{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,12]],"date-time":"2026-09-12T19:16:01Z","timestamp":1789240561545,"version":"build-2803163510"},"reference-count":32,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2026,4,18]],"date-time":"2026-04-18T00:00:00Z","timestamp":1776470400000},"content-version":"vor","delay-in-days":17,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2026,4,1]],"date-time":"2026-04-01T00:00:00Z","timestamp":1775001600000},"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":[[2026,4]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Improvement in exocrine pancreatic function in people with Cystic fibrosis (PwCF) after ivacaftor and lumacaftor\/ivacaftor has been documented. Data on triple therapy with elexacaftor\/tezacaftor\/ivacaftor (ETI) are limited.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>In this multicenter, prospective, observational study, we reviewed changes in fecal elastase\u20101 (FE\u20101) in the pediatric population with exocrine pancreatic insufficiency (EPI) after 2 years of ETI. Patients were classified according to FE\u20101: mild\u2010moderate EPI as 100\u2013199\u2009\u03bcg\/g and severe EPI as &lt;100\u2009\u03bcg\/g. Demographic, clinical, anthropometric, and body composition data were collected. Body composition was measured using bioelectrical impedance analysis.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Fifty\u2010three children were recruited (58.5% males). The median age was 12 years [IQR: 9.0\u201314.5], and 56.6% were homozygous for F508del. Values of FE\u20101 below fifteen were designated with a 0. At baseline, the median FE\u20101 was 0.00 [IQR: 0.00\u20130.00]. We observed a slight yet statistically significant improvement in FE\u20101 values 2 years after ETI (Wilcoxon test,\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&lt;\u20090.05). We classified patients into three groups based on FE\u20101 values. At baseline, 98% were in the severe EPI group (FE\u20101\u2009&lt;\u2009100\u2009\u03bcg\/g), the remaining 2% were in the mild\u2010moderate group (FE\u20101 100\u2013199\u2009\u03bcg\/g). Two years after triple therapy, six (11.4%) children showed a change in FE\u20101 to at least 100\u2009\u03bcg\/g from the baseline. Three (5.7%) had a change in FE\u20101\u2009\u2265\u2009200\u2009\u03bcg\/g from baseline, and the other three (5.7%) had a change in FE\u20101 between 100 and 199\u2009\u03bcg\/g. A significant reduction in daily lipase intake was observed (6346 vs. 5703\u2009L\/kg\/day;\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&lt;\u20090.05). BMI\n                      <jats:italic>Z<\/jats:italic>\n                      \u2010score improved over 2 years (\u22120.55 vs. \u22120.38;\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&lt;\u20090.05). Body composition data were available for 27 patients, showing increased fat mass and fat\u2010free mass after ETI therapy. No differences in body composition were found when stratified by EPI groups.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>CFTR modulators have improved pulmonary function and anthropometric measures in PwCF. Based on these findings, CFTR modulators may also enhance exocrine pancreatic function in pediatric patients.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/ppul.71625","type":"journal-article","created":{"date-parts":[[2026,4,18]],"date-time":"2026-04-18T15:30:09Z","timestamp":1776526209000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Exocrine Pancreatic Function in Pediatric Patients After Two Years of Triple CFTR Modulator Therapy: Is It Possible to Restore Pancreatic Function?"],"prefix":"10.1002","volume":"61","author":[{"given":"C. M. L\u00f3pez","family":"C\u00e1rdenes","sequence":"first","affiliation":[{"name":"Unidad de Fibrosis Qu\u00edstica. Hospital Universitario Ram\u00f3n y Cajal Madrid Spain"},{"name":"Ram\u00f3n y Cajal Institute of Sanitary Research (IRYCIS) Madrid Spain"},{"name":"Department of Medicine and Medical Specialities, Faculty of Medicine and Health Sciences University of Alcal\u00e1 Alcal\u00e1 de Henares Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S. Vicente","family":"Santamar\u00eda","sequence":"additional","affiliation":[{"name":"Unidad de Fibrosis Qu\u00edstica. Hospital Universitario Ram\u00f3n y Cajal Madrid Spain"},{"name":"Ram\u00f3n y Cajal Institute of Sanitary Research (IRYCIS) Madrid Spain"},{"name":"Department of Medicine and Medical Specialities, Faculty of Medicine and Health Sciences University of Alcal\u00e1 Alcal\u00e1 de Henares Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"R. Garc\u00eda","family":"Romero","sequence":"additional","affiliation":[{"name":"Unidad de Fibrosis Qu\u00edstica. Hospital Universitario Miguel Servet Zaragoza Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A. Tabares","family":"Gonz\u00e1lez","sequence":"additional","affiliation":[{"name":"Unidad de Fibrosis Qu\u00edstica. Hospital Universitario Ram\u00f3n y Cajal Madrid Spain"},{"name":"Ram\u00f3n y Cajal Institute of Sanitary Research (IRYCIS) Madrid Spain"},{"name":"Department of Medicine and Medical Specialities, Faculty of Medicine and Health Sciences University of Alcal\u00e1 Alcal\u00e1 de Henares Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C. Gasc\u00f3n","family":"Galindo","sequence":"additional","affiliation":[{"name":"Unidad de Fibrosis Qu\u00edstica. Hospital Universitario Ram\u00f3n y Cajal Madrid Spain"},{"name":"Ram\u00f3n y Cajal Institute of Sanitary Research (IRYCIS) Madrid Spain"},{"name":"Department of Medicine and Medical Specialities, Faculty of Medicine and Health Sciences University of Alcal\u00e1 Alcal\u00e1 de Henares Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"E. Blitz","family":"Castro","sequence":"additional","affiliation":[{"name":"Unidad de Fibrosis Qu\u00edstica. Hospital Universitario Ram\u00f3n y Cajal Madrid Spain"},{"name":"Ram\u00f3n y Cajal Institute of Sanitary Research (IRYCIS) Madrid Spain"},{"name":"Department of Medicine and Medical Specialities, Faculty of Medicine and Health Sciences University of Alcal\u00e1 Alcal\u00e1 de Henares Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J.","family":"Quaresma","sequence":"additional","affiliation":[{"name":"Unidad de Fibrosis Qu\u00edstica. Hospital Universitario Ram\u00f3n y Cajal Madrid Spain"},{"name":"Ram\u00f3n y Cajal Institute of Sanitary Research (IRYCIS) Madrid Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A. Morales","family":"Tirado","sequence":"additional","affiliation":[{"name":"Unidad de Fibrosis Qu\u00edstica. Hospital Universitario Ram\u00f3n y Cajal Madrid Spain"},{"name":"Ram\u00f3n y Cajal Institute of Sanitary Research (IRYCIS) Madrid Spain"},{"name":"Department of Medicine and Medical Specialities, Faculty of Medicine and Health Sciences University of Alcal\u00e1 Alcal\u00e1 de Henares Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M. Garriga","family":"Garc\u00eda","sequence":"additional","affiliation":[{"name":"Unidad de Fibrosis Qu\u00edstica. Hospital Universitario Ram\u00f3n y Cajal Madrid Spain"},{"name":"Servicio de Endocrinolog\u00eda y Nutrici\u00f3n. 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