Journals / Şişli Etfal Hastanesi Tıp Bülteni / 2004 / Cilt: 38 - Sayı: 1

Case report: Persistent mullerian duct syndrome

Olgu sunumu: Persistent müller kanalı sendromu (PMKS)

Pages
63–65
DOI
—
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AI Summary & Key Takeaways

AI Insight
⚡ TL;DR(Too Long; Didn't Read)

This is a comprehensive review of the literature with evaluation of the clinical features

📌 3 Key Takeaways

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Objective & Hypothesis:Persistent mullerian duct syndrome also named as hernia uteri inguinale is a form of internal male pseudohermafroditsm and may result from the failure of synthesis or release of AMH, defect of AMH receptors or the defect in the timing of the release of AMH.
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Key Findings & Data:Three month old male child operated for cryptorchidism and found out to be PMDS after the examination of the operation material is reported.
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Conclusion & Impact:This is a comprehensive review of the literature with evaluation of the clinical features
🔬Design:Review & Systematic Survey
📍Scope:Comprehensive Sample
⚙️Variables:Kriptorşidizm · Fıtık · kasık

Abstract

Persistent mullerian duct syndrome also named as hernia uteri inguinale is a form of internal male pseudohermafroditsm and may result from the failure of synthesis or release of AMH, defect of AMH receptors or the defect in the timing of the release of AMH. This syndrome is an. inherited disorder, it is usually transmitted as a recessive autosomal trait or X linked trait. Patients affected with PMDS are phcnotypic males with either bilateral cryptorchidism or unilateral testicular ectopia associated with inguinal hernia on the contralateral side. The presence of uterus and tubes is usually discovered at surgery. Three month old male child operated for cryptorchidism and found out to be PMDS after the examination of the operation material is reported. This is a comprehensive review of the literature with evaluation of the clinical features

Özet

Herni uteri inguinale olarak da bilmen Persistan Müller Kanal Sendromu (PMKS) internal erkek pseudohermafroditizmi-nin bir formudur ve AMH (Antimüllerian hormon) yetmezliği, AMH reseptör defekti veya sekresyonunda zamanlama def ekline bağlı olarak gelişir. Bu sendrom familyal olup muhtemelen otozomal resessif veya X' e bağlı resesif geçişlidir PMKS'I i hastalar fenotipik erkeklerdir. Bilateral kriptoorşitizm veya unilateral testiküler ektopi ile ilişkili kontrlateral inguinal herni mevcuttur. Uterus ve tubaların varlığı genelde cerrahi sırasında farkedilir. Kriptoorşitizm ön tanısıyla opere edilen üç aylık erkek çocukta operasyon materyalinin, incelenmesi sonucu PMKS tesbit edildi. Literatür bilgileri eşliğinde olguyu araştırmak ve birlikte tartışmak amacı ile sunuldu.

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Cite This Article

TANIK, C., TAŞKIN, T., YALÇIN, M. (2004). Case report: Persistent mullerian duct syndrome. Şişli Etfal Hastanesi Tıp Bülteni, Cilt: 38 - Sayı: 1, 63–65. https://ajindex.com/articles/363916

References (9)

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