The prion diseases or transmissible spongiform encephalopathies (TSEs), are a group of rare neurodegenerative disorders with a poor prognosis, correlated to the prion protein (PrP). In humans, these include three main categories: Sporadic Forms, Acquired Formsand Genetic Forms.
In animals – sheep, cattle, deer and other species – the prion diseases have different and specific designations: scrapie, bovine spongiform encephalopathy (BSE), chronic wasting disease. The first report of the disease prion in humans, Creutzfeldt-Jakob disease (CJD), appeared in 1920 (1-2), but only in 1982 Prusiner suggested the possibility that the transmissible agent was constituted by a protein without nucleic acids , partially resistant to proteolytic degradation (3).
17 January 2018
Epidemiological characteristics of human prion diseases
In the absence of specific therapies for human prion diseases, which are frequently associated with neurodegenerative disorders with fatal outcome, only an active surveillance can be used to monitor and prevent the transmission of such diseases. For this reason, starting from 1993, surveillance systems have been established in many countries. Moreover, there is urgent need […]
24 October 2017
Sporadic Creutzfeldt-Jakob Disease in 2 Plasma Product Recipients, United Kingdom
The work of Urwin reports the first two cases of Sporadic Creutzfeldt-Jakob disease in UK in patients with bleeding diathesis (one with hemophila B and one with von Willebrand disease) treated for long periods with plasma products; in both cases the clinical suspicion of Creutzfeldt-Jakob Disease was confirmed by biochemical and histopathologic findings. However, the […]
10 October 2016
Ten-year follow-up of two cohorts with an increased risk of variant CJD: donors to individuals who later developed variant CJD and other recipients of these at-risk donors.
Abstract BACKGROUND: Transmission of variant Creutzfeldt-Jakob disease (vCJD) through blood transfusion is implicated in three deaths and one asymptomatic infection. Based on this evidence, individuals assessed to be at increased risk of vCJD through donating blood transfused to individuals who later developed vCJD, or through being other recipients of such donors, are followed up to […]
Useful Links
- Recovery, safety, and tolerability of a solvent/detergent-treated and prion-safeguarded transfusion plasma in a randomized, crossover, clinical trial in healthy volunteers.
- TSE diagnostics: recent advances in immunoassaying prions
- Prospects for safe and effective vaccines against prion diseases.
- Pathogen inactivation and removal methods for plasma-derived clotting factor concentrates.
- How widespread is variant Creutzfeldt-Jakob disease?
- Development of Dose-Response Models of Creutzfeldt-Jakob Disease Infection in Nonhuman Primates for Assessing the Risk of Transfusion-Transmitted variant Creutzfeldt-Jakob Disease.
- TSE diagnostics: recent advances in immunoassaying prions.
- Removal of exogenous prion infectivity in leukoreduced red blood cells unit by a specific filter designed for human transfusion.
- Preclinical Detection of Variant CJD and BSE Prions in Blood.
- Evaluation of a test for its suitability in the diagnosis of variant Creutzfeldt-Jakob disease.
- Re-assessment of PrP(Sc) distribution in sporadic and variant CJD.
- Blood Test for Variant Creutzfeldt-Jakob Disease.
- Prions in the urine of patients with variant Creutzfeldt-Jakob disease.
- Lessons from the response to the threat of transfusion-transmitted vCJD in Ireland.
- Prions in the Urine of Patients with Variant Creutzfeldt–Jakob Disease
- Managing the risk of iatrogenic transmission of Creutzfeldt-Jakob disease in the UK.
- Parvovirus transmission by blood products – a cause for concern?
- Pathogen inactivation of whole blood and red cell components: An overview of concept, design, developments, criteria of acceptability and storage lesion
- Constant Transmission Properties of Variant Creutzfeldt-Jakob Disease in 5 Countries.
- Decontamination of prions in a plasma product manufacturing environment
- Variant Creutzfeldt-Jakob Disease With Extremely Low Lymphoreticular Deposition of Prion Protein
- Risk factors for variant Creutzfeldt-Jakob disease in dental practice: a case-control study
- Risk of transmission of Creutzfeldt-Jakob disease via blood and blood products. The French risk-analysis over the last 15 years
- The Management of Blood Safety in the Presence of Uncertain Risk: A United Kingdom Perspective
- Risk factors for variant Creutzfeldt-Jakob disease: a case-control study.
- Re-Assessment of PrPSc Distribution in Sporadic and Variant CJD
- The Genetics of Susceptibility to Variant Creutzfeldt-Jakob Disease
- Prion removal capacity of plasma protein manufacturing processes
- Highly Efficient Prion Transmission by Blood Transfusion
- Variant CJD and blood transfusion: are there additional cases?