Abstract
Background: Sickle cell anaemia (SCA) is a disorder of Mendelian autosomal recessive inheritance, characterised by abnormal haemoglobin synthesis resulting in multi-systemic manifestations. The kidneys are largely affected by this disorder, but overt features of kidney disease mostly manifest after the second decade, even though insult and sub-clinical features may occur during childhood. Unfortunately, investigating these sub-clinical features is not routinely done in resource-scarce settings, partly due to the low socioeconomic status of most of our patients and the overwhelmed health care workers. Objectives: To investigate glomerular dysfunction in children with SCA in the context of the resource-poor setting. Methodology: This cross-sectional study was conducted at the University of Maiduguri Teaching Hospital (UMTH), over 6 months. One hundred and ten SCA (Hb SS) children aged 3 – 14 years in steady-state constituted the cases, while 110 non-SCA (Hb AA) age and sex-matched, apparently healthy children formed the control. Anthropometry, blood pressure, urinalysis and serum creatinine of the subjects was done. Glomerular filtration rate (GFR) was estimated using the Schwartz formula. Results: The mean systolic blood pressure (SBP) ± SD of the cases and controls were 96.8±9.34mmHg and 99.14±13.44mmHg respectively, (p = 0.13). The mean diastolic BP ± SD of the cases and controls were 60.18±6.85mmHg and 64.35±8.23mmHg respectively, (p = 0.0001). Glomerular filtration rate was significantly higher among the cases than the controls, 126±32ml/min/1.73m2 and 93±16ml/min/1.73m2 respectively (p <0.001). Proteinuria was higher among the cases (8.2%), with one (0.9%) having nephrotic range proteinuria. Conclusions: The proteinuria and hyperfiltration found in some of the children with SCA in this study suggest that renal function abnormalities can be detected early in this group of children when appropriately and timely investigated.
Highlights
Various population-based studies reported an estimated prevalence of homozygous sickle cell disease to be between 1.6% and 3% in Nigerian newborns with the incidence to be approximately 150,000 newborns per annum.4-6It has been established that about 300,000 Sickle cell anaemia (SCA) children are born annually, and 75% of them are reported to be born in Sub-Saharan Africa.7A recent study in Maiduguri North-Eastern Nigeria showed a prevalence of 6.8% and this was attributed to the increased rate of intermarriages between ethnic groups that have the highest carrier rate of the disorder and consanguineous marriages in the north-eastern part of Nigeria.[8]
Even though Nigeria has the highest prevalence of SCA and the increased chance of chronic kidney disease (CKD), information about renal abnormalities in the paediatric population with SCA in our setting are largely under-reported, the paucity of paediatric nephrologists and haematologists in North-eastern Nigeria contribute to this reality.[11]
In a resource-constrained setting like ours, hyperfiltration is an appropriate indicator of deterioration in renal function in children with SCA, which occurs earlier than proteinuria and/or decreased creatinine clearance
Summary
Plasma Endothelin-1 (ET-1) levels are elevated in patients with SCD during steady-state periods as well as during acute vaso-occlusive crisis this ET-1 promotes sickling and tissue injury, induces vasoconstriction, inflammation, nociception as well as production of oxidant stress and the release of cytokines.[14]. The kidneys are largely affected by this disorder, but overt features of kidney disease mostly manifest after the second decade, even though insult and sub-clinical features may occur during childhood Investigating these sub-clinical features is not routinely done in resource-scarce settings, partly due to the low socioeconomic status of most of our patients and the overwhelmed health care workers. Conclusions: The proteinuria and hyperfiltration found in some of the children with SCA in this study suggest that renal function abnormalities can be detected early in this group of children when appropriately and timely investigated
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