Cystic fibrosis-related liver disease: Clinical presentations, diagnostic and monitoring approaches in the era of CFTR modulator therapies

Ursodeoxycholic acid and liver disease associated with cystic fibrosis: A multicenter cohort study

(2021) Carla Colombo et al.   Journal of Cystic Fibrosis

Lumacaftor-ivacaftor effects on cystic fibrosis-related liver involvement in adolescents with homozygous F508 del-CFTR

(2021) David Drummond et al.   Journal of Cystic Fibrosis

Hepatic manifestations of cystic fibrosis

(2020) Jeremy Dana et al.   CURRENT OPINION IN GASTROENTEROLOGY

Heterogeneous Liver on Research Ultrasound Identifies Children with Cystic Fibrosis at High Risk of Advanced Liver Disease: Interim Results of a Prospective Observational Case-Controlled Study

(2020) Marilyn J. Siegel et al.   JOURNAL OF PEDIATRICS

Nodular Regenerative Hyperplasia – an under-recognized vascular disorder of liver

(2020) Neha Bakshi et al.   PATHOLOGY RESEARCH AND PRACTICE

Cystic fibrosis transmembrane conductance regulator dysfunction in platelets drives lung hyperinflammation

(2020) Guadalupe Ortiz-Muñoz et al.   JOURNAL OF CLINICAL INVESTIGATION

A new definition for metabolic dysfunction-associated fatty liver disease: An international expert consensus statement

(2020) Mohammed Eslam et al.   JOURNAL OF HEPATOLOGY

Transjugular intrahepatic portosystemic shunt in non-cirrhotic portal hypertension related to cystic fibrosis in a lung transplant patient

(2020) A. Lupi et al.   Journal of Cystic Fibrosis

Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) in Human Lung Microvascular Endothelial Cells Controls Oxidative Stress, Reactive Oxygen-Mediated Cell Signaling and Inflammatory Responses

(2020) Maha Khalaf et al.   Frontiers in Physiology

Choline Supplementation in Cystic Fibrosis—The Metabolic and Clinical Impact

(2019) Wolfgang Bernhard et al.   Nutrients

A Multidisciplinary Approach to Pretransplant and Posttransplant Management of Cystic Fibrosis–Associated Liver Disease

(2019) A. Jay Freeman et al.   LIVER TRANSPLANTATION

Cystic fibrosis and portal hypertension: Distal splenorenal shunt can prevent the need for future liver transplant

(2019) Caroline Lemoine et al.   JOURNAL OF PEDIATRIC SURGERY

SERPINA1 Z allele is associated with cystic fibrosis liver disease

(2019) Pierre-Yves Boëlle et al.   GENETICS IN MEDICINE

Accuracy of Transient Elastography Data Combined With APRI in Detection and Staging of Liver Disease in Pediatric Patients With Cystic Fibrosis

(2019) Peter J. Lewindon et al.   Clinical Gastroenterology and Hepatology

Risk factors for adverse outcome in infancy in meconium ileus cystic fibrosis infants: A multicentre Italian study

(2019) Rita Padoan et al.   Journal of Cystic Fibrosis

Liver Ultrasound Patterns in Children with Cystic Fibrosis Correlate with Non-Invasive Tests of Liver Disease

(2019) Simon C. Ling et al.   JOURNAL OF PEDIATRIC GASTROENTEROLOGY AND NUTRITION

The emerging burden of liver disease in cystic fibrosis patients: A UK nationwide study

(2019) M. B. Toledano et al.   PLoS One

Porto-sinusoidal vascular disease: proposal and description of a novel entity

(2019) Andrea De Gottardi et al.   Lancet Gastroenterology & Hepatology

Real-Life Safety and Effectiveness of Lumacaftor-Ivacaftor in Patients with Cystic Fibrosis

(2019) Pierre-Régis Burgel et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Comparison between ROI-based and volumetric measurements in quantifying heterogeneity of liver stiffness using MR elastography

