| Autor: |
Campbell AD; Division of Pediatric Hematology/Oncology, University of Michigan, Ann Arbor, MI, USA. acampbell@childrensnational.org.; Center for Cancer and Blood Disorders, Children's National Medical Center, Washington, DC, USA. acampbell@childrensnational.org., Colombatti R; Clinic of Pediatric Hematology Oncology, Department of Women's and Child Health, Azienda Ospedaliera-Università di Padova, Padova, Italy., Andemariam B; Division of Hematology-Oncology, New England Sickle Cell Institute, Neag Comprehensive Cancer Center, UCONN Health, University of Connecticut, Farmington, CT, USA., Strunk C; ProMedica Russell J. Ebeid Children's Hospital, Toledo, OH, USA., Tartaglione I; Department of Women, Child and General and Specialized Surgery, University of Campania 'Luigi Vanvitelli', Naples, Italy., Piccone CM; Pediatric Hematology/Oncology, Rainbow Babies and Children's Hospital, Cleveland, OH, USA., Manwani D; Department of Pediatrics, Albert Einstein College of Medicine, Children's Hospital at Montefiore, Bronx, NY, USA., Asare EV; Ghana Institute of Clinical Genetics, Korle Bu Teaching Hospital, Accra, Ghana.; Department of Hematology, Korle-Bu Teaching Hospital, Accra, Ghana., Boruchov D; Department of Pediatrics, Connecticut Children's Medical Center, Hartford, CT, USA., Farooq F; Division of Pediatric Hematology/Oncology, University of Michigan, Ann Arbor, MI, USA., Urbonya R; Division of Pediatric Hematology/Oncology, University of Michigan, Ann Arbor, MI, USA., Boatemaa GD; Department of Physiology, University of Ghana Medical School, Accra, Ghana., Perrotta S; Department of Women, Child and General and Specialized Surgery, University of Campania 'Luigi Vanvitelli', Naples, Italy., Sainati L; Clinic of Pediatric Hematology Oncology, Department of Women's and Child Health, Azienda Ospedaliera-Università di Padova, Padova, Italy., Rivers A; Division of Pediatric Hematology/Oncology, University of Illinois-Chicago, Chicago, IL, USA., Rao S; Department of Child Health, Korle Bu Teaching Hospital, Accra, Ghana., Zempsky W; Department of Pediatrics, Connecticut Children's Medical Center, Hartford, CT, USA., Sey F; Ghana Institute of Clinical Genetics, Korle Bu Teaching Hospital, Accra, Ghana., Segbefia C; Department of Child Health, Korle Bu Teaching Hospital, Accra, Ghana., Inusa B; Department of Pediatric Haematology, Evelina Children's Hospital, Guy's and St. Thomas NHS Trust, London, UK., Antwi-Boasiako C; Department of Physiology, University of Ghana Medical School, Accra, Ghana. |
| Abstrakt: |
Millions are affected by sickle cell disease (SCD) worldwide with the greatest burden in sub-Saharan Africa. While its origin lies historically within the malaria belt, ongoing changes in migration patterns have shifted the burden of disease resulting in a global public health concern. We created the Consortium for the Advancement of Sickle Cell Research (CASiRe) to understand the different phenotypes of SCD across 4 countries (USA, UK, Italy, and Ghana). Here, we report the multi-generational ethnic and racial background of 877 SCD patients recruited in Ghana (n = 365, 41.6%), the USA (n = 254, 29%), Italy (n = 81, 9.2%), and the UK (n = 177, 20.2%). West Africa (including Benin Gulf) (N = 556, 63.4%) was the most common geographic region of origin, followed by North America (N = 184, 21%), Caribbean (N = 51, 5.8%), Europe (N = 27, 3.1%), Central Africa (N = 24, 2.7%), and West Africa (excluding Benin Gulf) (N = 21, 2.4%). SCD patients in Europe were primarily West African (73%), European (10%), Caribbean (8%), and Central African (8%). In the USA, patients were largely African American (71%), Caribbean (13%), or West African (10%). Most subjects identified themselves as Black or African American; the European cohort had the largest group of Caucasian SCD patients (8%), including 21% of the Italian patients. This is the first report of a comprehensive analysis of ethnicity within an international, transcontinental group of SCD patients. The diverse ethnic backgrounds observed in our cohort raises the possibility that genetic and environmental heterogeneity within each SCD population subgroup can affect the clinical phenotype and research outcomes. |
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