Publications
| Title | Abstract | Year Filter | PMID(sorted ascending) Filter |
|---|
| bovine spongiform encephalopathy and variant creutzfeldt-jakob disease. | 2004 | 15992264 | |
| australian sporadic cjd analysis supports endogenous determinants of molecular-clinical profiles. | to define the protease-resistant prion protein (prpres) types and associated clinical profiles in australian patients with sporadic creutzfeldt-jakob disease (cjd) to allow comparison with those reported from other continents and concomitantly reaffirm absence of variant cjd (vcjd). | 2005 | 16009895 |
| cerebrospinal fluid biomarkers in creutzfeldt-jakob disease. | creutzfeldt-jakob disease (cjd) is a rare neurodegenerative disorder. since the emergence of variant cjd (vcjd) vigilance concerning the disease's incidence has increased and the interest in accurate in vivo diagnosis has augmented. so far, a large number of biomarkers has been investigated as aid in the differential diagnosis of sporadic creutzfeldt-jakob disease (scjd) and vcjd. these include, among others, neuron-specific enolase (nse), microtubuli associated protein tau, s-100beta, amyloid-b ... | 2005 | 16023527 |
| leucoreduction and variant creutzfeldt-jakob disease. | 2005 | 16101813 | |
| accumulation of prion protein in the peripheral nervous system in human prion diseases. | after the finding that anti-prion antibodies stain sensory and sympathetic ganglia in variant creutzfeldt-jakob disease (vcjd), it was suggested that this localization supported the oral route of entry. however, prion accumulation subsequently also appeared in the peripheral nervous system (pns) in sporadic cases. this study aims at evaluating the extent of prion protein accumulation in the pns in all clinicopathologic subgroups of the disorder, with the exception of the familial and sporadic fo ... | 2005 | 16106220 |
| abnormal prion protein in the retina of the most commonly occurring subtype of sporadic creutzfeldt-jakob disease. | involvement of the eye has been reported in patients with variant creutzfeldt-jakob disease (vcjd), but there is disagreement on whether retinal involvement occurs in sporadic creutzfeldt-jakob disease (scjd). | 2005 | 16113366 |
| [update on transmissible spongiform subacute encephalopathies (tsse)]. | this update concerns human and ruminant transmissible spongiform subacute encephalopathies (tsse). the latest data on variant creutzfeldt-jakob disease confirm that new cases are less frequent than feared some years ago, but subclinical carriers could be a source of iatrogenic infection. the macaque is a good model of human oral transmission of bovine spongiform encephalopathy (bse). the latest data on bse in europe confirm the effectiveness of precautionary measures taken in 1996 and 2000. conc ... | 2005 | 16114866 |
| genotype frequencies at codon 129 of the prion protein gene in brazil: implications in susceptibility to variant creutzfeldt-jakob disease compared to european and asian populations. | a polymorphism at codon 129 of the prion protein gene has been shown to confer genetic susceptibility to prion diseases, and to influence the epidemic course of variant creutzfeldt-jakob disease. we employed a pcr-endonuclease digestion-based assay to investigate this genetic trait in brazil, and then compared our results to previously published data from several european and asian countries. | 2005 | 16119432 |
| the use of non-prion biomarkers for the diagnosis of transmissible spongiform encephalopathies in the live animal. | scrapie and bovine spongiform encephalopathy (bse) are major global concerns and the emergence of variant creutzfeldt-jakob disease (vcjd) has caused turmoil for blood transfusion services and hospitals worldwide. recent reports of iatrogenic cjd (icjd) cases following blood transfusions from transmissible spongiform encephalopathies (tse)-infected donors have fuelled this concern. major diagnostic tests for bse and scrapie are conducted post-mortem from animals in late stages of the disease. al ... | 2005 | 16120244 |
| bovine prion protein gene (prnp) promoter polymorphisms modulate prnp expression and may be responsible for differences in bovine spongiform encephalopathy susceptibility. | the susceptibility of humans to the variant creutzfeldt-jakob disease is greatly influenced by polymorphisms within the human prion protein gene (prnp). similar genetic differences exist in sheep, in which prnp polymorphisms modify the susceptibility to scrapie. however, the known coding polymorphisms within the bovine prnp gene have little or no effect on bovine spongiform encephalopathy (bse) susceptibility in cattle. we have recently found a tentative association between prnp promoter polymor ... | 2005 | 16141216 |
| inactivation of the bse agent by the heat and pressure process for manufacturing gelatine. | dietary exposure to the bovine spongiform encephalopathy (bse) agent is the probable cause of variant creutzfeldt-jakob disease in people. the industrial manufacturing process for the production of gelatine and colloidal protein by the heat and pressure process was downscaled accurately and its capacity to remove or inactivate bse infectivity was investigated. gelatine was made from bones experimentally contaminated with mouse brain infected with the 301v strain of mouse-passaged bse agent in wh ... | 2005 | 16157568 |
| community blood supply model: development of a new model to assess the safety, sufficiency, and cost of the blood supply. | through a combination of predonation donor screening and donated unit testing, the blood supply is safer than ever. however, as a result of increasingly stringent screening measures, one of the greatest threats may be an insufficient supply. the balance between safety and adequacy of the blood supply has not received enough attention. | 2005 | 16160212 |
| high levels of disease related prion protein in the ileum in variant creutzfeldt-jakob disease. | 2005 | 16162963 | |
| variant creutzfeldt-jakob disease and prions in the blood supply. | 2004 | 16163166 | |
| an immunoassay for the pathological form of the prion protein based on denaturation and time resolved fluorometry. | concern about the possible secondary spread of variant creutzfeldt-jakob disease (vcjd) through blood transfusion and blood products has increased the need for a sensitive and rapid test for the identification of prp(sc) in specimens collected non-invasively from living persons. furthermore, an accurate estimate of the prevalence of pre-clinical vcjd in the british population would be possible if there were such a test that could be applied to specimens available readily (e.g. blood and urine). ... | 2006 | 16219367 |
