{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,7]],"date-time":"2026-05-07T11:11:14Z","timestamp":1778152274151,"version":"3.51.4"},"reference-count":37,"publisher":"Wiley","license":[{"start":{"date-parts":[[2026,4,27]],"date-time":"2026-04-27T00:00:00Z","timestamp":1777248000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"},{"start":{"date-parts":[[2026,4,27]],"date-time":"2026-04-27T00:00:00Z","timestamp":1777248000000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["JPGN rep."],"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Objectives<\/jats:title>\n                    <jats:p>\n                      The efficacy of cystic fibrosis transmembrane conductance regulator (\n                      <jats:italic>CFTR<\/jats:italic>\n                      )\u2010modulator therapies in preventing or ameliorating cystic fibrosis liver disease (CFLD) by correcting\n                      <jats:italic>CFTR<\/jats:italic>\n                      in cholangiocytes is not well\u2010documented. This study aimed to assess liver function during CFTR\u2010modulators.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>\n                      Patients eligible to start Elexacaftor\/Tezacaftor\/Ivacaftor (July 2021\u2013December 2022), followed\u2010up at the Piedmont Cystic Fibrosis reference\n                      <jats:italic>Centre of the Citt\u00e0 della Salute e della Scienza, Turin<\/jats:italic>\n                      ,\n                      <jats:italic>Italy<\/jats:italic>\n                      , were enrolled through a prospective single\u2010center cohort study and evaluated using hematological exams and liver stiffness measurement (LSM) before treatment and at 1, 3, 6, and 12 months thereafter. The treated group was compared to a group of untreated people with cystic fibrosis (pwCF), evaluated at baseline and after 12 months.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Sixty\u2010nine treated pwCF, mean age 16.0 years, 51% males, were compared to 130 non\u2010treated pwCF, mean age 12.14 years, 49% males. Treated pwCF were classified by LSM as normal (&lt;5\u2009kPa) (\n                      <jats:italic>N<\/jats:italic>\n                      \u2009=\u200944, 64%), mild liver fibrosis (5\u20137\u2009kPa) (\n                      <jats:italic>N<\/jats:italic>\n                      \u2009=\u200916, 23%), or moderate\u2010severe fibrosis (&gt;7\u2009kPa) (\n                      <jats:italic>N<\/jats:italic>\n                      \u2009=\u20099, 13%). A significant trend in LSM reduction was observed at Month 12 in the subgroups with LSM 5\u20137\u2009kPa and LSM\u2009&gt;\u20097\u2009kPa (\u22121.05,\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.016 and \u22121.57,\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.022, respectively). PwCF without fibrosis at baseline and non\u2010treated pwCF showed no LSM change over time. Liver enzyme levels increased at Month 12,\u2009trend mirrored in non\u2010treated pwCF for AST level, remaining within normal ranges.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>During treatment, a significant LSM reduction was observed in pwCF with liver fibrosis at baseline, especially after Month 1. This rapid improvement could be multifactorial and may suggest a potential early response without significant liver toxicity.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/jpr3.70186","type":"journal-article","created":{"date-parts":[[2026,4,27]],"date-time":"2026-04-27T11:31:35Z","timestamp":1777289495000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Impact of cystic fibrosis transmembrane conductance regulator modulator therapies on liver stiffness and liver enzymes: An observational perspective single\u2010center cohort study"],"prefix":"10.1002","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6073-6608","authenticated-orcid":false,"given":"Laura","family":"Giugliano","sequence":"first","affiliation":[{"name":"Pediatric Gastroenterology Unit, Regina Margherita Children's Hospital Azienda Ospedaliera\u2010Universitaria Citt\u00e0 della Salute e della Scienza Turin Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Elisabetta","family":"Augustoni","sequence":"additional","affiliation":[{"name":"Pediatric Gastroenterology Unit, Regina Margherita Children's Hospital Azienda Ospedaliera\u2010Universitaria Citt\u00e0 della Salute e della Scienza Turin Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Antonio","family":"Pizzol","sequence":"additional","affiliation":[{"name":"Pediatric Gastroenterology Unit, Regina Margherita Children's Hospital Azienda Ospedaliera\u2010Universitaria Citt\u00e0 della Salute e della Scienza Turin Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anna","family":"Opramolla","sequence":"additional","affiliation":[{"name":"Pediatric Gastroenterology Unit, Regina Margherita Children's Hospital Azienda Ospedaliera\u2010Universitaria Citt\u00e0 della Salute e della Scienza Turin Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Cristina","family":"Chiad\u00f2","sequence":"additional","affiliation":[{"name":"Pediatric Gastroenterology Unit, Regina Margherita Children's Hospital Azienda Ospedaliera\u2010Universitaria Citt\u00e0 della Salute e della Scienza Turin Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anna","family":"Zagaria","sequence":"additional","affiliation":[{"name":"Pediatric Gastroenterology Unit, Regina Margherita Children's Hospital Azienda Ospedaliera\u2010Universitaria Citt\u00e0 della Salute e della Scienza Turin Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Irene","family":"Esposito","sequence":"additional","affiliation":[{"name":"Pediatric Pulmonology Unit, Regina Margherita Children Hospital Azienda Ospedaliera\u2010Universitaria Citt\u00e1 Della Salute e Della Scienza Turin Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Giulia","family":"Roberto","sequence":"additional","affiliation":[{"name":"Pediatric Pulmonology