Continuous nocturnal intragastric feeding for management of type 1 glycogen-storage disease

N Engl J Med. 1976 Feb 19;294(8):423-5. doi: 10.1056/NEJM197602192940805.

Abstract

The clinical and biochemical abnormalities associated with Type 1 glycogen-storage disease can be reversed by avoidance of hypoglycemia and secondary hormonal flux. Three patients with Type 1 disease were treated with intragastric infusions of a high glucose formula at night with three-hour starch feedings during the day. This regimen stabilized blood glucose levels above 70 mg per deciliter and decreased serum uric acid, triglyceride, lactate and serum oxalacetic transaminase levels, as well as hepatic size, in all patients. Increased linear growth rate (mean 1 cm per month) was associated with a decrease in mean plasma glucagon (from 190 to 40 pg per milliliter) and an increase in mean plasma insulin (from 19 to 43 muU per milliliter, [two patients]). These changes occurred within four weeks of beginning of treatment and continued with home treatment for 13 months. No complications resulted from tube placement daily by the patients. Type 1 disease can be managed by nighttime intragastric feeding and frequent daytime high starch meals.

MeSH terms

  • Adolescent
  • Aspartate Aminotransferases / blood
  • Blood Glucose / analysis
  • Child
  • Cholesterol / blood
  • Clofibrate / therapeutic use
  • Eating
  • Enteral Nutrition*
  • Female
  • Glucagon / blood
  • Glucose / administration & dosage
  • Glycogen Storage Disease Type I / blood
  • Glycogen Storage Disease Type I / diet therapy*
  • Glycogen Storage Disease Type I / drug therapy
  • Growth Hormone / blood
  • Humans
  • Hypoglycemia / prevention & control
  • Insulin / blood
  • Lactates / blood
  • Time Factors
  • Triglycerides / blood
  • Uric Acid / blood

Substances

  • Blood Glucose
  • Insulin
  • Lactates
  • Triglycerides
  • Uric Acid
  • Growth Hormone
  • Glucagon
  • Cholesterol
  • Aspartate Aminotransferases
  • Clofibrate
  • Glucose