{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,15]],"date-time":"2026-10-15T20:35:31Z","timestamp":1792096531010,"version":"4.4.0"},"reference-count":39,"publisher":"Elsevier BV","issue":"5","license":[{"start":{"date-parts":[[2025,9,1]],"date-time":"2025-09-01T00:00:00Z","timestamp":1756684800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2025,9,1]],"date-time":"2025-09-01T00:00:00Z","timestamp":1756684800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"}],"funder":[{"DOI":"10.13039\/501100004040","name":"KU Leuven","doi-asserted-by":"publisher","award":["S52174"],"award-info":[{"award-number":["S52174"]}],"id":[{"id":"10.13039\/501100004040","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100006282","name":"Koning Boudewijnstichting","doi-asserted-by":"publisher","award":["2020-J1810150-217926"],"award-info":[{"award-number":["2020-J1810150-217926"]}],"id":[{"id":"10.13039\/501100006282","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["clinicalkey.com","clinicalkey.com.au","clinicalkey.es","clinicalkey.fr","clinicalkey.jp","cysticfibrosisjournal.com","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Journal of Cystic Fibrosis"],"published-print":{"date-parts":[[2025,9]]},"DOI":"10.1016\/j.jcf.2025.04.002","type":"journal-article","created":{"date-parts":[[2025,5,8]],"date-time":"2025-05-08T17:33:09Z","timestamp":1746725589000},"page":"946-953","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":3,"title":["Pulmonary arteriole narrowing in end-stage cystic fibrosis lungs occurs with and without small airway disease"],"prefix":"10.1016","volume":"24","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-4145-491X","authenticated-orcid":false,"given":"Astrid","family":"Vermaut","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9348-8081","authenticated-orcid":false,"given":"Gitte","family":"Aerts","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1260-7083","authenticated-orcid":false,"given":"Lynn","family":"Willems","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4386-1746","authenticated-orcid":false,"given":"Vincent","family":"Geudens","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8471-3686","authenticated-orcid":false,"given":"Charlotte","family":"Hooft","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3806-4478","authenticated-orcid":false,"given":"Pieterjan","family":"Kerckhof","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0001-8346-9693","authenticated-orcid":false,"given":"Lise","family":"Vanvuchelen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0379-358X","authenticated-orcid":false,"given":"Marta","family":"Zapata-Ortega","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6176-0404","authenticated-orcid":false,"given":"Hanne","family":"Beeckmans","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3295-7179","authenticated-orcid":false,"given":"Xin","family":"Jin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0103-2517","authenticated-orcid":false,"given":"Yousry","family":"Mohamady","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7069-1535","authenticated-orcid":false,"given":"Jan","family":"Van Slambrouck","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6576-7814","authenticated-orcid":false,"given":"Lucia","family":"Aversa","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3981-4389","authenticated-orcid":false,"given":"Janne","family":"Verhaegen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1067-9179","authenticated-orcid":false,"given":"Emanuela E.","family":"Cortesi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Charlotte","family":"De Fays","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4246-6303","authenticated-orcid":false,"given":"Birgit","family":"Weynand","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1261-0992","authenticated-orcid":false,"given":"Dirk E.","family":"Van Raemdonck","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4261-7100","authenticated-orcid":false,"given":"Laurens J.","family":"Ceulemans","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9648-3497","authenticated-orcid":false,"given":"Wim