{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,12]],"date-time":"2026-03-12T14:14:57Z","timestamp":1773324897999,"version":"3.50.1"},"reference-count":16,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2014,8,18]],"date-time":"2014-08-18T00:00:00Z","timestamp":1408320000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["European J of Haematology"],"published-print":{"date-parts":[[2015,2]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Sickle cell disease (<jats:styled-content style=\"fixed-case\">SCD<\/jats:styled-content>) has extremely variable phenotypes, and several factors have been associated with the severity of the disease.<\/jats:p><\/jats:sec><jats:sec><jats:title>Objectives<\/jats:title><jats:p>To analyze the chronic complications of <jats:styled-content style=\"fixed-case\">SCD<\/jats:styled-content> and look for predictive risk factors for increased severity and number of complications.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Retrospective study including all children followed for <jats:styled-content style=\"fixed-case\">SCD<\/jats:styled-content> in the Paediatric Haematology Unit of a tertiary hospital in <jats:styled-content style=\"fixed-case\">P<\/jats:styled-content>ortugal, who completed 17\u00a0yr old between the years 2004 and 2013.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>We identified 44 patients, 55% female and 98% black. Chronic complications occurred in 80% of cases. Slight dilatation of the left ventricle was the most frequent complication (47.7%), followed by respiratory function disturbs (43.2%), microlithiasis or cholelithiasis (40.9%), increased flow velocity of cerebral arteries (31.8%), enuresis, delayed puberty and bone abnormalities (6.8% each), sickle cell retinopathy and leg ulcer (4.6% each) and recurrent priapism (2.3%). We identified a statistically significant association between leukocytes &gt;15\u00a0000\/\u03bcL and a higher number of hospitalizations (<jats:italic>P<\/jats:italic>\u00a0&lt;\u00a00.001) and chronic complications of the disease (<jats:italic>P<\/jats:italic>\u00a0=\u00a00.035). The occurrence of dactylitis in first year of life was also significantly associated with a higher number of hospitalizations (<jats:italic>P<\/jats:italic>\u00a0=\u00a00.004) and chronic complications (<jats:italic>P<\/jats:italic>\u00a0=\u00a00.018). The presence of \u03b1\u2010thalassemia was associated with a lower number of chronic complications (<jats:italic>P<\/jats:italic>\u00a0=\u00a00.036).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Leucocytosis and dactylitis in the first year of life can be predictors of <jats:styled-content style=\"fixed-case\">SCD<\/jats:styled-content> severity, while the presence of \u03b1\u2010thalassemia can be protective. The determination of early predictors of chronic complications of <jats:styled-content style=\"fixed-case\">SCD<\/jats:styled-content> may improve the comprehensive care of these patients.<\/jats:p><\/jats:sec>","DOI":"10.1111\/ejh.12411","type":"journal-article","created":{"date-parts":[[2014,7,10]],"date-time":"2014-07-10T15:27:45Z","timestamp":1405006065000},"page":"157-161","source":"Crossref","is-referenced-by-count":18,"title":["Sickle cell disease in children: chronic complications and search of predictive factors for adverse outcomes"],"prefix":"10.1111","volume":"94","author":[{"given":"In\u00eas Vaz","family":"Silva","sequence":"first","affiliation":[{"name":"Department of Paediatrics Hospital Vila Franca de Xira Vila Franca de Xira Portugal"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ana Filipa","family":"Reis","sequence":"additional","affiliation":[{"name":"Department of Paediatrics Hospital Garcia de Orta Almada Portugal"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Maria Jo\u00e3o","family":"Palar\u00e9","sequence":"additional","affiliation":[{"name":"Unit of Paediatric Haematology Department of Paediatrics Hospital de Santa Maria Centro Hospitalar Lisboa Norte EPE Lisboa Portugal"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anabela","family":"Ferr\u00e3o","sequence":"additional","affiliation":[{"name":"Unit of Paediatric Haematology Department of Paediatrics Hospital de Santa Maria Centro Hospitalar Lisboa Norte EPE Lisboa Portugal"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Teresa","family":"Rodrigues","sequence":"additional","affiliation":[{"name":"Laboratory of Biomathematics Faculdade de Medicina de Lisboa Lisboa Portugal"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anabela","family":"Morais","sequence":"additional","affiliation":[{"name":"Unit of Paediatric Haematology Department of Paediatrics Hospital de Santa Maria Centro Hospitalar Lisboa Norte EPE Lisboa Portugal"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2014,8,18]]},"reference":[{"key":"e_1_2_5_2_1","unstructured":"Prevalence of rare diseases: Bibliographic data Orphanet Report Series Rare Diseases collection Nov 2013 Number 1: Listed in alphabetical order of disease or group of diseases http:\/\/www.orpha.net\/orphacom\/cahiers\/docs\/GB\/Prevalence_of_rare diseases_ by_alphabetical_list.pdf"},{"key":"e_1_2_5_3_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2007-04-084921"},{"key":"e_1_2_5_4_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2006-02-005082"},{"key":"e_1_2_5_5_1","doi-asserted-by":"publisher","DOI":"10.1309\/YKN9-T1N3-DGGM-5KU1"},{"key":"e_1_2_5_6_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199406093302303"},{"key":"e_1_2_5_7_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM200001133420203"},{"key":"e_1_2_5_8_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-3016.2006.00692.x"},{"key":"e_1_2_5_9_1","unstructured":"VichinskyEP.Overview of the clinical manifestations of sickle cell disease[UpToDate Web site]. 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