| variant creutzfeldt-jakob disease: implications for the health care system. | the recognition of the first cases of variant creutzfeldt-jakob disease in the united kingdom (uk) in 1996 and the realisation that this new disease represented the human form of the cattle disease bse has prompted a considerable investment in research, particularly in the uk, europe and the united states (us). much has been learnt about this disease but much is still unknown. infectivity is not destroyed by conventional sterilisation and disinfection treatment methods. this, combined with the w ... | 2005 | 16222925 |
| a response to 'lymphocyte contamination of laryngoscope blades--a possible vector for transmission of variant creutzfeldt-jakob disease'. | 2005 | 16229718 | |
| variant creutzfeldt-jakob disease death, united states. | the only variant creutzfeldt-jakob disease (vcjd) patient identified in the united states died in 2004, and the diagnosis was confirmed by analysis of autopsy tissue. the patient likely acquired the disease while growing up in great britain before immigrating to the united states in 1992. additional vcjd patients continue to be identified outside the united kingdom, including 2 more patients in ireland, and 1 patient each in japan, portugal, saudi arabia, spain, and the netherlands. the reports ... | 2005 | 16229761 |
| dementia associated with infectious diseases. | at the turn of the last century, infectious diseases represented an important cause of health morbidity and behavioral changes. neurosyphilis, for example, was relatively common at the time and often led to the development of cognitive impairment and dementia. with the advent of effective antibiotic treatment, the association between infectious diseases and dementia became increasingly less frequent, although a resurgence of interest in this area has taken place during the past 15 years with the ... | 2005 | 16240484 |
| molecular evolution of the sheep prion protein gene. | transmissible spongiform encephalopathies (tses) are infectious, fatal neurodegenerative diseases characterized by aggregates of modified forms of the prion protein (prp) in the central nervous system. well known examples include variant creutzfeldt-jakob disease (vcjd) in humans, bse in cattle, chronic wasting disease in deer and scrapie in sheep and goats. in humans, sheep and deer, disease susceptibility is determined by host genotype at the prion protein gene (prnp). here i examine the molec ... | 2005 | 16243700 |
| prptse distribution in a primate model of variant, sporadic, and iatrogenic creutzfeldt-jakob disease. | human prion diseases, such as creutzfeldt-jakob disease (cjd), are neurodegenerative and fatal. sporadic cjd (scjd) can be transmitted between humans through medical procedures involving highly infected organs, such as the central nervous system. however, in variant cjd (vcjd), which is due to human contamination with the bovine spongiform encephalopathy (bse) agent, lymphoreticular tissue also harbors the transmissible spongiform encephalopathy-associated prion protein (prp(tse)), which poses a ... | 2005 | 16254368 |
| no clinical evidence of hidden vcjd in uk children. | between may 1997 and november 2004 this national prospective surveillance study identified 1007 children with "progressive intellectual and neurological deterioration" (pind). in most cases specific diagnoses were made, but of 92 undiagnosed children with pind 46 had died and only four underwent full necropsy. there was no clinical evidence of variant creutzfeldt-jakob disease (vcjd) in these undiagnosed cases, but without necropsy it is not possible to exclude vcjd completely. | 2006 | 16260461 |
| variant creutzfeldt-jakob disease transmission by plasma products: assessing and communicating risk in an era of scientific uncertainty. | a substantial body of animal data indicates that transmissible spongiform encephalopathies (tses) are transmitted through blood. these data have been augmented in the past year by reports that two recipients of red cells from donors with variant creutzfeldt-jakob disease (vcjd) in the united kingdom have acquired this infection. most of the blood donations collected in countries affected by bovine spongiform encephalopathy (bse) and vcjd also contribute plasma to fractionation pools. thus, a num ... | 2005 | 16262750 |
| prion biology in transfusion medicine: implications for lab testing. | although eerily silent for many years after the recognition of scrapie in 1759, tses remained present within the genome of some mammals. not since the mid-1950s when dr. carleton gadjusek visited the fore indians of new guinea to study kuru, however, has there been a more frenetic interest by governmental investigators. certainly, the u.k. experience has heralded a renewed interest in tses due to the notoriety associated with younger subjects succumbing to a variant cjd traced to the ingestion o ... | 2005 | 16265819 |
| ethical considerations in presymptomatic testing for variant cjd. | variant creutzfeldt-jakob disease (vcjd) is a fatal, transmissible, neurodegenerative disorder for which there is currently no effective treatment. vcjd arose from the zoonotic spread of bovine spongiform encephalopathy. there is now compelling evidence for human to human transmission through blood transfusions from presymptomatic carriers and experts are warning that the real epidemic may be yet to come. imperatives exist for the development of reliable, non-invasive presymptomatic diagnostic t ... | 2005 | 16269554 |
| risk assessment of variant creutzfeldt-jakob disease in cosmetics. | 2005 | 16276156 | |
| gerstmann-sträussler-scheinker disease amyloid protein polymerizes according to the "dock-and-lock" model. | prion protein (prp) amyloid formation is a central feature of genetic and acquired prion diseases such as gerstmann-sträussler-scheinker disease (gss) and variant creutzfeldt-jakob disease. the major component of gss amyloid is a prp fragment spanning residues approximately 82-146, which when synthesized as a peptide, readily forms fibrils featuring gss amyloid. the present study employed surface plasmon resonance (spr) to characterize the binding events underlying prp82-146 oligomerization at t ... | 2006 | 16286452 |
| risk factors for variant creutzfeldt-jakob disease: a case-control study. | to investigate the potential risk factors for variant creutzfeldt-jakob disease (vcjd) in the united kingdom. | 2006 | 16287153 |
