Progressive intrahepatic cholestasis (disorder)

Known as: PFIC1, cholestasis familial intrahepatic, Byler's syndrome 
A genetic syndrome affecting infants and children. It is characterized by chronic intrahepatic cholestasis usually progressing to cirrhosis within… (More)
National Institutes of Health

Papers overview

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Review
2014
Review
2014
Progressive familial intrahepatic cholestasis (PFIC) is a group of rare disorders which are caused by defect in bile secretion… (More)
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Highly Cited
2005
Highly Cited
2005
Mutations of the bile salt export pump (BSEP) or the multidrug resistance P-glycoprotein 3 (MDR3) are linked to impaired bile… (More)
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Highly Cited
2004
Highly Cited
2004
BACKGROUND & AIMS Progressive familial intrahepatic cholestasis (PFIC) and benign recurrent intrahepatic cholestasis (BRIC) are… (More)
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Highly Cited
2004
Highly Cited
2004
BACKGROUND/AIMS The incidence of intrahepatic cholangiocarcinoma (ICC) has been reported to be increasing in the USA. The aim of… (More)
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Highly Cited
2001
Highly Cited
2001
Clinical observations suggest a recent increase in intrahepatic biliary tract malignancies. Thus, our aim was to determine recent… (More)
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Highly Cited
2000
Highly Cited
2000
Intrahepatic cholestasis of pregnancy (ICP) is a liver disease of pregnancy with serious consequences for the mother and fetus… (More)
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Highly Cited
1999
Highly Cited
1999
BACKGROUND & AIMS Progressive familial intrahepatic cholestasis (PFIC), an inherited liver disease of childhood, is characterized… (More)
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Highly Cited
1998
Highly Cited
1998
The progressive familial intrahepatic cholestases (PFIC) are a group of inherited disorders with severe cholestatic liver disease… (More)
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Highly Cited
1998
Highly Cited
1998
Cholestasis, or impaired bile flow, is an important but poorly understood manifestation of liver disease. Two clinically distinct… (More)
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Highly Cited
1997
Highly Cited
1997
Byler disease (ByD) is an autosomal recessive disorder in which cholestasis of onset in infancy leads to hepatic fibrosis and… (More)
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