Persistent Müllerian duct syndrome: an update
نویسندگان
چکیده
منابع مشابه
Persistent müllerian duct syndrome--a case report.
An eighteen month old phenotypically and genotypically normal male child was admitted with a left inguinal hernia and a right undescended testis. At operation, he was found to have a uterus, bilateral fallopian tubes, and a vagina in the left hernial sac. Bilateral orchidopexies and excision of the persistent Mullerian duct structures were carried out. This rare case of persistent Mullerian duc...
متن کاملThe Persistent Müllerian Duct Syndrome: An Update Based Upon a Personal Experience of 157 Cases.
Male sex differentiation is driven by 2 hormones, testosterone and anti-müllerian hormone (AMH), responsible for the regression of müllerian ducts in male fetuses. Mutations inactivating AMH or its receptor AMHRII lead to the persistent müllerian duct syndrome (PMDS) in otherwise normally virilized 46,XY males. Our objective was to review the clinical, anatomical, and molecular features of PMDS...
متن کاملBilateral Cryptorchidism, a rare presentation for persistent Müllerian duct syndrome
Persistent Müllerian duct syndrome (PMDS) is a rare, sex-limited, autosomal recessive disorder representing male pseudo-hermaphroditism. It is observed in males with the presence of female reproductive organs such as the uterus, cervix, and bilateral fallopian tubes along with normally developed male reproductive organs. It generally occurs during embryogenesis due to mutation in anti-Müllerian...
متن کاملPersistent Müllerian duct syndrome: A case report and review
Persistent Mullerian duct syndrome is a rare form of internal male pseudohermaphroditism, in which Mullerian duct derivatives (uterus and fallopian tubes) are present in a genotypic (46XY) and phenotypic male. Over 150 cases have been reported, mainly from outside the African setting. This article presents an unexpected case encountered in an African setting. Handicaps in the management were un...
متن کاملThe management of the persistent Müllerian duct syndrome
OBJECTIVES To report the findings and management of patients with persistent Müllerian duct syndrome (PMDS). PATIENTS AND METHODS Nineteen phenotypically male patients (aged 8 months to 27 years) presented with testicular maldescent. All of them had normal male external genitalia. Two of them had had a previous diagnosis of persistent Müllerian structures. All patients were karyotyped, and ha...
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ژورنال
عنوان ژورنال: Reproduction, Fertility and Development
سال: 2019
ISSN: 1031-3613
DOI: 10.1071/rd17501