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疾病概览

Prion

克雅氏病

Creutzfeldt-Jakob disease

有证据支持的资料英文 7/7 个适用章节 · 中文 7/7 个适用章节更新于 2026年9月4日

克雅氏病(Creutzfeldt-Jakob disease, CJD)是一组朊蛋白相关的致死性神经退行性疾病,常以快速进展性痴呆和心理运动改变为主要表现[1][2][3]。现有综述提示其临床过程可迅速恶化,多数患者在临床起病后1年内死亡[1]。从公共卫生监测角度看,CJD总体罕见,但与医源性传播风险、长期潜伏以及散发型病例识别有关,因此需要持续警惕[1][3]

阅读完整临床与流行病学资料8
定义

克雅氏病是一类由错误折叠的朊蛋白(PrP Sc)相关的致死性朊蛋白病,病理过程涉及脑内异常朊蛋白沉积并导致神经退行性损害和海绵状改变[2][3]。文献将其分为散发型、遗传型和获得型等形式[2]。目前可获得资料显示,该病的临床识别和监测均受到其异质性、潜伏期长以及确证前诊断困难的影响[1][3]

临床特征

CJD的核心临床特征是快速进展性神经退行性综合征,表现为快速进展性痴呆,以及心理运动改变[1][2]。综述还提到可见认知功能障碍、运动障碍和行为异常等表现[2]。临床诊断线索通常结合特征性神经精神症状、脑脊液14-3-3蛋白、MRI和EEG等信息,而RT-QuIC等蛋白聚集/扩增检测被认为有助于临床前确证[3]。疾病预后不良,多数患者在临床起病后1年内死亡[1]

流行病学

现有综述指出,CJD总体罕见,其中尤以医源性CJD较为少见,但其公共卫生意义在于潜在的传播风险[1]。系统综述还提示,散发型CJD的发病率呈上升趋势[1]。文献同时指出,潜伏期可长达40年,提示既往暴露后的长期监测具有意义[1]。另一项综述强调,散发型病例多见于老年人,临床上可能与快速进展性痴呆相似,从而增加识别难度[1]

传播/暴露

可获得资料支持CJD存在医源性传播风险,且可在人体内无症状潜伏数十年后才表现为临床疾病[1]。这意味着公共卫生关注点主要在既往暴露史、潜伏期管理以及与医疗操作相关的风险识别,而不是常规社区传播[1]。关于其他具体传播途径、暴露场景或不同形式之间的差异性传播机制,所给来源中未提供足够细节。

高风险人群

现有资料中明确提到,散发型CJD多见于老年人[1]。此外,任何存在既往医源性暴露潜在风险的人群都需要在长期随访与监测框架下被关注,因为疾病可在人体内无症状潜伏多年甚至数十年[1]。关于更具体的高风险暴露群体或分层风险特征,所给来源尚未提供足够支持。

预防

来源提示,预防重点在于维持对CJD的持续警觉,以减少未来传播事件并加强相关监测[1][3]。对于已知或疑似散发型病例,更新后的诊断标准和RT-QuIC等技术可提高早期临床确认和监测能力,从而支持公共卫生处置[3]。除上述监测与识别措施外,关于具体暴露控制、器械处理或其他预防程序,本次来源未提供可直接引用的细节。

监测说明

在监测语境中,CJD应被理解为一种罕见但需要长期警惕的致死性朊蛋白病,尤其应关注散发型病例的识别及其与快速进展性痴呆的重叠[1][3]。由于可有长达数十年的无症状潜伏,病例监测不应仅依赖近期暴露史,而应结合临床演变、神经精神表现及辅助检测结果综合判断[1][3]。更新后的诊断与生物标志物应用被认为可改善早期确认、监测和相关研究评估[3]。 GlobalID 来源数据说明: 挪威 MSIS 发布的来源类别为“Prionsykdommer”(朊病毒病)。挪威已报告病例以 CJD 为主,但该类别及报告标准可以包含其他朊病毒病;因此应按官方来源类别解释,而不应假定每个数值都是已完成亚型确认的 CJD 病例。

