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Children with sickle cell disease who are underweight are at an increased risk of death in low-resource settings. The weight-for-age z-score can be used to screen for at-risk children over 5 years of age with sickle cell anemia. 

Children with sickle cell anemia and undernutrition, especially children aged between 5 and 12 years, may be at an increased risk of mortality in a low-resource setting. In sub-Saharan Africa, sickle cell anemia and undernutrition are common occurrences in children. While therapies have improved the survival of children with sickle cell anemia, there is still a high mortality rate in children with sickle cell anemia in this region.

In a recent study, researchers conducted a secondary analysis of subjects in the Primary Prevention of Stroke in Children with Sickle Cell Disease in Nigeria trial. The double-blind, randomized trial evaluated low-dose and moderate-dose hydroxyurea treatment in children with abnormal transcranial Doppler velocities and non-elevated transcranial Doppler velocities in northern Nigeria. Using the World Health Organization (WHO) growth reference, the nutritional status of the children was analyzed. 

Results of the analysis showed that the mean weight-for-age z-score was lower in children who died by the end of the study versus those who survived. Other baseline characteristics were not significantly different between the children who died and survived. In a pooled analysis, researchers found that being underweight increased the risk of death in children with sickle cell anemia. During treatment follow-up, children who were underweight were found to have a higher mortality rate than children who were not underweight. 

According to the study, underweight status is a risk factor for school-aged children with sickle cell anemia in Nigeria. The weight-for-age z-score can be a convenient and reproducible clinical measure for screening certain children with sickle cell anemia who may be at a greater risk of death. Evidence-based strategies are needed to track, manage, and prevent undernutrition in children with sickle cell anemia. 


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Klein, L. J., Abdullahi, S., Gambo, S., Stallings, V. A., Acra, S., Rodeghier, M., & DeBaun, M. R. (2022). Underweight children over 5 years with sickle cell anemia are at risk for early mortality in a low-resource setting. Blood Adv. https://doi.org/10.1182/bloodadvances.2022008623