixation and was able to be discharged home on the day of operation. He presented to the emergency department two days after his operation with seizure, tachycardia, tachypnea, and fever up to 38.6 degrees Celsius. He had progressive acute hypoxic respiratory failure requiring mechanical ventilation. Shortly after intubation, he developed pulmonary hemorrhage and anemia remedied with fresh frozen plasma, ventilator adjustments, and blood transfusion. A fast spin MRI of his head was negative. CT scan of his chest demonstrated bilateral patchy infiltrates; no pulmonary embolism was identified (). ECHO revealed mildly elevated tricuspid regurgitation velocities representing increased right ventricular and pulmonary artery pressures. He was placed on broad spectrum antibiotics for 72 hours but blood, urine, and mini BAL remained negative. Mechanical ventilation was able to be weaned and the patient was extubated on day five of admission. He was later transferred to the pediatric floor and later discharged home without any residual effects from the entire event.

[[11.0, 'year']]

M

{'4547466': 1, '19945913': 1, '23741098': 2, '29066903': 1, '11740000': 1, '19561953': 1, '5487573': 1, '34511509': 1, '19825491': 1, '25180103': 1, '22920089': 1, '30788612': 1, '23951421': 1, '20220408': 1, '30009059': 2}

{'6008769-1': 2, '3669557-1': 1}

6008772-1

comm/PMC006xxxxxx/PMC6008772.xml

Simultaneous Subconjunctival Triamcinolone and Bevacizumab Injections for Management of Blepharokeratoconjunctivitis in Children

Twin 1, a three-year-old Caucasian boy, was initially referred by his family physician with photophobia and frequent blinking of the left eye to Royal North Shore Hospital. He had varicella-zoster virus (VZV) keratitis of the left eye with positive VZV on Polymerase Chain Reaction (PCR) and was treated with intravenous acyclovir and topical acyclovir. His symptoms and follow-up examinations showed resolution.\nOne year from this presentation, best-corrected visual acuity (BCVA) in the right was 20/80 and could not be obtained in the left due to objection to occlusion. Examination under anaesthesia (EUA) revealed corneal neovascularization bilaterally (Figures -). There was blepharitis and conjunctivitis bilaterally, with a stromal infiltrate and overlying epithelial defect in the right eye. He was investigated for causes of interstitial keratitis and corneal neovascularization. Blood tests, and corneal and conjunctival swabs were unremarkable (). Given the results and EUA findings the diagnosis was staphylococcal hypersensitivity/chronic blepharokeratoconjunctivitis (BKC) secondary to meibomianitis, with corneal neovascularization and scarring [, ]. He was prescribed oral erythromycin (160mg, twice a day), chloramphenicol ointment (1%, three times a day) for both eyes, and topical fluorometholone (0.1%, twice a day) for the left eye.\nReview two weeks later showed progressive corneal neovascularization and infiltrate. Due to the progression despite conventional treatment and to aid compliance with the treatment, the decision was made for subconjunctival triamcinolone acetonide and bevacizumab, with regular EUA for treatment and monitoring of progress and side effects. Two weeks later, he received subconjunctival triamcinolone (4mg/0.1mL × 0.55mL) to the inferior conjunctiva and subconjunctival bevacizumab (2.5mg/0.1mL × 0.05mL) to the superior conjunctiva of the right eye. Extensive lid hygiene including 5% povidone-iodine lid scrubs and Meibomian gland expression was performed, and this was repeated at subsequent EUA. Intraocular pressures (IOP) by iCare® tonometer (iCare Finland) were 14mmHg in each eye. He continued on the same medication regimen and SteriLid (TheraTears®) two to three times per week.\nSix weeks after the injections, the mother of Twin 1 reported significant improvement in his symptoms. At EUA eight weeks after the injections, the right cornea looked markedly improved (Figures -). IOPs were 12mmHg in the right and 6mmHg in the left eye. Given that there had been dramatic improvement with no significant side effects observed, he was continued on the same regimen and the left eye was injected with the same dose of triamcinolone and bevacizumab.\nAt EUA 11 weeks after the left eye injections, there was quiescent interstitial keratitis. Adjunctive diathermy was applied to the patent superior and inferior feeder vessels in the left eye. Further subconjunctival triamcinolone (4mg/0.1mL × 0.5mL) was injected inferiorly in each eye. Binocular BCVA (due to objection to occlusion) was 20/63.\nAt EUA 5 months after the further bilateral triamcinolone injections, there was central corneal scarring and suppressed blood vessels bilaterally (Figures -). Diathermy was applied at the limbus to further feeder vessels of each eye. IOP was 16mmHg in the right and 19mmHg in the left eye. Binocular BCVA (due to objection to occlusion) 24 months after presentation was 20/63.

[[3.0, 'year']]

M

{'23485045': 1, '24858528': 1, '22459106': 1, '18054641': 1, '15774912': 1, '26058029': 1, '19021596': 1, '22898649': 1, '25014152': 1, '26634709': 1, '29967706': 2}

{}

6008780-1

comm/PMC006xxxxxx/PMC6008780.xml

Large Draining Focal Fibrous Hyperplasia Secondary to Periapical Granuloma

A 59-year-old man presented to the urgent care clinic at the School of Dentistry complaining of an upper lip mass for one-year duration. The mass started out as a small bump and had grown steadily since then. 3 months prior to his presentation, a draining parulis developed on the mass. The patient had no history of systemic symptoms such as fever, chills, weight loss, or fatigue. He was aware of a dark-colored “dead tooth” for several decades in the area of concern but denied any previous history of swelling in the area. The patient had recently moved to the United States from Nigeria and had previously been without access to adequate dental care. The patient said that a doctor in Nigeria told him that the lesion was likely cancerous.\nOn exam, there was a large, painless, fibrous, exophytic mass in the anterior maxillary labial vestibule (Figures and ). The base of the mass approximated the apex of tooth #8. A yellow purulent material was observed draining from the parulis (). Tooth #8 was discolored and was confirmed to be nonvital on pulp testing. There was a significant gap between teeth #7 and 8. Tooth #8 was displaced medially and was extruded relative to the adjacent dentition.\nA periapical radiograph revealed a large unilocular radiolucency associated with the apex of tooth #8 (). Cone-beam computed tomography again demonstrated a large cystic-appearing defect in the anterior maxilla with perforation of the buccal and palatal cortices . The lesion extended to the nasal floor on the ipsilateral side.\nThe patient was referred to the oral surgery department for excisional biopsy. After tooth #8 was removed, an incision was made around the base of the stalk that connected the mass to the labial and alveolar mucosa. Sharp dissection was used to free the mass, and the specimen was sent for histopathologic analysis. The mass communicated with a cystic lesion of the maxilla. The cyst was enucleated with a curette and also sent for pathology. Perforation of the cyst through the buccal and palatal cortices was noted during the procedure. Slight undermining of the wound margins allowed for closure with resorbable sutures.\nAt the patient's one-week follow-up (), he was doing very well. He reported minimal pain, no neurosensory disturbances, and no systemic or local symptoms of infection. He and his family were very relieved to learn that the lesion was benign. He was happy with his appearance after having the mass removed.

[[59.0, 'year']]

M

{'28149477': 1, '25364187': 1, '23289061': 1, '29967701': 2}

{}

6008794-1

comm/PMC006xxxxxx/PMC6008794.xml

Camptocormia in an Adolescent: A Case Report and Review of the Literature

A 15-year-old female with a past medical history of obesity presented to the emergency department with a marked 90-degree flexion at the thoracolumbar region. She was able to walk only by using a walker in an unusual manner; she placed her axillae on the hand grips. The patient described constant back pain almost daily for which she took ibuprofen with moderate relief. These symptoms began three years ago following a sexual assault and have progressed significantly. As per the patient's mother, the only time the patient's spine straightened out was during deep sleep. She had no neurologic complaints, specifically denying any sensory or motor deficits, fatigue, ptosis, or any complaints consistent with any form of endocrinopathy. Her family history included siblings with epilepsy, ADHD, and mood disorders.\nThe patient denied any substance abuse, including use of alcohol or cigarettes, as well as being sexually active. She reported no history of mood or psychotic symptoms and no suicide attempts.\nDespite the pain, however, the patient was not concerned with her condition. The patient herself was not interested in physical therapy or additional diagnostic testing or counseling despite her mother's encouragement. She refused a gynecological examination due to anxiety following having been a victim of sexual assault; it was unclear the extent of counseling, if any, she received following the assault.\nThe patient was a seventh grader at a local school where she participated in extracurricular activities. She reported being well accepted by her peers. She stated having a good social support network including friends and family. Her parents were separated and she lived with her mother and brother.\nPhysical exam revealed an obese (BMI 39.2) Caucasian female, leaning forward with the spine nearly ninety degrees at the waist. Extremities showed normal strength, sensation, and range of motion. The patient was unwilling to attempt spinal extension. Tenderness to palpation of the paraspinal muscles was present. The rest of the physical examination was unremarkable.\nUpon Pediatric Neurology consultation, no evidence of neuromuscular disease was found. Plain radiography of the cervical, thoracic, and lumbar spine was limited due to her deformity, but no abnormalities were noted. Magnetic resonance imaging of the same was unrevealing. Psychiatric consultation was obtained; they concluded that the patient's symptoms were likely manifestations of Conversion Disorder, following exclusion of organic causes.\nWhile she is currently receiving social services support and physical therapy; she declines psychiatric outpatient care at this time.

[[15.0, 'year']]

F

{'27668941': 1, '3143551': 1, '14286998': 1, '10423797': 1, '17516452': 1, '21916627': 1, '17712851': 1, '17922169': 1, '21016922': 1, '15322421': 1, '10348467': 1, '7631230': 1, '20644155': 1, '20761312': 1, '26900733': 1, '28416565': 1, '10939223': 1, '2527341': 1, '27314757': 1, '26506755': 1, '29971176': 2}

{}

6008800-1

comm/PMC006xxxxxx/PMC6008800.xml

An Ulcerated Ileal Gastrointestinal Stromal Tumor Disguised as Acute Appendicitis

A 28-year-old female with insignificant past medico surgical history presented with one day of acute onset pain in the periumbilical region that later migrated and confined to the RIF. She had associated intermittent fever, nausea, and loss of appetite. She did not have any urinary symptoms, bowel irregularities, or gynecological complaints. Abdominal examination was performed by two senior surgeons at two different occasions; the same day had findings of guarding and rebound tenderness at RIF. Hematological tests showed polymorphonuclear leukocytosis with left shift. Biochemical tests and urinalysis were normal. Urinary pregnancy test was negative. Abdominal radiographs were unremarkable. USG could not visualize appendix and was inconclusive except for probe tenderness in RIF. CT scan of the abdomen could not be done due to unavailability. A clinical diagnosis of acute appendicitis was made assigning an Alvarado score of 9/10. Laparotomy was performed using the Lanz incision in RIF. Intraoperatively appendix was found to be normal without evidence of inflammation or infection in RIF. In view of symptoms and signs, a possibility of other pathology was thought. Walking the bowel proximally up to 3 feet (1 m) did not show a Meckel's diverticulum or any other small bowel lesions. There were no obvious mesenteric lymph nodal enlargement and pelvic organs looked pristine. Approaching closure, just when the medial edge of the incision was retracted superomedially, a hemorrhagic lesion seemed to appear little deeper in the mid abdomen. Therefore, the incision was extended transversely from the medial edge to explore further. Entire bowel was explored and this revealed an ulcerated lesion measuring 7 × 5 cm arising from the antimesenteric border of the ileum 8 feet (2.5 m) from ICJ with localized interloop hemoperitoneum and inflammatory exudates as shown in . Resection of ileal segment containing the lesion was performed followed by restoration of bowel continuity and peritoneal toileting. The lesion was subsequently reported to be an ulcerated malignant ileal GIST.\nHistopathologically, gross examination confirmed the operative findings, and the cut section revealed a nodular lesion protruding out of the serosal surface measuring 7 × 5 cm along with 2 lymph nodes each measuring 2 × 1 cm.\nMicroscopically, the growth from the ileum had villous lining epithelium with focal ulceration. The submucosal region had a circumscribed nodule with proliferation of loosely cohesive spindle cells; some of which were arranged in vague storiform pattern and others in long fascicles. There were areas with epitheloid cells forming small anastomosing nests and cords. The areas in between these showed skenoid fibers along with focal areas of hemorrhage, infarction, and congestion as shown in . The mitotic figures were seen (8/50 high-power field). The lymph nodes were microscopically identified to be reactive, and the resected margins of the ileum were free of tumor.\nBased on tumor size and mitotic activity, possibility of a malignant GIST was suggested along with immunohistochemical analysis (CD117 and CD34) for further confirmation. The patient had an uneventful recovery and was discharged on the 8th postoperative day. She was advised to review a week later at the outpatients but failed to report. All possible contacts were used to trace her, but she remained inaccessible and lost to follow-up.

[[28.0, 'year']]

F

{'22349155': 1, '28119940': 1, '24500386': 1, '23317620': 1, '27900093': 1, '24829670': 2, '9409605': 1, '16960208': 1, '24379631': 1, '16285345': 1, '25833673': 1, '26170759': 1, '15666631': 1, '27990209': 2, '29971179': 2}

{'3990132-1': 1, '5139700-1': 1}

6008806-1

comm/PMC006xxxxxx/PMC6008806.xml

Vaginal Bleeding as Initial Presentation of an Aggressive Renal Cell Carcinoma: A Case Report and Review of the Literature

A 54-year-old, obese, Caucasian female, a current smoker, presented to the clinic with complaints of intermittent vaginal bleeding for approximately one week and that she “felt something” in her vaginal area. Her past medical history includes diabetes mellitus, hypertension, and an abdominal hysterectomy 30 years ago due to heavy menstrual bleeding of benign etiology. Upon examination, a pedunculated mass was found on the vaginal wall at approximately 7 o'clock. The mass, measuring 2.0 × 1.5 × 1.3 cm, was subsequently excised.\nHistologic examination revealed a clear cell carcinoma. Immunohistochemical stains were positive for CD10, PAX-8, and carbonic anhydrase 9/IX (CA-IX) and negative for CK7. A diagnosis of metastatic CCRCC was made (). Radiological studies then revealed a left renal mass, bilateral adrenal masses, and enlarged retroperitoneal lymph nodes. A subsequent left radical nephrectomy and adrenalectomy was performed. On gross examination, a unifocal tumor that measured 14.7 cm in its greatest dimension was identified. Pathologic examination confirmed the renal origin of the vaginal carcinoma. The nephrectomy diagnosis was established as CCRCC with rhabdoid differentiation and multinucleated giant tumor cells. The World Health Organization (WHO)/International Society of Urologic Pathologists (ISUP) grade was 4. The tumor invaded into the perinephric fat tissue, renal sinus, and major branches of the renal vein. Tumor necrosis and lymphovascular invasion were identified. All margins, including Gerota's fascia, ureteral and vascular, were free of malignancy. The left adrenal gland was involved with metastatic CCRCC. The final pathological stage was pT3a pNx pM1.\nMultiple pulmonary nodules, measuring up to 4 mm, and a right adrenal nodule were observed on imaging. The patient is now receiving adjuvant targeted therapy (Sunitinib 50 mg). The lung nodules are no longer seen, and the right adrenal nodule had decreased more than 30% in size. Interestingly, her vaginal mass recurred, increased in size, and changed in shape since her last resection. She, thus, underwent a partial excision of the new lesion on the vaginal wall. The pathologic examination revealed a CCRCC, with extensive rhabdoid differentiation this time ().

[[54.0, 'year']]

F

{'2237304': 1, '31916227': 1, '18794048': 1, '29140878': 1, '34040813': 2, '759654': 1, '22112490': 1, '22397650': 1, '23565309': 1, '23652071': 1, '14984968': 1, '26630224': 1, '25127094': 1, '29967708': 2}

{'8121607-1': 1}

6008810-1

comm/PMC006xxxxxx/PMC6008810.xml

Accidental Drowning: The Importance of Early Measures of Resuscitation for a Successful Outcome

A sixteen-year-old boy nonswimmer was hospitalized after drowning in cold river water. Based on data given by the passing eyewitness, the teenager jumped into the river to retrieve a sheep he was tending but immediately disappeared from the water surface. This youth of the same age promptly dived into the river, found the victim's body on the bottom at a depth of 2 metres, raised him, and swam to the shore, which was about 3 metres away. Based on the rescuer's statement, the drowning boy was unconscious, was not breathing, had blue lips and nail pates, and had very cold skin. Due to knowledge about First Aid acquired in high school, the rescuer started measures for basic life support (BLS (Basic Life Support)), freed the airway, performed several mouth to mouth breaths, and started chest compression. After 2-3 minutes the victim vomited, threw up water, coughed, and started breathing. In the next few minutes he regained consciousness. In the meantime, members of his family had been called and they brought the patient to our institution in a private vehicle.\nAt arrival he was somnolent, oriented, anxious, and pale, with tachypnea (number of respirations 30/min), with hypothermic-body temperature 35.1°C, shivering (whole body), and with the subjective sensation of cold. He was admitted to the JIT and the following was monitored: ECG TA, pulse, body temperature, and pulse oximetry. A nasogastric tube and a urinary catheter were placed. Auscultation of both lungs revealed weakened breathing sounds, especially in the basal and middle parts, with many early and late inspirium cracklings and low tone whistling. Cardiac action was arrhythmic accompanied by tachycardia up to 120/min, BP (blood pressure) 100/55 mmHg. There were no signs of neurological deficit or lateralization, GCS (Glasgow Coma Scale) 13/15.\nUsing transcutaneous pulse oximetry at admission we measured the following: O2 saturation, which was 80% (during the course of treatment it normalized); pH 7.32; O2 54.8 mm Hg; CO2 55 mm Hg; BE—1.60 mmol/L; C-reacting protein 96 mg/L; Er 5.11 × 1012/L; Hb 136 g/L; Le 14.3 × 109/L; glycemia 4.0 mmol/L. Chest radiography showed spotty shadows in the middle and inferior lobes with free costodiaphragmatic recess sinuses bilaterally.\nThe ECG at arrival indicated an irregular rhythm, HR (heart rate) 120/min with short-term atrial fibrillation that later corrected spontaneously into normal sinus rhythm.\nImmediately upon admission we continued reanimation. The patient was warmed up with thermal blankets. Oxygen therapy was applied using an oxygen mask with reservoir without rebreathing, at a flow rate of 8 l/min, decreasing to 6 l/min in order to maintain SpO2 ≥ 94%. Parenteral antibiotic therapy (III generation cephalosporin, aminoglycosides, and metronidazole) and parenteral rehydration were administered.\nOn the fifth day after admission the patient showed complete recovery, including withdrawal of the chest radiographic symptoms and no pathological signs were visible on physical examination of the lungs.\nHowever, during each attempt to get up, we registered atrial fibrillation or individual SVES (supraventricular extrasystole) and VES (ventricular extrasystole) triplets. On echocardiographic examination the structure of the left ventricular myocardium was hyperechogenic; echocardiography was performed with slightly restricted LVEF\nDuring halter ECG examination we registered multiple strings of VES triplets, frequency 150 per minute. An appropriate dose of Presolol was included in the therapy, with advice of strict inaction and avoidance of any kind of physical exertion.\nTwo months later the control echocardiography revealed that heart function had improved further, FS 0.34%; EF > 45%.

[[16.0, 'year']]

M

{'21615299': 1, '32482170': 1, '22250031': 1, '26477701': 1, '12848313': 1, '9349132': 1, '10376572': 1, '20498167': 1, '16302042': 1, '29974001': 2}

{}

6008859-1

comm/PMC006xxxxxx/PMC6008859.xml

Full Thickness Retinal Hole Formation after Nd:YAG Laser Hyaloidotomy in a Case with Valsalva Retinopathy

A 27-year-old male was referred to our hospital by a secondary care center due to a sudden onset of visual loss in his right eye, which occurred while weight-lifting in sports gym 4 days previously. The clinic referred the patient with fundus photographs which were screened 4 days before () and after the Nd: YAG laser hyaloidotomy treatment. Snellen acuity was 10/10 in both eyes. There was no afferent papillary defect and anterior segment examination was normal. The intraocular pressures were measured as 14 mmHg in both eyes.\nDilated fundus examinations revealed a normal macula in left eye. But there was a retinal pigment epithelium (RPE) alteration at the margin of the inferior temporal arterial vascular arcade in the right eye and resolved preretinal and subretinal hemorrhages were seen in the macula (). A diagnosis of Valsalva retinopathy was made based on the history and the treatment photographs of Nd:YAG laser hyaloidotomy (). The patient was also screened with fundus fluorescein angiography for any other vascular pathologies ().\nAt 1st month of examination all hemorrhages were resolved but RPE alterations were still at the margin of the inferior temporal arterial vascular arcade in the right eye (). The patient was screened with (OCTA) (OCTA; Avanti, Optovue RTVue XR). The OCTA images revealed 2 lesions. On en face OCT angiogram of OCTA full thickness retinal hole formation and ellipsoid zone damage at the superior and inferior margin of the inferior temporal arterial vascular arcade were seen (). Superficial vascular plexus was also damaged at that region. The projection of the evacuation of blood from subhyaloid space and the full thickness retinal hole formation were same ().

