An Autopsy Case of Fabry's Disease with Cardiac Manifestations

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概要

• 論文の詳細を見る

• We report an autopsy case of Fabrys disease with cardiac manifestations. Electron microscopic examination of rectal biopsy specimens revealed lamellar bodies and osmiophilic irregular bodies. Biochemical analysis showed low enzymatic activity of α-galactosidase A in plasma fluid. Microscopic examination on autopsy showed marked hypertrophy and vacuolation of cardiac muscle cells. Intracytoplasmic vacuolation was also found in glomerular epithelial or endothelial cells and smooth muscle cells of renal arteries, Meissners plexus in the submucosa of small and large intestines, and smooth muscle cells of arterioles in the cerebrum. The diagnosis of Fabrys disease was made. The patient died suddenly of cardiomyopathy. [J Clin Exp Hematopathol 49(2) : 121-123, 2009]

• 2009-11-01

著者

• Abe Masafumi

  First Department Of Pathology School Of Medicine Fukushima Medical University

• Abe Masafumi

  First Department Of Pathology Fukushima Medical University

• SUZUKI Osamu

  First Department of Pathology, School of Medicine, Fukushima Medical University

• Suzuki Osamu

  First Department Of Pathology School Of Medicine Fukushima Medical University

• Abe Masafumi

  First Department Of Pathology Fukushima Medical College

• ABE Masafumi

  First Department of Pathology, School of Medicine, Fukushima Medical University

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