[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.
D001827:Body Height; D002675:Child, Preschool; D004040:Dietary Carbohydrates / Q000008:administration & dosage; D004044:Dietary Proteins / Q000008:administration & dosage; D004750:Enteral Nutrition / Q000379:methods*; D005260:Female; D005526:Food, Formulated; D005953:Glycogen Storage Disease Type I / Q000628:therapy*; D006529:Hepatomegaly / Q000209:etiology / Q000628:therapy; D006701:Home Nursing; D006801:Humans; D007223:Infant; D013997:Time Factors
D004040:Dietary Carbohydrates; D004044:Dietary Proteins
Traitement en milieu familial de la glycogénose de type I par alimentation gastrique continue nocturne.