The hepatic fibrocystic diseases.
The hepatic fibrocystic diseases present with variable intrahepatic biliary abnormalities, which range from portal tract enlargement and fibrosis to cystic formations. They may present as autosomal recessive or dominant polycystic kidney diseases, with associated dilatation of the renal collecting system, or as incompletely characterized cystic diseases. Symptoms from the liver disease often result from complications of fibrosis or dilated ducts/cyst (sludge, lithiasis, infection). The treatment is supportive, with careful attention to associated renal disease. Liver transplantation is an option in selected patients.
Kerkar
Nanda
N
Department of Pediatrics, Mount Sinai School of Medicine, 1 Gustave L. Levy Place, New York, NY 10029, USA.
Norton
Karen
K
Suchy
Frederick J
FJ
D016767:Caroli Disease / Q000150:complications / Q000235:genetics*; D015529:Choledochal Cyst / Q000150:complications / Q000235:genetics; D003560:Cysts / Q000150:complications / Q000235:genetics*; D006801:Humans; D008107:Liver Diseases / Q000150:complications / Q000235:genetics*; D007690:Polycystic Kidney Diseases / Q000150:complications / Q000235:genetics*