| variant creutzfeldt-jakob disease in a transfusion recipient: coincidence or cause? | to date there have been four instances of infection transmitted through blood transfusions derived from individuals who later developed variant creutzfeldt-jakob disease (vcjd). the identification of further transmission of vcjd through this route would have important implications for risk assessment and public health. | 2010 | 20230536 |
| experimental transmission of bovine spongiform encephalopathy (bse) to cynomolgus macaques, a non-human primate. | bovine spongiform encephalopathy (bse) was transmitted to three macaques by intracerebral inoculation of a brain homogenate from affected cattle detected in japan. all monkeys developed abnormal behavioral signs, such as intermittent anorexia and hyperekplexia, around 24 months after inoculation. neuronal symptoms, such as tremor, myoclonic jerking, and paralysis, appeared 27-44 months after inoculation. these symptoms worsened and total paralysis ensued within a year after onset. the disease du ... | 2011 | 21266755 |
| [variant creutzfeld-jakob disease (vcjd) : epidemiology and prevention from human to human secondary transmission]. | in the wake of the bovine spongiform encephalopathy (bse) epidemic, variant creutzfeldt-jakob disease (vcjd) has emerged as a previously unknown prion disease of humans. the initial occurrence of vcjd was observed in 1995/1996, and, so far, a total of 219 vcjd cases have been reported worldwide from seven european and four non-european countries. of these, 172 cases were observed in the united kingdom. the exact prevalence of sub- or pre-clinical vcjd infections is unclear. despite effective mea ... | 2010 | 20449549 |
| detection of prion infection in variant creutzfeldt-jakob disease: a blood-based assay. | variant creutzfeldt-jakob disease (vcjd) is a fatal neurodegenerative disorder originating from exposure to bovine-spongiform-encephalopathy-like prions. prion infections are associated with long and clinically silent incubations. the number of asymptomatic individuals with vcjd prion infection is unknown, posing risk to others via blood transfusion, blood products, organ or tissue grafts, and contaminated medical instruments. we aimed to establish the sensitivity and specificity of a blood-base ... | 2011 | 21295339 |
| approaches to minimize infection risk in blood banking and transfusion practice. | the use of blood donor history and state-of-the-art fda-licensed serological and nucleic acid testing (nat) assays have greatly reduced the "infectious window" for several transfusion-transmitted pathogens. currently transmission of human immunodeficiency virus (hiv), human t-cell lymphotropic virus (htlv), hepatitis viruses and west nile virus are rare events. the seroprevalence of cytomegalovirus in the donor population is high and cytomegalovirus infection can cause significant complications ... | 2011 | 21303341 |
| [protection from bse : efforts, measures, success, and costs]. | by the mid 1980s, bovine spongiform encephalopathy (bse) emerged in the united kingdom (uk) and reached its peak in the early 1990s with up to 37,000 cases. in the year 2000, bse was diagnosed for the first time for a cow born in germany. since then, 413 cases of bse have been detected. about 10 years after the first bse cases were detected, variant creutzfeldt-jakob disease (vcjd), a new variant of creutzfeldt-jakob disease (cjd), was described in the uk. legal measures for protection from bse ... | 2010 | 20449555 |
| validation of diagnostic criteria for variant creutzfeldt-jakob disease. | variant creutzfeldt-jakob disease (vcjd), a novel form of human prion disease, was recognized in 1996. the disease affected a younger cohort than sporadic cjd, and the early clinical course was dominated by psychiatric and sensory symptoms. in an attempt to aid diagnosis and establish standardization between surveillance networks, diagnostic criteria were established. these were devised from the features of a small number of cases and modified in 2000 as the clinical phenotype was established. s ... | 2010 | 20517937 |
| the application of in vitro cell-free conversion systems to human prion diseases. | a key event in the pathogenesis of prion diseases is the conversion of the normal cellular isoform of the prion protein into the disease-associated isoform, but the mechanisms operating in this critical event are not yet fully understood. a number of novel approaches have recently been developed to study factors influencing this process. one of these, the protein misfolding cyclical amplification (pmca) technique, has been used to explore defined factors influencing the conversion of cellular pr ... | 2011 | 20535485 |
| detection of blood-transmissible agents: can screening be miniaturized? | transfusion safety relating to blood-transmissible agents is a major public health concern, particularly when faced with the continuing emergence of new infectious agents. these include new viruses appearing alongside other known reemerging viruses (west nile virus, chikungunya) as well as new strains of bacteria and parasites (plasmodium falciparum, trypanosoma cruzi) and finally pathologic prion protein (variant creutzfeldt-jakob disease). genomic mutations of known viruses (hepatitis b virus, ... | 2010 | 20546202 |
| a highly sensitive immunoassay for the detection of prion-infected material in whole human blood without the use of proteinase k. | the causal association of variant creutzfeldt-jakob disease (vcjd) with bovine spongiform encephalopathy has raised significant concerns for public health. assays for vcjd infection are vital for the application of therapeutics, for the screening of organ donations, and to maintain a safe blood supply. currently the best diagnostic tools for vcjd depend upon the detection of disease-associated prion protein (prp(sc) ), which is distinguished from normal background prp (prp(c) ) by proteinase k ( ... | 2010 | 20561299 |
