{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,5]],"date-time":"2026-10-05T01:49:48Z","timestamp":1791164988613,"version":"4.1.0"},"reference-count":29,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2026,5,8]],"date-time":"2026-05-08T00:00:00Z","timestamp":1778198400000},"content-version":"vor","delay-in-days":7,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2026,5,1]],"date-time":"2026-05-01T00:00:00Z","timestamp":1777593600000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"funder":[{"DOI":"10.13039\/501100000292","name":"Cystic Fibrosis Trust","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100000292","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Pulmonology"],"published-print":{"date-parts":[[2026,5]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Rationale<\/jats:title>\n                    <jats:p>Cystic fibrosis (CF) and primary ciliary dyskinesia (PCD) are characterized by neutrophilic airway inflammation but differ in clinical features.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Objectives<\/jats:title>\n                    <jats:p>We investigated relationships of pulmonary and systemic inflammatory markers with functional and structural lung disease.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Systemic (CRP, IgG, IL\u20106, and IL\u20108) and sputum (calprotectin, IL\u20106, and IL\u20108) markers were measured at baseline. Relationships were explored with spirometry, lung clearance index and lung computed tomography (CT) scores at baseline and with spirometry 10 years later.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      42 patients (21 CF, 21 PCD) of similar age (median CF 27\u2009y [12\u201359], PCD 27\u2009y [11\u201362]) and lung function (median ppFEV\n                      <jats:sub>1<\/jats:sub>\n                      CF 59% [40\u201399], PCD 67% [30\u2013101]) were enrolled in 2009. Systemic inflammation was significantly higher in CF (Neutrophils,\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&lt;\u20090.05, IL\u20106 and 8,\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&lt;\u20090.01). Conversely, sputum IL\u20106 and 8 were higher in PCD (\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&lt;\u20090.01). In CF, sputum IL\u20106 counter\u2010intuitively correlated with better CT scores (\n                      <jats:italic>r<\/jats:italic>\n                      \u2009=\u2009\u22120.51\/\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&lt;\u20090.05) at baseline and with better spirometry (\n                      <jats:italic>r<\/jats:italic>\n                      \u2009=\u20090.58,\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&lt;\u20090.05) 10 years later. Conversely, high sputum IL\u20106 at baseline was associated with worse lung function in PCD (\n                      <jats:italic>r<\/jats:italic>\n                      \u2009=\u20090.81\/\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.001) after 10 years.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>This study emphasizes the differences of functional and structural aspects in both diseases. Markers that predict long\u2010term outcome in PCD and in CF were identified. The most striking and unanticipated finding was that sputum IL\u20106 correlated with better CT scores and lung function in CF. This paradox merits further research but challenges whether sputum IL\u20106 in CF is always a bad prognostic indicator.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/ppul.71659","type":"journal-article","created":{"date-parts":[[2026,5,8]],"date-time":"2026-05-08T14:14:13Z","timestamp":1778249653000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Function and Structure Relationships With Inflammation Differ in Two Chronic Suppurative Lung Diseases"],"prefix":"10.1002","volume":"61","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1002-1185","authenticated-orcid":false,"given":"Teresa","family":"Fuchs","sequence":"first","affiliation":[{"name":"National Heart &amp; Lung Institute, Imperial College London London UK"},{"name":"Imperial Biomedical Research Centre London UK"},{"name":"Royal Brompton Hospital, Guy's &amp; St Thomas' Trust London UK"},{"name":"Department of Child and Adolescent Health Paediatrics III, Medical University of Innsbruck, Cystic Fibrosis Centre Innsbruck Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jacqueline","family":"Donovan","sequence":"additional","affiliation":[{"name":"National Heart &amp; Lung Institute, Imperial College London London UK"},{"name":"Imperial Biomedical Research Centre London UK"},{"name":"Royal Brompton Hospital, Guy's &amp; St Thomas' Trust London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrew","family":"Ives","sequence":"additional","affiliation":[{"name":"National Heart &amp; Lung Institute, Imperial College London London UK"},{"name":"Imperial Biomedical Research Centre London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Samantha","family":"Irving","sequence":"additional","affiliation":[{"name":"National Heart &amp; Lung Institute, Imperial College London London UK"},{"name":"Imperial Biomedical Research Centre London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gwyneth","family":"Davies","sequence":"additional","affiliation":[{"name":"Population Policy