No linkage between HLA and congenital adrenal hyperplasia due to 17-alpha-hydroxylase deficiency.

To the Editor: Congenital adrenal hyperplasia usually results from a deficiency of one of the enzymes involved in the synthesis of cortisol, aldosterone, and sex hormones. An unusual form of this disease is caused by 17-alpha-hydroxylase deficiency (17-OHDS). Affected females have signs of hypoestrogenism, and genetic males have signs of pseudohermaphroditism, because 17-alpha-hydroxylase is common to adrenals and gonads and its presence is required for the synthesis of sex hormones. Hypertension may be present because of an excess of mineralocorticoids other than aldosterone. The question of a linkage between the HLA complex and two other forms of congenital adrenal. © 1980, Massachusetts Medical Society. All rights reserved.