参考文献
  1. 1Uttley L et al. Creutzfeldt-Jakob disease: a systematic review of global incidence, prevalence, infectivity, and incubation. Lancet Infect Dis. 2020 Jan. PMID: 31876504. doi: 10.1016/S1473-3099(19)30615-2.PubMed: https://pubmed.ncbi.nlm.nih.gov/31876504/
  2. 2Noor H et al. Creutzfeldt-Jakob disease: A comprehensive review of current understanding and research. J Neurol Sci. 2024 Dec 15. PMID: 39546829. doi: 10.1016/j.jns.2024.123293.PubMed: https://pubmed.ncbi.nlm.nih.gov/39546829/
  3. 3Hermann P et al. Biomarkers and diagnostic guidelines for sporadic Creutzfeldt-Jakob disease. Lancet Neurol. 2021 Mar. PMID: 33609480. doi: 10.1016/S1474-4422(20)30477-4.PubMed: https://pubmed.ncbi.nlm.nih.gov/33609480/
  4. 4US Centers for Disease Control and Prevention. Classic Creutzfeldt-Jakob Disease | Classic CJD | CDC [Internet]. cited 4 Sept 2026.Available from: https://www.cdc.gov/creutzfeldt-jakob/about/index.html
编码登记
ICD-10
ICD-11
核心统计
累计病例
7K
峰值月份
2023-01
覆盖区间
37 个报告国家 · 1996-01-01 → 2026-09-01

数据覆盖

报告国家和地区

37 个国家/地区

月度变化趋势

数据获取

本页面数据索引,含来源链接和更新信息。

行数3,284
更新2026-09-01
范围
分块7
来源32 序列 · 818 观测
官方数据来源机构、频率与说明49
Austria

Austria AGES Radar for Infectious Diseases

Austria

来源
每月official_monthly_csv

AGES Radar monthly national source. Internal ingestion retains native categories; public release is disabled pending licence review.

Australia

Australia NINDSS

Australia

来源
每月Power BI 看板

Australian national notifiable diseases surveillance dashboard.

Australian Capital Territory, Australia

Australia NINDSS — Australian Capital Territory

Australian Capital Territory, Australia

来源
每月Power BI 看板

Australian NINDSS monthly state/territory notifications for Australian Capital Territory, kept separate from the Australia national aggregate.

New South Wales, Australia

Australia NINDSS — New South Wales

New South Wales, Australia

来源
每月Power BI 看板

Australian NINDSS monthly state/territory notifications for New South Wales, kept separate from the Australia national aggregate.

Northern Territory, Australia

Australia NINDSS — Northern Territory

Northern Territory, Australia

来源
每月Power BI 看板

Australian NINDSS monthly state/territory notifications for Northern Territory, kept separate from the Australia national aggregate.

Queensland, Australia

Australia NINDSS — Queensland

Queensland, Australia

来源
每月Power BI 看板

Australian NINDSS monthly state/territory notifications for Queensland, kept separate from the Australia national aggregate.

South Australia, Australia

Australia NINDSS — South Australia

South Australia, Australia

来源
每月Power BI 看板

Australian NINDSS monthly state/territory notifications for South Australia, kept separate from the Australia national aggregate.

Tasmania, Australia

Australia NINDSS — Tasmania

Tasmania, Australia

来源
每月Power BI 看板

Australian NINDSS monthly state/territory notifications for Tasmania, kept separate from the Australia national aggregate.

Victoria, Australia

Australia NINDSS — Victoria

Victoria, Australia

来源
每月Power BI 看板

Australian NINDSS monthly state/territory notifications for Victoria, kept separate from the Australia national aggregate.

