ReviewWorld journal of transplantation2016
Liver transplantation and the management of progressive familial intrahepatic cholestasis in children.
Review in World journal of transplantation, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 29 papers, 2 of them syntheses that pooled it.
What it found
Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.
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
29 citing papers in PubMed, 2 syntheses or guidelines pooled it, 61 citations in OpenAlex.
- Pooled it
- Epidemiology and burden of progressive familial intrahepatic cholestasis: a systematic review.Orphanet journal of rare diseases · 2021Pooled it
- Bile Acid Detergency as Determinant of Liver Pathology in a Humanized Mouse Model of Progressive Familial Intrahepatic Cholestasis Type 3.Cellular and molecular gastroenterology and hepatology · 2026Article
- ATP8B1 regulates PIP2 localization and cleavage of pyroptotic executioner Gasdermin D.Proceedings of the National Academy of Sciences of the United States of America · 2025Article
- Hepatocellular carcinoma associated with progressive intrahepatic familial cholestasis type 2: a case report.Clinical transplantation and research · 2024Article
- Pediatric Liver Transplantation: Selection Criteria and Post-transplant Medical Management.Indian journal of pediatrics · 2024Review
- Progressive Familial Intrahepatic Cholestasis-2 Mimicking Non-accidental Injury.ACG case reports journal · 2024Article
- Discrimination of Etiologically Different Cholestasis by Modeling Proteomics Datasets.International journal of molecular sciences · 2024Article
- Interim results from an ongoing, open-label, single-arm trial of odevixibat in progressive familial intrahepatic cholestasis.JHEP reports : innovation in hepatology · 2023Article
- Progressive Familial Intrahepatic Cholestasis: A Descriptive Study in a Tertiary Care Center.International journal of hepatology · 2023Article
- Development of the Patient- and Observer-Reported PRUCISION Instruments to Assess Pruritus and Sleep Disturbance in Pediatric Patients with Cholestatic Liver Diseases.Advances in therapy · 2022Review
- Pathogenic Novel Heterozygous Variant c.1076c>T p. (Ser359Phe) chr1: 120512166 in NOTCH2 Gene, Type 2 Alagille Syndrome Causing Neonatal Cholestasis: A Case Report.The American journal of case reports · 2022Article
- Liver transplantation in pediatric patients with progressive familial intrahepatic cholestasis: Single center experience of seven cases.Annals of hepato-biliary-pancreatic surgery · 2022Article
- Not only a small liver - The pathologist's perspective in the pediatric liver transplant setting.Pathologica · 2022Review
- Recent updates on progressive familial intrahepatic cholestasis types 1, 2 and 3: Outcome and therapeutic strategies.World journal of hepatology · 2022Review
- Clinical outcomes of surgical management for rare types of progressive familial intrahepatic cholestasis: a case series.Surgical case reports · 2022Article
- Long-Term Outcome after Liver Transplantation for Progressive Familial Intrahepatic Cholestasis.Medicina (Kaunas, Lithuania) · 2021Article
- Biopsy Validated Study of Biomarkers for Liver Fibrosis and Transplant Prediction in Inherited Cholestasis.Hepatology communications · 2020Article
- Potential of ileal bile acid transporter inhibition as a therapeutic target in Alagille syndrome and progressive familial intrahepatic cholestasis.Liver international : official journal of the International Association for the Study of the Liver · 2020Review
- Odevixibat and partial external biliary diversion showed equal improvement of cholestasis in a patient with progressive familial intrahepatic cholestasis.BMJ case reports · 2020Article
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
5 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Progressive familial intrahepatic cholestasis (PFIC) is a constellation of inherited disorders that result in the impairment of bile flow through the liver that predominantly affects children. The accumulation of bile results in progressive liver damage, and if left untreated leads to end stage liver disease and death. Patients often present with worsening jaundice and pruritis within the first few years of life. Many of these patients will progress to end stage liver disease and require liver transplantation. The role and timing of liver transplantation still remains debated especially in the management of PFIC1. In those patients who are appropriately selected, liver transplantation offers an excellent survival benefit. Appropriate timing and selection of patients for liver transplantation will be discussed, and the short and long term management of patients post liver transplantation will also be described.
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What OpenQuestion holds
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.