TY - JOUR
T1 - A study of the geographic distribution and associated risk factors of leg ulcers within an international cohort of sickle cell disease patients
T2 - the CASiRe group analysis
AU - Antwi-Boasiako, Charles
AU - Andemariam, Biree
AU - Colombatti, Raffaella
AU - Asare, Eugenia Vicky
AU - Strunk, Crawford
AU - Piccone, Connie M.
AU - Manwani, Deepa
AU - Boruchov, Donna
AU - Farooq, Fatimah
AU - Urbonya, Rebekah
AU - Wilson, Samuel
AU - Boatemaa, Gifty Dankwah
AU - Perrotta, Silverio
AU - Sainati, Laura
AU - Rivers, Angela
AU - Rao, Sudha
AU - Zempsky, William
AU - Ekem, Ivy
AU - Sey, Fredericka
AU - Segbefia, Catherine
AU - Inusa, Baba
AU - Tartaglione, Immacolata
AU - Campbell, Andrew D.
N1 - Publisher Copyright:
© 2020, Springer-Verlag GmbH Germany, part of Springer Nature.
PY - 2020/9/1
Y1 - 2020/9/1
N2 - 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 mass index were also associated with leg ulceration. Overall, our study identified a predominance of leg ulcers within male hemoglobin SS patients living in sub-Saharan Africa with renal dysfunction and increased hemolysis.
AB - 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 mass index were also associated with leg ulceration. Overall, our study identified a predominance of leg ulcers within male hemoglobin SS patients living in sub-Saharan Africa with renal dysfunction and increased hemolysis.
KW - Hemolysis
KW - International
KW - Leg ulcers
KW - Sickle cell
UR - http://www.scopus.com/inward/record.url?scp=85085081494&partnerID=8YFLogxK
U2 - 10.1007/s00277-020-04057-8
DO - 10.1007/s00277-020-04057-8
M3 - Article
C2 - 32377816
AN - SCOPUS:85085081494
SN - 0939-5555
VL - 99
SP - 2073
EP - 2079
JO - Annals of Hematology
JF - Annals of Hematology
IS - 9
ER -