| photo essay. mri and positron emission tomography findings in heidenhain variant creutzfeldt-jakob disease. | the typical presentation of heidenhain variant creutzfeldt-jakob disease (cjd) is a rapidly progressive visual loss in the setting of a relatively normal ophthalmologic examination. at presentation, patients with this uniformly fatal illness frequently demonstrate only minor cortical abnormalities on mri. here, we document the clinical presentation and imaging results of a patient with heidenhain variant cjd in whom abnormalities on positron emission tomographic imaging were more evident than ch ... | 2010 | 20581692 |
| prion interaction with the 37-kda/67-kda laminin receptor on enterocytes as a cellular model for intestinal uptake of prions. | enterocytes, a major cell population of the intestinal epithelium, represent one possible barrier to the entry of prions after oral exposure. we established a cell culture system employing enterocytes from different species to study alimentary prion interaction with the 37-kda/67-kda laminin receptor lrp/lr. human, bovine, porcine, ovine, and cervid enterocytes were cocultured with brain homogenates from cervid, sheep, and cattle suffering from chronic wasting disease (cwd), scrapie, and bovine ... | 2010 | 20603132 |
| molecular dynamics studies on the structural stability of wild-type dog prion protein. | prion diseases such as creutzfeldt-jakob disease, variant creutzfeldt-jakob diseases, gerstmann-sträussler-scheinker syndrome, fatal familial insomnia, kuru in humans, scrapie in sheep, bovine spongiform encephalopathy (or 'mad-cow' disease) and chronic wasting disease in cattle are invariably fatal and highly infectious neurodegenerative diseases affecting humans and animals. however, by now there have not been some effective therapeutic approaches to treat all these prion diseases. in 2008, ca ... | 2011 | 21469747 |
| heterozygosity at polymorphic codon 219 in variant creutzfeldt-jakob disease. | genetic variants of the prion protein gene (prnp) strongly determine susceptibility to prion diseases. all tested patients with definite variant creutzfeldt-jakob disease (vcjd) are homozygous for methionine at a common polymorphism at codon 129. a further genetic polymorphism at codon 219, a common variant in several asian populations, is considered protective against sporadic cjd. | 2010 | 20697057 |
| visual symptoms in the heidenhain variant of creutzfeldt-jakob disease. | the distinguishing feature in heidenhain variant creutzfeldt-jakob disease (hvcjd) is the presence of visual symptoms preceding the appearance of other clinical manifestations. the purpose of this report is to describe the broad range of visual symptomatology in a patient with hvcjd. | 2009 | 20704012 |
| increasing hybridoma viability and antibody repertoire after the cell fusion by the use of human plasma as an alternative supplement. | prion diseases such as bovine spongiform encephalopathy (bse) and new variant creutzfeldt-jakob disease (nvcjd) have caused a major safety concern in cell cultures using fetal calf serum (fcs). in this study, we found that screened and tested human plasma (hp) obtained from blood centers may be an ideal alternate nutrient substitute to fcs for culturing hybridoma. in addition to the inherent safety, a ten-fold increase in the fusion efficiency has been observed if the hp was used as the nutrient ... | 2010 | 20723546 |
| chronic wasting disease. | chronic wasting disease (cwd) is a prion disease of free-ranging and farmed ungulates (deer, elk, and moose) in north america and south korea. first described by the late e.s. williams and colleagues in northern colorado and southern wyoming in the 1970s, cwd has increased tremendously both in numerical and geographical distribution, reaching prevalence rates as high as 50% in free-ranging and >90% in captive deer herds in certain areas of usa and canada. cwd is certainly the most contagious pri ... | 2011 | 21598099 |
| prospective 10-year surveillance of human prion diseases in japan. | we analysed the epidemiological data and clinical features of patients with prion diseases that had been registered by the creutzfeldt-jakob disease surveillance committee, japan, over the past 10 years, since 1999. we obtained information on 1685 japanese patients suspected as having prion diseases and judged that 1222 patients had prion diseases, consisting of definite (n=180, 14.7%) and probable (n=1029, 84.2%) cases, except for dura mater graft-associated creutzfeldt-jakob disease which also ... | 2010 | 20855418 |
| review: contribution of transgenic models to understanding human prion disease. | transgenic mice expressing human prion protein in the absence of endogenous mouse prion protein faithfully replicate human prions. these models reproduce all of the key features of human disease, including long clinically silent incubation periods prior to fatal neurodegeneration with neuropathological phenotypes that mirror human prion strain diversity. critical contributions to our understanding of human prion disease pathogenesis and aetiology have only been possible through the use of transg ... | 2010 | 20880036 |
| large-scale immunohistochemical examination for lymphoreticular prion protein in tonsil specimens collected in britain. | there have been 173 cases of variant creutzfeldt-jakob disease (vcjd) in the uk, as of 5 july 2010, as a result of the bovine spongiform encephalopathy epidemic. the number of individuals subclinically infected with vcjd, and thus the eventual number of cases, remains, however, uncertain. in an attempt to address this problem, 63,007 tonsil tissue specimens were previously tested by enzyme immunoassay (eia) for the presence of disease-related prion protein (prp(res)) and found to be negative. to ... | 2010 | 20922767 |
