Gonadal dysgenesis and leucine-sensitive hypoglycemia

THE S Y N D R O M E of gonadal dysgenesis is characterized by short stature, sexual infantilism due to dysfunctional "streak" ovaries, and a broad spectrum of somatic abnormalities. Patients with Turner syndrome have been reported to have a greater frequency of diabetes mellitus, thyroiditis, and Addison disease. 1 Hypoglycemia and hyperinsulinism have not been documented previously. This report describes two children with Turner syndrome and leucine-sensitive hypoglycemia.