OBJECTIVES: I. Determine the dose of recombinant human insulin-like growth factor I that minimizes or decreases the need for exogenous glucose support without causing hypoglycemia.

PROTOCOL OUTLINE: This is a dose escalation study. Patients receive an escalating dose of recombinant human insulin-like growth factor I (IGF-I). IGF-I is given subcutaneously twice a day. The dose of IGF-I is increased each day for 4 days. Glucose is administered intravenously, when necessary, to prevent hypoglycemia. Following the study treatment patients resume prior medication and may undergo surgery.

Ages Eligible for Study:  up to  3 Months,  Genders Eligible for Study:  Both

Criteria

PROTOCOL ENTRY CRITERIA:

--Disease Characteristics--

• Diagnosis of hyperinsulinism (i.e., evidence of fasting hypoglycemia with inadequate suppression of insulin, normal pituitary and adrenal function, and increased insulin action)
• Intractable hypoglycemia (i.e., persistent IV glucose requirement for maintaining glucose levels greater than 60 mg/dL)
• Failed standard treatment regimen of diazoxide, octreotide, and frequent feedings to control hypoglycemia
• No other major medical problems