title: Dynamics of 24-hour pulsatile cortisol, 17-hydroxyprogesterone, and androstenedione release in prepubertal patients with nonclassic 21-hydroxylase deficiency and normal prepubertal children.
abstract: To assess whether the quantitative and qualitative aspects of cortisol, 17-hydroxyprogesterone (17-OHP), and androstenedione (D4A) secretion in patients with nonclassic congenital adrenal hyperplasia (NCCAH) differ from those in normal children, 24-hour serum concentrations of these steroids were measured in five prepubertal patients with NCCAH and five normal prepubertal children. Adrenal steroid profiles obtained by 30-minute sampling were analyzed by the Pulsar program. In comparison to normal children, the 24-hour quantitative parameters of 17-OHP and D4A secretion were significantly greater in NCCAH patients, but serum cortisol concentrations were similar in the two groups. When daytime and nighttime hormone releases were separately analyzed, a significant nocturnal elevation of the cortisol area under the curve above zero level (AUCo) and 12-hour mean and 17-OHP AUCo, AUC above baseline, mean peak height, amplitude, area, and 12-hour mean was detected in normal subjects only. Conversely, NCCAH patients exhibited an increased frequency and number of 17-OHP secretory peaks at night together with a reduction of the interpeak interval. No significant day/night differences in D4A concentrations were detected either in normals or in the patients. In conclusion, the results of the present study indicate that patients with NCCAH have a distinct pattern of adrenal steroid secretion characterized by a high-frequency 17-OHP release accompanied by a relative nocturnal cortisol deficiency.
authors.affiliation: Department of Pediatrics, University of Parma, Italy.
authors.forename: L
authors.initials: L
authors.lastname: Ghizzoni
authors.forename: S
authors.initials: S
authors.lastname: Bernasconi
authors.forename: R
authors.initials: R
authors.lastname: Virdis
authors.forename: A
authors.initials: A
authors.lastname: Vottero
authors.forename: M
authors.initials: M
authors.lastname: Ziveri
authors.forename: C
authors.initials: C
authors.lastname: Volta
authors.forename: L
authors.initials: L
authors.lastname: Iughetti
authors.forename: G
authors.initials: G
authors.lastname: Giovannelli
mesh_terms: D019326:17-alpha-Hydroxyprogesterone; D000312:Adrenal Hyperplasia, Congenital / Q000097:blood* / Q000503:physiopathology; D000324:Adrenocorticotropic Hormone / Q000494:pharmacology; D000375:Aging / Q000097:blood / Q000502:physiology; D000735:Androstenedione / Q000097:blood*; D002648:Child; D002940:Circadian Rhythm / Q000502:physiology*; D005260:Female; D006801:Humans; D006854:Hydrocortisone / Q000097:blood*; D006908:Hydroxyprogesterones / Q000097:blood*; D008297:Male; D011627:Puberty / Q000097:blood* / Q000502:physiology; D011863:Radioimmunoassay
chemical_list: D006908:Hydroxyprogesterones; D000735:Androstenedione; D019326:17-alpha-Hydroxyprogesterone; D000324:Adrenocorticotropic Hormone; D006854:Hydrocortisone