ReviewClinical reviews in allergy & immunology2015
Autoimmune BSEP disease: disease recurrence after liver transplantation for progressive familial intrahepatic cholestasis.
Review in Clinical reviews in allergy & immunology, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 22 papers.
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Who cites it
22 citing papers in PubMed, 65 citations in OpenAlex.
- Genetic Variants and Long-Term Outcomes in Korean Children with Progressive Familial Intrahepatic Cholestasis.Pediatric gastroenterology, hepatology & nutrition · 2025Article
- Article
- Successful Use of Bortezomib for Recurrent Progressive Familial Intrahepatic Cholestasis Type II After Liver Transplantation: A Pediatric Case with a 9-Year Follow-Up.Pediatric gastroenterology, hepatology & nutrition · 2024Article
- Diagnosis and management of Alagille and progressive familial intrahepatic cholestasis.Hepatology communications · 2023Article
- Cell-based BSEP trans-inhibition: A novel, non-invasive test for diagnosis of antibody-induced BSEP deficiency.JHEP reports : innovation in hepatology · 2023Article
- Genetic alterations and molecular mechanisms underlying hereditary intrahepatic cholestasis.Frontiers in pharmacology · 2023Review
- Gene Therapy for Acquired and Genetic Cholestasis.Biomedicines · 2022Review
- Clinical outcomes of surgical management for rare types of progressive familial intrahepatic cholestasis: a case series.Surgical case reports · 2022Article
- Case report: progressive familial intrahepatic cholestasis type 3 with compound heterozygous ABCB4 variants diagnosed 15 years after liver transplantation.BMC medical genetics · 2020Article
- Successful Treatment with Rituximab and Immunoadsorption for an Auto-Antibody Induced Bile Salt Export Pump Deficiency in a Liver Transplanted Patient.Pediatric gastroenterology, hepatology & nutrition · 2020Article
- [Inherited metabolic diseases and liver transplantation].Zhonghua gan zang bing za zhi = Zhonghua ganzangbing zazhi = Chinese journal of hepatology · 2019Review
- Mechanistic insights into geniposide regulation of bile salt export pump (BSEP) expression.RSC advances · 2018Article
- Can Bile Salt Export Pump Inhibition Testing in Drug Discovery and Development Reduce Liver Injury Risk? An International Transporter Consortium Perspective.Clinical pharmacology and therapeutics · 2018Review
- [Clinical and genetic analysis of an infant with progressive familial intrahepatic cholestasis type II].Zhongguo dang dai er ke za zhi = Chinese journal of contemporary pediatrics · 2018Article
- New Insights in Genetic Cholestasis: From Molecular Mechanisms to Clinical Implications.Canadian journal of gastroenterology & hepatology · 2018Review
- Progressive familial intrahepatic cholestasis: diagnosis, management, and treatment.Hepatic medicine : evidence and research · 2018Review
- Analysis of surgical interruption of the enterohepatic circulation as a treatment for pediatric cholestasis.Hepatology (Baltimore, Md.) · 2017Article
- Current and future therapies for inherited cholestatic liver diseases.World journal of gastroenterology · 2017Review
- Liver and Kidney on Chips: Microphysiological Models to Understand Transporter Function.Clinical pharmacology and therapeutics · 2016Review
- Liver transplantation for pediatric inherited metabolic disorders: Considerations for indications, complications, and perioperative management.Pediatric transplantation · 2016Review
Corrections and comments
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Authors and funding
8 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Severe cholestasis may result in end-stage liver disease with the need of liver transplantation (LTX). In children, about 10 % of LTX are necessary because of cholestatic liver diseases. Apart from bile duct atresia, three types of progressive familial intrahepatic cholestasis (PFIC) are common causes of severe cholestasis in children. The three subtypes of PFIC are defined by the involved genes: PFIC-1, PFIC-2, and PFIC-3 are due to mutations of P-type ATPase ATP8B1 (familial intrahepatic cholestasis 1, FIC1), the ATP binding cassette transporter ABCB11 (bile salt export pump, BSEP), or ABCB4 (multidrug resistance protein 3, MDR3), respectively. All transporters are localized in the canalicular membrane of hepatocytes and together mediate bile salt and phospholipid transport. In some patients with PFIC-2 disease, recurrence has been observed after LTX, which mimics a PFIC phenotype. It could be shown by several groups that inhibitory anti-BSEP antibodies emerge, which most likely cause disease recurrence. The prevalence of severe BSEP mutations (e.g., splice site and premature stop codon mutations) is very high in this group of patients. These mutations often result in the complete absence of BSEP, which likely accounts for an insufficient auto-tolerance against BSEP. Although many aspects of this "new" disease are not fully elucidated, the possibility of anti-BSEP antibody formation has implications for the pre- and posttransplant management of PFIC-2 patients. This review will summarize the current knowledge including diagnosis, pathomechanisms, and management of "autoimmune BSEP disease."
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.