17-ALPHA-HYDROXYLASE DEFICIENCY WITH PERSISTENCE OF MULLERIAN DUCTS IN A GENOTYPIC MALE AND PARADOXICAL ALDOSTERONE SECRETION
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AbstractWe report a case of congenital adrenal hyperplasia due to 17alpha-hydroxylase deficiency in a Chinese genotypic male patient. Despite the male genotype, normal female external genitalia were present and with the introduction of cyclical oestrogen therapy withdrawal bleeding occurred, confirming the presence of functional endometrial tissue. We believe this to be the first report of persistent Mullerian duct structures in a genotypic male with 17alpha-hydroxylase deficiency. It could be explained by either impaired secretion or impaired action of anti-Mullerian hormone. Further, contrary to the usual finding of suppressed aldosterone secretion, this patient had measurable levels of plasma aldosterone.
All Author(s) ListPANESAR NS, YEUNG VTF, CHAN JCN, SHEK CC, NICHOLLS MG, COCKRAM CS
Journal namePOSTGRADUATE MEDICAL JOURNAL
Year1993
Month2
Day1
Volume Number69
Issue Number808
PublisherBRITISH MED JOURNAL PUBL GROUP
Pages159 - 162
ISSN0032-5473
eISSN1469-0756
LanguagesEnglish-United Kingdom
Web of Science Subject CategoriesGeneral & Internal Medicine; Medicine, General & Internal; MEDICINE, GENERAL & INTERNAL

Last updated on 2020-18-01 at 02:12