Relation of Urinary 17-Ketogenic Steroids to Porter-Silber Chromogens in Certain Adrenal Cortical Disorders and “Idiopathic” Hirsutism

Abstract
Urinary 17-ketogenic steroids (17-KGS) were found to be disproportionately elevated compared to Porter-Silber chromogens (PSC) in 3 patients with congenital adrenal hyperplasia, 2 with adrenocortical carcinoma, 2 with virilizing adrenocortical hyperplasia and 21 with idiopathic hirsutism. Pregnanetriol accounted for the elevated 17-KGS only in the patients with congenital adrenal hyperplasia. In patients with idiopathic hirsutism, 12 of a group of 21 were observed to have a large excess of 17-KGS over PSC. Cortisol secretion rate was determined in only 1 patient in this subgroup, who had a high 17-KGS/PSC ratio which was found to be abnormally elevated to 37 mg/24 hr, yet the urinary PSC did not reveal this increased cortisol secretion although the 17-KGS suggested it. It appears that some patients with adrenal cortical disorders, and especially those with idiopathic hirsutism, metabolize cortisol excessively through pathways leading to excretion of adrenocorticoids, which are measured as 17-KGS but not as PSC, possibly cortolones. Determination of 17-KGS may reveal such cases not detected by urinary PSC. Conversely, elevation of 17-KGS instead of PSC in a hirsute woman should not be relied upon to confirm the diagnosis of Cushing's syndrome without a concurrent check on the PSC.