REDUCING BODY-LIKE INCLUSIONS IN SKELETAL-MUSCLE IN CHILDHOOD-ONSET ACID MALTASE DEFICIENCY

被引：12

作者：

JAY, V

CHRISTODOULOU, J

MERCERCONNOLLY, A

MCINNES, RR

机构：

[1] HOSP SICK CHILDREN,DIV CLIN GENET,TORONTO M5G 1X8,ONTARIO,CANADA

[2] UNIV TORONTO,TORONTO M5S 1A1,ONTARIO,CANADA

来源：

ACTA NEUROPATHOLOGICA | 1992年 / 85卷 / 01期

关键词：

MUSCLE; ULTRASTRUCTURE; LYSOSOME; METABOLIC DISORDER; GLYCOGENOSIS;

D O I：

10.1007/BF00304641

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

Unusual inclusions with some of the features of "reducing bodies" were encountered in the skeletal muscle biopsy of a 2.5-year-old boy with childhood-onset acid maltase deficiency. The biopsy revealed a vacuolar myopathy with lysosomal storage of glycogen and eosinophilic refractile inclusions in myofibers, which appeared dark blue with the menadione-nitroblue tetrazolium reaction. The significance of the association of inclusions with reducing properties in the setting of acid maltase deficiency is discussed.

引用

页码：111 / 115

页数：5

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