Congenital Adrenal Hyperplasia: Simultaneous Determination of Plasma Aldosterone and 17-Hydroxyprogesterone

Abstract

Plasma levels of aldosterne and 17-hydroxyprogesterone were determined in 5 just diagnosed cases of congenital adrenal hyperplasia due to 21-hydroxylase deficiency without salt-loss before onset of therapy, and in 14 children suffering from the same disorder, under glucocorticoid therapy before and after withdrawal of therapy for one week as well as before and after ACTH-stimulation. Furthermore, urinary pregnanetriol, pregnanetriolone, 17-ketosteroids and 17-ketogenic steroids were determined with and without therapy. Serum concentrations of 17-hydroxyprogesterone in patients before onset of therapy were at least 40 times above the normal range (normal range: 0.39 ± 0.23 ng/ml determined in 19 children of age 1–12 years), aldosterone was also elevated (range 16.5–84.4 ng/100 ml, normal aldosterone being 7.3 ± 4.7 ng/ml). In contrast, well controlled patients (judged on urinary pregnanetriol-excretion) had normal aldosterone (one exception) and 17-hydroxyprogesterone levels below 5 ng/ml. Both steroids were well stimulated by ACTH. In 3 poorly controlled cases elevated pregnanetriol and 17-hydroxyprogesterone values up to 130.9 ng/ml were found. Withdrawal of therapy resulted in pronounced rise of aldosterone and 17-hydroxyprogesterone. Exogenous ACTH had no detectable effect in 7 cases leading to further stimulation in the other children. We found an excellent correlation between plasma 17-hydroxyprogesterone and urinary pregnanetriol (r = 0.84, P < 0.003, n = 14). Fairly good correlation also exists between aldosterone and 17-hydroxyprogesterone values (r = 0.65, P < 0.01, n = 14) in patients before the onset and after withdrawal of therapy. The data confirm the value of 17-hydroxyprogesterone determinations for diagnosis and management of congenital adrenal hyperplasia on the one hand, and the compensatory elevated aldosterone production in the non-salt-losing form of the disorder on the other.

A Simple Procedure for the Radioimmunoassay of 17α-Hydroxyprogesterone in Serum: Comparison with an Immunological Purification Technique

“…The detection of a diminished 21-hydroxylase activity in the non-salt losing form of the disease in children and adults can be revealed by ACTH stimulation and concurrent measurement of blood levels of 17-0HP or pregnanetriol in urine Barnes and Atherden, 1972;Givens et al, 1975;Newmark et al, 1977;Parth et al, 1978). The monitoring of adequacy of treatment by the sequential measurement of 17-0HP in serum has been favoured (Loras et al, 1974;Hughes and Winter, 1978).…”

“…A number of radioimmunoassay methods have been described (Abraham et al, 1971;Youssefnejadian et al, 1972;Franks, 1974;Hami et al, 1975;Janne et al, 1975;Wickings, 1975;Kaplan, 1977;Forest and Cathiard, 1978;Hughes and Winter, 1978;Parth et al, 1978), but in the majority various degrees of purification of the serum extract are considered necessary. This detracts from the value of these methods when a result is required urgently.…”