Western Australia, Australia

Australia NINDSS — Western Australia

Western Australia, Australia

来源
每月Power BI 看板

Australian NINDSS monthly state/territory notifications for Western Australia, kept separate from the Australia national aggregate.

Belgium

ECDC Surveillance Atlas — Belgium Annual Baseline

Belgium

来源
每年official_ecdc_rest_aggregate

Belgium annual Member-State reported case counts from the ECDC Surveillance Atlas. Missing topic/year cells remain unknown and published zeroes are preserved. Data provided by ECDC based on data reported by EU/EEA Member States.

Bulgaria

ECDC Surveillance Atlas — Bulgaria Annual Baseline

Bulgaria

来源
每年official_ecdc_rest_aggregate

Bulgaria annual Member-State reported case counts from the ECDC Surveillance Atlas. Missing topic/year cells remain unknown and published zeroes are preserved. Data provided by ECDC based on data reported by EU/EEA Member States.

Brazil

Brazil DATASUS SINAN

Brazil

来源
每月ftp_dbc

Brazil Ministry of Health DATASUS/SINAN public DBC microdata aggregated to national monthly notification counts.

Canada

Canada PHAC CNDSS Annual

Canada

来源
ANNUAL网页

Canada is the national jurisdiction; subdivision feeds are registered separately.

Switzerland

Switzerland FOPH IDD

Switzerland

来源
每周rest_api

Switzerland FOPH/BAG IDD mandatory reporting API normalized to national case rows. Monthly series may use the dashboard CHFL aggregate where CH-only monthly series are not exposed.

Cyprus

ECDC Surveillance Atlas — Cyprus Annual Baseline

Cyprus

来源
每年official_ecdc_rest_aggregate

Cyprus annual Member-State reported case counts from the ECDC Surveillance Atlas. Missing topic/year cells remain unknown and published zeroes are preserved. Data provided by ECDC based on data reported by EU/EEA Member States.

Czechia

ECDC Surveillance Atlas — Czechia Annual Baseline

Czechia

来源
每年official_ecdc_rest_aggregate

Czechia annual Member-State reported case counts from the ECDC Surveillance Atlas. Missing topic/year cells remain unknown and published zeroes are preserved. Data provided by ECDC based on data reported by EU/EEA Member States.

Denmark

ECDC Surveillance Atlas — Denmark Annual Baseline

Denmark

来源
每年official_ecdc_rest_aggregate

Denmark annual Member-State reported case counts from the ECDC Surveillance Atlas. Missing topic/year cells remain unknown and published zeroes are preserved. Data provided by ECDC based on data reported by EU/EEA Member States.

Estonia

ECDC Surveillance Atlas — Estonia Annual Baseline

Estonia

来源
每年official_ecdc_rest_aggregate

Estonia annual Member-State reported case counts from the ECDC Surveillance Atlas. Missing topic/year cells remain unknown and published zeroes are preserved. Data provided by ECDC based on data reported by EU/EEA Member States.

Spain

ECDC Surveillance Atlas — Spain Annual Baseline

Spain

来源
每年official_ecdc_rest_aggregate

Spain annual Member-State reported case counts from the ECDC Surveillance Atlas. Missing topic/year cells remain unknown and published zeroes are preserved. Data provided by ECDC based on data reported by EU/EEA Member States.

France

ECDC Surveillance Atlas — France Annual Baseline

France

来源
每年official_ecdc_rest_aggregate

France annual Member-State reported case counts from the ECDC Surveillance Atlas. Missing topic/year cells remain unknown and published zeroes are preserved. Data provided by ECDC based on data reported by EU/EEA Member States.

United Kingdom

ECDC Surveillance Atlas — United Kingdom Annual Baseline

United Kingdom

来源
每年official_ecdc_rest_aggregate

United Kingdom annual Member-State reported case counts from the ECDC Surveillance Atlas. Missing topic/year cells remain unknown and published zeroes are preserved. Data provided by ECDC based on data reported by EU/EEA Member States.

