Internal medicine
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We report a case of granulomatosis with polyangiitis (GPA) presenting with hypertrophic pachymeningitis with a huge brain tumor-like lesion. A 57-year-old man acutely developed consciousness disturbance. Magnetic resonance imaging revealed a right frontal lobe mass with thickened, contrast-enhanced dura. ⋯ Histopathology of the excised brain tissues revealed thrombovasculitis with heavy neutrophilic infiltration in the pachy- and leptomeninges covering an ischemic cerebral cortex. The patient improved with corticosteroids and rituximab. Our case warrants considering GPA as a cause of hypertrophic pachymeningitis with brain-tumor like lesions.
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We encountered two consecutive cases of adult-onset Kawasaki disease (AKD) that were difficult to diagnose. In both cases, Kawasaki disease was not considered as a differential diagnosis in the early stages. ⋯ AKD has a minimal incidence rate and may have a clinical course different from that of childhood-onset Kawasaki disease. Therefore, it is necessary to incorporate Kawasaki disease into the differentiation of an adult fever and to consult with a pediatrician for its diagnosis.
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Exostosin 1 (EXT1) and exostosin 2 (EXT2)-associated membranous nephropathy (MN) may be associated with active autoimmune disease. We encountered an elderly man who presented with EXT1/EXT2-associated lupus-like MN with full house immune deposits, monoclonal gammopathy of uncertain significance and Sjögren's syndrome. ⋯ Although he did not fulfill the criteria of clinical systemic lupus erythematosus (SLE), he met a stand-alone renal criterion of the Systemic Lupus International Collaborating Clinics (SLICC) 2012. Whether or not a stand-alone renal criterion with EXT1/EXT2 positivity, as in the present patient, can efficiently guide decisions regarding the diagnosis and treatment of SLE remains a clinical dilemma.
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Case Reports
Complete Circumferential Endoscopic Submucosal Dissection for Esophageal Cancer Leaving an Island of Normal Mucosa.
Esophageal stricture caused by complete circumferential endoscopic submucosal dissection (ESD) of extensive esophageal squamous cell carcinoma (ESCC) is a major concern and can result in a low quality of life. Normal mucosa may remain within a complete circumferential lesion of ESCC in some cases. We herein report a case of ESCC in which a complete circumferential lesion was treated with ESD while leaving an island of normal mucosa within it. This case demonstrates that preserving areas of normal mucosa within lesions during complete circumferential ESD is not technically difficult and may be an effective measure for preventing esophageal stricture.
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The MYH7 R453 variant has been identified in inherited hypertrophic cardiomyopathy (HCM) and is associated with sudden death and a poor prognosis. The detailed clinical course of HCM with the MYH7 R453 variant, from a preserved to a reduced left ventricular ejection fraction, has not been reported. We identified the MYH7 R453C and R453H variants in three patients who progressively developed advanced heart failure requiring circulatory support and summarized the clinical course and echocardiographic parameters of these patients over the years. Because of the rapid disease progression, we consider genetic screening for patients with HCM imperative for future prognosis stratification.