| the risk of transmission of variant creutzfeldt-jakob disease via contact lenses and ophthalmic devices. | this review collated the available information regarding the risk of transmission of variant creutzfeldt-jakob disease (vcjd) via contact lenses and other ophthalmic devices. the topics examined include: the emerging background science of the unconventional infective agent, the prion, particularly those factors affecting transmission; the estimates of the number of undiagnosed infective individuals; and evidence of infectivity in the external eye. despite many uncertainties in the literature, we ... | 2002 | 16303485 |
| [prion diseases as zoonosis]. | prion diseases such as bovine spongiform encephalopathy (bse) have been recognized as zoonosis since the existence of variant creutzfeldt-jakob disease (vcjd) was reported in 1996. bse became a serious social problem even in japan after the first bse case was found in 2001. the incidence of bse in eu and uk appears declining, and the vcjd incidence also shows a tendency to decrease. on the contrary, fears for the spread of bse became actual problems: bse occurrence outside of eu, transmission of ... | 2005 | 16308529 |
| survey of zoonoses recorded in scotland between 1993 and 2002. | all the human and animal laboratory reports of zoonoses sent to health protection scotland between 1993 and 2002 were identified. there were 24,946 reports from veterinary laboratories, and 94,718 (20 per cent) of the 468,214 reports from medical laboratories were considered to be zoonotic. the most common reports of zoonoses from people were campylobacter, salmonella, cryptosporidium and giardia species and escherichia coli o157. the most common reports of zoonoses from animals were salmonella, ... | 2005 | 16311383 |
| pathological prion protein in muscles of hamsters and mice infected with rodent-adapted bse or vcjd. | recently, pathological prion protein (prp(tse)) was detected in muscle from sheep infected with scrapie, the archetype of transmissible spongiform encephalopathies (tses). this finding has highlighted the question of whether mammalian muscle may potentially also provide a reservoir for tse agents related to bovine spongiform encephalopathy (bse) and variant creutzfeldt-jakob disease (vcjd). here, results are reported from studies in hamsters and mice that provide direct experimental evidence, fo ... | 2006 | 16361438 |
| raised csf phospho-tau concentrations in variant creutzfeldt-jakob disease: diagnostic and pathological implications. | to investigate whether phosphorylated tau protein (tau-pt181) is increased in variant creutzfeldt-jakob disease (vcjd) and if the tau-pt181/tau protein ratio is useful for distinguishing between patients with and without cjd. | 2006 | 16361602 |
| pathogenesis and prevalence of variant creutzfeldt-jakob disease. | in the late 1980s and early 1990s, there was widespread exposure of the uk population to bovine spongiform encephalopathy (bse)-contaminated food products, which has led to over 150 deaths from variant creutzfeldt-jakob disease (vcjd). although the pathogenesis in humans is not fully understood, data from animal models and, to a lesser extent, patients with vcjd suggest that oral exposure to bse is rapidly followed by accumulation of prp(res) in gut-associated lymphoid tissue, then, after haemat ... | 2006 | 16362983 |
| infection and disease: cause and cure. | much can be learnt about the mechanisms by which micro-organisms cause disease from the ways that they interact with cells and tissues. this issue of the journal of pathology contains articles that address the roles that cell and tissue biology and pathology are playing in the elucidation of these mechanisms. a review of variant creutzfeldt-jakob disease is followed by a discussion of severe acute respiratory syndrome (sars). two articles on human papillomavirus (hpv) infection address the assoc ... | 2006 | 16362991 |
| [prion disease as infectious disease transmissible from animals to human]. | prion diseases such as bovine spongiform encephalopathy (bse) have been recognized as zoonosis since the existence of variant creutzfeldt-jakob disease (vcjd) was reported in 1996. after then, bse became a serious social problem all over the world. the incidence of bse in eu and uk appears declining, and the vcjd incidence also shows a tendency to decrease. on the contrary, fears for the spread of bse became actual problems: bse occurrence outside of eu, introduction of bse to other ruminants, a ... | 2005 | 16363697 |
| bovine spongiform encephalopathy statement of possible relation with new variant creutzfeldt-jakob disease: effects on the welfare of united kingdom cattle. | although measures to control bovine spongiform encephalopathy (bse) had been in force in the united kingdom for many years and had resulted in a marked decline in clinical cases, the announcement by the secretary of state for health on march 20, 1996, that a new variant form of creutzfeldt-jakob disease may be linked with exposure to bse, resulted in the introduction of several new control measures. these measures included a scheme banning human consumption of meat from cattle who were more than ... | 1998 | 16363943 |
| managing the risk of transmission of variant creutzfeldt jakob disease by blood products. | whereas plasma-derived clotting factor concentrates now have a very good safety record for not being infectious for lipid enveloped viruses, concern has arisen about the possibility that prion diseases might be transmitted by blood products. there is epidemiological evidence that classical sporadic creutzfeld jakob disease (cjd) is not transmitted by blood transfusion. there is now good evidence that the abnormal prion associated with variant cjd can be transmitted by transfusion of fresh blood ... | 2006 | 16371015 |
| risks and side effects of therapy with plasma and plasma fractions. | transfusion of plasma can lead to adverse reactions or events. immune-mediated reactions are most common--these include allergic and anaphylactic reactions, transfusion-related acute lung injury (trali) and haemolysis. they can range in severity from mild to fatal. fluid overload and citrate toxicity can occur after rapid or massive transfusion. in developed countries, microbial transmission rates are low because of donor selection and testing. pathogen reduction processes can be applied to eith ... | 2006 | 16377549 |
