ReviewHepatic medicine : evidence and research2018
Progressive familial intrahepatic cholestasis: diagnosis, management, and treatment.
Review in Hepatic medicine : evidence and research, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 42 papers, 1 of them a synthesis that pooled it.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
42 citing papers in PubMed, 1 synthesis or guideline pooled it.
- Epidemiology and burden of progressive familial intrahepatic cholestasis: a systematic review.Orphanet journal of rare diseases · 2021Pooled it
- Maralixibat for the treatment of PFIC: Long-term, IBAT inhibition in an open-label, Phase 2 study.Hepatology communications · 2022Trial
- Genetic and clinical spectrum of progressive familial intrahepatic cholestasis type 1: novel ATP8B1 variants and genotype-phenotype correlations.European journal of pediatrics · 2026Article
- Immunohistochemical Expression of HLA Classes I and II in Neonatal Cholestatic Liver Disease.Iranian journal of pathology · 2026Article
- Evaluation of miRNA Expression in Pediatric Cirrhosis Caused by BA and PFIC.BioMed research international · 2026Article
- Uncovering the Molecular Signatures of Rare Genetic Diseases in the Punjabi Population.International journal of molecular sciences · 2025Observational
- Odevixibat after liver transplant in patients with progressive familial intrahepatic cholestasis type 1: A case series.Journal of pediatric gastroenterology and nutrition · 2025Article
- Genotypes and different clinical variants between children and adults in progressive familial intrahepatic cholestasis: a state-of-the-art review.Orphanet journal of rare diseases · 2025Review
- Novel ABCB4 mutation in a female patient with progressive familial intrahepatic cholestasis type 3: a case report and literature review.Annals of medicine and surgery (2012) · 2025Article
- Case Report: Sustained biochemical remission following early initiation of odevixibat in an infant with monoallelic ABCB11 mutation and histologically confirmed PFIC2.Frontiers in pediatrics · 2025Article
- Food and Drug Administration Approval Summary: Odevixibat (Bylvay) for the Treatment of Pruritus With Progressive Familial Intrahepatic Cholestasis.Gastro hep advances · 2025Review
- Bile acids as therapeutic agents.Frontiers in pharmacology · 2025Review
- Case Report: A rare case of familial progressive cholestasis type 10 in an adult with heterozygous MYO5B variant.Frontiers in gastroenterology (Lausanne, Switzerland) · 2025Article
- Article
- Hepatocellular carcinoma associated with progressive intrahepatic familial cholestasis type 2: a case report.Clinical transplantation and research · 2024Article
- Progressive familial intrahepatic cholestasis type 4: a case report.Journal of medical case reports · 2024Article
- Article
- Article
- Odevixibat: A Novel Bile Salt Inhibitor Treatment for Pruritus in Progressive Familial Intrahepatic Cholestasis.Cureus · 2024Review
- Exceptional Liver Transplant Indications: Unveiling the Uncommon Landscape.Diagnostics (Basel, Switzerland) · 2024Review
Corrections and comments
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Authors and funding
2 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Progressive familial intrahepatic cholestasis (PFIC) is a group of autosomal recessive cholestatic liver diseases which are subgrouped according to the genetic defect, clinical presentation, laboratory findings and liver histology. Progressive liver fibrosis, cirrhosis, and end stage liver disease (ESLD) may eventually develop. PFIC was first described in Amish descendants of Jacob Byler, therefore it was originally called Byler disease. But it can be seen anywhere on the globe. This review summarizes the main features of the subtypes of the disease and discusses the current available diagnosis, conservative and surgical therapeutic options.
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Registered trials
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.