| dispersion of prion protein deposits around blood vessels in variant creutzfeldt-jakob disease. | in variant creutzfeldt-jakob disease (vcjd), a disease linked to bovine spongiform encephalopathy (bse), florid-type prion protein (prp(sc)) deposits are aggregated around the larger diameter (> 10 µm) cerebral microvessels. clustering of prp(sc) deposits around blood vessels may result from blood-borne prions or be a consequence of the cerebral vasculature influencing the development of the florid deposits. to clarify the factors involved, the dispersion of the florid prp(sc) deposits was studi ... | 2010 | 20924999 |
| conserved properties of human and bovine prion strains on transmission to guinea pigs. | the first transmissions of human prion diseases to rodents used guinea pigs (gps, cavia porcellus). later, transgenic mice expressing human or chimeric human/mouse prp replaced gps, but the small size of the mouse limits some investigations. to investigate the fidelity of strain-specific prion transmission to gps, we inoculated 'type 1' and 'type 2' prion strains into gps, and we measured the incubation times and determined the strain-specified size of the unglycosylated, protease-resistant (r) ... | 2011 | 21727894 |
| [anaesthetic management for caesarean delivery and creutzfeldt-jakob disease]. | variant creutzfeldt-jakob disease (vcjd) is the only form of prion diseases linked to bovine spongiform encephalopathy (bse). the surgical and anaesthetic management in patients having creutzfeldt-jakob disease is rare. maternofoetal and human transmission of creutzfeldt-jakob disease is still unknown. the principles for managing these new risks are not described in obstetric recommendations. we report the case of an 18-year-old woman, who developed the variant creutzfeldt-jakob disease during h ... | 2010 | 20934303 |
| the prion diseases. | the prion diseases are a family of rare neurodegenerative disorders that result from the accumulation of a misfolded isoform of the prion protein (prp), a normal constituent of the neuronal membrane. five subtypes constitute the known human prion diseases; kuru, creutzfeldt-jakob disease (cjd), gerstmann-sträussler-scheinker syndrome (gss), fatal insomnia (fi), and variant cjd (vcjd). these subtypes are distinguished, in part, by their clinical phenotype, but primarily by their associated brain ... | 2010 | 20938044 |
| differentiation of ruminant transmissible spongiform encephalopathy isolate types, including bovine spongiform encephalopathy and ch1641 scrapie. | with increased awareness of the diversity of transmissible spongiform encephalopathy (tse) strains in the ruminant population, comes an appreciation of the need for improved methods of differential diagnosis. exposure to bovine spongiform encephalopathy (bse) has been associated with the human tse, variant creutzfeldt-jakob disease, emphasizing the necessity in distinguishing low-risk tse types from bse. tse type discrimination in ruminants such as cattle, sheep, goats and deer, requires the app ... | 2010 | 20943889 |
| correlation of polydispersed prion protein and characteristic pathology in the thalamus in variant creutzfeldt-jakob disease: implication of small oligomeric species. | the vacuolation, neuronal loss and gliosis that characterize human prion disease pathology are accompanied by the accumulation of an aggregated, insoluble and protease-resistant form (termed prp(sc)) of the host-encoded normal cellular prion protein (prp(c)). in variant creutzfeldt-jakob disease the frontal cortex and cerebellum exhibit intense vacuolation and the accumulation of prp(sc) in the form of amyloid plaques and plaque-like structures. in contrast the posterior thalamus is characterize ... | 2010 | 21029243 |
| underestimation of the expression of cellular prion protein on human red blood cells. | recent transmissions of variant creutzfeldt-jakob disease by blood transfusion emphasize the need for the development of prion screening tests. the detection of prions in blood is complicated by the presence of poorly characterized cellular prion protein (prp(c) ) in both plasma and blood cells. according to published studies, most of prp(c) in blood cells resides in platelets (plts) and white blood cells. | 2010 | 21058954 |
| translocation of cellular prion protein to non-lipid rafts protects human prion-mediated neuronal damage. | prions are the causative agents of transmissible spongiform encephalopathies, such as variant creutzfeldt-jakob disease in humans. cellular prion proteins (prpc) connect with cholesterol- and glycosphingolipid-rich lipid rafts through association of their glycosyl-phosphatidylinositol (gpi) anchor with saturated raft lipids and interaction of their n-terminal regions. our previous study showed that cellular cholesterol enrichment preven ... | 2011 | 22179431 |
| a standardized comparison of commercially available prion decontamination reagents using the standard steel-binding assay. | prions are comprised principally of aggregates of a misfolded host protein and cause fatal transmissible neurodegenerative disorders of mammals, such as variant creutzfeldt-jakob disease in humans and bovine spongiform encephalopathy in cattle. prions pose significant public health concerns through contamination of blood products and surgical instruments, and can resist conventional hospital sterilization methods. prion infectivity binds avidly to surgical steel and can efficiently transfer infe ... | 2010 | 21084494 |
| preclinical deposition of pathological prion protein in muscle of experimentally infected primates. | prion diseases are transmissible fatal neurodegenerative disorders affecting humans and animals. a central step in disease progression is the accumulation of a misfolded form (prp(sc)) of the host encoded prion protein (prp(c)) in neuronal and non-neuronal tissues. the involvement of peripheral tissues in preclinical states increases the risk of accidental transmission. on the other hand, detection of prp(sc) in non-neuronal easy-accessible compartments such as muscle may offer a novel diagnosti ... | 2010 | 21085647 |
