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目的了解中国散发型克雅氏病(sporadic Creutzfeldt-Jakob disease,sCJD)患者生存情况及生存时间。方法选择2008年1月1日至2011年12月31日期间经我国CJD监测网络诊断的96例sCJD患者,对其家属进行电话随访,获得生存情况及死亡时间。同时从全国死因监测系统中补充患者相关资料,分析可能影响生存时间的因素。结果 96例sCJD患者全部在2年内死亡,其中位生存时间为6.1个月,生存时间短于3、6、12、24个月的累计百分比分别为15.6%、42.7%、74.0%和100%;首发症状、出现典型临床表现的数量对患者生存时间没有明显影响。结论中国sCJD患者的中位生存时间为6.1个月,整体生存时间<2年。
Objective To investigate the survival and survival of patients with sporadic Creutzfeldt-Jakob disease (sCJD) in China. Methods A total of 96 patients with sCJD diagnosed by CJD monitoring network in China from January 1, 2008 to December 31, 2011 were selected and followed up by phone to obtain their survival and death time. At the same time, the patient-related information was supplemented from the national cause of death monitoring system to analyze the factors that may affect the survival time. Results All 96 patients with sCJD died within 2 years. The median survival time was 6.1 months. The cumulative survival rates were 15.6%, 42.7%, 74.0% and 100%, respectively. The first symptom, the number of typical clinical manifestations did not significantly affect the patient’s survival time. Conclusions The median survival time of Chinese patients with sCJD is 6.1 months and the overall survival time is less than 2 years.