Age of first pain crisis and associated complications in the CASiRe international sickle cell disease cohort

Show simple item record Tartaglione, I. Strunk, C. Antwi-Boasiako, C. Andemariam, B. Colombatti, R. Asare, E.V. Piccone, C.M. Manwani, D. Boruchov, D. Tavernier, F. Farooq, F. Akatue, S. Oteng, B. Urbonya, R. Wilson, S. Owda, A. Bamfo, R. Boatemaa, G.D. Rao, Sudha Zempsky, W. Sey, F. Inusa, B.P.D. Perrotta, Silverio Segbefia, C. Campbell, A.D. 2021-04-06T17:34:59Z 2021-04-06T17:34:59Z 2021
dc.description Research Article en_US
dc.description.abstract Pain is a hallmark of Sickle Cell Disease (SCD) affecting patients throughout their life; the first pain crisis may occur at any age and is often the first presentation of the disease. Universal newborn screening identifies children with SCD at birth, significantly improving morbidity and mortality. Without early screening, diagnosis is generally made after disease manifestations appear. The Consortium for the Advancement of Sickle Cell Research (CASiRe) is an international collaborative group evaluating the clinical severity of subjects with SCD using a validated questionnaire and medical chart review, standardized across 4 countries (United States, United Kingdom, Italy and Ghana). We investigated the age of first pain crisis in 555 sickle cell subjects, 344 adults and 211 children. Median age of the first crisis in the whole group was 4 years old, 5 years old among adults and 2 years old among children. Patients from the United States generally reported the first crisis earlier than Ghanaians. Experiencing the first pain crisis early in life correlated with the genotype and disease severity. Early recognition of the first pain crisis could be useful to guide counseling and management of the disease. en_US
dc.language.iso en en_US
dc.publisher Blood Cells, Molecules and Diseases en_US
dc.subject Sickle cell disease en_US
dc.subject Pain crisis en_US
dc.subject Newborn screening en_US
dc.subject Complications en_US
dc.title Age of first pain crisis and associated complications in the CASiRe international sickle cell disease cohort en_US
dc.type Article en_US

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