■自1996年在英国爆发新的变异型CJD以来,已在全球范围内进行了Creutzfeldt-Jakob病(CJD)的流行病学研究。一个全国性的报告系统,克雅氏病监测单位(CJDSU),由台湾疾病控制中心指导,成立于1997年,旨在识别人类朊病毒疾病。
■从1998年到2017年,将647例病例转交给委员会确认。CJDSU的报告包括一份结构化问卷,记录临床,人口统计数据,和潜在的医源性暴露,以及临床和实验室检查的结果,包括血液和脑脊液检查,脑电图,和脑部磁共振成像。
■总共,356例(妇女,n=178)被确定为人类朊病毒病,97.4%(n=347)为散发性CJD,包括三个明确的,314可能,和30例可能的病例;1例可能的变异CJD和8例遗传形式的人类朊病毒疾病。第二个十年(0.95/1,000,000)的按年龄和性别划分的年平均发病率也明显高于第一个十年(0.63/1,000,000),发病率比为1.51。发病率随着年龄的增长而增加,在70-79岁的年龄达到顶峰。sCJD患者的10年生存曲线显示,5-,10年累积生存率为52%,5%,1%,分别。170例患者的PRNP多态性显示,M129M占98.8%,E219E占97.6%。
■2008年后发病率的显着增加表明医生对这种罕见疾病的认识有所提高。台湾sCJD患者的病程比其他国家长,这表明医疗保健系统的全面支持,以及台湾的临终关怀文化,可能会延长患有这种进行性和致命疾病的患者的生存时间。
UNASSIGNED: Epidemiologic studies of Creutzfeldt-Jakob disease (CJD) have been undertaken worldwide since the new variant CJD outbreak in 1996 in the United Kingdom. A nationwide report system, the Creutzfeldt-Jakob Disease Surveillance Unit (CJDSU), directed by the Centers for Disease Control of Taiwan, was established in 1997 to identify human prion diseases.
UNASSIGNED: From 1998 to 2017, 647 cases were referred to the committee for confirmation. The report to CJDSU included a structured questionnaire recording the clinical, demographic data, and potential iatrogenic exposure, and the results of the clinical and laboratory examination, including tests of blood and cerebrospinal fluid, electroencephalography, and brain magnetic resonance imaging.
UNASSIGNED: In total, 356 cases (women, n=178) were ascertained to be human prion diseases, and 97.4% (n=347) were sporadic CJD, including three definite, 314 probable, and 30 possible cases; one probable variant CJD and 8 cases of the genetic form human prion diseases. The age- and gender-specific average annual incidence were also significantly higher in the second decade (0.95/1,000,000) than in the first decade (0.63/1,000,000), with an incidence rate ratio of 1.51. The incidences increased with increasing age, reaching a peak at the age of 70-79 years. The 10-year survival curve for sCJD patients showed that the 1-, 5-, and 10-year cumulative survival rate were 52%, 5%, and 1%, respectively. PRNP polymorphisms in 170 patients showed that 98.8% were M129M and 97.6% E219E.
UNASSIGNED: The significant increase in incidence after 2008 suggests the increase in the awareness of this rare disease among physicians. The longer disease duration in patients with sCJD in Taiwan than in other countries indicates that the comprehensive support of the health care system, as well as the end-of-life care culture in Taiwan, may prolong survival time in patients with such a progressive and fatal disease.