Tags

Type your tag names separated by a space and hit enter

The incidence of late-onset congenital adrenal hyperplasia due to 3 beta-hydroxysteroid dehydrogenase deficiency among hirsute women.
Clin Endocrinol (Oxf). 1992 Apr; 36(4):383-8.CE

Abstract

OBJECTIVE

The present study was designed to determine the incidence of 3 beta-hydroxysteroid dehydrogenase deficiency (3 beta-HSD) in adult women with hyperandrogenism.

DESIGN AND PATIENTS

In 78 hirsute patients and 30 normal women in the same age range, an ACTH stimulation test was performed on day 5 of the cycle by administration of a single bolus of 0.25 mg ACTH-(1-24) at 0800 h.

MEASUREMENTS

The following steroids were measured before, 30 and 60 minutes after ACTH injection: delta 5-pregnenolone (delta 5-P), 17-hydroxy-delta 5-pregnenolone (17-OH delta 5-P), dehydroepiandrosterone (DHEA), delta 5-androstenediol, progesterone (P), 17-hydroxyprogesterone (17-OHP), androstenedione (A), testosterone (T) and cortisol.

RESULTS

Maximum ACTH-stimulated values of delta 5-steroids were in excess of the 90% confidence limits of the control group in 19 hirsute women. Ten patients had an isolated increase in delta 5-P, 17-OH delta 5-P, DHEA or delta 5-androstenediol. Nine patients had an increase in two delta 5 steroids and none had increased values of three or four delta 5 steroids. The ratios of 17-OH delta 5-P to 17-OHP, DHEA to A, delta 5-P to P and delta 5-androstenediol to T were increased in 5, 1, 1 and 1 patients respectively. No patient had elevated values of more than one ratio.

CONCLUSIONS

Using stringent diagnostic criteria, partial 3 beta-HSD deficiency was excluded in all 78 patients and therefore appears to be a rare disorder.

Authors+Show Affiliations

Mathieson J
Service d'Endocrinologie et des Maladies de la Reproduction, Hôpital Bicêtre, France.
Couzinet B
No affiliation info available
Wekstein-Noel S
No affiliation info available
Nahoul K
No affiliation info available
Turpin G
No affiliation info available
Schaison G
No affiliation info available

MeSH

3-Hydroxysteroid DehydrogenasesAdrenal Cortex HormonesAdrenal Hyperplasia, CongenitalAdrenocorticotropic HormoneAdultFemaleHirsutismHumansIncidenceStimulation, Chemical

Pub Type(s)

Comparative Study
Journal Article

Language

eng

PubMed ID

1330378

Citation

Mathieson, J, et al. "The Incidence of Late-onset Congenital Adrenal Hyperplasia Due to 3 Beta-hydroxysteroid Dehydrogenase Deficiency Among Hirsute Women." Clinical Endocrinology, vol. 36, no. 4, 1992, pp. 383-8.
Mathieson J, Couzinet B, Wekstein-Noel S, et al. The incidence of late-onset congenital adrenal hyperplasia due to 3 beta-hydroxysteroid dehydrogenase deficiency among hirsute women. Clin Endocrinol (Oxf). 1992;36(4):383-8.
Mathieson, J., Couzinet, B., Wekstein-Noel, S., Nahoul, K., Turpin, G., & Schaison, G. (1992). The incidence of late-onset congenital adrenal hyperplasia due to 3 beta-hydroxysteroid dehydrogenase deficiency among hirsute women. Clinical Endocrinology, 36(4), 383-8.
Mathieson J, et al. The Incidence of Late-onset Congenital Adrenal Hyperplasia Due to 3 Beta-hydroxysteroid Dehydrogenase Deficiency Among Hirsute Women. Clin Endocrinol (Oxf). 1992;36(4):383-8. PubMed PMID: 1330378.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - The incidence of late-onset congenital adrenal hyperplasia due to 3 beta-hydroxysteroid dehydrogenase deficiency among hirsute women. AU - Mathieson,J, AU - Couzinet,B, AU - Wekstein-Noel,S, AU - Nahoul,K, AU - Turpin,G, AU - Schaison,G, PY - 1992/4/1/pubmed PY - 1992/4/1/medline PY - 1992/4/1/entrez SP - 383 EP - 8 JF - Clinical endocrinology JO - Clin Endocrinol (Oxf) VL - 36 IS - 4 N2 - OBJECTIVE: The present study was designed to determine the incidence of 3 beta-hydroxysteroid dehydrogenase deficiency (3 beta-HSD) in adult women with hyperandrogenism. DESIGN AND PATIENTS: In 78 hirsute patients and 30 normal women in the same age range, an ACTH stimulation test was performed on day 5 of the cycle by administration of a single bolus of 0.25 mg ACTH-(1-24) at 0800 h. MEASUREMENTS: The following steroids were measured before, 30 and 60 minutes after ACTH injection: delta 5-pregnenolone (delta 5-P), 17-hydroxy-delta 5-pregnenolone (17-OH delta 5-P), dehydroepiandrosterone (DHEA), delta 5-androstenediol, progesterone (P), 17-hydroxyprogesterone (17-OHP), androstenedione (A), testosterone (T) and cortisol. RESULTS: Maximum ACTH-stimulated values of delta 5-steroids were in excess of the 90% confidence limits of the control group in 19 hirsute women. Ten patients had an isolated increase in delta 5-P, 17-OH delta 5-P, DHEA or delta 5-androstenediol. Nine patients had an increase in two delta 5 steroids and none had increased values of three or four delta 5 steroids. The ratios of 17-OH delta 5-P to 17-OHP, DHEA to A, delta 5-P to P and delta 5-androstenediol to T were increased in 5, 1, 1 and 1 patients respectively. No patient had elevated values of more than one ratio. CONCLUSIONS: Using stringent diagnostic criteria, partial 3 beta-HSD deficiency was excluded in all 78 patients and therefore appears to be a rare disorder. SN - 0300-0664 UR - https://www.unboundmedicine.com/medline/citation/1330378/The_incidence_of_late_onset_congenital_adrenal_hyperplasia_due_to_3_beta_hydroxysteroid_dehydrogenase_deficiency_among_hirsute_women_ DB - PRIME DP - Unbound Medicine ER -