Hepatic glycogenoses. Introduction
Guibaud, P.; Moatti, N.
Annales de Gastroenterologie et D'hepatologie 24(2): 61-62
1988
ISSN/ISBN: 0066-2070 PMID: 3164606 Document Number: 306222
Liver glycogenosis (GSD) are hereditary in diseases caused by deficiencies of the three major enzymatic systems involved degradation of glycogen: glucose-6-phosphatase (GSD VI). The aims of this paper are, in a first part, to summarize the biological and physiological aspects of these disorders in order to propose an update diagnostic process, and, in a second part, to point out the clinical features and the possible evolution of such patients becoming adults, according to the French experience.
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