L de Parscau — Page 4 | PubMed Health

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[Continuous nocturnal intragastric feeding in glycogenosis type I and III].

Three patients with type I glycogen storage disease (GT I) and four patients with type III glycogen storage disease (GT III) have been treated with nocturnal intragastric feeding and frequent daytime meals for 2 to 8.5 years. In all of them, hypoglycemia was well controlled. Patients with GT. I showed a normal growth rate and an improvement of serum lactate, triglycerides, cholesterol and urate. Patients with GT. III showed no consistent changes in serum triglycerides cholesterol and transaminases and no improvement of myocardiopathy.

Blood Glucose↗

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[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.

Glycogen Storage Disease↗