Human Prion Disorders: Review of the Current Literature and a Twenty-Year Experience of the National Surveillance Center in the Czech Republic

Result code in IS VaVaI

<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F00064190%3A_____%2F21%3AN0000023" target="_blank" >RIV/00064190:_____/21:N0000023 - isvavai.cz</a>

Alternative codes found

RIV/00216208:11120/21:43922427 RIV/00216208:11110/21:10432790 RIV/00064165:_____/21:10432790 RIV/00064173:_____/21:N0000148

Result on the web

<a href="http://dx.doi.org/10.3390/diagnostics11101821" target="_blank" >http://dx.doi.org/10.3390/diagnostics11101821</a>

DOI - Digital Object Identifier

<a href="http://dx.doi.org/10.3390/diagnostics11101821" target="_blank" >10.3390/diagnostics11101821</a>

Result language

angličtina

Original language name

Human Prion Disorders: Review of the Current Literature and a Twenty-Year Experience of the National Surveillance Center in the Czech Republic

Original language description

Human prion disorders (transmissible spongiform encephalopathies, TSEs) are unique, progressive, and fatal neurodegenerative diseases caused by aggregation of misfolded prion protein in neuronal tissue. Due to the potential transmission, human TSEs are under active surveillance in a majority of countries; in the Czech Republic data are centralized at the National surveillance center (NRL) which has a clinical and a neuropathological subdivision. The aim of our article is to review current knowledge about human TSEs and summarize the experience of active surveillance of human prion diseases in the Czech Republic during the last 20 years. Possible or probable TSEs undergo a mandatory autopsy using a standardized protocol. From 2001 to 2020, 305 cases of sporadic and genetic TSEs including 8 rare cases of Gerstmann-Straussler-Scheinker syndrome (GSS) were confirmed. Additionally, in the Czech Republic, brain samples from all corneal donors have been tested by the NRL immunology laboratory to increase the safety of corneal transplants since January 2007. All tested 6590 corneal donor brain tissue samples were negative for prion protein deposits. Moreover, the routine use of diagnostic criteria including biomarkers are robust enough, and not even the COVID-19 pandemic has negatively impacted TSEs surveillance in the Czech Republic.

Czech name

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Czech description

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Type

J_imp - Article in a specialist periodical, which is included in the Web of Science database

CEP classification

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OECD FORD branch

30218 - General and internal medicine

Project

Result was created during the realization of more than one project. More information in the Projects tab.

Continuities

P - Projekt vyzkumu a vyvoje financovany z verejnych zdroju (s odkazem do CEP)<br>I - Institucionalni podpora na dlouhodoby koncepcni rozvoj vyzkumne organizace

Publication year

2021

Confidentiality

S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů

Name of the periodical

DIAGNOSTICS

ISSN

2075-4418

e-ISSN

2075-4418

Volume of the periodical

11

Issue of the periodical within the volume

10

Country of publishing house

CH - SWITZERLAND

Number of pages

19

Pages from-to

Article Number1821

UT code for WoS article

000717044900001

EID of the result in the Scopus database

2-s2.0-85116591944