Abstract
BackgroundThe prevalence of Sickle cell disease is extremely high in Democratic Republic of Congo. Despite this high prevalence of the disease, data on renal abnormalities in children are rare.MethodThe study proposed to assess blood pressure, glomerular function, urea and uric acid levels in 65 steady state Congolese children with homozygous sickle cell disease and 67 normal controls.ResultsIn Hb-SS group, blood pressure level tended to be lower than Hb-AA groups but there was no statistically significant difference (p>0.05) between the two groups. The absolute values for GFR corrected for BSA were significantly higher in Hb-SS group compared to Hb-AA group (130.5±34.1 ml/min/1.73 m2 vs 113.7±24.5 ml/min/1.73 m2; p = 0.004). Children with Hb-SS were more likely to hyperfiltrate (30.8% of subjects) than children with Hb-AA (6.1% of subjects). Proteinuria was found in 4 (6.2%) children with Hb-SS. Uric acid level was significantly increased in children with Hb-SS compared to corresponding values in control group (4.4±1.3 mg/dl vs 3.5±1.1 mg/dl; p<0.001). Urea level was significantly decreased compared to corresponding values in Hb-AA group (15.3±8.3 mg/dl vs 22.9±10.1 mg/dl; p<0.001).ConclusionHyperfiltration, low creatinine, lower urea and high uric acid are more common in children with sickle cell disease than in normal controls.
Highlights
Sickle cell disease (SCD) is an autosomal recessive genetic condition due to a mutation in the beta-globin gene resulting in replacement of glutamic acid in position 6 of the beta-globin chain by valine resulting in an abnormal haemoglobin HbS molecule
Blood pressure In Hb-SS group, systolic and diastolic blood pressure level tended to be lower than Hb-AA groups
The absolute values for Glomerular Filtration Rate (GFR) corrected for body surface area (BSA) were significantly higher in Hb-SS group compared to Hb-AA group (Table-3)
Summary
Sickle cell disease (SCD) is an autosomal recessive genetic condition due to a mutation in the beta-globin gene resulting in replacement of glutamic acid in position 6 of the beta-globin chain by valine resulting in an abnormal haemoglobin HbS molecule. The sickle cell genes occur commonly in areas of the world with intense malaria transmission. Democratic Republic of Congo (DRC) has the second highest population of SCD patients in the whole world after Nigeria. The prevalence of SCD is extremely high with 25 to 30% of sickle cell trait carrier in the general population. The prevalence of Sickle cell disease is extremely high in Democratic Republic of Congo. Despite this high prevalence of the disease, data on renal abnormalities in children are rare
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