Abstract
Anthropometric indices are widely used to assess the health and nutritional status of children. We tested the hypothesis that the 2007 World Health Organization (WHO) reference for assessment of malnutrition in children with sickle cell anemia (SCA) overestimates the prevalence of severe malnutrition when compared to a previously constructed SCA-specific reference. We applied the WHO and SCA-specific references to children with SCA aged 5–12 years living in northern Nigeria (Primary Prevention of Stroke in Children with SCA in sub-Saharan Africa (SPRING) trial) to determine the difference in prevalence of severe malnutrition defined as body mass index (BMI) Z-score <−3 and whether severe malnutrition was associated with lower mean hemoglobin levels or abnormal transcranial Doppler measurements (>200 cm/s). A total of 799 children were included in the final analysis (median age 8.2 years (interquartile range (IQR) 6.4–10.4)). The application of the WHO reference resulted in lower mean BMI than the SCA-specific reference (−2.3 versus −1.2; p < 0.001, respectively). The use of the WHO reference when compared to the SCA-specific reference population also resulted in a higher prevalence of severe malnutrition (28.6% vs. 6.4%; p < 0.001). The WHO reference significantly overestimates the prevalence of severe malnutrition in children with SCA when compared to an SCA-specific reference. Regardless of the reference population, severe malnutrition was not associated with lower mean hemoglobin levels or abnormal transcranial Doppler (TCD) measurements.
Highlights
Sickle cell anemia (SCA) is one of the most common monogenic disorders [1] and disproportionately impacts countries in sub-Saharan Africa, like Nigeria
Given the magnitude of SCA coupled with the large number of children with severe malnutrition in sub-Saharan Africa
The prevalence of severe malnutrition in our study according to the SCA-specific growth parameters of children with SCA living in high-income setting was 6% vs. 28% based on the World Health Organization (WHO) reference
Summary
Sickle cell anemia (SCA) is one of the most common monogenic disorders [1] and disproportionately impacts countries in sub-Saharan Africa, like Nigeria. The effects of sickle cell disease (SCD) on nutritional status were first described in the 1950s and 1960s [5,6]. Children with SCD, similar to children with other chronic diseases, are at risk for delayed growth and pubertal maturity [7,8,9]. The largest prospective study of more than 2000 children and young adults with various sickle genotypes describing the natural history of SCD (Cooperative Study of Sickle Cell Disease (CSSCD)) demonstrated that individuals with HbSS and HbSβ0 thalassemia (referred to here as SCA) had lower weights and shorter heights compared to those with HbSC and HbSβ+ thalassemia. A comprehensive review of 46 anthropometric studies (26 cross-sectional and 20 longitudinal) revealed similar findings of growth failure worldwide among individuals with SCD [9]
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