FUNCTIONING PANCREATIC ISLET CELL ADENOMA. WITH LEUCINE-INDUCED HYPOGLYCEMIA.

Abstract

Cochrane, Payne, Simpkiss, and Wolff first described in 1956 1 a type of hypoglycemia in infants which was precipitated or aggravated by feedings containing L-leucine. Approximately 60 children with this familial disorder have been reported. 2 Weisenfeld and Goldner recently reviewed the incidence ofL-leucine sensitivity in adults. 3 They found five instances of leucine-induced hypoglycemia in parents of L-leucine-sensitive children. All were asymptomatic as adults, although three gave a history of childhood convulsions. Seven adults withL-leucine-sensitive hypoglycemia but without a family history of the disease have been reported. 3-7 These adults had adenomas or carcinomas of the islets of Langerhans. To our knowledge this report describes the eighth patient with organic hyperinsulinism in whom hypoglycemia was induced by the oral administration ofL-leucine. Her clinical history initially suggested a focal brain lesion. Report of a Case First Admission (May 31, 1962). —A 51-year-old, right-handed, white