A.","family":"Wuyts","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8263-0350","authenticated-orcid":false,"given":"Marianne","family":"Carlon","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3468-9251","authenticated-orcid":false,"given":"Robin","family":"Vos","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Natalie","family":"Lorent","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9652-7437","authenticated-orcid":false,"given":"Ghislaine","family":"Gayan-Ramirez","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2214-5879","authenticated-orcid":false,"given":"Laurent","family":"Godinas","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5576-9517","authenticated-orcid":false,"given":"Marijke","family":"Proesmans","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2303-2470","authenticated-orcid":false,"given":"Fran\u00e7ois","family":"Vermeulen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3961-1522","authenticated-orcid":false,"given":"Lieven J.","family":"Dupont","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6435-6901","authenticated-orcid":false,"given":"Bart M.","family":"Vanaudenaerde","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8138-5405","authenticated-orcid":false,"given":"Mieke","family":"Boon","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.jcf.2025.04.002_bib0001","doi-asserted-by":"crossref","first-page":"416","DOI":"10.1016\/j.jcf.2013.12.004","article-title":"Impact of pulmonary hypertension on survival in patients with cystic fibrosis undergoing lung transplantation: an analysis of the UNOS registry","volume":"13","author":"Hayes","year":"2014","journal-title":"J Cyst Fibrosis;"},{"key":"10.1016\/j.jcf.2025.04.002_bib0002","doi-asserted-by":"crossref","first-page":"652","DOI":"10.1097\/MCP.0b013e3283659e9f","article-title":"Pulmonary hypertension survival effects and treatment options in cystic fibrosis","author":"Tonelli","year":"2013","journal-title":"Curr Opin Pulm Med"},{"key":"10.1016\/j.jcf.2025.04.002_bib0003","doi-asserted-by":"crossref","first-page":"596","DOI":"10.1016\/S2213-2600(16)30107-2","article-title":"Pulmonary artery hypertension: an underrated disease manifestation in cystic fibrosis?","volume":"4","author":"Ratjen","year":"2016","journal-title":"Lancet Respirat"},{"key":"10.1016\/j.jcf.2025.04.002_bib0004","article-title":"Prognostic significance of pulmonary hypertension in patients with cystic fibrosis","author":"Li","year":"2018","journal-title":"Medicine"},{"key":"10.1016\/j.jcf.2025.04.002_bib0005","doi-asserted-by":"crossref","first-page":"532","DOI":"10.1016\/j.jcf.2012.04.012","article-title":"Pulmonary artery pressure in cystic fibrosis adults: characteristics, clinical correlates and long-term follow-up","volume":"11","author":"Bright-Thomas","year":"2012","journal-title":"J Cyst Fibrosis"},{"key":"10.1016\/j.jcf.2025.04.002_bib0006","doi-asserted-by":"crossref","DOI":"10.1183\/13993003.01253-2023","article-title":"Cystic fibrosis and the cardiovascular system: the unexpected heartache","author":"Hebestreit","year":"2023","journal-title":"Europ Respirat J"},{"key":"10.1016\/j.jcf.2025.04.002_bib0007","doi-asserted-by":"crossref","first-page":"1305","DOI":"10.3390\/life13061305","article-title":"Future comorbidities in an aging Cystic Fibrosis population","author":"Ticona","year":"2023","journal-title":"Life"},{"key":"10.1016\/j.jcf.2025.04.002_bib0008","doi-asserted-by":"crossref","first-page":"516","DOI":"10.1164\/rccm.201507-1281OC","article-title":"Morphometric analysis of explant lungs in cystic fibrosis","volume":"193","author":"Boon","year":"2016","journal-title":"Am J Respir Crit Care Med Am Thoracic Society;"},{"key":"10.1016\/j.jcf.2025.04.002_bib0009","doi-asserted-by":"crossref","first-page":"636","DOI":"10.1016\/S2213-2600(16)30105-9","article-title":"Pulmonary artery enlargement and cystic fibrosis pulmonary exacerbations: a cohort study","volume":"4","author":"Wells","year":"2016","journal-title":"Lancet Respir Med"},{"key":"10.1016\/j.jcf.2025.04.002_bib0010","doi-asserted-by":"crossref","first-page":"1023","DOI":"10.1113\/JP275860","article-title":"Angiogenesis in the lung","volume":"597","author":"Eldridge","year":"2019","journal-title":"J Physiol"},{"key":"10.1016\/j.jcf.2025.04.002_bib0011","doi-asserted-by":"crossref","DOI":"10.1152\/ajpcell.1998.275.6.C1555","article-title":"Characterization of CFTR expression and chloride channel activity in human endothelia","author":"Tousson","year":"1998","journal-title":"Am J Physiol"},{"key":"10.1016\/j.jcf.2025.04.002_bib0012","first-page":"58","article-title":"Involvement of CFTR in the pathogenesis of pulmonary arterial hypertension","author":"Le Ribeuz","year":"2021","journal-title":"Europ Respirat J Europ Respirat Society;"},{"key":"10.1016\/j.jcf.2025.04.002_bib0013","series-title":"Pulmonary artery enlargement is associated with pulmonary hypertension and decreased survival in severe cystic fibrosis: A cohort study","first-page":"15","author":"Zouk","year":"2020"},{"key":"10.1016\/j.jcf.2025.04.002_bib0014","first-page":"e401","article-title":"CT-measured pulmonary artery diameter as an independent predictor of pulmonary hypertension in cystic fibrosis","volume":"83","author":"Bakhshayeshkaram","year":"2018","journal-title":"Pol J Radiol Med Sci Int"},{"issue":"4","key":"10.1016\/j.jcf.2025.04.002_bib0015","doi-asserted-by":"crossref","first-page":"523","DOI":"10.1513\/AnnalsATS.202404-446OC","article-title":"Airway remodeling in cystic fibrosis is heterogeneous","volume":"22","author":"Vermaut","year":"2025","journal-title":"Ann Am Thorac Soc"},{"key":"10.1016\/j.jcf.2025.04.002_bib0016","series-title":"Small-Airway Obstruction and Emphysema in Chronic Obstructive Pulmonary Disease","author":"Mcdonough","year":"2011"},{"key":"10.1016\/j.jcf.2025.04.002_bib0017","doi-asserted-by":"crossref","first-page":"2220","DOI":"10.1016\/j.chest.2021.06.069","article-title":"Quantification of arterial and venous morphologic markers in pulmonary arterial hypertension using CT imaging","volume":"160","author":"Rahaghi","year":"2021","journal-title":"Chest"},{"key":"10.1016\/j.jcf.2025.04.002_bib0018","doi-asserted-by":"crossref","DOI":"10.1164\/rccm.202005-1671LE","article-title":"Pulmonary vascular pruning on computed tomography and risk of death in the Framingham heart study","author":"Synn","year":"2021","journal-title":"Am J Respir Crit Care Med"},{"key":"10.1016\/j.jcf.2025.04.002_bib0019","doi-asserted-by":"crossref","first-page":"865","DOI":"10.1016\/j.healun.2010.04.006","article-title":"Prevalence of pulmonary hypertension in end-stage cystic fibrosis and correlation with survival","volume":"29","author":"Tonelli","year":"2010","journal-title":"J Heart and Lung Transplant"},{"key":"10.1016\/j.jcf.2025.04.002_bib0020","doi-asserted-by":"crossref","first-page":"609","DOI":"10.1016\/j.healun.2013.03.006","article-title":"Pulmonary hypertension is a mild comorbidity in end-stage cystic fibrosis patients","volume":"32","author":"Belle-Van Meerkerk","year":"2013","journal-title":"J Heart and Lung Transplant"},{"key":"10.1016\/j.jcf.2025.04.002_bib0021","doi-asserted-by":"crossref","first-page":"1329","DOI":"10.1183\/09031936.00186908","article-title":"Pulmonary vascular dysfunction in endstage cystic fibrosis: role of NF-\u03bab and endothelin-1","volume":"34","author":"Henno","year":"2009","journal-title":"Europ Respirat J"},{"key":"10.1016\/j.jcf.2025.04.002_bib0022","series-title":"CFTR in the regulation of pulmonary vascular tone and remodeling","author":"Tabeling","year":"2021"},{"key":"10.1016\/j.jcf.2025.04.002_bib0023","doi-asserted-by":"crossref","first-page":"151","DOI":"10.4081\/monaldi.2019.1035","article-title":"Macro- and microvascular functions in cystic fibrosis adults without cardiovascular risk factors: A case-control study","volume":"89","author":"Vizzardi","year":"2019","journal-title":"Monaldi Arch Chest Disease;"},{"key":"10.1016\/j.jcf.2025.04.002_bib0024","first-page":"939","article-title":"Evidence of vascular endothelial dysfunction in young patients with cystic fibrosis","volume":"143","author":"Poore","year":"2013","journal-title":"Chest Am College Chest Phys"},{"key":"10.1016\/j.jcf.2025.04.002_bib0025","doi-asserted-by":"crossref","first-page":"3243","DOI":"10.1016\/j.bbadis.2017.08.011","article-title":"Mechanisms of endothelial cell dysfunction in cystic fibrosis","volume":"1863","author":"Totani","year":"2017","journal-title":"Biochim Biophys Acta Mol Basis Dis"},{"key":"10.1016\/j.jcf.2025.04.002_bib0026","doi-asserted-by":"crossref","DOI":"10.3389\/fphys.2017.00614","article-title":"Regulation of pulmonary vascular smooth muscle contractility in pulmonary arterial hypertension: implications for therapy","author":"Lyle","year":"2017","journal-title":"Front Physiol Frontiers Media S.A"},{"key":"10.1016\/j.jcf.2025.04.002_bib0027","doi-asserted-by":"crossref","DOI":"10.3390\/antiox8030056","article-title":"Regulation of smooth muscle cell proliferation by NADPH oxidases in pulmonary hypertension","author":"Huetsch","year":"2019","journal-title":"Antioxidants MDPI"},{"key":"10.1016\/j.jcf.2025.04.002_bib0028","first-page":"1","article-title":"Endothelial