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Congenital hepatic fibrosis is an inherited fibrocystic liver disease associated with proliferation of interlobular bile ducts within the portal areas and fibrosis that do not alter hepatic lobular architecture. The fibrosis would affect resistance in portal veins leading to portal hypertension. [citation needed]
Type V: Cystic dilatation of intrahepatic biliary ducts without extrahepatic duct disease. The presence of multiple saccular or cystic dilations of the intrahepatic ducts is known as Caroli's disease. [4] Type VI: An isolated cyst of the cystic duct is an extremely rare lesion. Only single case reports are documented in the literature.
Chronic liver diseases like chronic hepatitis, chronic alcohol abuse or chronic toxic liver disease may cause liver failure and hepatorenal syndrome; fibrosis and cirrhosis of liver; Cirrhosis may also occur in primary biliary cirrhosis. Rarely, cirrhosis is congenital.
Caroli disease (communicating cavernous ectasia, or congenital cystic dilatation of the intrahepatic biliary tree) is a rare inherited disorder characterized by cystic dilatation (or ectasia) of the bile ducts within the liver. There are two patterns of Caroli disease: focal or simple Caroli disease consists of abnormally widened bile ducts ...
This category reflects the organization of International Statistical Classification of Diseases and Related Health Problems, 10th Revision. Generally, diseases outlined within the ICD-10 codes K70-K77 within Chapter XI: Diseases of the digestive system should be included in this category.
Cystic fibrosis; D De Lange syndrome ... Huntington's disease; Hirschsprung's disease, ... List of ICD-9 codes 740–759: congenital anomalies; Rare disease
This category reflects the organization of International Statistical Classification of Diseases and Related Health Problems, 10th Revision. Generally, diseases outlined within the ICD-10 codes Q00-Q99 within Chapter XVII: Congenital malformations, deformations and chromosomal abnormalities should be included in this category.
Biliary atresia, also known as extrahepatic ductopenia and progressive obliterative cholangiopathy, is a childhood disease of the liver in which one or more bile ducts are abnormally narrow, blocked, or absent. It can be congenital or acquired. Biliary atresia is the most common reason for pediatric liver trasplantation in the United States. [2]