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Hypothalamic Endocrinopathy in Hand-Schüller-Christian Disease

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Grant support: U.S. Public Health Service Grants AM 14039, HD 08555, and RR40.

University of Pennsylvania School of Medicine;
Philadelphia, Pennsylvania

Ann Intern Med. 1978;88(4):512-513. doi:10.7326/0003-4819-88-4-512
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Hand-SchÜller-Christian disease is characterized by infiltration of multiple organs by well-differentiated histiocytes. Diabetes insipidus is a well-known manifestation of this disease, and anterior pituitary hormone deficiencies also occur (1). We describe a patient with Hand-Schüller-Christian disease whose hypothalamic-pituitary function was tested extensively. The results support the hypothesis that Hand-Schüller-Christian disease causes a hypothalamic endocrinopathy.

A 19-year-old man was referred for evaluation of polyuria and a goiter. He also had progressive exertional dyspnea and left axillary and right inguinal skin lesions.

The thyroid was symmetrically enlarged, firm, and smooth, each lobe measuring 10 x 5 cm. There were several left axillary


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