ReviewCellular and molecular gastroenterology and hepatology2019
Pathophysiology of Cystic Fibrosis Liver Disease: A Channelopathy Leading to Alterations in Innate Immunity and in Microbiota.
Review in Cellular and molecular gastroenterology and hepatology, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 26 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.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
26 citing papers in PubMed, 66 citations in OpenAlex.
- Elastic Fibers in the Pathogenesis of Cystic Fibrosis: Mechanisms, Consequences, and Therapeutic Implications.Life (Basel, Switzerland) · 2026Review
- Cholangiocyte biology in primary sclerosing cholangitis and other cholangiopathies: pathogenesis, clinical insights, and experimental tools.Physiological reviews · 2026Review
- Decoding liver injury in cystic fibrosis: How to tell drug-induced liver injury from cystic fibrosis liver disease.World journal of gastroenterology · 2026Review
- Unveiling the Immunopharmacological Mechanisms of Biejiajian Pills (BJJP) Underlying the Treatment of Hepatic Fibrosis: Insights From Network Pharmacology and Experimental Validation.Food science & nutrition · 2026Article
- Pathophysiology of cystic fibrosis-related liver disease.Current opinion in gastroenterology · 2026Review
- Impact of Cystic Fibrosis Transmembrane Conductance Regulator Modulating Therapies on Liver Transplant Outcomes.Gastro hep advances · 2026Article
- Cystic fibrosis liver disease progression in the era of elexacaftor-tezacaftor-ivacaftor.JHEP reports : innovation in hepatology · 2025Article
- Hepatocellular Carcinoma in Cystic Fibrosis Liver Disease.Gastro hep advances · 2025Article
- The ageing of people living with cystic fibrosis: what to expect now?European respiratory review : an official journal of the European Respiratory Society · 2024Review
- Genetic engineering drives the breakthrough of pig models in liver disease research.Liver research (Beijing, China) · 2024Review
- Prominent role of gut dysbiosis in the pathogenesis of cystic fibrosis-related liver disease in mice.Journal of hepatology · 2024Article
- Research progress and application of liver organoids for disease modeling and regenerative therapy.Journal of molecular medicine (Berlin, Germany) · 2024Review
- Diagnosis and Management of Simple and Complicated Meconium Ileus in Cystic Fibrosis, a Systematic Review.Diagnostics (Basel, Switzerland) · 2024Review
- Multidisciplinarity and Trandisciplinarity in the Diagnosis and Treatment of Pediatric Gastrointestinal Diseases.Diagnostics (Basel, Switzerland) · 2024Article
- Scoring Abdominal Symptoms in People with Cystic Fibrosis.Journal of clinical medicine · 2024Review
- Diagnosis, Management, and Prognosis of Cystic Fibrosis-Related Liver Disease in Children.Diagnostics (Basel, Switzerland) · 2024Review
- Article
- Diagnosis and Management of Gastrointestinal Manifestations in Children with Cystic Fibrosis.Diagnostics (Basel, Switzerland) · 2024Review
- Protective Effects and Mechanisms of Inhibiting Endoplasmic Reticulum Stress on Cold Seawater Immersion Combined with Hemorrhagic Shock.Journal of inflammation research · 2024Article
- Role of innate immunity and systemic inflammation in cystic fibrosis disease progression.Heliyon · 2023Review
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
Abstract
Cystic fibrosis (CF) is a monogenic disease caused by mutation of Cftr. CF-associated liver disease (CFLD) is a common nonpulmonary cause of mortality in CF and accounts for approximately 2.5%-5% of overall CF mortality. The peak of the disease is in the pediatric population, but a second wave of liver disease in CF adults has been reported in the past decade in association with an increase in the life expectancy of these patients. New drugs are available to correct the basic defect in CF but their efficacy in CFLD is not known. The cystic fibrosis transmembrane conductance regulator, expressed in the apical membrane of cholangiocytes, is a major determinant for bile secretion and CFLD classically has been considered a channelopathy. However, the recent findings of the cystic fibrosis transmembrane conductance regulator as a regulator of epithelial innate immunity and the possible influence of the intestinal disease with an altered microbiota on the liver complication have opened new mechanistic insights on the pathogenesis of CFLD. This review provides an overview of the current understanding of the pathophysiology of the disease and discusses a potential target for intervention.
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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.