Unit, Regina Margherita Children Hospital Azienda Ospedaliera\u2010Universitaria Citt\u00e1 Della Salute e Della Scienza Turin Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alice","family":"Ponte","sequence":"additional","affiliation":[{"name":"Pediatric Gastroenterology Unit, Regina Margherita Children's Hospital Azienda Ospedaliera\u2010Universitaria Citt\u00e0 della Salute e della Scienza Turin Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Andrea","family":"Evangelista","sequence":"additional","affiliation":[{"name":"Unit of Clinical Epidemiology, Citt\u00e0 della Salute e della Scienza di Torino Turin Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Paola","family":"Quarello","sequence":"additional","affiliation":[{"name":"Pediatric Onco\u2010Hematology, Regina Margherita Children's Hospital Azienda Ospedaliera\u2010Universitaria Citt\u00e0 della Salute e della Scienza Turin Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michele","family":"Pinon","sequence":"additional","affiliation":[{"name":"Pediatric Gastroenterology Unit, Regina Margherita Children's Hospital Azienda Ospedaliera\u2010Universitaria Citt\u00e0 della Salute e della Scienza Turin Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Pier Luigi","family":"Calvo","sequence":"additional","affiliation":[{"name":"Pediatric Gastroenterology Unit, Regina Margherita Children's Hospital Azienda Ospedaliera\u2010Universitaria Citt\u00e0 della Salute e della Scienza Turin Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2026,4,27]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMra2216474"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jhep.2021.09.042"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.prrv.2020.12.001"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1097\/MPG.0000000000000758"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1111\/liv.12113"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1097\/MPG.0000000000001448"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1155\/2016\/4592702"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0178784"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jhep.2022.06.017"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.14309\/ajg.0000000000000461"},{"key":"e_1_2_7_12_1","doi-asserted-by":"publisher","DOI":"10.1136\/heartjnl-2014-307385"},{"key":"e_1_2_7_13_1","doi-asserted-by":"publisher","DOI":"10.1097\/MPG.0000000000002872"},{"key":"e_1_2_7_14_1","doi-asserted-by":"publisher","DOI":"10.1002\/hep.23810"},{"key":"e_1_2_7_15_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.prrv.2024.07.004"},{"key":"e_1_2_7_16_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.prrv.2023.12.005"},{"key":"e_1_2_7_17_1","unstructured":"Kaftrio. European Medicines Agency (EMA). Accessed January 20 2025.https:\/\/www.ema.europa.eu\/en\/medicines\/human\/EPAR\/kaftrio"},{"key":"e_1_2_7_18_1","doi-asserted-by":"publisher","DOI":"10.1097\/HEP.0000000000000646"},{"key":"e_1_2_7_19_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2016.09.064"},{"key":"e_1_2_7_20_1","doi-asserted-by":"publisher","DOI":"10.1002\/hep.22376"},{"key":"e_1_2_7_21_1","doi-asserted-by":"publisher","DOI":"10.3748\/wjg.v21.i22.6931"},{"key":"e_1_2_7_22_1","doi-asserted-by":"publisher","DOI":"10.1111\/petr.13125"},{"key":"e_1_2_7_23_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jhep.2023.04.001"},{"key":"e_1_2_7_24_1","doi-asserted-by":"publisher","DOI":"10.3389\/fmolb.2023.1327958"},{"key":"e_1_2_7_25_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2024.01.001"},{"key":"e_1_2_7_26_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41598-024-68511-7"},{"key":"e_1_2_7_27_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2024.04.006"},{"key":"e_1_2_7_28_1","doi-asserted-by":"publisher","DOI":"10.1007\/s40265-023-01969-3"},{"key":"e_1_2_7_29_1","doi-asserted-by":"publisher","DOI":"10.1097\/HC9.0000000000000010"},{"key":"e_1_2_7_30_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2021.07.018"},{"key":"e_1_2_7_31_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2024.12.010"},{"key":"e_1_2_7_32_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2025.10.001"},{"key":"e_1_2_7_33_1","doi-asserted-by":"publisher","DOI":"10.4254\/wjh.v14.i2.411"},{"issue":"1","key":"e_1_2_7_34_1","first-page":"8","article-title":"Ivacaftor: a novel gene\u2010based therapeutic approach for cystic fibrosis","volume":"18","author":"Condren ME","year":"2013","journal-title":"J Pediatr Pharmacol Ther"},{"key":"e_1_2_7_35_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1908639"},{"issue":"7","key":"e_1_2_7_36_1","first-page":"500","article-title":"CFTR modulators for the treatment of cystic fibrosis","volume":"39","author":"Pettit RS","year":"2014","journal-title":"P & T"},{"key":"e_1_2_7_37_1","doi-asserted-by":"publisher","DOI":"10.3389\/fphar.2019.01662"},{"key":"e_1_2_7_38_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jhep.2023.02.006"}],"container-title":["JPGN Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/jpr3.70186","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/jpr3.70186","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/jpr3.70186","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,5,7]],"date-time":"2026-05-07T10:22:42Z","timestamp":1778149362000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/jpr3.70186"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,4,27]]},"references-count":37,"alternative-id":["10.1002\/jpr3.70186"],"URL":"https:\/\/doi.org\/10.1002\/jpr3.70186","archive":["Portico"],"relation":{},"ISSN":["2691-171X","2691-171X"],"issn-type":[{"value":"2691-171X","type":"print"},{"value":"2691-171X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,4,27]]},"assertion":[{"value":"2025-09-22","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-01-08","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-04-27","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"jpr3.70186"}}