Publications
| Title | Abstract | Year(sorted descending) Filter | PMID Filter |
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| molecular mechanisms of chronic wasting disease prion propagation. | prion disease epidemics, which have been unpredictable recurrences, are of significant concern for animal and human health. examples include kuru, once the leading cause of death among the fore people in papua new guinea and caused by mortuary feasting; bovine spongiform encephalopathy (bse) and its subsequent transmission to humans in the form of variant creutzfeldt-jakob disease (vcjd), and repeated examples of large-scale prion disease epidemics in animals caused by contaminated vaccines. the ... | 2017 | 28193766 |
| variant creutzfeldt-jakob disease in a patient with heterozygosity at prnp codon 129. | 2017 | 28099827 | |
| a heparin purification process removes spiked transmissible spongiform encephalopathy agent. | in 2000, bovine heparin was withdrawn from the us market for fear of contamination with bovine spongiform encephalopathy (bse) agent, the cause of variant creutzfeldt-jakob disease in humans. thus, us heparin is currently sourced only from pig intestines. availability of alternative sources of crude heparin, a life-saving drug, would benefit public health. bovine heparin is an obvious option, but bse clearance by the bovine heparin manufacturing process should be evaluated. to this end, using ha ... | 2017 | 28116677 |
| evaluation of rapid post-mortem test kits for bovine spongiform encephalopathy (bse) screening in japan: their analytical sensitivity to atypical bse prions. | a classical type of bovine spongiform encephalopathy (c-bse), recognized in 1987, had a large impact on public health due to its zoonotic link to variant creutzfeldt-jakob disease by the human consumption of dietary products contaminated with the c-bse prion. thus, a number of countries implemented bse surveillance using rapid post-mortem test kits that were approved for detection of the c-bse prion in the cattle brain. however, as atypical bse (l- and h-bse) cases emerged in subsequent years, t ... | 2017 | 28358272 |
| geographic exposure risk of variant creutzfeldt-jakob disease in us blood donors: a risk-ranking model to evaluate alternative donor-deferral policies. | variant creutzfeldt-jakob disease (vcjd) has been transmitted by blood transfusion (ttvcjd). the us food and drug administration (fda) recommends deferring blood donors who resided in or traveled to 30 european countries where they may have been exposed to bovine spongiform encephalopathy (bse) through beef consumption. those recommendations warrant re-evaluation, because new cases of bse and vcjd have markedly abated. | 2017 | 28261810 |
| detection and partial discrimination of atypical and classical bovine spongiform encephalopathies in cattle and primates using real-time quaking-induced conversion assay. | the transmission of classical bovine spongiform encephalopathy (c-bse) through contaminated meat product consumption is responsible for variant creutzfeldt-jakob disease (vcjd) in humans. more recent and atypical forms of bse (l-bse and h-bse) have been identified in cattle since the c-bse epidemic. their low incidence and advanced age of onset are compatible with a sporadic origin, as are most cases of creutzfeldt-jakob disease (cjd) in humans. transmissions studies in primates and transgenic m ... | 2017 | 28231300 |
| a closer look at prion strains: characterization and important implications. | prions are infectious proteins that are responsible for transmissible spongiform encephalopathies (tses) and consist primarily of scrapie prion protein (prp (sc) ), a pathogenic isoform of the host-encoded cellular prion protein (prp (c) ). the absence of nucleic acids as essential components of the infectious prions is the most striking feature associated to these diseases. additionally, different prion strains have been isolated from animal diseases despite the lack of dna or rna molecules. mo ... | 2017 | 23357828 |
| meeting report: pda virus and tse safety forum. | the report provides a summary of the presentations and discussions of the virus & tse (transmissible spongiform encephalopathy) safety forum 2011 that was organized by the parenteral drug association and held in barcelona, spain, on 28-30 june, 2011. the conference was accompanied by a workshop named "virus removal by filtration: trends and new developments." a summary of the workshop is provided as a separate report and will be published in this journal as well. the risk of virus contamination ... | 2016 | 23569070 |
| variant creutzfeldt-jakob disease deferral in canada: impact of stop dates. | to reduce the risk of variant creutzfeldt-jakob disease (vcjd) transmission via blood transfusion in canada, potential donors who spent a cumulative time in the united kingdom, western europe or saudi arabia are deferred. "stop dates" for accumulated time were later implemented for 3 months spent in the united kingdom or france (1980-1996) and for 5 years elsewhere in western europe (1980-2007); saudi arabia deferral was implemented with the "stop date" (1980-1996). we evaluated the long-term im ... | 2016 | 28151391 |
| creutzfeldt-jakob disease and blood transfusion: updated results of the uk transfusion medicine epidemiology review study. | this paper reports the results to 31 may 2015 of an ongoing uk study to look for additional cases of variant creutzfeldt-jakob disease (vcjd) transmission by blood transfusion, and to seek evidence whether other subtypes of creutzfeldt-jakob disease (cjd) may be transmissible via blood components. | 2016 | 26709606 |
| detection of prions in blood from patients with variant creutzfeldt-jakob disease. | human prion diseases are infectious and invariably fatal neurodegenerative diseases. they include sporadic creutzfeldt-jakob disease (scjd), the most common form, and variant cjd (vcjd), which is caused by interspecies transmission of prions from cattle infected by bovine spongiform encephalopathy. development of a biochemical assay for the sensitive, specific, early, and noninvasive detection of prions (prp(sc)) in the blood of patients affected by prion disease is a top medical priority to inc ... | 2016 | 28003548 |
| detection of prions in the plasma of presymptomatic and symptomatic patients with variant creutzfeldt-jakob disease. | variant creutzfeldt-jakob disease (vcjd) is a human prion disease resulting from the consumption of meat products contaminated by the agent causing bovine spongiform encephalopathy. evidence supporting the presence of a population of silent carriers that can potentially transmit the disease through blood transfusion is increasing. the development of a blood-screening assay for both symptomatic vcjd patients and asymptomatic carriers is urgently required. we show that a diagnostic assay combining ... | 2016 | 28003547 |
