Correlates of steady-state haematocrit and hepatosplenomegaly in children with sickle cell disease in Western Nigeria.

Autor: Brown BJ; Department of Paediatrics, University College Hospital, Ibadan, Nigeria., Fatunde OJ, Sodeinde O
Jazyk: angličtina
Zdroj: West African journal of medicine [West Afr J Med] 2012 Apr-Jun; Vol. 31 (2), pp. 86-91.
Abstrakt: Background: 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 socio-economic 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 socio-economic status have some modifying influences on the steady-state features of sickle cell disease in Nigerian children. In addition, increasing liver and spleen sizes seem to be related to a decreasing haematocrit.
Databáze: MEDLINE