[Clinical aspects of hepatic glycogenoses].
Hepatic glycogenosis, heterogeneous in their type, appear in children as an hepatomegaly discovered during manifestations of hypoglycemia and/or growth disorders, sometimes in the course of a systematic physical examination. A usually late puberty determines a transient aggravation of the height insufficiency. Persistence of a marked hepatomegaly and the development of lever adenomas are characteristic of type I glycogenosis. There, the metabolic imbalance (hyperlipoproteinemia and hyperuricemia, especially), lead to severe vascular and renal complications. Haematologic and sometimes infectious disorders may be added. In type III glycogenosis, the danger depends less on the liver fibrosis, usually minimal, than on the frequently associated cardio-vascular involvement. Type VI glycogenosis, usually have a favorable course. Current therapeutic progresses and a better care should result in a marked improvement of the evolution in type I and probably type III.
Guibaud
P
P
Unité de Génétique et d'Etude des Maladies Métaboliques de l'Enfant.
de Parscau
L
L
D006008:Glycogen Storage Disease / Q000378:metabolism* / Q000503:physiopathology; D006801:Humans; D008107:Liver Diseases / Q000378:metabolism* / Q000503:physiopathology
Aspects cliniques des glycogénoses hépatiques.