{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,2]],"date-time":"2026-07-02T22:51:02Z","timestamp":1783032662637,"version":"3.54.6"},"reference-count":98,"publisher":"Elsevier BV","license":[{"start":{"date-parts":[[2024,6,1]],"date-time":"2024-06-01T00:00:00Z","timestamp":1717200000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2024,6,1]],"date-time":"2024-06-01T00:00:00Z","timestamp":1717200000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2023,12,28]],"date-time":"2023-12-28T00:00:00Z","timestamp":1703721600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100000925","name":"National Health and Medical Research Council","doi-asserted-by":"publisher","award":["2020\/GNT1194358"],"award-info":[{"award-number":["2020\/GNT1194358"]}],"id":[{"id":"10.13039\/501100000925","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["clinicalkey.fr","clinicalkey.jp","clinicalkey.es","clinicalkey.com.au","clinicalkey.com","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Paediatric Respiratory Reviews"],"published-print":{"date-parts":[[2024,6]]},"DOI":"10.1016\/j.prrv.2023.12.005","type":"journal-article","created":{"date-parts":[[2023,12,28]],"date-time":"2023-12-28T05:33:48Z","timestamp":1703741628000},"page":"54-61","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":12,"special_numbering":"C","title":["Cystic fibrosis liver disease in the new era of cystic fibrosis transmembrane conductance regulator (CFTR) modulators"],"prefix":"10.1016","volume":"50","author":[{"given":"Jessica A.","family":"Eldredge","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mark R.","family":"Oliver","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chee Y.","family":"Ooi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"issue":"3","key":"10.1016\/j.prrv.2023.12.005_b0005","doi-asserted-by":"crossref","first-page":"175","DOI":"10.1038\/nrgastro.2015.226","article-title":"Cystic fibrosis from the gastroenterologist's perspective","volume":"13","author":"Ooi","year":"2016","journal-title":"Nat Rev Gastroenterol Hepatol"},{"issue":"19","key":"10.1016\/j.prrv.2023.12.005_b0010","doi-asserted-by":"crossref","first-page":"1992","DOI":"10.1056\/NEJMra043184","article-title":"Cystic fibrosis","volume":"352","author":"Rowe","year":"2005","journal-title":"N Engl J Med"},{"issue":"6824","key":"10.1016\/j.prrv.2023.12.005_b0015","doi-asserted-by":"crossref","first-page":"94","DOI":"10.1038\/35065099","article-title":"Aberrant CFTR-dependent HCO3- transport in mutations associated with cystic fibrosis","volume":"410","author":"Choi","year":"2001","journal-title":"Nature"},{"issue":"2","key":"10.1016\/j.prrv.2023.12.005_b0020","doi-asserted-by":"crossref","first-page":"218","DOI":"10.1016\/j.jcf.2017.11.019","article-title":"Up-to-date and projected estimates of survival for people with cystic fibrosis using baseline characteristics: A longitudinal study using UK patient registry data","volume":"17","author":"Keogh","year":"2018","journal-title":"J Cyst Fibros"},{"issue":"6","key":"10.1016\/j.prrv.2023.12.005_b0025","doi-asserted-by":"crossref","DOI":"10.3390\/genes11060589","article-title":"The Changing Epidemiology of Cystic Fibrosis: Incidence, Survival and Impact of the CFTR Gene Discovery","volume":"11","author":"Scotet","year":"2020","journal-title":"Genes (Basel)"},{"issue":"3","key":"10.1016\/j.prrv.2023.12.005_b0030","doi-asserted-by":"crossref","first-page":"670","DOI":"10.1183\/09031936.00119714","article-title":"A contemporary survival analysis of individuals with cystic fibrosis: a cohort study","volume":"45","author":"Stephenson","year":"2015","journal-title":"Eur Respir J"},{"issue":"4","key":"10.1016\/j.prrv.2023.12.005_b0035","doi-asserted-by":"crossref","first-page":"1648","DOI":"10.1002\/hep.30148","article-title":"Cystic Fibrosis Liver Disease: Outcomes and Risk Factors in a Large Cohort of French Patients","volume":"69","author":"Bo\u00eblle","year":"2019","journal-title":"Hepatology"},{"key":"10.1016\/j.prrv.2023.12.005_b0040","unstructured":"Cystic fibrosis Foundation 2021 Patient Registry: Annual Data Report. [Available from: https:\/\/www.cff.org\/sites\/default\/files\/2021-11\/Patient-Registry-Annual-Data-Report.pdf."