| Autor: |
Wolfsdorf JI; Department of Medicine, Children's Hospital, Boston, MA 02115., Rudlin CR, Crigler JF Jr |
| Jazyk: |
angličtina |
| Zdroj: |
The American journal of clinical nutrition [Am J Clin Nutr] 1990 Dec; Vol. 52 (6), pp. 1051-7. |
| DOI: |
10.1093/ajcn/52.6.1051 |
| Abstrakt: |
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. |
| Databáze: |
MEDLINE |
| Externí odkaz: |
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