ArticleClinical gastroenterology and hepatology : the official clinical practice journal of the American Gastroenterological Association2016
Features of Severe Liver Disease With Portal Hypertension in Patients With Cystic Fibrosis.
Article in Clinical gastroenterology and hepatology : the official clinical practice journal of the American Gastroenterological Association, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT00804583 (Genetic Modifiers of CF Liver Disease), which is not on this map. Cited by 38 papers, 1 of them a synthesis that pooled it.
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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.
Genetic Modifiers of CF Liver Disease
Who cites it
38 citing papers in PubMed, 1 synthesis or guideline pooled it, 135 citations in OpenAlex.
- Circulating biomarkers of antioxidant status and oxidative stress in people with cystic fibrosis: A systematic review and meta-analysis.Redox biology · 2020Pooled it
- From bilirubin to breakthroughs: Contemporary outcomes in pediatric liver transplantation for cystic fibrosis.Journal of pediatric gastroenterology and nutrition · 2026Article
- Maternal and fetal outcomes associated with CFTR modulator use during pregnancy: a scoping review.European respiratory review : an official journal of the European Respiratory Society · 2026Article
- Decoding liver injury in cystic fibrosis: How to tell drug-induced liver injury from cystic fibrosis liver disease.World journal of gastroenterology · 2026Review
- Screening and monitoring across the lifespan of an ageing cystic fibrosis population.ERJ open research · 2025Review
- Rare Mutation in Cystic Fibrosis as a Cause of Early-Onset Liver Disease and Esophageal Varices.Cureus · 2025Article
- Genetic modifiers of body mass index in individuals with cystic fibrosis.American journal of human genetics · 2024Article
- Heterogeneous liver on research ultrasound identifies children with cystic fibrosis at high risk of advanced liver disease.Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society · 2023Observational
- Ameliorating liver disease in an autosomal recessive polycystic kidney disease mouse model.American journal of physiology. Gastrointestinal and liver physiology · 2023Article
- Modifier Factors of Cystic Fibrosis Phenotypes: A Focus on Modifier Genes.International journal of molecular sciences · 2022Review
- Pleiotropic modifiers of age-related diabetes and neonatal intestinal obstruction in cystic fibrosis.American journal of human genetics · 2022Article
- Risk of variceal hemorrhage and pretransplant mortality in children with biliary atresia.Hepatology (Baltimore, Md.) · 2022Article
- Liver disease in cystic fibrosis patients in a tertiary care center in Saudi Arabia.International journal of pediatrics & adolescent medicine · 2022Article
- Cystic fibrosis related liver disease and endocrine considerations.Journal of clinical & translational endocrinology · 2022Article
- Understanding Cystic Fibrosis Comorbidities and Their Impact on Nutritional Management.Nutrients · 2022Review
- Intestinal Inflammation and Alterations in the Gut Microbiota in Cystic Fibrosis: A Review of the Current Evidence, Pathophysiology and Future Directions.Journal of clinical medicine · 2022Review
- Assessment of Liver Fibrosis with the Use of Elastography in Paediatric Patients with Diagnosed Cystic Fibrosis.Disease markers · 2022Article
- Cystic fibrosis associated liver disease in children.World journal of hepatology · 2021Review
- Non-invasive tools for detection of liver disease in children and adolescents with cystic fibrosis.Translational pediatrics · 2021Article
- Clinical use of shear-wave elastography for detecting liver fibrosis in children and adolescents with cystic fibrosis.Pediatric radiology · 2021Article
Corrections and comments
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Authors and funding
7 authors at 6 institutions in 4 countries.
Funding
Abstract
BACKGROUND &
aimsLiver disease is the third leading cause of death in patients with cystic fibrosis (CF), but features of patients with CF, severe liver disease, and portal hypertension have not been characterized fully.
methodsWe performed a retrospective analysis of data from 561 patients with CF (63% male, 99% with pancreatic insufficiency), liver disease (hepatic parenchymal abnormalities consistent with cirrhosis, confirmed by imaging), and portal hypertension (esophageal varices, portosystemic collaterals, or splenomegaly), with no alternate causes of liver disease. All patients were enrolled in the Genetic Modifier Study of Severe CF Liver Disease at 76 international centers, from January 1999 through July 2013.
resultsMale patients were diagnosed with liver disease at a younger age than female patients (10 vs 11 y; P = .01). Splenomegaly was observed in 99% of patients and varices in 71%. Levels of liver enzymes were near normal in most patients. Thrombocytopenia affected 70% of patients and was more severe in patients with varices (88 × 10(9)/L vs 145 × 10(9)/L; P < .0001). Ninety-one patients received liver transplants (16%), at a median age of 13.9 years. Compared with patients who did not receive liver transplants, patients who received liver transplants had lower platelet counts (78 × 10(9)/L vs 113 × 10(9)/L; P < .0001), higher international normalized ratios (P < .0001), and lower levels of albumin (P = .0002). The aminotransferase to platelet ratio index (APRi) and fibrosis index based on 4 factor (FIB-4) values were higher than the diagnostic thresholds for CF liver disease in 96% and in 90% of patients, respectively. Patients who received liver transplants or who had varices had higher APRi and FIB-4 values than patients who did not.
conclusionsIn patients with CF, severe liver disease develops early in childhood (approximately 10 years of age), and is more common in boys than in girls. Patients with varices and those who receive liver transplants have more abnormal platelet counts and APRi and FIB-4 scores.
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