Centre for Geographical Medicine (Coast) (CGMRC), Kenya Medical Research Institute (KEMRI), Kilifi, Kenya.
Br J Haematol. 2009 Sep;146(6):675-82. doi: 10.1111/j.1365-2141.2009.07771.x. Epub 2009 Jul 23.
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 micromol/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.
在全球范围内，镰状细胞病（SCD）在撒哈拉以南非洲的发病率最高，预后最差。然而，该地区很少有研究描述儿童 SCD 的临床特征。我们在肯尼亚基利菲的一家专科门诊对患有 SCD 的儿童进行了一项前瞻性观察研究。共有 124 名儿童（中位数年龄为 6.3 岁）纳入了该研究。41 名（33%）患儿存在脾肿大，25 名（20%）患儿存在肝肿大，且两种情况在所有年龄组中均较为常见。在 6%的就诊中发现疟原虫涂片阳性。平均血红蛋白浓度为 73 g/l，而非 SCD 对照组为 107 g/l（P < 0.001）。肝功能检查结果升高；血浆胆红素浓度为 46 μmol/l，天冬氨酸氨基转移酶为 124 iu/l。48 名（39%）儿童住院，2 名死亡。基利菲的 SCD 患儿贫血和肝功能紊乱程度与生活在发达国家的患者相似，但脾肿大持续到儿童后期。疟疾的患病率低于当地社区的预期患病率。本研究为东非农村地区 SCD 患儿的临床特征提供了有价值的数据。
