ISSN:
1432-0533
Keywords:
Muscle
;
Ultrastructure
;
Lysosome
;
Metabolic disorder
;
Glycogenosis
Source:
Springer Online Journal Archives 1860-2000
Topics:
Medicine
Notes:
Summary 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 childhoodonset 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.
Type of Medium:
Electronic Resource
URL:
http://dx.doi.org/10.1007/BF00304641
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