{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,26]],"date-time":"2026-09-26T05:26:27Z","timestamp":1790400387494,"version":"4.1.0"},"reference-count":34,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2020,5,16]],"date-time":"2020-05-16T00:00:00Z","timestamp":1589587200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.springer.com\/tdm"},{"start":{"date-parts":[[2020,5,16]],"date-time":"2020-05-16T00:00:00Z","timestamp":1589587200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/www.springer.com\/tdm"}],"funder":[{"DOI":"10.13039\/100006545","name":"National Institute on Minority Health and Health Disparities","doi-asserted-by":"publisher","award":["T37MD001425"],"award-info":[{"award-number":["T37MD001425"]}],"id":[{"id":"10.13039\/100006545","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["J. Racial and Ethnic Health Disparities"],"published-print":{"date-parts":[[2021,2]]},"DOI":"10.1007\/s40615-020-00762-2","type":"journal-article","created":{"date-parts":[[2020,5,16]],"date-time":"2020-05-16T14:02:35Z","timestamp":1589637755000},"page":"99-106","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":25,"title":["An Analysis of Racial and Ethnic Backgrounds Within the CASiRe International Cohort of Sickle Cell Disease Patients: Implications for Disease Phenotype and Clinical Research"],"prefix":"10.1007","volume":"8","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8829-0043","authenticated-orcid":false,"given":"Andrew D.","family":"Campbell","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Raffaella","family":"Colombatti","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Biree","family":"Andemariam","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Crawford","family":"Strunk","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Immacolata","family":"Tartaglione","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Connie M.","family":"Piccone","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Deepa","family":"Manwani","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eugenia Vicky","family":"Asare","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Donna","family":"Boruchov","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fatimah","family":"Farooq","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rebekah","family":"Urbonya","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gifty Dankwah","family":"Boatemaa","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Silverio","family":"Perrotta","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laura","family":"Sainati","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Angela","family":"Rivers","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sudha","family":"Rao","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"William","family":"Zempsky","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fredericka","family":"Sey","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Catherine","family":"Segbefia","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Baba","family":"Inusa","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Charles","family":"Antwi-Boasiako","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2020,5,16]]},"reference":[{"issue":"22","key":"762_CR1","doi-asserted-by":"publisher","first-page":"4331","DOI":"10.1182\/blood-2010-01-251348","volume":"115","author":"DJ Weatherall","year":"2010","unstructured":"Weatherall DJ. The inherited diseases of hemoglobin are an emerging global health burden. Blood. 