纯合子镰状细胞病患者生存率的提高：一项队列研究的经验教训
Improved survival in homozygous sickle cell disease: lessons from a cohort study.
作者信息
Lee A, Thomas P, Cupidore L, Serjeant B, Serjeant G
机构信息
MRC Laboratories, (Jamaica), University of the West Indies, Kingston, Jamaica.
出版信息
BMJ. 1995 Dec 16;311(7020):1600-2. doi: 10.1136/bmj.311.7020.1600.
OBJECTIVE
To examine whether simple interventions in a sickle cell clinic improve survival in sickle cell disease.
DESIGN
Survival curve analysis and hazard ratios in a cohort study followed from birth.
SETTING
MRC Laboratories (Jamaica) at the University of the West Indies, and Victoria Jubilee Hospital, Kingston, Jamaica.
SUBJECTS
315 patients with homozygous sickle cell disease detected during the screening of 100,000 consecutive non-operative deliveries between June 1973 and December 1981 at the main government maternity hospital, Kingston, Jamaica.
INTERVENTIONS
Prophylactic penicillin to prevent pneumococcal septicaemia, parental education in early diagnosis of acute splenic sequestration, close monitoring in sickle cell clinic.
MAIN OUTCOME MEASURES
Survival.
RESULTS
Survival appeared to improve, the log rank test for trend comparing the first, second, and last third of the study reaching borderline significance (P = 0.05). Combined deaths from acute splenic sequestration and pneumococcal septicaemia-meningitis declined significantly (test for trend, P = 0.02).
CONCLUSION
Early diagnosis and simple prophylactic measures significantly reduce deaths associated with homozygous sickle cell disease.
目的
研究镰状细胞病诊所的简单干预措施是否能提高镰状细胞病患者的生存率。
设计
一项队列研究中的生存曲线分析和风险比，从出生开始随访。
地点
西印度群岛大学的MRC实验室（牙买加）以及牙买加金斯敦的维多利亚禧年医院。
研究对象
1973年6月至1981年12月期间，在牙买加金斯敦主要政府妇产医院对100,000例连续非手术分娩进行筛查时检测出的315例纯合子镰状细胞病患者。
干预措施
预防性使用青霉素以预防肺炎球菌败血症，对家长进行急性脾滞留早期诊断的教育，在镰状细胞病诊所进行密切监测。
主要观察指标
生存率。
结果
生存率似乎有所提高，对研究前三分之一、中间三分之一和最后三分之一进行趋势比较的对数秩检验达到临界显著水平（P = 0.05）。急性脾滞留和肺炎球菌败血症 - 脑膜炎的合并死亡人数显著下降（趋势检验，P = 0.02）。
结论
早期诊断和简单的预防措施可显著降低与纯合子镰状细胞病相关的死亡。
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