دورية أكاديمية
أعرض في EDS

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

التفاصيل البيبلوغرافية
العنوان: Cystic fibrosis liver disease in the new era of cystic fibrosis transmembrane conductance regulator (CFTR) modulators.
المؤلفون: Eldredge JA; Department of Gastroenterology and Clinical Nutrition, Royal Children's Hospital, Melbourne, Australia. Electronic address: jessica.eldredge@rch.org.au., Oliver MR; Department of Gastroenterology and Clinical Nutrition, Royal Children's Hospital, Melbourne, Australia; Department of Paediatrics, Faculty of Medicine, The University of Melbourne, Melbourne, Australia. Electronic address: mark.oliver@rch.org.au., Ooi CY; Department of Gastroenterology, Sydney Children's Hospital Randwick, NSW, Australia; School of Clinical Medicine, Discipline of Paediatrics and Child Health, UNSW Medicine & Health, University of New South Wales, Sydney, Australia. Electronic address: keith.ooi@unsw.edu.au.
المصدر: Paediatric respiratory reviews [Paediatr Respir Rev] 2024 Jun; Vol. 50, pp. 54-61. Date of Electronic Publication: 2023 Dec 28.
نوع المنشور: Journal Article; Review
اللغة: English
بيانات الدورية: Publisher: W.B. Saunders Country of Publication: England NLM ID: 100898941 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1526-0550 (Electronic) Linking ISSN: 15260542 NLM ISO Abbreviation: Paediatr Respir Rev Subsets: MEDLINE
أسماء مطبوعة: Original Publication: London : W.B. Saunders, c2000-
مواضيع طبية MeSH: Cystic Fibrosis*/drug therapy , Cystic Fibrosis*/complications , Cystic Fibrosis*/physiopathology , Cystic Fibrosis Transmembrane Conductance Regulator*/genetics , Cystic Fibrosis Transmembrane Conductance Regulator*/metabolism , Liver Diseases*/metabolism , Liver Diseases*/etiology, Humans ; Liver Transplantation ; Benzodioxoles/therapeutic use ; Aminophenols/therapeutic use ; Quinolones/therapeutic use ; Aminopyridines/therapeutic use ; Pyrazoles/therapeutic use
مستخلص: 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), which will be of increasing relevance as survival increases in the new era of cystic fibrosis care. No medical therapy currently available has evidence to treat or prevent progression of liver disease. Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) modulators may be transformative on pulmonary, nutritional and quality of life, but direct effect on long term liver disease outcomes is not yet established. Drug-associated hepatic adverse effects may be common, and clinician familiarity with drug-monitoring recommendations is essential. Longitudinal studies are required to understand the effect of CFTR modulators on the incidence and natural history of CFLD, including with early treatment initiation, in established advanced liver disease, and post liver transplantation.
Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.
(Copyright © 2023 The Authors. Published by Elsevier Ltd.. All rights reserved.)
فهرسة مساهمة: Keywords: Cirrhosis; Cystic fibrosis liver disease; Cystic fibrosis transmembrane conductance regulator modulators; Portal hypertension
المشرفين على المادة: 126880-72-6 (Cystic Fibrosis Transmembrane Conductance Regulator)
0 (Benzodioxoles)
0 (Aminophenols)
0 (Quinolones)
0 (Aminopyridines)
0 (CFTR protein, human)
0 (Pyrazoles)
1Y740ILL1Z (ivacaftor)
تواريخ الأحداث: Date Created: 20240128 Date Completed: 20240529 Latest Revision: 20240702
رمز التحديث: 20240703
DOI: 10.1016/j.prrv.2023.12.005
PMID: 38281822
قاعدة البيانات: MEDLINE
الوصف
تدمد:1526-0550
DOI:10.1016/j.prrv.2023.12.005