},{"issue":"3","key":"10.1016\/j.prrv.2023.12.005_b0045","doi-asserted-by":"crossref","first-page":"455","DOI":"10.1016\/j.jcf.2019.09.016","article-title":"Occurrence, outcomes and predictors of portal hypertension in cystic fibrosis: A longitudinal prospective birth cohort study","volume":"19","author":"Cipolli","year":"2020","journal-title":"J Cyst Fibros"},{"issue":"10","key":"10.1016\/j.prrv.2023.12.005_b0050","doi-asserted-by":"crossref","first-page":"1076","DOI":"10.1001\/jama.2009.1295","article-title":"Genetic modifiers of liver disease in cystic fibrosis","volume":"302","author":"Bartlett","year":"2009","journal-title":"JAMA"},{"issue":"2","key":"10.1016\/j.prrv.2023.12.005_b0055","doi-asserted-by":"crossref","first-page":"116","DOI":"10.1016\/j.jcf.2012.11.010","article-title":"Cirrhosis and other liver disease in cystic fibrosis","volume":"12","author":"Flass","year":"2013","journal-title":"J Cyst Fibros"},{"issue":"6","key":"10.1016\/j.prrv.2023.12.005_b0060","doi-asserted-by":"crossref","first-page":"1374","DOI":"10.1002\/hep.1840360613","article-title":"Liver disease in cystic fibrosis: A prospective study on incidence, risk factors, and outcome","volume":"36","author":"Colombo","year":"2002","journal-title":"Hepatology"},{"key":"10.1016\/j.prrv.2023.12.005_b0065","doi-asserted-by":"crossref","unstructured":"Stonebraker JR, Ooi CY, Pace RG, Corvol H, Knowles MR, Durie PR, et al. Features of Severe Liver Disease With Portal Hypertension inPatients With Cystic Fibrosis. Clin Gastroenterol Hepatol. 2016; 14 (8): 1207-15.e3.","DOI":"10.1016\/j.cgh.2016.03.041"},{"issue":"1","key":"10.1016\/j.prrv.2023.12.005_b0070","doi-asserted-by":"crossref","first-page":"122","DOI":"10.1097\/MPG.0000000000001728","article-title":"Variceal Hemorrhage and Adverse Liver Outcomes in Patients With Cystic Fibrosis Cirrhosis","volume":"66","author":"Ye","year":"2018","journal-title":"J Pediatr Gastroenterol Nutr"},{"issue":"6","key":"10.1016\/j.prrv.2023.12.005_b0075","doi-asserted-by":"crossref","first-page":"796","DOI":"10.1097\/MPG.0000000000002694","article-title":"Cystic Fibrosis-related Liver Disease is Associated With Increased Disease Burden and Endocrine Comorbidities","volume":"70","author":"Singh","year":"2020","journal-title":"J Pediatr Gastroenterol Nutr"},{"issue":"Suppl 2","key":"10.1016\/j.prrv.2023.12.005_b0080","doi-asserted-by":"crossref","first-page":"S29","DOI":"10.1016\/S1569-1993(11)60006-4","article-title":"Best practice guidance for the diagnosis and management of cystic fibrosis-associated liver disease","volume":"10","author":"Debray","year":"2011","journal-title":"J Cyst Fibros"},{"key":"10.1016\/j.prrv.2023.12.005_b0085","article-title":"Cystic fibrosis screening, evaluation and management of hepatobiliary disease consensus recommendations","author":"Sellers","year":"2023","journal-title":"Hepatology"},{"issue":"1","key":"10.1016\/j.prrv.2023.12.005_b0090","doi-asserted-by":"crossref","first-page":"193","DOI":"10.1002\/hep.24014","article-title":"Importance of hepatic fibrosis in cystic fibrosis and the predictive value of liver biopsy","volume":"53","author":"Lewindon","year":"2011","journal-title":"Hepatology"},{"issue":"2","key":"10.1016\/j.prrv.2023.12.005_b0095","doi-asserted-by":"crossref","first-page":"e0116967","DOI":"10.1371\/journal.pone.0116967","article-title":"Intestinal lesions are associated with altered intestinal microbiome and are more frequent in children and young adults with cystic fibrosis and cirrhosis","volume":"10","author":"Flass","year":"2015","journal-title":"PLoS One"},{"issue":"Pt 2","key":"10.1016\/j.prrv.2023.12.005_b0100","first-page":"357","article-title":"Cystic fibrosis in adults: an autopsy study","volume":"14","author":"Vawter","year":"1979","journal-title":"Pathol Annu"},{"issue":"3","key":"10.1016\/j.prrv.2023.12.005_b0105","doi-asserted-by":"crossref","first-page":"1064","DOI":"10.1002\/hep.24183","article-title":"Noncirrhotic presinusoidal portal hypertension is common in cystic fibrosis-associated liver disease","volume":"53","author":"Witters","year":"2011","journal-title":"Hepatology"},{"issue":"10","key":"10.1016\/j.prrv.2023.12.005_b0110","doi-asserted-by":"crossref","first-page":"2134","DOI":"10.1016\/j.cgh.2018.10.046","article-title":"Obliterative Portal Venopathy Without Cirrhosis Is Prevalent in Pediatric Cystic Fibrosis Liver Disease With Portal Hypertension","volume":"17","author":"Wu","year":"2019","journal-title":"Clin Gastroenterol Hepatol"},{"issue":"10","key":"10.1016\/j.prrv.2023.12.005_b0115","doi-asserted-by":"crossref","first-page":"1342","DOI":"10.1002\/lt.24842","article-title":"Liver transplantation in adult cystic fibrosis: Clinical, imaging, and