[[27.0, 'year']]

M

{'24843309': 1, '19997743': 1, '7970521': 1, '4696667': 1, '19495839': 1, '23616766': 1, '23587284': 2, '17891339': 1, '25279780': 1, '23638430': 1, '19205491': 1, '10326976': 1, '11767030': 1, '9823347': 1, '23499439': 1, '2912113': 1, '14516831': 1, '29967707': 2}

{'3640895-1': 1}

6008866-1

comm/PMC006xxxxxx/PMC6008866.xml

Delayed Diagnosis of Acute Rheumatic Fever in a Patient with Multiple Emergency Department Visits

A 5-year-old boy was hospitalized with a 3-month history of intermittent fevers, fatigue, migratory joint pain, and weight loss. Three months ago, the patient was diagnosed with group A Streptococcus pharyngitis and was prescribed a 10-day course of amoxicillin. Two weeks later, he started having pain in his knees, elbows, shoulders, and neck. The patient was diagnosed with influenza and completed a course of oseltamivir. His joint pain and intermittent fever persisted. He began to develop malaise, weakness, and difficulty waking and was noted to have cough. During the next month, the patient was evaluated twice in different emergency departments with the same complaints. Evaluation at the emergency department revealed a mildly elevated C-reactive protein and erythrocyte sedimentation rate. Chest X-ray findings were consistent with pneumonia, and he was prescribed amoxicillin/clavulanic acid. Blood and urine cultures were negative. Three weeks later, the patient presented to his pediatrician with complaints of bilateral ear pain. His fevers, joint pain, difficulty walking, and fatigue persisted. During physical examination, the pediatrician noted a new systolic murmur. It prompted his hospitalization for further evaluation and management.\nOn examination, the patient appeared unwell, but nontoxic. His temperature was 96.8°F (36.0°C), heart rate was 123 beats/min, respiratory rate was 23/min, blood pressure was 109/65 mm Hg, and oxygen saturation was 98% on room air. His growth was plotted on the 25th percentile for weight and height. He had a V/VI holosystolic murmur with thrill at the mitral area with radiation to the axilla and interscapular area. The boy reported pain during active and passive motion of both knees and the neck. His left knee was slightly swollen. Examination of the skin revealed presence of small (1 centimeter in diameter) painless, firm nodules over extensor surfaces of ankles and elbows bilaterally, consistent with subcutaneous nodules.\nFurther review of the patient's history of present illness reveled noncompliance with previously prescribed treatment. Parents reported that the boy refused to take antibiotics. Laboratory evaluation showed the white blood cell count of 9.9 × 103 μ/L (9.9 × 109/L), platelet count of 495 × 103/µL (495 × 109/L), hemoglobin level of 11.2 g/dL (112 g/L), C-reactive protein level of 84.3 mg/L (802 nmol/L), and erythrocyte sedimentation rate of 93 mm/hr. Antistreptolysin O titers were 4,133 IU/ml (reference range, <150 IU/ml). Results of urinalysis were normal. Electrocardiography showed normal sinus rhythm with signs of left ventricular hypertrophy with volume overload with increased R wave voltage above 98th percentile for age in leads V5 and V6 and Q wave in lead V6 above 98th percentile for age (). Echocardiographic findings included moderate left atrial enlargement, severe mitral valve regurgitation (), and aortic valve regurgitation. The PR interval was within a normal limit of 0.120 seconds for 5-6-year-old children.\nBased on the history of preceding group A Streptococcus infection, clinical symptoms, elevated acute phase reactants, and echocardiographic findings, the patient was diagnosed with acute rheumatic fever. This patient received intramuscular penicillin G benzathine and was started on glucocorticoids for severe carditis. He also was prescribed aspirin for symptomatic management of arthritis with an initial dose at 75 mg/kg per day in divided doses every 6 hours during two days of hospitalization. The dose of aspirin at discharge was decreased to 50 mg/kg per day in divided doses every 8 hours. The patient was discharged with recommendations to continue secondary prophylaxis with penicillin G benzathine every 4 weeks for the next 10 years.

[[5.0, 'year']]

M

{'13689614': 1, '21386976': 1, '19246689': 1, '16253886': 1, '20177010': 1, '22534148': 1, '15382606': 1, '12131934': 1, '19880393': 1, '25926089': 1, '18392063': 1, '17547548': 1, '1404745': 1, '25908771': 1, '23091044': 1, '29974004': 2}

{}

6008880-1

comm/PMC006xxxxxx/PMC6008880.xml

Rupture of Hepatocellular Carcinoma after Transarterial Chemoembolization followed by Massive Gastric Bleeding

An 81-year-old man presented with abdominal discomfort and distention. His medical history was remarkable for hypertension and type 2 diabetes mellitus. He and his family denied alcohol abuse. Physical examination revealed hepatomegaly without jaundice, ascites, or hepatic encephalopathy. Laboratory data included platelet count 101,000/mm3, total bilirubin 0.9 mg/dl, AST 33 IU/L, ALT IU/L, and PT-INR 1.15. The serum AFP and PIVKA-II levels were 1081.0 ng/ml and 43 mAU/ml. Serologic tests for hepatitis B and C virus were negative. The diagnosis of nonalcoholic steatohepatitis with Child-Pugh A liver cirrhosis was made and four HCC lesions were found in segments II, VI, and VIII on the imaging. The largest one was located near the liver capsule in segment II measuring 6.5 cm in diameter. The other three lesions were less than 2 cm with one in segment VI and two in segment VIII. Segmental TACE with epirubicin and iodized oil was performed and he was discharged uneventfully.\nHe was doing well until he developed abdominal pain 15 days after TACE which was gradually getting worse emergency transport to the hospital. His temperature was 37.7°C, blood pressure 102/41 mmHg, and pulse rate 79/minute. On physical examination, the abdomen was distended and hard to palpation without rebound tenderness. His hemoglobin was 12.6 g/dl. A contrast enhanced computed tomography (CT) scan of the abdomen showed iodized oil and intraperitoneal free air with a rupture of the HCC in segment II into the peritoneal cavity adjacent to the gastric wall (). Extravasation of contrast medium was not seen. Emergent upper gastrointestinal endoscopy confirmed no gastric mucosal lesions or a site of perforation. Without evidence of septic shock or hemorrhage, surgical drainage and transcatheter arterial embolization (TAE) are considered less effective. He was treated nonoperatively with piperacillin and tazobactam. Although an abdominal abscess formed, he was discharged on hospital day 18 with continued antimicrobial therapy.\nFive weeks after undergoing TACE, he was readmitted with hematochezia and hemorrhagic shock. The hemoglobin level was dropped to 6.6 g/dl. A contrast enhanced CT scan demonstrated gastric extraluminal compression by an HCC lesion with extravasation of contrast medium into the stomach (). Emergent upper gastrointestinal endoscopy showed a submucosal tumor with central ulceration located on the anterior wall of the gastric body, corresponding to extraluminal compression by a HCC (). The hemorrhage from the ulcer was successfully controlled by hypertonic saline-epinephrine injection. Another submucosal tumor was found in the gastric fundus without ulcer formation (). The patient's condition stabilized and he was discharged for palliative care and died six weeks after undergoing TACE.

[[81.0, 'year']]

M

{'25762848': 1, '25682389': 1, '21699037': 1, '27930585': 1, '19154593': 2, '11802535': 1, '21611067': 1, '9607536': 1, '32455067': 2, '22648700': 1, '20698237': 1, '18926518': 1, '24384870': 1, '21431968': 1, '18849205': 1, '21960103': 1, '12143247': 1, '16621394': 1, '33955600': 1, '29974003': 2}

{'2647913-1': 1, '7243093-1': 1}

6008881-1

comm/PMC006xxxxxx/PMC6008881.xml

Central Pontine Myelinolysis in Pediatric Diabetic Ketoacidosis

A previously healthy 16-year-old female visitor from Japan presented to an adult emergency room (ER) with altered mental status and emesis. She was found lying on a bathroom floor in her hotel. There was a history of polyuria and polydipsia for 2 weeks and 8 to 10 kg weight loss during the previous month. Her vital signs upon arrival to the pediatric intensive care unit (PICU) were temperature of 98.1° Fahrenheit, heart rate 110/min, respiratory rate 26/min, blood pressure 140/81 mm of Hg, and 100% oxygen saturations without supplemental oxygen. Her capillary refill time was 4-5 seconds. Her weight was 57 kg. Her Glasgow Coma Scale (GCS) was 13 in the ER, which improved to 15 upon arrival to PICU. Skin rash with infected lesions was noted in her groin. The rest of her physical examination was unremarkable.\nWorkup in the ER showed hyperglycemia of 472 mg/dL, metabolic acidosis (pH 6.75, pCO2 18.4, pO2 149, HCO3 2.5, base deficit 32.6, anion gap 23.5), ketosis (beta-hydroxybutyrate 11.41), glucosuria, and ketonuria, which were consistent with diabetic ketoacidosis. Her white cell counts were 22.3 k/L, hemoglobin 15.8 g/dL, and hematocrit 47%. The rest of her workup was unremarkable.\nThe patient received fluid resuscitation with 30 mL/kg of 0.9% normal saline (NS) and 50 mEq of sodium bicarbonate in the ER. Continuous insulin infusion was started at 0.1 unit/kg/hr. After that she was started on intravenous fluids containing 0.45% saline and 75 mEq/L of sodium bicarbonate. This was administered at 150 mL/hr, which was 1.25 times the usual daily maintenance requirement for her weight. A consultation with our PICU was obtained at this stage. No further bicarbonate boluses were given. Her fluids were changed to isotonic fluid with potassium phosphate and potassium chloride and without any bicarbonate. It was administered at the rate of 1.5 times maintenance of daily requirement for weight. She was transferred to the PICU at this stage, where she continued to exhibit severe metabolic acidosis with pH 6.97, pCO2 26.7, HCO3 6.1, and base deficit of 24.5. The DKA management was continued with close monitoring and serial laboratory evaluations.\nAfter few hours of her arrival in the PICU, the patient became disoriented and confused. She was given one time 5 ml/kg of 3% hypertonic saline (HS) due to concern for cerebral edema and she responded well. Four hours later she developed lethargy followed by apnea. She was given additional doses of 3% HS bolus (5 mL/kg) and 0.8 g/kg of 20% mannitol. The hyperosmolar therapy was effective but only transiently; the patient eventually required intubation and mechanical ventilation for recurrent apnea.\nCT head obtained at that time showed a thin, right parietal subdural hemorrhage without any evidence of edema or mass effect. Of note, CT scan was obtained after the initiation of hyperosmolar therapy.\nHyperosmolar therapy was continued afterwards with 3% HS and 20% mannitol. It was guided with close monitoring of renal function including serum sodium and serum osmolarity. The doses and other parameters were maintained per standard of care [, ].\nThe patient's serum sodium was 135 upon arrival in the ER and 143 at around 11 hrs before the initiation of hyperosmolar therapy and reached to 160 at 71 hrs when hyperosmolar therapy was discontinued. It was normalized to 143 at 131 hrs of admission. Her osmolarity was 298, 305, 335, and 299 at those timings (sodium in mmol/L and osmolarity in msom/kg). The rate of glucose reduction was < 50 mg/dl/hr.\nMRI brain obtained at 60 hrs of admission showed mild edema of the cortex and sulci and diffuse edema of the pons and midbrain with restricted diffusion in the pons consistent with central pontine myelinolysis also known as osmotic demyelination syndrome. There was no significant change in the subdural hemorrhage (Figures –). MR angiogram showed no evidence of vessel abnormality.\nThe patient required continued mechanical ventilation and further management in the PICU. She became more responsive on day 5, and her support was gradually weaned until extubation on day 7. At that time she exhibited only mild residual dysmetria, trivial ataxia, mild left sided weakness, and neglect. All deficits subsequently improved.\nHer course was complicated by an isolated focal seizure that responded to standard antiepileptic medication. She also developed bilateral upper extremity deep venous thromboses (DVT). Hematology workup showed no underlying hypercoagulability. Thus the DVT was considered as another complication of DKA [–].\nHer overall hospital length of stay was 14 days, and by the date of discharge she was able to ambulate with a walker and speak and eat without difficulty.

[[16.0, 'year']]

F

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{}

6008890-1

comm/PMC006xxxxxx/PMC6008890.xml

Intra-Abdominal Hemorrhage following Cardiopulmonary Resuscitation: A Report of Two Cases

A previously healthy 29-year-old female was being transferred to the “Sismanoglio” General Hospital of Athens, Greece, due to pulmonary embolism (PE) 24 hours following a caesarian section. She had already received a bolus of intravenous 5000 E Heparin. The patient was hemodynamically unstable (blood pressure= 90/40 mmHg, heart rate= 140 beats/min, saturation= 85%, and breathing rate= 30 breaths/min) and afebrile (37 C). During the transportation, she suddenly went into cardiopulmonary arrest with pulseless electrical activity. CPR was immediately instituted according to advanced life support guidelines. Manual chest compressions and artificial ventilation were performed. The CPR was effective and the patient was then transferred to the Intensive Care Unit (ICU). About 3 hours later, she continued being hemodynamically unstable, her hematocrit had dropped 10 points (from 33% at ICU admission to 23% at that point), and she had a distended abdomen. She was transfused with two blood units and an emergency Diagnostic Peritoneal Lavage (DPL) was positive for intra-abdominal bleeding. At that point she was urgently taken into the operating theatre. An exploratory laparotomy was decided upon. About two liters of blood was removed from the abdomen. The intraoperative finding was an approximately 5 cm liver laceration at the left lobe, near the attachment point of the left coronary ligament. Liver suturing was performed and the intra-abdominal bleeding was successfully controlled. Unfortunately the patient passed away 3 hours after the operation. Cause of death was the massive pulmonary embolism.

[[29.0, 'year']]

F

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{'6008890-2': 2, '7751573-1': 1, '7751573-2': 1, '5644815-1': 1}

6008890-2

comm/PMC006xxxxxx/PMC6008890.xml

Intra-Abdominal Hemorrhage following Cardiopulmonary Resuscitation: A Report of Two Cases

A previously healthy 62-year-old female was transferred with an ambulance to the emergency department due to drowning at the sea. CPR had been instituted in situ by an experienced lifeguard. At presentation the patient was hemodynamically unstable (blood pressure= 100/55 mmHg, heart rate= 135 beats/min, and breaths= 25 per min) and afebrile (36.7 C). Her laboratory investigation showed hematocrit (Hct)= 23% and hemoglobin (Hgb)= 7.3 g/dL. Fluid resuscitation was performed and she was also transfused with 2 blood units, but she continued being hemodynamically unstable. After the transfusion she had Hct= 19% and Hgb= 6.4 g/dL. The abdominal ultrasonography revealed free intra-abdominal fluid. Bedsides, chest X-ray was negative for tympanic abdomen or pneumoperitoneum. At that point an exploratory laparotomy was decided upon. The site of bleeding was found to be vessels originating from the lesser curvature of the stomach, which were successfully ligated. The patient had an uneventful recovery and was discharged on the 12th postoperative day. Eight years after the event, she continues her everyday activities, without any limitations.

[[62.0, 'year']]

F

{'4604258': 1, '20956221': 1, '28092783': 1, '17640792': 1, '20064683': 1, '7458835': 1, '24487704': 1, '25771500': 1, '33364811': 2, '24996708': 1, '29057186': 2, '3608599': 1, '28122604': 1, '22942628': 1, '23474390': 1, '29974000': 2}

{'6008890-1': 2, '7751573-1': 1, '7751573-2': 1, '5644815-1': 1}

6008935-1

comm/PMC006xxxxxx/PMC6008935.xml

A rare case of perivascular epithelioid cell tumor (PEComa) of the greater omentum

A 49-year-old Japanese woman underwent an MRI examination in the referring hospital for an assessment of the orthopedic disease of her right hip joint, and the MRI revealed a tumor in her pelvis. She was referred to our hospital, where an MRI examination again showed tumor mobility (Fig. ), and a tumor derived from the intestinal tract was suspected.\nIn the contrast CT image (Fig. ), a mass approx. 45 mm in size was found on the right side of the pelvic floor, and in the blood vessel construction image, the tumor was nourished by vessels from the splenic artery to the greater omentum. In 18F-fluoro-2-deoxyglucose positron emission tomography/computed tomography (18F-FDG-PET/CT), no abnormal accumulation was observed (Fig. ). The results of complete blood cell counts and biochemical tests were all normal. Cancer antigen 19-9 (CA19-9) showed a mild elevation at 37.8 U/mL (normal range < 37.0 U/mL), and carcinogenic embryonic antigen (CEA) was normal at 0.8 ng/mL (normal range < 5.0 ng/mL). Based on all of these results, the preoperative diagnosis was a suspected omental primary tumor or accessory spleen.\nThere was a danger of torsion irrespective of the presence or absence of malignancy, and the patient desires surgery to remove the tumor; the surgery was conducted concurrently with the diagnosis and treatment. The surgery was a single-incision laparoscopic-assisted greater omental tumor resection. A longitudinal incision was made approx. 4 cm around the umbilicus. A multichannel port (x-Gate®, Sumitomo Bakelite, Tokyo) was inserted in the wound. Observation of the abdominal cavity revealed a red and solid tumor mass of approx. 4 cm. Adhesion with the surrounding tissue was not observed. The greater omentum and the tumor were guided out of the body through the gate hole (Fig. ), and the tumor was excised together with the greater omentum. The tumor, a light brown tinged solid mass with a smooth border, was 5.2 × 3.8 × 3.5 cm in size with a well-defined border (Fig. ).\nHistopathologically, the tumor involved large and small blood vessels (Fig. ), and tumor cells with eosinophilic cytoplasm were increased in number, in sheet form (Fig. ). In immunohistochemical staining, the tumor cells were found to be positive for HMB45 (Fig. ), Melan-A (Fig. ), and α-SMA (Fig. ), but negative for CD34, Desmin, c-kit, and s-100.\nBased on the above results, the tumor was diagnosed as an omental PEComa. There has been no recurrence at 16 months after the surgery.

[[49.0, 'year']]

F

{'16327428': 1, '24450662': 1, '32257249': 2, '20975340': 1, '24061520': 1, '34510959': 1, '24401515': 1, '26139507': 1, '16167542': 1, '28349318': 1, '25037302': 2, '31933841': 1, '18971775': 1, '27535361': 2, '34621837': 2, '25121029': 1, '27559437': 1, '18277881': 1, '31933807': 1, '16398680': 1, '15992696': 1, '9866769': 1, '26144278': 1, '29921303': 2}

{'7104191-1': 1, '8462230-1': 1, '4989355-1': 1, '4102875-1': 1}

6008944-1

comm/PMC006xxxxxx/PMC6008944.xml

Innovative Uses of Condom Uterine Balloon Tamponade for Postpartum Hemorrhage in India and Tanzania

A 26-year-old primigravida at 39-week gestation by date of last menstrual period, presented to the Muhimbili National Referral Hospital, Tanzania, in active labor. She had no significant past medical history and an uncomplicated antenatal course. Shortly after midnight she had a precipitous vaginal delivery. After delivery, the woman began hemorrhaging profusely. Her uterus was vigorously massaged, ten international units (IU) of intravenous oxytocin were administered, and the placenta manually removed. Despite the interventions, the patient continued to bleed and lost consciousness. On pelvic examination, the uterus was well contracted, bleeding superior to her cervix was minimal, and second degree bilateral vaginal sulcal lacerations were identified. Fluid resuscitation was initiated, and the patient was emergently taken to the operating theatre by the in-house Obstetrician and Gynecology resident for examination under anesthesia and repair of her vaginal lacerations. Despite attempts at suture repair, hemorrhage from the laceration sites continued. The senior Obstetrician and Gynecology consultant was also unsuccessful in gaining control of the hemorrhage and therefore placed an ESM-UBT device into her vagina. The ESM-UBT device was inflated with 300 cc of water and secured with vaginal packing. Hemorrhage ceased and antibiotics, intravenous fluids, and blood products were administered. The ESM-UBT device was removed after 48 hours, no further repair was necessary, and bleeding did not recur. The woman was discharged home on postpartum day two and at her six-week postpartum visit had fully recovered.

[[26.0, 'year']]

F

{'26223284': 1, '17362483': 1, '19155956': 1, '15411151': 1, '16234162': 1, '32293311': 2, '5070147': 1, '6650623': 1, '28675419': 1, '29967704': 2}

{'6008944-2': 2, '6008944-3': 2, '7161190-1': 1}

6008944-2

comm/PMC006xxxxxx/PMC6008944.xml

Innovative Uses of Condom Uterine Balloon Tamponade for Postpartum Hemorrhage in India and Tanzania

A 22-year-old gravida two para one woman, six-week pregnant by dating from her last menstrual period, presented to a private maternal health facility for termination of her pregnancy via dilation and suction curettage. Upon attempted cervical dilation the woman began hemorrhaging profusely. She was referred by ambulance to the Mahatma Gandhi Institute for Medical Sciences where she presented extremely pale, weak, and in shock with vital signs including a blood pressure of 80/30 mm/Hg and heart rate of 145 bpm. Outside imaging that was brought with the patient, when reviewed at the referral facility, clearly identified a cervical pregnancy. The patient was emergently taken to the operating theatre where resuscitation was initiated, and a rapid pelvic exam was performed. Examination revealed severe bleeding and a swollen cervix ballooned with clots. An ESM-UBT device was intentionally placed into the cervix, inflated with 200 cc of water which formed a dumbbell, filling the cervix and ballooning into the lower uterine segment and vagina. Vaginal packing was used to secure the uterine balloon's position. The woman was transferred to the intensive care unit where she received isotonic fluids and two units of blood. The ESM-UBT device was removed after 24 hours without any resumption of bleeding. There were no complications on six-week follow-up.

[[22.0, 'year']]

F

{'26223284': 1, '17362483': 1, '19155956': 1, '15411151': 1, '16234162': 1, '32293311': 2, '5070147': 1, '6650623': 1, '28675419': 1, '29967704': 2}

{'6008944-1': 2, '6008944-3': 2, '7161190-1': 1}

6008944-3

comm/PMC006xxxxxx/PMC6008944.xml

Innovative Uses of Condom Uterine Balloon Tamponade for Postpartum Hemorrhage in India and Tanzania

A 27-year-old gravida three para two woman, 23-week pregnant by date from her last menstrual period, presented to the Government Medical College Nagpur National Hospital, India, with vaginal bleeding. She had no significant past medical history. On physical examination, her fundal height was consistent with a 32-week uterus, there was moderate vaginal bleeding, and her cervix was closed. An ultrasound examination was remarkable for absence of a fetus and a solid collection of echoes with numerous anechoic spaces consistent with a molar pregnancy. The patient was taken to the operating theatre for cervical dilation and evacuation under general anesthesia. Following evacuation, the uterus was atonic and hemorrhage ensued with an estimated blood loss of greater than two liters. Bimanual uterine massage was performed, the bladder was drained, fluid resuscitation was initiated, and oxytocin (20 IU) and ergometrine (0.4 mg) were administered intravenously. Despite these interventions, profuse bleeding continued, and her vital signs deteriorated to a blood pressure of 84/60 and heart rate of 120. An ESM-UBT device was inserted into the uterus, inflated with 300 cc of water, and hemorrhage was immediately arrested. Her condition stabilized, and she was transfused blood. The ESM-UBT device was removed after 24 hours with no bleeding recurrence and the woman was discharged home two days thereafter. Pathology results confirmed a complete molar pregnancy. There were no complications on subsequent two- and six-week follow-up visits and she remains within their system for clinical monitoring throughout the year.