| absence of evidence for a causal link between bovine spongiform encephalopathy strain variant l-bse and known forms of sporadic creutzfeldt-jakob disease in human prp transgenic mice. | prions are proteinaceous pathogens responsible for subacute spongiform encephalopathies in animals and humans. the prions responsible for bovine spongiform encephalopathy (bse) are zoonotic agents, causing variant creutzfeldt-jakob disease (cjd) in humans. the transfer of prions between species is limited by a species barrier, which is thought to reflect structural incompatibilities between the host cellular prion protein (prp(c)) and the infecting pathological prp assemblies (prp(sc)) constitut ... | 2016 | 27681129 |
| a case cluster of variant creutzfeldt-jakob disease linked to the kingdom of saudi arabia. | as of mid-2016, 231 cases of variant creutzfeldt-jakob disease-the human form of a prion disease of cattle, bovine spongiform encephalopathy-have been reported from 12 countries. with few exceptions, the affected individuals had histories of extended residence in the uk or other western european countries during the period (1980-96) of maximum global risk for human exposure to bovine spongiform encephalopathy. however, the possibility remains that other geographic foci of human infection exist, ... | 2016 | 27671029 |
| prion diseases: immunotargets and therapy. | transmissible spongiform encephathalopathies or prion diseases are a group of neurological disorders characterized by neuronal loss, spongiform degeneration, and activation of astrocytes or microglia. these diseases affect humans and animals with an extremely high prevalence in some species such as deer and elk in north america. although rare in humans, they result in a devastatingly swift neurological progression with dementia and ataxia. patients usually die within a year of diagnosis. prion d ... | 2016 | 27529062 |
| are prions transported by plasma exosomes? | blood has been shown to contain disease-associated misfolded prion protein (prp(tse)) in animals naturally and experimentally infected with various transmissible spongiform encephalopathy (tse) agents, and in humans infected with variant creutzfeldt-jakob disease (vcjd). recently, we have demonstrated prp(tse) in extracellular vesicle preparations (evs) containing exosomes from plasma of mice infected with mouse-adapted vcjd by protein misfolding cyclic amplification (pmca). here we report the d ... | 2016 | 27499183 |
| guinea pig prion protein supports rapid propagation of bovine spongiform encephalopathy and variant creutzfeldt-jakob disease prions. | the biochemical and neuropathological properties of bovine spongiform encephalopathy (bse) and variant creutzfeldt-jakob disease (vcjd) prions are faithfully maintained upon transmission to guinea pigs. however, primary and secondary transmissions of bse and vcjd in guinea pigs result in long incubation periods of ∼450 and ∼350 days, respectively. to determine if the incubation periods of bse and vcjd prions could be shortened, we generated transgenic (tg) mice expressing guinea pig prion protei ... | 2016 | 27440899 |
| 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. | 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 further understand the risks of vcjd transmission through blood. | 2016 | 27432362 |
| clinical issues-may 2016. | variations in documenting surgical wound classification key words: surgical wound classification, clean, clean-contaminated, contaminated, dirty. wearing long-sleeved jackets while preparing and packaging items for sterilization key words: long-sleeved jackets, organic material, sterile processing. endoscopic transmission of prions key words: prions, high-risk tissue, low-risk tissue, creutzfeldt-jakob disease (cjd), variant creutzfeldt-jakob disease (vcjd). wearing gloves when handling flexible ... | 2016 | 27129755 |
| persistent residual contamination in endoscope channels; a fluorescence epimicroscopy study. | the increasing demand for endoscopic procedures poses new contamination challenges, given developing antimicrobial resistance worldwide and potential viral or prion diseases in populations at risk. we examined working channels from reusable luminal endoscopes used in recent years. | 2016 | 27110695 |
| meeting report: 2015 pda virus & tse safety forum. | the report provides a summary of the presentations at the virus & tse safety forum 2015 organized by the parenteral drug association (pda) and held in cascais, portugal, from 9 to 11 june, 2015. as with previous conferences of this series, the pda virus & tse safety forum 2015 provided an excellent forum for the exchange of information and opinions between the industry, research organizations, and regulatory bodies. regulatory updates on virus and tse safety aspects illustrating current topics o ... | 2016 | 27020643 |
| rapid and highly sensitive detection of variant creutzfeldt-jakob disease abnormal prion protein on steel surfaces by protein misfolding cyclic amplification: application to prion decontamination studies. | the prevalence of variant creutzfeldt-jakob disease (vcjd) in the population remains uncertain, although it has been estimated that 1 in 2000 people in the united kingdom are positive for abnormal prion protein (prptse) by a recent survey of archived appendix tissues. the prominent lymphotropism of vcjd prions raises the possibility that some surgical procedures may be at risk of iatrogenic vcjd transmission in healthcare facilities. it is therefore vital that decontamination procedures applied ... | 2016 | 26800081 |
| mononucleated blood cell populations display different abilities to transmit prion disease by the transfusion route. | previous experiments carried out in a sheep scrapie model demonstrated that the transfusion of 200 μl of prion-infected whole blood has an apparent 100% efficacy for disease transmission. these experiments also indicated that, despite the apparent low infectious titer, the intravenous administration of white blood cells (wbc) resulted in efficient disease transmission. in the study presented here, using the same transmissible spongiform encephalopathy (tse) animal model, our aim was to determine ... | 2016 | 26764000 |
| efficient propagation of variant creutzfeldt-jakob disease prion protein using the cell-protein misfolding cyclic amplification technique with samples containing plasma and heparin. | to prevent the iatrogenic spread of variant creutzfeldt-jakob disease (vcjd) between humans via blood products or transfusion, highly sensitive in vitro screening tests are necessary. protein misfolding cyclic amplification (pmca) is one such candidate test. however, plasma has been reported to inhibit the pmca reaction. therefore, we investigated the cell-pmca conditions that permit vcjd prion amplification in the presence of plasma. | 2016 | 26347231 |
| blood transmission studies of prion infectivity in the squirrel monkey (saimiri sciureus): the baxter study. | four secondary transmissions of variant creutzfeldt-jakob disease (vcjd) infectivity have been associated with the transfusion of nonleukoreduced red blood cells collected from vcjd patients during the asymptomatic phase of the disease. establishing efficient experimental models for assessing the risk of future transmissions of vcjd infectivity via blood transfusion is of paramount importance in view of a study of archived appendix samples in which the prevalence of asymptomatic vcjd infection i ... | 2016 | 26594017 |
