Creutzfeldt-Jakob disease surveillance in Australia: update to 31 December 2018
Creutzfeldt-Jakob disease surveillance in Australia: update to 31 December 2018
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DOI：
10.33321/cdi.2019.43.35
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发表时间：
2019-08-15
影响因子：
2.5
通讯作者：
Collins, Steven
中科院分区：
文献类型：
--
作者：
Stehmann, Christiane;Senesi, Matteo;Collins, Steven
位于墨尔本大学的澳大利亚国家克雅氏病登记处（ANCJDR）负责对人类朊病毒病（也称为传染性海绵状脑病）进行全国范围的监测，其中最常见的是克雅氏病（CJD）。国家监测涵盖1970年1月1日以来的时期，前瞻性监测从1993年10月1日开始。在这一前瞻性监测期间，在以下方面取得了相当大的进展：死前诊断、划分新的疾病亚型以及提高卫生保健机构对朊病毒疾病的认识。国家司法和刑事司法委员会的监督做法也相应地发生了变化和调整。本报告总结了ANCJDR从2018年1月1日至12月31日的活动。自ANCJDR于1997年9月开始在澳大利亚提供诊断性脑脊液（CSF）14-3-3蛋白检测以来，每年的转诊人数稳步增加。2018年，465份国内脑脊液标本被转诊进行14-3-3蛋白检测，78名疑似人朊病毒病患者被正式纳入国家登记册。截至2018年12月31日，78例疑似病例中的大多数仍被归类为“不完整”（42例），而11例病例通过详细的临床随访（1例）或神经病理学检查（10例）被排除; 15例病例被归类为“明确”，10例被归类为“可能”朊病毒疾病。所有疑似人类朊病毒病相关死亡中有62%接受了神经病理学检查。没有确诊变异型克雅二氏症病例。
Nationwide surveillance of human prion diseases (also known as transmissible spongiform encephalopathies), the most common being Creutzfeldt-Jakob disease (CJD), is performed by the Australian National Creutzfeldt-Jakob Disease Registry (ANCJDR), based at the University of Melbourne. National surveillance encompasses the period since 1 January 1970, with prospective surveillance occurring from 1 October 1993. Over this prospective surveillance period considerable developments have occurred in relation to pre-mortem diagnostics, the delineation of new disease subtypes and a heightened awareness of prion diseases in health care settings. Surveillance practices of the ANCJDR have evolved and adapted accordingly. This report summarises the activities of the ANCJDR from 1 January to 31 December 2018.Since the ANCJDR began offering diagnostic cerebrospinal fluid (CSF) 14-3-3 protein testing in Australia in September 1997, the annual number of referrals has steadily increased. In 2018, 465 domestic CSF specimens were referred for 14-3-3 protein testing and 78 persons with suspected human prion disease were formally added to the national register. The majority of the 78 suspect case notifications remain as of 31 December 2018 classified as "incomplete" (42 cases), while eleven cases were excluded by either detailed clinical follow-up (one case) or neuropathological examination (ten cases); 15 cases were classified as "definite" and ten as "probable" prion disease. Sixty-two percent of all suspected human prion disease related deaths underwent neuropathological examination. No cases of variant CJD were confirmed.