Rankine-Mullings Angela Edna, Logan Twila Mae, King Lesley-Gaye, Cunningham-Myrie Colette Andrea, Scott Clive Robert, Knight-Madden Jennifer Marcelle
Sickle Cell Unit, Caribbean Institute for Health Research, University of the West Indies, Kingston, Jamaica.
Mona School of Business and Management, University of the West Indies, Kingston, Jamaica.
BMC Pediatr. 2020 Aug 8;20(1):373. doi: 10.1186/s12887-020-02270-y.
The greatest disease burden of sickle cell disease occurs early in life. Understanding factors that reduce disease related events in this period is therefore important. Hence, we assessed the impact of early care at a specialist center on the incidence of acute events during the first five years.
This was a retrospective cohort study among Jamaican children with sickle cell disease. Medical records of patients born January, 2004 to December, 2009, who were registered at the Sickle Cell Unit, a specialist care facility, were abstracted for dates of initiation to care, first occurrence and frequency of the outcomes of interest (vaso-occlusive crises, acute splenic sequestration, acute chest syndrome, and infection). Patients were classified according to whether initiation of care was before (early) or after 5 months of age (late). Using standardized t-tests, χ2 tests, and a multiple-failure survival analysis the rates of acute events between groups were compared.
Of the total study group (n= 290), homozygous sickle cell disease accounted for 97% and 95% of the early (n=113) and late groups (n=177) respectively. The mean age of presentation in the early and late group was 0.2 and 2.3 years (p<0.01), with a mean length of follow-up of 5.2 and 3.2 years respectively (p<0.01). Vaso-occlusive crisis (n=880) and acute chest syndrome (n= 571) together accounted for 91.6% of the total number of events (n=1584). The risk of vaso-occlusive crisis and acute chest syndrome (among patients who presented with these acute events) was significantly higher in the "late" group, by 43% (Incidence rate ratio, (IRR) = 1.43, p<0.001); 95% CI (1.18-1.72) and 40% (IRR=1.40. p=0.002), 95% CI (1.12-1.75) respectively compared to "early" group. There was no difference in risk between groups for acute splenic sequestration and infection among persons presenting with these events.
The risk of acute events in children with sickle cell disease exposed to early care at a specialist care is significantly less. Therefore, widespread screening with rapid referral to a specialist center stands to reduce substantial morbidity in Jamaica and other regions with high prevalence of sickle cell disease.
镰状细胞病最大的疾病负担出现在生命早期。因此，了解在此期间减少疾病相关事件的因素非常重要。为此，我们评估了专科中心的早期护理对前五年急性事件发生率的影响。
这是一项针对牙买加镰状细胞病患儿的回顾性队列研究。提取了2004年1月至2009年12月在专科护理机构镰状细胞科登记的患者的医疗记录，以获取开始护理的日期、感兴趣的结局（血管闭塞性危机、急性脾滞留、急性胸综合征和感染）的首次发生情况及发生频率。患者根据开始护理是在5个月龄之前（早期）还是之后（晚期）进行分类。使用标准化t检验、χ2检验和多重失败生存分析比较组间急性事件的发生率。
在整个研究组（n = 290）中，纯合子镰状细胞病分别占早期组（n = 113）和晚期组（n = 177）的97%和95%。早期组和晚期组的平均就诊年龄分别为0.2岁和2.3岁（p<0.01），平均随访时间分别为5.2年和3.2年（p<0.01）。血管闭塞性危机（n = 880）和急性胸综合征（n = 571）共占事件总数（n = 1584）的91.6%。在出现这些急性事件的患者中，“晚期”组血管闭塞性危机和急性胸综合征的风险显著更高，分别比“早期”组高43%（发病率比，(IRR) = 1.43，p<0.001）；95%可信区间（1.18 - 1.72）和40%（IRR = 1.40，p = 0.002），95%可信区间（1.12 - 1.75）。在出现急性脾滞留和感染事件的患者中，两组之间的风险没有差异。
在专科护理机构接受早期护理的镰状细胞病患儿发生急性事件的风险显著更低。因此，在牙买加和其他镰状细胞病高发地区广泛开展筛查并迅速转诊至专科中心，有望大幅降低发病率。