| whole blood gene expression profiling in preclinical and clinical cattle infected with atypical bovine spongiform encephalopathy. | prion diseases, such as bovine spongiform encephalopathies (bse), are transmissible neurodegenerative disorders affecting humans and a wide variety of mammals. variant creutzfeldt-jakob disease (vcjd), a prion disease in humans, has been linked to exposure to bse prions. this classical bse (cbse) is now rapidly disappearing as a result of appropriate measures to control animal feeding. besides cbse, two atypical forms (named h- and l-type bse) have recently been described in europe, japan, and n ... | 2016 | 27073865 |
| exploring the zoonotic potential of animal prion diseases: in vivo and in vitro approaches. | following the discovery of a causal link between bovine spongiform encephalopathy (bse) in cattle and variant creutzfeldt-jakob disease (vcjd) in humans, several experimental approaches have been used to try to assess the potential risk of transmission of other animal transmissible spongiform encephalopathies (tses) to humans. experimental challenge of non-human primates, humanised transgenic mice and cell-free conversion systems have all been used as models to explore the susceptibility of huma ... | 2016 | 24549113 |
| disease burden of 32 infectious diseases in the netherlands, 2007-2011. | infectious disease burden estimates provided by a composite health measure give a balanced view of the true impact of a disease on a population, allowing the relative impact of diseases that differ in severity and mortality to be monitored over time. this article presents the first national disease burden estimates for a comprehensive set of 32 infectious diseases in the netherlands. | 2016 | 27097024 |
| shadoo/prp (sprn(0/0) /prnp(0/0) ) double knockout mice: more than zeroes. | shadoo (sho) is a brain glycoprotein with similarities to the unstructured region of prp (c) . frameshift alleles of the sho gene, sprn, are reported in variant creutzfeldt-jakob disease (vcjd) patients while sprn mrna knockdown in prp-null (prnp(0/0) ) embryos produces lethality, advancing sho as the hypothetical prp-like "pi" protein. also, sho levels are reduced as misfolded prp accumulates during prion infections. to penetrate these issues we created sprn null alleles (daude et al., proc. na ... | 2016 | 22929230 |
| clinical features of variant creutzfeldt-jakob disease. | since 1996, over one hundred cases of variant creutzfeldt-jakob disease have appeared, mostly in the united kingdom. in this review, we summarise the major clinical features of this progressive neurodegenerative condition and compare them with those of sporadic creutzfeldt-jakob disease. we emphasise the young age (median 26 years) at presentation and the dominant psychiatric/behavioural features, particularly depression. sensory symptoms are present initially in half the cases and florid psychi ... | 2016 | 11987139 |
| preclinical vcjd after blood transfusion in a prnp codon 129 heterozygous patient. | we report a case of preclinical variant creutzfeldt-jakob disease (vcjd) in a patient who died from a non-neurological disorder 5 years after receiving a blood transfusion from a donor who subsequently developed vcjd. protease-resistant prion protein (prp(res)) was detected by western blot, paraffin-embedded tissue blot, and immunohistochemistry in the spleen, but not in the brain. immunohistochemistry for prion protein was also positive in a cervical lymph node. the patient was a heterozygote a ... | 2015 | 15302196 |
| recent us case of variant creutzfeldt-jakob disease-global implications. | variant creutzfeldt-jakob disease (vcjd) is a rare, fatal prion disease resulting from transmission to humans of the infectious agent of bovine spongiform encephalopathy. we describe the clinical presentation of a recent case of vcjd in the united states and provide an update on diagnostic testing. the location of this patient's exposure is less clear than those in the 3 previously reported us cases, but strong evidence indicates that exposure to contaminated beef occurred outside the united sta ... | 2015 | 25897712 |
| susceptibility of european red deer (cervus elaphus elaphus) to alimentary challenge with bovine spongiform encephalopathy. | european red deer (cervus elaphus elaphus) are susceptible to the agent of bovine spongiform encephalopathy, one of the transmissible spongiform encephalopathies, when challenged intracerebrally but their susceptibility to alimentary challenge, the presumed natural route of transmission, is unknown. to determine this, eighteen deer were challenged via stomach tube with a large dose of the bovine spongiform encephalopathy agent and clinical signs, gross and histological lesions, presence and dist ... | 2015 | 25615837 |
| chronic wasting disease: fingerprinting the culprit in risk assessments. | transmissible spongiform encephalopathies (prion diseases) in animals may be associated with a zoonotic risk potential for humans as shown by the occurrence of variant creutzfeldt-jakob disease in the wake of the bovine spongiform encephalopathy epidemic. thus, the increasing exposure of humans in north america to cervid prions of chronic wasting disease (cwd) in elk and deer has prompted comprehensive risk assessments. the susceptibility of humans to cwd infections is currently under investigat ... | 2015 | 22453172 |
| an inventory of concerns behind blood safety policies in five western countries. | the availability of costly safety measures against transfusion-transmissible infections forces western countries to confront difficult ethical questions. how to decide about implementing such measures? when are such decisions justified? as a preliminary to addressing these questions, we assessed which concerns shape actual donor blood safety policymaking in five western countries. | 2015 | 26331441 |
| donor recruitment for fecal microbiota transplantation. | increasing demand for fecal microbiota transplantation (fmt) has created a need for stool banks sourced from long-term healthy donors. here, we describe our experience in recruiting and screening fecal donors. | 2015 | 26070003 |
| methods for differentiating prion types in food-producing animals. | prions are an enigma amongst infectious disease agents as they lack a genome yet confer specific pathologies thought to be dictated mainly, if not solely, by the conformation of the disease form of the prion protein (prp(sc)). prion diseases affect humans and animals, the latter including the food-producing ruminant species cattle, sheep, goats and deer. importantly, it has been shown that the disease agent of bovine spongiform encephalopathy (bse) is zoonotic, causing variant creutzfeldt jakob ... | 2015 | 26580664 |