(2019) Roya Rezvani Habibabadi et al.   EUROPEAN RADIOLOGY

The gut-liver axis in liver disease: pathophysiological basis for therapy

(2019) Agustín Albillos et al.   JOURNAL OF HEPATOLOGY

Efficacy and safety of the elexacaftor plus tezacaftor plus ivacaftor combination regimen in people with cystic fibrosis homozygous for the F508del mutation: a double-blind, randomised, phase 3 trial

(2019) Harry G M Heijerman et al.   LANCET

Elexacaftor–Tezacaftor–Ivacaftor for Cystic Fibrosis with a Single Phe508del Allele

(2019) Peter G. Middleton et al.   NEW ENGLAND JOURNAL OF MEDICINE

Quantification of hepatic steatosis with ultrasound: promising role of attenuation imaging coefficient in a biopsy-proven cohort

(2019) Marco Dioguardi Burgio et al.   EUROPEAN RADIOLOGY

Paracrine Fibroblast Growth Factor 1 Functions as Potent Therapeutic Agent for Intrahepatic Cholestasis by Downregulating Synthesis of Bile Acid

(2019) Huan Lin et al.   Frontiers in Pharmacology

Non-invasive evaluation of portal hypertension using shear-wave elastography: analysis of two algorithms combining liver and spleen stiffness in 191 patients with cirrhosis

(2018) L. Elkrief et al.   ALIMENTARY PHARMACOLOGY & THERAPEUTICS

Src kinase inhibition reduces inflammatory and cytoskeletal changes in ΔF508 human cholangiocytes and improves cystic fibrosis transmembrane conductance regulator correctors efficacy

(2018) Romina Fiorotto et al.   HEPATOLOGY

Variceal Hemorrhage and Adverse Liver Outcomes in Patients With Cystic Fibrosis Cirrhosis

(2018) Wen Ye et al.   JOURNAL OF PEDIATRIC GASTROENTEROLOGY AND NUTRITION

Role of vitamin D on gut microbiota in cystic fibrosis

(2018) Mansi Kanhere et al.   JOURNAL OF STEROID BIOCHEMISTRY AND MOLECULAR BIOLOGY

Increased platelet activation occurs in cystic fibrosis patients and correlates to clinical status

(2018) Ulrika Lindberg et al.   THROMBOSIS RESEARCH

The Efficacy of MRI in the diagnostic workup of cystic fibrosis-associated liver disease: A clinical observational cohort study

(2018) Sarah Poetter-Lang et al.   EUROPEAN RADIOLOGY

Cystic Fibrosis Liver Disease: Outcomes and Risk Factors in a Large Cohort of French Patients

(2018) Pierre-Yves Boelle et al.   HEPATOLOGY

Histology of portal vascular changes associated with idiopathic non-cirrhotic portal hypertension: nomenclature and definition

(2018) Maria Guido et al.   HISTOPATHOLOGY

Magnetic resonance elastography demonstrates elevated liver stiffness in cystic fibrosis patients

(2018) Don Hayes et al.   Journal of Cystic Fibrosis

Obliterative Portal Venopathy without Cirrhosis is Prevalent in Pediatric Cystic Fibrosis Liver Disease with Portal Hypertension

(2018) Hao Wu et al.   Clinical Gastroenterology and Hepatology

Modifier genes in cystic fibrosis-related liver disease

(2018) Dominique Debray et al.   CURRENT OPINION IN GASTROENTEROLOGY

Predictive factors for lumacaftor/ivacaftor clinical response

(2018) Alexandra Masson et al.   Journal of Cystic Fibrosis

Effect of NGM282, an FGF19 analogue, in primary sclerosing cholangitis: A multicenter, randomized, double-blind, placebo-controlled phase II trial

