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Case Reports
August 1940

HAND-SCHÜLLER-CHRISTIAN DISEASE: REPORT OF A CASE IN WHICH URINARY ANDROGENS WERE TITRATED

Author Affiliations

DURHAM, N. C.
From the Endocrine Division of the Department of Obstetrics and Gynecology and from the Department of Pediatrics, Duke University School of Medicine.

Am J Dis Child. 1940;60(2):352-358. doi:10.1001/archpedi.1940.02000020106010
Abstract

Rowland1 has suggested that Hand-Schüller-Christian disease is due to a primary constitutional alteration in the metabolism of lipoids, which results in an excessive accumulation of lipoids in the blood and in other tissue fluids and which gives rise to a massive storage or infiltration in various tissues of the body. Thus, this disease is viewed as belonging to a general group of lipoid disturbances which includes Niemann-Pick disease, Gaucher's disease and cutaneous xanthomatosis.

Most descriptions of the condition refer to associated alterations in endocrine functions. Christian2 regarded the disease as an "unusual syndrome of dyspituitarism." Diabetes insipidus, a common symptom, suggests possible involvement of the intermediate and the posterior lobes of the pituitary. The fact that the disease has been associated with intercurrent acromegaly, with adiposogenitalism, with dwarfism accompanied by genital hypoplasia and with pituitary cachexia (Simmonds' disease) is an additional reason for the belief that the pituitary

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