Open Access[AN AUTOPSY CASE OF GLYCOGEN AND NON‐GLYCOGEN POLYSACCHARIDE STORAGE DISEASE WITH CARDIOMEGALY
Author(s) -
Eto Komyo,
Takeuchi Tadao
Publication year - 1973
Publication title -
acta patholigica japonica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.73
H-Index - 74
ISSN - 0001-6632
DOI - 10.1111/j.1440-1827.1973.tb00785.x
Subject(s) - glycogen , autopsy , glycogen storage disease , pathology , polysaccharide , carbohydrate , biology , chemistry , medicine , endocrinology , biochemistry
An autopsy case of abnormal glycogen and non‐ glycogen polysaccharide deposition in a 7‐year‐old boy with extreme cardiomegaly was encountered. The accumulation of a non‐glycogen carbohydrate was seen in cardiac muscle fibers, skeletal muscle fibers, smooth muscle fibers of various organs, nerve tissues of brain, epithelial cells of liver, kidney and adrenal glands, and some other organs. Its histochemical properties were studied and it was categorized as an acidic mucopolysaccharide according to the classification suggested in the previous literatures. The electron microscopic features observed in the non‐glycogen storage were composed of randomly arranged filaments, somewhat different from amylopectinosis. Glycogen particles were occasionally found, but most of them were exhausted after death. Glycogenosomes were not identified and a‐glucoside could not be examined. Accordingly, a definite diagnosis of glycogenosis type II could not be made, but possible diagnosis of this disease with non‐glycogen polysaccharide storage was suggested by the autopsy findings and histochemical properties of storage substances.