| prion disease genetics. | prion diseases have stimulated intense scientific scrutiny since it was proposed that the infectious agent was devoid of nucleic acid. despite this finding, genetics has played a key role in understanding the pathobiology and clinical aspects of prion disease through the effects of a series of polymorphisms and mutations in the prion protein gene (prnp). the advent of variant creutzfeldt-jakob disease has confirmed one of the most powerful human genetic susceptibility factors, as all tested pati ... | 2006 | 16391566 |
| detection of type 1 prion protein in variant creutzfeldt-jakob disease. | molecular typing of the abnormal form of the prion protein (prp(sc)) has come to be regarded as a powerful tool in the investigation of the prion diseases. all evidence thus far presented indicates a single prp(sc) molecular type in variant creutzfeldt-jakob disease (termed type 2b), presumably resulting from infection with a single strain of the agent (bovine spongiform encephalopathy). here we show for the first time that the prp(sc) that accumulates in the brain in variant creutzfeldt-jakob d ... | 2006 | 16400018 |
| comparative evidence for a link between peyer's patch development and susceptibility to transmissible spongiform encephalopathies. | epidemiological analyses indicate that the age distribution of natural cases of transmissible spongiform encephalopathies (tses) reflect age-related risk of infection, however, the underlying mechanisms remain poorly understood. using a comparative approach, we tested the hypothesis that, there is a significant correlation between risk of infection for scrapie, bovine spongiform encephalopathy (bse) and variant cjd (vcjd), and the development of lymphoid tissue in the gut. | 2006 | 16405727 |
| short te quantitative proton magnetic resonance spectroscopy in variant creutzfeldt-jakob disease. | variant creutzfeldt-jakob disease (vcjd) is a fatal neurodegenerative disorder. clinical diagnosis is difficult in the early stages as the disease often presents with non-specific psychiatric and neurological symptoms. to investigate the diagnostic potential of quantitative short te in vivo mrs, and the nature and anatomical distribution of biochemical abnormalities in vcjd, localised single-voxel spectra (te/tr 30 ms/2,000 ms) were acquired from three brain regions: thalami, caudate nuclei and ... | 2006 | 16408201 |
| distinct glycoform ratios of protease resistant prion protein associated with prnp point mutations. | inherited prion diseases are neurodegenerative disorders caused by autosomal dominant mutations in the human prion protein gene (prnp). kindred with inherited prion disease can show remarkable phenotypic variability that has yet to be explained. here we report analysis of protease resistant disease-related prion protein (prp(sc)) isoforms from a range of inherited prion disease cases (point mutations p102l, d178n, e200k and 2-, 4- and 6-octapeptide repeat insertions) and show that the glycoform ... | 2006 | 16415305 |
| risks of transmission of variant creutzfeldt-jakob disease by blood transfusion. | variant creutzfeldt-jakob disease (vcjd) was first identified in 1996 in the uk, and results from human exposure to the bovine spongiform encephalopathy (bse) agent. vcjd has subsequently been identified in 10 additional countries, and numbers continue to increase in the uk. unlike other human prion diseases, infectivity and the disease-associated form of the prion protein are readily detected in lymphoid tissues in vcjd. in experimental bse infection in a sheep model, infectivity has been trans ... | 2005 | 16416391 |
| creutzfeldt-jakob disease and vision. | this review describes a group of diseases known as the transmissible spongiform encephalopathies (tses), which affect animals and humans. examination of affected brain tissue suggests that these diseases are caused by the acquisition and deposition of prion protein (prp). creutzfeldt-jakob disease (cjd) is the most important form of tse in humans with at least four different varieties of the disease. variant cjd (vcjd), a new form of the disease found in the uk, has several features that differ ... | 2006 | 16430434 |
| periodic electroencephalogram complexes in a patient with variant creutzfeldt-jakob disease. | based on the current criteria, the diagnosis of "possible" or "probable" variant creutzfeldt-jakob disease (vcjd) implies the absence of periodic sharp wave complexes (pswcs) in the electroencephalogram (eeg). to verify this point, we investigated the development of the eeg changes along the course of the disease in a pateint with vcjd. | 2006 | 16437565 |
| prions in skeletal muscles of deer with chronic wasting disease. | the emergence of chronic wasting disease (cwd) in deer and elk in an increasingly wide geographic area, as well as the interspecies transmission of bovine spongiform encephalopathy to humans in the form of variant creutzfeldt jakob disease, have raised concerns about the zoonotic potential of cwd. because meat consumption is the most likely means of exposure, it is important to determine whether skeletal muscle of diseased cervids contains prion infectivity. here bioassays in transgenic mice exp ... | 2006 | 16439622 |
| variant creutzfeldt-jakob disease: risk of transmission by blood transfusion and blood therapies. | in the last decade, a new variant of the human prion disease creutzfeldt-jakob disease (now known as variant cjd or vcjd) was identified and causally linked to dietary exposure to bovine spongiform encephalopathy (bse) during the 1980s and early 1990s. preliminary studies in animal models suggest that prions can be transmitted by blood. based on two recent reports of iatrogenic vcjd transmission by blood transfusion in humans, a department of health-sponsored risk assessment warned that recipien ... | 2006 | 16445812 |
| clinical implications of emerging pathogens in haemophilia: the variant creutzfeldt-jakob disease experience. | the impact of variant creutzfeldt-jakob disease (vcjd) on the clinical practice of haemophilia in the uk is coloured by the haemophilia community's experience of hepatitis c virus and human immunodeficiency virus (hiv) transmission via plasma-derived therapies in the 1980s, when the delay in recognizing and acting on the potential risks cost many patients their lives and left others to manage another chronic disease. this crisis prompted organisations such as the united kingdom haemophilia centr ... | 2006 | 16445813 |
| [variant creutzfeldt-jakob disease, 10 years later]. | 2006 | 16462658 | |