2010;115(22):4331\u20136.","journal-title":"Blood"},{"issue":"3","key":"762_CR2","doi-asserted-by":"publisher","first-page":"305","DOI":"10.1056\/NEJMc1705789","volume":"377","author":"FB Piel","year":"2017","unstructured":"Piel FB, Steinberg MH, Rees DC. Sickle cell disease. N Engl J Med. 2017;377(3):305.","journal-title":"N Engl J Med"},{"key":"762_CR3","doi-asserted-by":"publisher","unstructured":"Inusa BPD, Colombatti R. European migration crises: the role of national hemoglobinopathy registries in improving patient access to care. Pediatr Blood Cancer. 2017;64(7). https:\/\/doi.org\/10.1002\/pbc.26515.","DOI":"10.1002\/pbc.26515"},{"issue":"2","key":"762_CR4","doi-asserted-by":"publisher","first-page":"e80","DOI":"10.1016\/S2214-109X(13)70150-5","volume":"2","author":"FB Piel","year":"2014","unstructured":"Piel FB, Tatem AJ, Huang Z, Gupta S, Williams TN, Weatherall DJ. Global migration and the changing distribution of sickle haemoglobin: a quantitative study of temporal trends between 1960 and 2000. Lancet Glob Health. 2014;2(2):e80\u20139.","journal-title":"Lancet Glob Health"},{"issue":"4","key":"762_CR5","doi-asserted-by":"publisher","first-page":"236","DOI":"10.3109\/03630269.2014.927363","volume":"38","author":"TS Akingbola","year":"2014","unstructured":"Akingbola TS, Tayo BO, Salako B, Layden JE, Hsu LL, Cooper RS, et al. Comparison of patients from Nigeria and the USA highlights modifiable risk factors for sickle cell anemia complications. Hemoglobin. 2014;38(4):236\u201343.","journal-title":"Hemoglobin"},{"issue":"1","key":"762_CR6","first-page":"4","volume":"15","author":"SL Saraf","year":"2014","unstructured":"Saraf SL, Molokie RE, Nouraie M, Sable CA, Luchtman-Jones L, Ensing GJ, et al. Differences in the clinical and genotypic presentation of sickle cell disease around the world. Paediatr Respir Rev. 2014;15(1):4\u201312.","journal-title":"Paediatr Respir Rev"},{"issue":"2","key":"762_CR7","doi-asserted-by":"publisher","first-page":"210","DOI":"10.1111\/j.1365-2141.2006.05986.x","volume":"133","author":"AK Njamnshi","year":"2006","unstructured":"Njamnshi AK, Wonkam A, Djientcheu VP, Ongolo-Zogo P, Obama MT, Muna WFT, et al. Stroke may appear to be rare in Saudi-Arabian and Nigerian children with sickle cell disease, but not in Cameroonian sickle cell patients. Br J Haematol. 2006;133(2):210 author reply 211.","journal-title":"Br J Haematol"},{"key":"762_CR8","doi-asserted-by":"publisher","first-page":"36","DOI":"10.1186\/1824-7288-39-36","volume":"39","author":"M Montanaro","year":"2013","unstructured":"Montanaro M, Colombatti R, Pugliese M, Migliozzi C, Zani F, Guerzoni M, et al. Intellectual function evaluation of first generation immigrant children with sickle cell disease: the role of language and sociodemographic factors. Ital J Pediatr. 2013;39:36.","journal-title":"Ital J Pediatr"},{"issue":"4","key":"762_CR9","doi-asserted-by":"publisher","first-page":"626","DOI":"10.3324\/haematol.2016.153791","volume":"102","author":"P Maitra","year":"2017","unstructured":"Maitra P, Caughey M, Robinson L, Desai PC, Jones S, Nouraie M, et al. Risk factors for mortality in adult patients with sickle cell disease: a meta-analysis of studies in North America and Europe. Haematologica. 2017;102(4):626\u201336.","journal-title":"Haematologica"},{"issue":"3","key":"762_CR10","doi-asserted-by":"publisher","first-page":"225","DOI":"10.1111\/tme.12314","volume":"26","author":"Y Zheng","year":"2016","unstructured":"Zheng Y, Maitta RW. Alloimmunisation rates of sickle cell disease patients in the United States differ from those in other geographical regions. Transfus Med. 2016;26(3):225\u201330.","journal-title":"Transfus Med"},{"issue":"2","key":"762_CR11","doi-asserted-by":"publisher","first-page":"97","DOI":"10.5853\/jos.2014.16.2.97","volume":"16","author":"R Bavarsad Shahripour","year":"2014","unstructured":"Bavarsad Shahripour R, Mortazavi MM, Barlinn K, Keikhaei B, Mousakhani H, Azarpazhooh MR, et al. Can STOP trial velocity criteria be applied to Iranian children with sickle cell disease? J Stroke. 