{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,2]],"date-time":"2026-06-02T13:37:14Z","timestamp":1780407434422,"version":"3.54.1"},"reference-count":34,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2025,5,6]],"date-time":"2025-05-06T00:00:00Z","timestamp":1746489600000},"content-version":"vor","delay-in-days":5,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2025,5,1]],"date-time":"2025-05-01T00:00:00Z","timestamp":1746057600000},"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":[[2025,5]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Previous research showed that lung function abnormalities are common in infants with cystic fibrosis (IwCF) but real\u2010world data are missing.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>\n                      This single\u2010center retrospective study analyzed infant lung function results from IwCF born in 2012\u20132018. The tests were conducted at Great Ormond Street Hospital, London, as part of routine care at 3 months, 1 year, and 2 years of age. Z\u2010scores for SF\n                      <jats:sub>6<\/jats:sub>\n                      Lung Clearance Index (zLCI), plethysmographic FRC (zFRC\n                      <jats:sub>pleth<\/jats:sub>\n                      ) and FEV\n                      <jats:sub>0.5<\/jats:sub>\n                      were derived. Microbiology and antibiotics prescription from 3 months before lung function assessments, up to the closest medical review following the lung function encounter, were analyzed, along with changes in management advised by the physician.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      A total of 126 lung function encounters (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u200943 at 3 months, 46 at 1 year, 37 at 2 years) from 60 IwCF were included. LCI was abnormal (zLCI &gt;\u20091.96) in 31% (12\/39) of 3\u2010month\u2010olds (mean\u00b1 zLCI 1.21\u2009\u00b1\u20091.08), 28% (12\/43) of 1\u2010year\u2010olds and 19% (7\/36) of 2\u2010year\u2010olds (mean\u00b1 zLCI 1.13\u2009\u00b1\u20091.10). Among 74 cases with recent positive microbiology or abnormal chest findings at medical review, 100% (31\/31) of those with abnormal lung function and 86% (37\/43) of those with normal lung function (\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.04) had a recent antibiotic prescription or a change in clinical management. Conversely, in encounters with abnormal lung function but normal clinical findings, management changes occurred in only 12% (2\/16) of cases.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>In this real\u2010word cohort of IwCF, clinical management was mainly influenced by clinical findings and only marginally by abnormal lung function (elevated FRC or LCI).<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/ppul.71117","type":"journal-article","created":{"date-parts":[[2025,5,6]],"date-time":"2025-05-06T07:48:51Z","timestamp":1746517731000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Infant Lung Function in Cystic Fibrosis: A Real\u2010World Study"],"prefix":"10.1002","volume":"60","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5366-4594","authenticated-orcid":false,"given":"Michele","family":"Arigliani","sequence":"first","affiliation":[{"name":"Paediatric Respiratory Unit Great Ormond Street Hospital for Children NHS Foundation Trust London UK"},{"name":"Infection, Immunity and Inflammation Research and Teaching Department UCL Great Ormond Street Institute of Child Health (UCL GOS ICH) London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sidrah","family":"Chaudhry","sequence":"additional","affiliation":[{"name":"Paediatric Respiratory Unit Great Ormond Street Hospital for Children NHS Foundation Trust London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5647-2424","authenticated-orcid":false,"given":"Rossa","family":"Brugha","sequence":"additional","affiliation":[{"name":"Paediatric Respiratory Unit Great Ormond Street Hospital for Children NHS Foundation Trust London UK"},{"name":"Infection, Immunity and Inflammation Research and Teaching Department UCL Great Ormond Street Institute of Child Health (UCL GOS ICH) London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ranjan","family":"Suri","sequence":"additional","affiliation":[{"name":"Paediatric Respiratory Unit Great Ormond Street Hospital for Children NHS Foundation Trust London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paul","family":"Aurora","sequence":"additional","affiliation":[{"name":"Paediatric Respiratory Unit Great Ormond Street Hospital for Children NHS Foundation Trust London UK"},{"name":"Infection, Immunity and Inflammation Research and Teaching Department UCL Great Ormond Street Institute of Child Health (UCL GOS ICH) London 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