Abstract
Chronic or prolonged hypoglycemia can have devastating effects on a child's neurologic function. Support of normoglycemia is thus pivotal. We report the management of a child with familial nesidioblastosis who had failed to tolerate and respond to diazoxide therapy following a subtotal pancreatectomy at the third week of life. Long acting intramuscular zinc protamine glucagon was given with some success twice daily for 3 1/2 years along with frequent oral dextrose supplements. However, early morning hypoglycemia persisted, and wide fluctuations in plasma glucose levels occurred during 48 hours of frequent sampling (coefficient of variation 56.8%). At the fourth year of treatment circulating anti-insulin antibodies were found in our patient interfering with the plasma insulin determinations. Subsequently, we showed presence of trace amounts of insulin in the zinc-glucagon preparation Oral starch granules (1.25 tbsp/10 lb of body weight) were substituted for dextrose supplements and glucagon was discontinued. Over the subsequent months, plasma glucose concentrations have been more stable (coefficient of variation 23.6%) and within the normal range (70-120 mg/dl). We conclude that oral starch may be an effective treatment or therapeutic supplement in states of hyperinsulinism.
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Rose, S., Chrousos, G., Feuillan, P. et al. 1248 LONG-TERM ZINC PROTAMINE GLUCAGON AND ORAL STARCH IN IN THE MANAGEMENT OF FAMILIAL NESIDIOBLASTOSIS. Pediatr Res 19, 318 (1985). https://doi.org/10.1203/00006450-198504000-01278
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DOI: https://doi.org/10.1203/00006450-198504000-01278