The dietary treatment of children with type I glycogen storage disease with slow release carbohydrate.

Abstract:

:The effect of ingestion of uncooked cornstarch (2 g/kg body weight) in water, uncooked starch (1 g/kg) added to a meal, and glucose (2 g/kg) in water, was studied in eight patients with type IA glycogen storage disease (GSD) and one patient with type IB GSD. Blood glucose concentrations were determined at 30-min intervals during each tolerance test; blood lactate, blood insulin, and expiratory hydrogen were determined at 60-min intervals. The glucose levels remained in the normal range (greater than or equal to 1.8 mM) during approximately 6.5-9.0 h, 3.5-6.5 h, and 2.25-4.0 h during the three tolerance tests, respectively. The lactate levels differed markedly for the different tests per patient, and for the same type of test between the patients. Blood insulin concentrations after starch administration did not exceed values of 50 mU/liter above fasting levels and were markedly lower than those after glucose administration (maximum levels of 280 mU/liter). The expiratory hydrogen excretion did not increase or only slightly increased after cornstarch administration (less than 20 ppm).

journal_name

Pediatr Res

journal_title

Pediatric research

authors

Smit GP,Berger R,Potasnick R,Moses SW,Fernandes J

doi

10.1203/00006450-198409000-00015

subject

Has Abstract

pub_date

1984-09-01 00:00:00

pages

879-81

issue

9

eissn

0031-3998

issn

1530-0447

journal_volume

18

pub_type

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