Karaciğer ve safra yollarının kistik hastalıkları

Karaciğerin kistik hastalıkları süt çocukluğu ve çocukluk çağında tanı alırlar. Karaciğer ve safra yollarının kistik hastalıklarını koledok kistleri, otozomal çekinik (resesif) ve otozomal baskın (dominant) polikistik böbrek hastalığı, doğumsal hepatik fibroz ve Caroli hastalığı (karaciğer içi safra yollarının kistik genişlemesi) oluşturmaktadır. Koledok kisti ve Caroli hastalığı safra akımını engeller, kronik veya tıkayıcı kolestaza ve ilerleyici karaciğer hastalığına neden olur. Doğumsal hepatik fibroz ve polikistik böbrek hastalığında terminal interlobuler safra kanallarında kistik oluşumlar yer alır, kolestaz yoktur. Karaciğer ve safra yolları işlev bozukluğuna yol açmazlar.

Cystic diseases of the biliary tract and liver

Cystic diseases of liver are recognized in infancy and childhood initially. Cystic diseases of liver and biliary tract are choledocal cysts, autosomal recessive and autosomal dominant polycystic kidney disease, congenital hepatic fibrosis and Caroli disease (cystic dilatation of intrahepatic bile ducts). Choledochal cysts and Caroli disease do not allow biliary flow, cause chronic or obstructive cholestasis and progressive liver disease. In congenital hepatic fibrosis and polycystic kidney disease there is cystic formations at terminal interlobular bile ducts, but cholestasis is not seen. They don’t cause liver and biliary tract functional disturbances.

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