![]() The improvements in outcomes have been attributed in part to newborn screening and comprehensive care programs designed to prevent disease complications to the extent possible and to treat complications of disease when they occur. In high resource countries, mortality from SCD has decreased dramatically over the past five decades. In sub-Saharan Africa, it has been estimated that up to 90% of children born with SCD die before 5 years of age. The disease’s clinical hallmarks include acute painful crises and severe anemia. Since these processes can take place anywhere in the body, the disease is highly complex and characterized by multiple potential life-threatening complications that include acute splenic sequestration, aplastic crises, acute chest syndrome, infection, heart failure, and stroke. When this occurs, erythrocytes assume a misshapen and rigid form that promotes pathological processes leading to intravascular inflammation and occlusion of small blood vessels. The result is a change in the structure and dynamics of hemoglobin such that certain conditions including deoxygenation and acidosis predispose to hemoglobin polymerization. The condition derives from a point mutation of the β-globin gene found on the short arm of chromosome 11 through which the hydrophilic amino acid glutamic acid is substituted with the hydrophobic amino acid valine at the sixth position. Sickle cell disease (SCD) is the most common inherited blood disease worldwide, with the vast majority of cases occurring in sub-Saharan Africa. 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. 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 was a stark dearth of interventional studies that could be used to inform evidence-based changes in clinical practice. 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. ![]() 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. Studies assessing macro- and micronutrients generally had small sample sizes and were exploratory in nature. Findings consistently included growth impairment, especially among children and adolescents from sub-Saharan Africa. 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%). Most investigations (67%) were conducted in Nigeria. The search returned 526 articles, of which 76 were included in the final analyses. ![]() Databases used were Ovid Embase, Medline, Biosis and Web of Science. Nutritional status outcomes were defined as those that assessed dietary intakes, growth/anthropometry, or nutritional biomarkers. 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. The study was conducted using the Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) guidelines. ![]() 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. However, research that elucidates links between SCD pathophysiology and nutritional status in African patients is lacking. Sickle cell disease (SCD) is an inherited blood disorder that predominantly affects individuals in sub-Saharan Africa.
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