Internal medicine
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Case Reports
Status Cataplecticus with Rapid Eye Movement Sleep Excess in Late-onset Narcolepsy Type 1.
A 57-year-old man presented with difficulty speaking and walking along with increased daytime somnolence. His symptoms fluctuated throughout the day but never completely disappeared. A neurological examination revealed mild dysarthria, limb weakness, and staggering gait. ⋯ Human leukocyte antigen testing demonstrated DQB1*0602 positivity. His neurological symptoms were relieved by clomipramine. Thus, he was diagnosed with late-onset narcolepsy type 1 with status cataplecticus.
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A 44-year-old woman was admitted due to gross hematuria and progressive renal dysfunction. Poststreptococcal acute glomerulonephritis (PSAGN) was suspected due to her elevated anti-streptolysin O and anti-streptokinase titers and hypocomplementemia. A renal biopsy showed crescent formation and endocapillary hypercellularity with neutrophil infiltrate. ⋯ However, myeloperoxidase anti-neutrophil cytoplasmic antibody (ANCA) was positive, and peritubular capillaritis was observed. Furthermore, citrullinated histone H3-positive neutrophils were detected as markers for neutrophil extracellular trap formation. Therefore, she was diagnosed with ANCA-associated vasculitis superimposed on PSAGN that was the main contributor to her progressive renal injury.
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Case Reports
A Case of Severe Anemia Caused by Gastric Antral Vascular Ectasia and Autoimmune Gastritis.
An 80-year-old man presented to our hospital with general fatigue on exertion that had gradually worsened over 6 months. His blood test revealed severe anemia, and gastroscopy revealed findings consistent with gastric antral vascular ectasia (GAVE) and autoimmune gastritis. ⋯ The present case suggested that GAVE is triggered by autoimmune gastritis, and the mechanism is likely related to hypergastrinemia. The reporting of this rare case may help elucidate the cause of GAVE, which is currently unknown.
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Objective High-dose melphalan and autologous stem cell transplantation (ASCT) therapy for AL amyloidosis are now associated with reduced mortality based on the application of strict criteria. However, there is no long-term evidence concerning the performance of induction therapy with newer agents, such as bortezomib or daratumumab. Concerns regarding long-term relapse despite treatment with ASCT exist, and missing the opportunity to perform ASCT might occur if induction proves to not be efficacious and cardiac amyloidosis progression deprives the patients of a chance to receive ASCT. ⋯ Although there was no significant difference in the survival rates between the two groups, the time to next treatment or death was significantly better in the VAD+ASCT group than in the the frontline ASCT group. Acute kidney injury was the most frequent reason for failure to receive two courses of VAD, and early mortality was mainly due to gastrointestinal complications. Conclusion Considering that only those who underwent 2 courses of VAD experienced a 10-year renal response, induction therapy was deemed to be directly related to the long-term control of AL amyloidosis.
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A 60-year-old woman with a history of hypothyroidism was referred to our hospital for shortness of breath and a left ventricular ejection fraction (LVEF) of 13%, which required continuous dobutamine injection with intra-aortic balloon pump support. An endomyocardial biopsy obtained from the right ventricle revealed an infiltration of giant cells and eosinophils, indicating giant cell myocarditis. In addition to heart failure treatment, combined immunotherapy with steroids, tacrolimus, and intravenous immunoglobulin was administered. Transthoracic echocardiography demonstrated a dramatic improvement in the LVEF after this therapy, and the patient was discharged home without symptoms on day 72.