[[27.0, 'year']]

F

{'26223284': 1, '17362483': 1, '19155956': 1, '15411151': 1, '16234162': 1, '32293311': 2, '5070147': 1, '6650623': 1, '28675419': 1, '29967704': 2}

{'6008944-1': 2, '6008944-2': 2, '7161190-1': 1}

6008945-1

comm/PMC006xxxxxx/PMC6008945.xml

Isolated Polycystic Liver Disease: An Unusual Cause of Recurrent Variceal Bleed

A 67-year-old Caucasian female patient presented to the hospital with one day history of hematemesis and melena. She had two episodes of bright red emesis and four episodes of dark colored stools the day she presented, followed by a brief syncopal episode lasting for 10-15 seconds. She also complained of epigastric pain. She was diagnosed with polycystic liver disease and portal hypertension one year ago when she presented similarly with hematemesis and melena. At that time, she had an esophagogastroduodenoscopy (EGD) which showed esophageal varices and underwent banding. She denied any history of alcohol use, NSAID ingestion, or peptic ulcer disease. She was gravida 3, para 3, and had tubal ligation following her last pregnancy. She denied use of oral contraceptive pills or hormonal replacement therapy in the past. Her family history was significant for cystic liver disease and chronic liver failure in mother and maternal aunt.\nAt the time of presentation, she was afebrile (temperature of 98.7°F), tachycardic (heart rate of 110 beats/min), tachypneic (respiratory rate of 18/min), and hypotensive (blood pressure of 97/60 mmHg). Cardiopulmonary examination was normal. Abdominal examination showed mildly distended abdomen with epigastric and right upper quadrant tenderness, and bowel sounds were normal. Extremities showed bilateral 1+ pitting edema. Initial laboratory work showed hemoglobin of 7.7 g/dl, hematocrit of 24%, white blood count of 10.8 cells/mm3, and platelet count of 162 cells/mm3. Metabolic panel showed nonanion gap metabolic acidosis with serum bicarbonate of 17 mEq/L, normal serum BUN, and creatinine of 23 mg/dl and 0.57 mg/dl, respectively. Liver panel showed total bilirubin of 2.2 mg/dl, direct bilirubin of 0.4 mg/dl, albumin of 2.5 g/dl, alkaline phosphatase of 51U/L, AST of 22 U/L, and ALT of 16 U/L. Gamma-glutamyltransferase was measured to be 27 U/L and prothrombin time (INR) was measured to be 1.46.\nShe was aggressively resuscitated with fluids, pressors, and packed red blood cell (PRBC) transfusion. Octreotide and proton pump inhibitor infusions were started. Patient was electively intubated for hemodynamic instability and altered mental status. An EGD was done which showed four columns of large esophageal varices with red wale sign showing stigmata of high risk for bleeding and type 1 gastroesophageal varices (). Four bands were successfully applied starting from cardia up to midesophagus. A computed tomography (CT) of abdomen showed multiple cysts in the liver with evidence of portal hypertension in form of splenomegaly, ascites, and esophageal varices (). Liver surface was smooth and no thrombus was identified in hepatic veins and inferior vena cava. Bilateral kidneys were normal with no evidence of renal cysts (). Vital signs and hemoglobin were monitored closely. Patient was successfully extubated next day and pressors were weaned off. Patient did not have any further episode of hematemesis but continued to have melena. She slowly dropped her hemoglobin requiring transfusions with PRBC. An EGD was repeated which reveled 3 more columns of esophageal varices with intact bands from previous EGD. Four more bands were placed on remaining varices. Following repeat EGD patient's hemoglobin remained stable for rest of her hospital stay. During her hospital stay, she also underwent therapeutic paracentesis with removal of 330 ml of peritoneal fluid for worsening ascites (). Peritoneal fluid analysis showed serum ascites albumin gradient (SAAG) of more than 1.1 g/dl, normal white cell count, negative gram stain, and cultures. She was started on propranolol, furosemide, and spironolactone. She was discharged home with recommendations to follow up in outpatient clinic with a plan of repeat EGD in 3-4 weeks.

[[67.0, 'year']]

F

{'21191289': 1, '7890219': 1, '16614155': 1, '9393161': 1, '20219621': 1, '21105111': 1, '10821902': 1, '23109444': 1, '11535774': 1, '15133510': 1, '24886261': 1, '31505092': 1, '18199797': 1, '12529853': 1, '34754699': 2, '22773240': 1, '29971171': 2}

{'8571733-1': 1}

6008950-1

comm/PMC006xxxxxx/PMC6008950.xml

Duodenal Pyloric Gland Adenoma in a 59-Year-Old Asian Male

A 59-year-old Asian male presented to the gastroenterology office for a follow-up of a previously found duodenal lesion. He had an EGD at an outside facility 2 years earlier for evaluation of abdominal pain which showed a 1.2 cm duodenal polypoid lesion in the second portion of the duodenum. Pathology of the polyp revealed heterotopic gastric mucosa with benign lobules of gastric glands. He also was found to have chronically active H. pylori gastritis with numerous Helicobacter organisms and atrophic gastritis with patchy areas of intestinal metaplasia. At that time, he was instructed to follow up for a repeat endoscopy for surveillance in two years. As recommended, two years later he presented to our office for surveillance of his duodenal lesion. Due to his history of atrophic gastritis and a large duodenal lesion, we performed an upper endoscopy that showed atrophic gastritis, duodenitis, and a single 10 mm polyp in the second portion of the duodenum. The duodenal polyp was biopsied. Pathology revealed polypoid gastric metaplastic mucosa with focal epithelial atypia including nuclear enlargement, stratification, nucleoli, and few mitoses. The patient was referred for endoscopic ultrasound to further characterize the lesion and complete resection. Several weeks later he underwent an endoscopic ultrasound that revealed a 13-mm pedunculated and sessile polyp on the lateral wall opposite to the major papilla in the second portion of the duodenum (). The polyp was removed en bloc using a hot snare following a 4-mL saline lift. Two endoclips were placed for hemostasis. Pathology revealed a pyloric gland adenoma (Figures , , and ) with predominantly low grade dysplasia (Figures and ) and some small areas of focal high grade dysplasia. The slides were read by the in-house pathologist and were also reviewed by a GI expert pathologist at the University of Michigan. The patient was recommended to follow up for surveillance upper endoscopy in 6 months due to the areas of high grade dysplasia found on pathology. He was lost to follow-up for over one year. Fifteen months after the PGA was removed, the patient did follow-up for a surveillance upper endoscopy. A duodenal scar was found at the site of the PGA and a biopsy was taken from the site. There was no evidence of residual pyloric gland adenoma. The patient was then recommended to follow up for surveillance endoscopy in 5 years in accordance with the ASGE guidelines for surveillance of gastric adenomatous polyps.

[[59.0, 'year']]

M

{'795617': 1, '18830123': 1, '16564854': 1, '12715167': 1, '29971172': 2}

{}

6008951-1

comm/PMC006xxxxxx/PMC6008951.xml

Candida glabrata: A Unique Cause of Necrotizing Urethritis

The patient is a 56-year-old man with a past medical history significant for poorly controlled insulin-dependent type 2 diabetes mellitus, hypertension, hypothyroidism, and meatal stenosis with incomplete bladder emptying who presented the University Medical Center emergency room with a seven-day history of perineal pain and fever. One week prior to presentation, he was seen in the emergency room with the same symptoms and was placed on a 14-day course of ciprofloxacin for a suspected urinary tract infection. Also, the patient was admitted to the medical intensive care unit 10 months previously for severe sepsis secondary to Candida glabrata urinary tract infection with candidemia. The patient denied penile trauma, gross hematuria, and dysuria. Upon presentation, he was hemodynamically stable with a blood pressure of 145/77 mmHg, and he had mild tachycardia with a heart rate of 110 beats per minute and a fever of 101.4 F. On physical exam, the patient had mild tenderness to palpation at the penoscrotal junction with induration, no crepitus was palpated, and there were no skin changes. His laboratory values were significant for an elevated white blood cell count (WBC) of 20,000/μL, a creatinine level of 1.4 mg/dL, and a hemoglobin A1c level of 9.4%, and all other values were within normal limits. On computerized tomography (CT) scan, emphysematous changes were noted with in the ventral portion of the penile shaft with air at the bulb of the corpus spongiosum (Figures and ).\nThe patient had a postvoid residual of 320 ml; thus, a transurethral Foley catheter was placed with withdrawal of 700 ml of clear yellow urine which was sent for culture. The patient was begun on intravenous (IV) meropenem, fluconazole, and daptomycin. Repeat CT scan on hospital day 1 was largely unchanged, and the patient remained febrile to 102.3 F. On hospital day 2, meropenem and daptomycin were discontinued, and the patient was switched to piperacillin/tazobactam based on recommendations from the Infectious Disease team. A pelvic magnetic resonance imaging (MRI) with and without gadolinium was obtained at this time which showed reduced blood flow to the corpus spongiosum compared to the corpus cavernosum on T1-weighted imaging with gadolinium ().\nThe patient was then taken to the operating room on hospital day 3 due to worsening penile pain and induration, a persistently elevated white blood cell and continued fever. On cystourethroscopy, the urethral mucosa appeared dusky from the anterior to the membranous urethra. A longitudinal incision was made in the perineum, the urethra was palpated and noted to be indurated and firm, and thus the bulbospongiosus muscle was split and a longitudinal incision was made in Buck's fascia with expulsion of approximately 10 ml of purulent fluid which was sent for culture and gram stain. The remaining necrotic spongiosal tissue was debrided with blunt dissection, a penrose drain was placed within the defect, and the skin was closed loosely. On postoperative day 2, his white blood count decreased to 13,000/μL and he remained afebrile for 48 hours. The patient's intraoperative wound culture was found to be positive, and using automated Matrix Assisted Laser Desorption Ionization Time of Flight (MALDI-TOF) mass spectrometry, the organism Candida glabrata was identified by our microbiology laboratory. The wound culture result was identical to his urine culture. The patient was then switched to micafungin based on the Infectious Disease team's recommendations. He was subsequently discharged to home on postoperative day 14 on a 14-day course of oral voriconazole with the transurethral Foley in place.

[[56.0, 'year']]

M

{'452226': 1, '23995534': 1, '18324496': 1, '21895858': 1, '9880475': 1, '24198996': 2, '26171271': 1, '19153361': 1, '18923769': 1, '29971173': 2}

{'3808107-1': 1}

6009011-1

comm/PMC006xxxxxx/PMC6009011.xml

An Interesting Case and Literature Review of a Coronary Stent Fracture in a Current Generation Platinum Chromium Everolimus-Eluting Stent

The following case is of a 66-year-old man with a complicated cardiac history, who for some time has been experiencing limiting angina despite being on maximally titrated medical therapy of aspirin, prasugrel, carvedilol, ranolazine, isosorbidemononitrate (sustained release), atorvastatin, lisinopril, nitroglycerin patch, and as needed sublingual nitroglycerin. His cardiac history includes extensive coronary artery disease (CAD). \nStatus post coronary artery bypass graft 15 years ago (CABG: reverse saphenous vein graft to first and second obtuse marginal arteries in a sequential fashion and reverse saphenous vein graft to posterior descending artery and posterolateral artery in a sequential fashion) Status post redo CABG 2 years ago (CABG: left internal mammary artery [LIMA] to the left anterior descending artery [LAD]) Status post multiple stents\nHe also has a history of hypertension, diabetes (on metformin), dyslipidemia, and prior bilateral carotid endarterectomy. He was evaluated by cardiothoracic surgery 8 months prior, and they recommended against reoperation.\nAbout 4 months prior, he underwent another coronary angiography. This angiogram showed a relatively long 95% stenosis in the mid-LAD. The stenosis extended from the mid-LAD to a bit passed the LIMA anastomosis, with retrograde flow into the LIMA (). Some tenting of the LAD at the anastomotic site was also noted. Several predilations were performed at 8 and 10 atmospheres with no significant improvement to the stenosis. A 2.25 × 28 mm Synergy drug-eluting stent (Boston Scientific) was then deployed at 12 atmospheres and postdilated with 2.25 × 20 mm noncompliant balloon at 14 atmospheres. The stent was successfully deployed in the mid-LAD with no residual stenosis. A slight kink in the distal one-third of the stent at the LIMA insertion point was noted (). further demonstrates the movement of the intact stent during diastole and systole. The patient's symptoms temporarily improved.\nA short time after, he presented again with rest pain. His vital signs were stable. Physical examinations, including cardiac exams, were unimpressive. Electrocardiogram (EKG) showed normal sinus rhythm without significant ST/T changes. Two troponin levels drawn 12-hour apart were both normal. Repeat coronary angiography was performed. It revealed a mid-LAD stent fracture with 70% in-stent restenosis (fractional flow reserve of 0.78) (). Coronary intervention was performed and a Xience Alpine DES was deployed inside the fractured Synergy DES with TIMI III flow and no residual stenosis (). The patient tolerated the procedure and was discharged in stable condition.

[[66.0, 'year']]

M

{'28582037': 1, '23011266': 1, '26985210': 2, '19477386': 1, '27499060': 1, '23818756': 1, '14696169': 1, '34908931': 1, '24720422': 1, '20920641': 1, '22431372': 1, '21939948': 1, '28923786': 1, '26138831': 1, '27853098': 1, '18508140': 1, '19909872': 1, '12192018': 1, '29967699': 2}

{'4791650-1': 1}

6009022-1

comm/PMC006xxxxxx/PMC6009022.xml

Lipschütz Genital Ulceration as Initial Manifestation of Primary Sjögren's Syndrome

A 26-year-old-woman, with history of labial herpes and asthmatic bronchitis, presented with bilateral retroocular pain, odynophagia, fever, vaginal discomfort and vulvar ulcers. The ulcers continued to progress despite treatment with nonsteroidal anti-inflammatory drugs and valacyclovir, prescribed at the primary healthcare site. Two days later, after initial consultation at our emergency room, she was admitted immediately at the Vulvar Clinic of our institution, with increasing vulvar pain, without other symptoms. Physical examination of the vulva showed extended vulvar oedema and kissing pattern ulcers on labia minora and majora, vagina and cervix (). Inguinal lymph nodes were also bilaterally swollen. The patient denied the use of other medications and sexual activity in more than 6 months. First blood tests only showed C-reactive protein 12.35 mg/dL (normal: <0.5 mg/dL). Serologies for herpes virus 1 and 2, Ebstein-Barr virus (EBV; IgG+), cytomegalovirus, mycoplasma pneumoniae, parvovirus B19, toxoplasmosis, rubella, hepatitis, human immunodeficiency virus, and syphilis (using the Venereal Disease Research Laboratory test) were negative. After this, a multidisciplinary approach was performed. When directly asked, she complained about mild eye dryness and she often felt a discomfort of dry mouth. There was no familial history of autoimmune diseases, but her father had some episodes of oral aphthosis. Immunological examination was positive for rheumatoid factor (RF – 22.3 UI; normal: <15 UI), anti-nuclear antibodies [ANA (speckled, titer 1:320)] and antibodies to SSA/Ro (SSA 3+/ Ro52KD 3+)—initial screening step of ANA by indirect immunofluorescence on HEp-2 cells (Euroimmun ®, Germany); autoantibodies confirmation assay by line immunoblot (ANA profile 3 - Euroimmun ®, Germany)—antibodies detected on strips were evaluated semiquantitatively (negative, 1+, 2++, and 3+++). Anti-SSB/La, anti-RNP, anti-Sm, anti-dsDNA, antineutrophil cytoplasmic, anticardiolipin, and anti-beta(2)-glycoprotein1 antibodies were negative. Serum C3 level was 1.79 g/L (normal: 0.9-1.8 g/L) and C4 level was 0.31 g/L (0.1-0.4 g/L). Immunoglobulins (IgG, IgM, IgA) were measured and a high IgG level was found (20.50 g /L; normal: 7-16 g/L). Erythrocyte sedimentation rate was also high (45 mm/h; normal: <16 mm/h). Lupus anticoagulant and HLA-B27 were both negative. Ophthalmological evaluation was refused by the patient. Labial salivary gland (LSG) biopsy revealed focal lymphocytic sialadenitis (FLS), with a focus score (FS) =1 (per 4 mm2) obtained by four LSGs (3-5 mm). No other histopathological features were reported. The patient was diagnosed as having pSS on the basis of dry eyes and dry mouth, positive anti-SSA/Ro antibody, and typical histopathologic abnormalities on LSG biopsy. Prednisolone 20 mg/day was prescribed and vulvar healing appeared within 2 weeks with partial loss of left labia ().

[[26.0, 'year']]

F

{'16966020': 1, '22622341': 1, '27532148': 1, '26297242': 1, '19643667': 1, '27965259': 1, '26691952': 1, '26231345': 1, '20462661': 1, '25295212': 2, '21480190': 1, '29967703': 2}

{'4175910-1': 1}

6009024-1

comm/PMC006xxxxxx/PMC6009024.xml

Dislodged Bonded Molar Tube into Wound during Orthognathic Surgery

A 25-year-old gentleman presented to our clinic with a complaint of recurrent pain and swelling on his right cheek of three-month duration. He visited a general practitioner each time, and the condition was resolved with analgesic and antibiotics. However, his symptoms got worse and he attended our Oral Surgery Clinic for consultation.\nThe patient is a fit and healthy young man with no relevant medical history and no known history of allergy. Past surgical history revealed that he had underwent bimaxillary orthognathic surgery one and half year earlier in a local hospital. Although the postoperative period was uneventful, the surgical team informed him that there was a dislodged orthodontic appliance in his right cheek that must have occurred during the operation. The team explained to the patient that this accident was realized later on the next day after the surgery when the molar tube from the right maxillary second molar was found missing, and its presence was confirmed high up in the right maxillary-zygomatic buttress area shown in the postoperative X-ray image taken on the next day following the surgery. A series of further postoperative radiographs confirmed its location, lying outside the right maxillary antrum. Due to the pronounced postoperative facial oedema at that time, no attempt was made to remove the appliance. The absence of sign and symptoms during further follow-up sessions confirmed the decision to leave it in-situ with continuous clinical observation.\nOn examination, there was no extraoral swelling noted. The mandible and maxilla seemed firm indicating good healing following previous mandibular saggital split and maxillary Le Fort I osteotomy sites and a stable class I dental occlusion. Intraorally, there was a sinus with slight pus discharge on the upper right buccal sulcus region adjacent to the upper right first premolar. All teeth in that quadrant were firm and vital. Tenderness was elicited upon palpation on the upper right vestibular region. We suspected the sinus track may originate from the dislodged appliance embedded in the cheek soft tissue. A periapical view was then taken with gutta-percha inserted into the sinus for foreign body localization purpose. The radiograph revealed the gutta-percha pointed towards the site of titanium plate and screws placed used for rigid fixation, and with the molar orthodontic tube appliance in its vicinity (). A cone beam CT was performed to provide a 3D detailed location of the appliance (Figures and ) and confirmed it to be located outside the maxillary antrum.\nThe presence of the molar orthodontic tube foreign body reaction was suspected as the most probable cause of the recurrent right cheek pain and swelling associated with an intraoral discharging sinus. Exploration of the site was performed through the sulcular incision under general anesthesia. The dislodged molar tube was identified lying on the zygomatic bone just beneath the raised flap. It was removed by dividing some surrounding fibrous tissue strands. Just below it, one titanium straight bone plate with four screws used for fixing the previous Le Fort I osteotomy site was inspected and found to be rigidly embedded in normal bone. However, a decision was made to remove them based on the fact that they are present in an infected area. (). The Le Fort I osteotomy site showed good healing with new bone formation. Patient had an uneventful recovery thereafter, and the orthognathic surgical team who attended him previously was informed of his progress.

[[25.0, 'year']]

M

{'28217303': 1, '23631975': 1, '21353938': 1, '17545294': 1, '26008665': 1, '18675211': 1, '17545300': 1, '23814204': 1, '22971264': 1, '16125398': 1, '10358250': 1, '3057951': 1, '29971169': 2}

{}

6009035-1

comm/PMC006xxxxxx/PMC6009035.xml

Robotic enucleation for pediatric insulinoma with MEN1 syndrome: a case report and literature review

A 9-year-old girl was admitted due to repeated loss of consciousness, concomitant with a pale face, palpitations, and convulsions, which had persisted for 2 years and had been aggravated during the previous 2 months. These symptoms occurred automatically. The patient denied experiencing any sweating, nausea, vomiting, trembling, or an obvious sense of hunger before meals. The patient was previously misdiagnosed with epilepsy in another hospital, but no abnormal findings were detected on a 24-h electroencephalogram at our hospital. Her abdominal perfusion CT showed a highly perfused nodule within the pancreatic tail; A magnetic resonance scan confirmed the location of this nodule and indicated that its size was 11.6 × 13.2 mm (Fig. -). Additionally, 68Ga-exendin 4 PET-CT showed a region in the pancreatic tail with abnormally high metabolism and overexpression of the glucagon-like peptide-1 receptor (Fig. -). Lab testing showed a low fasting blood glucose (BG) of 2.2 mmol/L (reference range: 3.9–6.1 mmol/L), a high proinsulin level of 4455.9 pg/mL (reference range: 30–180 ng/mL), a normal C-peptide level of 2.56 ng/mL (reference range: 0.8–4.2 ng/mL), a serum insulin level of 15.35 μIU/mL (reference range: 5.2–17.2 μIU/mL), and a gastrin level of 92.6 pg/mL (reference range: < 100 pg/mL). These results confirmed a diagnosis of insulinoma. Imaging examination showed no abnormalities indicative of parathyroid adenoma or malignancy in the pituitary or adrenal glands. Lab testing showed normal levels of parathyroid hormone (PTH), blood calcium, phosphate, follicle-stimulating hormone (FSH), growth hormone (GH), prolactin (PRL), adrenocorticotropic hormone (ACTH), 24-h urinary free cortisol (24 hUFC), and serum cortisol. Her luteinizing hormone (LH) level was 0.24 IU/L (reference range: 2.12–10 IU/L during the follicular phase), which was considered related to her age.\nPreoperative preparation: To avoid recurrent symptoms and to maintain her fasting BG at a tolerably low level, the patient was given regular snacks before bedtime. BG can be controlled at a level between 50 and 60 mg/dL preoperatively.\nSurgical procedure: The patient underwent minimally invasive insulinoma enucleation surgery under the Da Vinci robot-assisted system with intraoperative ultrasound (IOUS) connected. The patient was put in a head-low, feet-high and left-lateral position. The robotic system was positioned at the head of the patient, while the assistant surgeon stood between the patient’s legs. Abdominal exploration via laparoscopy was conducted, and no obvious abnormalities were found. The robotic lens and operating arms were docked. The gastrocolic ligament was dissected with an ultrasonically activated scalpel. The head of the pancreas was exposed by grasping the colon downward and lifting the stomach. Towards the tail of the pancreas, we separated and exposed the spleen. The surgeon then controlled the ultrasound probe, exploring the tumor from the pancreatic tail to the head and the uncinated process with the assistance of a prograsp clamp. A quasi-circular, hypoechoic lesion was found at the end of the pancreas with a diameter of approximately 10 mm and a clear boundary. We marked the normal pancreatic tissue around the lesion with an electrotome, and while dividing the pancreas sequentially, suction was used continually to visualize the tumor capsule. Precise positioning was achieved using IOUS, and the tumor was completely resected along the capsule (Fig. -). A peritoneal drainage tube was placed. The surgery went well, lasting 65 min (skin to skin), and the volume of intraoperative bleeding was 5 mL. Intraoperative BG is documented in Table .\nAfter surgery, the patient was given liquid diet on POD2. The drain was clean and was removed on POD4, and the patient gradually resumed her normal diet. She was discharged to home on POD6. During the following 1.5 years, the patient had no recurrence of the disease. No postoperative complication occurred, such as pancreatic fistula or pancreatic function deficiency.\nPathological examination showed that the tumor was a pancreatic neuroendocrine tumor (Grade 2 with a Ki-67 index of 4%) (Fig. -). This tumor was positive for CgA, Syn, and AE1/AE3 (Fig. -). Insulin staining was partially positive (Fig. ), while gastrin, glucagon, and somatostatin staining were negative (Fig. -).\nSanger-directed sequencing for the MEN1 gene mutation was performed on a peripheral blood sample, revealing a homozygous pathogenic mutation of c247_250delCTGT (p.Ile85Serfs*33) (Fig. ). This point mutation was also detected in the frozen tissue of the patient.