pathological evidence of obliterative portal venopathy","volume":"23","author":"Hillaire","year":"2017","journal-title":"Liver Transpl"},{"issue":"3","key":"10.1016\/j.prrv.2023.12.005_b0120","doi-asserted-by":"crossref","first-page":"286","DOI":"10.1007\/s00439-003-0963-z","article-title":"Increased prevalence of CFTR mutations and variants and decreased chloride secretion in primary sclerosing cholangitis","volume":"113","author":"Sheth","year":"2003","journal-title":"Hum Genet"},{"issue":"Suppl 2","key":"10.1016\/j.prrv.2023.12.005_b0125","doi-asserted-by":"crossref","first-page":"S62","DOI":"10.1016\/j.jcf.2017.07.006","article-title":"Gallbladder and bile duct disease in Cystic Fibrosis","volume":"16","author":"Assis","year":"2017","journal-title":"J Cyst Fibros"},{"issue":"2","key":"10.1016\/j.prrv.2023.12.005_b0130","doi-asserted-by":"crossref","first-page":"122","DOI":"10.1093\/jnci\/djs481","article-title":"Cancer risk in cystic fibrosis: a 20-year nationwide study from the United States","volume":"105","author":"Maisonneuve","year":"2013","journal-title":"J Natl Cancer Inst"},{"issue":"8","key":"10.1016\/j.prrv.2023.12.005_b0135","doi-asserted-by":"crossref","first-page":"1153","DOI":"10.1136\/gut.2004.062786","article-title":"Patterns of GI disease in adulthood associated with mutations in the CFTR gene","volume":"56","author":"Wilschanski","year":"2007","journal-title":"Gut"},{"issue":"2","key":"10.1016\/j.prrv.2023.12.005_b0140","doi-asserted-by":"crossref","first-page":"88","DOI":"10.1097\/MOG.0000000000000508","article-title":"Modifier genes in cystic fibrosis-related liver disease","volume":"35","author":"Debray","year":"2019","journal-title":"Curr Opin Gastroenterol"},{"issue":"5","key":"10.1016\/j.prrv.2023.12.005_b0145","doi-asserted-by":"crossref","first-page":"1705","DOI":"10.1016\/S0002-9440(10)61117-0","article-title":"The role of hepatic stellate cells and transforming growth factor-beta(1) in cystic fibrosis liver disease","volume":"160","author":"Lewindon","year":"2002","journal-title":"Am J Pathol"},{"issue":"2","key":"10.1016\/j.prrv.2023.12.005_b0150","doi-asserted-by":"crossref","first-page":"533","DOI":"10.1002\/hep.22637","article-title":"Fibrogenesis in pediatric cholestatic liver disease: role of taurocholate and hepatocyte-derived monocyte chemotaxis protein-1 in hepatic stellate cell recruitment","volume":"49","author":"Ramm","year":"2009","journal-title":"Hepatology"},{"issue":"2","key":"10.1016\/j.prrv.2023.12.005_b0155","doi-asserted-by":"crossref","first-page":"420","DOI":"10.1016\/j.jhep.2021.09.042","article-title":"Cystic fibrosis-related liver disease: Clinical presentations, diagnostic and monitoring approaches in the era of CFTR modulator therapies","volume":"76","author":"Dana","year":"2022","journal-title":"J Hepatol"},{"issue":"5","key":"10.1016\/j.prrv.2023.12.005_b0160","doi-asserted-by":"crossref","first-page":"1797","DOI":"10.1007\/s10620-022-07812-1","article-title":"Cystic Fibrosis-Related Gut Dysbiosis: A Systematic Review","volume":"68","author":"Caley","year":"2023","journal-title":"Dig Dis Sci"},{"issue":"1","key":"10.1016\/j.prrv.2023.12.005_b0165","doi-asserted-by":"crossref","first-page":"2156254","DOI":"10.1080\/19490976.2022.2156254","article-title":"Diet and the gut-lung axis in cystic fibrosis - direct & indirect links","volume":"15","author":"McKay","year":"2023","journal-title":"Gut Microbes"},{"issue":"1","key":"10.1016\/j.prrv.2023.12.005_b0170","doi-asserted-by":"crossref","first-page":"18593","DOI":"10.1038\/s41598-019-55028-7","article-title":"Gut Microbiota in Children With Cystic Fibrosis: A Taxonomic and Functional Dysbiosis","volume":"9","author":"Coffey","year":"2019","journal-title":"Sci Rep"},{"key":"10.1016\/j.prrv.2023.12.005_b0175","doi-asserted-by":"crossref","first-page":"24857","DOI":"10.1038\/srep24857","article-title":"Disrupted progression of the intestinal microbiota with age in children with cystic fibrosis","volume":"6","author":"Nielsen","year":"2016","journal-title":"Sci Rep"},{"issue":"3","key":"10.1016\/j.prrv.2023.12.005_b0180","doi-asserted-by":"crossref","DOI":"10.3390\/jcm11030649","article-title":"Intestinal Inflammation and Alterations in the Gut Microbiota in Cystic Fibrosis: A Review of the Current Evidence, Pathophysiology and Future Directions","volume":"11","author":"Tam","year":"2022","journal-title":"J Clin