Improved survival in homozygous sickle cell disease: Lessons from a cohort study

Improved survival in homozygous sickle cell disease: Lessons from a cohort study
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DOI:
10.1136/bmj.311.7020.1600
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发表时间:
1995-12-16
影响因子:
--
通讯作者:
Serjeant, G
Serjeant, G
中科院分区:
医学1区
文献类型:
--
作者:
Lee, A;Thomas, P;Serjeant, G

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目的:探讨镰状细胞临床的简单干预措施是否能提高镰状细胞病患者的生存率。从出生开始的队列研究中的设计-生存曲线分析和风险比。环境-西印度群岛大学的mrc实验室(牙买加)和牙买加金斯敦的维多利亚朱比利医院。研究对象:对1973年6月至1981年12月在牙买加金斯顿主要政府妇产医院连续10万例非手术分娩的315例纯合子镰状细胞病患者进行筛查。干预措施-预防性青霉素预防肺炎球菌败血症,父母教育早期诊断急性脾隔离,密切监测镰状细胞临床,主要结局指标-生存。结果:生存率出现改善,第一、第二和最后三分之一的研究比较趋势的对数秩检验达到临界显著性(P=0.05)。急性脾隔离和肺炎球菌败血症-脑膜炎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纯合子镰状细胞病的死亡率。
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.Subject-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.