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[Home treatment of type I glycogenosis by continuous nocturnal gastric feeding]
Insights
Continuous nocturnal intragastric feeding with high glucose formula effectively manages type I glycogen-storage disease in children. This home-based treatment stabilizes blood glucose, improves growth, and reduces metabolic abnormalities, offering a practical alternative to surgery.
Area of Science:
- Metabolic disorders
- Pediatric endocrinology
- Nutritional therapy
Background:
- Type I glycogen-storage disease (GSD Ia) presents significant metabolic challenges, including hypoglycemia and hepatomegaly.
- Traditional management often requires frequent hospitalizations and invasive procedures.
Observation:
- Two children with GSD Ia were treated at home with continuous nocturnal intragastric feeding of a high glucose formula.
- This was complemented by frequent daytime feeding and a high-carbohydrate meal post-infusion.
Findings:
- The regimen led to rapid hospital discharge and return to school for both children.
- Stable blood glucose levels, improved fasting and exercise tolerance, decreased serum triglycerides, cholesterol, uric acid, and lactate, and reduced liver size were observed.
- Remarkable increases in linear growth rate and insulin-glucagon ratio were noted, with no gastric tube complications.
Implications:
- Home-based nocturnal intragastric feeding is an effective, practical, and well-tolerated long-term therapy for GSD Ia.
- This approach offers a reliable, less invasive, and cost-effective alternative to surgical interventions like portocaval shunting.
- Continuous treatment is recommended through adolescence for sustained benefits.
Abstract:
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.