{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,21]],"date-time":"2026-10-21T13:43:21Z","timestamp":1792590201384,"version":"4.5.0"},"reference-count":17,"publisher":"Wiley","license":[{"start":{"date-parts":[[2026,10,21]],"date-time":"2026-10-21T00:00:00Z","timestamp":1792540800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2026,10,21]],"date-time":"2026-10-21T00:00:00Z","timestamp":1792540800000},"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":["Acta Paediatrica"],"abstract":"ABSTRACT<\/jats:title>\n \n Aim<\/jats:title>\n Elexacaftor\/Tezacaftor\/Ivacaftor (ETI) has markedly improved clinical outcomes in cystic fibrosis (CF). However, its effects on gastrointestinal (GI) function, nutrition and pancreatic enzyme therapy in children remain insufficiently described. This study aimed to characterise GI\u2010related changes during the first year of ETI treatment in Swedish children with CF.<\/jats:p>\n <\/jats:sec>\n \n Methods<\/jats:title>\n In this prospective, multicentre study at three paediatric CF centres, children aged 6\u201317\u2009years who initiated ETI were assessed at baseline, 6 and 12\u2009months. Outcomes included GI symptoms and the use of GI\u2010related medication, health\u2010related quality of life (CFQ\u2010R), nutritional status, PERT use and faecal elastase. Longitudinal changes were analysed using generalised least squares regression.<\/jats:p>\n <\/jats:sec>\n \n Results<\/jats:title>\n \n Sixty\u2010two participants (mean age 11.7\u2009years) completed follow\u2010up. The total mean combined number of reported GI symptoms declined from 1.5 to 0.9 (\n p<\/jats:italic>\n \u2009=\u20090.005), although no individual symptom changed significantly after 12\u2009months of ETI treatment (\n p<\/jats:italic>\n \u2009>\u20090.05). CFQ\u2010R Digestive scores improved by 7.8 points (\n p<\/jats:italic>\n \u2009=\u20090.002). PERT dose decreased by 1.3\u2009\u00d7\u200910\n 3<\/jats:sup>\n \u2009units\/kg\/day (\n p<\/jats:italic>\n \u2009<\u20090.001), while BMI z\u2010scores and faecal elastase remained unchanged.\n <\/jats:p>\n <\/jats:sec>\n \n Conclusion<\/jats:title>\n ETI therapy reduced gastrointestinal symptoms and improved digestive quality of life in children with CF, accompanied by lower PERT use but unchanged pancreatic function and growth.<\/jats:p>\n <\/jats:sec>","DOI":"10.1111\/apa.70807","type":"journal-article","created":{"date-parts":[[2026,10,21]],"date-time":"2026-10-21T13:06:08Z","timestamp":1792587968000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Changes in Gastrointestinal Symptoms Among Children With Cystic Fibrosis During 12\u2009Months of Elexacaftor\/Tezacaftor\/Ivacaftor Therapy: A Swedish Multicentre Study"],"prefix":"10.1111","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0194-7365","authenticated-orcid":false,"given":"Marcus","family":"Svedberg","sequence":"first","affiliation":[{"name":"Department of Paediatrics, Institute of Clinical Sciences Sahlgrenska Academy, Gothenburg University Sweden"},{"name":"Department of Paediatrics Queen Silvia's Children's Hospital Gothenburg Sweden"},{"name":"Centre for Digital Health Sahlgrenska University Hospital Gothenburg Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ellen Karlge","family":"Nilsson","sequence":"additional","affiliation":[{"name":"Department of Paediatrics Queen Silvia's Children's Hospital Gothenburg Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Per","family":"Ekman","sequence":"additional","affiliation":[{"name":"Statistiska Konsultgruppen Sweden AB