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

Raquel Huerta, Didier Dewailly, Christine Decanter, Eric S. Knochenhauer, Larry R. Boots, Ricardo Azziz

Research output: Contribution to journalArticle

16 Citations (Scopus)

Abstract

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.

Original languageEnglish (US)
Pages (from-to)329-334
Number of pages6
JournalFertility and sterility
Volume74
Issue number2
DOIs
StatePublished - Aug 1 2000

Fingerprint

Steroid 21-Hydroxylase
Adrenocorticotropic Hormone
Hyperplasia
Hydrocortisone
17-alpha-Hydroxyprogesterone
17-alpha-Hydroxypregnenolone
Pregnenolone
Dehydroepiandrosterone
Cortodoxone
Cosyntropin
Androstenedione
Serum
Progesterone
Steroids
Outcome Assessment (Health Care)
Prospective Studies

Keywords

  • 21-hydroxylase
  • Adrenal
  • Adrenal hyperplasia
  • Cortisol
  • Excess androgens
  • Hirsutism

ASJC Scopus subject areas

  • Reproductive Medicine
  • Obstetrics and Gynecology

Cite this

Adrenocortical hyperresponsivity to adrenocorticotropic hormone : A mechanism favoring the normal production of cortisol in 21-hydroxylase-deficient nonclassic adrenal hyperplasia. / Huerta, Raquel; Dewailly, Didier; Decanter, Christine; Knochenhauer, Eric S.; Boots, Larry R.; Azziz, Ricardo.

In: Fertility and sterility, Vol. 74, No. 2, 01.08.2000, p. 329-334.

Research output: Contribution to journalArticle

Huerta, Raquel ; Dewailly, Didier ; Decanter, Christine ; Knochenhauer, Eric S. ; Boots, Larry R. ; Azziz, Ricardo. / Adrenocortical hyperresponsivity to adrenocorticotropic hormone : A mechanism favoring the normal production of cortisol in 21-hydroxylase-deficient nonclassic adrenal hyperplasia. In: Fertility and sterility. 2000 ; Vol. 74, No. 2. pp. 329-334.
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title = "Adrenocortical hyperresponsivity to adrenocorticotropic hormone: A mechanism favoring the normal production of cortisol in 21-hydroxylase-deficient nonclassic adrenal hyperplasia",
abstract = "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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T2 - A mechanism favoring the normal production of cortisol in 21-hydroxylase-deficient nonclassic adrenal hyperplasia

AU - Huerta, Raquel

AU - Dewailly, Didier

AU - Decanter, Christine

AU - Knochenhauer, Eric S.

AU - Boots, Larry R.

AU - Azziz, Ricardo

PY - 2000/8/1

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N2 - 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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KW - 21-hydroxylase

KW - Adrenal

KW - Adrenal hyperplasia

KW - Cortisol

KW - Excess androgens

KW - Hirsutism

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