Circadian levels of 17-OH progesterone(17-OHP),testosterone(T) and cortisol(F) in children with congenital adrenal hyperplasia(CAH) with and without treatment

Abstract

Top of pageAbstract In 9 girls and 2 boys age 2 9/12 to 16 7/12 yrs.with CAH (21 hydroxylase deficiency) blood was drawn in 9o min intervals over a 24h period for determination of 17-OHP,T and F; 17 ketosteroids (17-KS) and pregnantriol (P3) were measured in the 24h urine. These parameters were determined after interruption of therapy as well as under different therapeutic regimens. 17-OHP had marked diurnal variations;after treatment there was an inverse course of 17-OHP to the substituted corticosteroids;when the lower dose was given in the evening the onset of the nocturnal 17-OHP rise occured earlier and the levels were even higher;with all therapeutic regimens the T peak values were found during the night.The plasmaF-levels distinctly reflected the orally administered dose of corticosteroids;in the untreated cases a decreased diurnal rhythm was observed.There was a good correlation between T and 17-KS as well as between 17-OHP and P3,respectively.We conclude that: 1. the daytime of 17-OHP sampling and the interval to the drug administration should be standardized; 2. Corticosteroids should be administered 3 times daily with the highest dose in the evening.