Abstract

Hyperinsulinemic infants develop severe hypoglycemia due to a major suppression of glucose production by the liver. Treatment of this condition is made difficult in a subset of patients by the limited efficacy of drugs, such as diazoxide or corticosteroids. The need for maintaining a large supply of glucose in these patients requires continuous nasogastric feeding and sometimes prolonged peripheral or central venous catheterization. We have used the subcutaneous infusion of glucagon via a small insulin pump in an attempt to maintain BG within normal limits in 5 hypersinulinemic infants previously treated with 15 mg/kg.d diazoxide. Glucagon was infused continuously as a 1 mg/ml 0.5 N saline solution at a rate of 0.37±0.17 mg/kg.d during the 2-12 weeks preceeding subtotal pancreatectomy. No side effects were detected. BG increased from 50±28 to 110±57 mg/dl (p<0.001), with a consistent effect in all treated children. Less than 6±4% BG values fell below 45 mg/dl versus 41±14% (p<0.025) under previous therapy. Mean insulin values were as expected unchanged (10±7.3 versus 18±13 μU/ml before). During this period, feeding could be discontinued and diminished down to 0.65±0.1 g carbohydrates/kg.h; versus 0.93 ± 0.1 g/kg.h before. Because of its simplicity and reliability, we propose that the subcutaneous continuous infusion of glucagon should be largely employed for the control of hypoglycemia in infants developing severe hyperinsulinism and/or resistance to diazoxide therapy.

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