Progressive Familial Intrahepatic Cholestasis

Neonatal cholestasis : How do I approach?

Source Event :Case discussions in Pediatric Liver Diseases organised by Children's Liver Foundation and Indian Academy of pediatric, Mumbai on 14th and 15th December 2013.
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Presented by
Dr Avinash Khade
Moderated by
Dr S K Yachha
Panelists
Dr Malathi Sathiyasekaran
Dr Girish Gupte
Dr Ashley D'Cruz

Odevixibat treatment in progressive familial intrahepatic cholestasis: a randomised, placebo-controlled, phase 3 trial

Impact of progressive familial intrahepatic cholestasis on caregivers: caregiver-reported outcomes from the multinational PICTURE study

Biliary Diversion in Progressive Familial Intrahepatic Cholestasis: A Systematic Review and Meta-Analysis

Progressive familial intrahepatic cholestasis - farnesoid X receptor deficiency due to NR1H4mutation: A case report

Progressive Familial Intrahepatic Cholestasis Associated With USP53 Gene Mutation in a Brazilian Child

Potential of Ileal Bile Acid Transporter Inhibition as a Therapeutic Target in Alagille Syndrome and Progressive Familial Intrahepatic Cholestasis

Liver Involvement in Children with Alpha-1 Antitrypsin Deficiency: A Multicenter Study

Successful Treatment with Rituximab and Immunoadsorption for an Auto-Antibody Induced Bile Salt Export Pump Deficiency in a Liver Transplanted Patient.

Effects of partial internal biliary diversion on long-term outcomes in patients with progressive familial intrahepatic cholestasis: experience in 44 patients

NR1H4-related Progressive Familial Intrahepatic Cholestasis 5: Further Evidence for Rapidly Progressive Liver Failure.

Novel ATP8B1 Gene Mutations in a Child with Progressive Familial Intrahepatic Cholestasis Type 1.

Alloimmunity and Cholestasis After Liver Transplantation in Children With Progressive Familial Intrahepatic Cholestasis.

Systematic review of progressive familial intrahepatic cholestasis.

Quality of Life in Patients with Progressive Familial Intrahepatic Cholestasis: No Difference between Post Liver Transplantation and Post Partial External Biliary Diversion.

Rescue of defective ATP8B1 trafficking by CFTR correctors as a therapeutic strategy for familial intrahepatic cholestasis

Possible Phenylacetate Hepatotoxicity during 4-Phenylbutyrate Therapy of Byler Disease.

Targeted pharmacotherapy in progressive familial intrahepatic cholestasis type 2: Evidence for improvement of cholestasis with 4-phenylbutyrate

Functional analysis of ABCB4 mutations relates clinical outcomes of progressive familial intrahepatic cholestasis type 3 to the degree of MDR3 floppase activity

Progressive familial Intrahepatic Cholestasis

Partial Internal Biliary Diversion: A Solution for Intractable Pruritus in Progressive Familial Intrahepatic Cholestasis Type 1

Hepatocellular Carcinoma in a Boy with Progressive familial Intrahepatic Cholestasis Type II: Challenging Identification: Case report

Therapeutic interventions in progressive familial intrahepatic cholestasis: experience from a tertiary care centre in north India

Progressive Familial Intrahepatic Cholestasis

What is PFIC ?

PFIC as the name stands for is a rare progressive disorder affecting liver due to defect in secretion of bile. As an inherited inability to secrete bile , it accumulates in the liver causing damage and ultimately end stage liver failure.

 

PFIC is Autosomal recessive disorder and can affect both males and females equally. It is a rare disease affecting about 1 per 1,00,000 births around the world.

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