Müller-Gang Persistence Syndrome
Classification according to ICD-11 | |
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LD2A.Y | Other specified malformative disorders of sex development |
ICD-11 ( WHO version 2019) |
When Persistent Müllerian duct syndrome (engl. PMDS Persistent = Müllerian duct syndrome) remain in a man who is otherwise normal genitals which the Mullerian ducts are made. The reason is either a structural anomaly or a lack of anti- Müllerian hormone (AMH) or its receptor AMHRII.
Affected individuals have normally developed male sexual organs, but also have female organs and tissues that are derived from Müller's ducts. This leads to infertility in most cases.
literature
- N. Josso, C. Belville, N. di Clemente, JY Picard: AMH and AMH receptor defects in persistent Müllerian duct syndrome. In: Hum Reprod Update . 2005 Jul-Aug; 11 (4), pp. 351-356. Epub 2005 May 5th Review. PMID 15878900
Web links
- Müller-Gang Persistence Syndrome. In: Online Mendelian Inheritance in Man . (English)