Publications
| Title | Abstract | Year(sorted ascending) Filter | PMID Filter |
|---|
| decontamination of medical devices: legislation and compliance to practice. | the amount of legislation and guidelines published on decontamination reflects the current attitude of politicians and the general public to the desire to reduce the risk of infection. incidents, such as bovine spongiform encephalopathy (bse) in cattle with the subsequent risk of transmission of variant creutzfeldt-jakob disease (vcjd), require greater compliance by healthcare workers to the guidance both in the hospital and the wider community. the change in care provision and the demands of go ... | 2004 | 15549014 |
| advances in screening test development for transmissible spongiform encephalopathies. | the blood of patients with transmissible spongiform encephalopathy or prion disease can no longer be considered free of infectivity. there have been two recent reports of highly probable transfusion-associated iatrogenic variant creutzfeldt-jakob disease infections, and there is supporting experimental evidence of scrapie transmission by the transfusion of blood from sheep with naturally occurring disease. in the absence of a preclinical diagnostic test for transmissible spongiform encephalopath ... | 2004 | 15566331 |
| social and environmental risk factors in the emergence of infectious diseases. | fifty years ago, the age-old scourge of infectious disease was receding in the developed world in response to improved public health measures, while the advent of antibiotics, better vaccines, insecticides and improved surveillance held the promise of eradicating residual problems. by the late twentieth century, however, an increase in the emergence and re-emergence of infectious diseases was evident in many parts of the world. this upturn looms as the fourth major transition in human-microbe re ... | 2004 | 15577934 |
| effect of protease treatment on plasma infectivity in variant creutzfeldt-jakob disease mice. | the emergence of variant creutzfeldt-jakob disease (vcjd) and of a probable transmission of the disease through blood transfusion from a presymptomatic case has underlined the need for a reliable, sensitive, and specific screening test. this study was initiated to explain why attempts to identify protease-resistant prion protein (prpres) following treatment with proteinase k (pk) in blood or blood components have so far failed. | 2004 | 15584983 |
| protease-resistant human prion protein and ferritin are cotransported across caco-2 epithelial cells: implications for species barrier in prion uptake from the intestine. | foodborne transmission of bovine spongiform encephalopathy (bse) to humans as variant creutzfeldt-jakob disease (cjd) has affected over 100 individuals, and probably millions of others have been exposed to bse-contaminated food substances. despite these obvious public health concerns, surprisingly little is known about the mechanism by which prp-scrapie (prp(sc)), the most reliable surrogate marker of infection in bse-contaminated food, crosses the human intestinal epithelial cell barrier. here ... | 2004 | 15601934 |
| the modern landscape of transfusion-related iatrogenic creutzfeldt-jakob disease and blood screening tests. | the idea that blood in naturally occurring transmissible spongiform encephalopathies is not infectious has imploded in the face of recent transmissions from the blood of naturally occurring scrapie in sheep and of variant creutzfeldt-jakob disease in humans. although donor exclusion criteria ensure that the number of any further iatrogenic cases will be small, the risk of future blood-borne disease transmissions could be entirely eliminated by a diagnostic preclinical screening test. a variety o ... | 2004 | 15666660 |
| variant creutzfeldt-jakob disease and the acquired and transmissible spongiform encephalopathies. | in 1995 a fatal epidemic of spongiform encephalopathy appeared in great britain. the new epidemic condition was clinically and pathologically similar to creutzfeldt-jakob disease (cjd), a rare sporadic encephalopathy, and was called "variant cjd" (vcjd). the vcjd epidemic was detected by active epidemiologic surveillance, which had been set up in response to a british epizootic of another new disease called "bovine spongiform encephalopathy" (bse). widespread cattle exposure to the bse agent (a ... | 2004 | 14986255 |
| creutzfeldt-jakob disease in ireland: epidemiological aspects 1980-2002. | surveillance for creutzfeldt-jakob disease (cjd) has been carried out in the republic of ireland since 1980. initial surveillance was passive and based on consented autopsy confirmation of cjd in patients in whom there was a high index of clinical suspicion. since 1999, an active surveillance programme involving formal notification of all suspect cjd cases has been in place. the annual mortality rate has increased from 0.34 cases/million in 1980 to 1.27 cases/million in 2001. in all, 29 cases ha ... | 2004 | 14988606 |
| specificity of lymphoreticular accumulation of prion protein for variant creutzfeldt-jakob disease. | immunocytochemical accumulation of prion protein (prp) in lymphoid tissues is a feature of variant creutzfeldt-jakob disease (vcjd) that has been used both to aid in the diagnosis of patients and as a basis of large scale screening studies to assess the prevalence of preclinical disease in the uk. however, the specificity of this approach is unknown. | 2004 | 14990604 |
