Paediatrics, Mzuzu Central Hospital, Mzuzu, Northern Region, Malawi.
Biological Sciences, Mzuzu University Faculty of Health Sciences, Mzuzu, Northern Region, Malawi.
BMJ Paediatr Open. 2021 Sep 8;5(1):e001097. doi: 10.1136/bmjpo-2021-001097. eCollection 2021.
Sickle cell disease (SCD) remains a major cause of childhood mortality and morbidity in Malawi. However, literature to comprehensively describe the disease in the paediatric population is lacking.
A retrospective review of clinical files of children with SCD was conducted. Descriptive statistics were performed to summarise the data. χ or Fisher's exact test was used to look for significant associations between predictor variables and outcome variables (case fatality and length of hospital stay). Predictor variables that were significantly associated with outcome variables (p≤0.05) in a χ or Fisher's exact test were carried forward for analysis in a binary logistic regression. A multivariable binary logistic regression was used to identify covariates that independently predicted length of hospital stay.
There were 16 333 paediatric hospitalisations during the study period. Of these, 512 were patients with SCD representing 3.1% (95% CI: 2.9%- 3.4%). Sixty-eight of the 512 children (13.3%; 95% CI: 10.5% - 16.5%) were newly diagnosed cases. Of these, only 13.2% (95% CI: 6.2% - 23.6%) were diagnosed in infancy. Anaemia (94.1%), sepsis (79.5%) and painful crisis (54.3%) were the most recorded clinical features. The mean values of haematological parameters were as follows: haemoglobin (g/dL) 6.4 (SD=1.9), platelets (×10/L) 358.8 (SD=200.9) while median value for white cell count (×10/L) was 23.5 (IQR: 18.0-31.2). Case fatality was 1.4% (95% CI: 0.6% - 2.8%)and 15.2% (95% CI: 12.2% -18.6%) of the children had a prolonged hospital stay (>5 days). Patients with painful crisis were 1.7 (95% CI: 1.02 - 2.86) times more likely to have prolonged hospital stay than those without the complication.
Anaemia, sepsis and painful crisis were the most common clinical features paediatric patients with SCD presented with. Patients with painful crisis were more likely to have prolonged hospital stay. Delayed diagnosis of SCD is a problem that needs immediate attention in this setting. Although somewhat encouraging, the relatively low in-hospital mortality among SCD children may under-report the true mortality from the disease considering community deaths and deaths occurring before SCD diagnosis is made.
镰状细胞病（SCD）仍然是马拉维儿童死亡和发病的主要原因。然而，缺乏全面描述儿科人群疾病的文献。
对患有 SCD 的儿童的临床档案进行回顾性分析。采用描述性统计方法对数据进行总结。χ检验或 Fisher 确切检验用于寻找预测变量和结局变量（病死率和住院时间）之间的显著关联。在 χ检验或 Fisher 确切检验中与结局变量显著相关的预测变量（p≤0.05），则进行二项逻辑回归分析。采用多变量二项逻辑回归分析确定独立预测住院时间的协变量。
研究期间共有 16333 例儿科住院患者。其中，512 例为 SCD 患儿，占 3.1%（95%CI：2.9%-3.4%）。512 例患儿中，有 68 例（13.3%；95%CI：10.5%-16.5%）为新诊断病例。其中，只有 13.2%（95%CI：6.2%-23.6%）在婴儿期被诊断。贫血（94.1%）、败血症（79.5%）和疼痛危象（54.3%）是最常见的临床特征。血液学参数的平均值如下：血红蛋白（g/dL）6.4（SD=1.9），血小板（×10/L）358.8（SD=200.9），白细胞计数（×10/L）中位数为 23.5（IQR：18.0-31.2）。病死率为 1.4%（95%CI：0.6%-2.8%），15.2%（95%CI：12.2%-18.6%）的患儿住院时间延长（>5 天）。发生疼痛危象的患儿比无并发症的患儿更有可能延长住院时间（1.7 倍；95%CI：1.02-2.86）。
贫血、败血症和疼痛危象是儿科 SCD 患者最常见的临床表现。发生疼痛危象的患儿更有可能延长住院时间。SCD 的延迟诊断是该地区需要立即关注的问题。尽管情况有所好转，但 SCD 患儿的住院死亡率相对较低，可能低估了该疾病的实际死亡率，因为包括社区死亡和在做出 SCD 诊断之前发生的死亡。
