King L, Fraser R, Forbes M, Grindley M, Ali S, Reid M
Sickle Cell Unit, Tropical Medicine Research Institute, University of the West Indies, Mona, Kingston, Jamaica, West Indies.
J Med Screen. 2007;14(3):117-22. doi: 10.1258/096914107782066185.
The aim of this study was to evaluate the existing newborn sickle haemoglobinopathy screening programme in Jamaica.
A retrospective analysis of infants screened during the period 8 November 1995 to 22 July 2006 was performed. Patient data for analyses was restricted to patients with homozygous (Hb SS) sickle cell disease. Published data from the Jamaican Sickle Cell Cohort Study was used to make comparisons with the study sample.
The study sample consisted of 435 patients with Hb SS disease. Acute chest syndrome was the most common clinical (non-death) event accounting for approximately 50% of all events. Acute splenic sequestration, no longer a significant cause of mortality, was responsible for approximately 32% of clinical events. Seven deaths (1.8%) occurred during the study period compared with 17.6% to the same age in the Jamaican Sickle Cell Cohort Study. There was a lower proportion of hospital admissions and episodes of serious illness in the study group compared with controls.
Survival estimates for the study sample showed improvement compared with the Jamaican Sickle Cell Cohort Study. This study continues to demonstrate the benefits of, and as such shows support for, newborn screening and early interventions in sickle cell disease. In addition, it highlights some of the areas for continued focus and research development. Although the current system is providing an essential and beneficial service, the study emphasizes the need for newborn screening programmes to be comprehensive care systems to be fully effective.
本研究旨在评估牙买加现有的新生儿镰状血红蛋白病筛查项目。
对1995年11月8日至2006年7月22日期间接受筛查的婴儿进行回顾性分析。用于分析的患者数据仅限于纯合子（Hb SS）镰状细胞病患者。牙买加镰状细胞队列研究的已发表数据用于与研究样本进行比较。
研究样本包括435例Hb SS病患者。急性胸综合征是最常见的临床（非死亡）事件，约占所有事件的50%。急性脾滞留不再是主要的死亡原因，约占临床事件的32%。研究期间有7例死亡（1.8%），而牙买加镰状细胞队列研究中同年龄组的死亡率为17.6%。与对照组相比，研究组的住院率和重病发作比例较低。
与牙买加镰状细胞队列研究相比，研究样本的生存估计有所改善。本研究继续证明了新生儿筛查和镰状细胞病早期干预的益处，并因此表明了对其的支持。此外，它突出了一些需要持续关注和研究发展的领域。尽管当前系统提供了一项基本且有益的服务，但该研究强调新生儿筛查项目需要成为全面的护理系统才能充分有效。
