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

J Dana, D Debray, A Beaufrère, S Hillaire, M Fabre… - Journal of …, 2022 - Elsevier
Cystic fibrosis (CF) is the most common autosomal recessive disease in the Caucasian
population. Cystic fibrosis-related liver disease (CFLD) is defined as the pathogenesis …

[HTML][HTML] PROMISE: working with the CF community to understand emerging clinical and research needs for those treated with highly effective CFTR modulator therapy

DP Nichols, SH Donaldson, CA Frederick… - Journal of Cystic …, 2021 - Elsevier
Highly effective CFTR modulator drug therapy is increasingly available to those with cystic
fibrosis. Multiple observational research studies are now being conducted to better …

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

MB Toledano, SK Mukherjee, J Howell, D Westaby… - PLoS …, 2019 - journals.plos.org
Objective Cystic fibrosis associated liver disease (CFLD) is the third largest cause of
mortality in CF. Our aim was to define the burden of CFLD in the UK using national registry …

Cystic fibrosis screening, evaluation, and management of hepatobiliary disease consensus recommendations

ZM Sellers, DN Assis, SM Paranjape, M Sathe… - Hepatology, 2024 - journals.lww.com
Cystic fibrosis (CF) may cause a spectrum of hepatobiliary complications, including portal
hypertension, multilobular cirrhosis, and liver failure. Current guidelines on the detection …

[HTML][HTML] Cystic fibrosis liver disease in the new era of cystic fibrosis transmembrane conductance regulator (CFTR) modulators

JA Eldredge, MR Oliver, CY Ooi - Paediatric Respiratory Reviews, 2024 - Elsevier
Cystic fibrosis liver disease (CFLD) is characterised by a wide heterogenity of manifestations
and severity. It represents a major cause of morbidity in people with cystic fibrosis (PwCF) …

Multisystemic effects of elexacaftor–tezacaftor–ivacaftor in adults with cystic fibrosis and advanced lung disease

PR Burgel, JL Paillasseur, I Durieu… - Annals of the …, 2024 - atsjournals.org
Rationale: Limited data exist on the safety and effectiveness of elexacaftor-tezacaftor-
ivacaftor (ETI) in people with cystic fibrosis (pwCF) and advanced lung disease. Objectives …

[HTML][HTML] Pathophysiology of cystic fibrosis liver disease: a channelopathy leading to alterations in innate immunity and in microbiota

R Fiorotto, M Strazzabosco - Cellular and Molecular Gastroenterology and …, 2019 - Elsevier
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 …

Modifier factors of cystic fibrosis phenotypes: a focus on modifier genes

J Mésinèle, M Ruffin, L Guillot, H Corvol - International Journal of …, 2022 - mdpi.com
Although cystic fibrosis (CF) is recognized as a monogenic disease, due to variants within
the CFTR (Cystic Fibrosis Transmembrane Regulator) gene, an extreme clinical …

Liver fibrosis in children: a comprehensive review of mechanisms, diagnosis, and therapy

E Ozdogan, C Arikan - Clinical and Experimental Pediatrics, 2022 - pmc.ncbi.nlm.nih.gov
Chronic liver disease incidence is increasing among children worldwide due to a multitude
of epidemiological changes. Most of these chronic insults to the pediatric liver progress to …

[HTML][HTML] Guidelines for the management of cholestatic liver diseases (2021)

L Lu, Chinese Medical Association - Journal of Clinical and …, 2022 - ncbi.nlm.nih.gov
Abstract In 2015, the Chinese Society of Hepatology and the Chinese Society of
Gastroenterology issued a consensus statement on the diagnosis and management of …