Abstract
Parents of CF children are often over-enthusiastic to any new modality of management. This study reports adverse metabolic and psychological effects of a liquid formula in 4 children (ages 16, 13, 8 and 5 yrs) with CF who were placed on the diet by their parents. The 16 yr old developed recurrent hypoglycemia while the 13 yr old developed hyperglycemia and glycosuria, all of which disappeared upon returning to a conventional diet. Simultaneous determinations of blood glucose (BG), immuno-reactive insulin (IRI), and immune-reactive glucagon (IRG) over a 5 hr period following the formula were compared with those following a standard glucose tolerance test (GTT). An exaggerated hyperglycemia (peak 226-486 vs 136-288 mg%) at 60' was followed by a 2nd peak at 2-4 hrs (120-178 vs 61-103 mg%). The IRI and IRG were all low and flat except in the 16 yr old, who had high levels. It is postulated that the high carbohydrates (85%) in the diet precipitated the higher peak of BG and that rapid absorption of aminoacids (8%) and fat (7%) may account for the prolonged hyperglycemia through continuous conversion to glucose. Only the 16 yr old consented to repeat tests on and off the formula. The 13 yr old refused to resume the diet. Parental fears prohibited the testing of the 8 and 5 yr olds on the conventional diet lest the children not return to the formula. All chlldren accepted the diet to please their parents. However, both adolescents felt “trapped between the diet and death” and were relieved when the formula was discontinued for “metabolic” reasons.
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Wang, CI., Zeltzer, L. & Kogut, M. METABOLIC AND EMOTIONAL IMPACT OP AN ELEMENTARY DIET IN CYSTIC FIBSOSIS (CF). Pediatr Res 11, 384 (1977). https://doi.org/10.1203/00006450-197704000-00088
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DOI: https://doi.org/10.1203/00006450-197704000-00088