2014;16(2):97\u2013101.","journal-title":"J Stroke"},{"issue":"6","key":"762_CR12","doi-asserted-by":"publisher","first-page":"966","DOI":"10.1111\/bjh.13916","volume":"172","author":"J Sommet","year":"2016","unstructured":"Sommet J, Alberti C, Couque N, Verlhac S, Haouari Z, Mohamed D, et al. Clinical and haematological risk factors for cerebral macrovasculopathy in a sickle cell disease newborn cohort: a prospective study. Br J Haematol. 2016;172(6):966\u201377.","journal-title":"Br J Haematol"},{"key":"762_CR13","first-page":"25","volume":"11","author":"KC Duru","year":"2015","unstructured":"Duru KC, Noble JA, Guindo A, Yi L, Imumorin IG, Diallo DA, et al. Extensive genomic variability of knops blood group polymorphisms is associated with sickle cell disease in Africa. Evol Bioinformatics Online. 2015;11:25\u201333.","journal-title":"Evol Bioinformatics Online"},{"key":"762_CR14","doi-asserted-by":"publisher","DOI":"10.7717\/peerj.799","volume":"3","author":"JA Noble","year":"2015","unstructured":"Noble JA, Duru KC, Guindo A, Yi L, Imumorin IG, Diallo DA, et al. Interethnic diversity of the CD209 (rs4804803) gene promoter polymorphism in African but not American sickle cell disease. PeerJ. 2015;3:e799.","journal-title":"PeerJ"},{"issue":"6","key":"762_CR15","doi-asserted-by":"publisher","first-page":"1058","DOI":"10.1111\/bjh.15250","volume":"184","author":"J Kanter","year":"2019","unstructured":"Kanter J, Heath LE, Knorr J, Agbenyega ET, Colombatti R, Dampier C, et al. Novel findings from the multinational DOVE study on geographic and age-related differences in pain perception and analgesic usage in children with sickle cell anaemia. Br J Haematol. 2019;184(6):1058\u201361.","journal-title":"Br J Haematol"},{"key":"762_CR16","unstructured":"Health, N.I.O. National Institutes of Health (2001) NIH policy and guidelines on the inclusion of women and minorities as subjects in clinical research 2001; Available from: https:\/\/grants.nih.gov\/policy\/inclusion\/women-and-minorities.htm. Accessed 28 Nov 2017."},{"key":"762_CR17","unstructured":"National Center on Birth Defects and Developmental Disabilities, C.f.D.C.a.P. 2019; Available from: https:\/\/www.cdc.gov\/ncbddd\/sicklecell\/data.html. Accessed 21 Oct 2019."},{"issue":"19","key":"762_CR18","doi-asserted-by":"publisher","first-page":"2100","DOI":"10.1182\/blood-2018-09-876508","volume":"133","author":"L De Franceschi","year":"2019","unstructured":"De Franceschi L, et al. Access to emergency departments for acute events and identification of sickle cell disease in refugees. Blood. 2019;133(19):2100\u20133.","journal-title":"Blood"},{"key":"762_CR19","doi-asserted-by":"publisher","unstructured":"Inusa BPD, et al. Geographic differences in phenotype and treatment of children with sickle cell anemia from the multinational DOVE study. J Clin Med. 2019;8(11). https:\/\/doi.org\/10.3390\/jcm8112009.","DOI":"10.3390\/jcm8112009"},{"issue":"9","key":"762_CR20","doi-asserted-by":"publisher","first-page":"e271","DOI":"10.1371\/journal.pmed.0040271","volume":"4","author":"L Braun","year":"2007","unstructured":"Braun L, Fausto-Sterling A, Fullwiley D, Hammonds EM, Nelson A, Quivers W, et al. Racial categories in medical practice: how useful are they? PLoS Med. 2007;4(9):e271.","journal-title":"PLoS Med"},{"issue":"12","key":"762_CR21","doi-asserted-by":"publisher","first-page":"1253","DOI":"10.15537\/smj.2018.12.23113","volume":"39","author":"EY Moussa","year":"2018","unstructured":"Moussa EY, Yassine NM, Borjac JM. New variants in beta globin gene among the Palestinian refugees with sickle cell disease in Lebanon. Saudi Med J. 2018;39(12):1253\u20138.","journal-title":"Saudi Med J"},{"key":"762_CR22","doi-asserted-by":"publisher","first-page":"115","DOI":"10.1016\/j.actatropica.2018.02.030","volume":"182","author":"A