| transmission of scrapie prions to primate after an extended silent incubation period. | classical bovine spongiform encephalopathy (c-bse) is the only animal prion disease reputed to be zoonotic, causing variant creutzfeldt-jakob disease (vcjd) in humans and having guided protective measures for animal and human health against animal prion diseases. recently, partial transmissions to humanized mice showed that the zoonotic potential of scrapie might be similar to c-bse. we here report the direct transmission of a natural classical scrapie isolate to cynomolgus macaque, a highly rel ... | 2015 | 26123044 |
| the transmissible spongiform encephalopathies of livestock. | prion diseases or transmissible spongiform encephalopathies (tses) are fatal protein-misfolding neurodegenerative diseases. tses have been described in several species, including bovine spongiform encephalopathy (bse) in cattle, scrapie in sheep and goats, chronic wasting disease (cwd) in cervids, transmissible mink encephalopathy (tme) in mink, and kuru and creutzfeldt-jakob disease (cjd) in humans. these diseases are associated with the accumulation of a protease-resistant, disease-associated ... | 2015 | 25991695 |
| generation of a persistently infected mdbk cell line with natural bovine spongiform encephalopathy (bse). | bovine spongiform encephalopathy (bse) is a zoonotic transmissible spongiform encephalopathy (tse) thought to be caused by the same prion strain as variant creutzfeldt-jakob disease (vcjd). unlike scrapie and chronic wasting disease there is no cell culture model allowing the replication of proteinase k resistant bse (prpbse) and the further in vitro study of this disease. we have generated a cell line based on the madin-darby bovine kidney (mdbk) cell line over-expressing the bovine prion prote ... | 2015 | 25647616 |
| the canadian management of bovine spongiform encephalopathy in historical and scientific perspective, 1990-2014. | on february 11, 2015, the canadian food inspection agency announced that a cow born and raised in alberta had tested positive for bovine spongiform encephalopathy (bse), commonly known as mad cow disease. bse is a prion disease of cattle that, when transmitted to humans, produces a fatal neurodegenerative disease known as variant creutzfeldt-jakob disease. we believe that this latest case of bse in canadian cattle suggests the timeliness of a review of the management of bse in canada from a hist ... | 2015 | 26357946 |
| leukoreduction and blood-borne vcjd transmission risk. | risk assessments for transmission of variant creutzfeldt-jakob disease predicted that leukocyte reduction would be inefficient at preventing transmission of the disease by blood transfusion. nevertheless, approximately 14 years ago, a significant proportion of european countries decided to implement leukocyte reduction treatment within their human blood supplies in order to mitigate the risk of human prion disease transmission. | 2015 | 25402620 |
| is the continued use of uk plasma sourced cryoprecipitate justified? | 2015 | 25302409 | |
| prospects for safe and effective vaccines against prion diseases. | prion diseases are subacute neurodegenerative diseases that affect humans and animals. an abnormally folded isoform (prp(sc)) of the host cellular prion protein is considered to constitute the major, if not sole, component of the infectious prion. the occurrence of variant creutzfeldt-jakob disease in humans most likely arose due to consumption of food contaminated with bovine spongiform encephalopathy prions. the demonstration that some prion infections may have the capacity to transmit to othe ... | 2015 | 25266267 |
| development of a bifunctional filter for prion protein and leukoreduction of red blood cell components. | leukofiltration of blood components is currently implemented worldwide as a precautionary measure against white blood cell-associated adverse effects and the potential transmission of variant creutzfeldt-jakob disease (vcjd). a newly developed bifunctional filter (sepacell prima, asahi kasei medical) was assessed for prion removal, leukoreduction (lr), and whether the filter significantly affected red blood cells (rbcs). | 2015 | 25178436 |
| bank vole prion protein as an apparently universal substrate for rt-quic-based detection and discrimination of prion strains. | prions propagate as multiple strains in a wide variety of mammalian species. the detection of all such strains by a single ultrasensitive assay such as real time quaking-induced conversion (rt-quic) would facilitate prion disease diagnosis, surveillance and research. previous studies have shown that bank voles, and transgenic mice expressing bank vole prion protein, are susceptible to most, if not all, types of prions. here we show that bacterially expressed recombinant bank vole prion protein ( ... | 2015 | 26086786 |
| a prion reduction filter does not completely remove endogenous prion infectivity from sheep blood. | variant creutzfeldt-jakob disease (vcjd) is a transmissible spongiform encephalopathy affecting humans, acquired initially through infection with bovine spongiform encephalopathy (bse). a small number of vcjd cases have been acquired through the transfusion of blood from asymptomatic donors who subsequently developed vcjd. filter devices that selectively bind the infectious agent associated with prion disease have been developed for removal of infection from blood. this study independently asses ... | 2015 | 26032915 |
| comparative quantitative study of 'signature' pathological lesions in the hippocampus and adjacent gyri of 12 neurodegenerative disorders. | the hippocampus (hc) and adjacent gyri are implicated in dementia in several neurodegenerative disorders. to compare hc pathology among disorders, densities of 'signature' pathological lesions were measured at a standard location in eight brain regions of 12 disorders. principal components analysis of the data suggested that the disorders could be divided into three groups: (1) alzheimer's disease (ad), down's syndrome (ds), sporadic creutzfeldt-jakob disease, and variant creutzfeldt-jakob disea ... | 2015 | 25929331 |
| foodborne-transmitted prions from the brain of cows with bovine spongiform encephalopathy ascend in afferent neurons to the simian central nervous system and spread to tonsils and spleen at a late stage of the incubation period. | protease-resistant prion protein (prp(res)) accumulation in lymphoreticular tissues indicates prion infection. to date, tonsillectomy and appendectomy samples have been used in population prevalence surveys to detect clinically silent carriers of variant creutzfeldt-jakob disease (vcjd). however, the temporal sequence of prion spread in the human body is still not known. we therefore traced the temporal-spatial pattern of prp(res) accumulation in the body of a simian vcjd model. | 2015 | 25895987 |
