{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,17]],"date-time":"2026-08-17T14:54:35Z","timestamp":1786978475768,"version":"build-2736575974"},"reference-count":31,"publisher":"Wiley","issue":"7","license":[{"start":{"date-parts":[[2021,3,26]],"date-time":"2021-03-26T00:00:00Z","timestamp":1616716800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Pulmonology"],"published-print":{"date-parts":[[2021,7]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>\n                      Normal values (&gt;80%) of Forced Expiratory Volume in one second (FEV\n                      <jats:sub>1<\/jats:sub>\n                      ) in patients with cystic fibrosis (CF) may lead to the interpretation that there is no lung disease. This study is a comprehensive analysis of lung involvement in CF patients having normal FEV\n                      <jats:sub>1<\/jats:sub>\n                      .\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Patients were recruited from two CF centers: Hadassah Medical Center, Jerusalem and Vall d' Hebron Hospital, Barcelona. Lung disease was assessed by lung clearance index (LCI), chest CT\u2010Brody score, respiratory cultures, number of pulmonary exacerbations (PEx), and days of antibiotic treatment in the year before the assessment.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Of the 247 patients, 89 (36%) had FEV\n                      <jats:sub>1<\/jats:sub>\n                      \u226580% and were included in the study (mean age, 17.6; range, 4.25\u201349 years). Chronic\n                      <jats:italic>Pseudomonas aeruginosa<\/jats:italic>\n                      infection was found in 21%, and 31% had at least one major PEx in the year before the study. Abnormally elevated LCI was found in 86% of patients, ranging between 7.52 and 18.97, and total Brody score (TBS) was abnormal in 92% (range, 5.0\u201396.5). Patients with chronic\n                      <jats:italic>P. aeruginosa<\/jats:italic>\n                      had significantly higher LCI (\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u2009.01) and TBS (\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u2009.02) which were associated with more major PEx (\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&lt;\u2009.01 and\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u2009.01, respectively) and more days of intravenous (IV) antibiotic treatment in the preceding year (\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u2009.03 and\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u2009.001, respectively).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>\n                      Most CF patients with normal FEV\n                      <jats:sub>1<\/jats:sub>\n                      have already physiological and structural lung abnormalities which were associated with more PEx and IV antibiotic treatment. Further studies are needed to determine if better adherence to the currently used therapies and the new cystic fibrosis transmembrane modulators will prevent the progression of lung disease.\n                    <\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/ppul.25371","type":"journal-article","created":{"date-parts":[[2021,3,11]],"date-time":"2021-03-11T05:59:46Z","timestamp":1615442386000},"page":"2007-2013","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":11,"title":["<i>How abnormal is the normal?<\/i>\n                    Clinical characteristics of CF patients with normal FEV\n                    <sub>1<\/sub>"],"prefix":"10.1002","volume":"56","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-7119-5675","authenticated-orcid":false,"given":"Malena","family":"Cohen\u2010Cymberknoh","sequence":"first","affiliation":[{"name":"Pediatric Pulmonology Unit and Cystic Fibrosis Center, Hadassah Medical Center and 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Medicine Hebrew University of Jerusalem  Jerusalem Israel"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Angeles","family":"Spangenberg","sequence":"additional","affiliation":[{"name":"Pediatric Pulmonology Unit and Cystic Fibrosis Center Hospital Universitari Vall d' Hebron  Barcelona Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laura","family":"Garriga","sequence":"additional","affiliation":[{"name":"Pediatric Pulmonology Unit and Cystic Fibrosis Center Hospital Universitari Vall d' Hebron  Barcelona Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Iris","family":"Eisenstadt","sequence":"additional","affiliation":[{"name":"Department of Physiotherapy and Cystic Fibrosis Center Hadassah\u2010Hebrew University Medical Center  Jerusalem Israel"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tomer","family":"Israeli","sequence":"additional","affiliation":[{"name":"Department of Physiotherapy and Cystic 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