The role of adrenocorticotropin testing in evaluating girls with premature adrenarche and hirsutism/oligomenorrhea.
To identify biochemical predictors for future development of hirsutism and/or oligomenorrhea (H/O) in girls with premature adrenarche (PA), we performed dexamethasone-suppressed ACTH stimulation tests in girls with PA (n = 46), young women (n = 44) with H/O, and adult women (n = 31). Cortisol, androstenedione, dehydroepiandrosterone, and 17-hydroxyprogesterone were measured. Seven girls with PA (15%) and seven with H/O (16%) had evidence of nonclassical adrenal steroid biosynthetic defects [nonclassical congenital adrenal hyperplasia (NCAH)]. Twenty-five girls with PA (54%) and 28 girls with H/O (64%) had the moderately elevated 17-hydroxyprogesterone response to ACTH that has been reported in obligate heterozygotes for 21-hydroxylase deficiency. There were no clinical features that distinguished the girls with NCAH from the others. ACTH testing is an important tool in distinguishing those girls with PA and H/O who have NCAH. Although we could find no differences in other adrenal steroid hormones that might predict which of the other girls with PA might late develop H/O, black girls comprised a substantially smaller fraction of the population with H/O than of the population with PA (2% vs. 26%; chi 2 = 8.5; P less than 0.005). This observation suggests that PA, in blacks who do not have NCAH, is more likely to be a benign condition/than in other ethnic groups.
Hawkins
L A
LA
Department of Pediatrics, Schneider Children's Hospital of Long Island, Jewish Medical Center, New Hyde Park, New York 11042.
Chasalow
F I
FI
Blethen
S L
SL
D019326:17-alpha-Hydroxyprogesterone; D000152:Acne Vulgaris / Q000209:etiology; D000293:Adolescent; D000302:Adrenal Cortex / Q000254:growth & development*; D000303:Adrenal Cortex Diseases / Q000175:diagnosis / Q000503:physiopathology*; D000312:Adrenal Hyperplasia, Congenital; D000324:Adrenocorticotropic Hormone; D000328:Adult; D002648:Child; D003687:Dehydroepiandrosterone / Q000097:blood; D003907:Dexamethasone; D005260:Female; D006628:Hirsutism / Q000175:diagnosis / Q000503:physiopathology*; D006801:Humans; D006908:Hydroxyprogesterones / Q000097:blood*; D008572:Menarche; D009839:Oligomenorrhea / Q000175:diagnosis / Q000503:physiopathology*; D013256:Steroids / Q000096:biosynthesis
D006908:Hydroxyprogesterones; D013256:Steroids; D003687:Dehydroepiandrosterone; D019326:17-alpha-Hydroxyprogesterone; D003907:Dexamethasone; D000324:Adrenocorticotropic Hormone