{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,18]],"date-time":"2026-08-18T04:50:29Z","timestamp":1787028629562,"version":"build-2736575974"},"reference-count":36,"publisher":"MDPI AG","issue":"23","license":[{"start":{"date-parts":[[2022,11,22]],"date-time":"2022-11-22T00:00:00Z","timestamp":1669075200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Regione Campania","award":["L. 548\/94"],"award-info":[{"award-number":["L. 548\/94"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>The last ten years have been characterized by an enormous step forward in the therapy and management of patients with Cystic Fibrosis (CF), thanks to the development and combination of Cystic Fibrosis Transmembrane Receptor (CFTR) correctors and potentiators. Specifically, the last approved triple combination elexacaftor\/tezacaftor\/ivacaftor has been demonstrated to improve lung function in CF patients with both homozygous Phe508del and Phe508del\/minimal function genotypes. Here we have assessed the effect of elexacaftor\/tezacaftor\/ivacaftor in patients carrying the Phe508del\/minimal function genotype (n = 20) after one year of treatments on liver function and nutrient absorption with a focus on lipid metabolism. We show that weight, BMI, and albumin significantly increase, suggesting a positive impact of the treatment on nutrient absorption. Furthermore, cholesterol levels as a biomarker of lipid metabolism increased significantly after one year of treatment. Most importantly, we suggest that these results were not dependent on the diet composition, possibly indicating that the drug improves the hepatic synthesis and secretion of proteins and cholesterol.<\/jats:p>","DOI":"10.3390\/jcm11236900","type":"journal-article","created":{"date-parts":[[2022,11,23]],"date-time":"2022-11-23T03:15:24Z","timestamp":1669173324000},"page":"6900","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":35,"title":["Cystic Fibrosis Patients with F508del\/Minimal Function Genotype: Laboratory and Nutritional Evaluations after One Year of Elexacaftor\/Tezacaftor\/Ivacaftor Treatment"],"prefix":"10.3390","volume":"11","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-4246-6883","authenticated-orcid":false,"given":"Vincenzo","family":"Carnovale","sequence":"first","affiliation":[{"name":"Department of Translational Medical Science, University of Naples Federico II, 80131 Naples, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1121-7315","authenticated-orcid":false,"given":"Filippo","family":"Scial\u00f2","sequence":"additional","affiliation":[{"name":"Department of Translational and Medical Science, University of Campania \u201cL. Vanvitelli\u201d, 80131 Naples, Italy"},{"name":"CEINGE\u2014Biotecnologie Avanzate, 80131 Naples, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1500-0746","authenticated-orcid":false,"given":"Monica","family":"Gelzo","sequence":"additional","affiliation":[{"name":"CEINGE\u2014Biotecnologie Avanzate, 80131 Naples, Italy"},{"name":"Department of Molecular Medicine and Medical Biotechnology, University of Naples Federico II, 80131 Naples, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8909-9726","authenticated-orcid":false,"given":"Paola","family":"Iacotucci","sequence":"additional","affiliation":[{"name":"Department of Clinical Medicine and Surgery, University of Naples Federico II, 80131 Naples, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0596-2933","authenticated-orcid":false,"given":"Felice","family":"Amato","sequence":"additional","affiliation":[{"name":"CEINGE\u2014Biotecnologie Avanzate, 80131 Naples, Italy"},{"name":"Department of Molecular Medicine and Medical Biotechnology, University of Naples Federico II, 80131 Naples, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1614-6816","authenticated-orcid":false,"given":"Federica","family":"Zarrilli","sequence":"additional","affiliation":[{"name":"CEINGE\u2014Biotecnologie Avanzate, 80131 Naples, Italy"},{"name":"Department of Molecular Medicine and Medical Biotechnology, University of Naples Federico II, 80131 Naples, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Assunta","family":"Celardo","sequence":"additional","affiliation":[{"name":"Department of Translational Medical Science, University of Naples Federico II, 80131 Naples, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giuseppe","family":"Castaldo","sequence":"additional","affiliation":[{"name":"CEINGE\u2014Biotecnologie Avanzate, 80131 Naples, Italy"},{"name":"Department of Molecular Medicine and Medical Biotechnology, University of Naples Federico II, 80131 Naples, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4720-1320","authenticated-orcid":false,"given":"Gaetano","family":"Corso","sequence":"additional","affiliation":[{"name":"Department of Clinical and Experimental Medicine, University of Foggia, 71122 Foggia, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2022,11,22]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"93","DOI":"10.1007\/s00018-016-2391-y","article-title":"Role of CFTR in epithelial physiology","volume":"74","author":"Gray","year":"2017","journal-title":"Cell. 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