PEDIATRICS Vol. 65 No. 4 April 1980, pp. 777-781
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Neonatal Salt Loss in the Hypertensive Form of Congenital Adrenal Hyperplasia

J. H. Holcombe MD1, B. S. Keenan MD1, B. L. Nichols MD1, R. T. Kirkland MD1, and G. W. Clayton MD1

1 Department of Pediatrics, Section of Nutrition and Gastoenterology and Section of Endocrinology, Baylor College of Medicine, Houston

The 11beta-hydroxylase deficiency (11OHD) form of congenital adrenal hyperplasia is diagnosed infrequently during the newborn period. A child presumed to have the 21-hydroxylase deficiency form of congenital adrenal hyperplasia was studied extensively as an infant. The diagnosis was based on ambiguous genitalia, elevated 17-ketosteroids, evidence of urinary 11-ketopregnanetriol, and salt loss. Severe hypertension was detected at 11 years, and 11beta-hydroxylase deficiency was confirmed with elevated plasma 11-deoxycorticosterone and 11-deoxycortisol, low cortisol, and normalization of blood pressure following glucocorticoid replacement. Impaired aldosterone biosynthesis and salt loss were demonstrated during dexamethasone therapy. Salt loss during infancy does not distinguish between the 11beta- and 21-hydroxylase deficiency forms of congenital adrenal hyperplasia.

Submitted on January 15, 1979
Accepted on June 18, 1979