Med"},{"issue":"2","key":"10.1016\/j.prrv.2023.12.005_b0185","doi-asserted-by":"crossref","first-page":"6","DOI":"10.1007\/s11894-017-0546-0","article-title":"The Enigmatic Gut in Cystic Fibrosis: Linking Inflammation, Dysbiosis, and the Increased Risk of Malignancy","volume":"19","author":"Garg","year":"2017","journal-title":"Curr Gastroenterol Rep"},{"issue":"2","key":"10.1016\/j.prrv.2023.12.005_b0190","doi-asserted-by":"crossref","first-page":"197","DOI":"10.1016\/j.jcmgh.2019.04.013","article-title":"Pathophysiology of Cystic Fibrosis Liver Disease: A Channelopathy Leading to Alterations in Innate Immunity and in Microbiota","volume":"8","author":"Fiorotto","year":"2019","journal-title":"Cell Mol Gastroenterol Hepatol"},{"key":"10.1016\/j.prrv.2023.12.005_b0195","doi-asserted-by":"crossref","unstructured":"Fiorotto R, Scirpo R, Trauner M, Fabris L, Hoque R, Spirli C, et al. Loss of CFTR affects biliary epithelium innate immunity and causes TLR4-NF-\u03baB-mediated inflammatory response in mice. Gastroenterology. 2011; 141 (4): 1498-508, 508.e1-5.","DOI":"10.1053\/j.gastro.2011.06.052"},{"issue":"12","key":"10.1016\/j.prrv.2023.12.005_b0200","doi-asserted-by":"crossref","first-page":"1533","DOI":"10.1002\/hep4.1266","article-title":"Diet-Induced Dysbiosis and Genetic Background Synergize With Cystic Fibrosis Transmembrane Conductance Regulator Deficiency to Promote Cholangiopathy in Mice","volume":"2","author":"Debray","year":"2018","journal-title":"Hepatol Commun"},{"issue":"1","key":"10.1016\/j.prrv.2023.12.005_b0205","doi-asserted-by":"crossref","first-page":"113","DOI":"10.1097\/MPG.0000000000000758","article-title":"Increase of Serum \u03b3-Glutamyltransferase Associated With Development of Cirrhotic Cystic Fibrosis Liver Disease","volume":"61","author":"Bodewes","year":"2015","journal-title":"J Pediatr Gastroenterol Nutr"},{"issue":"3","key":"10.1016\/j.prrv.2023.12.005_b0210","doi-asserted-by":"crossref","first-page":"351","DOI":"10.1097\/MPG.0000000000002413","article-title":"Liver Ultrasound Patterns in Children With Cystic Fibrosis Correlate With Noninvasive Tests of Liver Disease","volume":"69","author":"Ling","year":"2019","journal-title":"J Pediatr Gastroenterol Nutr"},{"issue":"1","key":"10.1016\/j.prrv.2023.12.005_b0215","doi-asserted-by":"crossref","first-page":"139","DOI":"10.1016\/j.jcf.2016.08.002","article-title":"Prevalence of elevated liver enzymes in children with cystic fibrosis diagnosed by newborn screen","volume":"16","author":"Woodruff","year":"2017","journal-title":"J Cyst Fibros"},{"issue":"6","key":"10.1016\/j.prrv.2023.12.005_b0220","doi-asserted-by":"crossref","first-page":"2301","DOI":"10.1002\/hep.30156","article-title":"MicroRNA Sequencing Identifies a Serum MicroRNA Panel, Which Combined With Aspartate Aminotransferase to Platelet Ratio Index Can Detect and Monitor Liver Disease in Pediatric Cystic Fibrosis","volume":"68","author":"Calvopina","year":"2018","journal-title":"Hepatology"},{"issue":"5","key":"10.1016\/j.prrv.2023.12.005_b0225","doi-asserted-by":"crossref","first-page":"1576","DOI":"10.1002\/hep.28016","article-title":"Aspartate aminotransferase to platelet ratio and fibrosis-4 as biomarkers in biopsy-validated pediatric cystic fibrosis liver disease","volume":"62","author":"Leung","year":"2015","journal-title":"Hepatology"},{"key":"10.1016\/j.prrv.2023.12.005_b0230","doi-asserted-by":"crossref","unstructured":"Lewindon PJ, Puertolas-Lopez MV, Ramm LE, Noble C, Pereira TN, Wixey JA, et al. Accuracy of Transient Elastography Data Combined With APRI in Detection and Staging of Liver Disease in Pediatric Patients With Cystic Fibrosis. Clin Gastroenterol Hepatol. 2019; 17 (12): 2561-9.e5.","DOI":"10.1016\/j.cgh.2019.03.015"},{"issue":"3","key":"10.1016\/j.prrv.2023.12.005_b0235","doi-asserted-by":"crossref","first-page":"449","DOI":"10.1016\/j.jcf.2019.06.017","article-title":"Supersonic shear-wave elastography and APRI for the detection and staging of liver disease in pediatric cystic fibrosis","volume":"19","author":"Calvopina","year":"2020","journal-title":"J Cyst Fibros"},{"key":"10.1016\/j.prrv.2023.12.005_b0240","doi-asserted-by":"crossref","unstructured":"Leung DH, Ye W, Molleston JP, Weymann A, Ling S, Paranjape SM, et al. Baseline Ultrasound and Clinical Correlates in Children with Cystic Fibrosis. J Pediatr. 