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

Service d'Endocrinologie et des Maladies de la Reproduction, Hôpital Bicêtre, France.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

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_ L2 - https://onlinelibrary.wiley.com/resolve/openurl?genre=article&sid=nlm:pubmed&issn=0300-0664&date=1992&volume=36&issue=4&spage=383 DB - PRIME DP - Unbound Medicine ER -