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

Hepatic manifestations of cystic fibrosis

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

A longitudinal assessment of non-invasive biomarkers to diagnose and predict cystic fibrosis-associated liver disease

(2020) Wikrom Karnsakul et al.   Journal of Cystic Fibrosis

Reduced Intestinal Inflammation with Lumacaftor/Ivacaftor in Adolescents with Cystic Fibrosis

(2020) Candice Tétard et al.   JOURNAL OF PEDIATRIC GASTROENTEROLOGY AND NUTRITION

Safety, pharmacokinetics, and pharmacodynamics of lumacaftor and ivacaftor combination therapy in children aged 2–5 years with cystic fibrosis homozygous for F508del-CFTR: an open-label phase 3 study

(2019) John J McNamara et al.   Lancet Respiratory Medicine

New pediatric percentiles of liver enzyme serum levels (alanine aminotransferase, aspartate aminotransferase, γ-glutamyltransferase): Effects of age, sex, body mass index, and pubertal stage

(2018) Sarah Bussler et al.   HEPATOLOGY

MR Spectroscopy–derived Proton Density Fat Fraction Is Superior to Controlled Attenuation Parameter for Detecting and Grading Hepatic Steatosis

(2018) Jurgen Henk Runge et al.   RADIOLOGY

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

(2018) Pierre-Yves Boelle et al.   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

Cystic Fibrosis–related Liver Disease

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Optimized LC-MS/MS Method for the High-throughput Analysis of Clinical Samples of Ivacaftor, Its Major Metabolites, and Lumacaftor in Biological Fluids of Cystic Fibrosis Patients

(2017) Elena K. Schneider et al.   Jove-Journal of Visualized Experiments

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

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Diagnosis of cirrhosis and portal hypertension: imaging, non-invasive markers of fibrosis and liver biopsy

(2017) Bogdan Procopet et al.   Gastroenterology Report

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

(2016) Romina Fiorotto et al.   HEPATOLOGY

Development of HPLC and LC–MS/MS methods for the analysis of ivacaftor, its major metabolites and lumacaftor in plasma and sputum of cystic fibrosis patients treated with ORKAMBI or KALYDECO

(2016) Elena K. Schneider et al.   JOURNAL OF CHROMATOGRAPHY B-ANALYTICAL TECHNOLOGIES IN THE BIOMEDICAL AND LIFE SCIENCES

Aspartate aminotransferase to platelet ratio and fibrosis-4 as biomarkers in biopsy-validated pediatric cystic fibrosis liver disease

(2015) Daniel H. Leung et al.   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

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

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

Performance of the aspartate aminotransferase-to-platelet ratio index for the staging of hepatitis C-related fibrosis: An updated meta-analysis

(2010) Zhong-Hua Lin et al.   HEPATOLOGY