Adrenocortical hyperresponsivity to adrenocorticotropic hormone: A mechanism favoring the normal production of cortisol in 21-hydroxylase-deficient nonclassic adrenal hyperplasia

Academic Article


  • Objective: To test the hypothesis that patients with nonclassic adrenal hyperplasia (NCAH) exhibit a generalized exaggeration in their response to ACTH stimulation that favors the normal production of F. Patients with 21-hydroxylase (21-OH)-deficient NCAH do not demonstrate cortisol (F) deficiency. Design: Prospective controlled study.Setting: Tertiary university clinic.Patient(s): Twenty-four untreated patients with NCAH diagnosed by a 17α-hydroxyprogesterone (17-HP) level of >30.3 nmol/L (>10 ng/mL), and 37 age- and body mass-matched healthy eumenorrheic nonhirsute controls. Intervention(s): All study subjects underwent a 60 minute acute stimulation using 0.25 mg of ACTH-(1-24) i.v. Main Outcome Measure(s): Basal and stimulated serum levels of pregnenolone (PREG), 17-hydroxypregnenolone (17-HPREG), dehydroepiandrosterone (DHA), progesterone (P4), 17-HP, androstenedione (A4), 11-deoxycortisol (S), and cortisol (F). Result(s): The median basal (i.e., Steroid0) or ACTH-stimulated (i.e., Steroid60) serum levels of PREG, 17-HPREG, DHA, P4, 17-HP, A4 and, most importantly, S were higher in NCAH patients than in controls. In contrast, the levels of F at either 0 minute or 60 minutes of stimulation were similar between NCAH and control women. The proportion of NCAH patients with stimulated steroids levels of >the 95th percentile of controls were as follows: 84.21% for PREG60, 87.5% for 17-HPREG60, 95.8% for DHA60, 89.5% for P460, 100% for 17-HP60, 91.7% for A460, 29.2% for S60, and 4.1% for F60.Conclusion(s): A generalized adrenocortical hyperresponsivity to ACTH stimulation seems to be present in patients with 21-OH-deficient NCAH, with an exaggerated production of S evident in approximately 30%. The excess production of S in these NCAH patients may, in part, account for their normal F production. Copyright (C) 2000 American Society for Reproductive Medicine.
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    Author List

  • Huerta R; Dewailly D; Decanter C; Knochenhauer ES; Boots LR; Azziz R
  • Start Page

  • 329
  • End Page

  • 334
  • Volume

  • 74
  • Issue

  • 2