| prions in dentistry--what are they, should we be concerned, and what can we do? | to briefly review the characteristics of prions, the risk of transmission and implications for infection control in dentistry. | 2006 | 16480606 |
| vcjd prion acquires altered virulence through trans-species infection. | variant creutzfeldt-jakob disease (vcjd) appears to be caused by infection with the bovine spongiform encephalopathy (bse) agent. to date, all patients with vcjd are homozygous for methionine at codon 129 of the prp gene. to investigate the relationship between polymorphism at codon 129 and susceptibility to bse or vcjd prions, we performed splenic follicular dendritic cell assay with humanized knock-in mice through peripheral infection. all humanized knock-in mice showed little or no susceptibi ... | 2006 | 16480953 |
| new thoughts on cjd, surgical instruments and disease transmission. | for most people, the threat of widespread transmission of variant creutzfeldt-jakob disease in humans as a result of bovine spongiform encephalopathy has passed into the history books. but concerns about transmission of the disease during certain medical procedures still exist. | 2006 | 16483102 |
| detection and localization of prpsc in the skeletal muscle of patients with variant, iatrogenic, and sporadic forms of creutzfeldt-jakob disease. | variant creutzfeldt-jakob disease (vcjd) differs from other human prion diseases in that the pathogenic prion protein prp(sc) can be detected to a greater extent at extraneuronal sites throughout the body, principally within lymphoid tissues. however, a recent study using a high-sensitivity western blotting technique revealed low levels of prp(sc) in skeletal muscle from a quarter of swiss patients with sporadic cjd (scjd). this posed the question of whether prp(sc) in muscle could also be detec ... | 2006 | 16507908 |
| the alternative role of 14-3-3 zeta as a sweeper of misfolded proteins in disease conditions. | here, we propose a novel hypothesis that 14-3-3 zeta might act as a sweeper of misfolded proteins by facilitating the formation of aggregates, which are referred to as inclusion bodies. studies on the localization of the 14-3-3 proteins in different types of inclusion bodies in the brain including neurofibrillary tangle in alzheimer's disease, pick bodies in pick's disease, lewy body-like hyaline inclusions in sporadic amyotrophic lateral sclerosis, prion/florid plaques in sporadic/variant creut ... | 2006 | 16516399 |
| the first japanese case of variant creutzfeldt-jakob disease showing periodic electroencephalogram. | 2006 | 16530582 | |
| the effects of leukodepletion on the generation and removal of microvesicles and prion protein in blood components. | universal leukodepletion (ld) has been implemented in the united kingdom to reduce the risk of transfusion-transmitted variant creutzfeldt-jakob disease. if ld causes microvesiculation of blood cells, however, potentially infectious membrane-associated prion could reach the final products. | 2006 | 16533284 |
| predictive ability of sequential surveys in determining donor loss from increasingly stringent variant creutzfeldt-jakob disease deferral policies. | predonation screening questions about travel increase the safety of the blood supply from diseases such as variant creutzfeldt-jakob disease (vcjd) and malaria. this study examines the ability of sequential surveys to predict actual travel deferrals and the operational validity of travel questions. | 2006 | 16533291 |
| diagnostics for tse agents. | bovine spongiform encephalopathy (bse) is a fatal acquired neuro-degenerative disease in cattle, belonging to the group of transmissible spongiform encephalopathies (tses) or prion diseases. since its first recognition in the u.k. in 1986, bse has raised great public health concerns because the bse agent has been shown to cause variant creutzfeldt jakob disease (vcjd) in humans. with the introduction of mandatory active surveillance programmes in the european union the need to develop rapid test ... | 2006 | 16566455 |
| the neuropathologic phenotype of experimental ovine bse is maintained after blood transfusion. | iatrogenic transmission by blood transfusion has been described in cases of human variant creutzfeldt-jakob disease (vcjd), experimental ovine bovine spongiform encephalopathy (bse), and natural sheep scrapie, demonstrating that blood in these prion diseases is infectious. however, the possible effect of the transfusion, derived from differences in the inoculum (blood) and the route of infection (intravenous), on the pathologic phenotype of the disease in the recipients is not known. this study ... | 2006 | 16569766 |
| donor selection criteria to maximize double platelet products (dpp) by platelet apheresis. | variant creutzfeldt-jakob disease brought us to perform a study to diminish donor exposure from transfusion of platelet concentrates. the current study aimed to develop donor selection criteria that maximize the likelihood of deriving single donor platelets and producing double platelet products (dpp). donors were recruited among plasmapheresis donors and among other donors when the selected donors did not show up. donor precount and body weight and haematocrit were examined as determinants of h ... | 2006 | 16574489 |
| what can we learn from the oral intake of prions by sheep? | the central nervous system is the ultimate target of prions, the agents responsible for fatal neurodegenerative diseases known as transmissible spongiform encephalopathies (tses). the neuro-invasive phase and its related clinical signs take place after a long incubation period. during this asymptomatic phase, however, active transport and replication of the infectious agent take place in peripheral sites. the oral infection route has been extensively studied because of its implication in the rec ... | 2006 | 16575798 |
| new variant creutzfeldt-jakob disease: a controversial but potential blood transfusion risk. | 2003 | 16583978 | |
| preparation of soluble infectious samples from scrapie-infected brain: a new tool to study the clearance of transmissible spongiform encephalopathy agents during plasma fractionation. | concern about the safety of blood, blood components, and plasma-derived products with respect to prions has increased since the report of two blood-related infections of variant creutzfeldt-jakob disease in the united kingdom. efforts were directed toward the development of procedures able to remove or inactivate prions from blood components or plasma-derived products with brain fractions of transmissible spongiform encephalopathy (tse)-infected rodents as spiking materials. these spiking materi ... | 2006 | 16584444 |