| [variant creutzfeldt-jakob disease]. | 2004 | 15011360 | |
| distribution of a bovine spongiform encephalopathy-derived agent over ion-exchange chromatography used in the preparation of concentrates of fibrinogen and factor viii. | the risk of haemophiliacs contracting variant creutzfeldt-jakob disease (vcjd) via treatment with factor viii concentrates is not known. therefore, in order to determine the extent to which the vcjd agent might be removed during the preparation of factor viii concentrate, the partitioning of a bovine spongiform encephalopathy (bse)-derived agent was measured over the main purification step used to prepare the scottish national blood transfusion service high-purity factor viii concentrate (libera ... | 2004 | 15023177 |
| leucodepletion for transmissible spongiform encephalopathies. | transmissible spongiform encephalopathies (tses) have been recognised around the world for many years. creutzfeldt-jakob disease (cjd), one of the human forms of tse, has been studied widely and thus far has not proved a great threat to human health. the emergence of two new tses--bovine spongiform encephalopathy (bse) in cattle and variant creutzfeldt-jakob disease (vcjd) in humans in the uk--has caused great concern. bse has had an economic impact and vcjd is a threat to human health. it has b ... | 2004 | 15058746 |
| singled out? | the increasing use of single use medical devices is being driven by a growing awareness of iatrogenic (from the greek; caused by the doctor) and nosocomial infections. public health perceptions relating to transmissible spongiform encephalopathies, specifically variant creutzfeldt-jakob disease (vcjd), the human immunodeficiency virus (hiv) and hepatitis b are high on the political agenda and a matter of concern to healthcare professionals. | 2004 | 15060930 |
| proceedings of a consensus conference: the screening of blood donors for variant cjd. | since the identification, in 1996, of the first case of variant creutzfeldt-jakob disease (vcjd) in humans various approaches have been implemented and/or proposed to prevent this disease from being transfusion transmitted. in addition, a variety of possible laboratory-based approaches have been developed and will continue to be developed for the vcjd screening of blood donors. various issues related to the implementation of such vcjd testing is likely to assume greater importance as diagnostic ... | 2004 | 15067588 |
| aspects of risk assessment and risk management of nosocomial transmission of classical and variant creutzfeldt-jakob disease with special attention to german regulations. | 2004 | 15077406 | |
| technical aspects of the development and validation of tests for variant creutzfeldt-jakob disease in blood transfusion. | the development of tests for variant creutzfeldt-jakob disease in the context of blood transfusion is technically complicated by a number of factors, including the long asymptomatic period and uncertainty as to whether infectivity is present in human blood at all. the specific needs of a donor test impose constraints. it is argued that the only possible analyte will be blood, and that while the initial work will involve animal studies, these will provide only an approximate guide. a rapid infect ... | 2004 | 15078250 |
| silent prions lying in wait: a two-hit model of prion/amyloid formation and infection. | diseases such as type 2 diabetes, alzheimer's and parkinson's are associated with the formation of amyloid. the transmissible spongiform encephalopathies, such as variant creutzfeldt-jakob disease, are believed to result from infectious forms of amyloid proteins termed prions. the ability of amyloid to initiate spontaneously and in the case of prions, to transfer successfully from one host to another, has been hard to fully rationalize. in this paper we use a mathematical model to explore the id ... | 2004 | 15095987 |
| estimating blood donor loss due to the variant cjd travel deferral. | the fda recommended new travel deferrals in may 2002 to prevent the potential transmission of variant cjd (vcjd). the predicted impact of such deferrals on the blood supply was controversial. | 2004 | 15104643 |
| mammalian brain consumption by blood donors in the united states: brains today, deferred tomorrow? | theoretical concerns of possible variant cjd (vcjd) transmission by transfusion have led to deferral of us donors potentially exposed to the bovine spongiform encephalopathy agent. although the efficacy of these policies is unknown, impact on blood collections has been substantial. under the precautionary principle, deferral of donors consuming bovine (or other mam-malian) brains, possibly contaminated with the vcjd agent, might be considered. blood donors were surveyed to determine lifetime mam ... | 2004 | 15104646 |
| current concepts and controversies in prion immunopathology. | scrapie in sheep and new variant creutzfeldt-jakob disease in humans are typically initiated by extracerebral exposure to prions. both exhibit early prion accumulation in sites of the peripheral lymphoreticular system, such as splenic or lymph nodal germinal centers. in germinal centers, follicular dendritic cells (fdcs), whose development and maintenance depend on lymphotoxin and tumor necrosis factor signaling, are believed to be the main cell type for efficient prion replication in the periph ... | 2004 | 15126687 |