Gothenburg Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9447-663X","authenticated-orcid":false,"given":"Henrik","family":"Imberg","sequence":"additional","affiliation":[{"name":"Statistiska Konsultgruppen Sweden AB Gothenburg Sweden"},{"name":"Department of Molecular and Clinical Medicine, Institute of Medicine, Sahlgrenska Academy University of Gothenburg Gothenburg Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2026,10,21]]},"reference":[{"issue":"10059","key":"e_1_2_11_2_1","doi-asserted-by":"crossref","first-page":"2519","DOI":"10.1016\/S0140-6736(16)00576-6","article-title":"Cystic Fibrosis","volume":"388","author":"Elborn J. S.","year":"2016","journal-title":"Lancet"},{"issue":"12","key":"e_1_2_11_3_1","doi-asserted-by":"crossref","first-page":"1522","DOI":"10.1164\/rccm.202102-0509OC","article-title":"A Phase 3 Open\u2010Label Study of Elexacaftor\/Tezacaftor\/Ivacaftor in Children 6 Through 11\u2009Years of Age With Cystic Fibrosis and at Least One F508del Allele","volume":"203","author":"Zemanick E. T.","year":"2021","journal-title":"American Journal of Respiratory and Critical Care Medicine"},{"issue":"19","key":"e_1_2_11_4_1","doi-asserted-by":"crossref","first-page":"1809","DOI":"10.1056\/NEJMoa1908639","article-title":"Elexacaftor\u2010Tezacaftor\u2010Ivacaftor for Cystic Fibrosis With a Single Phe508del Allele","volume":"381","author":"Middleton P. G.","year":"2019","journal-title":"New England Journal of Medicine"},{"issue":"5","key":"e_1_2_11_5_1","doi-asserted-by":"crossref","first-page":"894","DOI":"10.1016\/j.jcf.2025.09.003","article-title":"The Novel CFAbd\u2010Score.Kid(\u24b8) Reveals a Significant Decline of Abdominal Symptoms in Children With Cystic Fibrosis Aged 6 Through 11\u2009Years on Elexacaftor\/Tezacaftor\/Ivacaftor \u2010 First Results","volume":"24","author":"Mainz J. G.","year":"2025","journal-title":"Journal of Cystic Fibrosis"},{"key":"e_1_2_11_6_1","doi-asserted-by":"crossref","first-page":"787","DOI":"10.1016\/j.jcf.2025.01.010","article-title":"Longer Term Follow\u2010Up of Abdominal Symptoms (CFAbd\u2010Score) After Initiation of Elexacaftor \/ Tezacaftor \/ Ivacaftor in Adults With Cystic Fibrosis","volume":"24","author":"Caley L. R.","year":"2025","journal-title":"Journal of Cystic Fibrosis"},{"issue":"3","key":"e_1_2_11_7_1","doi-asserted-by":"crossref","first-page":"474","DOI":"10.1016\/j.jcf.2023.10.001","article-title":"Reduction in Abdominal Symptoms (CFAbd\u2010Score), Faecal M2\u2010Pyruvate\u2010Kinase and Calprotectin Over One Year of Treatment With Elexacaftor\u2010Tezacaftor\u2010Ivacaftor in People With CF Aged >\/=12\u2009Years \u2010 The RECOVER Study","volume":"23","author":"Mainz J. G.","year":"2024","journal-title":"Journal of Cystic Fibrosis"},{"key":"e_1_2_11_8_1","doi-asserted-by":"crossref","DOI":"10.3389\/fphar.2022.877118","article-title":"Elexacaftor\u2010Tezacaftor\u2010Ivacaftor Treatment Reduces Abdominal Symptoms in Cystic Fibrosis\u2010Early Results Obtained With the CF\u2010Specific CFAbd\u2010Score","volume":"13","author":"Mainz J. G.","year":"2022","journal-title":"Frontiers in Pharmacology"},{"issue":"6","key":"e_1_2_11_9_1","doi-asserted-by":"crossref","first-page":"326","DOI":"10.1097\/00063198-199811000-00003","article-title":"Measurement of Quality of Life in Cystic Fibrosis","volume":"4","author":"Quittner A. L.","year":"1998","journal-title":"Current Opinion in Pulmonary Medicine"},{"issue":"5","key":"e_1_2_11_10_1","doi-asserted-by":"crossref","first-page":"600","DOI":"10.1111\/j.1651-2227.2006.tb02290.x","article-title":"Growth From Birth to Age 19 for Children in Sweden Born in 1981: Descriptive Values","volume":"95","author":"Werner