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Benefits in the ketogenic diet regime upon drug-resistant epileptic encephalopathy of a de novo NBEA pathogenic different.
A 46-year-old woman with exacerbating hemoptysis and dyspnea was diagnosed with diffuse alveolar hemorrhage (DAH). High doses of glucocorticoids were initiated, but afterward, paroxysmal hypertension (210/140 mmHg) with headache and abdominal pain appeared. A 50-mm left adrenal tumor with an intense uptake by iodine-123 metaiodobenzylguanidine scintigraphy and catecholamine hypersecretion revealed complication with pheochromocytoma. Because high doses of glucocorticoids, sometimes required for DAH, can provoke life-threatening paroxysmal hypertension in pheochromocytoma and paraganglioma (PPGL), our case suggests that PPGL needs to be recognized as the cause of DAH and should be detected with whole-body imaging before starting glucocorticoids.Objective Since patients with thoracic aortic aneurysm (TAA)/abdominal aortic aneurysm (AAA) are often complicated with coronary artery disease, it is common for those patients to undergo percutaneous coronary intervention (PCI). The ankle brachial index (ABI) is usually measured in patients with TAA/AAA to screen the presence of peripheral arterial disease. The present study investigated the association between the ABI and clinical outcomes following PCI in patients with TAA/AAA. Methods and Material We divided 200 TAA/AAA patients who underwent PCI into a normal ABI group (n=137) and an abnormal ABI group (n=63) according to the ABI cut-off level of 1.00. The primary endpoint was one-year major adverse cardiovascular events (MACE), defined as the composite of cardiovascular death, non-fetal myocardial infarction, stroke, target vessel revascularization, and hospitalization for heart failure. Results Mean ABIs in the normal and abnormal ABI groups were 1.12±0.09 and 0.86±0.11, respectively (p less then 0.01). Kaplan-Meier curves showed MACE were more frequent in the abnormal ABI group than in the normal ABI group (p=0.01). A multivariate Cox hazard analysis revealed that an abnormal ABI was significantly associated with 1-year MACE (vs. ABI ≥1.0 HR 3.02, 95% confidence interval 1.00-9.08, p=0.049). Conclusion Among patients with TAA/AAA who underwent PCI, abnormal ABI was significantly associated with 1-year MACE, suggesting the utility of the ABI measurement in this high-risk population.Objective Endoscopic papillary large-balloon dilation (EPLBD) with limited endoscopic sphincterotomy (EST) is widely used for removing multiple large common bile duct (CBD) stones. However, the safety and effectiveness of immediate EPLBD after limited EST and EPLBD at an interval after limited EST is unclear. Thus, this multicenter retrospective study was conducted to examine this matter. Methods Propensity score-matching was performed to adjust the baseline characteristics between the immediate and interval EPLBD groups. We compared the incidence of post-endoscopic retrograde cholangiopancreatography (ERCP) complications and the early outcomes of ERCP between the 2 matched groups, which comprised 66 patients each. Results The complete stone clearance rate in each study group was 100%. The overall incidence of post-ERCP complications in the propensity score-matched interval and immediate EPLBD groups was 3/33 (9.1%) and 1/33 (3.0%), respectively (p=0.61). The immediate EPLBD group had significantly fewer mean ERCP sessions for complete stone removal and a significantly lower rate of endoscopic mechanical lithotripsy (EML) usage than the interval EPLBD group (1.6 vs. 2.4 sessions, p less then 0.001; and 4/33 [12.1%] vs. 12/33 [36.4%], p=0.042, respectively). Conclusion The incidence of post-ERCP complications in the immediate EPLBD group was not significantly different from that in the interval EPLBD group. Compared with interval EPLBD, immediate EPLBD may result in a reduced number of ERCP sessions for complete stone clearance and reduce the rate of EML usage.A 70-year-old woman was hospitalized with dyspnea. A transthoracic echocardiogram indicated an elevated systolic pulmonary artery pressure, and the cytology specimens obtained using a pulmonary artery catheter confirmed adenocarcinoma metastasis. Diffusion- weighted whole-body imaging with background body signal suppression (DWIBS) detected high-signal-intensity lesions in the urinary bladder. The patient died of respiratory failure and a postmortem examination was performed. Tumor cells in the bladder were immunohistochemically positive for GATA3, indicating micropapillary urothelial carcinoma, which is a rare variant of urothelial carcinoma and considered an adenocarcinoma subtype. This case is the first autopsy case of PTTM associated with micropapillary urothelial carcinoma of the urinary bladder.Good's syndrome is associated with thymoma and acquired immunodeficiency. A 54-year-old man visited our hospital with a complaint of cough. Chest imaging revealed diffuse nodular shadows and anterior mediastinal mass. Hypogammaglobulinemia and a decreased B lymphocyte count were found by a laboratory evaluation. The lung nodules markedly regressed after immunoglobulin therapy. The mediastinal mass and remaining nodule were surgically resected and diagnosed as a type AB thymoma and a necrotizing epithelioid granuloma with T lymphocyte-dominant alveolitis, respectively. selleck chemicals The overall appearances of these lesions were mostly in line with the spectrum of granulomatous-lymphocytic interstitial lung disease associated with Good's syndrome.We herein report an extremely rare case of adenocarcinoma of the minor duodenal papilla (MiDP) which was successfully treated by endoscopic mucosal resection (EMR). An asymptomatic 84-year-old man underwent upper gastrointestinal endoscopy, which revealed a slightly elevated lesion at the MiDP. The biopsy findings were suggestive of adenocarcinoma. Computed tomography, magnetic resonance images and endoscopic ultrasonography did not reveal pancreatic tumor infiltration nor any apparent distant metastases. Therefore, we treated the lesion using EMR with complete resection. No recurrence or metastasis has been detected at 13 months after EMR. Total resection of the MiDP can thus serve as a relatively safe and simple treatment.Objective This study examined whether or not the Digestive Disease Week-Japan (DDW-J) 2004 scale proposed over 15 years ago can be applied to current cases of drug-induced liver injury (DILI). Patients and Methods The new patients group included 125 patients from 2012 to 2019 and was divided into 2 subgroups 96 patients in the new DILI group and 29 patients in the new non-DILI group. Similarly, the old patients group included 105 patients from 1997 to 2002 and was divided into 2 subgroups 59 patients in the old DILI group and 46 patients in the old non-DILI group. Patients were assessed by the DDW-J 2004 scale; those with a score ≥3 were defined as having DILI. Results The total score of the new DILI group was significantly lower than that of the old DILI group (6 [1-11] vs. 6 [3-9], p=0.004). The sensitivity, specificity, positive predictive value, and negative predictive value (NPV) were 94.8%, 65.6%, 90.1%, and 79.2%, respectively, in the new patients group and 100%, 91.4%, 93.7%, and 100%, respectively, in the old patients group.
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