Term Name: progressive familial intrahepatic cholestasis
Synonyms: Byler disease, PFIC
Definition: An intrahepatic cholestasis characterized by early onset of chronic unremitting cholestasis of hepatocellular origin that progresses to hepatic fibrosis, cirrhosis, and end-stage liver disease before adulthood.
Ontology: Human Disease [DOID:0070221]   ( DOID:0070221 )

Relationships
is a type of: intrahepatic cholestasis
has subtype: progressive familial intrahepatic cholestasis 1 progressive familial intrahepatic cholestasis 2 progressive familial intrahepatic cholestasis 3 progressive familial intrahepatic cholestasis 4 progressive familial intrahepatic cholestasis 5