ArticlePloS one2017
Assessment of pathologic increase in liver stiffness enables earlier diagnosis of CFLD: Results from a prospective longitudinal cohort study.
Article in PloS one, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 14 papers.
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14 citing papers in PubMed, 44 citations in OpenAlex.
- Prospective study of quantitative liver MRI in cystic fibrosis: feasibility and comparison to PUSH cohort ultrasound.Pediatric radiology · 2023Trial
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- Age-Dependent Systemic Regulation of C1q/TNF-Related Protein 3 and Progranulin in Patients with Cystic Fibrosis: Biomarkers or Therapeutic Targets?Biomedicines · 2026Article
- Screening and monitoring across the lifespan of an ageing cystic fibrosis population.ERJ open research · 2025Review
- The effect of elexacaftor-tezacaftor-ivacaftor on liver stiffness in children with cystic fibrosis.Journal of pediatric gastroenterology and nutrition · 2025Article
- New guidelines for screening, evaluation and management of hepatobiliary disease in cystic fibrosis.Hepatobiliary surgery and nutrition · 2024Article
- Cystic fibrosis hepatobiliary involvement: an update on imaging in diagnosis and monitoring.Pediatric radiology · 2024Review
- Evidence and therapeutic implications of biomechanically regulated immunosurveillance in cancer and other diseases.Nature nanotechnology · 2024Review
- Update on Cystic Fibrosis in Pediatric Patients.Current gastroenterology reports · 2023Review
- Assessment of Liver Fibrosis with the Use of Elastography in Paediatric Patients with Diagnosed Cystic Fibrosis.Disease markers · 2022Article
- Assessment of Selected Parameters of Liver Fibrosis and Inflammation in Patients with Diagnosed Cystic Fibrosis.Mediators of inflammation · 2020Article
- Cystic Fibrosis Liver Disease: Know More.Oman medical journal · 2019Review
- Advances in diagnosis of chronic liver diseases in pediatric patients.World journal of pediatrics : WJP · 2018Review
- Liver disease in patients with cystic fibrosis.Current opinion in gastroenterology · 2018Review
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Authors and funding
8 authors at 2 institutions in 1 country.
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No grant is acknowledged in the PubMed record.
Abstract
About 30% of patients with Cystic Fibrosis (CF) develop CF-associated liver disease (CFLD). Recent studies have shown that transient elastography (TE), as a method to quantify liver stiffness, allows non-invasive diagnosis of CFLD in adults and children with CF. Within this study we aimed to prospectively identify patients at risk for development of CFLD by longitudinal analysis of liver stiffness and fibrosis scores in a 5-year follow-up. 36 pediatric and 16 adult patients with initial liver stiffness below the cut-off value indicative of CFLD (6.3 kPa) were examined by transient elastography for 4-5 years. TE, APRI-, and FIB-4-scores were assessed and compared by Kruskal-Wallis test and receiver operating characteristic (ROC)-analysis. Frequencies were compared by Chi2-test. Among the 36 patients participating in this study, a subgroup of 9 patients developed liver stiffness >6.3 kPa after 4-5 years with an increase of ΔTE >0.38 kPa/a (the group with increasing liver stiffness was labelled TEinc). APRI- and FIB-4 scores confirmed the rationale for grouping. The frequency of CFLD assessed by conventional diagnosis was significantly higher in TEinc-group compared to the control group (TEnorm). None of the adult CF patients matched criteria for TEinc-group. For the first time it was shown that the non-invasive longitudinal assessment of TE allows identification of patients with progression of CFLD in a subgroup of juvenile but not in adult CF patients. Comparing TE to conventional fibrosis-scores underlined the strength of the continuous assessment of liver stiffness for the exact diagnosis of progressive CFLD. The newly described cut-off for pathologic increase of liver stiffness, ΔTEcutoff = 0.38kPa/a, might enable to detect developing CFLD using consequent follow up TE measurements before reaching the level of stiffness indicating established CFLD. Nevertheless, the limited size of the analyzed cohort should encourage a prospective, multi-center, long term follow up study to confirm the suggested cut-off for the rise in liver stiffness.
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