{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T15:18:13Z","timestamp":1791299893499,"version":"4.1.0"},"reference-count":50,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2014,5,16]],"date-time":"2014-05-16T00:00:00Z","timestamp":1400198400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["American J Hematol"],"published-print":{"date-parts":[[2014,8]]},"abstract":"<jats:p>Many patients with sickle cell disease (SCD) have a reduced exercise capacity and abnormal lung function. Cardiopulmonary exercise testing (CPET) can identify causes of exercise limitation. Forty\u2010four consecutive SCD patients (27 HbSS, 11 HbSC, and 6 HbS\u2010beta thalassemia) with a median age (interquartile range) of 26 (21\u201341) years underwent pulmonary function tests, CPET, chest x\u2010ray, and echocardiography to further characterize exercise limitation in SCD. Peak oxygen uptake (V\u2032O<jats:sub>2<\/jats:sub>\u2010peak), expressing maximum exercise capacity, was decreased in 83% of the studied patients. V\u2032O<jats:sub>2<\/jats:sub>\u2010peak correlated with hemoglobin levels (<jats:italic>R<\/jats:italic>\u2009=\u20090.440, <jats:italic>P<\/jats:italic>\u2009=\u20090.005), forced vital capacity (FVC) (<jats:italic>R<\/jats:italic>\u2009=\u20090.717, <jats:italic>P<\/jats:italic>\u2009&lt;\u20090.0001). Cardiothoracic ratio on chest x\u2010ray inversely correlated with FVC (<jats:italic>R<\/jats:italic>\u2009=\u2009\u22120.637, <jats:italic>P<\/jats:italic>\u2009&lt;\u20090.001). According to criteria for exercise limitation, the patients were limited in exercise capacity due to anemia (<jats:italic>n<\/jats:italic>\u2009=\u200917), cardiovascular dysfunction (<jats:italic>n<\/jats:italic>\u2009=\u20092), musculoskeletal function (<jats:italic>n<\/jats:italic>\u2009=\u200910), pulmonary ventilatory abnormalities (<jats:italic>n<\/jats:italic>\u2009=\u20091), pulmonary vascular exercise limitation (<jats:italic>n<\/jats:italic>\u2009=\u20091), and poor effort (<jats:italic>n<\/jats:italic>\u2009=\u20093). In the present study we demonstrate that anemia is the most important determinant of reduced exercise tolerance observed in SCD patients without signs of pulmonary hypertension. We found a strong correlation between various parameters of lung volume and cardiothoracic ratio and we hypothesize that cardiomegaly and relative small chest size may be important causes of the impairment in pulmonary function, that is, reduced long volumes and diffusion capacity, in SCD. Taking into account anthropomorphic differences between SCD patients and controls could help to interpret lung function studies in SCD better. Am. J. Hematol. 89:819\u2013824, 2014. \u00a9 2014 Wiley Periodicals, Inc.<\/jats:p>","DOI":"10.1002\/ajh.23752","type":"journal-article","created":{"date-parts":[[2014,5,3]],"date-time":"2014-05-03T08:48:42Z","timestamp":1399106922000},"page":"819-824","source":"Crossref","is-referenced-by-count":46,"title":["Exercise tolerance, lung function abnormalities, anemia, and cardiothoracic ratio in sickle cell patients"],"prefix":"10.1002","volume":"89","author":[{"given":"Eduard J.","family":"van Beers","sequence":"first","affiliation":[{"name":"Department of Hematology, Academic Medical Center University of Amsterdam Amsterdam The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mart N.","family":"van der Plas","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine Academic Medical Center University of Amsterdam Amsterdam The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Erfan","family":"Nur","sequence":"additional","affiliation":[{"name":"Department of Hematology, Academic Medical Center University of Amsterdam Amsterdam The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Harm\u2010Jan","family":"Bogaard","sequence":"additional","affiliation":[{"name":"Department of Pulmonary Medicine VU University Medical Center Amsterdam The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Reindert P.","family":"van Steenwijk","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine Academic Medical Center University of Amsterdam Amsterdam The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bart J.","family":"Biemond","sequence":"additional","affiliation":[{"name":"Department of Hematology, Academic Medical Center University of Amsterdam Amsterdam The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paul","family":"Bresser","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine Academic Medical Center University of Amsterdam Amsterdam The Netherlands"},{"name":"Department of Respiratory Medicine Onze Lieve Vrouwe Gasthuis Amsterdam The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2014,5,16]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.200601-125OC"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1005565"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.3233\/CH-2011-1465"},{"issue":"11","key":"e_1_2_7_5_1","first-page":"1021","article-title":"Longitudinal exercise hemodynamics in children with sickle cell anemia","volume":"138","author":"Alpert BS","year":"1984","journal-title":"Am J Dis Child"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1096-8652(199606)52:2<96::AID-AJH5>3.0.CO;2-0"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1097\/00005768-200004000-00002"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.2002036"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V56.6.1127.1127"},{"issue":"2","key":"e_1_2_7_10_1","first-page":"643","article-title":"The acute chest syndrome in sickle cell disease: Incidence and risk factors","volume":"84","author":"Castro O","year":"1994","journal-title":"The Cooperative Study of Sickle Cell Disease. 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