2015; 167 (4): 862-8.e2.","DOI":"10.1016\/j.jpeds.2015.06.062"},{"issue":"3","key":"10.1016\/j.prrv.2023.12.005_b0245","doi-asserted-by":"crossref","first-page":"215","DOI":"10.1016\/j.jcf.2007.08.001","article-title":"Comparison of ultrasound and biopsy findings in children with cystic fibrosis related liver disease","volume":"7","author":"Mueller-Abt","year":"2008","journal-title":"J Cyst Fibros"},{"issue":"3","key":"10.1016\/j.prrv.2023.12.005_b0250","doi-asserted-by":"crossref","DOI":"10.1016\/j.clinre.2021.101855","article-title":"Comparison of Transient Elastography, ShearWave Elastography, Magnetic Resonance Elastography and FibroTest as routine diagnostic markers for assessing liver fibrosis in children with Cystic Fibrosis","volume":"46","author":"Dana","year":"2022","journal-title":"Clin Res Hepatol Gastroenterol"},{"issue":"6","key":"10.1016\/j.prrv.2023.12.005_b0255","doi-asserted-by":"crossref","first-page":"e54","DOI":"10.1016\/j.jcf.2018.08.010","article-title":"Magnetic resonance elastography demonstrates elevated liver stiffness in cystic fibrosis patients","volume":"17","author":"Hayes","year":"2018","journal-title":"J Cyst Fibros"},{"issue":"3","key":"10.1016\/j.prrv.2023.12.005_b0260","doi-asserted-by":"crossref","first-page":"339","DOI":"10.1016\/j.jcf.2020.06.006","article-title":"The Emergence of Elastography for Cystic Fibrosis Liver Disease","volume":"19","author":"Masand","year":"2020","journal-title":"J Cyst Fibros"},{"issue":"12","key":"10.1016\/j.prrv.2023.12.005_b0265","doi-asserted-by":"crossref","first-page":"6477","DOI":"10.1007\/s00330-019-06331-4","article-title":"Prospective comparison of transient, point shear wave, and magnetic resonance elastography for staging liver fibrosis","volume":"29","author":"Lefebvre","year":"2019","journal-title":"Eur Radiol"},{"issue":"4","key":"10.1016\/j.prrv.2023.12.005_b0270","doi-asserted-by":"crossref","first-page":"640","DOI":"10.1002\/lt.25421","article-title":"A Multidisciplinary Approach to Pretransplant and Posttransplant Management of Cystic Fibrosis-Associated Liver Disease","volume":"25","author":"Freeman","year":"2019","journal-title":"Liver Transpl"},{"issue":"9","key":"10.1016\/j.prrv.2023.12.005_b0275","first-page":"Cd000222","article-title":"Ursodeoxycholic acid for cystic fibrosis-related liver disease","volume":"9","author":"Cheng","year":"2017","journal-title":"Cochrane Database Syst Rev"},{"issue":"2","key":"10.1016\/j.prrv.2023.12.005_b0280","doi-asserted-by":"crossref","first-page":"220","DOI":"10.1016\/j.jcf.2021.03.014","article-title":"Ursodeoxycholic acid and liver disease associated with cystic fibrosis: A multicenter cohort study","volume":"21","author":"Colombo","year":"2022","journal-title":"J Cyst Fibros"},{"issue":"3","key":"10.1016\/j.prrv.2023.12.005_b0285","doi-asserted-by":"crossref","first-page":"808","DOI":"10.1002\/hep.23082","article-title":"High-dose ursodeoxycholic acid for the treatment of primary sclerosing cholangitis","volume":"50","author":"Lindor","year":"2009","journal-title":"Hepatology"},{"issue":"3","key":"10.1016\/j.prrv.2023.12.005_b0290","first-page":"Cd012056","article-title":"Interventions for preventing and managing advanced liver disease in cystic fibrosis","volume":"3","author":"Palaniappan","year":"2020","journal-title":"Cochrane Database Syst Rev"},{"issue":"3","key":"10.1016\/j.prrv.2023.12.005_b0295","doi-asserted-by":"crossref","first-page":"243","DOI":"10.1002\/lt.22240","article-title":"Liver transplantation in patients with cystic fibrosis: analysis of United Network for Organ Sharing data","volume":"17","author":"Mendizabal","year":"2011","journal-title":"Liver Transpl"},{"key":"10.1016\/j.prrv.2023.12.005_b0300","doi-asserted-by":"crossref","first-page":"S480","DOI":"10.1016\/S0168-8278(23)01202-3","article-title":"THU-522 - Liver transplantation in patients with cystic fibrosis: 30 years of experience in Australia and New Zealand","volume":"78","author":"Wilson","year":"2023","journal-title":"J Hepatol"},{"issue":"8","key":"10.1016\/j.prrv.2023.12.005_b0305","doi-asserted-by":"crossref","first-page":"954","DOI":"10.1111\/j.1399-3046.2010.01412.x","article-title":"A review of abdominal organ transplantation in cystic fibrosis","volume":"14","author":"Lu","year":"2010","journal-title":"Pediatr Transplant"},{"issue":"5","key":"10.1016\/j.prrv.2023.12.005_b0310","doi-asserted-by":"crossref","first-page":"1620","DOI":"10.1111\/ajt.13630","article-title":"Under Utilization of Pancreas Transplants in Cystic Fibrosis Recipients in the United Network Organ Sharing (UNOS) Data 1987\u20132014","volume":"16","author":"Usatin","year":"2016","journal-title":"Am