Abstract
Plasma levels of aldosterone(PA) and 17hydroxyprogesterone(17-OHP), urinary pregnanetriol (PT), pregnanetriolone and 17-ketosteroids were determined in 19 children with CAH. 17-OHP in 5 cases before onset of therapy was at least 40 times above normal range, PA also being elevated. Well controlled patients had normal PA and 17-OHP below 5 ng/ml. Both steroids were well stimulable by ACTH. In 3 poorly controlled cases elevated PT and 17-OHP up to 131 ng/ml were found, Withdrawal of therapy resulted in pronounced rise in PA and 17-OHP. Additional exogeneous ACTH had no effect in 7 cases leading to further stimulation in the other children. Correlation between 17-OHP and PT was excellent (r=0,84, p<0,003) and fairly good between PA and 17-OHP (r=0,65, p<0,01). The data confirm the value of 17-OHP determinations for diagnosis and management of CAH on the one hand, and the compensatory elevated aldosterone production on the other.
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