Short Stature and Growth Hormone Deficiency Due to Histiocytosis X

Abstract

Diabetes insipidus (DI) is a common complication of histiocytosis X. Anterior pituitary function has not been previously systematically studied. Both anterior and posterior pituitary function has been evaluated in 11 children with histiocytosis. The following methods were used. Insulin hypoglycemia and arginine infusion for HGH, serum PBI, T4, and 191I uptake for thyroid function, and metyrapone stimulation for adrenal function. Concentrating ability was used as an index of vasopressin release. Gonadotropin was assessed by physical examination only. In 6 patients, only vasopressin and growth hormone (GH) response was studied. In 5 thyroid and adrenocortical evaluation was alos obtianed. Of the 11 children, 4 were of normal height for age, while 7 were below the 3rd percentile. Those children with normal height all had normal GH response. Two of them had DI with normal thyroid and adrenal function. Of the 7 children with retardation of linear growth, 6 were found to be GH deficient. The 1 child with normal GH had a strong family history of short stature. Five had DI and all were GH deficient. Thyroid studies were normal in 2 and adrenal studies in 3 of these. One child had histiocytosis of the thyroid gland with goiter and hypothyroidism. In 3 children of adolescent age with short stature, GH deficiency and DI, puberty was delayed. This study documents that histocytosis may involve the anterior and/or posterior pituitary. An adequate clinical evaluation should include study of the anterior pituitary.