| unsuccessful intraventricular pentosan polysulphate treatment of variant creutzfeldt-jakob disease. | pentosan polysulphate, delivered by chronic intraventricular infusion, has been proposed as a potential therapy for human prion disease. the first treated patient is still alive several years after treatment started. here we describe in detail a case of variant creutzfeldt-jakob disease in which this treatment was started at a relatively early stage but had no definite clinical benefit. the patient died from disease progression 16 months after diagnosis and 5 months after pentosan polysulphate t ... | 2006 | 16598408 |
| variant creutzfeldt-jakob disease: prion protein genotype analysis of positive appendix tissue samples from a retrospective prevalence study. | to perform prion protein gene (prnp) codon 129 analysis in dna extracted from appendix tissue samples that had tested positive for disease associated prion protein. | 2006 | 16606639 |
| the expanding universe of prion diseases. | prions cause fatal and transmissible neurodegenerative disease. these etiological infectious agents are formed in greater part from a misfolded cell-surface protein called prp(c). several mammalian species are affected by the diseases, and in the case of "mad cow disease" (bse) the agent has a tropism for humans, with negative consequences for agribusiness and public health. unfortunately, the known universe of prion diseases is expanding. at least four novel prion diseases--including human dise ... | 2006 | 16609731 |
| a note on parameter estimation for variant creutzfeldt-jakob disease epidemic models. | a recent series of papers has raised issues regarding estimation of the key epidemiological parameters of variant creutzfeldt-jakob disease (vcjd) from fitting survival models to case data. in particular, it was stated that the scale of the epidemic cannot be estimated and must be fixed in any analysis. we show that this problem is an artefact of the approximate likelihood used in these papers to facilitate model-fitting, and is not a concern if estimation is based on the full likelihood. we als ... | 2007 | 16612835 |
| predicting susceptibility and incubation time of human-to-human transmission of vcjd. | identification of possible transmission of variant creutzfeldt-jakob disease (vcjd) via blood transfusion has caused concern over spread of the disease within the human population. we aimed to model iatrogenic spread to enable a comparison of transmission efficiencies of vcjd and bovine spongiform encephalopathy (bse) and an assessment of the effect of the codon-129 polymorphism on human susceptibility. | 2006 | 16632309 |
| new ratios for the detection and classification of cjd in multisequence mri of the brain. | we present a method for the analysis of deep grey brain nuclei for accurate detection of human spongiform encephalopathy in multisequence mri of the brain. we employ t1, t2 and flair-t2 mr sequences for the detection of intensity deviations in the internal nuclei. the mr data are registered to a probabilistic atlas and normalised in intensity prior to the segmentation of hyperintensities using a foveal model. anatomical data from a segmented atlas are employed to refine the registration and remo ... | 2005 | 16685996 |
| molecular aspects of disease pathogenesis in the transmissible spongiform encephalopathies. | the transmissible spongiform encephalopathies (tse), or prion diseases, are a group of rare, fatal, and transmissible neurodegenerative diseases of mammals for which there are no known viral or bacterial etiological agents. the bovine form of these diseases, bovine spongiform encephalopathy (bse), has crossed over into humans to cause variant creutzfeldt-jakob disease. as a result, bse and the tse diseases are now considered a significant threat to human health. understanding the basic mechanism ... | 2006 | 16691009 |
| endoplasmic reticulum stress features are prominent in alzheimer disease but not in prion diseases in vivo. | prion diseases and alzheimer disease (ad) share a variety of clinical and neuropathologic features (e.g. progressive dementia, accumulation of abnormally folded proteins in diseased tissue, and pronounced neuronal loss) as well as pathogenic mechanisms like generation of oxidative stress molecules and complement activation. recently, it was suggested that neuronal death in ad may have its origin in the endoplasmic reticulum (er). cellular stress conditions can interfere with protein folding and ... | 2006 | 16691116 |
| protease-resistant prion protein in lymphoreticular tumors of variant creutzfeldt-jakob disease mice. | we report protease-resistant prion protein (prpres) in spontaneous lymphoreticular tumors of mice infected with the agent of variant creutzfeldt-jakob disease (vcjd). prpres may accumulate in lymphoreticular system tumors of asymptomatic persons with vcjd. the statistical power of estimates of vcjd prevalence might be increased by expanding screening to include samples of lymphoreticular neoplasms. | 2006 | 16704797 |
| a new human genotype prone to variant creutzfeldt-jakob disease. | 2006 | 16709965 | |
| blood audit evidenced-based cross-match requesting for lumbar spine surgery. | complete audit cycle. | 2006 | 16721282 |
| two cases of variant creutzfeldt-jakob disease (vcjd) referred to the department of community mental health, aldershot garrison in 2003. | in the year 2003 the department of community mental health (dcmh) at aldershot garrison received referrals of two soldiers, a sergeant and a lance corporal, who presented with a complex picture of neurological and psychiatric symptoms. both had been investigated very thoroughly by neurologists who, owing to the mainly negative results of their investigations, were unable to make a diagnosis. of the two patients one had also been assessed as a psychiatric in-patient in a civilian hospital and had ... | 2006 | 16749468 |
| surface decontamination of surgical instruments: an ongoing dilemma. | the issues of cross-infection and the survival of variant creutzfeldt jakob disease (vcjd) on surgical instruments have highlighted the importance of cleanliness of multiple-use surgical instruments. the aim of this study was to assess the levels of total protein contamination on a wide range of surgical instruments as an indication of the effectiveness of routine cleaning and disinfection in hospitals. anonymized trays of wrapped and autoclaved instruments were supplied to two laboratories for ... | 2006 | 16759745 |