| removal of transmissible spongiform encephalopathy prion from large volumes of cell culture media supplemented with fetal bovine serum by using hollow fiber anion-exchange membrane chromatography. | cases of variant creutzfeldt-jakob disease in people who had consumed contaminated meat products from cattle with bovine spongiform encephalopathy emphasize the need for measures aimed at preventing the transmission of the pathogenic prion protein (prpsc) from materials derived from cattle. highly stringent scrutiny is required for fetal bovine serum (fbs), a growth-medium supplement used in the production of parenteral vaccines and therapeutic recombinant proteins and in the ex vivo expansion o ... | 2015 | 25874629 |
| mri detection of prion protein plaques in variant creutzfeldt-jakob disease. | 2015 | 25846996 | |
| rare structural genetic variation in human prion diseases. | prion diseases are a diverse group of neurodegenerative conditions, caused by the templated misfolding of prion protein. aside from the strong genetic risk conferred by multiple variants of the prion protein gene (prnp), several other variants have been suggested to confer risk in the most common type, sporadic creutzfeldt-jakob disease (scjd) or in the acquired prion diseases. large and rare copy number variants (cnvs) are known to confer risk in several related disorders including alzheimer's ... | 2015 | 25726360 |
| evaluation of the protection of primates transfused with variant creutzfeldt-jakob disease-infected blood products filtered with prion removal devices: a 5-year update. | analysis of archived appendix samples reveals that one in 2000 individuals in the united kingdom may carry the infectious prion protein associated with variant creutzfeldt-jakob disease (vcjd), raising questions about the risk of transfusion transmission from apparently healthy carriers. blood leukoreduction shows limited efficiency against prions. therefore, in absence of antemortem diagnostic tests, prion removal filters, including the p-capt filter were designed to improve blood transfusion s ... | 2015 | 25647476 |
| variant creutzfeldt-jakob disease in older patients. | 2015 | 25609647 | |
| batch recall of french plasma-derived products due to variant creutzfeldt-jakob disease risk: the psychological impact on haemophilic patients, changes in their therapeutic demands and behaviour and ethical considerations. | the choice of plasma-derived products (pdp) vs. recombinant products (rp) for treating haemophilia is influenced by the infectious and perceived safety of the products. batch recall of pdp due to the risk of variant creutzfeldt-jakob disease (vcjd) may have unfavourable psychological impacts on haemophilia patients and influence their product preferences. this study aimed to assess the psychological impact of batch recalls of pdp in six haemophilia patients and their therapeutic demands, and to ... | 2015 | 25545300 |
| the end of the bse saga: do we still need surveillance for human prion diseases? | the epidemics of classical bovine spongiform encephalopathy (bse) and variant creutzfeldt-jakob disease (vcjd) related to bse-infected food are coming to an end. the decline in concern about these diseases may invite complacency and questions whether surveillance for human prion diseases is still necessary. this article reviews the main points of surveillance and why it is still needed: animal sources for human prion infection other than bse cannot be excluded; the potentially increasing circula ... | 2015 | 26715203 |
| donating blood for research: a potential method for enhancing customer satisfaction of permanently deferred blood donors. | each year, a large number of individuals in australia are deferred from donating blood. a deferral may have a negative impact on donor satisfaction and subsequent word-of-mouth communication. the australian red cross blood service (the blood service) is, therefore, investigating options for managing service interactions with deferred donors to maintain positive relationships. while public research institutes in australia have established independent research donor registries, other countries pro ... | 2015 | 26674813 |
| preclinical detection of infectivity and disease-specific prp in blood throughout the incubation period of prion disease. | variant creutzfeldt-jakob disease (vcjd) is a fatal neurodegenerative disorder characterised by accumulation of pathological isoforms of the prion protein, prp. although cases of clinical vcjd are rare, there is evidence there may be tens of thousands of infectious carriers in the united kingdom alone. this raises concern about the potential for perpetuation of infection via medical procedures, in particular transfusion of contaminated blood products. accurate biochemical detection of prion infe ... | 2015 | 26631638 |
| overview and evaluation of 15 years of creutzfeldt-jakob disease surveillance in belgium, 1998-2012. | in 1998, following the detection of variant creutzfeldt-jakob disease (vcjd) in the uk, belgium installed a surveillance system for creutzfeldt-jakob disease (cjd). the objectives of this system were to identify vcjd cases and detect increases in cjd incidence. diagnostic confirmation of cjd is based on autopsy after referral by neurologists. reference centres perform autopsies and report to the surveillance system. the aim of this study was to assess whether the system met its objectives and to ... | 2015 | 26630984 |
| live kidney donation from a person with haemophilia. | there are many documented cases of a person with haemophilia successfully receiving a solid organ transplant, including liver and kidney. however, there is no literature reporting live organ donation by a person with haemophilia. presumably, this is because the associated risks of excessive bleeding, inhibitor development after a period of intensive treatment with factor replacement and the possibility of variant creutzfeldt-jakob disease transmission in those previously treated with blood produ ... | 2015 | 26628308 |
| the role of the donor liaison officer at pluslife (perth bone and tissue bank inc.), western australia. | femoral head donation at the time of hip replacement surgery provides a much needed resource of bone allograft to orthopaedic surgeons. prior to 2005, potential femoral head donors were identified and consented in the hospital setting on the day of surgery. this resulted in over 40 % of donations failing post operatively suggesting that more effort could be given to pre-operative screening resulting in substantial savings in the cost associated with collection and testing of donors who were subs ... | 2015 | 25080848 |
| blood reference materials from macaques infected with variant creutzfeldt-jakob disease agent. | variant creutzfeldt-jakob disease (vcjd) is a fatal neurodegenerative infection that can be transmitted by blood and blood products from donors in the latent phase of the disease. currently, there is no validated antemortem vcjd blood screening test. several blood tests are under development. any useful test must be validated with disease-relevant blood reference panels. | 2015 | 25154296 |
