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A case of late-onset congenital adrenal hyperplasia due to partial 3 beta-hydroxysteroid dehydrogenase deficiency.
Endocr J. 1993 Feb; 40(1):107-9.EJ

Abstract

Late-onset congenital adrenal hyperplasia due to 3 beta-hydroxysteroid dehydrogenase deficiency has been reported with increasing frequency, but only a few adult women have been found to have this disorder in Japan. We report a 26-year-old Japanese hirsute woman with partial 3 beta-hydroxysteroid dehydrogenase deficiency. The diagnosis was based on significantly increased ratios of 17-hydroxypregnenolone to 17-hydroxyprogesterone and of dehydroepiandrosterone to androstenedione after administration of ACTH. Hirsutism improved with the administration of dexamethasone (0.5 mg) every evening. Since routine assay of delta 5-steroid metabolites has become available, the incidence of this disorder will increase. Diagnostic effort should be attempted since the disorder is treatable with low-dose dexamethasone.

Authors+Show Affiliations

Department of Endocrinology, Kobe City General Hospital, Japan.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

7951484

Citation

Hattori, N, et al. "A Case of Late-onset Congenital Adrenal Hyperplasia Due to Partial 3 Beta-hydroxysteroid Dehydrogenase Deficiency." Endocrine Journal, vol. 40, no. 1, 1993, pp. 107-9.
Hattori N, Ishihara T, Moridera K, et al. A case of late-onset congenital adrenal hyperplasia due to partial 3 beta-hydroxysteroid dehydrogenase deficiency. Endocr J. 1993;40(1):107-9.
Hattori, N., Ishihara, T., Moridera, K., Hino, M., Ikekubo, K., & Kurahachi, H. (1993). A case of late-onset congenital adrenal hyperplasia due to partial 3 beta-hydroxysteroid dehydrogenase deficiency. Endocrine Journal, 40(1), 107-9.
Hattori N, et al. A Case of Late-onset Congenital Adrenal Hyperplasia Due to Partial 3 Beta-hydroxysteroid Dehydrogenase Deficiency. Endocr J. 1993;40(1):107-9. PubMed PMID: 7951484.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - A case of late-onset congenital adrenal hyperplasia due to partial 3 beta-hydroxysteroid dehydrogenase deficiency. AU - Hattori,N, AU - Ishihara,T, AU - Moridera,K, AU - Hino,M, AU - Ikekubo,K, AU - Kurahachi,H, PY - 1993/2/1/pubmed PY - 1993/2/1/medline PY - 1993/2/1/entrez SP - 107 EP - 9 JF - Endocrine journal JO - Endocr J VL - 40 IS - 1 N2 - Late-onset congenital adrenal hyperplasia due to 3 beta-hydroxysteroid dehydrogenase deficiency has been reported with increasing frequency, but only a few adult women have been found to have this disorder in Japan. We report a 26-year-old Japanese hirsute woman with partial 3 beta-hydroxysteroid dehydrogenase deficiency. The diagnosis was based on significantly increased ratios of 17-hydroxypregnenolone to 17-hydroxyprogesterone and of dehydroepiandrosterone to androstenedione after administration of ACTH. Hirsutism improved with the administration of dexamethasone (0.5 mg) every evening. Since routine assay of delta 5-steroid metabolites has become available, the incidence of this disorder will increase. Diagnostic effort should be attempted since the disorder is treatable with low-dose dexamethasone. SN - 0918-8959 UR - https://www.unboundmedicine.com/medline/citation/7951484/A_case_of_late_onset_congenital_adrenal_hyperplasia_due_to_partial_3_beta_hydroxysteroid_dehydrogenase_deficiency_ L2 - https://joi.jlc.jst.go.jp/JST.Journalarchive/endocrj1993/40.107?from=PubMed DB - PRIME DP - Unbound Medicine ER -