Sickle cell disease and cardiovascular health in children: a systematic review of early myocardial damage, biomarkers, and preventive strategies in low-resource settings
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Sickle Cell Disease (SCD) is one of the most prevalent inherited hemoglobinopathies worldwide, with sub-Saharan Africa bearing the highest burden. In Nigeria, where an estimated 150,000 children are born annually with SCD, the cardiovascular consequences of chronic hemolytic anemia, recurrent vaso-occlusive episodes, and endothelial dysfunction remain underappreciated. Children with SCD are at heightened risk of myocardial ischemia, silent cardiac damage, and progressive ventricular dysfunction, yet routine surveillance protocols are absent in most Nigerian tertiary health facilities. This systematic review synthesizes current evidence on the prevalence of early myocardial injury in pediatric SCD populations, evaluates the utility of high-sensitivity troponin assays and advanced echocardiographic modalities (speckletracking imaging), and examines the potential role of prophylactic therapies such as hydroxyurea, chronic transfusion regimens, and targeted antioxidant interventions in mitigating silent cardiac events. Particular attention is directed toward knowledge gaps specific to the Nigerian health system, where diagnostic limitations, workforce shortages, and competing infectious disease priorities impede cardiovascular screening. The review highlights that while advanced imaging may not be universally accessible, simple preventive measures - hydroxyurea, L-glutamine, transfusion, aggressive hydration, and infection prevention - can be initiated at the first point of contact even in rural settings. The review concludes with a proposed interdisciplinary framework to guide future implementation research in resource-constrained environments.
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