B.","year":"2006","journal-title":"Acta Paediatrica"},{"issue":"6","key":"e_1_2_11_11_1","doi-asserted-by":"crossref","first-page":"1324","DOI":"10.1183\/09031936.00080312","article-title":"Multi\u2010Ethnic Reference Values for Spirometry for the 3\u201095\u2010Yr Age Range: The Global Lung Function 2012 Equations","volume":"40","author":"Quanjer P. H.","year":"2012","journal-title":"European Respiratory Journal"},{"issue":"3","key":"e_1_2_11_12_1","doi-asserted-by":"crossref","DOI":"10.1002\/ppul.71038","article-title":"Impact of Elexacaftor\/Tezacaftor\/Ivacaftor on Microbiology and Antibiotic Utilization in People With Cystic Fibrosis","volume":"60","author":"Williams C. L.","year":"2025","journal-title":"Pediatric Pulmonology"},{"issue":"5","key":"e_1_2_11_13_1","doi-asserted-by":"crossref","first-page":"984","DOI":"10.1016\/j.jcf.2024.08.001","article-title":"A Longitudinal Study Assessing the Impact of Elexacaftor\/Tezacaftor\/Ivacaftor on Gut Transit and Function in People With Cystic Fibrosis Using Magnetic Resonance Imaging (MRI)","volume":"23","author":"Yule A.","year":"2024","journal-title":"Journal of Cystic Fibrosis"},{"key":"e_1_2_11_14_1","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 S.","year":"2016","journal-title":"Scientific Reports"},{"issue":"5","key":"e_1_2_11_15_1","doi-asserted-by":"crossref","first-page":"886","DOI":"10.1016\/j.jcf.2025.07.010","article-title":"Validation of the CFAbd\u2010Score.Kid(\u24b8), a Novel Gastrointestinal Patient Reported Outcome Measure, Specific for Children With Cystic Fibrosis","volume":"24","author":"Sadrieh P.","year":"2025","journal-title":"Journal of Cystic Fibrosis"},{"issue":"4","key":"e_1_2_11_16_1","doi-asserted-by":"crossref","first-page":"419","DOI":"10.1007\/s40271-019-00361-2","article-title":"Validity and Reliability of a Novel Multimodal Questionnaire for the Assessment of Abdominal Symptoms in People With Cystic Fibrosis (CFAbd\u2010Score)","volume":"12","author":"Jaudszus A.","year":"2019","journal-title":"Patient"},{"key":"e_1_2_11_17_1","doi-asserted-by":"crossref","DOI":"10.1016\/j.bpg.2022.101788","article-title":"Digestive Outcomes in Cystic Fibrosis","volume":"56","author":"Ley D.","year":"2022","journal-title":"Best Practice & Research. Clinical Gastroenterology"},{"issue":"3","key":"e_1_2_11_18_1","doi-asserted-by":"crossref","first-page":"557","DOI":"10.1016\/j.clnu.2016.03.004","article-title":"ESPEN\u2010ESPGHAN\u2010ECFS Guidelines on Nutrition Care for Infants, Children, and Adults With Cystic Fibrosis","volume":"35","author":"Turck D.","year":"2016","journal-title":"Clinical Nutrition"}],"container-title":["Acta Paediatrica"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/apa.70807","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1111\/apa.70807","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/apa.70807","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,10,21]],"date-time":"2026-10-21T13:06:10Z","timestamp":1792587970000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/apa.70807"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,10,21]]},"references-count":17,"alternative-id":["10.1111\/apa.70807"],"URL":"https:\/\/doi.org\/10.1111\/apa.70807","archive":["Portico"],"relation":{},"ISSN":["0803-5253","1651-2227"],"issn-type":[{"value":"0803-5253","type":"print"},{"value":"1651-2227","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,10,21]]},"assertion":[{"value":"2025-11-11","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-10-16","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-10-21","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"apa.70807"}}