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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… 
National Institutes of Health

Related topics

Related topics

15 relations
ATP8B1, 2097T-C, +2Autosomal recessive inheritanceCholestasis, Progressive Familial Intrahepatic, 2Congenital hypofibrinogenemia

Broader (2)

CholestasisIntrahepatic Cholestasis

Papers overview

Semantic Scholar uses AI to extract papers important to this topic.
Highly Cited
2012
Highly Cited
2012
Surgical Approach for Long-term Survival of Patients With Intrahepatic Cholangiocarcinoma: A Multi-institutional Analysis of 434 Patients.
OBJECTIVES To examine the outcomes of a hepatectomy for intrahepatic cholangiocarcinoma (IHC) and to clarify the prognostic… 
Highly Cited
2006
Highly Cited
2006
Liver-Infiltrating Lymphocytes in Chronic Human Hepatitis C Virus Infection Display an Exhausted Phenotype with High Levels of PD-1 and Low Levels of CD127 Expression
ABSTRACT The majority of people infected with hepatitis C virus (HCV) fail to generate or maintain a T-cell response effective… 
Highly Cited
2005
Highly Cited
2005
Expression and localization of hepatobiliary transport proteins in progressive familial intrahepatic cholestasis
Mutations of the bile salt export pump (BSEP) or the multidrug resistance P‐glycoprotein 3 (MDR3) are linked to impaired bile… 
Highly Cited
2003
Highly Cited
2003
Farnesoid X Receptor Activates Transcription of the Phospholipid Pump MDR3*
The human multidrug resistance gene MDR3 encodes a P-glycoprotein that belongs to the ATP-binding cassette transporter family… 
Highly Cited
2002
Highly Cited
2002
Bile acid-activated nuclear receptor FXR suppresses apolipoprotein A-I transcription via a negative FXR response element.
Serum levels of HDL are inversely correlated with the risk of coronary heart disease. The anti-atherogenic effect of HDL is… 
Review
2001
Review
2001
Overview: ABC Transporters and Human Disease
ABC transporters are found in all known organisms, and approximately 1,100 different transporters belonging to this family have… 
Highly Cited
2001
Highly Cited
2001
Proliferative activity of intrahepatic colorectal metastases after preoperative hemihepatic portal vein embolization
Although hemihepatic portal vein embolization (PVE) has been used preoperatively to extend indications for hepatectomy in… 
Highly Cited
1994
Highly Cited
1994
Choledochal Cyst Disease A Changing Pattern of Presentation
ObjectiveThe authors compared the presentation, treatment, and long-term outcome of children and adults with choledochal cysts… 
Highly Cited
1993
Highly Cited
1993
Compartmentalization of T lymphocytes to the site of disease: intrahepatic CD4+ T cells specific for the protein NS4 of hepatitis C virus in patients with chronic hepatitis C
The adult liver is an organ without constitutive lymphoid components. Therefore, any intrahepatic T cell found in chronic… 
Highly Cited
1973
Highly Cited
1973
Progressive familial intrahepatic cholestasis.
Two brothers with an early onset of fluctuating jaundice, pruritus, malabsorption, and rickets are reported. Biochemical features… 
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