Nasr","year":"2018","unstructured":"Nasr A, Saleh AM, Eltoum M, Abushouk A, Hamza A, Aljada A, et al. Antibody responses to P. falciparum apical membrane antigen 1(AMA-1) in relation to haemoglobin S (HbS), HbC, G6PD and ABO blood groups among Fulani and Masaleit living in Western Sudan. Acta Trop. 2018;182:115\u201323.","journal-title":"Acta Trop"},{"issue":"6","key":"762_CR23","doi-asserted-by":"publisher","first-page":"2016","DOI":"10.1111\/trf.15197","volume":"59","author":"LA Boateng","year":"2019","unstructured":"Boateng LA, Campbell AD, Davenport RD, Osei-Akoto A, Hugan S, Asamoah A, et al. Red blood cell alloimmunization and minor red blood cell antigen phenotypes in transfused Ghanaian patients with sickle cell disease. Transfusion. 2019;59(6):2016\u201322.","journal-title":"Transfusion"},{"key":"762_CR24","doi-asserted-by":"publisher","first-page":"192","DOI":"10.1016\/j.dib.2016.11.082","volume":"10","author":"I Armenis","year":"2017","unstructured":"Armenis I, Kalotychou V, Tzanetea R, Kollia P, Kontogeorgiou Z, Anastasopoulou D, et al. Data on eNOS T786 and G894T polymorphisms and peripheral blood eNOS mRNA levels in sickle cell disease. Data Brief. 2017;10:192\u20137.","journal-title":"Data Brief"},{"issue":"2","key":"762_CR25","doi-asserted-by":"publisher","first-page":"75","DOI":"10.1016\/j.hemonc.2017.09.002","volume":"11","author":"RA Afifi","year":"2018","unstructured":"Afifi RA, et al. CD209-336A\/G promotor polymorphism and its clinical associations in sickle cell disease Egyptian pediatric patients. Hematol Oncol Stem Cell Ther. 2018;11(2):75\u201381.","journal-title":"Hematol Oncol Stem Cell Ther"},{"key":"762_CR26","doi-asserted-by":"publisher","first-page":"17","DOI":"10.1016\/j.niox.2016.11.002","volume":"62","author":"I Armenis","year":"2017","unstructured":"Armenis I, Kalotychou V, Tzanetea R, Kollia P, Kontogeorgiou Z, Anastasopoulou D, et al. Prognostic value of T786C and G894T eNOS polymorphisms in sickle cell disease. Nitric Oxide. 2017;62:17\u201323.","journal-title":"Nitric Oxide"},{"issue":"1","key":"762_CR27","first-page":"35","volume":"13","author":"RAA Afifi","year":"2019","unstructured":"Afifi RAA, Sedky YM, Abd-ELKareem H, Botros SKA. IL-Ibeta+3954 C\/T polymorphism and its clinical associations in Egyptian sickle cell disease patients. Int J Hematol Oncol Stem Cell Res. 2019;13(1):35\u201341.","journal-title":"Int J Hematol Oncol Stem Cell Res"},{"key":"762_CR28","doi-asserted-by":"publisher","unstructured":"Antwi-Boasiako C, et al. Association between eNOS gene polymorphism (T786C and VNTR) and sickle cell disease patients in Ghana. Diseases. 2018;6(4). https:\/\/doi.org\/10.3390\/diseases6040090.","DOI":"10.3390\/diseases6040090"},{"issue":"1","key":"762_CR29","doi-asserted-by":"publisher","first-page":"8874","DOI":"10.1038\/s41598-019-45081-7","volume":"9","author":"F Pereira","year":"2019","unstructured":"Pereira F, et al. A systematic literature review on the European, African and Amerindian genetic ancestry components on Brazilian health outcomes. Sci Rep. 2019;9(1):8874.","journal-title":"Sci Rep"},{"key":"762_CR30","doi-asserted-by":"publisher","unstructured":"Wonkam A, Makani J. Sickle cell disease in Africa: an urgent need for longitudinal cohort studies. Lancet Glob Health. 