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11 beta-hydroxylase deficiency in hyperandrogenism.
Fertil Steril. 1991 Apr; 55(4):733-41.FS

Abstract

OBJECTIVE

to determine the 11-deoxycortisol (S) response and incidence of 11 beta-hydroxylase deficiency in hyperandrogenism.

DESIGN

Hyperandrogenic women prospectively and consecutively underwent acute adrenal stimulation studies.

SETTING

Tertiary institution.

PATIENTS

Two hundred sixty women complaining of hirsutism and/or hyperandrogenic oligomenorrhea were studied, excluding five unrelated families (1.9% of total) suffering from 21-hydroxylase deficient late-onset adrenal hyperplasia. Forty-one healthy premenopausal eumenorrheic women served as controls.

MAIN OUTCOME MEASURES

Only two unrelated women (0.8%) had a poststimulation or net increment S level value greater than or equal to threefold the upper 95th percentile of controls and were presumed to suffer from 11 beta-hydroxylase deficient late-onset adrenal hyperplasia. One hundred nine (42%) of hyperandrogenic women had at least one S value above the 95th percentile of controls. These women also demonstrated higher basal (F0) and stimulated cortisol levels, but a similar increment compared with controls.

RESULTS

Patients with high S measures had higher testosterone, dehydroepiandrosterone sulfate, and androstenedione levels, but similar luteinizing hormone/follicle-stimulating hormone ratios, than hyperandrogenic cohorts with no abnormal S measures. Basal values of S (S0), F0, or S0/F0 were not useful to predict an abnormal S response to stimulation.

CONCLUSIONS

Although adrenocortical hyperactivity was present in 42% of our hyperandrogenic patients, only 0.8% were presumed to suffer from 11 beta-hydroxylase deficient late-onset adrenal hyperplasia. A systemic search for this deficiency in hyperandrogenism is probably unwarranted.

Authors+Show Affiliations

Azziz R
Department of Obstetrics and Gynecology, University of Alabama, Birmingham 35294.
Boots LR
No affiliation info available
Parker CR
No affiliation info available
Bradley E
No affiliation info available
Zacur HA
No affiliation info available

MeSH

Adrenal GlandsAdrenal Hyperplasia, CongenitalAdultAndrogensCortodoxoneFemaleHirsutismHormonesHumansHyperplasiaMenstruation DisturbancesPredictive Value of TestsProspective StudiesReference Values

Pub Type(s)

Comparative Study
Journal Article
Research Support, U.S. Gov't, P.H.S.

Language

eng

PubMed ID

2009997

Citation

Azziz, R, et al. "11 Beta-hydroxylase Deficiency in Hyperandrogenism." Fertility and Sterility, vol. 55, no. 4, 1991, pp. 733-41.
Azziz R, Boots LR, Parker CR, et al. 11 beta-hydroxylase deficiency in hyperandrogenism. Fertil Steril. 1991;55(4):733-41.
Azziz, R., Boots, L. R., Parker, C. R., Bradley, E., & Zacur, H. A. (1991). 11 beta-hydroxylase deficiency in hyperandrogenism. Fertility and Sterility, 55(4), 733-41.
Azziz R, et al. 11 Beta-hydroxylase Deficiency in Hyperandrogenism. Fertil Steril. 1991;55(4):733-41. PubMed PMID: 2009997.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - 11 beta-hydroxylase deficiency in hyperandrogenism. AU - Azziz,R, AU - Boots,L R, AU - Parker,C R,Jr AU - Bradley,E,Jr AU - Zacur,H A, PY - 1991/4/1/pubmed PY - 2001/3/28/medline PY - 1991/4/1/entrez SP - 733 EP - 41 JF - Fertility and sterility JO - Fertil Steril VL - 55 IS - 4 N2 - OBJECTIVE: to determine the 11-deoxycortisol (S) response and incidence of 11 beta-hydroxylase deficiency in hyperandrogenism. DESIGN: Hyperandrogenic women prospectively and consecutively underwent acute adrenal stimulation studies. SETTING: Tertiary institution. PATIENTS: Two hundred sixty women complaining of hirsutism and/or hyperandrogenic oligomenorrhea were studied, excluding five unrelated families (1.9% of total) suffering from 21-hydroxylase deficient late-onset adrenal hyperplasia. Forty-one healthy premenopausal eumenorrheic women served as controls. MAIN OUTCOME MEASURES: Only two unrelated women (0.8%) had a poststimulation or net increment S level value greater than or equal to threefold the upper 95th percentile of controls and were presumed to suffer from 11 beta-hydroxylase deficient late-onset adrenal hyperplasia. One hundred nine (42%) of hyperandrogenic women had at least one S value above the 95th percentile of controls. These women also demonstrated higher basal (F0) and stimulated cortisol levels, but a similar increment compared with controls. RESULTS: Patients with high S measures had higher testosterone, dehydroepiandrosterone sulfate, and androstenedione levels, but similar luteinizing hormone/follicle-stimulating hormone ratios, than hyperandrogenic cohorts with no abnormal S measures. Basal values of S (S0), F0, or S0/F0 were not useful to predict an abnormal S response to stimulation. CONCLUSIONS: Although adrenocortical hyperactivity was present in 42% of our hyperandrogenic patients, only 0.8% were presumed to suffer from 11 beta-hydroxylase deficient late-onset adrenal hyperplasia. A systemic search for this deficiency in hyperandrogenism is probably unwarranted. SN - 0015-0282 UR - https://www.unboundmedicine.com/medline/citation/2009997/11_beta_hydroxylase_deficiency_in_hyperandrogenism_ DB - PRIME DP - Unbound Medicine ER -