| prion infectivity in variant creutzfeldt-jakob disease rectum. | disease-related prion protein (prp(sc)) is readily detectable in lymphoreticular tissues in variant creutzfeldt-jakob disease (vcjd), but not in other forms of human prion disease. this distinctive pathogenesis, with the unknown population prevalence of asymptomatic vcjd infection, has led to significant concerns that secondary transmission of vcjd prions will occur through a wide range of surgical procedures. to date prp(sc):prion infectivity ratios have not been determined in vcjd, and it is u ... | 2007 | 16763054 |
| clusterin expression in follicular dendritic cells associated with prion protein accumulation. | peripheral accumulation of abnormal prion protein (prp) in variant creutzfeldt-jakob disease and some animal models of transmissible spongiform encephalopathies (tses) may occur in the lymphoreticular system. within the lymphoid tissues, abnormal prp accumulation occurs on follicular dendritic cells (fdcs). clusterin (apolipoprotein j) has been recognized as one of the molecules associated with prp in tses, and clusterin expression is increased in the central nervous system where abnormal prp de ... | 2006 | 16767691 |
| an overview of prion biology and the role of blood filtration in reducing the risk of transfusion-transmitted variant creutzfeldt-jakob disease. | prions are infectious proteins believed to be responsible for a variety of progressive and fatal neurodegenerative diseases, collectively referred to as transmissible spongiform encephalopathies (tse). by 1996, it was recognized that ingestion of beef from cattle afflicted with a tse known as bovine spongiform encephalopathy, could result in a devastating human tse known as variant creutzfeldt-jakob disease (vcjd). two recent reports of probable transfusion-transmitted vcjd have raised concerns ... | 2006 | 16787827 |
| kuru in the 21st century--an acquired human prion disease with very long incubation periods. | kuru provides the principal experience of epidemic human prion disease. its incidence has steadily fallen after the abrupt cessation of its route of transmission (endocannibalism) in papua new guinea in the 1950s. the onset of variant creutzfeldt-jakob disease (vcjd), and the unknown prevalence of infection after the extensive dietary exposure to bovine spongiform encephalopathy (bse) prions in the uk, has led to renewed interest in kuru. we investigated possible incubation periods, pathogenesis ... | 2006 | 16798390 |
| variant creutzfeldt-jakob disease in the united kingdom and elsewhere: situation at the end of 2005. | 2006 | 16801715 | |
| dissociation of pathological and molecular phenotype of variant creutzfeldt-jakob disease in transgenic human prion protein 129 heterozygous mice. | all neuropathologically confirmed cases of variant creutzfeldt-jakob disease (vcjd), characterized by abundant florid plaques and type 4 disease-related prion protein (prp(sc)) in the brain, have been homozygous for methionine at polymorphic residue 129 of prnp. the distinctive neuropathological and molecular phenotype of vcjd can be faithfully recapitulated in prnp-null transgenic mice homozygous for human prp m129 but not v129, where a distinct prion strain is propagated. here we model suscept ... | 2006 | 16809423 |
| size frequency distributions of the florid prion protein aggregates in variant creutzfeldt-jakob disease follow a power-law function. | the objective was to test the hypothesis that the size frequency distributions of the prion protein (prp) plaques in cases of variant creutzfeldt-jakob disease (vcjd) follow a power-law function. the design was a retrospective neuropathological study. the patients were 11 cases of clinically and neuropathologically verified vcjd. size distributions of the diffuse and florid-type plaques were measured in several areas of the cerebral cortex and hippocampus from each case and a power-law function ... | 2006 | 16816906 |
| variant cjd (vcjd) and bovine spongiform encephalopathy (bse): 10 and 20 years on: part 1. | from 1986 more than 184,000 cattle in the uk and islands (of which >1,880 have been detected by active surveillance using rapid tests) and approaching 5,500 elsewhere have been confirmed with bse. the original 1988 ban on the use of ruminant-derived protein in ruminant feed has been upgraded and now prohibits the use of any processed animal protein in feed for any farmed food animal. as a result of rigorous enforcement this reinforced ban is now regarded as fully effective from 1 aug. 1996. reas ... | 2006 | 16823691 |
| variant cjd (vcjd) and bovine spongiform encephalopathy (bse): 10 and 20 years on: part 2. | up until february 2006, variant cjd (vcjd), the human disease associated with transmission of bse from cattle, has been confirmed in 160 patients resident in the uk and 28 elsewhere, some of whom have never visited the uk. cases have been reported in france (16 cases), ireland (3), usa (2), canada, italy, japan, the netherlands, portugal, saudi arabia and spain (1 each). the presumed main period of hazard for ingestion of the bse agent in bovine products in the uk is 1984-89, or perhaps up to 19 ... | 2006 | 16823692 |
| can a second wave of new variant of the cjd be discarded in absence of observation of clinical non met-met cases? | presently, all patients with clinical variant creutzfeldt-jakob disease in the united kingdom have been met-met at codon 129 of the prp gene. there is much worry about the possibility of a second wave of the epidemic in the 60% of the united kingdom population which are not met-met. | 2006 | 16830965 |
| cerebral amyloidoses: molecular pathways and therapeutic challenges. | alzheimer disease (ad) and creutzfeldt-jakob disease (cjd) are sporadic and genetic neurodegenerative conditions characterized by brain accumulation and deposition of protein aggregates. in ad, the key pathogenic event is linked to the formation of a 4-kda amyloid beta (abeta) peptide, generated by sequential cleavages of the amyloid precursor protein (app). in cjd and other prion diseases, the process is initiated by conformational changes of the cellular prion protein, or prp(c), into a beta-s ... | 2006 | 16842201 |
