[Long-term course of hepatic glycogenosis. A retrospective study of 76 cases].
In order to study the long term outcome of hepatic glycogen storage diseases, a national retrospective inquiry gathered 76 patients older than 12 years. In adolescents and adults, hypoglycemia, failure to thrive, pubertal delay, hepatomegaly and metabolic disturbances are major in type I, intermediary in type III and mild in type "VI+IX". Spontaneous improvement of these symptoms is noted in older patients. Beside these classical signs, anemia, high blood pressure, renal failure and persistent hypercholesterolemia were reported in some type I glycogen storage disease and bad school and professional results in type III. The knowledge of these complications should lead to a better management of these patients.
de Parscau
L
L
Service de Pédiatrie, Hôpital Debrousse, Lyon.
Guibaud
P
P
Labrune
P
P
Odièvre
M
M
D000293:Adolescent; D000328:Adult; D002648:Child; D005260:Female; D006008:Glycogen Storage Disease / Q000150:complications / Q000401:mortality / Q000503:physiopathology*; D006801:Humans; D008107:Liver Diseases / Q000150:complications / Q000401:mortality / Q000503:physiopathology*; D008297:Male; D008875:Middle Aged; D015337:Multicenter Studies as Topic; D012189:Retrospective Studies; D013997:Time Factors
Evolution à long terme des glycogénoses hépatiques. Etude rétrospective de 76 observations.