{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,8]],"date-time":"2026-03-08T22:00:29Z","timestamp":1773007229814,"version":"3.50.1"},"reference-count":48,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2026,2,13]],"date-time":"2026-02-13T00:00:00Z","timestamp":1770940800000},"content-version":"vor","delay-in-days":12,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2026,2,1]],"date-time":"2026-02-01T00:00:00Z","timestamp":1769904000000},"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":["Pediatric Pulmonology"],"published-print":{"date-parts":[[2026,2]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>For people with cystic fibrosis (pwCF) carrying susceptible mutations, elexacaftor\/tezacaftor\/ivacaftor (ETI) has changed the course of their lives. Organ manifestations critically affecting both respiratory and digestive tracts have been found to improve considerably. Consequently, ETI\u2010associated reductions in sinonasal symptoms (SNS) deriving from the upper respiratory tract are consistently reported. However, although substantial ETI\u2010related declines in the burden of abdominal symptoms (AS) have been reported using the CFAbd\u2010Score, their correlation with SNS changes has not been studied.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Aims<\/jats:title>\n                    <jats:p>Assessing correlations between ETI\u2010related changes in pwCF SNS and AS.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>The German adapted version of the Sinonasal\u2010Outcome\u2010Test 20 (SNOT\u201020\u2010GAV) and the CFAbd\u2010Score, the first CF\u2010specific gastrointestinal (GI) patient\u2010reported outcome measure developed and validated following FDA guidelines, were simultaneously administered. PwCF completing both paired questionnaires, at least once before and after ETI initiation, were included.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      In two German CF\u2010centers,\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u200931 pwCF (18 f, mean\u2010age 13 years) were included, completing\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u2009106 paired questionnaires. During ETI therapy, significant decreases (\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&lt;\u20090.05) were observed for the mean total CFAbd\u2010Score (10.9\u21925.8pts[\u221247%]) as well as all sub\u2010scores (\u2018pain\u2019: \u221261%, \u2018disorders of bowel movement\u2019: \u221256%, \u2018GERD\u2019: \u221254%, \u2018disorders of appetite\u2019: \u221250% and \u2018QoL\u2010impairment\u2019:\u221267%). Analogously, SNS significantly improved (total SNOT: 10.1\u21923.4pts[\u221266%] and its 3 domains: primary and secondary nasal symptoms, and SNS\u2010related QoL [\u221268%\/\u221281%\/\u221254%]). However, the correlation between changes in symptoms related to upper\u2010respiratory and GI tracts was only modest.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>The burden of symptoms captured with the CFAbd\u2010Score and the SNOT\u201020\u2010GAV significantly and markedly decreased after ETI initiation. The modest association between changes in both organ\u2010specific symptom scores supports the previously reported individualized electrophysiological responses observed in nasal\u2010potential and intestinal\u2010current measurements.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/ppul.71507","type":"journal-article","created":{"date-parts":[[2026,2,13]],"date-time":"2026-02-13T14:00:48Z","timestamp":1770991248000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Relating Improvement in Gastrointestinal (CFAbd\u2010Score\n                    <sup>\u00a9<\/sup>\n                    ) and Sinonasal (SNOT) Symptoms During Therapy With Elexacaftor\/Tezacaftor\/Ivacaftor in CF Patients"],"prefix":"10.1002","volume":"61","author":[{"given":"Jochen G.","family":"Mainz","sequence":"first","affiliation":[{"name":"CF\u2010Center, Klinikum Westbrandenburg, Campus Brandenburg an der Havel Brandenburg an der Havel Germany"},{"name":"Medizinische Hochschule Brandenburg (MHB), Universit\u00e4tsklinikum Brandenburg an der Havel Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Vivien","family":"Greiner","sequence":"additional","affiliation":[{"name":"CF\u2010Center, Klinikum Westbrandenburg, Campus Brandenburg an der Havel Brandenburg an der Havel Germany"},{"name":"Medizinische Hochschule Brandenburg (MHB), Universit\u00e4tsklinikum Brandenburg an der Havel Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Pauline","family":"Sadrieh","sequence":"additional","affiliation":[{"name":"CF\u2010Center, Klinikum Westbrandenburg, Campus Brandenburg an der Havel Brandenburg an der Havel Germany"},{"name":"Medizinische Hochschule Brandenburg (MHB), Universit\u00e4tsklinikum Brandenburg an der Havel Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Louise","family":"Polte","sequence":"additional","affiliation":[{"name":"CF\u2010Center, Klinikum Westbrandenburg, Campus Brandenburg an der Havel Brandenburg an der Havel Germany"},{"name":"Medizinische Hochschule Brandenburg (MHB), Universit\u00e4tsklinikum Brandenburg an der Havel Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ingo","family":"Baumann","sequence":"additional","affiliation":[{"name":"Department of Otorhinolaryngology University Hospital Heidelberg Heidelberg Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Franziska","family":"Duckstein","sequence":"additional","affiliation":[{"name":"CF\u2010Center, Klinikum Westbrandenburg, Campus Brandenburg an der Havel Brandenburg an der Havel Germany"},{"name":"Medizinische Hochschule Brandenburg (MHB), Universit\u00e4tsklinikum Brandenburg an der Havel Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anton","family":"Barucha","sequence":"additional","affiliation":[{"name":"CF\u2010Center, Klinikum Westbrandenburg, Campus Brandenburg an der Havel Brandenburg an der Havel Germany"},{"name":"Medizinische Hochschule Brandenburg (MHB), Universit\u00e4tsklinikum Brandenburg an der Havel Germany"},{"name":"Department of Gastroenterology Brandenburg Medical School (Theodor Fontane) Brandenburg an der Havel Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Suzanne","family":"van Dullemen","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Division of Pneumology, Allergology, Infectious Diseases, Gastroenterology Goethe University Frankfurt a.M. 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