Current Treatment Options for Cystic Fibrosis-Related Liver Disease

Overview

Journal Int J Mol Sci

Publisher MDPI

Specialties Biochemistry
Chemistry
Molecular Biology

Date 2020 Nov 18

PMID 33202578

Citations 11

Authors

Katharina Staufer

Affiliations

Soon will be listed here.

Abstract

Cystic Fibrosis-related liver disease (CFLD) has become a leading cause of morbidity and mortality in patients with Cystic Fibrosis (CF), and affects children and adults. The understanding of the pathogenesis of CFLD is key in order to develop efficacious treatments. However, it remains complex, and has not been clarified to the last. The search for a drug might be additionally complicated due to the diverse clinical picture and lack of a unified definition of CFLD. Although ursodeoxycholic acid has been used for decades, its efficacy in CFLD is controversial, and the potential of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) modulators and targeted gene therapy in CFLD needs to be defined in the near future. This review focuses on the current knowledge on treatment strategies for CFLD based on pathomechanistic viewpoints.

Citing Articles

Diagnosis, Management, and Prognosis of Cystic Fibrosis-Related Liver Disease in Children.

Anton-Paduraru D, Azoicai A, Trofin F, Murgu A, Mindru D, Bocec A Diagnostics (Basel). 2024; 14(5).

PMID: 38473009 PMC: 10930689. DOI: 10.3390/diagnostics14050538.

Sotagliflozin attenuates liver-associated disorders in cystic fibrosis rabbits.

Liang X, Hou X, Bouhamdan M, Sun Y, Song Z, Rajagopalan C JCI Insight. 2024; 9(6).

PMID: 38358827 PMC: 10972622. DOI: 10.1172/jci.insight.165826.

Liver magnetic resonance elastography and fat fraction in pediatric patients with cystic fibrosis versus healthy children.

Tan A, Demirtas D, Hizarcioglu-Gulsen H, Karakaya J, Isiyel E, Ozen H Pediatr Radiol. 2023; 54(2):250-259.

PMID: 38133654 DOI: 10.1007/s00247-023-05832-1.

Evolving Nutritional Needs in Cystic Fibrosis.

Frantzen T, Barsky S, LaVecchia G, Marowitz M, Wang J Life (Basel). 2023; 13(7).

PMID: 37511806 PMC: 10381916. DOI: 10.3390/life13071431.

Cystic fibrosis rabbits develop spontaneous hepatobiliary lesions and CF-associated liver disease (CFLD)-like phenotypes.

Wu Q, Liang X, Hou X, Song Z, Bouhamdan M, Qiu Y PNAS Nexus. 2023; 2(1):pgac306.

PMID: 36712930 PMC: 9832953. DOI: 10.1093/pnasnexus/pgac306.

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