(2018) Gideon M. Hirschfield et al.   JOURNAL OF HEPATOLOGY

Clinical expression of cystic fibrosis in a large cohort of Italian siblings

(2018) Vito Terlizzi et al.   BMC Pulmonary Medicine

Adult-onset cystic fibrosis liver disease: Diagnosis and characterization of an underappreciated entity

(2017) Christopher Koh et al.   HEPATOLOGY

Prevalence of elevated liver enzymes in children with cystic fibrosis diagnosed by newborn screen

(2017) Samantha A. Woodruff et al.   Journal of Cystic Fibrosis

Liver disease in cystic fibrosis presents as non-cirrhotic portal hypertension

(2017) Peter Witters et al.   Journal of Cystic Fibrosis

Gallbladder and bile duct disease in Cystic Fibrosis

(2017) David N. Assis et al.   Journal of Cystic Fibrosis

nor Ursodeoxycholic acid improves cholestasis in primary sclerosing cholangitis

(2017) Peter Fickert et al.   JOURNAL OF HEPATOLOGY

Correlation of Transient Elastography With Severity of Cystic Fibrosis–related Liver Disease

(2017) Amal Aqul et al.   JOURNAL OF PEDIATRIC GASTROENTEROLOGY AND NUTRITION

Cystic Fibrosis–related Liver Disease

(2017) Dominique Debray et al.   JOURNAL OF PEDIATRIC GASTROENTEROLOGY AND NUTRITION

Liver transplantation in adult cystic fibrosis: Clinical, imaging, and pathological evidence of obliterative portal venopathy

(2017) Sophie Hillaire et al.   LIVER TRANSPLANTATION

Increased Soluble VCAM-1 and Normal P-Selectin in Cystic Fibrosis: a Cross-Sectional Study

(2017) Jan K. Nowak et al.   LUNG

Tezacaftor–Ivacaftor in Residual-Function Heterozygotes with Cystic Fibrosis

(2017) Steven M. Rowe et al.   NEW ENGLAND JOURNAL OF MEDICINE

Spin-echo Echo-planar Imaging MR Elastography versus Gradient-echo MR Elastography for Assessment of Liver Stiffness in Children and Young Adults Suspected of Having Liver Disease

(2017) Suraj D. Serai et al.   RADIOLOGY

Ultrasound Elastography: Review of Techniques and Clinical Applications

(2017) Rosa M.S. Sigrist et al.   Theranostics

Efficacy and safety of lumacaftor and ivacaftor in patients aged 6–11 years with cystic fibrosis homozygous for F508del-CFTR : a randomised, placebo-controlled phase 3 trial

(2017) Felix Ratjen et al.   Lancet Respiratory Medicine

Assessment of pathologic increase in liver stiffness enables earlier diagnosis of CFLD: Results from a prospective longitudinal cohort study

(2017) Victoria Klotter et al.   PLoS One

Features of Severe Liver Disease With Portal Hypertension in Patients With Cystic Fibrosis

(2016) Jaclyn R. Stonebraker et al.   Clinical Gastroenterology and Hepatology

The cystic fibrosis transmembrane conductance regulator controls biliary epithelial inflammation and permeability by regulating Src tyrosine kinase activity

(2016) Romina Fiorotto et al.   HEPATOLOGY

A Placebo-Controlled Trial of Obeticholic Acid in Primary Biliary Cholangitis

(2016) Frederik Nevens et al.   NEW ENGLAND JOURNAL OF MEDICINE

Diagnosing native liver fibrosis and esophageal varices using liver and spleen stiffness measurements in biliary atresia: a pilot study

(2016) Hirofumi Tomita et al.   PEDIATRIC RADIOLOGY

Spleen Size Is Significantly Influenced by Body Height and Sex: Establishment of Normal Values for Spleen Size at US with a Cohort of 1200 Healthy Individuals

(2016) Kai Uwe Chow et al.   RADIOLOGY

Longitudinal Transient Elastography Measurements Used in Follow-up for Patients with Cystic Fibrosis