[[9.0, 'year']]

F

{'24423286': 1, '21640474': 1, '26174862': 1, '20860043': 1, '26986124': 2, '27539217': 1, '23604412': 1, '24599222': 1, '31179254': 1, '25134801': 1, '28477012': 1, '34423159': 1, '29302427': 1, '18156937': 1, '22785103': 1, '22528577': 1, '18038712': 1, '27480993': 1, '25437309': 1, '24399015': 1, '29121944': 2, '28628713': 1, '22576843': 1, '19405259': 1, '28225638': 1, '26756113': 1, '24314196': 1, '25633730': 1, '20525548': 1, '20833329': 1, '29921249': 2}

{'5680789-1': 1, '4839905-1': 1}

6009036-1

comm/PMC006xxxxxx/PMC6009036.xml

Jaundice caused by protrusion of a hepatic cyst into common bile duct that was resolved by choledochoscopic needle-knife electrotomy: a case report

A 41-year-old woman presented to our hospital for further evaluation and management of persistent liver dysfunction, painless xanthochromia, and skin itching for 3 months.\nOn admission, a physical examination showed severe icteric sclera and skin without abdominal tenderness or positive shifting dullness. Liver function showed severe hepatic injury and obstructive jaundice (Table ). US showed a 5.0 × 5.3 cm, well-circumscribed anechoic lesion with many separations, increased through-transmission of sound, and no evidence of mural nodularity. Epigastric enhanced CT showed a cystic mass with sharply defined margins and smooth, thin, non-reinforced walls in the left medial liver lobe, and obstruction of the biliary tract. The contents of the mass were homogeneous, with water-density lesions, suggesting that it was a cyst. MRI showed a homogeneously hypointense lesion on T1-weighted imaging and this was homogeneously hyperintense on T2-weighted imaging. US, CT, and magnetic resonance cholangiopancreatography (MRCP) showed a slightly ectatic right hepatic duct, a greatly ectatic left hepatic duct and common bile duct, an enlarged gallbladder, and a normal distal end of the choledochus (Fig. ). Possible reasons for biliary obstruction were bile duct tumor, common bile duct stones, or compression of the common bile duct by a hepatic cyst.\nEndoscopic retrograde cholangiography (ERC) was performed to define the cause of obstruction. This procedure showed that the right hepatic duct was ectatic, but the left hepatic duct and common bile duct were not observed. There were no stone, but a large lump was observed in the common bile duct, which suggested suspicious bile duct tumor. Endoscopic sphincterotomy, endoscopic retrograde biliary drainage(ERBD), and endoscopic nasobiliary draingage(ENBD) were successfully performed to drain bile for severe hepatic injury and jaundice, rather than laparotomy, because of coagulation disorders (Fig. ). After these procedures, the icteric sclera and skin gradually faded, liver function was obviously improved, and coagulation disorders were close to normal levels (Table ).\nFour weeks later, an open operation was performed for suspicion of bile duct tumor. In exploration of the common bile duct by a choledochoscope, a large pedunculated lump protruding into the common bile duct from the left hepatic duct was discovered. This lump was completely removed from the root of the lump by endoscopic needle-knife electrotomy without active hemorrhage (Fig. ). The morphology of the lump was similar to the gallbladder with a capsule wall and capsular space (Fig. ). The choledochoscope was inserted into the hepatic duct from the stump of the lump and there was no viscous liquid secreted by cystadenoma or cystadenocarcinoma. Postoperative pathology of the lump suggested a hepatic cyst wall without heterocysts or tumor cells (Fig. ). A T tube was retained in the common bile duct in order to prevent bile leakage and observe the recovery of the root incision of the lump and postoperative bleeding.\nTwo months later, all of the liver function indices recovered to the normal range (Table ). Epigastric US and CT showed a normal hepatic duct and the hepatic cyst was the same size compared with preoperatively. A choledochoscopic examination was performed through a T-tube fistula. There was no evidence of bile duct tumor. The choledochoscope could enter into the cyst from the defect of the bile duct wall, and the mucosa around the incision recovered well, with no signs of stricture.\nThree years after the procedure, the patient was in a good general condition, without signs of cholestasis or bile duct stones. The cyst had barely changed (Fig. ).

[[41.0, 'year']]

F

{'18316254': 1, '22961971': 1, '3946368': 1, '25765565': 1, '23396748': 1, '15983777': 1, '29047328': 1, '24770829': 1, '24098524': 1, '27153771': 1, '18090983': 1, '28733730': 1, '3350261': 1, '2648477': 1, '2669463': 1, '23445783': 1, '14709695': 1, '23154870': 1, '17356842': 1, '27861347': 1, '15900063': 1, '28739769': 1, '23819056': 1, '29921238': 2}

{}

6009975-1

comm/PMC006xxxxxx/PMC6009975.xml

An unusual case of ectopic corticotrophin-releasing hormone syndrome caused by an adrenal noncatecholamine-secreting pheochromocytoma: a case report

Prior to this admission, a 27-year-old woman sought medical attention at a local hospital because of facial redness and edema caused by eating a mango. She was treated with dexamethasone (5 mg intravenously daily) for five days. The patient gradually developed a round face, acne, hirsutism, hypokalemia, and 5 kg of weight loss over the course of one month since receiving dexamethasone. Upon hospitalization, the patient presented with a one-month history of facial edema, weight loss, and acne. She had no family history of Cushing’s syndrome, pheochromocytoma, or multiple endocrine neoplasia type 2. A 5.5-cm mass located in the right posterior lobe of the liver was detected by ultrasound in a routine physical examination 16 months ago at a local hospital; however, the patient had no symptom and did not seek further medical attention at that time.\nThe patient’s blood pressure was 120/75 mmHg in both arms in the supine position, with a regular pulse of 76 bpm. Her height was 164 cm and her weight was 48 kg (body mass index:17.8 kg/m2). The patient had “moon face” and severe facial edema, beard, central deposition of fat with slim extremities and atrophic muscles, and no pretibial edema. The skin was diffused with acne and both armpits had hyperpigmentaion.\nLaboratory tests revealed marked hypokalemia (2.1 mmol/L; normal range, 3.5–5.5 mmol/L), which could not be normalized with oral and intravenous potassium supplementation until spironolactone was added. A 75-g oral glucose tolerance test confirmed diabetes mellitus with a fasting blood glucose level of 9.19 mmol/l and a 2-h glucose level of 21.66 mmol/L, with a HbA1c level of 6.3%. She was started on insulin aspart30 (48 U daily).\nEndocrinological investigation identified severe hypercortisolism with loss of circadian rhythm. Plasma ACTH level was elevated to 1157 pg/mL, confirming ACTH-dependent Cushing’s syndrome. Except for testosterone, the catecholamine metabolites, growth hormone, calcitonin, and prolactin levels were all within the normal range (Table ).\nMRI and contrasted MRI revealed no pituitary adenoma and EAS was considered. Abdominal contrast-enhanced CT scanning revealed that the mass lesion was located in the right adrenal gland and not in the right hepatic posterior lobe. The mass was a heterogeneous solid tumor which was mildly enhanced with some patchy nonenhancing areas (Fig. ). PET/CT showed moderate fluorodeoxyglucose (FDG) uptake in the mass (Fig. ). Both contrasted CT and PET-CT revealed bilateral adrenal hyperplasia, but the neck, thorax, and pelvis were normal.\nWe prepared to do inferior petrosal sinus sampling (IPSS) for ACTH assays, adrenal vein sampling for ACTH assays to make out the origin of ACTH. But the patient’s general condition rapidly deteriorated after admission, Adrenal tumorectomy was performed. During surgery, when the right adrenal mass was mobilized, no hypertensive crisis occurred. A 6.5-cm black mass was found arising from the medial branch of the right adrenal gland (Fig. ). Hematoxylin-eosin staining of the tumor revealed that most of the cells were chromaffin-like cells. In addition, there were multifocal oval eosinophilic cells under the tumor capsule (Fig. ). Immunohistochemical staining showed positive staining for chromogranin A (CgA) (Fig. ) and CD56 (Fig. ), with a Ki67 labeling index of approximately 16% (Fig. ) for chromaffin-like cells. No positive ACTH immunostaining was noticed (Fig. ). Positive immunostaining for CRH and Melan-A (Fig. and ) and negative immunostaining for CgA and CD56 were found in the eosinophilic cells, indicating that CRH production is indeed derived from the tumor’s peripheral cells.\nOne day after surgery, ACTH levels had decreased from 715 to 14.3 pg/ml and serum cortisol level had decreased from more than 50 to 10.4 μg/dl. One week postoperatively, blood potassium and glucose levels normalized without the need for medication. The signs and symptoms of Cushing’s syndrome gradually disappeared within two months, and the hydrocortisone supplementation (initial dosage was 60 mg daily and tapered gradually) was discontinued seven weeks postoperatively. CT revealed that the left adrenal gland was almost reduced to the normal size three months later. The patient is currently under regular follow-up and remains well nine months after surgery.

[[27.0, 'year']]

F

{'19351607': 1, '19246974': 1, '1283118': 1, '18264630': 1, '15251695': 1, '16303835': 1, '17039427': 1, '27699708': 2, '7921204': 1, '26433209': 1, '7491544': 1, '11182843': 1, '31434676': 1, '3035046': 1, '18209859': 1, '29921267': 2}

{'5225211-1': 1, '5225211-2': 1}

6010360-1

comm/PMC006xxxxxx/PMC6010360.xml

Dapsone-induced Methemoglobinemia in a Child with End-stage Renal Disease: A Brief Review

A four-year-old girl with a history of asphyxiating thoracic dysplasia (Jeune syndrome) and end-stage renal disease, with a post-renal transplant, was on immunosuppressive therapy for chronic rejection. She was admitted to our tertiary care pediatric intensive care unit (PICU) with cardiorespiratory failure. The child was intubated and started on mechanical ventilation. The child was on daily peritoneal dialysis for renal failure and on dapsone for Pneumocystis prophylaxis for the last six months. During the hospital stay, she developed multiple complications, including sepsis, electrolyte imbalance, and thrombocytopenia. She was on various medications, including fentanyl, midazolam, mycophenolate, prednisolone, pantoprazole, cefepime, and dapsone. During the third week of hospital stay, the child had diarrhea and then developed metabolic acidosis. She was also noted to have cyanosis, low oxygen saturation (82% to 87%) by pulse oximetry (peripheral capillary oxygen saturation (SPO2)), despite the escalation of inspired oxygen to 100%. A blood gas analysis consistent with metabolic acidosis (ph 7.23, oxygen partial pressure (PaO2) 125 mmHg, partial pressure of carbon dioxide in arterial blood (PaCo2) 42 mmHg, HCo3 14.6, bases excess (BE) -9). With cyanosis, low oxygen saturation by pulse oximetry (SPO2) in the setting of normal oxygen partial pressure (PaO2) methemoglobinemia was suspected. Co-oximetry was ordered, which revealed a methemoglobin level of 21.5% (normal range 0 to 2%). Laboratory workup ruled out hemolysis. Since the child had end-stage renal disease, methylene blue was not considered as a treatment option, and she was started on daily ascorbic acid 500 mg via a gastrostomy tube. Dapsone was discontinued and pentamidine nebulization monthly started for Pneumocystis prophylaxis. Serial methemoglobin levels on co-oximetry showed an improving trend. Within 48 hours, the methemoglobinemia resolved and the level came down to 0.4 mg/dl. Ascorbic acid was continued for four more days and discontinued. Off ascorbic acid, the methemoglobin level remained less than 0.3 mg/dl. After a meticulous review of the medications and workup, we concluded that dapsone was the probable cause of methemoglobinemia in this child.

[[4.0, 'year']]

F

{'24310318': 1, '10533013': 1, '16212642': 1, '6228217': 1, '8200173': 1, '22024786': 1, '26134197': 1, '17272524': 1, '18376092': 1, '29930890': 2}

{}

6010361-1

comm/PMC006xxxxxx/PMC6010361.xml

Post Vaccination Guillain Barre Syndrome: A Case Report

A 15-year-old girl presented to the emergency department with a history of progressive lower limbs weakness for 10 days, inability to pass urine and intermittent grade 6/10 lower abdominal pain for two days. She had no associated symptoms like fever, rash, headache, backache or blurring of vision. No antecedent respiratory tract infection or diarrheal illness. Past medical history was insignificant. About 24 days prior to these symptoms, she received neural tissue sheep brain anti-rabies vaccine following a dog bite. On physical examination, the patient was not in acute distress. She was afebrile and her vitals were: a) Blood pressure: 130/80 mm Hg; b) Respiratory rate: 18 breaths/minute; c) Heart rate: 102 bpm.\nNeurological examination revealed no facial asymmetry and intact cranial nerves. Motor system examination of the lower limbs revealed following: a) Power of the knee: grade ⅕ below the knees; b) Power of the hips: grade ⅕ of the hip flexors and extensors bilaterally; c) Tone and reflexes of the legs: hypotonia of legs bilaterally with loss of knee and ankle reflexes bilaterally; d) Joint position and vibration sense: reduced joint position and vibration was noticed, and there was hyperesthesia of the soles bilaterally.\nUpper limbs were normal on physical examination. The cardiorespiratory system was unremarkable on examination. On abdominal examination, urinary bladder was palpable above the symphysis pubis and was tender on palpation.\nLaboratory investigations revealed hemoglobin of 12.5 g/dl with the hematocrit of 44%; TLC was 6500/cmm and ESR at the first hour using Westergren method was 23 mm. Cerebrospinal fluid examination (CSF) revealed clear fluid, normal opening pressure, glucose 75 mg/dl (normal range: 45–100 mg/dl), proteins 2.5 g/l (normal range: 0.18–0.45 g/dl) and WBCs 2/cmm (albuminocytologic dissociation).\nBased on physical examination and CSF findings a provisional diagnosis of Guillain–Barre syndrome was made. Electrophysiological study (confirmatory test) revealed demyelinating polyneuropathy consistent with Guillain–Barre syndrome. Plasmapheresis and physical therapy sessions resulted in a nearly complete recovery in six to eight months.

[[15.0, 'year']]

F

{'34480607': 1, '34867899': 1, '8685108': 1, '34567447': 2, '9562667': 1, '22694000': 1, '32409144': 1, '11291988': 1, '8036829': 1, '34934821': 1, '19388722': 1, '29930889': 2}

{'8462911-1': 1}

6010465-1

comm/PMC006xxxxxx/PMC6010465.xml

Signaling pathway screening platforms are an efficient approach to identify therapeutic targets in cancers that lack known driver mutations: a case report for a cancer of unknown primary origin

A 68-year old gentleman presented with an isolated right axillary mass in October 2014. An axillary biopsy revealed poorly-differentiated (Grade 3, Ki67 85%) neuroendocrine carcinoma of unknown origin (positive for CD56, chromagranin and synaptophysin; negative for TTF1, CDX2 and Merkel Cell Polyomavirus). Positron emission technology/computed tomography demonstrated a right axillary mass and no other identifiable sites of disease. The patient received six cycles of carboplatin and etoposide chemotherapy between Oct 2014 and April 2015 with RECIST partial response after three cycles but with evidence of tumor growth after cycle 6. He was referred to the Experimental Cancer Medicine Team and consented to TARGET in June 2015. In parallel he was referred for surgical resection of the isolated axillary mass which achieved a complete resection and permitted access to fresh tissue for PDX and translational research in July 2015.\nFollowing a brief disease-free period, the patient relapsed with metastatic nodules within the retroperitoneum in December 2015. The patient was treated with capecitabine and temozolamide between March and October 2016 with best response of progressive disease. Treatment was switched to interferon and sandostatin and his disease has remained stable by RECIST 1.1 criteria on this combination at the time of writing.

[[68.0, 'year']]

M

{'31817861': 1, '19505943': 1, '16452206': 1, '21478889': 1, '29082359': 1, '22294718': 1, '26758680': 1, '22637570': 1, '32753716': 1, '30580378': 1, '28365644': 1, '18725988': 1, '22500797': 1, '32640668': 1, '28886003': 1, '20601685': 1, '26469047': 1, '19451168': 1, '27827359': 1, '24880617': 1, '25448010': 1, '26715644': 1, '26370156': 1, '26839307': 1, '29056346': 1, '20644199': 1, '27663479': 1, '19179188': 1, '29951225': 2}

{}

6010516-1

comm/PMC006xxxxxx/PMC6010516.xml

Deficiency of Adenosine Deaminase 2 in Adult Siblings: Many Years of a Misdiagnosed Disease With Severe Consequences

A 40-year-old Caucasian male, with a prior diagnosis of PAN, presented in January 2016 with large volume hematemesis from esophageal varices related to portal hypertension. Throughout his childhood he had chronic arthralgia and swelling of his ankles diagnosed as juvenile idiopathic arthritis. By age 4, he manifested developmental delays, including difficulty with ambulation. At the age of 20, he developed recurrent ischemic strokes causing persistent dysarthria, ataxia, and weakness. During that time, he developed recurrent violaceous subcutaneous lesions that would ulcerate, but were responsive to glucocorticoids. He was diagnosed with PAN based on skin biopsies. He required a long-term maintenance dose of prednisone 7.5 mg daily, as reduction would led to recurrent skin lesions. In childhood, he developed poorly explained visual loss without evidence of inflammatory eye disease on physical exam. His visual acuity had been stable for the last couple years prior to presentation. His history was also remarkable for recurrent mouth and genital ulcers, which were also responsive to glucocorticoids. He had lymphopenia (absolute lymphocytes count 400/μl [1000-4800], CD3 422/μl [600-2990], CD4 370/μl [440-2160], CD8 49/μl [120-1320], CD19 11/μl [100-700]), yet normal lymphocyte proliferation studies to mitogens. Additionally, he had hypogammaglobulinemia (IgG 383 mg/dl [762-1488], IgA 46 [70-390], IgM <20 [38-328]), but had protective antibody levels to both tetanus and pneumococcus. There was no history of recurrent infections.

[[40.0, 'year']]

M

{'32845415': 1, '30386947': 1, '18647320': 1, '34122398': 1, '25457153': 1, '30644014': 1, '24552285': 1, '26867732': 1, '27130863': 1, '31848804': 1, '27059682': 1, '24552284': 1, '23420139': 1, '29391253': 1, '32743516': 1, '32276138': 1, '29271561': 1, '29411230': 1, '15182332': 1, '31945408': 1, '28974505': 1, '27514238': 1, '32638197': 1, '32892503': 1, '31015188': 1, '29963054': 2}

{'6010516-2': 2}

6010516-2

comm/PMC006xxxxxx/PMC6010516.xml

Deficiency of Adenosine Deaminase 2 in Adult Siblings: Many Years of a Misdiagnosed Disease With Severe Consequences

A 43-year-old Caucasian male, brother to Patient 1, had a similar presentation. At 5 years of age, he was diagnosed with juvenile idiopathic arthritis after presenting with polyarthritis. At the age of 8, he was diagnosed with stage IV Hodgkin’s lymphoma (records were not available). He was initially treated with radiation therapy alone, but later transitioned to chemotherapy [initially with MOPP (mechlorethamine, vincristine, procarbazine, and prednisone) and later converted to ABVD (doxorubicin, bleomycin, vinblastine, and dacarbazine)] with remission achieved 1 year later. At age 24, he developed sudden onset left-sided persistent numbness followed by bilateral sensorineural hearing loss with near complete left-sided deafness. At age 26, he presented with bright red blood in his stool, which required resection of 6 inches of small intestines for reasons that were unclear. By age 29, he developed heart failure and pulmonary hypertension presumed secondary to ABVD treatment. His history was also significant for superior vena cava occlusion, presumed secondary to a port catheter, and esophageal varices. Immunologic evaluation revealed normal immunoglobulins, lymphocyte proliferation to mitogens, and lymphocyte subsets except for a slightly low CD8 count (83/μl). He had protective antibody levels to tetanus.\nBased on these presentations, there was a high suspicion for DADA2. Written informed consent was obtained for each patient, as well as their parents for genetic testing, and for the publication of this case report. ADA2 exon sequencing was performed on both patients and their unaffected parents as previously described (). Adenosine deaminase 2 levels were tested only in Patient 2 with comparisons made to DADA2 patients, adult carriers for ADA2 mutations and adult healthy controls.

[[43.0, 'year']]

M

{'32845415': 1, '30386947': 1, '18647320': 1, '34122398': 1, '25457153': 1, '30644014': 1, '24552285': 1, '26867732': 1, '27130863': 1, '31848804': 1, '27059682': 1, '24552284': 1, '23420139': 1, '29391253': 1, '32743516': 1, '32276138': 1, '29271561': 1, '29411230': 1, '15182332': 1, '31945408': 1, '28974505': 1, '27514238': 1, '32638197': 1, '32892503': 1, '31015188': 1, '29963054': 2}

{'6010516-1': 2}

6010554-1

comm/PMC006xxxxxx/PMC6010554.xml

A Craniopharyngioma Associated With Elevated Cerebrospinal Fluid HCG Concentrations Misdiagnosed as a Germinoma

A 35-year-old female was admitted to our hospital complaining of an intermittent headache for 5 years, menopause for more than 2 months, and blurred vision and lactation for more than 1 month. Five years prior, the patient began suffering from intermittent and gradually deteriorating occipital and frontal headaches. One month prior to admission, she had impaired vision and an absent right temporal view and simultaneous bilateral galactorrhea, without purulent or bloody secretions. Since the onset of symptoms, the patient experienced no nausea or vomiting, sleepiness, chills, hair loss, or fatigue. Computed tomography (CT) showed an irregular cystic solid mass in the suprasellar area. The size of the mass was 1.4*1 cm and could be markedly enhanced. The CT value of the solid part of the lesion on plain CT was 40 Hounsfield units (HU); in the enhancement, the value was 74 HU, and the cystic part showed no enhancement. The boundary of the lesion was less clear, the density was not uniform, and no obvious calcification was observed. The cavernous sinus was involved. There were no abnormalities in the ventricular system, sulcus, brain split and pool. Moreover, there was no shift in the midline structure, and no abnormalities were found in the skull structure. The sinuses exhibited no obvious abnormalities. Magnetic resonance imaging (MRI) of the brain and pituitary showed an irregular cystic solid mass in the suprasellar area, involving in the pituitary stalk, and the boundary between the lesion and pituitary was unclear. In addition, the mass pressed on the optic chiasm, and the solid part of the lesion was significantly enhanced, while the cystic part showed no enhancement. There was no abnormal signal in the brain or paranasal sinuses nor signs of calcification (Figure ). Auxiliary examinations revealed that the patient's urine volume and specific gravity were within the normal range (urine volume: 1,800 mL/day; urine specific gravity: 1.015). A pituitary function evaluation showed hyperprolactinemia (45.47 μg/L, normal range for female: 2.8–29.2 U/L) and intact growth hormone (GH) and gonadotropin function. The insulin-like growth factor 1 (IGF-1) level was 120 ng/ml (109–284 ng/ml). The pituitary-thyroid and pituitary-adrenal axis were intact (Tables –). The blood sedimentation and CRP level were normal, and indicators related to tuberculosis and other infections were negative. Because of the thickening of the pituitary stalk, CSF analysis was performed to identify the nature of the mass lesion before any invasive procedures were performed. The results of CSF analysis were normal except for a significantly increased human HCG (18.54 U/L, normal range: 0–5 U/L) level. However, the serum HCG and α-fetoprotein (AFP) levels were normal. A biopsy was recommended to clarify the diagnosis, but the patient declined. Considering the possibility of a germinoma, radiotherapy was recommended. No regression of the intracranial mass and no resolution of the headache were observed after 10 radiotherapy treatments (total 10 Gy). Therefore, the patient was admitted a second time. Apart from a deficiency in GH and hypogonadotropic hormones, the other axes were intact (Tables –). The IGF-1 level was 89 ng/ml (109–284 ng/ml). A repeated CSF analysis indicated there was no obvious decrease in the HCG level in the CSF (17.41 U/L, normal range: 0–5 U/L). An MRI showed no signs of shrinkage of the mass (Figure ). Due to the enlarged intracranial mass, the patient was transferred to the neurosurgical department for total resection of the mass. The pathological analysis was indicative of a craniopharyngioma (Figure ).\nUnder a microscope, the lateral fissure was separated at the saddle area, and CSF was released. The brain tissue collapsed, and a portion of the temporal lobe was removed. The tumor was located in the suprasellar area. The tumor was removed from the second gap along the longitudinal axis of the pituitary stalk and was frozen for analysis. The tumor and hypothalamus were closely related. Hemostasis was achieved, and the gauze inventory was correct. The surgery was complete following strict closure of the dura mater, bone flap reduction and fixation, suturing of the temporalis muscle and scalp, and placement of an external dura mater drain. The tumor specimens were sent for pathological analysis.\nAfter surgery, the patient did well and had no complaints of headache, vomiting, polyuria, polydipsia, sleepiness, or anorexia. She did not receive any hormone replacement. One year later, she was admitted to our endocrinology department for re-evaluation. Her urine volume and specific gravity were within the normal range (urine volume: 1,500 mL/day; urine specific gravity: 1.017). The urine osmotic pressure was 752 mOsm/L (0–1,000 mOsm/L), the blood osmolality was 292 mOsm/L, and a pituitary stimulation test revealed normal frontal lobe function except for an impaired GH level (Tables , ). The IGF-1 level was 92 ng/ml. HCG levels in the serum and CSF were within normal limits (serum HCG: 0.1 U/L; CSF HCG: 0.31 U/L). The slightly increased prolactin detected preoperatively had decreased to within the normal range. Cranial and pituitary MRI showed no expansion of the sellar region, no signs of a local tumor, no abnormal enhancement, a clear boundary of the optic chiasm, and no signs of tumor recurrence (Figure ).

