Urinary Steroid Patterns and Loss of Salt in Congenital Adrenal Hyperplasia

Abstract
Excretion of aldosterone, pregnanetriol, pregnanediol, 17-ketosteroids, tetrahydrocortlsone (THE: 3[beta],17,21-trihydroxy-5[beta]-pregnane-ll,20-dione) and tetrahydro-11-deoxycortisol (THS; 3[alpha],17,21-trihydroxy-5[beta]-pregnan-20-one) was measured in 13 patients with congenital adrenal hyperplasia. An attempt was made to correlate the steroid excretion pattern with the tendency to lose salt. Nine of the patients maintained normal levels of serum sodium and excreted normal or increased amounts of aldosterone. Most of them excreted increased amounts of aldosterone when maintained on a low-salt diet. Four of the 13 patients were unable to maintain normal levels of serum sodium; one excreted no detectable of aldosterone and 3 excreted essentially normal amounts. The salt losers also excreted very small quantities of THE. There appeared to be a good correlation between the tendency to lose salt and the ability of the patient to increase urinary aldosterone excretion in response to salt depletion or to the administration of adrenocorticotrophin. There also appeared to be a good correlation between loss of salt and the ratio of urinary aldosterone to urinary pregnanetriol.