Abstract
Globally, sickle cell disease (SCD) has its highest prevalence and worst prognosis in sub-Saharan Africa. Nevertheless, relatively few studies describe the clinical characteristics of children with SCD in this region. We conducted a prospective observational study of children with SCD attending a specialist out-patient clinic in Kilifi, Kenya. A total of 124 children (median age 6·3 years) were included in the study. Splenomegaly was present in 41 (33%) subjects and hepatomegaly in 25 (20%), both being common in all age groups. A positive malaria slide was found at 6% of clinic visits. The mean haemoglobin concentration was 73 g/l, compared to 107 g/l in non-SCD controls (P < 0·001). Liver function tests were elevated; plasma bilirubin concentrations were 46 μmol/l and aspartate aminotransferase was 124 iu/l. Forty-eight (39%) children were admitted to hospital and two died. Children with SCD in Kilifi have a similar degree of anaemia and liver function derangement to patients living in developed countries, but splenomegaly persists into later childhood. The prevalence of malaria was lower than expected given the prevalence in the local community. This study provides valuable data regarding the clinical characteristics of children living with SCD in a rural setting in East Africa.
Highlights
The prevalence of sickle cell trait (HbAS) in this area is 15% (Williams et al, 2005) and homozygosity for HbS (HbSS) is the only form of sickle cell disease (SCD) that has been documented in the local community
In our cohort splenomegaly persisted until late childhood such that the spleen was still palpable in more than one-third of 10-year olds, an observation that is in accordance with previous studies conducted in sub-Saharan Africa (Esan, 1966; Adekile et al, 1988, 1993; Thuilliez & Vierin, 1997; Mouele et al, 1999)
Some reports have attributed the persistence of splenomegaly in African children with SCD to the effects of malaria, in this study we did not find an association with either the number of episodes of malaria or level of parasitaemia
Summary
The study was conducted at Kilifi District Hospital (KDH), which is located 60 km to the north of Mombasa on the Kenyan coast. KDH is the main district level government inpatient facility, serving a community of about 100 000 children under 15 years of age. First published online 23 July 2009 doi:10.1111/j.1365-2141.2009.07771.x Study subjects. Our study involved children attending the SCD outpatient clinic at KDH. All subjects were
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