Aydın ERDEMİR, Sefa KELEKÇİ, Ebru TÜRKOĞLU, Hese COŞAR, Zelal KAHRAMANER, Sümer SÜTÇÜOĞLU, Çağdaş ÇÖLLÜOĞLU, SEFA KURT, Esra ARUN ÖZER

Prune-Belly Sendromlu Bir Olguda Erken Prenatal Girişim ile Başarılı Tedavi

Prune-Belly Sendromu (PBS) abdominal kasların hipoplazisi, üriner system anomalileri ve bilateral inmemiş testis ile karakterize konjenital bir hastalıktır. Üriner sistem tutulumu %60 mortaliteye sahip olan son dönem böbrek hastalığı ve pulmoner hipoplazi komplikasyonları ile en önemli prognostik faktördür. PBS’nin kalıtım şekli bilinmemesine rağmen, hastalığın bir genetik temeli olduğu düşünülmektedir. Antenatal dönemde üriner sistemdeki obstrüksiyonun giderilmesi hastaların yaşam şansını arttırmaktadır. Biz prenatal dönemde vezikoamniyotik şant uygulanan ve böylece az etkilenen ailesel bir PBS olgusu sunduk

Anahtar Kelimeler:

Abdominal kas hipoplazisi, Eagle Barrett Sendromu, Prune-Belly Sendromu, üriner sistem obstrüksiyonu, vezikoamniyotik şant

Prune-Belly Sendromlu Bir Olguda Erken Prenatal Girişim ile Başarılı Tedavi,

Prune-Belly Syndrome (PBS) is a congenital disorder characterized by abdominal wall musculature deficiency, urinary tract anomalies and bilateral cryptorchidism. Urinary tract disease is the most important prognostic factor with the complications of pulmonary hypoplasia and end stage renal disease resulting in a mortality rate of 60%. Although the mode of inheritance of PBS is unclear, it has been hypothesized that PBS has a genetic basis. In the antenatal period relieving urinary tract obstruction can improve the survival rate of patients with PBS. We report a neonate with familial prune-belly syndrome who was applied vesico-amniotic shunt and so was affected slightly

Keywords:

Deficiency of abdominal musculature, Eagle Barrett syndrome, Prune-Belly syndrome, urinary tract obstruction, vesico-amniotic shunt,

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