Clinical profile of paediatric sickle cell disease at a reference hospital in North–eastern Nigeria

Background: Sickle cell disorders (SCD) are the commonest inherited haemoglobin (Hb) disorders. Globally, about 300,000 babies are born annually with these conditions. The clinical profiles of SCD have been described in many parts of the world. These however have not been adequately investigated in some parts of Nigeria. This study evaluated the clinical features, Hb phenotype and complications of children with SCD being managed at a Paediatric haematology clinic in Northern Nigeria. Methods: A prospective observational study of steady state SCD patients aged 2 – 16 years, was conducted from January, 2019  -October, 2020. Historical and examination findings were documented. Analysis of data was done with the Statistical Package for Social Sciences (SPSS) version 20.0. Results: One hundred children were studied. Mean age was 6.97 ± 3.63 years. Male: female ratio was 1.6:1.Mean weight and mean height were lower than reference standards (t= -1.20, p= 0.14), (t= - 0.66, p= 0.27) respectively. Mean age at diagnosis was 13.24 ±14.83 months, and at first presentation was 13.86 ±17.51 months, 58.5% of subjects were adequately vaccinated for age, while 92% had a Hb phenotype of SS. Acute chest syndrome occurred more frequently in children aged 9-12 years (χ2= 11.59, P ˂ 0.001), and in those with severe bacterial infections (χ2= 7.41, p= 0.006). Conclusion: The complications of Paediatric SCD in this part of North-Eastern Nigeria mirrors those in other parts of the country. Socio-economic class, Hb phenotype and vaccination status had no influence on the development of complications.