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Otozomal Dominant Polikistik Böbre k Hastalığı Tedavisinde Yeni Ufuklar

New Horizons in the Treatment of Autosomal Dominant Polycystic Kidney Disease

Journal Name:

  • Türk Nefroloji, Diyaliz ve Transplantasyon Dergisi

Publication Year:

  • 2007
Author NameUniversity of AuthorFaculty of Author
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Abstract (Original Language): 
Otozomal dominant polikistik böbrek hastalığı (ODPBH) en sık görülen (prevalans 1/400-1/1000) herediter böbrek hastalığıdır (1). Yaşamın herhangi bir evresinde ortaya çıkabilir- Özellikle böbrek ve karaciğer gibi duktus içeren organlarda kist oluşumu ve gastrointestinal ve kardiyovasküler anomali-lcriyle kendini gösterir. Batı ülkelerinde renal rep-lasman tedavisi gören hastalarının %8-10'unda kronik böbrek yetmezliğinin nedeni ODPBH'dir (1). Bu oran ülkemizde %5.3 civarındadır (2). Çeşitli klinik serilerde hastaların %50'sinde son döneni böbrek yetmezliğinin (SDBY) 57-73 yaşları arasında ortaya çıktığı bildirilmiştir. PKD1 genotipi, erkek cinsiyet, 30 yaşından önce tanı konulması, ilk hematüri atağının 30 yaşından önce olması, sistemik hipertansiyonun 35 yaşından önce ortaya çıkması ve böbrek boyutların büyüklüğü SDBY'ye gidişi hızlandıran risk faktörleridir (3) Ayrıca hiperlipidemi ve düşük HDL seviyesinin de prognozu kötüleştirdiği bildirilmiştir.
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35-39
  • Turkish

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