The incidence of BSE in Europe is in continued decline. At present, iatrogenic transmission from person to person is considered a serious threat to public health. This report of the International Society of Blood Transfusion Working Party on Transmissible Spongiform Encephalopathy will focus on the state of the art in relation to blood components and plasma safety. Latest information on the pathogenicity of the infectious agent, the frequency and dynamics of infection in blood and transfusion transmissibility will be documented. Preventive measures including donor deferral policies, technologies for prion removal from labile blood components and for prion detection in plasma, the absence of a sensitive and rapid reference assay able to confirm the positive results from any putative blood screening assay will be updated. At last, as many uncertainties remain and a number of assumptions await confirmation, the areas to continue to explore are listed.
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http://dx.doi.org/10.1111/j.1423-0410.2009.01161.x | DOI Listing |
Cureus
December 2024
Medicine, King Saud University Medical School, Riyadh, SAU.
Creutzfeldt-Jakob disease (CJD) is a rare, rapidly progressive, and incurable neurodegenerative disorder caused by prions. It is invariably fatal and classified under transmissible spongiform encephalopathies. This case report presents a 66-year-old Saudi female who was admitted to the neurology department due to a rapidly advancing cognitive decline.
View Article and Find Full Text PDFAnimals (Basel)
December 2024
Korea Zoonosis Research Institute, Jeonbuk National University, Iksan 54531, Republic of Korea.
Prion diseases, or transmissible spongiform encephalopathies (TSEs), are a group of invariably fatal neurodegenerative disorders. One of the candidate genes involved in prion diseases is the shadow of the prion protein () gene. Raccoon dogs, a canid, are considered to be a prion disease-resistant species.
View Article and Find Full Text PDFAnimals (Basel)
December 2024
Department of Epidemiological Sciences, Animal and Plant Health Agency, Addlestone KT15 3NB, UK.
Atypical scrapie (AS) is a transmissible spongiform encephalopathy (TSE) that affects sheep and goats. Low within-flock incidence suggests that AS is not transmissible between animals, and testing of all animals that exit positive flocks for two years following detection (i.e.
View Article and Find Full Text PDFiScience
December 2024
Université Paris-Saclay, INRAe, UVSQ, VIM, 78350 Jouy-en-Josas, France.
Prion diseases, or transmissible spongiform encephalopathies (TSEs), are neurodegenerative disorders caused by the accumulation of misfolded conformers (PrP) of the cellular prion protein (PrP). During the pathogenesis, the PrP seeds disseminate in the central nervous system and convert PrP leading to the formation of insoluble assemblies. As for conventional infectious diseases, variations in the clinical manifestation define a specific prion strain which correspond to different PrP structures.
View Article and Find Full Text PDFPathology
November 2024
National CJD Research & Surveillance Unit, Centre for Clinical Brain Sciences, Chancellor's Building, University of Edinburgh, Edinburgh BioQuarter, Edinburgh, United Kingdom; Academic Department of Neuropathology, Centre for Clinical Brain Sciences, Chancellor's Building, University of Edinburgh, Edinburgh BioQuarter, Edinburgh, United Kingdom.
Human prion diseases are a rare group of transmissible neurodegenerative conditions which are classified according to their aetiology as sporadic, genetic or acquired forms. Creutzfeldt-Jakob disease (CJD) is the most common form of human prion disease, with the sporadic form accounting for ∼85% of all reported cases. While advances have been made in the development of clinical tools and biomarkers in the diagnosis of prion disease, allowing greater diagnostic certainty for surveillance purposes, definitive diagnosis requires neuropathological examination of the brain at postmortem.
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