URINARY STEROIDS IN THE HYPERTENSIVE FORM OF CONGENITAL ADRENAL HYPERPLASIA*†

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Abstract
Four patients with the hypertensive form of virilizing adrenal hyperplasia were studied and compared with 6 patients with the simple virilizing type, and 7 others with the salt-losing type of this syndrome. The urinary patterns of excretion of total 17-ketosteroids, pregnanetriol, tetrahydrocortisol, tetrahydrocortisone, tetrahydro-S and tetrahydrodesoxycorticosterone were compared in each type of manifestation of this syndrome. The results show a significant variation in the degree of enzymatic defects present, with the majority of patients demonstrating an ability to perform the sequential enzymatic processes of hydroxylation necessary for the synthesis of cortisol. Prolonged stimulation with ACTH did not significantly increase this ability. In 2 siblings with the hypertensive form of the syndrome, clinical manifestation appeared only after puberty, indicating that this disorder may be a cause of acquired hypertension in the adult. The rarity of this abnormality is indicated by the negative results in a survey of a large hypertension clinic and a female hirsutism clinic. Values for urinary 17-ketosteroids and pregnanetriol may be misleading in the diagnosis of the hypertensive adrenogenital syndrome, for these may be within normal ranges even though virilization and hypertension are progressing. Fractionation of the urinary corticosteroids was necessary to prove the diagnosis in 2 cases.

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