Nutritional perspectives on sickle cell disease in Africa: a systematic review

Abstract

Background Sickle cell disease (SCD) is an inherited blood disorder that predominantly affects individuals in sub-Saharan Africa. However, research that elucidates links between SCD pathophysiology and nutritional status in African patients is lacking. This systematic review aimed to assess the landscape of studies in sub-Saharan Africa that focused on nutritional aspects of SCD, and highlights gaps in knowledge that could inform priority-setting for future research. Methods The study was conducted using the Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) guidelines. Inclusion criteria comprised original, peer-reviewed research published between January 1995 and November 2020 involving individuals in Africa with any phenotypic variant of SCD and at least one nutritional status outcome. Nutritional status outcomes were defined as those that assessed dietary intakes, growth/anthropometry, or nutritional biomarkers. Databases used were Ovid Embase, Medline, Biosis and Web of Science. Results The search returned 526 articles, of which 76 were included in the final analyses. Most investigations (67%) were conducted in Nigeria. Studies were categorized into one of three main categories: descriptive studies of anthropometric characteristics (49%), descriptive studies of macro- or micronutrient status (41%), and interventional studies (11%). Findings consistently included growth impairment, especially among children and adolescents from sub-Saharan Africa. Studies assessing macro- and micronutrients generally had small sample sizes and were exploratory in nature. Only four randomized trials were identified, which measured the impact of lime juice, long-chain fatty acids supplementation, ready-to-use supplementary food (RUSF), and oral arginine on health outcomes. Conclusions The findings reveal a moderate number of descriptive studies, most with small sample sizes, that focused on various aspects of nutrition and SCD in African patients. There was a stark dearth of interventional studies that could be used to inform evidence-based changes in clinical practice. Findings from the investigations were generally consistent with data from other regional settings, describing a significant risk of growth faltering and malnutrition among individuals with SCD. There is an unmet need for clinical research to better understand the potential benefits of nutrition-related interventions for patients with SCD in sub-Saharan Africa to promote optimal growth and improve health outcomes.

Relevance of Plasma Homocysteine and Methylenetetrahydrofolate Reductase 677TT Genotype in Sickle Cell Disease: A Systematic Review and Meta-Analysis

“…With regards to VOC, our meta-analysis found a neutral effect size between SCD children and controls, the wide heterogeneity decreasing after removal of the studies from India [ 29 ] and from Nigeria [ 23 , 24 , 53 ], implying that these latter contributed to the heterogeneity, possibly via the same nutritional and vitamin deficiencies alluded to earlier [ 62 ]. It should be noted that in the VOC comparison, SCD patients were unmatched, preventing the capture of the same genetic, oxidative, and nutritional background of the participants before and after crisis, that might have allowed a better interpretation of the results.…”

Describing the growth and nutritional status of sickle cell disease children and adolescents with reference to WHO growth standards in Cameroon

“…Stunting, wasting, being underweight, and micronutrient deficiencies are the four main ways in which undernutrition presents itself [ 11 ]. In Cameroon, few studies have been conducted on the growth and nutritional status of children with SCD [ 12 , 13 ]. Ngo Um Sap et al found a prevalence of underweight of 4% in children with SCD under 5 years of age [ 13 ].…”

Perfil nutricional e sua relação com marcadores de gravidade em adultos com anemia falciforme

“…Sickle cell anemia (SCA), the most prevalent hereditary hemoglobinopathy in the world, with an autosomal recessive character, resulting from a mutation in the β-globin gene, characterized by homozygosity for the βS allele (Nartey et al, 2021;Sunnd et al, 2019;Piccin et al, 2019). In the SCA, the most severe form of sickle cell disease (SCD), the erythrocytes acquire a sickle shape, a cellular expression of the polymerization of deoxygenated hemoglobin S (HbS), a process that leads to hemolysis and adhesion of erythrocytes to the vascular endothelium, which results in obstruction, chronic inflammation and recurrent episodes of blood vessel occlusion (Piccin et al, 2019;Salinas Cisneros & Thein, 2020).…”

“…SCA is a multisystemic pathology, that has high morbidity and mortality rates and is a severe public health problem (Abdul-Hussein et al, 2021;Nartey et al, 2021). It predominantly affects people of African, Indian and Arabic descent, with most cases occurring in Sub-Saharan Africa (Nartey et al, 2021;Inusa et al, 2019).…”

“…SCA is a multisystemic pathology, that has high morbidity and mortality rates and is a severe public health problem (Abdul-Hussein et al, 2021;Nartey et al, 2021). It predominantly affects people of African, Indian and Arabic descent, with most cases occurring in Sub-Saharan Africa (Nartey et al, 2021;Inusa et al, 2019). In this scenario, barriers to a better health care stand out, such as low socioeconomic status and precarious health conditions, which affects the nutritional status and hemoglobin levels of patients with SCD (Animasahun et al, 2011).…”