ReviewWorld journal of hepatology2022
Recent updates on progressive familial intrahepatic cholestasis types 1, 2 and 3: Outcome and therapeutic strategies.
Review in World journal of hepatology, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT05704517 (Progressive Familial Intrahepatic Cholestasis in Indian Children - Establishing an Indian PFIC Registry), which is not on this map. Cited by 22 papers.
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.
Progressive Familial Intrahepatic Cholestasis in Indian Children - Establishing an Indian PFIC Registry
Who cites it
22 citing papers in PubMed, 40 citations in OpenAlex.
- Novel genetic variants and atypical phenotypes in pediatric progressive familial intrahepatic cholestasis.Pediatric research · 2026Article
- A Hematologic Masquerader: Progressive Familial Intrahepatic Cholestasis Type 3 Presenting as Anemia, Hepatosplenomegaly, and Recurrent Bleeding in a Child.Clinical case reports · 2026Article
- Altered Bile Acid Transport in Liver Disease.Biomedicines · 2026Review
- Remarkable Response to Odevixibat in an Adult With Progressive Familial Intrahepatic Cholestasis Type 1 and Intractable Pruritus.ACG case reports journal · 2026Article
- ATP-binding Cassette Transporter Defects and Their Roles in Hepatic Diseases.Journal of clinical and translational hepatology · 2026Review
- Functional inactivation of MDR3 caused by a homozygousFrontiers in genetics · 2026Article
- Therapeutic modulation of the gut microbiota by traditional Chinese medicine in the management of cholestatic liver injury.Frontiers in cellular and infection microbiology · 2026Review
- Atypical presentation of pediatric acute hepatitis A: Is the situation alarming?World journal of virology · 2025Review
- Rare liver diseases - Etiology, diagnosis and management: A review.Biomolecules & biomedicine · 2025Review
- An overview of paediatric autoimmune and genetic cholestatic liver disease for the adult physician.Clinical medicine (London, England) · 2025Review
- Real-world experience with odevixibat in children with progressive familial intrahepatic cholestasis.JHEP reports : innovation in hepatology · 2025Article
- Liver fibrosis negatively impacts in vivo gene transfer to murine hepatocytes.Nature communications · 2025Article
- 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
- Recurrent Jaundice Unraveled: A Case of Benign Recurrent Intrahepatic Cholestasis (BRIC) in an Indian Patient.Cureus · 2024Article
- Progressive familial intrahepatic cholestasis type 4: a case report.Journal of medical case reports · 2024Article
- Progressive Familial Intrahepatic Cholestasis: A Descriptive Study in a Tertiary Care Center.International journal of hepatology · 2023Article
- Clinical and genetic characterization of pediatric patients with progressive familial intrahepatic cholestasis type 3 (PFIC3): identification of 14 novel ABCB4 variants and review of the literatures.Orphanet journal of rare diseases · 2022Review
- Article
- Long-Term Results of Pediatric Liver Transplantation for Progressive Familial Intrahepatic Cholestasis.Journal of clinical medicine · 2022Article
- Pediatric acute viral hepatitis with atypical variants: Clinical dilemmas and natural history.World journal of hepatology · 2022Review
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
2 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Recent evidence points towards the role of genotype to understand the phenotype, predict the natural course and long term outcome of patients with progressive familial intrahepatic cholestasis (PFIC). Expanded role of the heterozygous transporter defects presenting late needs to be suspected and identified. Treatment of pruritus, nutritional rehabilitation, prevention of fibrosis progression and liver transplantation (LT) in those with end stage liver disease form the crux of the treatment. LT in PFIC has its own unique issues like high rates of intractable diarrhoea, growth failure; steatohepatitis and graft failure in PFIC1 and antibody-mediated bile salt export pump deficiency in PFIC2. Drugs inhibiting apical sodium-dependent bile transporter and adenovirus-associated vector mediated gene therapy hold promise for future.
Indexed as
Identifiers
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.