2019;7(10):e1310\u2013e1311. https:\/\/doi.org\/10.1016\/S2214-109X(19)30364-X.","DOI":"10.1016\/S2214-109X(19)30364-X"},{"issue":"5","key":"762_CR31","doi-asserted-by":"publisher","first-page":"429","DOI":"10.1056\/NEJMoa1611770","volume":"376","author":"KI Ataga","year":"2017","unstructured":"Ataga KI, Kutlar A, Kanter J, Liles D, Cancado R, Friedrisch J, et al. Crizanlizumab for the prevention of pain crises in sickle cell disease. N Engl J Med. 2017;376(5):429\u201339.","journal-title":"N Engl J Med"},{"issue":"7","key":"762_CR32","doi-asserted-by":"publisher","first-page":"625","DOI":"10.1056\/NEJMoa1512021","volume":"374","author":"MM Heeney","year":"2016","unstructured":"Heeney MM, Hoppe CC, Abboud MR, Inusa B, Kanter J, Ogutu B, et al. A multinational trial of prasugrel for sickle cell vaso-occlusive events. N Engl J Med. 2016;374(7):625\u201335.","journal-title":"N Engl J Med"},{"key":"762_CR33","doi-asserted-by":"publisher","first-page":"10","DOI":"10.1016\/j.scr.2018.09.011","volume":"33","author":"GLS Martins","year":"2018","unstructured":"Martins GLS, Paredes BD, Azevedo CM, Sampaio GLDA, Nonaka CKV, Cavalcante BRR, et al. Generation of integration-free iPS cell lines from three sickle cell disease patients from the state of Bahia, Brazil. Stem Cell Res. 2018;33:10\u20134.","journal-title":"Stem Cell Res"},{"key":"762_CR34","doi-asserted-by":"publisher","first-page":"509","DOI":"10.1056\/NEJMoa1903212","volume":"381","author":"E Vichinsky","year":"2019","unstructured":"Vichinsky E, Hoppe CC, Ataga KI, Ware RE, Nduba V, el-Beshlawy A, et al. A phase 3 randomized trial of Voxelotor in sickle cell disease. N Engl J Med. 2019;381:509\u201319.","journal-title":"N Engl J Med"}],"container-title":["Journal of Racial and Ethnic Health Disparities"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s40615-020-00762-2.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s40615-020-00762-2\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s40615-020-00762-2.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,5,15]],"date-time":"2021-05-15T19:28:50Z","timestamp":1621106930000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1007\/s40615-020-00762-2"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,5,16]]},"references-count":34,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2021,2]]}},"alternative-id":["762"],"URL":"https:\/\/doi.org\/10.1007\/s40615-020-00762-2","relation":{},"ISSN":["2197-3792","2196-8837"],"issn-type":[{"value":"2197-3792","type":"print"},{"value":"2196-8837","type":"electronic"}],"subject":[],"published":{"date-parts":[[2020,5,16]]},"assertion":[{"value":"19 January 2020","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"6 April 2020","order":2,"name":"revised","label":"Revised","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"13 April 2020","order":3,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"16 May 2020","order":4,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Compliance with Ethical Standards"}},{"value":"A Campbell: research funding and consultancy from Global Blood Therapeutics (GBT), Novartis, Cyclerion; consultancy from BluebirdBio; D Manwani: research funding from Grifols; consultancy for Novartis, Pfizer, Global Blood Therapeutics; B Andemariam: consultancy for Novartis, Pfizer, NovoNordisk, Emmaus, Cyclerion, Terumo, Sanofi; DSMB: Global Blood Therapeutics; research funding: Imara; B Inusa: education funding: Novartis AstraZeneca, Global Blood Therapeutics, Celgene, Vertex; C. Strunk: consultancy Global Blood Therapeutics and Novartis; R Colombatti: research funding: Global Blood Therapeutics, Novartis. No disclosures to declare from the other co-authors.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflict of Interest"}},{"value":"Except at two sites (Guys and St Thomas Hospital, UK; Azienda Ospedaliera-Universit\u00e0 di Padova, Italy) that had a previously Institutional Review Board\u2013approved Sickle Cell Disease Registry, Institutional Review Board approval was obtained at each site and written informed consent was obtained by each patient and\/or parent\/guardian.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Informed Consent and Research Involving Human Participants and\/or Animals"}}]}}