Hypertensive Virilizing Adrenal Hyperplasia with Minimal Impairment of Synthetic Route to Cortisol

Publisher Website
Google Scholar
Abstract
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 after therapy. The results suggest either the formation of analternate pathway to cortisol synthesis, or the existence of a form of VAH with two independent 11-B hydroxylating systems, exhibiting only minimal impairment of the synthetic route to cortisol. The latter would support the presence of two independent 11-B hydroxylating systems in the normal human adrenalThis has been suggested by Zachmann et al. (J Clin Endocrinol Metab33: 501, 1971) to be true in infancy. Our observations on an adult indicate that these two systems may not be transitory, but persist into adulthood.

This publication has 0 references indexed in Scilit: