Sickle cell disease clinical phenotypes in children from South.Western, Nigeria

Abstract

Background: The clinical phenotypes of children with sickle cell disease (SCD) are poorly described in many sub-Saharan countries including Nigeria.
Objectives: The objective was to highlight various clinical phenotypes of SCD in children and investigate the influence of sociodemographic indices on the development of SCD complications.
Methods: We carried out a cross.sectional study of 240 pediatric patients attending the sickle cell clinic and the emergency room in a teaching hospital in South.Western Nigeria over a 12.month period. The clinical phenotypes and severity of the disease were documented, and the  influence of sociodemographic variables was investigated.
Results: The five leading clinical phenotypes in our patients were  significant pain episodes, that is, vaso.occlusive crisis in 159 (66.3%); anemic crisis in 62 (25.8%); severe bacterial infections, 57 (23.8%); acute chest syndrome (ACS), 27 (11.3%) and stroke, 7 (2.9%). Forty.two  (33.1%) had a previous history of dactylitis (hand.foot syndrome). Other clinical phenotypes such as avascular necrosis of the femur, 4 (1.7%); nephropathy, 2 (0.8%); priapism, gallstone and chronic leg ulcer, one (0.4%) each, were not commonly seen. More children with a history of asthma had ACS. Furthermore, high steady.state white blood cell count was associated with severe disease.
Conclusion: The clinical phenotypes of SCD in children from South.Western Nigeria are highly variable with the disease manifesting very early and about 10% having significant complications. Sociodemographic  characteristics appear to have little influence on the development of SCD complications among our patients, but age and low-socioeconomic class are associated with anemic crisis.

Key words: Clinical phenotypes, Nigeria, sickle cell disease,  sociodemographic variables