AJCN Tufts Nutrition Symposium, Boston Sept 24-26
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American Journal of Clinical Nutrition, Vol 52, 1051-1057, Copyright © 1990 by The American Society for Clinical Nutrition, Inc

ORIGINAL RESEARCH COMMUNICATIONS

Physical growth and development of children with type 1 glycogen- storage disease: comparison of the effects of long-term use of dextrose and uncooked cornstarch

JI Wolfsdorf, CR Rudlin and JF Crigler Jr
Department of Medicine, Children's Hospital, Boston, MA 02115.

Thirteen patients with type 1 glycogen-storage disease (GSD-1) were studied to compare the effects on biochemical control and growth of 2 y of therapy with intermittent feedings of uncooked cornstarch (UCS) at the fasting glucose production rate and therapy with continuous overnight glucose (COG) and dextrose feedings during the day. Mean biochemical abnormalities for the groups were minimized but not normalized by either COG or UCS. Growth progressed normally when COG was started by 1.2 y of age and normal growth rate was maintained by UCS. Weight increased from 101 +/- 3% ideal body weight at onset of COG to 127 +/- 5% during COG and the first year of UCS therapy but did not increase further in the second year. When growth failure occurred before onset of COG [-3.7 SD score for chronological age (SDSCA)], only partial correction (-1.9 SDSCA) to genetic potential for height occurred. Intermittent feeding of UCS provides an effective alternative to COG for the treatment of GSD-1.




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Copyright © 1990 by The American Society for Nutrition