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The epidemiology of sickle cell disease in children recruited in infancy in Kilifi, Kenya: A prospective cohort study

The Lancet Global Health Sep 23, 2019

Uyoga S, Macharia AW, Mochamah G, et al. - Through a prospective cohort study conducted on members of the Kilifi Genetic Birth Cohort Study (KGBCS) on the Indian Ocean coast of Kenya, researchers defined the comparative incidence of specific clinical outcomes among 15,737 children aged between birth and 5 years with and without sickle cell disease, who lived within the Kilifi area of Kenya. In children with sickle cell disease vs those without sickle cell disease, mortality was higher. Among children with sickle cell disease, in those who recruited at the clinic and in those with higher levels of hemoglobin F (HbF), mortality was lower. The incidence of admission to hospital was also greater in children with sickle cell disease in comparison with the children without sickle cell disease. Severe anemia was the most prevalent cause for admission to hospital among those with sickle cell disease. In those with an enrollment HbF level above the median and those who were homozygous for α-thalassemia, admission to hospital was lower. However, in young children with sickle cell disease in this Kenyan cohort, morbidity and mortality were high, both were decreased by early diagnosis and supportive care. The weight must now move in the direction of early discovery and prevention of long-term complications of sickle cell disease.
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