Abstract
In three patients with GSD I, three with GSD III and one with PCD (age 4-20 y) nocturnal drip feeding (duration 2-8 y) was replaced by administration of uncooked corn starch in curd. To reach comparable serum glucose levels over night and in the morning (70-100 mg/dl) as during drip feeding, the starch had to be given 1-2 × per night. Patients aged below 14 y received 0.5-2.0 g/kg at 9 p.m. and an additional 3.0-4.0 g of starch/kg at midnight, whereas the two oldest patients got a single dose of 3.0–4.0 g/kg at midnight only. Caloric intake over night was 100-120 % of that during drip feeding. In addition, three of the younger patients obtained 1.0-2.0 g starch/kg one hour after breakfest and lunch. Following a period of 1.5-2.5 y no significant change occured in serum lactate, alanine, GOT, GPT, uric, acid, triglyceride, cholesterol values and in the growth rate when compared to the last two years during drip feeding. The high starch doses were tolerated without side effects.
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Ullrich, K., Van Teeflen-Heithoff, A. 92 A GLYCOGEN STORAGE DISEASE TYPE I AND III(GSDI, III) AND PYRUVATE CARBOXYLASE DEFICIENCY (PCD): NOCTURNAL GASTRIC DRIP FEEDING VERSUS ADMINISTRATION OF UNCOOKED CORN STARCH!. Pediatr Res 20, 1049 (1986). https://doi.org/10.1203/00006450-198610000-00146
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DOI: https://doi.org/10.1203/00006450-198610000-00146