and Practice, UCL Great Ormond Street Institute of Child Health London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Claire","family":"Hogg","sequence":"additional","affiliation":[{"name":"National Heart &amp; Lung Institute, Imperial College London London UK"},{"name":"Imperial Biomedical Research Centre London UK"},{"name":"Royal Brompton Hospital, Guy's &amp; St Thomas' Trust London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eric","family":"Alton","sequence":"additional","affiliation":[{"name":"National Heart &amp; Lung Institute, Imperial College London London UK"},{"name":"Imperial Biomedical Research Centre London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gemma","family":"Wilson","sequence":"additional","affiliation":[{"name":"National Heart &amp; Lung Institute, Imperial College London London UK"},{"name":"Imperial Biomedical Research Centre London UK"},{"name":"Royal Brompton Hospital, Guy's &amp; St Thomas' Trust London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrew","family":"Bush","sequence":"additional","affiliation":[{"name":"National Heart &amp; Lung Institute, Imperial College London London UK"},{"name":"Imperial Biomedical Research Centre London UK"},{"name":"Royal Brompton Hospital, Guy's &amp; St Thomas' Trust London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jane C.","family":"Davies","sequence":"additional","affiliation":[{"name":"National Heart &amp; Lung Institute, Imperial College London London UK"},{"name":"Imperial Biomedical Research Centre London UK"},{"name":"Royal Brompton Hospital, Guy's &amp; St Thomas' Trust London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2026,5,8]]},"reference":[{"key":"e_1_2_11_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(20)32542-3"},{"key":"e_1_2_11_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.rmed.2023.107169"},{"key":"e_1_2_11_4_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201301-0059CI"},{"key":"e_1_2_11_5_1","doi-asserted-by":"publisher","DOI":"10.1378\/chest.13-1162"},{"key":"e_1_2_11_6_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.200303-365OC"},{"key":"e_1_2_11_7_1","doi-asserted-by":"publisher","DOI":"10.3390\/ijms21093331"},{"key":"e_1_2_11_8_1","doi-asserted-by":"publisher","DOI":"10.1038\/cmi.2017.118"},{"key":"e_1_2_11_9_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.24129"},{"key":"e_1_2_11_10_1","doi-asserted-by":"publisher","DOI":"10.1378\/chest.129.1.118"},{"key":"e_1_2_11_11_1","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.01390-2015"},{"key":"e_1_2_11_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2023.09.008"},{"key":"e_1_2_11_13_1","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.01090-2016"},{"key":"e_1_2_11_14_1","doi-asserted-by":"publisher","DOI":"10.1136\/thorax.55.3.198"},{"key":"e_1_2_11_15_1","doi-asserted-by":"publisher","DOI":"10.1111\/cei.13624"},{"key":"e_1_2_11_16_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1569-1993(02)00141-8"},{"key":"e_1_2_11_17_1","doi-asserted-by":"publisher","DOI":"10.1034\/j.1399-3003.1999.14b17.x"},{"key":"e_1_2_11_18_1","doi-asserted-by":"publisher","DOI":"10.1513\/AnnalsATS.202204-314OC"},{"key":"e_1_2_11_19_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2021.10.009"},{"key":"e_1_2_11_20_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.20050"},{"key":"e_1_2_11_21_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2010.12.004"},{"key":"e_1_2_11_22_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201203-0507OC"},{"key":"e_1_2_11_23_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1301725"},{"key":"e_1_2_11_24_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2024.01.002"},{"key":"e_1_2_11_25_1","doi-asserted-by":"publisher","DOI":"10.1016\/S2213-2600(16)30048-0"},{"key":"e_1_2_11_26_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbadis.2010.04.005"},{"key":"e_1_2_11_27_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbamcr.2011.01.034"},{"key":"e_1_2_11_28_1","doi-asserted-by":"publisher","DOI":"10.1164\/ajrccm.157.6.9704086"},{"key":"e_1_2_11_29_1","doi-asserted-by":"publisher","DOI":"10.1513\/AnnalsATS.202507-817OC"},{"key":"e_1_2_11_30_1","doi-asserted-by":"publisher","DOI":"10.3390\/cells13020166"}],"container-title":["Pediatric Pulmonology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ppul.71659","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/ppul.71659","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ppul.71659","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,7,22]],"date-time":"2026-07-22T13:09:59Z","timestamp":1784725799000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/ppul.71659"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,5]]},"references-count":29,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2026,5]]}},"alternative-id":["10.1002\/ppul.71659"],"URL":"https:\/\/doi.org\/10.1002\/ppul.71659","archive":["Portico"],"relation":{},"ISSN":["8755-6863","1099-0496"],"issn-type":[{"value":"8755-6863","type":"print"},{"value":"1099-0496","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,5]]},"assertion":[{"value":"2025-10-24","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-05-05","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-05-08","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"e71659"}}