| laminar distribution of the pathological changes in sporadic and variant creutzfeldt-jakob disease. | the laminar distributions of the pathological changes in the cerebral cortex were compared in the prion diseases sporadic creutzfeldt-jakob disease (scjd) and variant cjd (vcjd). first, in some cortical regions, the vacuolation ("spongiform change") was more generally distributed across the cortex in scjd. second, there was greater neuronal loss in the upper cortex in vcjd and in the lower cortex in scjd. third, the "diffuse" and "florid" prion protein (prp(sc)) deposits were more frequently dis ... | 2010 | 21209711 |
| experimental interspecies transmission studies of the transmissible spongiform encephalopathies to cattle: comparison to bovine spongiform encephalopathy in cattle. | prion diseases or transmissible spongiform encephalopathies (tses) of animals include scrapie of sheep and goats; transmissible mink encephalopathy (tme); chronic wasting disease (cwd) of deer, elk and moose; and bovine spongiform encephalopathy (bse) of cattle. the emergence of bse and its spread to human beings in the form of variant creutzfeldt-jakob disease (vcjd) resulted in interest in susceptibility of cattle to cwd, tme and scrapie. experimental cross-species transmission of tse agents p ... | 2011 | 21908269 |
| leu138 in bovine prion peptide fibrils is involved in seeding discrimination related to codon 129 m/v polymorphism in the prion peptide seeding experiment. | the risk of acquiring variant creutzfeldt-jakob disease is closely related to polymorphism at codon 129 of the human prion gene, because almost all variant creutzfeldt-jakob disease patients are met/met homozygotes. although animal transmission experiments corroborated this seeding discrimination, the origin of the differential seeding efficiency of the bovine prion seed for human codon 129 polymorphism remained elusive. here, we used a short prion protein (prp) peptide as a model system to test ... | 2011 | 21920025 |
| rna integrity in post mortem human variant creutzfeldt-jakob disease (vcjd) and control brain tissue. | k. r. sherwood, m. w. head, r. walker, c. smith, j. w. ironside and j. k. fazakerley (2011) neuropathology and applied neurobiology37, 633-642 rna integrity in post mortem human variant creutzfeldt-jakob disease (vcjd) and control brain tissue aims: to determine premortem and post mortem factors affecting quality and yield of rna isolated from the unique archived brain material in the uk national creutzfeldt-jakob disease surveillance unit brain and tissue bank and to compare this to control bra ... | 2011 | 21251044 |
| atypical l-type bovine spongiform encephalopathy (l-bse) transmission to cynomolgus macaques, a non-human primate. | a low molecular weight type of atypical bovine spongiform encephalopathy (l-bse) was transmitted to two cynomolgus macaques by intracerebral inoculation of a brain homogenate of cattle with atypical bse detected in japan. they developed neurological signs and symptoms at 19 or 20 months post-inoculation and were euthanized 6 months after the onset of total paralysis. both the incubation period and duration of the disease were shorter than those for experimental transmission of classical bse (c-b ... | 2011 | 21266763 |
| the risk of variant creutzfeldt-jakob disease among uk patients with bleeding disorders, known to have received potentially contaminated plasma products. | summary. the risk of variant creutzfeldt-jakob disease (vcjd) from potentially infected plasma products remains unquantified. this risk has been assessed for 787 uk patients with an inherited bleeding disorder prospectively followed-up for 10-20 years through the uk haemophilia centre doctors' organisation (ukhcdo) surveillance study. these patients had been treated with any of 25 'implicated' clotting factor batches from 1987 to 1999, which included in their manufacture, plasma from eight dono ... | 2011 | 21342369 |
| universal white blood cell reduction in europe: has transmission of variant creutzfeldt-jakob disease been prevented? | universal white blood cell (wbc) reduction was introduced in europe to prevent transmission of variant creutzfeldt-jakob disease (vcjd) by transfusion. findings from rodent models indicate that wbc reduction should not prevent vcjd transmission because the residual plasma infectivity suffices to infect transfusion recipients even under optimistic infectivity assumptions. although infectivity in human blood may not partition in the manner in which it is distributed in rodents, prion-reduction fil ... | 2011 | 21345641 |
| biological effects and use of prpsc- and prp-specific antibodies generated by immunization with purified full-length native mouse prions. | the prion agent is the infectious particle causing spongiform encephalopathies in animals and humans and is thought to consist of an altered conformation (prp(sc)) of the normal and ubiquitous prion protein prp(c). the interaction of the prion agent with the immune system, particularly the humoral immune response, has remained unresolved. here we investigated the immunogenicity of full-length native and infectious prions, as well as the specific biological effects of the resulting monoclonal ant ... | 2011 | 21345946 |
| Comparison of candidate vCJD in vitro diagnostic assays using identical sample sets. | Background and Objectives With four transfusion related transmissions of variant Creutzfeldt-Jakob Disease (vCJD), three of which developed clinical disease and the other died of other causes but was positive for markers of infection, there is an increased urgency to identify and implement a test for blood donor screening. With limited amounts of blood samples from vCJD cases available test evaluation is challenging. Alternative approaches are therefore needed. Control and vCJD tissues homogena ... | 2011 | 22126309 |