[[35.0, 'year']]

F

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{}

6010806-1

comm/PMC006xxxxxx/PMC6010806.xml

Novel application of cryobiopsy in the diagnosis of pulmonary alveolar proteinosis

A 54-year-old male businessman presented to our clinic with progressive shortness of breath for the last three months. He also complained of intermittent cough with yellowish sputum and dyspnoea upon exertion for half a year. He had a history of well-controlled hypertension and smoking one pack of cigarettes per day. Family history was remarkable for adenocarcinoma of the lung. Travel history was remarkable for travel to Yunnan, China, in the past year.\nHe first visited a pulmonologist in a local clinic a month prior to admission, where chest X-ray demonstrated increased infiltration over bilateral lung fields. Oral azithromycin was administered, but his dyspnoea progressed. He then visited our clinic for a second opinion. Upon examination, he denied fever, joint pain, dry eyes or mouth, muscle weakness, pitting oedema over lower legs, and skin rash. Physical examination was unremarkable, except chest auscultation demonstrating fine crackles over bilateral lung fields. Chest X-ray showed diffuse interstitial infiltration bilaterally (Fig. ). He was admitted for further workup and monitoring of his respiratory performance.\nDuring hospitalization, the patient had no fever, and respiratory pattern was smooth. Peripheral capillary oxygen saturation (SpO2) was 95% under ambient air. Serum white blood cell count showed no leucocytosis or left shift. Autoimmune markers, including rheumatoid factor, antinuclear antibody, anti-Smith antibody, anti-ribonucleoprotein antibody, anti-SSA, anti-SSB, and anti-Scl-70, were all negative. Polymerase chain reaction of Pneumocystis jiroveci from induced sputum was negative. One of three sets of sputum culture demonstrated non-tuberculous mycobacteria, which was later attributed to environmental contamination given his symptoms and image findings. Chest CT disclosed extensive patchy ground-glass opacities superimposed with thickened interlobular septa and intralobular lines over both lung fields, demonstrating a “crazy paving” pattern (Fig.). Pulmonary function test showed normal spirometry and moderately reduced diffusing capacity of the lungs for carbon monoxide (DLCO) on single breath diffusing capacity test (5.42 mmol/min/kPa, correlated with 57.7% of predicted value).\nBronchoscopy indicated no endobronchial lesions. A radial probe endobronchial ultrasound (EBUS) showed a “blizzard sign”, suggesting ground-glass opacity over the superior segment of the right lower lobe (Fig.) . Brush cytology, bronchoalveolar lavage (BAL), and biopsy were performed. Brush cytology was negative for malignant cells.\nMacroscopically, the BAL specimen was clear in appearance. Microbiological workup of BAL was negative for presence of bacteria, tubercolosis (TB), non-tuberculous mycobacteria (NTM), fungus, and Pneumocystis jiroveci. We performed both a forceps biopsy and a cryobiopsy on the larger-sized lesions.\nPathological report of the four 0.2 × 0.2 × 0.1 cm tissue fragments obtained by forceps biopsy indicated only chronic inflammation.\nOn the other hand, the four 0.5 × 0.5 × 0.2 cm tissue fragments acquired by cryobiopsy revealed periodic acid-Schiff (PAS)-positive granular proteinaceous exudate with mild interstitial lymphocytic infiltration filling the alveolar spaces consistent with PAP (Fig.).\nWe discussed the treatment options with the patient, and whole lung lavage was advised. The patient declined our suggested treatment and opted for close surveillance. He was discharged with outpatient follow up. Later, his shortness of breath spontaneously resolved, and serial chest X-rays showed resolution of the interstitial infiltration of the lungs.

[[54.0, 'year']]

M

{'27514590': 1, '29021953': 1, '27660154': 1, '26991519': 1, '14695413': 1, '24862226': 1, '21632797': 1, '8620721': 1, '13552931': 1, '28399377': 1, '32643644': 2, '29938112': 2}

{'7507936-1': 1}

6011099-1

comm/PMC006xxxxxx/PMC6011099.xml

EBV Infection in XLP1 Manifested Solely by Behavioral Aggression and Effective Treatment Using Rituximab

A 5-year-old male initially came to the attention of the immunology service at 2 years of age with a history of recurrent sinopulmonary infections and a family history of XLP1 (nonsense mutation c. 191G>A in the SH2D1A gene). Of note, his uncle with XLP1 had a history of EBV-related central nervous system (CNS) lymphoma. SAP expression was found to be absent in NK and CD8 cells, confirming the diagnosis of XLP1. NKT cells were undetectable. Profound hypogammaglobulinemia was also noted. He was monitored and maintained on monthly intravenous immunoglobulin (IVIG) infusions with only minor breakthrough sinopulmonary infections. Adherence to IVIG was suboptimal due to social reasons. The family deferred stem cell transplantation. At the age of 5 years, he presented with acute behavioral changes, manifesting as uncontrolled aggression, requiring inpatient psychiatric treatment. Basic labs including complete blood count and comprehensive metabolic panel were normal. Specifically, no cytopenias, no transaminitis, and normal inflammatory markers including C-reactive protein (CRP) and platelet count were noted. MRI of the brain showed multiple small nonenhancing foci on T2 FLAIR sequence, mostly in the frontal lobes and scattered throughout the subcortical white matter (). The lesions remained unchanged on repeat MRI at 2 weeks and again at 4 months following presentation, suggesting that these lesions may be consistent with glial scars without acute inflammation of the brain. PCR revealed presence of EBV in the blood (2300 copies/ml). Cerebrospinal fluid (CSF) also demonstrated EBV (73 copies/ml). CSF was otherwise normal apart from only slightly elevated CSF WBC (8 cells/mm3), of which 79% were lymphocytes and 21% monocytes. Intravenous gamma globulin 1 g/kg and intravenous ganciclovir 10 mg/kg/day were started immediately. However, despite 14 days of ganciclovir treatment, EBV counts remained significantly elevated. Rituximab was administered on days 16 and 23 of admission. Prednisolone 1 mg/kg/day was given daily on days 16 through 23, and IVIG 1 g/kg was repeated on day 23. Valganciclovir 45 mg/kg/day was continued for 4 months after ganciclovir treatment. Prior to rituximab infusion, EBV counts had reached 2300 copies of EBV genome/mL of blood. Five days after the second rituximab infusion CD19+ and CD20+ lymphocytes and EBV PCR were found to be absent in the blood and remained absent for 5 months, at which time hematopoietic stem cell transplant (HSCT) was performed ( and ). IgM to EBV was undetectable at the time of presentation, and the patient never seroconverted.

[[5.0, 'year']]

M

{'17066466': 1, '15749842': 1, '7506951': 1, '25085526': 1, '22613797': 1, '15738056': 1, '15494422': 1, '20926771': 1, '7522629': 1, '12670406': 1, '6306053': 1, '11049992': 1, '15711562': 1, '25613729': 1, '22069374': 1, '29977631': 2}

{}

6011100-1

comm/PMC006xxxxxx/PMC6011100.xml

Glucose Management during Insulinoma Resection Using Real-Time Subcutaneous Continuous Glucose Monitoring

A 12-year-old girl (height, 144 cm; weight, 40 kg) was presented to the hospital with an episode of seizure with impaired consciousness. Her BG at that time was 60 mg/dL (normal value of casual BG: 70–200 mg/dL). Glucose was administered and she recovered consciousness. She had no significant comorbidities prior to hospital admission. From her family history and further investigation, she was diagnosed as having insulinoma and hyperparathyroidism in MEN-1. Arterial phase images of computed tomography showed a vascularity-rich tumor of 20 mm in diameter located in the head of the pancreas. The feeding artery of the tumor was not clearly demonstrated by angiography and selective arterial calcium injection. We considered that extended surgical procedures such as pancreatoduodenectomy might be required depending on intraoperative findings; therefore, open abdominal surgery rather than laparoscopic surgery was scheduled. Her intact parathyroid hormone level was 66.0 pg/ml (normal value: 10–65 pg/mL) and her adjusted serum calcium level was 10.3 mg/dL (normal value: 8.7–9.9 mg/dL), although parathyroid ultrasound examination revealed no parathyroid tumor. Other tumors complicated with MEN-1 were not detected. The results of other preoperative examinations were unremarkable. On the day before surgery, an Enlite™ sensor of MiniMed™ 620G (Medtronic Diabetes, Northridge, CA, USA) SCGM system was inserted into her upper arm. Although MiniMed 620G was combined with an insulin pump, we did not use the pump. The SCGM system was calibrated as recommended by the manufacturer with capillary BG measured by OneTouch® UltraVue™ Blood Glucose Meter (Johnson & Johnson, New Brunswick, NJ, USA).\nNo premedication was given and she walked into the operating room. Capillary BG was 80 mg/dL and the SCGM system was calibrated. Continuous glucose infusion was started at 4.6 g/hr, and general anesthesia was induced with 80 mg of propofol, 50 mcg of fentanyl, and 0.02 mcg/kg/min of remifentanil. Muscle relaxation was obtained by administration of 30 mg of rocuronium, and the trachea was intubated. After induction of general anesthesia, an epidural catheter was inserted at the eighth and ninth thoracic interspace. Anesthesia was maintained with 1.7% sevoflurane, 0.01–0.02 mcg/kg/min of remifentanil, and intermittent thoracic epidural administration of 0.25% levobupivacaine (3 ml). An arterial catheter was inserted, and arterial BG measured by an ABL800 Flex Blood Gas Analyzer (Radiometer, Brea, CA, USA) was 71 mg/dL, which was compatible with IG of 73 mg/dL. An additional 2 g of glucose was given intravenously before the start of surgery, and IG increased from 77 mg/dL to 101 mg/dL at 20 minutes (). After that, IG mildly decreased and returned to almost the same level after 1 hour. The surgeon informed us that the tumor would be removed by enucleation shortly. Just before tumor resection, arterial BG was 76 mg/dL and IG was 80 mg/dL. At 20 minutes after tumor resection, IG showed a rapid increase. We therefore decreased the continuous glucose infusion rate to the usual dose of 1 g/hr, and IG gradually decreased and was stabilized at about 140 mg/dL. During surgery, blood pressure was between 90/50 and 120/70 mmHg, and heart rate was between 60 and 90 bpm. She was extubated in the operating room and transferred to the general surgery ward. The operation time was 2 h and 24 min, and the anesthesia time was 3 h and 53 min. Postoperative pain was controlled well by continuous epidural analgesia (12 mcg of fentanyl and 0.2% levobupivacaine at 4 ml/hr) and administration of 600 mg of acetaminophen (every 6 hr). Her postoperative course was uneventful and she was discharged on POD 10.

[[12.0, 'year']]

F

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{'7325648-1': 1}

6011104-1

comm/PMC006xxxxxx/PMC6011104.xml

Renal Cell Carcinoma in a Horseshoe Kidney Treated with Laparoscopic Partial Nephrectomy

An 83-year-old woman was referred to our department for further examination of a left renal tumor 20 mm in diameter in her horseshoe kidney. She had no remarkable medical history except for uterine cancer at 49 years of age. The laboratory data showed slight anemia and a low liver function (Hb 12.3 g/dL, AST 52 IU/L, ALT44IU/L, and LDH 201 IU/L).\nContrast-enhanced computed tomography (CT) showed a renal tumor covered with a capsule and buried by nearly 30%. The tumor was supplied by four arteries toward the left kidney (). We set the laparoscopic ports as shown in . We first approached intraperitoneally and then cut the peritoneal and approached to the renal helium. We encountered a thin artery and cut it after confirming the supplied area by clamping. We then clamped the main artery and cut the tumor with a 1 mm surgical margin. No urinary tract leakage was observed. After coagulation using bipolar forceps, we sutured using 1-0 Vicryl (Ethicon, Cincinnati, OH, USA) then sprayed with Arista AH (BARD; Warwick, RI, USA). We then fixed the thread as shown in . After confirming the lack of active bleeding, we placed a drainage tube and closed the incision. The total operation time was 2 h 39 min, with 11 min of clamping.\nA histopathological examination revealed clear cell carcinoma (grade 2, INFα, v[−], pT1a). The patient was discharged 11 days after surgery and has been free from recurrence for 43 months.

[[83.0, 'year']]

F

{'3054787': 1, '14744359': 1, '29027269': 1, '17494112': 1, '18786208': 1, '32743445': 2, '32489122': 2, '32743446': 1, '28332239': 1, '33817203': 2, '19958148': 1, '10625187': 1, '11992090': 1, '25553168': 1, '30959826': 1, '31186978': 2, '22924304': 1, '30896647': 1, '29288785': 1, '32904068': 1, '29977636': 2}

{'6521524-1': 1, '7292058-1': 1, '7273767-1': 1, '7874816-1': 1}

6011110-1

comm/PMC006xxxxxx/PMC6011110.xml

Nerve Surgery to Treat Intractable Genitofemoral Neuropathic Pain following Laparoscopic Live Kidney Donation

A 50-year-old man underwent live laparoscopic donor nephrectomy (LLDN), through transperitoneal approach, on his left kidney as a donor for his sister. This procedure went uneventful and the patient was discharged 3 days after surgery. However, within one week after the procedure the patient experienced pain at the left side of the scrotum and left upper leg. During physical examination, a hypersensitive scrotum (allodynia) was noted whereas no other abnormalities were seen. The urologist was consulted but no urologic complications were found. The neuropathic pain area befitted the genitofemoral nerve (GFN). Conservative treatment was initiated with neuroleptic drugs and blocks of the L1/L2 dorsal roots with local anaesthetics and pulsed radiofrequency lesioning. These treatments did not result in significant pain relief and he could not work. Three years after the start of the neuropathic pain, operative treatment aiming at pain relief was initiated. In a multidisciplinary meeting it was concluded that a nerve entrapment of the GFN was suspected to be the most likely cause of the patient's neuropathic pain. Eventually, an exploratory transperitoneal laparoscopy was performed. After extensive adhesiolysis, a hemoclip used for closure of the ureter at the time of nephrectomy was identified in close relation to the GFN and removed. Due to the local scarring it could not be surgically assessed to which extent the GFN was actually damaged or a neuroma was formed. It seemed, however, highly likely that the pain originated from the GFN in this area. We therefore cut the GFN proximally in a normal looking segment (). Pathological analysis of the tissue confirmed this to contain nerve tissue. Within 2 weeks following surgery, the patient was completely pain-free and could return to his normal activities.

[[50.0, 'year']]

M

{'11572904': 1, '26895841': 1, '15472550': 1, '21336213': 1, '10414548': 1, '27815571': 1, '17693290': 1, '29977640': 2}

{}

6011111-1

comm/PMC006xxxxxx/PMC6011111.xml

Inhaling Difluoroethane Computer Cleaner Resulting in Acute Kidney Injury and Chronic Kidney Disease

A 32-year-old Caucasian male with a known history of depression presented to the emergency department accompanied by police after a violent outburst following prolonged DFE abuse during a suicide attempt. On admission, he was emotionally labile and had contusions on his left shoulder and upper extremities due to a physical altercation with police. He was initially confused and verbally abusive, but within six hours he was oriented, cooperative, and able to provide a reliable history.\nThe patient stated that he had huffed keyboard cleaner several days per week for the past year; however, the frequency had increased over the last month to several times daily. Other than depression, the patient had no other known medical problems. He denied any personal or family history of kidney disease. The patient denied drinking alcohol, and he reported smoking one-half pack of cigarettes per day for the previous two years. He denied any other substance abuse except for DFE. He reported no use of nonsteroidal anti-inflammatory (NSAID) medications. During this episode of difluoroethane abuse, he had no loss of consciousness, but experienced frightening visual and auditory hallucinations as well as anxiety that persisted even after returning to his self-reported baseline mental status.\nInitial labs revealed a WBC count of 21,000 with polymorphonuclear predominance of 81%, an elevated creatinine of 1.5mg/dL with no known baseline, BUN of 10mg/dL, GFR of 54 mL/min/1.73 m2, lactic acidosis, creatine kinase of 350 U/L, and a carboxyhemoglobin level of 3.1%. Urinalysis was significant for 3+ proteinuria, moderate blood, 14 RBCs, 3 WBCs, and 3 hyaline casts. He had a negative urine drug screen.\nThe patient received single renally adjusted doses of Vancomycin and Piperacillin-Tazobactam, intravenous 0.9% saline, and oxygen by nasal cannula while in the emergency department prior to admission. Over the next 24 hours, he received intravenous fluids and oxygen with subsequent normalization of lactic acid, creatine kinase, and leukocyte count. His creatinine down trended to 1.3mg/dL. However, 36 hours into admission, his serum creatinine inexplicably rose to 2.3 with a rise in carboxyhemoglobin to 3.6%, while his urine output remained stable (). Repeat urinalysis showed a small amount of blood with RBC of 2, negative proteinuria, and no evidence of infection.\nHis normal saline infusion was transitioned to bicarbonate with saline, but was discontinued after the patient developed pruritus. Ultimately, he was transitioned to normal saline at 75 ml/hour. A renal ultrasound showed increased echogenicity of both kidneys consistent with medical renal disease, without change in size, atrophy, or cystic lesions (). Thus, it is likely that the patient had some degree of chronic kidney disease (CKD) prior to admission. For the next three days, he continued to receive intravenous normal saline with subsequent improvement of creatinine and normalization of his urinalysis and was discharged with a creatinine of 1.6.

[[32.0, 'year']]

M

{'26613951': 1, '10630273': 1, '8812268': 1, '20822675': 1, '15171188': 1, '23034927': 1, '21904803': 1, '33824835': 2, '33092229': 1, '30648044': 2, '21295430': 1, '27449958': 1, '29977633': 2}

{'8012268-1': 1, '6318142-1': 1}

6011113-1

comm/PMC006xxxxxx/PMC6011113.xml

Removal of Implant and New Rehabilitation for Better Esthetics

A 44-year-old female patient reported to the Department of Oral and Maxillofacial Surgery, and Periodontology at the Dental School of Ribeirao Preto, University of São Paulo for a placement of implants. During the initial examination, it was observed that the right upper central incisor had an unsatisfactory temporary Richmond type crown that was changed by a metal core, post, and new temporary crown. The new temporary crown was fitted to the removable partial denture that was used by the patient (). Surgical guides were made and the patient was referred for implant placement, in the region of teeth 12, 35, 36, 44, and 46.\nAfter approximately 8 months, it was verified that the implant located in the anterior region was positioned further apical and buccal () making it impossible to fabricate a successful implant crown from an esthetic point of view.\nThe implant was gradually removed using the implant removal instrument (Implant Retrieval Tool; Nobel Biocare), with antirotational movement (). Following this surgical procedure, a new implant was installed (cone morse 3.5 × 11 mm, Conexão, Brazil), with a torque of 30 N, and positioned in the mesiodistal direction. Specifically, the implant was placed closer to tooth 14 in the buccal-palatine direction on the bone ridge (located around 1 mm for the palatal from an imaginary curvature passing through the buccal surfaces of the present teeth and 1 mm infraosseo in the coronoapical direction) ().\nAfter the surgery, the removable partial denture was removed. This was followed by the fabrication of a temporary fixed partial prosthesis with retention used during the osseointegration phase on teeth 11 and 14 (): this was only for esthetic purpose. This was in addition to the installation of prostheses on the implants in the regions of teeth 35, 36, 44, and 46.\nImmediately after the second stage surgery, the temporary fixed partial denture was performed using the healing cap as an abutment and a lateral incisor as a mesial cantilever. Following the rehabilitation procedures, this temporary fixed partial denture was changed to a provisional fixed prosthesis with the mesial cantilever screwed into the implant. During this phase, periodic acrylic resin implements were added to the temporary prosthesis to promote gingival conditioning (Figures and ) and black triangle closure (Figures and ) and also to improve gingival esthetics.\nAt the end of two months, the implant prosthesis with a mesial cantilever was installed (). This case report was approved by the institution's Ethics Committee on Human Research and followed the ethical principles of the Declaration of Helsinki, in addition to complying with specific legislation.

