{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,11,1]],"date-time":"2025-11-01T02:44:19Z","timestamp":1761965059444,"version":"3.40.5"},"reference-count":35,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2019,2,10]],"date-time":"2019-02-10T00:00:00Z","timestamp":1549756800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/501100003593","name":"Conselho Nacional de Desenvolvimento Cient\u00edfico e Tecnol\u00f3gico","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100003593","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Br J Haematol"],"published-print":{"date-parts":[[2019,4]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>Pulmonary complications are frequent in patients with sickle cell disease (<jats:styled-content style=\"fixed-case\">SCD<\/jats:styled-content>), but few studies have described lung pathology in <jats:styled-content style=\"fixed-case\">SCD<\/jats:styled-content>. We studied the lung tissue of 30 deceased <jats:styled-content style=\"fixed-case\">SCD<\/jats:styled-content> patients (1994\u20132012). Demographics, genotype, clinical characteristics, cause of death and associated conditions are presented. We quantified the presence of pulmonary arterial changes, thrombosis and venous thickening. Alveolar capillary abnormalities were demonstrated using <jats:styled-content style=\"fixed-case\">CD<\/jats:styled-content>34 expression and confocal microscopy. Autopsy and echocardiography reports were reviewed to classify heart abnormalities. Tissue expression of markers of endothelial activation (vascular cell adhesion molecule 1, intercellular adhesion molecule 1 and vascular endothelial growth factor) was quantified in pulmonary vessels. Median age was 33\u00a0years; genotype was <jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content> in 19, <jats:styled-content style=\"fixed-case\">SC<\/jats:styled-content> in 7 and S\u03b2 in 4, and there were 18 males. Hypertensive arterial changes were present in 76% of the patients, recent thrombosis in 80% and old thrombosis in 43%. Venous thickening was present in 23% and pulmonary capillary haemangiomatosis foci in 87%. Ten percent of the patients presented right ventricular hypertrophy. There was no increased expression of endothelial activation markers when compared to controls. <jats:styled-content style=\"fixed-case\">SCD<\/jats:styled-content> affects the whole pulmonary vascular tree and reflects the multiple burden on lung vasculature imposed by the disease upon time.<\/jats:p>","DOI":"10.1111\/bjh.15795","type":"journal-article","created":{"date-parts":[[2019,2,11]],"date-time":"2019-02-11T00:51:20Z","timestamp":1549846280000},"page":"317-326","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":11,"title":["Clinical relevance of pulmonary vasculature involvement in sickle cell disease"],"prefix":"10.1111","volume":"185","author":[{"given":"German R.","family":"Carstens","sequence":"first","affiliation":[{"name":"Department of Pathology Faculdade de Medicina Universidade de S\u00e3o Paulo S\u00e3o Paulo SP Brazil"},{"name":"Hospital Eduardo Sch\u00fctz Schroeder Puerto Montt Chile"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Bianca B. 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