| methionine 129 variant of human prion protein oligomerizes more rapidly than the valine 129 variant: implications for disease susceptibility to creutzfeldt-jakob disease. | the human prp gene (prnp) has two common alleles that encode either methionine or valine at codon 129. this polymorphism modulates disease susceptibility and phenotype of human transmissible spongiform encyphalopathies, but the molecular mechanism by which these effects are mediated remains unclear. here, we compared the misfolding pathway that leads to the formation of beta-sheet-rich oligomeric isoforms of the methionine 129 variant of prp to that of the valine 129 variant. we provide evidence ... | 2004 | 15131108 |
| molecular diagnostic tools in creutzfeldt-jakob disease and other prion disorders. | clinical criteria and cerebrospinal fluid biomarkers for the diagnosis of human prion diseases (sporadic, iatrogenic or variant creutzfeldt-jakob disease and genetic inherited transmissible spongiform encephalopathies) are now widely available and show a sensitivity and specificity of approximately 98%. final diagnosis of prion diseases is obtained by post-mortem examination upon identification of the pathological conformer of the prion protein (prpsc) in the brain. several diagnostic kits are n ... | 2004 | 15137902 |
| polymorphisms of the prion protein gene (prnp) in a korean population. | human prion protein gene (prnp) has been considered to be involved in the susceptibility of humans to prion diseases. polymorphisms of methionine (met)/valine (val) at codon 129 and of glutamic acid (glu)/lysine (lys) at codon 219 are thought to play an important role in susceptibility to sporadic, iatrogenic and variant creutzfeldt-jakob disease (cjd). although the genotype distribution of polymorphisms in prnp open reading frame (orf) has been reported in many european populations, among asian ... | 2004 | 15148589 |
| clinical features of variant creutzfeldt-jakob disease. | the possibility that a new form of human prion disease, variant creutzfeldt-jakob disease (vcjd) had occurred in the uk was first raised by the identification of a small number of cases with unusual clinical characteristics. atypical features included a young age at death, a predominantly psychiatric presentation, a relatively extended duration of illness and the absence of the 'typical' periodic electroencephalogram seen in sporadic cjd. diagnostic criteria for vcjd have now been formulated and ... | 2004 | 15148990 |
| neuropathology and molecular biology of variant creutzfeldt-jakob disease. | the neuropathological features of human prion diseases are spongiform change, neuronal loss, astrocytic proliferation and the accumulation of prp(sc), the abnormal isoform of prion protein (prp). the pattern of brain involvement is remarkably variable and is substantially influenced by the host prp genotype and prp(sc) isotype. variant creutzfeldt-jakob disease (vcjd) is a novel human prion disease which results from exposure to the bovine spongiform encephalopathy (bse) agent. the neuropatholog ... | 2004 | 15148991 |
| the epidemiology of variant creutzfeldt-jakob disease. | variant creutzfeldt-jakob disease (vcjd) was identified as a new disease in 1996. it was linked to infection with the bovine spongiform encephalopathy (bse) agent although the epidemiological evidence for this was not strong, but later strain typing studies confirmed the association. the disease has affected predominantly young adults whose dietary and other characteristics are unexceptional compared to control groups, other than that all patients to date have been methoinine homozygous at codon ... | 2004 | 15148992 |
| molecular aspects of disease pathogenesis in the transmissible spongiform encephalopathies. | the transmissible spongiform encephalopathy (tse) diseases are a group of rare, fatal, and transmissible neurodegenerative diseases that include kuru and creutzfeldt-jakob disease (cjd) in humans, scrapie in sheep, transmissible mink encephalopathy (tme), and chronic wasting disease (cwd) in mule deer and elk. over the last 20 yr, they have gone from a fascinating but relatively obscure group of diseases to one that is a major agricultural and economic problem as well as a threat to human health ... | 2004 | 15156065 |
| prpsc accumulation in myocytes from sheep incubating natural scrapie. | because variant creutzfeldt-jakob disease (vcjd) in humans probably results from consumption of products contaminated with tissue from animals with bovine spongiform encephalopathy, whether infectious prion protein is present in ruminant muscles is a crucial question. here we show that experimentally and naturally scrapie-affected sheep accumulate the prion protein prp(sc) in a myocyte subset. in naturally infected sheep, prp(sc) is detectable in muscle several months before clinical disease ons ... | 2004 | 15156203 |
| resistance to scrapie in prp arr/arq heterozygous sheep is not caused by preferential allelic use. | in sheep, susceptibility to scrapie, which is similar to human prion diseases such as kuru and variant creutzfeldt-jakob disease (vcjd), is determined by prion protein (prp) gene (prnp) polymorphisms. sheep with genotype arq/arq, denoting polymorphisms at codons 136, 154, and 171, are susceptible, whereas those with genotypes arr/arq and arr/arr are resistant, indicating dominance of arr over the arq allele. | 2004 | 15166274 |
