Dynamics of 24-hour pulsatile cortisol, 17-hydroxyprogesterone, and androstenedione release in prepubertal patients with nonclassic 21-hydroxylase deficiency and normal prepubertal children

Metabolism. 1994 Mar;43(3):372-7. doi: 10.1016/0026-0495(94)90107-4.

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.

Publication types

  • Comparative Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • 17-alpha-Hydroxyprogesterone
  • Adrenal Hyperplasia, Congenital* / blood*
  • Adrenal Hyperplasia, Congenital* / physiopathology
  • Adrenocorticotropic Hormone / pharmacology
  • Aging / blood
  • Aging / physiology
  • Androstenedione / blood*
  • Child
  • Circadian Rhythm / physiology*
  • Female
  • Humans
  • Hydrocortisone / blood*
  • Hydroxyprogesterones / blood*
  • Male
  • Puberty / blood*
  • Puberty / physiology
  • Radioimmunoassay

Substances

  • Hydroxyprogesterones
  • Androstenedione
  • 17-alpha-Hydroxyprogesterone
  • Adrenocorticotropic Hormone
  • Hydrocortisone