我国96例散发型克雅氏病患者随访调查及生存分析

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。

Follow up of 96 sporadic Creutzfeldt-Jakob disease cases in China

Objective To evaluate the survive of sporadic Creutzfeldt-Jakob disease (sCJD) cases in China. Methods All 96 sCJD patients reported by national CJD surveillance network between 1 January 2008 and 31 December 2011 were enrolled in this study. The follow up of their survival status/length were made by telephone call to the patients' family members. The epidemiological and clinical information of the patients were obtained from CJD surveillance network and the association between clinical manifestations and survival length were analyzed. Results All the 96 sCJD patients died within 2 years after onset. The median of the survival length was 6.1 months. The cumulative proportions of the survival length less than 3, 6, 12 and 24 months were 15.6%, 42.7%, 74.0% and 100%, respectively. The initial manifestation and the frequency of typical clinical symptom had no significant effect on the patients' survival length. Conclusion All the sCJD patients in China died within 2 years after onset, the median of the survival length was 6.1 months. The general socio-demographic characteristics and clinical features of the patients did not influence the survival length of the patients.