Correlates of Steady-State Haematocrit and Hepatosplenomegaly in Children with Sickle Cell Disease in Western Nigeria

Abstract

Backgroup: Sickle cell disease is a common genetic disorder in Nigeria.
Objectives: To determine the steady state haematocrit, liver size and spleen size in children with sickle cell disease and the factors that influence them.
Methods: This was a retrospective study of children with sickle cell disorders who attended the anaemia clinic of the Children’s Outpatient Department, University College Hospital, Ibadan between the years 2000–2009. Relevant data extracted from their case notes included socio- demographic variables, haemoglobin phenotype, steady state haematocrit and liver and splenic sizes. Means were compared with t-test and correlation tested with Pearson correlation. Statistical significance was set at p<0.05.
Results: A total of 415 (Male: female ratio 1.1:1) children were studied and 385 (92.8%) and 30 (7.2%) of the children were of haemoglobin (Hb) SS and Hb SC phenotypes respectively. Their ages ranged from 0.5–17 years with a mean (SD) of 7.3(4.4) years. Mean (SD) steady state haematocrit for children with HbSC was 28.3(4.5) % and significantly higher than 24.1 (3.7) % in HbSS. Mean steady state haematocrit was also higher in children from higher than lower socioeconomic classes. There was a negative correlation of haematocrit with age, with hepatomegaly and splenomegaly. Steady state hepatomegaly occurred more frequently in HbSS than in HbSC.
Conclusion: Haemoglobin phenotype, age and socioeconomic status have some modifying influences on the steadystate features of sickle cell disease in Nigerian children. In addition, increasing liver and spleen sizes seem to be related to a decreasing haematocrit.

Keywords: Sickle cell; steady-state; haematocrit; hepatosplenomegaly