| conformation as therapeutic target in the prionoses and other neurodegenerative conditions. | neurodegenerative conditions are increasing in prevalence as the average human life expectancy rises. alzheimer's disease (ad) is the fourth commonest cause of death in the united states; the recent outbreak of new variant creutzfeldt-jakob disease (nvcjd) has raised the specter of a large population being at risk to develop this prionosis. the pathogenesis of many neurodegenerative diseases is now recognized to be associated with abnormalities of protein conformation. a common theme in these di ... | 2001 | 21374507 |
| notification and support for people exposed to the risk of creutzfeldt-jakob disease (cjd) (or other prion diseases) through medical treatment (iatrogenically). | creutzfeldt-jakob disease (cjd) and variant cjd (vcjd) are rare and always-fatal diseases transmissible via certain medical procedures. if a person is exposed to the disease risk through medical treatment, they may need to be notified of this to prevent them passing the risk to others in healthcare settings and to enable additional infection control measures to be put in place for certain procedures. as cjd is incurable, and unable to be screened for or effectively treated, communicating this ri ... | 2011 | 21412905 |
| The structural stability of wild-type horse prion protein. | Prion diseases (e.g. Creutzfeldt-Jakob disease (CJD), variant CJD (vCJD), Gerstmann-Straussler-Scheinker syndrome (GSS), Fatal Familial Insomnia (FFI) and Kuru in humans, scrapie in sheep, bovine spongiform encephalopathy (BSE or 'mad-cow' disease) and chronic wasting disease (CWD) in cattles) are invariably fatal and highly infectious neurodegenerative diseases affecting humans and animals. However, by now there have not been some effective therapeutic approaches or medications to treat all the ... | 2011 | 21875155 |
| bse safety standards: an evaluation of public health policies of japan, europe, and usa. | since the advent of bovine spongiform encephalopathy (bse) in the united kingdom in 1986, new bse cases have recently become rare. however, in japan and the united states, positive cases have started to be seen recently. the rise in bse cases paved the way for the human form of this disease, the variant creutzfeldt-jakob disease (vcjd). the observed trends in the uk may be attributed to effective implementation of public health policies coupled with increased vigilance through advancement in sci ... | 2005 | 21432135 |
| detection of prion protein in urine-derived injectable fertility products by a targeted proteomic approach. | iatrogenic transmission of human prion disease can occur through medical or surgical procedures, including injection of hormones such as gonadotropins extracted from cadaver pituitaries. annually, more than 300,000 women in the united states and canada are prescribed urine-derived gonadotropins for infertility. although menopausal urine donors are screened for symptomatic neurological disease, incubation of creutzfeldt-jakob disease (cjd) is impossible to exclude by non-invasive testing. risk of ... | 2011 | 21448279 |
| The molecular epidemiology of variant CJD. | The emergence of the novel prion diseases bovine spongiform encephalopathy (BSE) and, subsequently, variant Creutzfeldt-Jakob disease (vCJD) in epidemic forms has attracted much scientific attention. The oral transmission of these disorders, the causative relationship of vCJD to BSE and the resistance of the transmissible agents in both disorders to conventional forms of decontamination has caused great public health concern. The size of the still emerging vCJD epidemic is thankfully much lower ... | 2011 | 21915360 |
| prion disease blood test using immunoprecipitation and improved quaking-induced conversion. | abstract a key challenge in managing transmissible spongiform encephalopathies (tses) or prion diseases in medicine, agriculture, and wildlife biology is the development of practical tests for prions that are at or below infectious levels. of particular interest are tests capable of detecting prions in blood components such as plasma, but blood typically has extremely low prion concentrations and contains inhibitors of the most sensitive prion tests. one of the latter tests is quaking-induced co ... | 2011 | 21558432 |
| 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 |
| heparin enhances the cell-protein misfolding cyclic amplification efficiency of variant creutzfeldt-jakob disease. | highly sensitive in vitro screening tests are required to prevent the iatrogenic spread of variant creutzfeldt-jakob disease (vcjd). protein misfolding cyclic amplification (pmca) is a candidate for such a test, but the sensitivity of this method is insufficient. polyanions were reported to enhance pmca efficiency, but their effects on vcjd are unclear. we developed a cell-pmca of vcjd, wherein cell lysate containing exogenously expressed human prp was used as substrates, to investigate the effe ... | 2011 | 21565253 |
| 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 |
| atypical prion diseases in humans and animals. | although prion diseases, such as creutzfeldt-jakob disease (cjd) in humans and scrapie in sheep, have long been recognized, our understanding of their epidemiology and pathogenesis is still in its early stages. progress is hampered by the lengthy incubation periods and the lack of effective ways of monitoring and characterizing these agents. protease-resistant conformers of the prion protein (prp), known as the "scrapie form" (prp(sc)), are used as disease markers, and for taxonomic purposes, in ... | 2011 | 21598097 |
| 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 |