| white blood cell-based detection of asymptomatic scrapie infection by ex vivo assays. | prion transmission can occur by blood transfusion in human variant creutzfeldt-jakob disease and in experimental animal models, including sheep. screening of blood and its derivatives for the presence of prions became therefore a major public health issue. as infectious titer in blood is reportedly low, highly sensitive and robust methods are required to detect prions in blood and blood derived products. the objectives of this study were to compare different methods--in vitro, ex vivo and in viv ... | 2014 | 25122456 |
| blood test for variant creutzfeldt-jakob disease--reply. | 2014 | 25111211 | |
| blood test for variant creutzfeldt-jakob disease. | 2014 | 25111210 | |
| genotype-dependent molecular evolution of sheep bovine spongiform encephalopathy (bse) prions in vitro affects their zoonotic potential. | prion diseases are rare fatal neurological conditions of humans and animals, one of which (variant creutzfeldt-jakob disease) is known to be a zoonotic form of the cattle disease bovine spongiform encephalopathy (bse). what makes one animal prion disease zoonotic and others not is poorly understood, but it appears to involve compatibility between the prion strain and the host prion protein sequence. concerns have been raised that the united kingdom sheep flock may have been exposed to bse early ... | 2014 | 25100723 |
| prions in the urine of patients with variant creutzfeldt-jakob disease. | prions, the infectious agents responsible for transmissible spongiform encephalopathies, consist mainly of the misfolded prion protein (prp(sc)). the unique mechanism of transmission and the appearance of a variant form of creutzfeldt-jakob disease, which has been linked to consumption of prion-contaminated cattle meat, have raised concerns about public health. evidence suggests that variant creutzfeldt-jakob disease prions circulate in body fluids from people in whom the disease is silently inc ... | 2014 | 25099577 |
| managing the risk of iatrogenic transmission of creutzfeldt-jakob disease in the uk. | with the emergence of bovine spongiform encephalopathy (bse) and variant creutzfeldt-jakob disease (cjd) in the uk, there is concern about iatrogenic transmission, and the approach to managing this risk is unique. | 2014 | 25082752 |
| detection of infectivity in blood of persons with variant and sporadic creutzfeldt-jakob disease. | we report the presence of infectivity in erythrocytes, leukocytes, and plasma of 1 person with variant creutzfeldt-jakob disease and in the plasma of 2 in 4 persons whose tests were positive for sporadic creutzfeldt-jakob disease. the measured infectivity levels were comparable to those reported in various animals with transmissible spongiform encephalopathies. | 2014 | 24377668 |
| evidence of subclinical prion disease in aged mice following exposure to bovine spongiform encephalopathy. | the occurrence of variant creutzfeldt-jakob (vcjd) disease in humans was almost certainly the result of consumption of food contaminated with bovine spongiform encephalopathy (bse) prions. despite probable widespread exposure of the uk population to bse-contaminated food in the 1980s, vcjd has been identified predominantly in young individuals, and there have been fewer cases of clinical disease than anticipated. the reasons for this are uncertain. following peripheral exposure, many prions repl ... | 2014 | 24123519 |
| removal of exogenous prion infectivity in leukoreduced red blood cells unit by a specific filter designed for human transfusion. | five cases of variant creutzfeldt-jakob disease (vcjd) infections were attributed to infusion of contaminated blood components, turning to real the interhuman transmissibility of this prion disease from asymptomatic carriers. preventive policies rely on exclusion from blood donation and benefit of leukoreduction initially implemented against leukotropic viruses. in the absence of available antemortem diagnostic tests, the updated prevalence of silent vcjd infections (1/2000 in the united kingdom ... | 2014 | 24117735 |
| ultrasensitive detection of prp(sc) in the cerebrospinal fluid and blood of macaques infected with bovine spongiform encephalopathy prion. | prion diseases are characterized by the prominent accumulation of the misfolded form of a normal cellular protein (prp(sc)) in the central nervous system. the pathological features and biochemical properties of prp(sc) in macaque monkeys infected with the bovine spongiform encephalopathy (bse) prion have been found to be similar to those of human subjects with variant creutzfeldt-jakob disease (vcjd). non-human primate models are thus ideally suited for performing valid diagnostic tests and dete ... | 2014 | 25024281 |
| infectious prion diseases in humans: cannibalism, iatrogenicity and zoonoses. | in contrast with other neurodegenerative disorders associated to protein misfolding, human prion diseases include infectious forms (also called transmitted forms) such as kuru, iatrogenic creutzfeldt-jakob disease and variant creutzfeldt-jakob disease. the transmissible agent is thought to be solely composed of the abnormal isoform (prp(sc)) of the host-encoded prion protein that accumulated in the central nervous system of affected individuals. compared to its normal counterpart, prp(sc) is β-s ... | 2014 | 24956437 |
| preclinical detection of variant cjd and bse prions in blood. | the emergence of variant creutzfeldt jakob disease (vcjd) is considered a likely consequence of human dietary exposure to bovine spongiform encephalopathy (bse) agent. more recently, secondary vcjd cases were identified in patients transfused with blood products prepared from apparently healthy donors who later went on to develop the disease. as there is no validated assay for detection of vcjd/bse infected individuals the prevalence of the disease in the population remains uncertain. in that co ... | 2014 | 24945656 |
| temporal dynamics of hippocampal neurogenesis in chronic neurodegeneration. | the study of neurogenesis during chronic neurodegeneration is crucial in order to understand the intrinsic repair mechanisms of the brain, and key to designing therapeutic strategies. in this study, using an experimental model of progressive chronic neurodegeneration, murine prion disease, we define the temporal dynamics of the generation, maturation and integration of new neurons in the hippocampal dentate gyrus, using dual pulse-chase, multicolour γ-retroviral tracing, transmission electron mi ... | 2014 | 24941947 |
| update on the provisional estimation of developing iatrogenic variant creutzfeldt-jakob disease from human islet cell transplantation procedures. | 2014 | 24933255 | |
| variant cjd and blood transfusion: are there additional cases? | in this study, we compare variant creutzfeldt-jakob disease (vcjd) cases definitely linked to blood transfusion, those with a history of blood transfusion in which no donor has developed vcjd and primary cases with no history of blood transfusion. the aim is to determine whether there are any differences in the demographics or clinical phenotype in these groups that might suggest additional cases of transfusion transmission of vcjd. | 2014 | 24916465 |
