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Persistent Müllerian duct syndrome (PMDS) is the presence of Müllerian duct derivatives (fallopian tubes, uterus, and/or the upper part of the vagina) in what would be considered a genetically and otherwise physically normal male animal by typical human based standards. In humans, PMDS typically is due to an autosomal recessive congenital disorder and is considered by some to be a form of pseudohermaphroditism due to the presence of Müllerian derivatives. PMDS can also present in non-human animals.

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  • Müller-Gang-Persistenzsyndrom (de)
  • Síndrome del conducto mülleriano persistente (es)
  • Syndrome des canaux de Müller persistants (fr)
  • 지속성 뮐러관 증후군 (ko)
  • Persistent Müllerian duct syndrome (en)
  • Синдром персистирующих мюллеровых протоков (ru)
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  • Beim Müller-Gang-Persistenzsyndrom (engl. PMDS = Persistent müllerian duct syndrome) bleiben bei einem Mann, der ansonsten normale Genitalien aufweist, die Müller'schen Gänge bestehen. Der Grund ist entweder eine Strukturanomalie oder ein Mangel an Anti-Müller-Hormon (AMH) bzw. seines Rezeptors AMHRII. Betroffene Individuen haben normal entwickelte männliche Geschlechtsorgane, besitzen aber außerdem weibliche Organe und Gewebe, die sich von den Müllerschen Gängen ableiten. Dies führt in den meisten Fällen zu Unfruchtbarkeit. (de)
  • Le syndrome des canaux de Müller persistants, ou syndrome de persistance des canaux de Müller est une malformation congénitale rare atteignant les organes génitaux masculins. La persistance des canaux de Müller au cours du développement embryonnaire entraîne la formation d'un utérus et de trompes de Fallope conjointement à des organes génitaux externes masculins (pénis et scrotum) normaux. Il existe également une cryptorchidie (testicules en position intra-abdominale), et parfois une hernie inguinale ; l'infertilité est fréquente. Dans 45 % des cas, une mutation du gène de l'hormone anti-müllérienne est retrouvée. La transmission se fait sur un mode autosomique récessif. (fr)
  • 지속성 뮐러관 증후군은 남성에게 이 사라지지 않아 자궁 등을 가진 증상이다. 유전자 이상으로 인해 항뮐러관 호르몬의 합성이나 작용에 문제가 생겨서 생긴다. (ko)
  • El síndrome del conducto de Müller persistente es una de las anomalías que pueden dar durante el desarrollo de los embriones tanto humanos como de otros mamíferos. El sistema de Müller es el precursor de los órganos femeninos y el sistema de Wolff el de los órganos masculinos. Para que un embrión se desarrolle como macho necesita que dos hormonas (testosterona y dihodrosterona) actúen sobre células "diana" del aparato de Wolff, propiciando el desarrollo de los órganos sexuales internos del macho, mientras que otra hormona ( la inhibidora del sistema de Müller) inhibe el desarrollo de los órganos sexuales femeninos. Como su propio nombre indica, este síndrome provoca que se desarrollen los órganos masculinos, pero no inhibe el desarrollo de los femeninos, lo que dará lugar a individuos con (es)
  • Persistent Müllerian duct syndrome (PMDS) is the presence of Müllerian duct derivatives (fallopian tubes, uterus, and/or the upper part of the vagina) in what would be considered a genetically and otherwise physically normal male animal by typical human based standards. In humans, PMDS typically is due to an autosomal recessive congenital disorder and is considered by some to be a form of pseudohermaphroditism due to the presence of Müllerian derivatives. PMDS can also present in non-human animals. (en)
  • Синдром персистирующих мюллеровых протоков (СПМП) — состояние, при котором в мужском (генетически и физиологически) организме присутствуют производные мюллеровых протоков (фаллопиевые трубы, матка и/или верхней части влагалища). У людей СПМП обычно возникает из-за аутосомно-рецессивной врождённой аномалии и считается интерсекс-вариацией из-за присутствия мюллеровых производных. (ru)
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