URINARY KETOSTEROID EXCRETION PATTERNS IN CONGENITAL ADRENAL HYPERPLASIAF*

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Abstract
THE present concepts of the aberrant mechanisms in congenital adrenal hyperplasia have recently been summarized by Jailer et al. (1), Bongiovanni and Eberlein (2) and Dorfman (3). The basic defect is thought to be the inability of the adrenal cortices to produce adequate amounts of hydrocortisone. The low levels of blood hydrocortisone lead to a compensatory increase in secretion of corticotropin by the anterior pituitary gland, and consequent adrenal hyperplasia. The hyperplastic adrenal cortices, unable to effect the adequate synthesis of hydrocortisone, secrete, among other steroids, androgen(s) or androgen precursor(s) which bring about the clinical picture. The identity of the androgenic compounds which induce the signs of virilism is uncertain. Dorfman (4) believed that the androgenicity could be attributed to the C19 adrenal steroids such as 4-androstene-3,17-dione (androstenedione) and 11-hydroxyandrostene-3,17-dione (11-hydroxyandrostenedione), whereas Jailer et al. (1) held that the intermediates such as 17-hydroxyprogesterone and 21-desoxyhydrocortisone or their metabolites such as androsterone and 11-hydroxyandrosterone, could account for the virilization.