Table 3.

Clinical features of PFIC subtypes

	PFIC type 1	PFIC type 2	PFIC type 3
Age of onset	Early infancy	Early infancy	Later in childhood or young adulthood
Extrahepaticmanifestations	Watery diarrhea Pancreatitis Sensorineural hearing loss	Uncommon	None
Pruritus	Severe	Very severe	Moderate
Cholesterol stone formation	Absent	Increased	Increased
Progression of PFIC subtypes	Liver cirrhosis and rapid progression to ESLD Patients do not have increased risk for development of liver tumors.	Progression even more quickly to end-stage liver disease, require liver transplantation during the first decade of life. Risk of development of liver tumors are high.	Insidious Risk of development of liver tumors are mildly increased.

Abbreviations: ESLD, end-stage liver disease; PFIC, progressive familial intrahepatic cholestasis.