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The measurement of 125I-insulin specific binding to erythrocytes obtained from seven infants and children with various hypoglycemic syndromes showed a significant increase in six patients with recurrent, documented, symptomatic hypoglycemia (percent specific 125I-insulin binding 9 to 14 versus 6.1 +/- 1.4% mean +/- 2 S.D. for 13 controls). The increase was due to an increased number of receptors sites per cell rather than to increased affinity for insulin. The patients included three children with nesidioblastosis, all after 90% pancreatectomy, two with leucine sensitivity, and two with glycogen storage disease type I. One of the patients with leucine sensitivity, who for 2 years before the study had no hypoglycemia, had normal insulin values (less than 10 microunits/ml). Thus, a symptomatic hypoglycemia correlated better with increased 125I-insulin binding than with plasma insulin values. Furthermore, Diazoxide therapy in two patients caused a mild but consistent decrease in the number of insulin receptor sites, and the institution of continuous nocturnal nasogastric feedings in a patient with glycogen storage disease type I was followed by amelioration of the hypoglycemia and a marked increase in 125I-insulin specific binding from 5.2 to 9.5%.

Increased 125I-Insulin Receptor Binding to Erythrocytes of Hypoglycemic Infants and Children