Greece

ECDC Surveillance Atlas — Greece Annual Baseline

Greece

来源
每年official_ecdc_rest_aggregate

Greece annual Member-State reported case counts from the ECDC Surveillance Atlas. Missing topic/year cells remain unknown and published zeroes are preserved. Data provided by ECDC based on data reported by EU/EEA Member States.

Hong Kong, China

Hong Kong, China CHP Notifiable Diseases

Hong Kong, China

来源
每月开放数据

Hong Kong, China CHP annual notifiable infectious disease CSVs normalized to national monthly totals

Croatia

ECDC Surveillance Atlas — Croatia Annual Baseline

Croatia

来源
每年official_ecdc_rest_aggregate

Croatia annual Member-State reported case counts from the ECDC Surveillance Atlas. Missing topic/year cells remain unknown and published zeroes are preserved. Data provided by ECDC based on data reported by EU/EEA Member States.

Hungary

ECDC Surveillance Atlas — Hungary Annual Baseline

Hungary

来源
每年official_ecdc_rest_aggregate

Hungary annual Member-State reported case counts from the ECDC Surveillance Atlas. Missing topic/year cells remain unknown and published zeroes are preserved. Data provided by ECDC based on data reported by EU/EEA Member States.

Ireland

Ireland HPSC Notifiable Diseases Hub

Ireland

来源
每周official_arcgis_feature_service

Ireland HPSC national weekly notifiable-disease totals from the official ArcGIS FeatureServer. Internal ingestion is enabled; public release remains disabled pending written redistribution permission.

Iceland

冰岛卫生署年度传染病看板

Iceland

来源
每年Power BI 看板

冰岛全国分病种年度病例通报;历史值可能追溯修订,并保持年度总量口径。

Iceland

冰岛卫生署性病监测看板

Iceland

来源
月度事实(按季度发布)Power BI 看板

实验室与登记监测的月度性病诊断事实;官方看板按季度发布。

Iceland

冰岛卫生署呼吸道感染周度看板

Iceland

来源
每周Power BI 看板

按 ISO 周记录的呼吸道疾病诊断数;住院、样本和疫苗指标不计入诊断数。

Iceland

冰岛卫生署历史传染病登记

Iceland

来源
年度与月度官方 Excel

1997—2021 年官方历史登记表,年度与月度事实按原始粒度保留。

Iceland

冰岛卫生署历史 ICD 临床月报

Iceland

来源
每月官方 Excel

Saga 电子病历 ICD 就诊数;与登记通报不可比并保持独立。

Italy

ECDC Surveillance Atlas — Italy Annual Baseline

Italy

来源
每年official_ecdc_rest_aggregate

Italy annual Member-State reported case counts from the ECDC Surveillance Atlas. Missing topic/year cells remain unknown and published zeroes are preserved. Data provided by ECDC based on data reported by EU/EEA Member States.

Japan

日本 NIID/JIHS 周报

Japan

来源
每周网页

日本 NIID/JIHS 周度传染病监测数据。

South Korea

Korea KDCA EID

South Korea

来源
每月open_api_or_portal_download

Korea KDCA notifiable infectious disease OpenAPI or portal/KOSIS downloads aggregated to national monthly notification counts.

Liechtenstein

ECDC Surveillance Atlas — Liechtenstein Annual Baseline

Liechtenstein

来源
每年official_ecdc_rest_aggregate

Liechtenstein annual Member-State reported case counts from the ECDC Surveillance Atlas. Missing topic/year cells remain unknown and published zeroes are preserved. Data provided by ECDC based on data reported by EU/EEA Member States.

Lithuania

ECDC Surveillance Atlas — Lithuania Annual Baseline

Lithuania

来源
每年official_ecdc_rest_aggregate

Lithuania annual Member-State reported case counts from the ECDC Surveillance Atlas. Missing topic/year cells remain unknown and published zeroes are preserved. Data provided by ECDC based on data reported by EU/EEA Member States.