(2016) Stephanie Van Biervliet et al.   ULTRASOUND IN MEDICINE AND BIOLOGY

Down-regulation of IL-8 by high-dose vitamin D is specific to hyperinflammatory macrophages and involves mechanisms beyond up-regulation ofDUSP1

(2015) N Dauletbaev et al.   BRITISH JOURNAL OF PHARMACOLOGY

Stimulation of nuclear receptor peroxisome proliferator-activated receptor-γ limits NF-κB-dependent inflammation in mouse cystic fibrosis biliary epithelium

(2015) Roberto Scirpo et al.   HEPATOLOGY

Expanding consensus in portal hypertension

(2015) Roberto de Franchis   JOURNAL OF HEPATOLOGY

Increase of Serum Gamma Glutamyltransferase (GGT) Associated With the Development of Cirrhotic Cystic Fibrosis Liver Disease

(2015) Frank A.J.A. Bodewes et al.   JOURNAL OF PEDIATRIC GASTROENTEROLOGY AND NUTRITION

Baseline Ultrasound and Clinical Correlates in Children with Cystic Fibrosis

(2015) Daniel H. Leung et al.   JOURNAL OF PEDIATRICS

Lumacaftor–Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del CFTR

(2015) Claire E. Wainwright et al.   NEW ENGLAND JOURNAL OF MEDICINE

Hepatic and Splenic Acoustic Radiation Force Impulse Shear Wave Velocity Elastography in Children with Liver Disease Associated with Cystic Fibrosis

(2015) Teresa Cañas et al.   Biomed Research International

Noninvasive Methods, including Transient Elastography, for the Detection of Liver Disease in Adults with Cystic Fibrosis

(2015) Matthew D Sadler et al.   Canadian Journal of Gastroenterology and Hepatology

Intestinal Lesions Are Associated with Altered Intestinal Microbiome and Are More Frequent in Children and Young Adults with Cystic Fibrosis and Cirrhosis

(2015) Thomas Flass et al.   PLoS One

The clinical significance of the gut microbiota in cystic fibrosis and the potential for dietary therapies

(2014) Li Li et al.   CLINICAL NUTRITION

A Vitamin D Receptor/SMAD Genomic Circuit Gates Hepatic Fibrotic Response

(2013) Ning Ding et al.   CELL

Disrupted tight junctions in the small intestine of cystic fibrosis mice

(2013) Robert C. De Lisle   CELL AND TISSUE RESEARCH

Evidence of Vascular Endothelial Dysfunction in Young Patients With Cystic Fibrosis

(2013) Spencer Poore et al.   CHEST

Non-invasive measurement of liver and pancreas fibrosis in patients with cystic fibrosis

(2013) Mireen Friedrich-Rust et al.   Journal of Cystic Fibrosis

Magnetic resonance elastography of liver: Technique, analysis, and clinical applications

(2013) Sudhakar K. Venkatesh et al.   JOURNAL OF MAGNETIC RESONANCE IMAGING

Utility of transient elastography in the non-invasive evaluation of cystic fibrosis liver disease

(2013) Matthew T. Kitson et al.   LIVER INTERNATIONAL

Nonalcoholic Fatty Liver Disease: MR Imaging of Liver Proton Density Fat Fraction to Assess Hepatic Steatosis

(2013) An Tang et al.   RADIOLOGY

Increased arterial stiffness in children with cystic fibrosis: Table 1–

(2012) Tobias Buehler et al.   EUROPEAN RESPIRATORY JOURNAL

Gut dysbiosis in cystic fibrosis

(2012) Pauline D. Scanlan et al.   Journal of Cystic Fibrosis

Cancer Risk in Cystic Fibrosis: A 20-Year Nationwide Study From the United States

(2012) Patrick Maisonneuve et al.   JNCI-Journal of the National Cancer Institute

