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PubMed1986

[Home treatment of type I glycogenosis by continuous nocturnal gastric feeding].

Two children with type I glycogen-storage disease were treated at home with continuous nocturnal intragastric feeding, using a high glucose formula. The children were 6.5 years and 32 months old respectively when initiating treatment, and they have now been treated for 24 and 18 months. A high carbohydrate meal was given soon after stopping the nocturnal infusion, and this was followed by frequent daytime feeding. In this way, the children were rapidly discharged from hospital and soon returned to school. This regimen stabilized blood glucose levels, avoiding hypoglycemic complications, and improved tolerance to fasting and exercise. Moreover it decreased serum triglyceride, cholesterol, uric acid and lactate levels as well as liver size. The increase in linear growth rate was remarkable and was associated with an increase in insulin-glucagon ratio. No complications resulted from the gastric tube. The method proved to be effective, simple, practical and acceptable by children and their parents. In addition, it is relatively inexpensive and represents a reliable long-term alternative therapy to portocaval shunting for patients with type I glycogen-storage disease. The nocturnal infusions should be continued until after adolescence.

Body Height↗
PubMed1988

[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.

Adolescent↗