to mesenchymal transition in pulmonary vascular diseases","author":"Yun","year":"2020","journal-title":"Biomedicines"},{"key":"10.1016\/j.jcf.2025.04.002_bib0029","doi-asserted-by":"crossref","first-page":"144","DOI":"10.1016\/j.pupt.2014.09.003","article-title":"Pulmonary vascular changes in asthma and COPD","volume":"29","author":"Harkness","year":"2014","journal-title":"Pulm Pharmacol Ther"},{"key":"10.1016\/j.jcf.2025.04.002_bib0030","doi-asserted-by":"crossref","DOI":"10.1016\/j.rmed.2023.107499","article-title":"Potential systemic effects of acquired CFTR dysfunction in COPD","author":"Miravitlles","year":"2024","journal-title":"Respir Med"},{"key":"10.1016\/j.jcf.2025.04.002_bib0031","doi-asserted-by":"crossref","DOI":"10.1172\/JCI170502","article-title":"The vascular perspective on acute and chronic lung disease","author":"Borek","year":"2023","journal-title":"J Clin Investigat"},{"key":"10.1016\/j.jcf.2025.04.002_bib0032","doi-asserted-by":"crossref","first-page":"745","DOI":"10.1016\/j.bbrc.2007.02.166","article-title":"Intrinsic pro-angiogenic status of cystic fibrosis airway epithelial cells","volume":"356","author":"Verhaeghe","year":"2007","journal-title":"Biochem Biophys Res"},{"key":"10.1016\/j.jcf.2025.04.002_bib0033","doi-asserted-by":"crossref","first-page":"1553","DOI":"10.1183\/09031936.00164212","article-title":"CFTR dysfunction induces vascular endothelial growth factor synthesis in airway epithelium","volume":"42","author":"Martin","year":"2013","journal-title":"Europ Respirat J"},{"key":"10.1016\/j.jcf.2025.04.002_bib0034","first-page":"7","article-title":"Decoding\u201d angiogenesis: new facets controlling endothelial cell behavior","author":"Sewduth","year":"2016","journal-title":"Front Physiol"},{"key":"10.1016\/j.jcf.2025.04.002_bib0035","doi-asserted-by":"crossref","first-page":"820","DOI":"10.1016\/S2213-2600(23)00259-X","article-title":"Pulmonary hypertension associated with lung disease: new insights into pathomechanisms, diagnosis, and management","author":"Olsson","year":"2023","journal-title":"Lancet Respir Med"},{"key":"10.1016\/j.jcf.2025.04.002_bib0036","doi-asserted-by":"crossref","DOI":"10.1016\/j.athoracsur.2003.12.096","article-title":"Barazzone-Argiroffo C. Aerosolized iloprost as a bridge to lung transplantation in a patient with cystic fibrosis and pulmonary hypertension","volume":"78","author":"Tissi\u00e8res","year":"2004","journal-title":"Annals Thoracic Surg"},{"key":"10.1016\/j.jcf.2025.04.002_bib0037","first-page":"1828","volume":"33","author":"Temur","year":"2023"},{"key":"10.1016\/j.jcf.2025.04.002_bib0038","first-page":"61","article-title":"Diagnosis of pulmonary hypertension with cardiac MRI: derivation and validation of regression models","volume":"290","author":"Johns","year":"2019","journal-title":"Radiol Radiolog Society North Am"},{"key":"10.1016\/j.jcf.2025.04.002_bib0039","doi-asserted-by":"crossref","first-page":"1575","DOI":"10.1007\/s00330-005-2721-1","article-title":"Assessment of hemodynamic changes in the systemic and pulmonary arterial circulation in patients with cystic fibrosis using phase-contrast MRI","volume":"15","author":"Ley","year":"2005","journal-title":"Eur Radiol"}],"container-title":["Journal of Cystic Fibrosis"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1569199325007684?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1569199325007684?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2026,5,28]],"date-time":"2026-05-28T07:36:40Z","timestamp":1779953800000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S1569199325007684"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,9]]},"references-count":39,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2025,9]]}},"alternative-id":["S1569199325007684"],"URL":"https:\/\/doi.org\/10.1016\/j.jcf.2025.04.002","relation":{},"ISSN":["1569-1993"],"issn-type":[{"value":"1569-1993","type":"print"}],"subject":[],"published":{"date-parts":[[2025,9]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Pulmonary arteriole narrowing in end-stage cystic fibrosis lungs occurs with and without small airway disease","name":"articletitle","label":"Article Title"},{"value":"Journal of Cystic Fibrosis","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.jcf.2025.04.002","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2025 Published by Elsevier B.V. on behalf of European Cystic Fibrosis Society.","name":"copyright","label":"Copyright"}]}}