A study of the geographic distribution and associated risk factors of leg ulcers within an international cohort of sickl
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ORIGINAL ARTICLE
A study of the geographic distribution and associated risk factors of leg ulcers within an international cohort of sickle cell disease patients: the CASiRe group analysis Charles Antwi-Boasiako 1 & Biree Andemariam 2 & Raffaella Colombatti 3 & Eugenia Vicky Asare 4 & Crawford Strunk 5 & Connie M. Piccone 6 & Deepa Manwani 7 & Donna Boruchov 8 & Fatimah Farooq 9 & Rebekah Urbonya 9 & Samuel Wilson 9 & Gifty Dankwah Boatemaa 1 & Silverio Perrotta 10 & Laura Sainati 3 & Angela Rivers 11 & Sudha Rao 12 & William Zempsky 8 & Ivy Ekem 13 & Fredericka Sey 14 & Catherine Segbefia 15 & Baba Inusa 16 & Immacolata Tartaglione 10 & Andrew D. Campbell 9,17 Received: 13 January 2020 / Accepted: 25 April 2020 # Springer-Verlag GmbH Germany, part of Springer Nature 2020
Abstract Vasculopathy is a hallmark of sickle cell disease ultimately resulting in chronic end organ damage. Leg ulcer is one of its sequelae, occurring in ~ 5–10% of adult sickle cell patients. The majority of leg ulcer publications to date have emanated from single center cohort studies. As such, there are limited studies on the geographic distribution of leg ulcers and associated risk factors worldwide. The Consortium for the Advancement of Sickle Cell Research (CASiRe) was formed to improve the understanding of the different phenotypes of sickle cell disease patients living in different geographic locations around the world (USA, UK, Italy, Ghana). This cross-sectional cohort sub-study of 659 sickle cell patients aimed to determine the geographic distribution and risk factors associated with leg ulcers. The prevalence of leg ulcers was 10.3% and was associated with older age, SS genotype, male gender, and Ghanaian origin. In fact, the highest prevalence (18.6%) was observed in Ghana. Albuminuria, proteinuria, increased markers of hemolysis (lower hemoglobin, higher total bilirubin), lower oxygen saturation, and lower body
* Andrew D. Campbell [email protected] 1
Department of Physiology, University of Ghana Medical School, Accra, Ghana
2
New England Sickle Cell Institute, Division of Hematology-Oncology, Neag Comprehensive Cancer Center, UCONN Health, University of Connecticut, Farmington, CT, USA
3
Clinic of Pediatric Hematology Oncology, Department of Women’s and Child Health, Azienda Ospedaliera-Università di Padova, Padova, Italy
9
Division of Pediatric Hematology/Oncology, University of Michigan, Ann Arbor, MI, USA
10
Department of Women, Child and General and Specialized Surgery, University of Campania “Luigi Vanvitelli”, Naples, Italy
11
Division of Pediatric Hematology/Oncology, University of IllinoisChicago, Chicago, IL, USA
12
Department of Child Health, Korle Bu Teaching Hospital, Accra, Ghana
13
Department of Haematology, University of Cape Coast, Cape Coast, Ghana
14
Ghana Institute of Clinical Genetics, Korle Bu Teaching Hospital, Accra, Ghana
15
Department of Child Health, Korle Bu Teaching Hospital, University of Ghana Medical School, Accra, Ghana
4
Department of Hematology, Korle-Bu Teachin
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