J Transplant"},{"issue":"9","key":"10.1016\/j.prrv.2023.12.005_b0315","doi-asserted-by":"crossref","first-page":"e14371","DOI":"10.1111\/ctr.14371","article-title":"Pancreas transplantation for cystic fibrosis: A frequently missed opportunity","volume":"35","author":"Fridell","year":"2021","journal-title":"Clin Transplant"},{"issue":"8","key":"10.1016\/j.prrv.2023.12.005_b0320","doi-asserted-by":"crossref","first-page":"2288","DOI":"10.1073\/pnas.1525093113","article-title":"Effective treatment of steatosis and steatohepatitis by fibroblast growth factor 1 in mouse models of nonalcoholic fatty liver disease","volume":"113","author":"Liu","year":"2016","journal-title":"Proc Natl Acad Sci U S A"},{"issue":"3","key":"10.1016\/j.prrv.2023.12.005_b0325","doi-asserted-by":"crossref","first-page":"601","DOI":"10.1016\/j.cell.2013.03.028","article-title":"A vitamin D receptor\/SMAD genomic circuit gates hepatic fibrotic response","volume":"153","author":"Ding","year":"2013","journal-title":"Cell"},{"issue":"10","key":"10.1016\/j.prrv.2023.12.005_b0330","doi-asserted-by":"crossref","first-page":"1193","DOI":"10.1164\/rccm.201910-1943SO","article-title":"Cystic Fibrosis: Emergence of Highly Effective Targeted Therapeutics and Potential Clinical Implications","volume":"201","author":"Mall","year":"2020","journal-title":"Am J Respir Crit Care Med"},{"issue":"18","key":"10.1016\/j.prrv.2023.12.005_b0335","doi-asserted-by":"crossref","first-page":"1663","DOI":"10.1056\/NEJMoa1105185","article-title":"A CFTR potentiator in patients with cystic fibrosis and the G551D mutation","volume":"365","author":"Ramsey","year":"2011","journal-title":"N Engl J Med"},{"issue":"19","key":"10.1016\/j.prrv.2023.12.005_b0340","doi-asserted-by":"crossref","first-page":"1809","DOI":"10.1056\/NEJMoa1908639","article-title":"Elexacaftor-Tezacaftor-Ivacaftor for Cystic Fibrosis with a Single Phe508del Allele","volume":"381","author":"Middleton","year":"2019","journal-title":"N Engl J Med"},{"issue":"5","key":"10.1016\/j.prrv.2023.12.005_b0345","doi-asserted-by":"crossref","first-page":"529","DOI":"10.1164\/rccm.202108-1986OC","article-title":"Clinical Effectiveness of Elexacaftor\/Tezacaftor\/Ivacaftor in People with Cystic Fibrosis: A Clinical Trial","volume":"205","author":"Nichols","year":"2022","journal-title":"Am J Respir Crit Care Med"},{"issue":"10212","key":"10.1016\/j.prrv.2023.12.005_b0350","doi-asserted-by":"crossref","first-page":"1940","DOI":"10.1016\/S0140-6736(19)32597-8","article-title":"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","volume":"394","author":"Heijerman","year":"2019","journal-title":"Lancet"},{"issue":"4","key":"10.1016\/j.prrv.2023.12.005_b0355","doi-asserted-by":"crossref","first-page":"730","DOI":"10.1016\/j.jcf.2023.03.002","article-title":"Real-world safety and effectiveness of elexacaftor\/tezacaftor\/ivacaftor in people with cystic fibrosis: Interim results of a long-term registry-based study","volume":"22","author":"Bower","year":"2023","journal-title":"J Cyst Fibros"},{"key":"10.1016\/j.prrv.2023.12.005_b0360","doi-asserted-by":"crossref","DOI":"10.3389\/fped.2022.914790","article-title":"The Exocrine Pancreas in Cystic Fibrosis in the Era of CFTR Modulation: A Mini Review","volume":"10","author":"McKay","year":"2022","journal-title":"Front Pediatr"},{"issue":"2","key":"10.1016\/j.prrv.2023.12.005_b0365","doi-asserted-by":"crossref","first-page":"258","DOI":"10.1016\/j.jcf.2021.09.001","article-title":"The effect of elexacaftor\/tezacaftor\/ivacaftor (ETI) on glycemia in adults with cystic fibrosis","volume":"21","author":"Scully","year":"2022","journal-title":"J Cyst Fibros"},{"issue":"2","key":"10.1016\/j.prrv.2023.12.005_b0370","doi-asserted-by":"crossref","first-page":"282","DOI":"10.1016\/j.jcf.2022.10.003","article-title":"Elexacaftor\/tezacaftor\/ivacaftor and gastrointestinal outcomes in cystic fibrosis: Report of promise-GI","volume":"22","author":"Schwarzenberg","year":"2023","journal-title":"J Cyst Fibros"},{"key":"10.1016\/j.prrv.2023.12.005_b0375","doi-asserted-by":"crossref","DOI":"10.3389\/fphar.2022.877118","article-title":"Elexacaftor-Tezacaftor-Ivacaftor Treatment Reduces Abdominal Symptoms in Cystic Fibrosis-Early results Obtained With the CF-Specific CFAbd-Score","volume":"13","author":"Mainz","year":"2022","journal-title":"Front Pharmacol"},{"issue":"2","key":"10.1016\/j.prrv.2023.12.005_b0380","doi-asserted-by":"crossref","first-page":"e76","DOI":"10.1016\/j.jhep.2023.02.006","article-title":"Effects