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Development in a Patient With Salt-Losing Congenital Adrenal Hyperplasia

Arthur Bauman, MD; Caroline G. Bauman, MD

JAMA. 1982;248(23):3140-3141.

	Since this article does not have an abstract, we have provided the first 150 words of the full text PDF and any section headings.

CONGENITAL adrenal hyperplasia (CAH) is an uncommon disorder, but the incidence of virilizing adrenocortical neoplasms, benign and malignant, is even less common.¹ The occurrence of both conditions in the same patient^2-5 raises the question of whether or not the association is coincidental.

The patient described herein was born with a salt-losing form of CAH, probably caused by a severe 21-hydroxylase deficiency; despite adequate control, a virilizing carcinoma of the adrenal cortex developed.

Report of a Case

An 11-year-old girl was admitted to the White Plains (NY) Hospital Medical Center because of progressive virilization associated with strikingly increasing levels of urinary 17-ketosteroids (17-KS).

During the first week of life, the patient had had persistent vomiting, weakness, and convulsions. Serum sodium level had been 126 mEq/L, and serum potassium level, 9.2 mEq/L. The clitoris had been enlarged and the labia majora fused. A cystourethrogram had shown dye in a vaginal . . . [Full Text PDF of this Article]

Author Affiliations
From the Section of Endocrinology, Department of Medicine (Dr A. Bauman), and the Department of Pediatrics (Dr C. G. Bauman), White Plains (NY) Hospital Medical Center.

Footnotes
Reprint requests to 115 Davis Ave, White Plains, NY 10605 (Dr A. Bauman).

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