| rapid screening and confirmatory methods for biochemical diagnosis of human prion disease. | transmissible spongiform encephalopathies (tses) are characterised by accumulation of an abnormal isoform of prion protein (prp(sc)), mainly in the brain but also in various peripheral tissues. home-made assays consisting of non-standardised protocols are used currently for laboratory diagnosis of human tse. the purpose of the present study was to test the ability of two commercial assays, tesee™ cjd elisa and tesee™ western blot, to detect prpsc in cerebral and lymphoid tissues of tse patients. ... | 2011 | 21619894 |
| structural and functional neuroimaging in human prion diseases. | introduction: prion diseases are neurodegenerative disorders resulting from the accumulation of a misfolded isoform of the cellular prion protein (prpc). they can occur as acquired, sporadic or hereditary forms. although prion diseases show a wide range of phenotypic variations, pathological features and clinical evolution, they are all characterised by a common unfavourable course and a fatal outcome. review summary: some variants, such as kuru, have practically disappeared, while others, for e ... | 2011 | 21621879 |
| estimation of variant creutzfeldt-jakob disease infectivity titers in human blood. | background: blood of individuals with variant creutzfeldt-jakob disease (vcjd) is infectious but the titer is unknown. current estimates of possible vcjd infectivity titers in blood have largely relied on an assumption that the titers of vcjd agent in human blood are likely to be similar to those in blood of rodents infected with model transmissible spongiform encephalopathy agents, assayed by intracerebral inoculations of rodents of the same species. study design and methods: we analyzed publis ... | 2011 | 21645006 |
| biochemical and strain properties of cjd prions: complexity versus simplicity. | prions, the agents responsible for transmissible spongiform encephalopathies, are infectious proteins consisting primarily of scrapie prion protein (prp(sc) ), a misfolded, beta-sheet enriched and aggregated form of the host-encoded cellular prion protein (prp(c) ). their propagation is based on an autocatalytic prp conversion process. despite the lack of a nucleic acid genome, different prion strains have been isolated from animal diseases. increasing evidence supports the view that strain-spec ... | 2011 | 21790605 |
| the risk to human islet cell transplant recipients of acquiring variant creutzfeldt-jakob disease: a provisional quantitative risk assessment. | | 2011 | 21691192 |
| Candidate cell substrates, vaccine production, and transmissible spongiform encephalopathies. | Transmissible spongiform encephalopathy (TSE) agents have contaminated human tissue-derived medical products, human blood components, and animal vaccines. The objective of this study was to determine the potential susceptibility to infection of 5 cell lines used or proposed for manufacture of biological products, as well as other lines. Cell lines were exposed to the infectious agents of sporadic and variant Creutzfeldt-Jakob disease and bovine spongiform encephalopathy (BSE). Exposed cultures w ... | 2011 | 22172513 |
| Limited efficacy of steam sterilization to inactivate vCJD infectivity. | The transmission of bovine spongiform encephalopathy (BSE) to humans as variant Creutzfeldt-Jakob Disease (vCJD) raised concerns about potential secondary transmissions due to the resistance of the agents causing transmissible spongiform encephalopathies (TSEs), sometimes known as prions, to commonly used methods of sterilization, notably steam sterilization (or autoclaving). It has been suggested that surgical instruments and other medical devices might retain sufficient infected tissue debris ... | 2012 | 22099953 |
| 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 |
| inhibitors of gastric acid secretion increase the risk of prion infection in mice. | gastric juice is a unique combination of hydrochloric acid and the proteolytic enzyme pepsin. its main function is to inactivate ingested microorganisms. prions cause fatal transmissible degenerative encephalopathies in animals and man. these diseases have attracted attention due to the proposed link between bovine spongiform encephalopathy in cattle and the occurrence of a new variant creutzfeldt-jakob disease in humans where the most probable route of transmission is via contaminated food. the ... | 2011 | 21936725 |
| 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 |
| squirrel monkeys (saimiri sciureus) infected with the agent of bovine spongiform encephalopathy develop tau pathology. | squirrel monkeys (saimiri sciureus) were infected experimentally with the agent of classical bovine spongiform encephalopathy (bse). two to four years later, six of the monkeys developed alterations in interactive behaviour and cognition and other neurological signs typical of transmissible spongiform encephalopathy (tse). at necropsy examination, the brains from all of the monkeys showed pathological changes similar to those described in variant creutzfeldt-jakob disease (vcjd) of man, except t ... | 2011 | 22018806 |
| in vitro detection of prionemia in tse-infected cervids and hamsters. | blood-borne transmission of infectious prions during the symptomatic and asymptomatic stages of disease occurs for both human and animal transmissible spongiform encephalopathies (tses). the geographical distribution of the cervid tse, chronic wasting disease (cwd), continues to spread across north america and the prospective number of individuals harboring an asymptomatic infection of human variant creutzfeldt-jakob disease (vcjd) in the united kingdom has been projected to be ~1 in 3000 reside ... | 2013 | 24224043 |