of CFTR modulator therapies on liver stiffness and bile flow: A single-centre experience","volume":"79","author":"Calvo","year":"2023","journal-title":"J Hepatol"},{"issue":"4","key":"10.1016\/j.prrv.2023.12.005_b0385","doi-asserted-by":"crossref","first-page":"878","DOI":"10.1111\/liv.15544","article-title":"Increase of liver stiffness and altered bile acid metabolism after triple CFTR modulator initiation in children and young adults with cystic fibrosis","volume":"43","author":"Schnell","year":"2023","journal-title":"Liver Int"},{"issue":"2","key":"10.1016\/j.prrv.2023.12.005_b0390","doi-asserted-by":"crossref","first-page":"e0010","DOI":"10.1097\/HC9.0000000000000010","article-title":"Effects of CFTR modulators on serum biomarkers of liver fibrosis in children with cystic fibrosis","volume":"7","author":"Levitte","year":"2023","journal-title":"Hepatol Commun"},{"issue":"2","key":"10.1016\/j.prrv.2023.12.005_b0395","doi-asserted-by":"crossref","first-page":"263","DOI":"10.1016\/j.jcf.2023.01.012","article-title":"Characterization of CFTR mutations in people with cystic fibrosis and severe liver disease who are not eligible for CFTR modulators","volume":"22","author":"Colombo","year":"2023","journal-title":"J Cyst Fibros"},{"issue":"21","key":"10.1016\/j.prrv.2023.12.005_b0400","doi-asserted-by":"crossref","first-page":"2024","DOI":"10.1056\/NEJMoa1709847","article-title":"Tezacaftor-Ivacaftor in Residual-Function Heterozygotes with Cystic Fibrosis","volume":"377","author":"Rowe","year":"2017","journal-title":"N Engl J Med"},{"issue":"17","key":"10.1016\/j.prrv.2023.12.005_b0405","doi-asserted-by":"crossref","first-page":"1612","DOI":"10.1056\/NEJMoa1807120","article-title":"VX-445-Tezacaftor-Ivacaftor in Patients with Cystic Fibrosis and One or Two Phe508del Alleles","volume":"379","author":"Keating","year":"2018","journal-title":"N Engl J Med"},{"issue":"12","key":"10.1016\/j.prrv.2023.12.005_b0410","doi-asserted-by":"crossref","first-page":"1522","DOI":"10.1164\/rccm.202102-0509OC","article-title":"A Phase 3 Open-Label Study of Elexacaftor\/Tezacaftor\/Ivacaftor in Children 6 through 11 Years of Age with Cystic Fibrosis and at Least One F508del Allele","volume":"203","author":"Zemanick","year":"2021","journal-title":"Am J Respir Crit Care Med"},{"issue":"11","key":"10.1016\/j.prrv.2023.12.005_b0415","doi-asserted-by":"crossref","first-page":"1361","DOI":"10.1164\/rccm.202202-0392OC","article-title":"Efficacy and Safety of Elexacaftor\/Tezacaftor\/Ivacaftor in Children 6 Through 11 Years of Age with Cystic Fibrosis Heterozygous for F508del and a Minimal Function Mutation: A Phase 3b, Randomized, Placebo-controlled Study","volume":"206","author":"Mall","year":"2022","journal-title":"Am J Respir Crit Care Med"},{"issue":"3","key":"10.1016\/j.prrv.2023.12.005_b0420","doi-asserted-by":"crossref","first-page":"267","DOI":"10.1016\/S2213-2600(21)00454-9","article-title":"Efficacy and safety of elexacaftor plus tezacaftor plus ivacaftor versus tezacaftor plus ivacaftor in people with cystic fibrosis homozygous for F508del-CFTR: a 24-week, multicentre, randomised, double-blind, active-controlled, phase 3b trial","volume":"10","author":"Sutharsan","year":"2022","journal-title":"Lancet Respir Med"},{"issue":"9","key":"10.1016\/j.prrv.2023.12.005_b0425","doi-asserted-by":"crossref","first-page":"815","DOI":"10.1056\/NEJMoa2100665","article-title":"Triple Therapy for Cystic Fibrosis Phe508del-Gating and -Residual Function Genotypes","volume":"385","author":"Barry","year":"2021","journal-title":"N Engl J Med"},{"issue":"2","key":"10.1016\/j.prrv.2023.12.005_b0430","doi-asserted-by":"crossref","first-page":"e99","DOI":"10.1016\/j.jcf.2021.07.001","article-title":"Drug-induced liver injury from elexacaftor\/ivacaftor\/tezacaftor","volume":"21","author":"Lowry","year":"2022","journal-title":"J Cyst Fibros"},{"key":"10.1016\/j.prrv.2023.12.005_b0435","article-title":"Delayed hepatic necrosis in a cystic fibrosis patient taking Elexacaftor\/Tezacaftor\/Ivacaftor (Kaftrio)","volume":"34","author":"Salehi","year":"2021","journal-title":"Respir Med Case Rep"},{"key":"10.1016\/j.prrv.2023.12.005_b0440","first-page":"A2113-A","article-title":"Acute Liver Failure in a Patient with Cystic Fibrosis Taking Triple Combination Modulator. TP35 TP035 challenging cases in patients with bronchiectasis","author":"Sharma","year":"2021","journal-title":"Am Thorac Soc Int Conf Abstracts Am Thorac Soc"},{"issue":"3","key":"10.1016\/j.prrv.2023.12.005_b0445","doi-asserted-by":"crossref","first-page":"220","DOI":"10.1056\/NEJMoa1409547","article-title":"Lumacaftor-Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del CFTR","volume":"373","author":"Wainwright","year":"2015","journal-title":"N Engl J Med"},{"key":"10.1016\/j.prrv.2023.12.005_b0450","unstructured":"ORKAMBI [prescribing information]. Boston MVPIA."