Learn About Progressive Familial Intrahepatic Cholestasis Type 1

What is the definition of Progressive Familial Intrahepatic Cholestasis Type 1?

Progressive familial intrahepatic cholestasis (PFIC) is a disorder that causes progressive liver disease, which typically leads to liver failure. In people with PFIC, liver cells are less able to secrete a digestive fluid called bile. The buildup of bile in liver cells causes liver disease in affected individuals.

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What are the causes of Progressive Familial Intrahepatic Cholestasis Type 1?

Mutations in the ATP8B1, ABCB11, and ABCB4 genes can cause PFIC.

How prevalent is Progressive Familial Intrahepatic Cholestasis Type 1?

PFIC is estimated to affect 1 in 50,000 to 100,000 people worldwide. PFIC type 1 is much more common in the Inuit population of Greenland and the Old Order Amish population of the United States.

Is Progressive Familial Intrahepatic Cholestasis Type 1 an inherited disorder?

This condition is inherited in an autosomal recessive pattern, which means both copies of the gene in each cell have mutations. The parents of an individual with an autosomal recessive condition each carry one copy of the mutated gene, but they typically do not show signs and symptoms of the condition.

Who are the top Progressive Familial Intrahepatic Cholestasis Type 1 Local Doctors?
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King's College London

Institute Of Liver Studies 
London, ENG, GB 

Richard Thompson is in London, United Kingdom. Thompson is rated as an Elite expert by MediFind in the treatment of Progressive Familial Intrahepatic Cholestasis Type 1. He is also highly rated in 12 other conditions, according to our data. His top areas of expertise are Progressive Familial Intrahepatic Cholestasis Type 1, Cholestasis, Bile Duct Obstruction, and Biliary Atresia.

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Hepatology, Feeding Disorders And Pediatrics, The Children's Memorial Health Institute

Warsaw, MZ, PL 

Irena Jankowska is in Warsaw, Poland. Jankowska is rated as an Elite expert by MediFind in the treatment of Progressive Familial Intrahepatic Cholestasis Type 1. She is also highly rated in 19 other conditions, according to our data. Her top areas of expertise are Progressive Familial Intrahepatic Cholestasis Type 1, Cholestasis, Bile Duct Obstruction, and Progressive Familial Intrahepatic Cholestasis Type 2.

 
 
 
 
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Assistance Publique Hôpitaux De Paris

Inserm Umrs 1193, Paris Saclay University 
Orsay, FR 

Emmanuel Gonzales is in Orsay, France. Gonzales is rated as an Elite expert by MediFind in the treatment of Progressive Familial Intrahepatic Cholestasis Type 1. He is also highly rated in 11 other conditions, according to our data. His top areas of expertise are Cholestasis, Progressive Familial Intrahepatic Cholestasis Type 1, Progressive Familial Intrahepatic Cholestasis Type 2, and Bile Duct Obstruction.

What are the latest Progressive Familial Intrahepatic Cholestasis Type 1 Clinical Trials?
An Open-label Extension Study to Evaluate Long-term Efficacy and Safety of A4250 in Children With Progressive Familial Intrahepatic Cholestasis Types 1 and 2 (PEDFIC 2)
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Gastrostomy-Biliary Diversion: Innovative Management for Children With Bile Canalicular Transport Disorders
Who are the sources who wrote this article ?

Published Date:updated Last, December

Published By: National Institutes of Health

What are the Latest Advances for Progressive Familial Intrahepatic Cholestasis Type 1?
Long-Term Outcomes of Patients With Progressive Familial Intrahepatic Cholestasis After Biliary Diversion.
Partial External Biliary Diversion for Severe Diarrhea After Liver Transplant in Patients with Progressive Familial Intrahepatic Cholestasis Type 1.
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Biliary diversion in progressive familial intrahepatic cholestasis: a systematic review and meta-analysis.