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Late onset adrenal hyperplasia due to 3 beta-hydroxy-delta 5-steroid dehydrogenase deficiency in north Indian hirsute women.
Indian J Med Res. 1997 Jan; 105:27-31.IJ

Abstract

The presence of late onset 3 beta-hydroxy steroid dehydrogenase (3 beta-HSD) type of congenital adrenal hyperplasia was studied in 58 north Indian hirsute women. The age range of these patients was 15 to 42 yr. Fifty two per cent of these patients had body mass index > 25. Basal serum testosterone, luteinizing hormone, follicle stimulating hormone, dehydroepiandrosterone sulphate (DHEAS), and 17 hydroxy progesterone (17 OHP) were estimated. All the patients underwent adrenocorticotropin (ACTH) stimulation test after an overnight dexamethasone suppression for the estimation of DHEAS, 17 OHP, and 17 hydroxy pregnenolone (delta 5-17p). Five (8.6%) hirsute women showed an exaggerated 17 OHP response to ACTH indicating 21-hydroxylase deficiency. Eight (13.8%) hirsute women had elevated basal DHEAS and ACTH-stimulated DHEAS as well as delta 5-17P responses indicative of 3 beta-HSD deficiency. In one patient hirsutism was the presenting manifestation of tumoural hyperandrogenism. Our findings indicate the presence of both 21-hydroxylase and 3 beta-HSD deficiency in north Indian hirsute women, with, 3 beta-HSD deficiency being the major cause of hirsutism in this population.

Authors+Show Affiliations

Department of Endocrinology, All India Institute of Medical Sciences, New Delhi.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

9029832

Citation

Sundararaman, P G., et al. "Late Onset Adrenal Hyperplasia Due to 3 Beta-hydroxy-delta 5-steroid Dehydrogenase Deficiency in North Indian Hirsute Women." The Indian Journal of Medical Research, vol. 105, 1997, pp. 27-31.
Sundararaman PG, Ammini AC, Khurana ML, et al. Late onset adrenal hyperplasia due to 3 beta-hydroxy-delta 5-steroid dehydrogenase deficiency in north Indian hirsute women. Indian J Med Res. 1997;105:27-31.
Sundararaman, P. G., Ammini, A. C., Khurana, M. L., Karmarkar, M. G., & Ahuja, M. M. (1997). Late onset adrenal hyperplasia due to 3 beta-hydroxy-delta 5-steroid dehydrogenase deficiency in north Indian hirsute women. The Indian Journal of Medical Research, 105, 27-31.
Sundararaman PG, et al. Late Onset Adrenal Hyperplasia Due to 3 Beta-hydroxy-delta 5-steroid Dehydrogenase Deficiency in North Indian Hirsute Women. Indian J Med Res. 1997;105:27-31. PubMed PMID: 9029832.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Late onset adrenal hyperplasia due to 3 beta-hydroxy-delta 5-steroid dehydrogenase deficiency in north Indian hirsute women. AU - Sundararaman,P G, AU - Ammini,A C, AU - Khurana,M L, AU - Karmarkar,M G, AU - Ahuja,M M, PY - 1997/1/1/pubmed PY - 1997/1/1/medline PY - 1997/1/1/entrez SP - 27 EP - 31 JF - The Indian journal of medical research JO - Indian J Med Res VL - 105 N2 - The presence of late onset 3 beta-hydroxy steroid dehydrogenase (3 beta-HSD) type of congenital adrenal hyperplasia was studied in 58 north Indian hirsute women. The age range of these patients was 15 to 42 yr. Fifty two per cent of these patients had body mass index > 25. Basal serum testosterone, luteinizing hormone, follicle stimulating hormone, dehydroepiandrosterone sulphate (DHEAS), and 17 hydroxy progesterone (17 OHP) were estimated. All the patients underwent adrenocorticotropin (ACTH) stimulation test after an overnight dexamethasone suppression for the estimation of DHEAS, 17 OHP, and 17 hydroxy pregnenolone (delta 5-17p). Five (8.6%) hirsute women showed an exaggerated 17 OHP response to ACTH indicating 21-hydroxylase deficiency. Eight (13.8%) hirsute women had elevated basal DHEAS and ACTH-stimulated DHEAS as well as delta 5-17P responses indicative of 3 beta-HSD deficiency. In one patient hirsutism was the presenting manifestation of tumoural hyperandrogenism. Our findings indicate the presence of both 21-hydroxylase and 3 beta-HSD deficiency in north Indian hirsute women, with, 3 beta-HSD deficiency being the major cause of hirsutism in this population. SN - 0971-5916 UR - https://www.unboundmedicine.com/medline/citation/9029832/Late_onset_adrenal_hyperplasia_due_to_3_beta_hydroxy_delta_5_steroid_dehydrogenase_deficiency_in_north_Indian_hirsute_women_ DB - PRIME DP - Unbound Medicine ER -