Neurologic complications of sickle cell disease in Africa

Abstract

Objective:To summarize prevalence data on the neurologic complications of sickle cell disease (SCD) in Africa.Methods:We searched EMBASE, PubMed, and African Index Medicus to identify all relevant articles published from inception to May 31, 2016. Each study was reviewed for methodologic quality. A random-effects model was used to estimate the prevalence of neurologic complications of SCD across studies.Results:Thirty-one studies were included. Methodologic quality was high or moderate in 90% of studies. Stroke, conditional and abnormal cerebral blood flow, seizures, and headache were the complications most frequently reported, with overall prevalence rates of 4.2%, 10.6%, 6.1%, 4.4%, and 18.9%, respectively. Some complications, like silent brain infarcts, peripheral neuropathies, neurocognitive deficits, or moyamoya disease, have been rarely or not studied at all in the African setting. Incidence data were scarce and of poor quality.Conclusions:The burden of neurologic complications of SCD is important in Africa and most likely underestimated. A better evaluation of this burden requires larger prospective studies using standard up-to-date screening methods. Accessibility to diagnostic tools such as neuroimaging, transcranial Doppler, EEG, and neuropsychological evaluation, as well as to preventive and therapeutic interventions and trained health care providers, should be improved in routine clinical practice.

Stroke Prevalence in Children With Sickle Cell Disease in Sub-Saharan Africa: A Systematic Review and Meta-Analysis

“…Our estimate of stroke prevalence is similar to that reported in a recent review of SCD stroke combining children and adults across Africa. 12 Similar to our findings, they also reported a higher stroke prevalence in studies utilizing diagnostic imaging and clear diagnostic criteria. Our systematic review and meta-analysis summarize what is known about pediatric SCD stroke in SSA.…”

“…A recent study reviewing neurologic complications of SCD in Africa highlights the interest in this area of research. 12 , 13 …”

Sickle cell disease, sickle trait and the risk for venous thromboembolism: a systematic review and meta-analysis

“…When deoxygenated, hemoglobin S (HbS) polymerizes to form a fibrous network responsible for red cell rigidity, hemolysis, increased blood viscosity, poor microvascular blood flow and vessel occlusion [ 3 ]. Acute and chronic vessel occlusion could cause significant complications in various organs including the brain (strokes or silent brain infarcts), the kidneys (renal infarction with papillary necrosis or medullar fibrosis), the bones (aseptic osteonecrosis, pain crises), the spleen (spleen infarcts), the retina (retinopathy), the lungs (acute chest syndrome) or the male external genitalia (priapism) [ 3 – 5 ]. Some studies have also suggested an increased risk of venous thromboembolism (VTE) in patients with SCD or sickle cell trait (SCT) [ 6 – 9 ].…”

Annual stroke incidence in Nigerian children with sickle cell disease and elevated TCD velocities treated with hydroxyurea

“…Sickle cell disease (SCD) remains the leading cause of stroke in Black children. 1,2 Nigeria has the largest burden of SCD in the world, with about 150,000 babies born annually with the disorder. 3,4 Hospitalbased studies have reported stroke prevalence rates of 6-8% in Nigerian children with SCD.…”