title: Blunted adrenarche in patients with classical congenital adrenal hyperplasia due to 21-hydroxylase deficiency.
abstract: Dehydroepiandrosterone sulfate (DHEAS) concentration levels were measured by a specific RIA in 23 female and 7 male patients with classical congenital adrenal hyperplasia (CAH) salt-losing type due to steroid 21-hydroxylase (21-OH) deficiency. The patients were divided into four groups by age and the DHEAS concentrations (mean +/- SD; micrograms/dl) for each age group were: 5 to 8 years (10.2 +/- 6.5; range 5-23 micrograms/dl); 8 to 10 years (18.3 +/- 14.9; range 5-44 micrograms/dl); 10 to 18 years (41.9 +/- 40; range 5-160 micrograms/dl); and above 18 years (49.7 +/- 65.9; range 9 to 242 micrograms/dl). These DHEAS levels were compared to age-matched normal values and it was found that DHEAS concentrations in 29 of the 30 patients were less than or in the lower part of the normal range for all age groupings. The DHEAS levels did not correlate with 17 alpha-hydroxyprogesterone (17-OHP), androstenedione, and testosterone values. The data indicate that blunted adrenarche occurs in classical CAH patients with 21-OH deficiency.
authors.affiliation: Children's Hospital of Buffalo, Department of Pediatrics, School of Medicine, State University of New York at Buffalo 14222, USA.
authors.forename: E P
authors.initials: EP
authors.lastname: Sellers
authors.forename: M H
authors.initials: MH
authors.lastname: MacGillivray
mesh_terms: D019326:17-alpha-Hydroxyprogesterone; D000293:Adolescent; D000302:Adrenal Cortex / Q000254:growth & development*; D000312:Adrenal Hyperplasia, Congenital / Q000503:physiopathology; D000328:Adult; D000735:Androstenedione / Q000097:blood; D002648:Child; D002675:Child, Preschool; D003687:Dehydroepiandrosterone / Q000031:analogs & derivatives / Q000097:blood; D019314:Dehydroepiandrosterone Sulfate; D005260:Female; D006801:Humans; D006908:Hydroxyprogesterones / Q000097:blood; D008297:Male; D012016:Reference Values; D013739:Testosterone / Q000097:blood
chemical_list: D006908:Hydroxyprogesterones; D013739:Testosterone; D000735:Androstenedione; D003687:Dehydroepiandrosterone; D019314:Dehydroepiandrosterone Sulfate; D019326:17-alpha-Hydroxyprogesterone