Pituitary Function in Hand-Schüller-Christian Disease

Abstract

Anterior and posterior pituitary function was evaluated in 13 patients with Hand–Schuller–Christian disease, including eight with growth retardation. Growth-hormone reserve, determined by insulin-induced hypoglycemia, arginine tolerance test or pyrogen stimulation, was deficient in seven of the eight short patients and in three of the patients whose disease developed after adult height had been attained. Tests of gonadotropin secretion and adrenocortical and thyroid function gave normal results. A dehydration test demonstrated complete diabetes insipidus in eight patients and incomplete diabetes insipidus in two. Growth-hormone deficiency was usually found when diabetes insipidus was present. Both abnormalities may be accounted for by a single hypothalamic lesion. The short stature frequently observed in patients with this disease may be in part secondary to quantitative growth-hormone deficiency.