[[44.0, 'year']]

F

{'29237427': 1, '10946340': 1, '28301683': 1, '23619748': 1, '12216916': 1, '28758035': 2, '31729576': 1, '26688293': 1, '23057026': 1, '15635945': 1, '19089213': 1, '23062142': 1, '14560479': 1, '24176547': 1, '20228977': 1, '29484714': 1, '26913807': 1, '26794049': 1, '26059796': 1, '24245461': 1, '16865133': 1, '26313019': 1, '17293814': 1, '9710822': 1, '29977626': 2}

{'5516738-1': 1, '5516738-2': 1}

6011114-1

comm/PMC006xxxxxx/PMC6011114.xml

Aesthetic Rehabilitation of a Severe Dental Fluorosis Case with Ceramic Veneers: A Step-by-Step Guide

A 26-year-old Yemeni male patient from Taiz Province was referred to the restorative dental clinics at King Saud University, Saudi Arabia. His chief complaint was an unpleasant smile caused by generalized tooth discoloration. His medical history was irrelevant. The fluoride level in the water around Taiz Province is >3.6 mg/l [].\nClinical examination revealed generalized fluorosis with loss of the outermost enamel in irregular areas involving less than half of the entire surface, as well as changes in the morphology caused by merging pits and marked attrition (). In this case, based on the Thylstrup and Fejerskov index (TFI) for dental fluorosis classification, the dental fluorosis was classified as TFI = 7 [].\nAfter the clinical examination, radiographs, preoperative photographs, and upper and lower alginate impressions for diagnostic models were taken. The patient was presented with treatment options, which included ceramic or composite veneers, along with the advantages and disadvantages of each option. The patient agreed to smile enhancement using ceramic veneers for his upper teeth given that he desired an optimum aesthetic and a long-term result. The veneers would be placed on the patient's upper teeth, from his upper right 2nd premolar to upper left 2nd premolar. The patient decided to postpone veneering his lower teeth, given his limited financial capacity. Diagnostic models were analyzed to evaluate the occlusion, and a diagnostic wax-up was made of white-colored wax. The use of the wax-up allows the patient to preview the desired appearance of his teeth, and this wax-up is also essential for the fabrication of a clear matrix for temporary restorations.\nThe desired shade was selected using the VITAPAN classical shade guide (VITA Zahnfabrik, Germany). The enamel of the eight maxillary teeth was prepared using a flat-end tapered diamond bur to a depth of 0.5–0.75 mm facial reduction with 1.5 mm incisal reduction (). A chamfer finish line was maintained at the level of the gingival margin. The proximal margin was extended into the facial and gingival embrasures.\nFollowing tooth preparation, gingival retraction was achieved using retraction cords (Ultrapak Cord #00, Ultradent Products Inc., South Jordan, UT, USA) soaked in a hemostatic agent. Impressions were taken with a polyvinylsiloxane material (Virtual, Ivoclar Vivadent, Amherst, NY). The impression material was manipulated according to the manufacturer's instructions. Temporization was performed by spot etching on the facial surface of each prepared tooth with 37% phosphoric acid (Total Etch, Ivoclar Vivadent, Schaan, Liechtenstein). Bonding agent (OptiBond Solo Plus, Kerr, Orange, CA, USA) was applied on the enamel-etched spots and light cured for 20 seconds using a high-intensity light-emitting diode (LED) curing light (Elipar S10, 3M ESPE, MN, USA). The clear matrix that was previously fabricated was loaded with a temporization material (Protemp Plus, 3M ESPE, MN, USA) and placed over the prepared teeth. Light curing was done for 10 seconds per tooth. Then, the matrix was gently teased away from the prepared teeth. A number 12 scalpel blade was used to remove the partially cured temporization material. Facial and lingual embrasures were refined with a thin diamond disk, the occlusion was adjusted, and the temporary restorations were polished using polishing discs and points ().\nCeramic veneers were fabricated with a lithium disilicate-reinforced glass ceramic material (IPS e.max Press, Ivoclar Vivadent, Schaan, Liechtenstein). Temporary veneers were removed, and the teeth were cleaned using pumice. Ceramic veneers were tried-in using a transparent shade try-in paste (Variolink Veneer try-in paste, Ivoclar Vivadent, Schaan, Liechtenstein) to assess marginal adaptation and shade.\nAfterwards, veneers were prepared for bonding. Fitting surfaces of the veneers were etched with hydrofluoric acid (Porcelain Etchant 9.5%, Bisco Inc., Schaumburg, IL, USA) for 60 seconds, washed under running water for another 60 seconds, and dried with an air syringe. A layer of silane coupling agent (Monobond Plus, Ivoclar Vivadent, Schaan, Liechtenstein) was applied on the veneers' fitting surfaces and gently air-dried after one minute. Then, the prepared teeth were etched using 37% phosphoric acid for 30 seconds, rinsed, and dried. A clear mylar strip was placed interproximally to prevent inadvertent bonding to the adjacent tooth and to facilitate the subsequent removal of excess resin cement in the embrasures. A layer of bonding agent (Adhese Universal, Ivoclar Vivadent, Schaan, Liechtenstein) was applied on the prepared tooth surfaces and air-thinned. Then, Heliobond (Ivoclar Vivadent, Schaan, Liechtenstein) was placed on the prepared tooth surfaces. The inner surface of the veneers was covered with light-cured resin cement (Variolink Veneer, transparent shade, Ivoclar Vivadent, Schaan, Liechtenstein). Veneers were positioned appropriately on the teeth by applying gentle pressure, following which excess resin cement was carefully removed with an explorer before light curing. Light curing was first performed for 2 seconds, and the excess resin cement was removed with a microbrush. After that, each veneer was light-cured from the facial aspect for 40 seconds and from the lingual aspect for 40 seconds. The two veneers of the central incisors were first simultaneously cemented. This was followed by cementation of the veneers of the two lateral incisors. Then, the veneers of the two canines were cemented. Finally, veneers for the first and second premolars were cemented simultaneously on each side.\nMinimal gingival flash of the resin luting cement was removed with a number 12 scalpel blade. A flame-shaped fine diamond bur was used to finish the ceramic margins and to contour the embrasure surfaces. Occlusion was assessed and adjusted. Flossing was performed to ensure interproximal contact patency. Ceramic polishing was performed using a series of polishing cups and points (OptraFine polishing system, Ivoclar Vivadent, Schaan, Liechtenstein). Interproximal contacts were finished with finishing and polishing strips. Final surface lustre was achieved by using a diamond polishing paste with a rubber prophylaxis cup. The postoperative clinical photographs are shown in . The patient was satisfied with the final result ().

[[26.0, 'year']]

M

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{}

6011115-1

comm/PMC006xxxxxx/PMC6011115.xml

Evaluation of Tumor Viability for Primary and Bone Metastases in Metastatic Castration-Resistant Prostate Cancer Using Whole-Body Magnetic Resonance Imaging

A 75-year-old male visited a urological practitioner because of nocturia. An elevated serum prostate specific antigen (PSA) level of 76.2 ng/mL was observed, and digital rectal examination showed diffuse induration of the prostate. Pelvic MRI demonstrated extensive high signal of the prostate in diffusion-weighted imaging (DWI) (). Fluorodeoxyglucose-positron emission tomography/CT revealed multiple spine and pelvic bone and para-aortic and pelvic lymph node metastases (Figures and ). Based on these findings, the patient was diagnosed with metastatic prostate cancer (cT3aN1M1b) and treated without prostate needle biopsy, with primary androgen deprivation therapy (ADT), including a GnRH antagonist (degarelix) and anti-androgen agent (bicalutamide). The PSA level immediately declined and reached nadir (0.23 ng/mL) after 8 months. However, 15 months after the start of ADT (PSA level, 2.33 ng/mL), the patient was diagnosed with CRPC and referred to our hospital.\nTo evaluate the patient's current disease status, we performed CT, bone scan, and WB-MRI. We observed discrepancies between the WB-MRI, bone scan, and CT. CT showed multiple osteoblastic lesions in the spine and pelvic bone (Figures and ) and shrunken para-aortic lymph nodes. Bone scan similarly showed multiple accumulations at the same bone sites as the CT (). However, these osteoblastic lesions showed almost no high signal in DWI of WB-MRI, suggesting that the lesions did not have viable tumor cells (Figures –). In contrast, the primary lesion had a diffuse high signal remaining in DWI of WB-MRI ().\nWe then performed histopathological examinations of both the prostate and the vertebra. The prostate needle biopsy demonstrated that 10 of 12 cores had viable prostate cancer cells (). Meanwhile, we performed the CT-guided needle biopsy from the osteoblastic lesion of the second lumbar vertebra, which was diagnosed with bone metastasis by CT and bone scintigraphy, to exclude vertebral bone metastasis of prostate cancer by an orthopedic unit of another hospital, which the patient visited owing to lumbago and bilateral lower limb paralysis. Histopathological examination of the osteoblastic lesion demonstrated no malignant cells (). Considering the results of the bone needle biopsy, we comprehensively reviewed the patient's image findings. A CT scan in the second lumbar vertebra revealed an osteoblastic change (885.4 Hounsfield unit), T1- and T2-weighted imaging of MRI revealed low signal intensity, and the ADC value was low (0.498 × 10−3 mm2/s); however, b = 0 and b = 1200 of DWI exhibited no high signal intensity, suggesting a benign osteoblastic change induced by the treatment effect (Figures –). With orthopedic conservative therapy in accordance with the diagnosis of lumbar hernia, the lumbago and bilateral lower limb paralysis completely resolved.\nBased on the findings of our WB-MRI and histopathological examinations, we reasoned that viable tumor cells inducing disease progression may primarily exist in the primary lesions and not in the metastatic lesions. Then, to control the overall disease, we changed the patient's medication from bicalutamide to enzalutamide for potentially existing micrometastases and added prostate RT (74 Gy). After this, his elevated PSA immediately declined and was controlled at a level of <0.2 ng/mL.

[[75.0, 'year']]

M

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{}

6011119-1

comm/PMC006xxxxxx/PMC6011119.xml

Aspirated Almond Masquerading as an Obstructing Endobronchial Mass Suspicious for Lung Cancer

A 64-year-old-man with an eighty-pack-year smoking history presented with insidious onset of worsening shortness of breath over a period of 6 months. He had previously undergone ablation for atrial fibrillation, as well as surgery and radiation for prostate cancer. Six months before presenting to our institution, he had fallen and fractured 2 right-sided ribs and developed an upper respiratory tract infection one month later. Over the next 3 months, the patient continued to experience intermittent fevers, chills, cough, wheezing, and exertional dyspnea.\nThe patient was initially treated with oral antibiotics for presumed community-acquired pneumonia; however, his symptoms persisted despite multiple courses of antibiotics and inhaled bronchodilators, and he was ultimately hospitalized. Serum serology for coccidioidomycosis was negative. Sputum cultures grew normal upper respiratory flora. His chest radiograph showed a left lower lobe (LLL) infiltrate. A computed tomogram (CT) of the chest showed a LLL consolidation, with evidence of narrowing of the LLL bronchus (). There was an abrupt cutoff in the LLL bronchus, but no endobronchial lesion was detected (). A bronchoscopy performed at the hospital showed a “rounded, nonulcerating, pink mass-like growth” obstructing the LLL bronchus. Brushings from the growth were negative for malignancy, but no biopsies were obtained. He was referred to our center for evaluation of this endobronchial lesion.\nUpon further examination, the patient recalled a significant decline in his exercise tolerance about 6 months prior to the current presentation. His pulmonary function tests showed severe airflow obstruction, with FEV1 of 1.53 L (42% predicted), which was a decline from his previous FEV1 of 2.2 L (63% predicted) 6 months earlier. The patient's physical examination revealed decreased breath sounds at the lower left lung base, with wheezing limited to the LLL. Although he had continued to smoke for the past 6 months, he had quit smoking 3 weeks prior to the current presentation. Based on his symptom complex, worsening airflow obstruction, and the mass seen on prior bronchoscopy, the likely differential diagnoses included primary lung cancer, lung carcinoid tumor, and foreign body aspiration. Although foreign body aspiration was unlikely, the development and progression of his symptoms over just 6 months would also be unusual for primary lung cancer or for lung carcinoid tumor.\nThe patient underwent another bronchoscopy, which revealed a narrow LLL bronchus, with a fleshy endobronchial mass lesion (). Multiple fine needle aspirates were obtained using a Wang needle (CONMED, Utica, NY), but every pass was positive for dense acute inflammation on rapid onsite evaluation. Multiple endobronchial biopsies were then obtained, and 2 pearly white structures with a brown detached cover were revealed (). The object was extracted in its entirety using a Zero Tip Airway Retrieval Basket (Boston Scientific, Marlborough, MA) and biopsy forceps; the object was identified as an aspirated almond. An ultrathin bronchoscope was then used to examine the airways distal to the aspirated almond, and there was no evidence of any additional foreign material. A bronchoalveolar lavage was performed at the end of the case, and specimens were sent for culture. Pathology of the extracted foreign body was consistent with vegetable matter (), and bronchoalveolar lavage cultures were negative for bacterial or fungal organisms.\nA 10-day course of amoxicillin-clavulanate was prescribed due to the imaging evidence of aspiration pneumonia secondary to almond aspiration. On follow-up examination one month later, he reported drastically increased exercise tolerance and his cough and wheezing had disappeared; meanwhile, pulmonary function tests showed an increase in his FEV1 to 2.12 L (60% predicted), and a repeat flexible bronchoscopy revealed no bronchial stricture. The patient shared that while he was recovering from his rib fractures, his favorite snack (enjoyed in the supine position) was almonds. While foreign body aspiration for 6 months could lead to irreversible parenchymal changes, a follow-up CT scan at 3 months revealed complete resolution of parenchymal infiltrates in this case (). At the time of his 6-month follow-up, his exercise tolerance had returned to baseline and he had successfully quit smoking.

[[64.0, 'year']]

M

{'25969517': 1, '29221325': 1, '33430107': 2, '10334153': 1, '16088484': 1, '11444115': 1, '9176648': 1, '28066626': 1, '29977638': 2}

{'7827418-1': 1}

6011120-1

comm/PMC006xxxxxx/PMC6011120.xml

Maturogenesis of an Immature Dens Evaginatus Nonvital Premolar with an Apically Placed Bioceramic Material (EndoSequence Root Repair Material®): An Unexpected Finding

A healthy 11-year-old girl was presented to the National University of Malaysia (UKM) Paediatric Dental Clinic with a referral for further management of pulp necrosis of an immature lower right second premolar (tooth 45), secondary to the fractured tubercle of dens evaginatus. Two weeks earlier, she had treatment at a general dental clinic for pain related to tooth 45. Tooth 45 had spontaneous and lingering pain following cold and thermal stimuli. The tooth was diagnosed to have symptomatic irreversible pulpitis, and root canal therapy was initiated. The canal was accessed, and pulp extirpation performed before the placement of intracanal nonsetting calcium hydroxide by the general dental practitioner (GDP).\nAt the time of current assessment, her tooth-related symptoms had completely resolved. General oral examination showed the presence of generalised mild gingivitis with a basic periodontal examination (BPE) score of 1 in all sextants. The patient's oral hygiene was fair with a plaque score of 30%. The patient is still in her mixed dentition with the presence of the primary maxillary canines. Her upper dental arch was well aligned, and mild crowding of anterior teeth was noted in the lower arch. Tooth 45 has an occlusal glass ionomer dressing of the access cavity made for the pulp extirpation earlier by the GDP (). Cold and electric pulp sensibility testings showed positive responses to all fully erupted premolars indicative of tooth vitality expect for tooth 45. Tooth 45 also has slight tenderness to percussion. Periapical radiograph of tooth 45 showed an immature root with convergent open apex and small periapical radiolucency. The pulp space of tooth 45 is of an even width from the coronal to the apical portion (). Based on the assessments, tooth 45 was diagnosed with pulp necrosis secondary to fractured dens evaginatus and symptomatic apical periodontitis.\nOn the day of initial assessment, tooth 45 was isolated with rubber dam after infiltration of local anaesthetic solution (2% lidocaine with 1 : 80000 adrenaline). Pulp chamber was reentered through the previously prepared access cavity. The root canal was exposed and irrigated with saline. After that, the canal was dried with paper points and the tooth working length was estimated with a K-file No. 60. A working length, 2 mm short of the apical opening, was determined (17 mm). The canal was gently prepared with the K-file No. 60 and then irrigated with a copious volume of 1.5% sodium hypochlorite (NaOCI). After drying the wet canal with paper points, nonsetting calcium hydroxide was placed into the canal and the access cavity was double sealed with Cavit™ 3M, USA, and glass ionomer cement (GIC) (Riva Self Cure™ SDI, Australia).\nTwo weeks later, the tooth was reassessed for any signs and symptoms of infection. The tooth was no longer tender to percussion, and there was no indication of infection-related signs and symptoms. After isolation with a rubber dam, the root canal of tooth 45 was reaccessed and irrigated with a copious volume 1.5% NaOCl to remove the nonsetting calcium hydroxide. Then, the canal was irrigated with sterile water and dried with paper points. Subsequently, the canal was irrigated with 17% EDTA (Pulpdent™, Watertown, Massachusetts) for a minute and dried with paper points. Finally, under the guidance of a dental operating microscope (Carl Zeiss Surgical GmbH, S100), the apical region was filled using the EndoSequence (BC RRM-Fast Set Putty, Brasseler, USA) material up to 4 mm thickness to create an apical seal (). The orifice of the root canal was double sealed with a cotton pellet, temporary filling material (Cavit 3M, USA), and GIC (Riva Self Cure SDI, Australia).\nOnce again, the root canal was reaccessed two weeks later, irrigated with 1.5% NaOCl, and dried with paper points. Next, the dried canal was obturated with thermoplasticised gutta-perca using the Obtura III Max System (Obtura Spartan® Endodontics) (). After that, the access cavity was double sealed with GIC (Riva Self Cure SDI, Australia) and nanohybrid composite (AURA™ SDI, Australia), respectively.\nFollowing the obturation, tooth 45 was reviewed at three-month and six-month intervals. During both reviews, tooth 45 was asymptomatic. However, at the six-month review, a periapical radiograph of tooth 45 showed an unexpected finding. The apical root of tooth 45 continued to grow beyond the apexification level with a normal periodontal ligament space and lamina dura. No evidence of periapical radiolucency was noted (). However, regular annual monitoring of tooth 45 is essential to ensure that the coronal seal is intact and no apical complication further arises.

[[11.0, 'year']]

F

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{}

6011124-1

comm/PMC006xxxxxx/PMC6011124.xml

Primary Angiosarcoma of the Breast after Bilateral Breast Reduction

The patient is a 50-year-old African American female with a history of bilateral breast reduction twelve years ago, iron deficiency anemia, and obesity, who presented to the surgeon's office complaining of tenderness of her right breast. The patient reported that recently she had been developing keloids along the scar of the right breast with some areas having a blue hue; her left breast was unremarkable. She noticed that after wearing a sports bra there was increased pressure and abrasions to the keloid, leading to cellulitis and edema. She was previously treated with two courses of antibiotics for what was presumed to be an infected keloidal scar of her right breast but with minimal improvement. On exam, she had a large 10 cm diameter keloidal region on the inferior and lateral aspect of the right breast with edema and cellulitis. The keloidal area had no palpable fluctuance; she exhibited no nipple discharge or palpable adenopathy of the right axilla ().\nThe patient had a benign-appearing mammogram 8 months prior, and all of her screening mammograms since her breast reduction have been without signs of malignancy. Another mammogram was ordered but was not performed due to patient discomfort. An ultrasound of the breast was preformed and suggested marked edema and skin thickening suggestive of infection but no definitive fluid collection or underlying suspicious mass was observed.\nThe patient underwent a right breast partial mastectomy for cosmesis and resection of the infected keloidal area. Intraoperatively, the mass was highly vascular, firm, but not fixed to the chest wall. Postoperatively, the pathology revealed a high-grade primary angiosarcoma of the breast with negative margins.\nPatient underwent a computed tomography of the chest, abdomen, and pelvis, which did not show any evidence of gross metastatic disease. The patient then underwent completion mastectomy and scheduled for adjuvant chemotherapy with combination gemcitabine and Taxotere, followed by radiation.

[[50.0, 'year']]

F

{'18521899': 1, '21779229': 1, '28794852': 1, '6402946': 1, '27191361': 1, '27042570': 1, '17541936': 1, '21697045': 1, '27128306': 1, '16000250': 1, '16288486': 1, '3179927': 1, '25659906': 1, '18809609': 1, '27099969': 1, '20537949': 1, '22611503': 1, '31360438': 2, '25504856': 1, '22907853': 1, '29977639': 2}

{'6649797-1': 1}

6011125-1

comm/PMC006xxxxxx/PMC6011125.xml

Endocarditis Caused by Gram-Negative Moraxella osloensis in an Immunocompetent Patient: First Case Report in Latin America

The 41-year-old male patient F.C.F. showed no comorbidities and denied alcoholism, smoking, and taking regular medication. He started having fever, myalgia, anorexia, vomiting, jaundice, and colure on July 14, 2016. He took antiviral drugs with monohydrate dipyrone (750 mg/day) associated to chlorpheniramine maleate (6 mg/day) and paracetamol (>4 g/day) at his peril. He denied taking any other drugs on that occasion. Because his clinical condition had not improved, he went to the hospital, where he underwent medical tests such as complete blood count (CBC): hemoglobin 13.2 g/dL, hematocrit 39.6%, platelets 343,000/mm3, white blood cells (leucocytes 5200/mm3), liver function Aspartate transaminase (AST) 39.8 U/L, Alanine transaminase (ALT) 40.1 U/L, gamma-glutamyl transferase (GGT) 346 U/L, and alkaline phosphatase 98.5 U/L. In addition, the NS1 test for dengue was negative and the abdominal ultrasound showed the liver with diffuse increase in parenchymal echogenicity, indicating mild/moderate steatosis. The patient was diagnosed with hepatitis due to the clinical signs assessed, results of laboratory tests, and excessive intake of paracetamol. The patient was hospitalized, and after 4 days of intravenous hydration and clinical assessment, he was discharged from the hospital.\nFifteen days after his discharge, the patient related that he had daily high fever, appetite loss, sweating, weight loss (8 kg), and progressive worsening. On August 6, 2016, the infectious disease specialist ordered new medical tests when examining the patient. The results revealed that blood count was hemoglobin 10.2 g/dL; hematocrit 30.7%; red blood cells 4.16 million/mm3; and platelets 200,000/mm3. It also revealed anisocytosis, microcytosis, and hypochromia; white blood cells were leucocytes 9700/mm3, banded neutrophils 6%, segmented neutrophils 76%, and lymphocytes 10%. Liver function tests indicated aspartate transaminase (AST) 64 U/L, alanine transaminase (ALT) 85 U/L, gamma-glutamyl transpeptidase (GGT) 560 U/L, and lactic dehydrogenase 248 U/L. C-reactive protein was 134.29 mg/L. Serologies were ordered for leptospirosis, rickettsialpox, hepatitis, syphilis, Zika virus, Chikungunya, Epstein-Barr virus, cytomegalovirus, toxoplasmosis, and human immunodeficiency virus (HIV). All of them had negative results.\nFurthermore, blood cultures were conventionally performed using the Bact/Alert 3D 120 automated microbial detection system. The initial incubation was performed with chocolate agar medium, in 5–10% CO2 at 35°C–37°C for 24 hours. Three blood samples were collected in the first 24 hours, at a minimum 15-minute interval between them, using venous puncture in different places. The colonies isolated on chocolate agar plates were identified by colonial morphology, gram stain, and oxidase reaction. Colonies appeared smooth, round, uniform, grey/brown, and 1 mm in diameter. The oxidase test and tributyrin agar test were positive. This result was positive for the presence of the microorganism M. osloensis in the three samples.\nTransesophageal echocardiography (TEE) was ordered, and it showed prolapsed mitral valve involving the posterior leaflet (P2 segment) with an echogenic structure adhered to the atrial face, with approximate dimensions of 0.47 × 0.85 cm, compatible with endocardial vegetation, determining failure of coaptation and a moderate degree of valvular insufficiency and eccentric jet. Intracavitary thrombi were absent, and systolic and diastolic functions of the left ventricle were normal. According to the criteria of Habib et al. [], the diagnosis of IE was confirmed, and the patient was hospitalized for intravenous antibiotic therapy.\nAntibiotic therapy was prescribed from August 8 to September 28 with the administration of vancomycin for four days, gentamicin for 10 days, and ampicillin for 6 days, and after the result of blood culture, ceftriaxone was introduced for 36 days. Remission of signs and symptoms was observed as of August 12. Laboratory tests results were better, and blood cultures were sterile. Theses exams were repeated on August 21 and on September 15. Their results showed no alteration.\nAfter completing the therapeutic treatment with specific antibiotics, the patient underwent serial laboratory tests and was hospitalized. Another TEE was carried (09/21), and it revealed chordal rupture and moderate mitral regurgitation. Surgical correction was performed.\nOn September 26, the patient underwent implantation of a mitral valve prosthesis. Surgery was performed without any complications. However, neither the culture nor the pathological anatomy of the surgically removed valve was performed. On October 06, the patient was discharged from the hospital with a prescription for oral anticoagulant warfarin.\nEighteen months after the endocarditis episode, the patient was asymptomatic, in good physical condition. He attends follow-up visits with a cardiologist at the same hospital.

