I Avivi1, S Pollack, O Gideoni, S Linn, Z Blumenfeld. 1. Rambam Medical Center, Bruce Rappaport Faculty of Medicine, Technion-Israel Institute of Technology, Haifa, Israel.
Abstract
OBJECTIVE: To evaluate the reliability of the ACTH test as a means for detection of late onset congenital adrenal hyperplasia (CAH) and discriminating it from polycystic ovary syndrome (PCOS), by repeating the test after 6 months of cyproterone acetate and ethinyl E2 treatment. DESIGN: Follow-up comparison study. SETTING: Reproductive Endocrinology in an university tertiary center. PATIENTS: Thirty-one young women with hirsutism, oligoamenorrhea, and acne, 21 of them detected as late onset CAH, and 10 as non-late onset CAH (PCOS). INTERVENTION: Cyproterone acetate and ethinyl E2 treatment for > or = 6 months. The ACTH test, before and after 6 months of cyproterone acetate + ethinyl E2 treatment, and human leukocyte antigen (HLA) typing. MAIN OUTCOME MEASURE: The ACTH test interpretation correlated to HLA typing. RESULTS: By repeating the ACTH stimulation test in the 31 women (after cyproterone acetate + ethinyl E2 administration), we found a diminution in the rate of accumulation of 17 alpha-hydroxyprogesterone (delta 17-OHP) + P, in all 21-hydroxylase late onset CAH cases. As a result of treatment with cyproterone acetate + ethinyl E2, a decrease in the accumulation rate of 17-OHP + P, below the discriminative value for late onset CAH (6.5 ng/dL per minute), was noted among 12 of 21 women defined primarily as late onset CAH. Among the nine other women, a decrease in the accumulation rate of 17-OHP + P was noted, however not < 6.5 ng/dL per minute. CONCLUSIONS: The interpretation of delta 17-OHP + P for the diagnosis of late onset CAH may be too sensitive as to the correct clinical diagnosis of late onset CAH. By repeating the ACTH test after 6 months of treatment with cyproterone acetate-ethinyl E2, specificity and accuracy may be improved.
OBJECTIVE: To evaluate the reliability of the ACTH test as a means for detection of late onset congenital adrenal hyperplasia (CAH) and discriminating it from polycystic ovary syndrome (PCOS), by repeating the test after 6 months of cyproterone acetate and ethinyl E2 treatment. DESIGN: Follow-up comparison study. SETTING: Reproductive Endocrinology in an university tertiary center. PATIENTS: Thirty-one young women with hirsutism, oligoamenorrhea, and acne, 21 of them detected as late onset CAH, and 10 as non-late onset CAH (PCOS). INTERVENTION: Cyproterone acetate and ethinyl E2 treatment for > or = 6 months. The ACTH test, before and after 6 months of cyproterone acetate + ethinyl E2 treatment, and human leukocyte antigen (HLA) typing. MAIN OUTCOME MEASURE: The ACTH test interpretation correlated to HLA typing. RESULTS: By repeating the ACTH stimulation test in the 31 women (after cyproterone acetate + ethinyl E2 administration), we found a diminution in the rate of accumulation of 17 alpha-hydroxyprogesterone (delta 17-OHP) + P, in all 21-hydroxylase late onset CAH cases. As a result of treatment with cyproterone acetate + ethinyl E2, a decrease in the accumulation rate of 17-OHP + P, below the discriminative value for late onset CAH (6.5 ng/dL per minute), was noted among 12 of 21 women defined primarily as late onset CAH. Among the nine other women, a decrease in the accumulation rate of 17-OHP + P was noted, however not < 6.5 ng/dL per minute. CONCLUSIONS: The interpretation of delta 17-OHP + P for the diagnosis of late onset CAH may be too sensitive as to the correct clinical diagnosis of late onset CAH. By repeating the ACTH test after 6 months of treatment with cyproterone acetate-ethinyl E2, specificity and accuracy may be improved.
Authors: C Manieri; C Di Bisceglie; R Fornengo; T Grosso; E Zumpano; F Calvo; E Berardengo; M Volante; M Papotti Journal: J Endocrinol Invest Date: 1998-02 Impact factor: 4.256