Islet cell tumor in the neonate. Studies in carbohydrate metabolism and therapeutic response.

Abstract

The earliest documented case of hypoglycemia resulting from a functioning islet cell adenoma is reported. Hypoglycemia was diagnosed at 3 hours of age in a male infant with a birth weight of 3,177 gm (7 lb). The pregnancy and delivery had been uneventful. The infant was considered to have severe idiopathic hypoglycemia of infancy. Pertinent laboratory features included the absence of documented hyperinsulinism, elevated plasma growth hormone, symptomatic hypoglycemia following oral glucose load, standard meal and glucagon administration, leucine and insulin sensitivity, and a normal response to I.V. tolbutamide. The clinical course was characterized by persistent hypoglycemia despite a therapeutic regimen consisting of ACTH, epinephrine, ephiedrine, glucagon, human growth hormone, chlorothiazides, trichlormethiazides, and diazoxide of optimum dosage and duration. Preoperatively, the growth pattern was along the 3rd percentile, skeletal maturation was less than birth level at age 6 months, mental age was two months behind the chronologic age, and the EEG was abnormal. After subtotal pancreatectomy at age 73½ months, the growth pattern reached the 75th percentile, skeletal maturation improved to the 6-month level within a 7-month postoperative period, mental age became consistent with chronologic age, and the EEG returned to normal.