| accelerated, spleen-based titration of variant creutzfeldt-jakob disease infectivity in transgenic mice expressing human prion protein with sensitivity comparable to that of survival time bioassay. | the dietary exposure of the human population to the prions responsible for the bovine spongiform encephalopathy (bse) epizooty has led to the emergence of variant creutzfeldt-jakob disease (vcjd). this fatal, untreatable neurodegenerative disorder is a growing public health concern because the prevalence of the infection seems much greater than the disease incidence and because secondary transmission of vcjd by blood transfusion or use of blood products has occurred. a current limitation in vari ... | 2014 | 24850746 |
| increased susceptibility of transgenic mice expressing human prp to experimental sheep bovine spongiform encephalopathy is not due to increased agent titre in sheep brain tissue. | bovine spongiform encephalopathy (bse) in cattle and variant creutzfeldt-jakob disease in humans have previously been shown to be caused by the same strain of transmissible spongiform encephalopathy agent. it is hypothesized that the agent spread to humans following consumption of food products prepared from infected cattle. despite evidence supporting zoonotic transmission, mouse models expressing human prion protein (hutg) have consistently shown poor transmission rates when inoculated with ca ... | 2014 | 24828334 |
| distinct transmissibility features of tse sources derived from ruminant prion diseases by the oral route in a transgenic mouse model (tgovprp4) overexpressing the ovine prion protein. | transmissible spongiform encephalopathies (tses) are a group of fatal neurodegenerative diseases associated with a misfolded form of host-encoded prion protein (prp). some of them, such as classical bovine spongiform encephalopathy in cattle (bse), transmissible mink encephalopathy (tme), kuru and variant creutzfeldt-jakob disease in humans, are acquired by the oral route exposure to infected tissues. we investigated the possible transmission by the oral route of a panel of strains derived from ... | 2014 | 24797075 |
| a refined method for estimating the size of the potential blood donor pool in australia. | to be eligible to donate blood, potential donors must meet certain eligibility criteria to ensure safety to the donor and to the blood supply. in australia, there is no reliable estimate of the size of the donor-eligible population. this study uses a refinement to a published method to determine the population prevalence of donor-exclusion factors and subsequently estimates the size of the potential donor pool in australia. | 2014 | 24796704 |
| investigation of variant creutzfeldt-jakob disease implicated organ or tissue transplantation in the united kingdom. | person-to-person transmission of variant creutzfeldt-jakob disease (vcjd) has occurred through blood transfusion and could also theoretically occur as a result of the transplantation of organs or tissues. this study aimed to investigate whether there were transplant-associated vcjd cases in the united kingdom (uk). | 2014 | 24770619 |
| human prion diseases and the risk of their transmission during anatomical dissection. | prion diseases (or transmissible spongiform encephalopathies) are a unique group of fatal progressive neurodegenerative diseases of the central nervous system. the infectious agent is hypothesized to consist solely of a highly protease-resistant misfolded isoform of the host prion protein. prions display a remarkable degree of resistance to chemical and physical decontamination. many common forms of decontamination or neutralization used in infection control are ineffective against prions, excep ... | 2014 | 24740900 |
| risk assessment for transmission of variant creutzfeldt-jakob disease by transfusion of red blood cells in the united states. | variant creutzfeldt-jakob disease (vcjd) is transmitted by blood transfusion. to mitigate the risk of transfusion-transmitted vcjd (ttvcjd), the us food and drug administration has recommended deferral of potential at-risk blood donors, but some risk remains. we describe a quantitative risk assessment to estimate residual, postdeferral ttvcjd risk in the united states. | 2014 | 24689837 |
| population screening for variant creutzfeldt-jakob disease using a novel blood test: diagnostic accuracy and feasibility study. | our study indicates a prototype blood-based variant creutzfeldt-jakob disease (vcjd) assay has sufficient sensitivity and specificity to justify a large study comparing vcjd prevalence in the united kingdom with a bovine spongiform encephalopathy-unexposed population. in a clinical diagnostic capacity, the assay's likelihood ratios dramatically change an individual's pretest disease odds to posttest probabilities and can confirm vcjd infection. | 2014 | 24590363 |
| postmortem findings in a case of variant creutzfeldt-jakob disease treated with intraventricular pentosan polysulfate. | a small number of patients with variant creutzfeldt-jakob disease (vcjd) have been treated with intraventicular pentosan polysulfate (ipps) and extended survival has been reported in some cases. to date, there have been no reports on the findings of postmortem examination of the brain in treated patients and the reasons for the extended survival are uncertain. we report on the neuropathological findings in a case of vcjd treated with pps. | 2014 | 24554103 |
| variant creutzfeldt-jakob disease with extremely low lymphoreticular deposition of prion protein. | human transmission of bovine spongiform encephalopathy causes the fatal neurodegenerative condition variant creutzfeldt-jakob disease (vcjd) and, based on recent human prevalence studies, significant subclinical prion infection of the uk population. to date, all clinical cases have been fatal, totaling 228 mostly young adults residing in the united kingdom. | 2014 | 24445428 |
| [creutzfeldt-jakob disease in a person over the age of 80]. | creutzfeldt-jakob disease is a rare neurodegenerative disease that can occur in four forms. the sporadic form, the familial form, the iatrogenic form and variant creutzfeldt-jakob disease. the sporadic form is seen in about 1 per million people per year and is associated with rapidly progressive cognitive decline, psychiatric and neurological symptoms. the diagnosis and counseling of patients is a major challenge because of the complex and heterogeneous presentation, especially if the presentati ... | 2014 | 24430016 |
| human tonsil-derived follicular dendritic-like cells are refractory to human prion infection in vitro and traffic disease-associated prion protein to lysosomes. | the molecular mechanisms involved in human cellular susceptibility to prion infection remain poorly defined. this is due, in part, to the absence of any well characterized and relevant cultured human cells susceptible to infection with human prions, such as those involved in creutzfeldt-jakob disease. in variant creutzfeldt-jakob disease, prion replication is thought to occur first in the lymphoreticular system and then spread into the brain. we have, therefore, examined the susceptibility of a ... | 2014 | 24183781 |
