Hypertensive virilizing adrenal hyperplasia with minimal impairment of synthetic route to cortisol.

One of the first described cases of hypertensive virilizing adrenal hyperplasia (VAH)(Pediatrics 8: 805, 1951) has been followed from age 2½ until age 26. Bloodpressure as an infant was 150/90, and at age 25 was 220/160. During childhood the patient was lost tofollow-up for prolonged periods, and received no therapy from age 20 to 25. At this time 24 h urinary excretion of 17-ketosteroids was 89 mg; tetrahydro 11-deoxycortisol (tetrahydro S), 47 mg and pregnanetriol 5.7 mg Hourly measurements of several plasma steroids utilizing sephadex LH 20 chromatography and competitive protein binding were made during 24 h; concentration ranges were as follows (μg/100 ml): 11-deoxycortisol 8–40; cortisol 0–48;corticosterone 0–1.5; deoxycorticosterone 1–18. Plasma cortisol, especially showed a significantmorning impairment, but reached normal and even markedly elevated levels during the day and early evening. Urinary cyclic AMP per 24 h ranged from 5.3 to 11.6 n mol/mg creatinine before therapy, and was 1.9 n mol afte...