},{"key":"10.1016\/j.prrv.2023.12.005_b0455","unstructured":"Canadian clinical consensus guideline for initiation, monitoring and discontinuation of CFTR modulator therapies for patients with cystic fibrosis. Cystic Fibrosis Canada, July 2021 (https:\/\/www.cysticfibrosis.ca\/uploads\/CFC%20Modulator%20Guidelines.pdf)."},{"issue":"6","key":"10.1016\/j.prrv.2023.12.005_b0460","doi-asserted-by":"crossref","first-page":"817","DOI":"10.1007\/s13318-022-00791-8","article-title":"Phase 1 Study to Assess the Safety and Pharmacokinetics of Elexacaftor\/Tezacaftor\/Ivacaftor in Subjects Without Cystic Fibrosis With Moderate Hepatic Impairment","volume":"47","author":"Viswanathan","year":"2022","journal-title":"Eur J Drug Metab Pharmacokinet"},{"issue":"2","key":"10.1016\/j.prrv.2023.12.005_b0465","doi-asserted-by":"crossref","first-page":"212","DOI":"10.1016\/j.jcf.2021.07.018","article-title":"Lumacaftor-ivacaftor effects on cystic fibrosis-related liver involvement in adolescents with homozygous F508 del-CFTR","volume":"21","author":"Drummond","year":"2022","journal-title":"J Cyst Fibros"},{"issue":"12","key":"10.1016\/j.prrv.2023.12.005_b0470","doi-asserted-by":"crossref","first-page":"761","DOI":"10.4254\/wjh.v11.i12.761","article-title":"Lumacaftor\/ivacaftor therapy is associated with reduced hepatic steatosis in cystic fibrosis patients","volume":"11","author":"Kutney","year":"2019","journal-title":"World J Hepatol"},{"issue":"5","key":"10.1016\/j.prrv.2023.12.005_b0475","doi-asserted-by":"crossref","first-page":"578","DOI":"10.1097\/MPG.0000000000000765","article-title":"Improvement of hepatic steatosis in cystic fibrosis with ivacaftor therapy","volume":"60","author":"Hayes","year":"2015","journal-title":"J Pediatr Gastroenterol Nutr"},{"issue":"2","key":"10.1016\/j.prrv.2023.12.005_b0480","doi-asserted-by":"crossref","first-page":"256","DOI":"10.1016\/j.jcf.2023.01.007","article-title":"Longitudinal effects of elexacaftor\/tezacaftor\/ivacaftor on liver tests at a large single adult cystic fibrosis centre","volume":"22","author":"Tewkesbury","year":"2023","journal-title":"J Cyst Fibros"},{"issue":"2","key":"10.1016\/j.prrv.2023.12.005_b0485","doi-asserted-by":"crossref","first-page":"411","DOI":"10.1002\/ppul.25779","article-title":"The use of elexacaftor\/tezacaftor\/ivacaftor in patients with cystic fibrosis postliver transplant: A case series","volume":"57","author":"Ragan","year":"2022","journal-title":"Pediatr Pulmonol"},{"issue":"2","key":"10.1016\/j.prrv.2023.12.005_b0490","doi-asserted-by":"crossref","first-page":"227","DOI":"10.1016\/j.jcf.2021.07.017","article-title":"Use of elexacaftor\/tezacaftor\/ivacaftor in liver transplant patients with cystic fibrosis","volume":"21","author":"McKinzie","year":"2022","journal-title":"J Cyst Fibros"}],"container-title":["Paediatric Respiratory Reviews"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1526054223000878?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1526054223000878?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2024,5,27]],"date-time":"2024-05-27T21:58:07Z","timestamp":1716847087000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S1526054223000878"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,6]]},"references-count":98,"alternative-id":["S1526054223000878"],"URL":"https:\/\/doi.org\/10.1016\/j.prrv.2023.12.005","relation":{},"ISSN":["1526-0542"],"issn-type":[{"value":"1526-0542","type":"print"}],"subject":[],"published":{"date-parts":[[2024,6]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Cystic fibrosis liver disease in the new era of cystic fibrosis transmembrane conductance regulator (CFTR) modulators","name":"articletitle","label":"Article Title"},{"value":"Paediatric Respiratory Reviews","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.prrv.2023.12.005","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2023 The Authors. Published by Elsevier Ltd.","name":"copyright","label":"Copyright"}]}}