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Polikistik Böbrek Hastalığında Kistogenezde Yenilikler

Novel Approaches for the Cystogenesis in Polycystic Kidney Disease

Journal Name:

  • Türk Nefroloji, Diyaliz ve Transplantasyon Dergisi

Publication Year:

  • 2007
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Abstract (Original Language): 
Otozomal dominant polikistik böbrek hastalığı en .sık karşılaşılan herediter renal kistik hastalıktır ve 400 ile 1000 canlı doğumdan birinde rastlanılır (1,2). Orak hücreli anemiden 10 kat, kistik fibrozdan 15 kat daha sıktır (1-4). Sistemik bir hastalıktır ve son dönem böbrek yetersizliğinin %5-10'undan sorumludur (1-4). Polikistik böbrek hastalığından sorumlu genler tanımlanmıştır. Olguların %85-90'ından sorumlu gen 16. kromozomun kısa kolunda yerleşmiştir. PKD1 geni olarak adlandırılan bu gen polikistin 1 proteinini kodlar. Dördüncü kromozomun uzun kolunda yer alan PKD2 geni ise olguların %10-15'inden sorumludur ve polikislin 2 adlı proteinin yapımını kontrol eder (5). Bazı araştırıcılar tarafından varlığı kabul edilen PKD3 geni ise daha İzole edilememiştir. Her bir genin hastalığında klinik tabloda farklılık dikkati çeker. PKD2 geni hastalığında kistler daha ileri yaşta ortaya çıkar ve böbrek yetersizliği ileri yaşta problem olur. Yapılan bir çalışmada, son dönem böbrek yetersizliği gelişme yaşı PKD2 geni olanlarda 69, PKD1 geni olanlarda İse 57 olarak bulunmuştur (6).
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REFERENCES

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10
Türk
Nefroloj
i Diyaliz ve Transplantasyon Dergisi /Official Journal of the Turkish Society of Nephrology
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