| molecular cloning and sequence analysis of prion protein gene in xiji donkey in china. | prion diseases are a group of human and animal neurodegenerative disorders caused by the deposition of an abnormal isoform prion protein (prp(sc)) encoded by a single copy prion protein gene (prnp). prion disease has been reported in many herbivores but not in equus and the species barrier might be playing a role in resistance of these species to the disease. therefore, analysis of genotype of prion protein (prp) in these species may help understand the transmission of the disease. xiji donkey i ... | 2013 | 23954254 |
| chronic wasting disease and atypical forms of bovine spongiform encephalopathy and scrapie are not transmissible to mice expressing wild-type levels of human prion protein. | the association between bovine spongiform encephalopathy (bse) and variant creutzfeldt-jakob disease (vcjd) has demonstrated that cattle transmissible spongiform encephalopathies (tses) can pose a risk to human health and raises the possibility that other ruminant tses may be transmissible to humans. in recent years, several novel tses in sheep, cattle and deer have been described and the risk posed to humans by these agents is currently unknown. in this study, we inoculated two forms of atypica ... | 2012 | 22495232 |
| comparison of nanofiltration efficacy in reducing infectivity of centrifuged versus ultracentrifuged 263k scrapie-infected brain homogenates in "spiked" albumin solutions. | background: the safety of plasma-derived products is of concern for possible transmission of variant creutzfeldt-jakob disease. the absence of validated screening tests requires the use of procedures to remove or inactivate prions during the manufacture of plasma-derived products to minimize the risk of transmission. these procedures need proper validation studies based on spiking human plasma or intermediate fractions of plasma fractionation with prions in a form as close as possible to that pr ... | 2011 | 22082124 |
| genome wide association studies and prion disease. | over the last decade remarkable advances in genotyping and sequencing technology have resulted in hundreds of novel gene associations with disease. these have typically involved high frequency alleles in common diseases and with the advent of next generation sequencing, disease causing recessive mutations in rare inherited syndromes. here we discuss the impact of these advances and other gene discovery methods in the prion diseases. several quantitative trait loci in mouse have been mapped and t ... | 2011 | 21844666 |
| isolation of prion with bse properties from farmed goat. | transmissible spongiform encephalopathies are fatal neurodegenerative diseases that include variant creutzfeldt-jakob disease in humans, scrapie in small ruminants, and bovine spongiform encephalopathy (bse) in cattle. scrapie is not considered a public health risk, but bse has been linked to variant creutzfeldt-jakob disease. small ruminants are susceptible to bse, and in 2005 bse was identified in a farmed goat in france. we confirm another bse case in a goat in which scrapie was originally di ... | 2011 | 22172149 |
| Genome-wide association study in multiple human prion diseases suggests genetic risk factors additional to PRNP. | Prion diseases are fatal neurodegenerative diseases of humans and animals caused by the misfolding and aggregation of prion protein (PrP). Mammalian prion diseases are under strong genetic control but few risk factors are known aside from the PrP gene locus (PRNP). No genome-wide association study (GWAS) has been done aside from a small sample of variant Creutzfeldt-Jakob disease. We conducted GWAS of sporadic CJD, variant CJD, iatrogenic CJD, inherited prion disease, kuru and resistance to kuru ... | 2011 | 22210626 |
| demographics of successful, unsuccessful and deferral visits at six blood centers over a 4-year period. | background: descriptions of donor demographics are of value in formulating recruitment and retention strategies. the demographics of successful (sv), unsuccessful (uv; meaning a nonuseable unit), and deferred (dv) donor visits over a 4-year period were investigated using retrovirus epidemiology donor study (reds)-ii databases. study design and methods: fourteen deferral categories were created that included low hematocrit (hct) or hemoglobin (hb), feeling unwell, malaria travel, malaria other, c ... | 2011 | 21950621 |
| all clinically-relevant blood components transmit prion disease following a single blood transfusion: a sheep model of vcjd. | variant cjd (vcjd) is an incurable, infectious human disease, likely arising from the consumption of bse-contaminated meat products. whilst the epidemic appears to be waning, there is much concern that vcjd infection may be perpetuated in humans by the transfusion of contaminated blood products. since 2004, several cases of transfusion-associated vcjd transmission have been reported and linked to blood collected from pre-clinically affected donors. using an animal model in which the disease mani ... | 2011 | 21858015 |
| the effects of host age on the transport of complement-bound complexes to the spleen and the pathogenesis of intravenous scrapie infection. | infections with variant creutzfeldt-jakob disease (vcjd) have almost exclusively occurred in young patients, but the reasons for this age distribution are uncertain. our data suggest that the pathogenesis of many peripherally acquired transmissible spongiform encephalopathy (tse) agents is less efficient in aged individuals. four vcjd cases linked to transfusion of vcjd-contaminated blood or blood products have been described. three cases occurred in elderly patients, implying that intravenous e ... | 2012 | 22031932 |
| consumers' understanding and concerns about bovine spongiform encephalopathy (bse): comparison among canadian, american, and japanese consumers. | in spite of much analysis of the impact of bovine spongiform encephalopathy (bse) on consumer perceptions and meat purchases, there has been little explicit analysis of the level of bse knowledge. in this study the role of knowledge about bse was examined in canada, the united states, and japan. in addition, the level of knowledge was linked to human health concerns regarding bse and whether there is agreement with paying a premium for beef with bse animal tests. from a public policy perspective ... | 2011 | 22043916 |
