Abstract
BackgroundAlterations in the structure of haemoglobin (Hb) are usually brought about by point mutations affecting one or, in some cases, two codons encoding amino acids of the globin chains. One in three Ghanaians are said to have sickle cell disorders, whereas malaria continues to be one of the leading causes of mortality among children. This study determined the prevalence of sickle cell disorders and malaria infection among children aged 1–12 years in the Volta Region.MethodsThis was a community-based cross-sectional survey that involved 938 children aged 1–12 years selected from three districts, one each from the 3 geographical zones of the Volta Region using a multistage sampling method. Demographic information was collected using a standard questionnaire and anthropometric indices were measured. Isoelectric focusing (IEF) electrophoresis was used to determine the Hb genotypes and sub-microscopic parasites were determined by PCR.ResultsThe prevalence of sickling screening positive was 16.0% with an overall prevalence of sickle cell disorders being 2.0%. Among the individual genotypes making up the sickle cell disorders, genotype HbSF was the highest (0.9% as compared to 0.2%; HbSS, 0.6%; HbSC and 0.3%; HbSCF). Microscopic Plasmodium falciparum parasitaemia was detected among 5.5% of the children and 14.2% sub-microscopic prevalence by PCR. Children with sickle cell disorders were more likely to have sub-microscopic parasitaemia (AOR = 5.51 95%CI (2.15, 14.10), p < 0.001) as well as anaemia (AOR = 3.03 95% CI (1.04, 8.82), p = 0.042), compared to those with normal genotypes. There was no significant difference observed between sickle cell disorders and growth and development of the children screened.ConclusionsSickle cell disorders were significantly associated with sub-microscopic parasitaemia as well as anaemia in this study. Establishment of sickle cell clinics in the district and regional hospitals will help in the management of children with the disorder and also generate a national database on sickle cell disorders. National neonatal screening policies must also be put in place to help in early detection and management of these disorders.
Highlights
Alterations in the structure of haemoglobin (Hb) are usually brought about by point mutations affect‐ ing one or, in some cases, two codons encoding amino acids of the globin chains
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The resulting valine is a hydrophobic amino acid unlike the glutamic acid, which is hydrophilic. This hydrophobic amino acid interacts with another haemoglobin leading to the polymerization of deoxygenated red blood cell, causing the shape of the normal haemoglobin to be distorted into sickle shaped, which results in the obstruction or occlusion of the blood vessels [5, 6]
Summary
Alterations in the structure of haemoglobin (Hb) are usually brought about by point mutations affect‐ ing one or, in some cases, two codons encoding amino acids of the globin chains. Sickle-cell disorders are types of haemoglobin disorders, which occur due to mutations in one of the globin subunits of haemoglobin (Hb), resulting in a change in amino acid sequence [1]. The sickle haemoglobin (HbS) in sickle cell disorders results from an amino acid substitution at the sixth residue of the β-globin subunit; valine for glutamic acid [1, 4]. The resulting valine is a hydrophobic amino acid unlike the glutamic acid, which is hydrophilic This hydrophobic amino acid interacts with another haemoglobin leading to the polymerization of deoxygenated red blood cell, causing the shape of the normal haemoglobin to be distorted into sickle shaped, which results in the obstruction or occlusion of the blood vessels [5, 6]. Some common complications include frequent attacks of severe pain called crises, acute severe anaemia, malaria and other infections, stroke (especially among children), acute lung damage, development of cerebrovascular disease and cognitive impairment [7, 8]
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