Luxembourg

ECDC Surveillance Atlas — Luxembourg Annual Baseline

Luxembourg

来源
每年official_ecdc_rest_aggregate

Luxembourg annual Member-State reported case counts from the ECDC Surveillance Atlas. Missing topic/year cells remain unknown and published zeroes are preserved. Data provided by ECDC based on data reported by EU/EEA Member States.

Latvia

ECDC Surveillance Atlas — Latvia Annual Baseline

Latvia

来源
每年official_ecdc_rest_aggregate

Latvia annual Member-State reported case counts from the ECDC Surveillance Atlas. Missing topic/year cells remain unknown and published zeroes are preserved. Data provided by ECDC based on data reported by EU/EEA Member States.

Malta

ECDC Surveillance Atlas — Malta Annual Baseline

Malta

来源
每年official_ecdc_rest_aggregate

Malta annual Member-State reported case counts from the ECDC Surveillance Atlas. Missing topic/year cells remain unknown and published zeroes are preserved. Data provided by ECDC based on data reported by EU/EEA Member States.

Netherlands

ECDC Surveillance Atlas — Netherlands Annual Baseline

Netherlands

来源
每年official_ecdc_rest_aggregate

Netherlands annual Member-State reported case counts from the ECDC Surveillance Atlas. Missing topic/year cells remain unknown and published zeroes are preserved. Data provided by ECDC based on data reported by EU/EEA Member States.

Norway

Norway FHI MSIS Statistics Bank

Norway

来源
每月official_json_api

Norway FHI MSIS national monthly notifications from the official Allvis frontend API; adapter contract checks guard the unversioned endpoint.

Poland

ECDC Surveillance Atlas — Poland Annual Baseline

Poland

来源
每年official_ecdc_rest_aggregate

Poland annual Member-State reported case counts from the ECDC Surveillance Atlas. Missing topic/year cells remain unknown and published zeroes are preserved. Data provided by ECDC based on data reported by EU/EEA Member States.

Portugal

ECDC Surveillance Atlas — Portugal Annual Baseline

Portugal

来源
每年official_ecdc_rest_aggregate

Portugal annual Member-State reported case counts from the ECDC Surveillance Atlas. Missing topic/year cells remain unknown and published zeroes are preserved. Data provided by ECDC based on data reported by EU/EEA Member States.

Romania

ECDC Surveillance Atlas — Romania Annual Baseline

Romania

来源
每年official_ecdc_rest_aggregate

Romania annual Member-State reported case counts from the ECDC Surveillance Atlas. Missing topic/year cells remain unknown and published zeroes are preserved. Data provided by ECDC based on data reported by EU/EEA Member States.

Sweden

Sweden Public Health Agency SmiNet

Sweden

来源
每月official_html_csv

Sweden SmiNet national monthly reported cases from the official Public Health Agency statistics pages; public release is enabled with closed-month publication and revisable recent-month refreshes.

Slovenia

ECDC Surveillance Atlas — Slovenia Annual Baseline

Slovenia

来源
每年official_ecdc_rest_aggregate

Slovenia annual Member-State reported case counts from the ECDC Surveillance Atlas. Missing topic/year cells remain unknown and published zeroes are preserved. Data provided by ECDC based on data reported by EU/EEA Member States.

Slovakia

ECDC Surveillance Atlas — Slovakia Annual Baseline

Slovakia

来源
每年official_ecdc_rest_aggregate

Slovakia annual Member-State reported case counts from the ECDC Surveillance Atlas. Missing topic/year cells remain unknown and published zeroes are preserved. Data provided by ECDC based on data reported by EU/EEA Member States.

Taiwan, China

Taiwan, China CDC NIDSS

Taiwan, China

来源
每月开放数据

Taiwan, China monthly notifiable infectious disease open-data CSV feed.

分块公开数据

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