[[41.0, 'year']]

M

{'8075270': 1, '1004377': 1, '15080311': 1, '27695673': 2, '10619749': 1, '11981649': 1, '7107844': 1, '26376092': 1, '5278896': 1, '7087280': 1, '26320109': 1, '23109812': 1, '24790917': 1, '20829400': 1, '26373316': 1, '25352503': 1, '16213607': 1, '29977621': 2}

{'5043396-1': 1}

6011128-1

comm/PMC006xxxxxx/PMC6011128.xml

Secret Sarcoma: A Cardiac Mass Disguised as Influenza

A 50-year-old Asian male with a past medical history of supraventricular tachycardia and obstructive sleep apnea on CPAP at night presented with one month of intermittent flu-like symptoms, orthopnea, and dyspnea on exertion. At the onset of these symptoms, he presented to a walk-in clinic and was diagnosed with influenza. He was treated symptomatically and noted improvement, but one week later he had a recurrence of symptoms while playing volleyball. From that time on, he noticed dyspnea on exertion, continued malaise, fevers, and diffuse joint pains so he presented multiple times to outpatient providers. He received doxycycline without improvement, and follow-up testing showed a mild leukocytosis, negative EBV, and an unremarkable chest X-ray. He was diagnosed with lingering postviral symptoms from influenza. He ultimately presented as a walk-in patient to the cardiology clinic when he started having chest tightness, palpitations, and his dyspnea progressed to occurring at rest, relieved only with a tripod position.\nEKG on presentation () showed right axis deviation and abnormal ST-T wave segments in V1 through V3 which was new compared with a prior EKG. Due to the concern for pulmonary embolism, a CT angiogram of the chest was obtained which displayed moderate bilateral pleural effusions, a mass in the right ventricle, and a mass in the left atrium extending through the mitral valve invading into the left ventricle (). Echocardiogram exhibited normal LVEF but some mitral valve occlusion due to the mass. Cardiac MRI was obtained () and confirmed the masses. The patient required debulking of the left atrial tumor, and pathology revealed an undifferentiated, high-grade pleomorphic sarcoma. Due to tumor infiltration into the left pulmonary veins, as well as focal areas of uptake in the small bowel at a site of intussusception, he was started on pembrolizumab chemotherapy with concurrent radiation therapy to the heart and small bowel.

[[50.0, 'year']]

M

{'19464464': 1, '16003450': 1, '21720466': 1, '8947350': 1, '1728367': 1, '232754': 1, '8215825': 1, '9726764': 1, '31089431': 2, '29977622': 2}

{'6476112-1': 1}

6011133-1

comm/PMC006xxxxxx/PMC6011133.xml

A Rare Adrenal Incidentaloma That Mimics Adrenocortical Carcinoma

A 64-year-old white male with no prior medical history presented to his primary care physician for routine follow-up. There was no history of hypertension. During work-up for elevated liver transaminases, he was found to have hepatitis C. Before initiation of Harvoni, he underwent CT imaging of the abdomen with contrast which found a 5 × 6.7 × 7 cm right adrenal mass (). On physical examination, he was afebrile with a pulse of 47 and normotensive at 118/68. His abdominal exam was nontender, nondistended, without masses, or hernias. Review of systems was negative for abdominal pain, hypertension, weakness, palpitations, headache, diaphoresis, or weight gain. He was a current smoker with a 33 pack-year history. He had no history of endocrine disease. His family history was significant only for a father with pancreatic cancer. His remaining laboratory values were within normal values including a normal potassium value. The patient was seen by the endocrine service for evaluation, and biochemical work-up revealed that the ACTH level was 9.1 pg/ml (nl 7.2–63.3); AM cortisol was normal at 10.01 mcg/dl, and 24-hour urine metanephrines was less than 50 mcg (nl).\nOn CT imaging, the right adrenal mass contained scattered calcifications with small regions of fat. It enhanced in a peripheral globular fashion with central progression. The absolute contrast washout of 22.9% was indeterminate for adrenal adenoma (). The mass was noted to abut but did not appear to invade the adjacent liver, right kidney, and inferior vena cava. There was no adenopathy or free fluid. There was no evidence of metastatic disease.\nDue to the size and atypical features of the mass, right adrenalectomy was performed. An open thoracoabdominal approach was chosen due to the patient's low lying costal margin, the size of the mass, and retrocaval extension of the mass medially towards the vertebral body. The patient recovered well postoperatively and was discharged four days after surgery. The resected specimen weighed 126 grams and measured 7.5 × 6.5 × 4.7 cm on gross pathology (). Within the specimen was a 6.4 × 5.5 × 4.7 cm intraparenchymal nodule, which on histologic examination proved to be a cavernous hemagioma (). The patient has had no evidence of recurrence for nearly 18 months.

[[64.0, 'year']]

M

{'27070729': 1, '22623268': 1, '26793524': 1, '22863384': 1, '26600897': 1, '10732270': 1, '27390021': 1, '31949968': 2, '17287480': 1, '8379804': 1, '24441435': 1, '16699294': 1, '24865537': 1, '22019159': 1, '29984033': 2}

{'6944974-1': 1}

6011134-1

comm/PMC006xxxxxx/PMC6011134.xml

The Rising Triad of Cesarean Scar Pregnancy, Placenta Percreta, and Uterine Rupture: A Case Report and Comprehensive Review of the Literature

A 34-year-old Gravida 11 Para 3073 at 16 weeks and 1 day gestation presented to the emergency room of an outside hospital with a 2-day history of progressively worsening nausea, vomiting, and diarrhea, exacerbated by eating. The pregnancy had been unremarkable. Her past medical history included endometriosis and infertility. Her past surgical history was significant for two cesarean sections and left salpingo-oophorectomy secondary to an ectopic pregnancy. Physical exam elicited severe, diffuse abdominal tenderness. Fetal heart tones were taken to be in the 140s and positive fetal movement was reported. Laboratory investigations, including complete blood count, comprehensive metabolic panel, amylase, and lipase, were within normal limits. The ER physician's leading differential diagnosis was of gastrointestinal etiology. An MRI and MRCP were performed to rule out appendicitis and gallbladder disease. The MRI was notable for a large amount of intraperitoneal fluid of unknown etiology; an intrauterine fetus was visualized.\nThe patient continued to experience intractable pain, worse with movement and breathing, despite IV pain medication. At that point she has been at the outside facility for approximately 12 hours. The patient was transferred to our facility under the joint care of the Obstetrics/Gynecology and General Surgery teams. Upon arrival, the patient's hemodynamic status had deteriorated. She presented with tachycardia, dyspnea, chest pain, and worsening abdominal pain. Her hemoglobin had fallen from 11.7 g/dL to 7.9 g/dL. Transabdominal ultrasound imaging revealed a single intrauterine pregnancy that was positioned low in the uterus, with marked thinning of the anterior myometrium at the site of the pregnancy, and significant hemoperitoneum. Fetal heart tones were steady in the 140s. The MRI images were reevaluated prior to surgery (see ).\nAt this point, the patient was taken for emergency laparotomy and the staff Gynecologic Oncologist was consulted. The patient underwent a modified radical hysterectomy with right ureteral lysis and cystotomy with bladder repair. The intraoperative findings were consistent for a placenta percreta and uterine rupture with a 2 x 1 cm defect in the right lower uterine segment. There were significant intra-abdominal blood and evidence of invasion of the placenta into the posterior aspect of the bladder. Total estimated blood loss for the surgery was 3,150 mL. The patient received 900 mL of cell saver and 1 unit packed red blood cells (PRBC) intraoperatively.\nThe patient was admitted to the ICU following surgery. She was transferred out of the unit on postoperative day 1. Two more units of PRBC were transfused over the course of the postoperative period. She was discharged on postoperative day 4 after having met her postoperative milestones. Due to the cystotomy, she was discharged with Foley urinary catheter in place for a minimum of 7 days with cystogram scheduled prior to removal. Patient was referred for grief counseling.\nPathologic examination of the uterus included placenta percreta with uterine rupture (see for gross specimen). There was absence of decidua identified in the lower uterine segment in the area of the uterine rupture.

[[34.0, 'year']]

F

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{}

6011142-1

comm/PMC006xxxxxx/PMC6011142.xml

Misinterpretation of Psychiatric Illness in Deaf Patients: Two Case Reports

The first case concerns a 26-year-old prelingually Deaf male, with a prior history of Tourette's syndrome, bipolar disorder, and HIV, who was placed under a Baker Act at a local hospital for “acting erratic and psychotic.” A Baker Act is a 72-hour involuntary psychiatric hold within the state of Florida that can be initiated by healthcare professionals and police officers in the event of a patient being a danger to self or others. The preliminary diagnosis on the involuntary form, as per the emergency room physician, was “psychosis.” The patient was subsequently given an emergency treatment order of intramuscular lorazepam and was transferred to a psychiatric hospital where he was observed by nursing as “calm and nonthreatening.”\nPrior to initial psychiatric interview, an ASL-interpreter was called to assist. The patient asked where he was at and became angry after discovering the truth of his hospitalization. He reported he initially came to the hospital as he had been having anxiety and physical pain attributed to his Tourette's Disorder. He reported his neurologist had him on carisoprodol and diazepam to help relieve these symptoms, but that they were stopped one month prior. The family was called and stated there was questionable abuse of medications but they were adamant that he was safe for himself and others.\nWhen the patient was seen by the ED physician initially there was no interpreter present. The patient reported becoming frustrated and was trying to sign aggressively which he believes was misinterpreted. He also expressed in spoken word to the staff there that he had been “hearing voices” secondary to his pain level. He purportedly was never told what was occurring prior to seeing the interpreter at the transfer facility nearly 12 hours later. The patient adamantly denied SI, HI, AVH, or mania and maintained a linear and coherent thought process. He expressed a history of bipolar disorder which had been diagnosed after a similar incident in the past. He had been on several antipsychotics previously but had not taken any for several years without incident. He had only been taking anxiolytics and pain meds for multiple years which he felt stable on, as well as antiretrovirals for his HIV diagnosis.\nThe patient later admitted that he had been buying oxycodone off the street since his neurologist had stopped prescribing medications due to questionable abuse. A clinical opiate withdrawal scale was performed and was only positive for minor anxiety elevation. A full medical workup was performed and excluded any medical causes to his admission. Through further interview, OCD was excluded as a diagnosis but substance use disorder remained high on the differential for his current and past behavior. The patient was kept overnight for observation and discharged the next morning following positive report from staff. He was given extensive education on substance use as well as coping strategies to prevent readmissions. Upon discharge “unspecified psychosis” was given as his diagnosis.

[[26.0, 'year']]

M

{'16510696': 1, '6731642': 1, '17903259': 1, '23507810': 1, '33170422': 1, '23538291': 1, '21327903': 1, '34948509': 1, '21700590': 1, '12114142': 1, '22423884': 1, '29984029': 2}

{'6011142-2': 2}

6011142-2

comm/PMC006xxxxxx/PMC6011142.xml

Misinterpretation of Psychiatric Illness in Deaf Patients: Two Case Reports

The second case involves a 30-year-old Deaf, Hispanic male who presented to the Emergency Department after his mother reported that the he was behaving oddly and not taking his risperidone. Per reports, the patient was talking to his mother about going places in a UFO and exhibiting disorganized and illogical behaviors. He was subsequently placed under a Baker Act by the emergency room physician who documented that the patient was exhibiting auditory hallucinations. Initially an interpreter was brought to the hospital prior to his admission. Per the ASL-interpreter, the patient stated that he felt “fine and not crazy” and that all of these events are happening because his mother does not “understanding Deaf culture.” He also conveyed that he did not like to take his meds because they interfered with him being able to drink alcohol and caused drowsiness.\nUpon initial psychiatric interview an interpreter was not present as the hospital only agreed to set periods of time for the interpreter. As an effort to communicate, questions were prepared for the patient to answer via written responses. highlights a portion of the questions and answers that were constructed. From the responses he maintained bizarre delusions but denied current SI, HI, or AVH. When the ASL-interpreter arrived, the patient appeared jovial and yearned to express himself. The interpreter stated she had difficulties reading his rapid signing at first and had to have him slow down several times. However she did note that this was a common occurrence when addressing Deaf individuals.\nWith the interpreter's assistance, the patient was answering questions logically with a linear thought process. He reported that he had been diagnosed with schizophrenia as a teenager after having several interpersonal issues with his mother. She is Spanish speaking only and he stated that she has never fully understood how to communicate effectively with him. He had been taking risperidone for several years but was tired of continuing with the medication due to the side effects of drowsiness and weight gain, which he was never able to fully discuss with his psychiatrist. Patient reported he was in an ASL school and learning a career in massage therapy. After meeting a girlfriend there he began to develop a sense of independence that he reported his mother disapproved of. This caused an altercation that he reports his mother misinterpreted which precipitated his admission.\nThe patient continued to express that he was abducted by aliens as a child and could understand their language, but besides this he expressed no other psychotic processes. He was observed for two days without medications and remained calm/cooperative but was unable to participate in most activities due to limitations of the interpreter availability. After a family session was completed the patient was discharged home with plans to follow up with his community psychiatrist. The patients' diagnosis was changed to delusional disorder upon his discharge.

[[30.0, 'year']]

M

{'16510696': 1, '6731642': 1, '17903259': 1, '23507810': 1, '33170422': 1, '23538291': 1, '21327903': 1, '34948509': 1, '21700590': 1, '12114142': 1, '22423884': 1, '29984029': 2}

{'6011142-1': 2}

6011145-1

comm/PMC006xxxxxx/PMC6011145.xml

Cycling Induced Spontaneous Coronary Artery Dissection in a Healthy Male

A 36-year-old male, a seasoned cyclist with no past medical history, presents to the emergency department with complaints of lightheadedness and diaphoresis after a bicycle fall. Patient was participating in a bicycle race when another rider ahead of him fell causing the patient to swerve to avoid him. Patient states that he fell on his left side and hit a tree with his right leg. Patient was wearing a helmet and did not suffer any chest or head trauma. After the fall, he felt lightheaded and diaphoretic and complained of mid back pain. Patient denied any chest pains or shortness of breath. Patient was subsequently brought to the hospital directly following the accident by ambulance.\nIn the emergency department, patient was noted to be in no acute distress; initial blood pressure was 128/69 mmHg with pulse of 65 beats per minute. He was afebrile, not tachypneic, and well appearing with marked right thigh swelling and tenderness to his medial thigh. Given the dizziness and diaphoresis initially, patient had an ECG performed which showed lateral ST segment elevation () and had a subsequent troponin I that was positive, 0.49ng/mL, with a Creatine Phosphokinase (CPK) of 617 U/L.\nThere was initial concern for a possible cardiac contusion, although the patient had no chest wall trauma and thus was admitted for further evaluation. As an inpatient, an echocardiogram was performed demonstrating normal right and left ventricular function and trace pericardial effusion while the patients troponin continued to trend upwards towards a maximum of 21ng/mL. He was loaded with Aspirin and Clopidogrel as well as initiation of a heparin infusion, Lisinopril, and a Beta Blocker. Coronary angiography was subsequently performed demonstrating a spontaneous coronary artery dissection of left anterior descending coronary artery. No further diagnostic study was performed at that time. Further history revealed that he took multiple caffeine Jello shots and drank a large cup of coffee prior to participation in the race. He denied cocaine, amphetamine, or other performance enhancing drug use ().\nThe patient's CPK and troponin trended downwards on conservative medical management and his back pain resolved; therefore a stent was not placed. The patient was visiting from outside the area; discharge planning included repeat coronary angiography in 6 weeks and instructions that he will not be able to perform competitive cycling again. Should his dissection extend at that period of time or patient become symptomatic, stent placement would be considered. Patient was to continue the Aspirin and Clopidogrel until the repeat angiography was performed. Patient was discharged with plans to follow up with a cardiologist in his home state. Multiple follow-up phone calls made us unable to reach the patient and he was subsequently lost to follow-up.

[[36.0, 'year']]

M

{'12403896': 1, '23272729': 2, '7606975': 1, '18830003': 1, '14653061': 1, '16645363': 1, '17762352': 1, '25442447': 1, '22800851': 1, '28766909': 1, '27023796': 1, '20440039': 1, '22045090': 1, '28402237': 1, '29479014': 1, '23339097': 1, '7721492': 1, '25060027': 1, '29984009': 2}

{'3539931-1': 1}

6011150-1

comm/PMC006xxxxxx/PMC6011150.xml

A Rare Case of Complete Heart Block in a Young Patient

A 30-year-old male with history of active smoking (1 pack per day for 10 years) and external hemorrhoids came to the preop anesthesia clinic for anesthesia evaluation fitness and was found to have high blood pressure (BP) (234/144). He was referred immediately to the emergency room (ER) for BP control. In the ER, BP was 221/125 and heart rate (HR) was 50 beats/minute. Routine electrocardiogram (EKG) showed 3rd-degree heart block (TDHB) and left ventricular hypertrophy (LVH) with strain pattern (). He denied chest pain, palpitation, dyspnea, dizziness, or syncope. The patient was started on antihypertensive medication for BP control and was admitted to the cardiology ward for evaluation and management of complete heart block. Further physical exam revealed absent arterial pulses except the left radial pulse which was weak. BP was significantly different between both upper limbs and between upper and lower limbs (right upper limb 126/86 and lower limb 85/54, left upper limb 145/85 and lower limb 75/50). His initial blood work showed mild renal impairment.\nComputerized tomography (CT) thoracic aortogram was done to rule out coarctation of the aorta, which was normal; CT coronary angiogram showed no evidence of coronary artery disease (CAD). Magnetic resonance imaging (MRI) of the heart was normal as well. Transthoracic echocardiogram (TTE) showed moderate hypokinesia of the left ventricle (LV), ejection fraction (EF) 35–40%, grade 2/4 diastolic dysfunction, and moderate concentric LVH. Holter monitoring did not reveal any pauses. Ultrasound/Doppler of the kidneys showed increased parenchymal echogenicity with poorly defined corticomedullary junction impressive of renal parenchymal disease. CT abdominal aortogram showed large thrombus seen in the abdominal aorta starting at the level of renal arteries completely occluding the aorta and common iliac arteries with no blood flow seen beyond the renal artery level up to the aortic bifurcation; moderate to severe stenosis is noted at the origin of both renal arteries because of thrombus (Figures and ). Multiple abdominal collaterals are seen with multiple collaterals in the anterior and lateral abdominal wall and paraspinal collaterals (). Extensive blood work-up including thyroid function test and autoimmune and thrombophilia work-up was all unremarkable. No cause of aortic thrombosis and TDHB was identified.\nInitial recommendation of the vascular surgeon was to follow up in the clinic with no intervention as it is a chronic process, and the patient was asymptomatic. Since the patient had uncontrolled hypertension despite being on maximum doses of four antihypertensive medications, eventually he underwent percutaneous stenting of bilateral renal arteries which was followed by an improvement in the BP and renal function and reduction in doses of antihypertensive medications. The patient also underwent permanent pacemaker insertion for TDHB. The patient was also placed on warfarin and was advised to see the vascular surgeon after 3 months. Unfortunately, the patient did not follow up.

[[30.0, 'year']]

M

{'23473760': 1, '21263005': 1, '23801822': 1, '21665919': 1, '17859070': 1, '5775291': 1, '16508591': 1, '15583080': 1, '17896999': 1, '10447229': 1, '18498951': 1, '2957590': 1, '11220567': 1, '29984004': 2}

{}

6011152-1

comm/PMC006xxxxxx/PMC6011152.xml

Never Too Old? Occurrence of Medulloblastoma in the Elderly beyond the 70th Year of Life

Case 1 concerned a 71-year-old male presenting with gait difficulties and vertigo. Cranial MRI revealed a low and focal contrast-enhancing nodular tumor in the left cerebellar hemisphere and upper vermis (). The lesion appeared hypointense relative to gray matter on T1 weighted (T1w) images and moderately hyperintense on T2w images. Tumor margins were best displayed on diffusion weighted images (DWI). The suspected clinical diagnosis was metastasis from an unknown primary tumor. Microsurgical resection of the tumor was performed. Histopathological work-up revealed a highly cellular tumor consisting of small cells with scant cytoplasm and round-oval or pleomorphic, hyperchromatic cells in the cerebellum. A part of the tumor showed a nodular architecture and a desmoplastic component (Gomori staining). Some tumor cells expressed the neuronal differentiation marker synaptophysin. The diagnosis was paucinodular desmoplastic MB (WHO grade IV). Tumor cells showed nuclear YAP1 and cytoplasmic GAB1 staining, while nuclear staining for ß-catenin and staining for p53 was negative. There was no MYC- or MYCN-amplification detectable (). The patient's condition was stable in the continuing course; however a week later he was affected by a severe pneumonia and died due to respiratory insufficiency.

