Epidemiological characteristics of human prion diseases.

Epidemiological characteristics of human prion diseases.
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人类朊病毒疾病的流行病学特征。

DOI:
10.1186/s40249-016-0143-8
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发表时间:
2016-06-02
影响因子:
8.1
通讯作者:
Dong XP
Dong XP
中科院分区:
医学1区
文献类型:
--
作者:
Chen C;Dong XP

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人类朊病毒病是一组可传染、进行性且总是致命的神经退行性疾病,包括库鲁病、克雅氏病 (CJD)、格斯特曼-斯特劳斯勒-沙因克综合征和致命的家族性失眠症。人类朊病毒病每年影响全世界每百万人约 1-2 人,以散发、遗传和获得性形式发生。这些疾病引起了科学界和公众的关注,不仅因为它们神秘的病原体,而且因为自变异型克雅氏病出现以来,它们对公众健康构成了相当大的威胁。目前还没有针对朊病毒疾病的具体治疗和预防干预措施,因此对人类朊病毒疾病的主动监测对于疾病控制和预防至关重要。自1993年以来,许多国家和地区建立了克雅氏病监测系统,并开展了多个长期跨国合作项目。本文对不同国家和地区各种人类朊病毒病的流行病学特征和主动监测系统进行了总结和综述。本文的在线版本 (doi:10.1186/s40249-016-0143-8) 包含补充材料,可供授权用户使用。
Human prion diseases are a group of transmissible, progressive, and invariably fatal neurodegenerative disorders, which include Kuru, Creutzfeldt-Jakob disease (CJD), Gerstmann-Sträussler-Scheinker syndrome, and fatal familial insomnia. Human prion diseases affect approximately 1–2 persons per million worldwide annually, occurring in sporadic, inherited, and acquired forms. These diseases have attracted both scientific and public attention not only because of their mysterious pathogen, but also due to their considerable threat to public health since the emergence of the variant CJD. There are still no specific therapeutic and prophylactic interventions available for prion diseases, thus active surveillance of human prion diseases is critical for disease control and prevention. Since 1993, CJD surveillance systems have been established in many countries and regions, and several long-term multinational cooperative projects have been conducted. In this paper, the epidemiological characteristics of various human prion diseases and the active surveillance systems pertaining to them in different countries and regions are summarized and reviewed. The online version of this article (doi:10.1186/s40249-016-0143-8) contains supplementary material, which is available to authorized users.