[Hepatic glycogenoses. Introduction].
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.
Guibaud
P
P
Hôpital Debrousse, Lyon.
Moatti
N
N
D006008:Glycogen Storage Disease / Q000145:classification*; D006801:Humans; D008107:Liver Diseases / Q000145:classification*
Les glycogénoses hépatiques. Avant-propos.