
Persistent Mullerian Duct Syndrome: A Rare Case of an Adult Infertile Male with Bilateral Cryptorchidism
Author(s) -
Prakash Sankapal,
Venkat Arjunrao Gite,
Mayank Agrawal,
Mahesh S. Sane,
Atul Singal
Publication year - 2021
Publication title -
journal of reproduction and infertility
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.495
H-Index - 22
eISSN - 2251-676X
pISSN - 2228-5482
DOI - 10.18502/jri.v22i3.6722
Subject(s) - male pseudohermaphroditism , medicine , gynecology , anti müllerian hormone , infertility , exploratory laparotomy , hysterectomy , mullerian ducts , uterus , hormone , surgery , pregnancy , biology , genetics
Persistent mullerian duct syndrome (PMDS) is a very rare form of internal male pseudohermaphroditism in individuals who are phenotypically males with 46 XY karyotypes harboring internal female reproductive organs which are Mullerian derivatives. It occurs as a defect in the genes coding for the Mullerian inhibiting substance (MIS) or the anti Mullerian hormone (AMH) receptor, ultimately leading to failure of regression of Mullerian ducts.