Functioning Islet Cell Adenoma with a Note about Leucine Sensitivity

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Abstract
Two cases of spontaneous hypoglycemia due to functioning islet cell adenoma are described. In case 1 extensive laboratory examinations of liver and endocrine functions were normal. A convulsion was precipitated by a 20 hour fast when the blood sugar measured 30 mg/100 ml and the serum level for insulin-like activity was elevated. Whereas glucagon elevated the blood sugar levels to normal, cortisone and epinephrine did not. Leucine 0.32 g/kg body weight caused a 26% decrease in blood sugar levels. Case 2 suffered a convulsion after 21 hours of fast when the blood sugar level was 17 mg/100 ml. Leucine (0.15 g/kg) depressed the blood sugar levels by 85% at 75 minutes when convulsion occurred. A postoperative leucine test was negative. Both cases illustrate the importance of a prolonged fast in establishing the diagnosis of functioning islet cell tumor. The demonstration of leucine sensitivity in case 2 and the absence of it in case 1 indicate that leucine sensitivity is not specific for the leucine sensitive hypoglycemia of childhood and does not rule out or confirm pancreatic islet cell tumor as a cause of hypoglycemia. The possibility that leucine in some instances can be a direct stimulus for insulin production is suggested.

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