A PPARγ–FGF1 axis is required for adaptive adipose remodelling and metabolic homeostasis

(2012) Johan W. Jonker et al.   NATURE

Performance of Transient Elastography for the Staging of Liver Fibrosis in Patients with Chronic Hepatitis B: A Meta-Analysis

(2012) Young Eun Chon et al.   PLoS One

Non-Invasive Evaluation of Cystic Fibrosis Related Liver Disease in Adults with ARFI, Transient Elastography and Different Fibrosis Scores

(2012) Thomas Karlas et al.   PLoS One

Obliterative Portal Venopathy: Findings at CT Imaging

(2012) Anne-Sophie Glatard et al.   RADIOLOGY

Liver disease in cystic fibrosis

(2011) Marion Rowland et al.   CURRENT OPINION IN PULMONARY MEDICINE

Loss of CFTR Affects Biliary Epithelium Innate Immunity and Causes TLR4–NF-κB—Mediated Inflammatory Response in Mice

(2011) Romina Fiorotto et al.   GASTROENTEROLOGY

Vitamin D receptor agonists inhibit pro-inflammatory cytokine production from the respiratory epithelium in cystic fibrosis

(2011) P. McNally et al.   Journal of Cystic Fibrosis

Transient elastography and portal hypertension in pediatric patients with cystic fibrosis

(2011) A.C. Malbrunot-Wagner et al.   Journal of Cystic Fibrosis

Best practice guidance for the diagnosis and management of cystic fibrosis-associated liver disease

(2011) Dominique Debray et al.   Journal of Cystic Fibrosis

Liver disease in adult patients with cystic fibrosis: A frequent and independent prognostic factor associated with death or lung transplantation

(2011) Ariane Chryssostalis et al.   JOURNAL OF HEPATOLOGY

Transcriptional Basis for Hepatic Fibrosis in Cystic Fibrosis–associated Liver Disease

(2011) Tamara N. Pereira et al.   JOURNAL OF PEDIATRIC GASTROENTEROLOGY AND NUTRITION

Secondary causes of nonalcoholic fatty liver disease

(2011) Jacob M. Kneeman et al.   Therapeutic Advances in Gastroenterology

Cystic fibrosis transmembrane conductance regulator (CFTR) expression in human platelets: impact on mediators and mechanisms of the inflammatory response

(2010) Domenico Mattoscio et al.   FASEB JOURNAL

Importance of hepatic fibrosis in cystic fibrosis and the predictive value of liver biopsy

(2010) Peter J. Lewindon et al.   HEPATOLOGY

The biliary HCO3− umbrella: A unifying hypothesis on pathogenetic and therapeutic aspects of fibrosing cholangiopathies

(2010) Ulrich Beuers et al.   HEPATOLOGY

Evidence of Intestinal Inflammation in Patients With Cystic Fibrosis

(2010) Steven L Werlin et al.   JOURNAL OF PEDIATRIC GASTROENTEROLOGY AND NUTRITION

Liver transplantation in patients with cystic fibrosis: Analysis of united network for organ sharing data

(2010) Manuel Mendizabal et al.   LIVER TRANSPLANTATION

Pharmacological correction of a defect in PPAR-γ signaling ameliorates disease severity in Cftr-deficient mice

(2010) Gregory S Harmon et al.   NATURE MEDICINE

Inflammatory cytokines in vascular dysfunction and vascular disease

(2009) Alexander H. Sprague et al.   BIOCHEMICAL PHARMACOLOGY

Genetic Modifiers of Liver Disease in Cystic Fibrosis

(2009) Jaclyn R. Bartlett   JAMA-JOURNAL OF THE AMERICAN MEDICAL ASSOCIATION

Comparison of ultrasound and biopsy findings in children with cystic fibrosis related liver disease

(2007) Peter R. Mueller-Abt et al.   Journal of Cystic Fibrosis