{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,17]],"date-time":"2026-08-17T14:52:18Z","timestamp":1786978338504,"version":"build-2736575974"},"reference-count":33,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2024,4,12]],"date-time":"2024-04-12T00:00:00Z","timestamp":1712880000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"DOI":"10.13039\/100000050","name":"National Heart, Lung, and Blood Institute","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000050","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000897","name":"Cystic Fibrosis Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000897","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Pulmonology"],"published-print":{"date-parts":[[2024,6]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Cystic fibrosis (CF) is caused by CF transmembrane conductance regulator (CFTR) gene mutations producing dysfunctional CFTR proteins leading to progressive clinical disease. Elexacaftor\u2010tezacaftor\u2010ivacaftor (ETI) remarkably improves lung disease but is associated with substantial weight gain.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Study Design and Methods<\/jats:title>\n                    <jats:p>We performed a single\u2010center longitudinal study predicting 6\u2010month weight gain after ETI initiation. We used linear mixed effects modeling (LME) to determine association of ETI treatment with changing body mass index (BMI). Using linear regression, we examined BMI prediction models with distinct combinations of main effects to identify a model useful for patient counseling. We used up to eight commonly observed clinical characteristics as input variables (age, sex, percent predicted FEV1 [FEV1%], F508del homozygous state, pancreatic sufficiency, HgbA1c, prior modulator use and prior year number of pulmonary exacerbations).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      We evaluated 154 patients (19\u201073 years old, 54% female, FEV\n                      <jats:sub>1<\/jats:sub>\n                      %\u2009=\u200919\u2212121, 0\u22126 prior year pulmonary exacerbations). LME demonstrated an association between ETI use and weight increases. Exhaustive testing suggested a parsimonious linear regression model well\u2010fitted to data that is potentially useful for counseling. The two variable model shows that on average, BMI decreases by 0.045 (95% Confidence Interval [CI]\u2009=\u2009\u22120.069 to \u22120.021,\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&lt;\u20090.001) for every year of age and increases by 0.322 (CI\u2009=\u20090.142 to 0.502,\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.001) for each additional prior year exacerbation at the time of ETI initiation.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Interpretation<\/jats:title>\n                    <jats:p>Young patients with many prior year pulmonary exacerbations likely have the largest 6 month weight gain after starting ETI.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/ppul.26982","type":"journal-article","created":{"date-parts":[[2024,4,12]],"date-time":"2024-04-12T09:24:06Z","timestamp":1712913846000},"page":"1724-1730","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":14,"title":["Predicting weight gain in patients with cystic fibrosis on triple combination modulator"],"prefix":"10.1002","volume":"59","author":[{"ORCID":"https:\/\/orcid.org\/0009-0004-9353-6986","authenticated-orcid":false,"given":"Kelly L.","family":"Stewart","sequence":"first","affiliation":[{"name":"The Adult Cystic Fibrosis Center, Division of Respiratory, Critical Care and Occupational Pulmonary Medicine, Department of Internal Medicine, School of Medicine University of Utah Salt Lake City Utah USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0705-715X","authenticated-orcid":false,"given":"Rhonda","family":"Szczesniak","sequence":"additional","affiliation":[{"name":"Division of Biostatistics &amp; Epidemiology, Division of Pulmonary Medicine Cincinnati Children's Hospital Medical Center Cincinnati OH USA"},{"name":"Department of Pediatrics University of Cincinnati Cincinnati OH USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0121-4847","authenticated-orcid":false,"given":"Theodore G.","family":"Liou","sequence":"additional","affiliation":[{"name":"The Adult Cystic Fibrosis Center, Division of Respiratory, Critical Care and Occupational Pulmonary Medicine, Department of Internal Medicine, School of Medicine University of Utah Salt Lake City Utah USA"},{"name":"The Center for Quantitative Biology University of Utah Salt Lake City Utah USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2024,4,12]]},"reference":[{"key":"e_1_2_12_2_1","doi-asserted-by":"publisher","DOI":"10.1001\/archpedi.1938.01980140114013"},{"key":"e_1_2_12_3_1","unstructured":"Cystic Fibrosis Foundation Patient Registry 2021Annual Data Report.Cystic Fibrosis Foundation; 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