[[71.0, 'year']]

M

{'19118784': 1, '27588198': 1, '17487854': 1, '26026861': 1, '12377339': 1, '26706607': 1, '27106407': 1, '29984017': 2}

{'6011152-2': 2}

6011152-2

comm/PMC006xxxxxx/PMC6011152.xml

Never Too Old? Occurrence of Medulloblastoma in the Elderly beyond the 70th Year of Life

Case 2 was a 72-year-old male who was referred to the hospital because of change of personality and loss of weight. Cranial MRI showed a large low contrast-enhancing mass in the right cerebellar hemisphere consisting of a lateral solid component and a small medial cystic. The tumor caused occlusive hydrocephalus but no surrounding edema (). MR revealed diffusion restriction of the solid tumor part and peripheral susceptibility effects, e.g., hemosiderin deposits. Once again, the first suspected diagnosis was metastasis without presence of any neoplasm in the patient history; the second radiological diagnosis was MB. The possibility of a high-grade glioma was discussed but neglected due to its rare occurrence in the cerebellum in this age group. Prior to surgery an external ventricular drainage was inserted. Complete tumor resection was performed. Histopathological examination showed a highly cellular cerebellar tumor consisting of sheets of uniform cells with a high nuclear/cytoplasmic ratio and round to oval hyperchromatic nuclei. Many tumor cells reacted for synaptophysin. There was no evidence of a nodular or desmoplastic component in the Gomori staining. The diagnosis was that of a classical MB (WHO grade IV) (). The tumor cells did not show staining for YAP1, GAB1, and p53 or nuclear staining for ß-catenin. Evidence of MYC- or MYCN-amplification was not found. The postoperative course was uneventful and the ventricular drainage was removed without evidence of an enlarged ventricular system. However, the patient was found dead seven days later in his room. The cause of unexpected death could not be clarified, since an autopsy was not allowed.

[[72.0, 'year']]

M

{'19118784': 1, '27588198': 1, '17487854': 1, '26026861': 1, '12377339': 1, '26706607': 1, '27106407': 1, '29984017': 2}

{'6011152-1': 2}

6011155-1

comm/PMC006xxxxxx/PMC6011155.xml

Septic Infrapatellar Bursitis in an Immunocompromised Female

An 86-year-old female with a history of metastatic ovarian cancer presented to the ED with painful bilateral lower extremity edema and a left lateral leg ulceration. Her metastatic ovarian cancer had been diagnosed by malignant pleural effusion five months earlier, and she had completed neoadjuvant chemotherapy with carboplatin and Taxol approximately one week prior to this presentation. She was admitted to the hospital and started on cefazolin for left lower extremity cellulitis on hospital day one.\nOn admission, plain films and ultrasound did not reveal any evidence of osteomyelitis, fracture, DVT, or abscess to the left lower extremity. On exam, she had 3+ pitting edema below the knee bilaterally as well as chronic venous stasis changes. The patient also had a venous ulcer (approximately 2 cm in diameter) on the anterolateral aspect of the distal third of her left lower leg. At the time of admission, this venous ulcer had some serous weeping but no purulent drainage or fluctuance on examination. Her initial Laboratory Risk Indicator for Necrotizing Fasciitis (LRINEC) score was 4, suggesting a low risk for necrotizing fasciitis; however, on hospital day 3, her CRP began to uptrend and she became febrile. At this point, her antibiotics were switched from cefazolin to vancomycin to cover MRSA.\nOn hospital day five, the patient was noted to have a new erythematous area over the anterior left knee, inferior to the patella (). Ultrasound revealed a small fluid collection superficial to the patellar tendon in the infrapatellar region measuring 3.3 × 2.5 × 0.4 cm (). The infrapatellar bursa was aspirated and sent for culture. The patient was started on piperacillin-tazobactam, given the patient's immunocompromised status and subsequent risk for atypical and gram-negative organisms.\nAn MRI was performed on hospital day seven (this was delayed due to the patient's pacemaker) but did not reveal any evidence of osteomyelitis. The patient was clinically improved after starting piperacillin-tazobactam, and vancomycin was discontinued on hospital day seven. On hospital day eight, aspirate cultures returned with Pseudomonas aeruginosa; she was stable for discharge at that time and was sent out with a ten-day course of levofloxacin (culture was pan-sensitive) and close follow-up with infectious disease.

[[86.0, 'year']]

F

{'19038882': 1, '28555416': 1, '24371726': 1, '26745168': 1, '21628647': 1, '22957333': 1, '24305696': 1, '11254233': 1, '23188870': 1, '7667644': 1, '16297744': 1, '28290630': 1, '21183828': 1, '29984025': 2}

{}

6011161-1

comm/PMC006xxxxxx/PMC6011161.xml

Tibia Adamantinoma Resection and Reconstruction with a Custom-Made Total Tibia Endoprosthesis: A Case Report with 8-Year Follow-Up

A 48-year-old female with known breast carcinoma was screened for possible dissemination with whole-body computed tomography (CT) and a bone scintigraphy scan. The bone scan revealed a tumor in the entire right tibia. The patient reported no symptoms from the tibia tumor. A plain X-ray and magnetic resonance image (MRI) confirmed an intraosseal tumor that extended from 4 cm below the knee joint proximally to about 4 cm from the ankle joint distally (). An open biopsy confirmed an adamantinoma histology. Different treatment options were thoroughly discussed with the patient, including a lower leg amputation with disarticulation of the knee, a total tibia resection and reconstruction with a tibia allograft, or a custom-made tibia EPR, which was eventually selected.\nThe tumor was resected with an extensive anteromedial approach, and the defect was reconstructed with a custom-made, silver-coated, modular endoprosthesis of the Modular Universal Tumor and Revision System (Implantcast®, Buxtehüde, Germany) (). The knee joint was reconstructed with a metal-on-poly articulation with a (unique) metal-on-metal hinge mechanism (). The ankle joint was reconstructed with a metal-on-poly hinge joint with a talar replacement, stabilized with a trans-talar and trans-calcanear hydroxyapatite-coated stem. A supplementary screw was used to add stability in the subtalar joint. The endoprosthesis was enveloped in a Trevira (Implantcast®) tube to facilitate the attachment of soft tissues and the patella tendon (). A microvascular latissimus dorsi musculocutaneous flap was anastomosed to the tibia artery (end-to-side) and concomitant vein and wrapped around the prosthesis to avoid dead space and allow tension-free closure. In addition, a medial gastrocnemius muscle flap was transposed to cover the patellar tendon region; this was covered with a meshed split-thickness skin graft.\nA histological analysis of the resected specimen showed an adamantinoma that had spread throughout the entire tibia and the margins were wide: an unaffected periosteum as an anatomic barrier and a minimum of clear soft tissue margin of 3 mm. Due to intracortical location of the tumor, no massive muscle excision was needed.\nThe knee joint was immobilized in extension for 6 weeks to facilitate patella ligament attachment to the tube. Then, the joint was gradually mobilized. After 6 months, the patient's gait was almost normal, with mild limping. Local MRI and chest radiographs performed during the 8 years of follow-up showed no signs of local recurrence or distant metastasis. No loosening of the stem or other mechanical problem was reported. In a routine follow-up, in addition to radiographs, we used a local MRI with a metal artifact reduction sequence (MARS) technique [], which enables to observe local recurrences of the tumor around the massive titanium endoprosthesis.\nEight years postoperatively, the patient had most of the time no pain and could mobilize freely. The patient resumed working full-time as a kindergarten teacher, and she has maintained her previous active lifestyle (except downhill skiing). On her latest follow-up visit (at 8 years), the knee range of motion was 0–105 degrees, ankle dorsiflexion was 5 degrees, and ankle flexion was 35 degrees. In the latest follow-up visit, we used 4 patient-related outcome (PRO) measures. The Musculoskeletal Society Tumor Score (MSTS) was 77%; the Oxford Knee score (OKS) was 35/48; the Toronto Extremity Salvage Score (TESS) was 80/100; and the 15D was 0.87/1.\nThe metal-on-metal prosthesis caused an increase in metal ion concentrations: cobalt was 6 ppb and chromium was 8 ppb. The silver coating created a mild, local skin argyria pigmentation, with cosmetic discomfort [] ().

[[48.0, 'year']]

F

{'18057365': 1, '20119985': 1, '17368533': 1, '34007499': 2, '23814255': 1, '23369129': 1, '28088378': 1, '2743266': 1, '10954102': 1, '19048403': 1, '17375547': 1, '24378391': 1, '28899125': 1, '16470458': 1, '28343825': 1, '18060426': 1, '25628291': 1, '22675031': 1, '18279517': 1, '34326904': 2, '28265758': 1, '31190111': 1, '29984021': 2}

{'8110409-1': 1, '8110409-2': 1, '8311747-1': 1}

6011162-1

comm/PMC006xxxxxx/PMC6011162.xml

Graves' Disease Presenting with Periodic Paralysis to the Emergency Department

A 26-year-old male healthy Asian student presented to an emergency department in Australia with sudden onset generalised weakness affecting predominantly his lower limbs after eating dinner. The patient reported difficulty standing and difficulty lifting his arms without any muscle pain or paraesthesia, headache, or back pain. Although he had experienced multiple similar episodes over the past month, these had been less severe and always self-resolved. It was unclear to the patient if these episodes of weakness were associated with food intake or exercise.\nOn further questioning, the patient reported 15-kilogram weight loss over the past three months and a four-day history of nonbloody diarrhoea, which resolved one week prior to presentation. He had otherwise been well and was playing soccer regularly. He had no relevant family history, was on no regular medications, and denied using illicit drugs.\nOn examination, the patient appeared mildly diaphoretic but was afebrile. Heart rate was irregular, at 92 beats per minute and blood pressure was 118/60 mmHg. He had a normal respiratory rate at 18 breaths per minute with oxygen saturations of 98% on room air. Neurological examination revealed symmetrical proximal weakness of upper and lower limbs with normal tone, reflexes, and sensation. The decrease in power was more noticeable in the lower limbs compared to the upper limbs. In addition, there was a mildly enlarged painless thyroid gland, with a slight hand tremor, but no signs of thyroid acropachy or thyroid eye disease. Heart sounds were normal with no murmurs, gallops, or rubs, and lung fields were clear to auscultation and percussion. There was no abdominal tenderness to palpation.\nBedside electrocardiogram revealed atrial flutter with a variable ventricular rate. Initial biochemistry showed hypokalaemia with potassium of 2.3 mmol/L (reference range: 3.5–5.2 mmol/L) and hypomagnesaemia with magnesium of 0.59 mmol/L (reference range: 0.70–1.10 mmol/L). Plasma glucose level was 7.1 mmol/L. Complete blood count, urea and creatinine, liver function, phosphate, and creatinine kinase were unremarkable.\nHypokalaemic periodic paralysis was suspected and thyroid function testing was performed. This revealed hyperthyroidism with a TSH of <0.01 mU/L (reference range: 0.40–4.00 mU/L), T4 of 44 pmol/L (reference range: 9–19 pmol/L), and T3 of 25 pmol/L (reference range: 3.0–5.5 pmol/L), suggesting a diagnosis of TPP. TSH receptor antibodies were elevated at >40 U/L (reference range: <1.8 U/L), confirming a diagnosis of Graves' disease. This was further supported by a radionuclide thyroid scan showing diffuse uptake in the thyroid gland. Antithyroperoxidase antibodies, antithyroglobulin antibodies, and urinary electrolytes were not measured in this case.\nInitial treatment in the emergency department included 40 mmol of intravenous KCl and 10 mmol of intravenous MgSO4. The patient's weakness resolved during inpatient admission. Potassium was 4.7 mmol/L and magnesium was 0.88 mmol/L on repeat testing 6 hours after the initial biochemical tests. Thereafter, potassium remained normal throughout the admission, without rebound hyperkalaemia or the need for further replacement. The patient was discharged home the next day on Carbimazole 20 mg twice a day and Propranolol 20 mg three times a day. No potassium supplementation was prescribed on discharge and he was not started on anticoagulation for the atrial flutter due to his low risk of thromboembolism.\nOne month after discharge, the patient reported no further episodes of weakness. His T4 and T3 had normalised, although TSH remained at <0.01 mU/L. His potassium was normal at 4.1 mmol/L. Ongoing monitoring of thyroid function was recommended, with a plan for radioiodine ablation or thyroid surgery if antithyroid medical therapy was unsuccessful. However, further follow-up information was not attainable as the patient returned to his home overseas.

[[26.0, 'year']]

M

{'20089526': 1, '13481091': 1, '22460532': 1, '32443393': 2, '2818118': 1, '24695373': 1, '23939916': 1, '30567254': 1, '6017520': 1, '16608889': 1, '29984010': 2}

{'7277936-1': 1, '7277936-2': 1, '7277936-3': 1}

6011163-1

comm/PMC006xxxxxx/PMC6011163.xml

Diffuse Alveolar Hemorrhage Induced by Vaping

Thirty-three-year-old male with diabetes and seizure disorder presented to the emergency department (ED) with worsening dyspnea and hemoptysis. Two weeks prior to his ED presentation, he was treated with antibiotics for community acquired pneumonia with minimal improvement. Upon further inquiry, patient admitted to vaping for the past 2 months with overtly increased exposure time and has experimented on new flavors. He denied previous or current recreational drug use. CT scan of the chest showed diffuse ground glass opacities and bilateral patchy consolidation (). He had worsening hypoxia that required noninvasive ventilation. His echocardiogram was otherwise normal. Bronchoscopic examination failed to demonstrate airway lesions. Bronchoalveolar lavage (BAL) revealed increasing blood in four sequential aliquots confirming diagnosis of DAH (). BAL cell count showed greater than 30,000 RBCs and 800 WBCs, 42% neutrophils, 36% lymphocytes, 1% eosinophils, and 21% macrophages. All inflammatory serologies were unremarkable: erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), rheumatoid factor (RF), antinuclear antibody (ANA), and anti-antineutrophil cytoplasmic antibodies (ANCA). In addition, serum eosinophil count, anti-glomerular basement membrane (GBM) antibodies, and anti-phospholipid antibodies were all normal. Urine toxicology screen which includes amphetamines, cannabinoids, and cocaine was negative. There was no microbiologic growth on all BAL specimens. Patient was treated with pulse dose steroids after DAH was confirmed with BAL aliquots (). He underwent right wedge resection lung biopsy which revealed evidence of bland pulmonary hemorrhage () with no evidence of capillaritis or diffuse alveolar damage (DAD). Prussian blue iron staining was also noted which reflects old hemorrhage (). His symptoms improved with complete resolution of alveolar hemorrhage on chest CT scan after 2 weeks (). His steroids were tapered quickly and he has not used a personal vaporizer since then.

[[33.0, 'year']]

M

{'24732162': 1, '32025438': 2, '33102141': 1, '26776875': 1, '33560943': 1, '32685366': 1, '31899452': 1, '33110741': 1, '28235965': 1, '32016924': 1, '31753841': 1, '31956478': 1, '21943539': 1, '32030277': 1, '32584238': 1, '31897566': 1, '32243764': 1, '32802422': 1, '33194132': 1, '33186869': 1, '32246394': 1, '23678356': 1, '25136064': 1, '31667071': 1, '32230711': 1, '32211613': 1, '32690998': 1, '34360499': 1, '20442117': 1, '33100546': 1, '31570493': 1, '28392919': 1, '33753133': 1, '32462343': 1, '28642230': 1, '31583455': 1, '25456810': 1, '33717828': 1, '27472774': 1, '32440996': 1, '32327029': 1, '24462024': 1, '32393606': 1, '25381306': 1, '29984031': 2}

{'6991143-1': 1}

6011165-1

comm/PMC006xxxxxx/PMC6011165.xml

Bradycardia during Induction Therapy with All-trans Retinoic Acid in Patients with Acute Promyelocytic Leukemia: Case Report and Literature Review

A 41-year-old man without any underlying diseases such as cardiovascular disease was hospitalized for spontaneous gum bleeding, epistaxis, and lower limb ecchymosis. Laboratory data on the admission date showed leukocytosis (WBC, 15,820/mm3; promyelocyte, 66%, with Auer rod), anemia (Hb, 9.5 g/dL), thrombocytopenia (PLT, 22,000/mm3), and abnormal coagulation profile (fibrinogen, 29 mg/dL; fibrin degradation product (FDP), 68.5 mcg/mL; d-dimer, 19.81 mcg/mL; prothrombin time (PT), 20.5 sec; international normalized ratio (INR), 1.92; partial thromboplastin time (PTT), 29 sec). Other laboratory data were as follows: C-reactive protein (CRP), 8.97 mg/dL; total bilirubin (T-bil), 0.73 mg/dL; aspartate aminotransferase (AST), 60 U/L; alanine transferase (ALT), 100 U/L; and serum creatinine (Scr), 0.9 mg/dL. His baseline electrocardiogram (ECG) was normal (). Bone marrow aspiration and biopsy disclosed APL with PML-RARα. ATRA at a dose of 45 mg/m2/day (40 mg twice daily) was administered. On the third day of therapy, oxygen saturation abruptly dropped to 90% without oxygen supplementation. Chest X-ray (CXR), ECG, and echocardiography did not show any abnormalities. In order to prevent DS, intravenous methylprednisolone was administered at a daily dose of 80 mg–120 mg according to the clinical signs and symptoms. Idarubicin (12 mg/m2/dose) was administered starting on the fourth day for four doses. WBC progressively elevated to 46,830/mm3; therefore, 1000 mg hydroxyurea twice daily was also added starting on the seventh day.\nOn the 11th day of therapy, WBC and promyelocyte decreased to 6,310/mm3 and 14%, respectively. ATO infusion at a dose of 0.15 mg/kg was initiated; however, dizziness and chest pain occurred during infusion. ECG showed complete AV block with a heart rate (HR) of 40–50 beats/min (bpm) (). The electrolytes and liver function tests were all within normal limits (Na, 138 mmol/L; K, 4.6 mmol/L; Ca, 2.29 mmol/L; Mg, 0.95 mmol/L; T-bil, 0.77 mg/dL; AST, 15 U/L; ALT, 14 U/L). Thus, ATO and ATRA were immediately discontinued, and aminophylline was administered.\nAfter interruption of ATRA for three doses, complete AV block persisted, but his HR partially recovered to 60 bpm. ATRA was rechallenged with 40 mg once daily on the 13th day of therapy under relatively stable vital signs. On the following morning, his HR decreased to 30 bpm and he required a nonrebreathing mask to maintain oxygen saturation. CXR showed bilateral pulmonary edema, and echocardiogram revealed a large amount of pericardial effusion with signs of cardiac tamponade. In addition, ECG showed complete AV block with QRS widening, and acute renal failure also occurred (Scr, 1.4 mg/dL; blood urea nitrogen (BUN), 45.2 mg/dL; Na, 133 mmol/L; K, 5.2 mmol/L; Ca, 2.22 mmol/L; Mg, 0.93 mmol/L; P, 4.9 mg/dL; T-bil, 1.22 mg/dL; ALT, 17 U/L). Therefore, ARTA was immediately postponed, and a temporary pacemaker was inserted. Steroid treatment was switched to dexamethasone (8 mg twice daily) because of suspected DS. Pericardiocentesis and thoracentesis were performed. Two days after stopping ATRA, ECG showed sinus bradycardia with a first-degree AV block and diffuse ST elevation. The Naranjo adverse drug reaction probability scale [] indicated a score of 7 for ATRA, suggesting it was the probable cause of arrhythmia in this episode.\nThe PML-RARα mutant was still detected using polymerase chain reaction (PCR) of blood sampled on the 19th day of therapy. Consequently, ATRA was resumed the following day with 10 mg twice daily. His HR decreased from 70–90 bpm to 60–70 bpm with stable vital signs. ECG demonstrated sinus rhythm with first-degree AV block. The dose of ATRA was gradually titrated up to 30 mg twice daily on the 24th day. Sinus tachycardia with occasional premature ventricular complexes was noted. The pacemaker was removed on the 27th day. During follow-up, ECG showed normal sinus rhythm (), and echocardiogram revealed minimal pericardial effusion.\nComplete molecular remission was documented by bone marrow aspiration done on the 38th day of therapy using flow cytometry and PCR for PML-RARα. Thereafter, the patient received three cycles of consolidation with ATRA-based chemotherapy. No further bradycardia episode was reported. The patient has been free of leukemia for 1.5 years after the diagnosis.

[[41.0, 'year']]

M

{'24627526': 1, '25726499': 1, '22715121': 1, '21364078': 1, '18299451': 1, '8691592': 1, '10789823': 1, '22778455': 1, '24659681': 1, '6956755': 1, '7249508': 1, '8606532': 1, '16447716': 1, '23767209': 1, '21220600': 1, '1597247': 1, '29984015': 2}

{}

6011167-1

comm/PMC006xxxxxx/PMC6011167.xml

Heat Stroke with Status Epilepticus Secondary to Posterior Reversible Encephalopathy Syndrome (PRES)

A 20-year-old fit and healthy man presented with sudden collapse after running for 3 hours under the hot sun (ambient temperature 39°C) during a marathon competition. After finishing his run for 35 km, he felt unwell and collapsed. He was brought to the hospital immediately. No measures were taken to lower his temperature during the 30-minute transferral to the hospital. Upon arrival to the hospital, he was confused (Glasgow coma scale: 13/15, E4M5V4). His blood pressure was 100/60 mmHg, heart rate 120/min, rectal temperature 42.2°C (axillary temperature 41.5°C). He was given 2 L of intravenous normal saline and rapidly cooled with ice pillow and ice water-soaked towel. He was then transferred to ICU for close monitoring. His rectal temperature was brought down to 39°C (axillary temperature 38.5°C) in an hour time. He was then transferred to ICU.\nHis cerebral CT scan, electrocardiogram, chest X-ray, and bedside echocardiogram were unremarkable. Laboratory results was shown in .\nHe remained oliguric (urine output < 10 ml/hour) despite initial fluid resuscitation. Hemodialysis was commenced on Day 1 of admission. On Day 3 of admission, his arterial blood pH, renal panel, and electrolytes were all normalized and he had regained urine output (urine output 10–20 ml/hour). He was fully conscious and alert (GCS 15/15).\nUnfortunately, on Day 4 of admission, he started to complain of severe headache followed by 6 episodes of generalized tonic-clonic seizures, each episode lasting for 1-2 minutes, in a one-hour time. There was no regaining of consciousness in between seizures. The episodes were associated with drooling of saliva, tongue biting, and postictal drowsiness. He was intubated and started on intravenous midazolam infusion, intravenous phenytoin, and intravenous levetiracetam.\nElectroencephalogram done on the same day showed generalized continuous slowing. However, no epileptic activity was observed during the bedside EEG monitoring. MRI brain done on the same day () showed bilateral, symmetrical vasogenic oedema involving both frontal and posterior parieto-occipital, cerebellar hemispheres, and predominantly the cortex and subcortical white matter regions. DWI and ADC sequence showed no area of restricted diffusion. The finding was consistent with posterior reversible encephalopathy syndrome (PRES).\nHe had another 2 episodes of generalized tonic-clonic seizures on Day 5 of admission that lasted less than 1 minute. Loading dose of intravenous sodium valproate 1 g was added. His repeat blood tests, including renal panel, complete blood count, pH, and electrolytes, were all normal.\nOn Day 6 of admission, he was able to produce 3 L of urine per day and hemodialysis was stopped. His midazolam infusion was weaning off slowly. He was fit-free since then. On Day 7 of admission, he was extubated and transferred to general ward. His antiepileptics agents were changed to oral form. His seizure was well controlled with oral phenytoin, valproate, and levetiracetam.\nA repeat MRI brain done on Day 14 of admission () showed near complete resolution of the previous vasogenic oedema. He was discharged well with oral valproic acid and levetiracetam.

[[20.0, 'year']]

M

{'31928528': 1, '12067009': 1, '32292535': 1, '15952443': 1, '16046331': 1, '17698535': 1, '15248787': 1, '26184985': 1, '29984005': 2}

{}