Combined persistent Mullerian duct syndrome, transverse testicular ectopia and mosaic Klinefelter's syndrome

被引：0

作者：

Rehman, Abdul ^{[1
]}

Hasan, Zubair ^{[2
]}

Amanat, Samina ^{[3
]}

Shaukat, Tauseef ^{[1
]}

Saeed, Aisha ^{[1
]}

Jamil, Kashif ^{[1
]}

Zaidi, Amjad ^{[1
]}

Akram, M. ^{[1
]}

机构：

[1] Pakistan Atom Energy Commiss Hosp, Dept Gen Surg, Islamabad, Pakistan

[2] Pakistan Atom Energy Commiss Hosp, Dept Adm, Islamabad, Pakistan

[3] Pakistan Atom Energy Commiss Hosp, Dept Pathol, Islamabad, Pakistan

来源：

JCPSP-JOURNAL OF THE COLLEGE OF PHYSICIANS AND SURGEONS PAKISTAN | 2008年 / 18卷 / 06期

关键词：

persistent Mullerian duct syndrome; male pseudohermaphroditism; transverse testicular ectopia; mosaicism; Klinefelter's syndrome;

D O I：

暂无

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Persistent Mullerian Duct Syndrome (PMDS), a rare form of male pseudohermaphroditism, is characterized by the persistence of Mullerian duct structures (uterus, fallopian tubes and upper two-thirds of vagina) in otherwise normally-virilized males (Karyotype 46XY). Patients suffering from PMDS present with cryptorchidism, inguinal hernia and infertility. Diagnosis is established when Mullerian duct structures are discovered either during ultrasonography for localization of undescended testis(s), during surgical exploration for cryptorchidism or herniorrhaphy (hernii uteri inguinalis). Presence of both testes on one side of the scrotum is known as Transverse Testicular Ectopia (TTE). Co-existence of PMDS and transverse testicular ectopia in a patient of mosaic Klinefelter's syndrome (Karyotype 46XY/47XXY) is a unique genetic association.

引用

页码：375 / 377

页数：3

共 10 条

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