| risk of prion disease transmission through bovine-derived bone substitutes: a systematic review. | background: despite the causal association between variant creutzfeldt - jakob disease and bovine spongiform encephalopathy (bse), bovine origin graft materials are widely used during dental surgical procedures. the aim of this study was to assess the risk of bse transmission through anorganic bovine bone substitutes. methods: electronic database of medline was searched to identify relevant studies regarding our focused questions, presence of bse prion infectivity in raw bovine bone, bse prion i ... | 2011 | 22171533 |
| The Management of Blood Safety in the Presence of Uncertain Risk: A United Kingdom Perspective. | Millions of patients in the UK benefit from the use of both plasma derivatives and blood components that are seen as critical interventions in current medicine. Measures are in place to significantly reduce the risks associated with blood transfusion and plasma derivatives; however, these measures themselves are not risk free. Over the past 20 years, advances in technology and regulation have seen major reductions in the risks associated with transfusion. International blood services, industry, ... | 2011 | 22126710 |
| transcriptional modulation in a leukocyte-depleted splenic cell population during prion disease. | prion replication in the periphery precedes neuroinvasion in many experimental rodent scrapie models, and in natural sheep scrapie and chronic wasting disease (cwd) in cervids. prions propagate in the germinal centers of secondary lymphoid organs and are strongly associated with follicular dendritic cells (fdc) and possibly circulating dendritic cells and macrophages. given the importance of lymphoid organs in prion disease transmission and pathogenesis, gene expression studies may reveal host f ... | 2011 | 22043911 |
| Epidemiological indication for a role of sheep in the emergence of variant Creutzfeldt-Jakob disease. | | 2012 | 21945301 |
| 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 |
| prion diseases as transmissible zoonotic diseases. | prion diseases, also called transmissible spongiform encephalopathies (tses), lead to neurological dysfunction in animals and are fatal. infectious prion proteins are causative agents of many mammalian tses, including scrapie (in sheep), chronic wasting disease (in deer and elk), bovine spongiform encephalopathy (bse; in cattle), and creutzfeldt-jakob disease (cjd; in humans). bse, better known as mad cow disease, is among the many recently discovered zoonotic diseases. bse cases were first repo ... | 0 | 24159531 |
| 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 |
| 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 |
| variant creutzfeldt-jakob disease: an update. | variant creutzfeldt-jakob disease (vcjd) is a novel human prion disease caused by the bovine spongiform encephalopathy agent. most cases have occurred in the uk, with smaller numbers in 11 other countries. all definite vcjd cases have occurred in methionine homozygotes at codon 129 in the prion protein gene. following oral infection, the vcjd agent appears to replicate in lymphoid tissues during the asymptomatic phase of the incubation period. at present, four probable cases of vcjd infection ha ... | 2012 | 22505363 |
| 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 |
| 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 |
| 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 |
| prion transmission prevented by modifying the β2-α2 loop structure of host prpc. | zoonotic prion transmission was reported after the bovine spongiform encephalopathy (bse) epidemic, when >200 cases of prion disease in humans were diagnosed as variant creutzfeldt-jakob disease. assessing the risk of cross-species prion transmission remains challenging. we and others have studied how specific amino acid residue differences between species impact prion conversion and have found that the β2-α2 loop region of the mouse prion protein (residues 165-175) markedly influences infection ... | 2014 | 24431459 |
| 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 |
| prion disease: chemotherapeutic strategies. | prion diseases, also known as transmissible spongiform encephalopathies, are invariably fatal neurodegenerative diseases for which there are no efficacious treatments. thousands of compounds have been screened for anti-prion effect, and yet of those that have effect in vitro, very few show effect in vivo, especially if administered in the later stages of disease. however, with new techniques for early diagnosis being developed, and with further insight into the pathogenesis of early disease, inc ... | 2012 | 22420513 |
| could immunomodulation be used to prevent prion diseases? | all prion diseases are currently without effective treatment and are universally fatal. the underlying pathogenesis of prion diseases (prionoses) is related to an autocatalytic conformational conversion of prp(c) (c for cellular) to a pathological and infectious conformer known as prp(sc) (sc for scrapie) or prp(res) (res for proteinase k resistant). the past experience with variant creutzfeldt-jakob disease, which originated from bovine spongiform encephalopathy, as well as the ongoing epidemic ... | 2012 | 22397565 |
| 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 |
| 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 |
| 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 |
| 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 |
| variant creutzfeldt-jakob disease in a patient with heterozygosity at prnp codon 129. | | 2017 | 28099827 |