| social cognitive predictors of intention to test for variant creutzfeldt-jakob disease in those affected by haemophilia and other clotting disorders. | individuals with bleeding disorders are at increased risk of variant creutzfeldt-jakob disease. this study explored social cognitive predictors of screening intentions. ninety men and women with bleeding disorders, recruited through the haemophilia society, completed an extended theory of planned behaviour questionnaire to predict intention to screen for variant creutzfeldt-jakob disease. extended theory of planned behaviour variables accounted for 57 per cent of the variance in intention. self- ... | 2014 | 23520351 |
| assessment of prion reduction filters in decreasing infectivity of ultracentrifuged 263k scrapie-infected brain homogenates in "spiked" human blood and red blood cells. | the safety of red blood cells (rbcs) is of concern because of the occurrence of four transfusion-transmitted variant creutzfeldt-jakob disease (vcjd) cases in the united kingdom. the absence of validated screening tests requires the use of procedures to remove prions from blood to minimize the risk of transmission. these procedures must be validated using infectious prions in a form that is as close as possible to one in blood. | 2014 | 23915063 |
| evidence for zoonotic potential of ovine scrapie prions. | although bovine spongiform encephalopathy (bse) is the cause of variant creutzfeldt jakob disease (vcjd) in humans, the zoonotic potential of scrapie prions remains unknown. mice genetically engineered to overexpress the human prion protein (tghu) have emerged as highly relevant models for gauging the capacity of prions to transmit to humans. these models can propagate human prions without any apparent transmission barrier and have been used used to confirm the zoonotic ability of bse. here we s ... | 2014 | 25510416 |
| first demonstration of transmissible spongiform encephalopathy-associated prion protein (prptse) in extracellular vesicles from plasma of mice infected with mouse-adapted variant creutzfeldt-jakob disease by in vitro amplification. | the development of variant creutzfeldt-jakob disease (vcjd) in three recipients of non-leukoreduced red blood cells from asymptomatic donors who subsequently developed the disease has confirmed existing concerns about the possible spread of transmissible spongiform encephalopathies (tses) via blood products. in addition, the presence of disease-associated misfolded prion protein (prp(tse)), generally associated with infectivity, has been demonstrated in the blood of vcjd patients. however, its o ... | 2014 | 25157106 |
| development of dose-response models of creutzfeldt-jakob disease infection in nonhuman primates for assessing the risk of transfusion-transmitted variant creutzfeldt-jakob disease. | estimates for the risk of transmitting variant creutzfeldt-jakob disease (vcjd) via blood transfusion have relied largely on data from rodent experiments, but the relationship between dose (amount of infected blood) and response (vcjd infection) has never been well quantified. the goal of this study was to develop a dose-response model based on nonhuman primate data to better estimate the likelihood of transfusion-transmitted vcjd (ttvcjd) in humans. our model used dose-response data from nonhum ... | 2014 | 25231313 |
| esophageal capsule endoscopy is a useful tool in patients with hemophilia. | esophageal capsule endoscopy (ece) is considered to be an alternative to conventional esophagogastroduodenoscopy (egd); however, its indications continue to expand. this report presents results from the use of ece in patients with hemophilia at a tertiary referral academic center over a 7-year period. a total of 16 patients with hemophilia, who were all at risk of new-variant creutzfeldt-jakob disease due to previous treatment with uk plasma-derived pooled blood products, underwent a total of 28 ... | 2014 | 25208029 |
| molecular modeling of prion transmission to humans. | using different prion strains, such as the variant creutzfeldt-jakob disease agent and the atypical bovine spongiform encephalopathy agents, and using transgenic mice expressing human or bovine prion protein, we assessed the reliability of protein misfolding cyclic amplification (pmca) to model interspecies and genetic barriers to prion transmission. we compared our pmca results with in vivo transmission data characterized by attack rates, i.e., the percentage of inoculated mice that developed t ... | 2014 | 25279820 |
| variant cjd. 18 years of research and surveillance. | it is now 18 years since the first identification of a case of vcjd in the uk. since that time, there has been much speculation over how vcjd might impact human health. to date there have been 177 case reports in the uk and a further 51 cases worldwide in 11 different countries. since establishing that bse and vcjd are of the same strain of agent, we have also shown that there is broad similarity between uk and non-uk vcjd cases on first passage to mice. transgenic mouse studies have indicated t ... | 2014 | 25495404 |
| complex proteinopathy with accumulations of prion protein, hyperphosphorylated tau, α-synuclein and ubiquitin in experimental bovine spongiform encephalopathy of monkeys. | proteins aggregate in several slowly progressive neurodegenerative diseases called 'proteinopathies'. studies with cell cultures and transgenic mice overexpressing mutated proteins suggested that aggregates of one protein induced misfolding and aggregation of other proteins as well - a possible common mechanism for some neurodegenerative diseases. however, most proteinopathies are 'sporadic', without gene mutation or overexpression. thus, proteinopathies in wt animals genetically close to humans ... | 2014 | 24769839 |
| estimation of the exposure of the uk population to the bovine spongiform encephalopathy agent through dietary intake during the period 1980 to 1996. | although the incidence of variant creutzfeldt-jakob disease (vcjd) has declined to 1 since 2012 in the uk, uncertainty remains regarding possible future cases and the size of the subclinical population that may cause secondary transmission of the disease through blood transfusion. estimating the number of individuals who were exposed to the bovine spongiform encephalopathy (bse) infectious agent and may be susceptible to vcjd will help to clarify related public health concerns and plan strategie ... | 2014 | 24736322 |
| infections and exposures: reported incidents associated with unsuccessful decontamination of reusable surgical instruments. | reusable surgical instruments provide a potential route for the transmission of pathogenic agents between patients in healthcare facilities. as such, the decontamination process between uses is a vital component in the prevention of healthcare-associated infections. this article reviews reported outbreaks and incidents associated with inappropriate, inadequate, or unsuccessful decontamination of surgical instruments, indicating potential pitfalls of decontamination practices worldwide. to the au ... | 2014 | 25287950 |