Polysaccharide (amylopectin-like) storage myopathy histochemical ultrastructural and biochemical studies.

J F Pellissier, T de Barsy, J Bille, G Serratrice, M Toga
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引用次数: 24

Abstract

A case of an adult polysaccharide myopathy is reported in a patient with progressive muscular atrophy and weakness of limb girdles. Histochemistry and electron microscopy showed in some muscle fibers, a storage material composed of amylopectin-like filaments. Biochemical results were normal and no enzyme deficiency was found. This case is compared with three other published cases. Pathological conditions with amylopectin or amylopectin-like storage material are reviewed.

多糖(支链淀粉样)储存肌病的组织化学、超微结构和生化研究。
一例成人多糖肌病报告在病人进行性肌肉萎缩和四肢无力的腰带。组织化学和电子显微镜显示,在一些肌肉纤维中,有一种由支链淀粉样细丝组成的储存物质。生化结果正常,未发现酶缺乏症。本案例与其他三个已发表的案例进行了比较。对支链淀粉或支链淀粉样储存材料的病理情况进行综述。
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