| projections of the future course of the primary vcjd epidemic in the uk: inclusion of subclinical infection and the possibility of wider genetic susceptibility. | the incidence of variant creutzfeldt-jakob disease (vcjd) in the united kingdom appears to be in decline, with only four deaths reported this year (to 6 september 2004). however, results of a survey of lymphoreticular tissues have suggested a substantially higher prevalence of vcjd than expected from the clinical data alone. there are two plausible explanations for this discrepancy: first, a proportion of those infected will not develop clinical disease (subclinical infection); and second, the g ... | 2005 | 16849160 |
| differentiation of scjd and vcjd forms by automated analysis of basal ganglia intensity distribution in multisequence mri of the brain--definition and evaluation of new mri-based ratios. | we present a method for the analysis of basal ganglia (including the thalamus) for accurate detection of human spongiform encephalopathy in multisequence magnetic resonance imaging (mri) of the brain. one common feature of most forms of prion protein diseases is the appearance of hyperintensities in the deep grey matter area of the brain in t2-weighted magnetic resonance (mr) images. we employ t1, t2, and flair-t2 mr sequences for the detection of intensity deviations in the internal nuclei. fir ... | 2006 | 16894998 |
| quantitative evaluation of prion inactivation comparing steam sterilization and chemical sterilants: proposed method for test standardization. | prions are notoriously resistant to inactivation. to prevent accidental transmission of variant creutzfeldt-jakob disease (vcjd), various decontamination procedures have been adopted for re-usable medical devices by the authorities of countries at risk. as the vcjd agent in humans has a wide tissue distribution, practical methods of prion decontamination urgently need to be standardized, as do other sterilization and disinfection procedures (european committee for standardization). this article ... | 2006 | 16895739 |
| clinical findings and diagnostic tests in creutzfeldt-jakob disease and variant creutzfeldt-jakob disease. | sporadic creutzfeldt-jakob disease (scjd) is a rare transmissible disease caused by accumulation of pathological prion protein in the cns. scjd typically affects patients in their sixties. the median disease duration in scjd (6 months) is shorter than in variant creutzfeldt-jakob disease (vcjd) (14 months). the clinical diagnosis in scjd is supported by the detection of periodic sharp and slow wave complexes (pswc) in the electroencephalogram, 14-3-3 proteins in the cerebrospinal fluid (csf) and ... | 2004 | 16903140 |
| an outline of the neuropathology of transmissible spongiform encephalopathies (prion diseases). | we review here the basic neuropathology of transmissible spongiform encephalopathies (tse) or prion diseases. the classic hallmark of tse neuropathology is a combination (in different proportions in different diseases) of spongiform change, astrocytosis, neuronal loss and amyloid plaques. immunohistochemically, accumulation of the abnormal isoform of prion protein (prp(sc) or prp(d)) is regarded as a diagnostic for tse. we also review the peculiarities of kuru, variant creutzfeldt-jakob disease ... | 2004 | 16903141 |
| tubulovesicular structures--the ultrastructural hallmark for transmissible spongiform encephalopathies or prion diseases. | tubulovesicular structures (particles--tvs) are the only ultrastructural marker for all transmissible spongiform encephalopathies (tses) or prion disease as seen by thin section electron microscopy. the latter is stressed as opposed to negative-staining techniques. tvs are spheres or short rods of approximately 27-35 nm in diameter. what is particularly interesting, this size of tvs is also the size of filter cut-off as judged from ultrafiltration studies and the size of the smallest infectious ... | 2004 | 16903145 |
| amyloid plaques in transmissible spongiform encephalopathies (prion diseases). | amyloid plaques are encountered in all cases of kuru and gerstmann-straussler-scheinker disease (gss) and in some 10-15% of sporadic creutzfeldt-jakob disease (scjd) cases. in variant creutzfeldt-jakob (vcjd) the particular type of plaque known as "florid" or "daisy" plaque exists in 100% of cases. by electron microscopy several types of amyloid plaque were delineated, corresponding to those seen by prp immunohistochemistry. unicentric "kuru" plaques consisted of stellate arrangements (stars or ... | 2004 | 16903146 |
| [creutzfeldt-jakob disease--the past or the future]. | some recent views on ethiopathogenesis and epidemiology of four main forms of cjd, based on up to-day experiences and expectations for the future, are presented. the sporadic form of the disease (scjd) displays a stable morbidity--ca. 1 case/1 million population yearly. the reasons of its so constant appearance remain still unknown. the familial forms of cjd (fcjd) depending upon more than 40 mutations in prnp gene known today are inherited as autosomal dominant train. the clinical and neuropath ... | 2006 | 16909780 |
| does the surface property of a disposable applanation tonometer account for its underestimation of intraocular pressure when compared with the goldmann tonometer? | disposable tonometers are increasingly being adopted partly because of concerns over the transmission of variant creutzfeldt-jakob disease and partly for convenience. recently, we have found one such tonometer (tonojet by luneau ophthalmologie, france) underestimated the intraocular pressure (iop). | 2007 | 16912886 |
| methods to minimize the risks of creutzfeldt-jakob disease transmission by surgical procedures: where to set the standard? | new prion-related disorders have emerged over the past 20 years, of which the most notable in the human context is variant creutzfeldt-jakob disease (cjd). this disorder is a challenge to medical and public health professionals seeking early detection and diagnosis, provision of therapy, and support for persons affected and a better understanding of transmission risks. the risk of iatrogenic transmission of the disease remains a significant threat, given the well documented cases of cjd transmis ... | 2006 | 16912952 |
| a novel copper-hydrogen peroxide formulation for prion decontamination. | with the appearance of variant creutzfeldt-jakob disease (cjd) and the detection of infectious prions in the peripheral organs of persons with sporadic cjd, the development of decontamination methods that are compatible with medical equipment has become a major issue. here, we show that a formulation of copper metal ions in combination with hydrogen peroxide dramatically reduces the level of prion protein (prp)(sc) (the scrapie isoform of prp) present in homogenates of samples from prion-infecte ... | 2006 | 16941355 |