| prion protein heterogeneity in sporadic but not variant creutzfeldt-jakob disease: uk cases 1991-2002. | human prion diseases can occur as an idiopathic disorder (sporadic creutzfeldt-jakob disease) or can be acquired, as is the case for variant creutzfeldt-jakob disease. these disorders are characterized by the accumulation of a protease-resistant form of the host-encoded prion protein termed prp(sc) in the brains of affected individuals. prp(sc) has been proposed to be the principal, if not sole, component of the infectious agent, with its accumulation in the central nervous system the primary ev ... | 2004 | 15174020 |
| vaccines for conformational disorders. | neurodegenerative disorders are becoming increasingly common and an ever greater healthcare burden, as the average age in western populations rises. many of these are conformational disorders, which are characterized by the accumulation of a host protein that undergoes a structural change increasing its beta-sheet content, rendering it toxic. the most common of these illnesses is alzheimer's disease. prion diseases are also conformational disorders, which are currently less common than alzheimer ... | 2004 | 15176944 |
| why aren't we more ahead? the risk of variant creutzfeldt-jakob disease from eating bovine spongiform encephalopathy-infected foods: still undetermined. | 2004 | 15180097 | |
| the unrecognised french bse epidemic. | in france, implementation of systematic screening programs in 2000, as a complement to the mandatory reporting of animals with clinical signs of bse (passive surveillance), revealed certain limitations of the mandatory system. indeed, systematic screening showed that some bse cases were not detected by the clinical surveillance system, implying considerable bse case under-reporting throughout the epidemic. as the most likely explanation for variant creutzfeldt-jakob disease (vcjd) is exposure to ... | 2004 | 15210083 |
| prevalence of lymphoreticular prion protein accumulation in uk tissue samples. | this study aims to provide an estimate of the number of individuals in the uk who may be incubating variant creutzfeldt-jakob disease and at risk of causing iatrogenic spread of the disease. lymphoreticular accumulation of prion protein is a consistent feature of variant creutzfeldt-jakob at autopsy and has also been demonstrated in the pre-clinical phase. immunohistochemical accumulation of prion protein in the lymphoreticular system remains the only technique that has been shown to predict neu ... | 2004 | 15221931 |
| informed consent for blood transfusion: should the possibility of prion risk be included? | the emergence of bovine spongiform encephalopathy (bse) in british cattle has received significant media attention since its discovery in 1986. transmission of this prion from cattle to humans has been documented, and the bse prion is believed to be the causative agent for variant creutzfeldt-jakob disease (vcjd). evidence of this spread is a significant threat to public health, and, although there has never been a proven case, there is a theoretical risk of transmission between humans by blood ... | 2004 | 15248167 |
| comparison of demographic and donation profiles and transfusion-transmissible disease markers and risk rates in previously transfused and nontransfused blood donors. | increasing concern about transfusion transmission of variant creutzfeldt-jakob disease has resulted in indefinite deferral of transfused donors in france and the uk. little is known, however, about the impact of indefinite deferral of transfused donors on blood safety and availability in the us. | 2004 | 15265131 |
| prion diseases: update on mad cow disease, variant creutzfeldt-jakob disease, and the transmissible spongiform encephalopathies. | transmissible spongiform encephalopathies (tses) are a group of progressive, fatal neurodegenerative disorders that share a common spongiform histopathology. tses may be transmitted in a sporadic, familial, iatrogenic, or zoonotic fashion. the putative infectious agent of tse, the prion, represents a novel paradigm of infectious disease with disease transmission in the absence of nucleic acid. several small but spectacular epidemics of tses in man have prompted widespread public health and food ... | 2004 | 15265460 |
| targeting prion amyloid deposits in vivo. | the diagnosis of prion diseases in humans is challenging due to a lack of specific and sensitive non-invasive tests. many forms of human prion disease including variant creutzfeldt-jakob disease (vcjd), gerstmann-sträussler-scheinker (gss) syndrome, and 10% of sporadic cjd cases are associated with amyloid deposition. several positron emission tomography (pet) ligands have recently been developed to directly image beta-amyloid associated with alzheimer disease. one of them, methoxy-x04, is a flu ... | 2004 | 15290902 |
| bovine spongiform encephalopathy and creutzfeldt-jakob disease: facts and uncertainties underlying the causal link between animal and human diseases. | following an outbreak of bovine spongiform encephalopathy (bse) in dairy cows in the united kingdom (uk), 153 definite and probable human cases of new variant creutzfeldt-jakob disease (nvcjd) have been reported, almost exclusively in the uk. although exposure to the bse agent is the most plausible interpretation for the occurrence of nvcjd, the causal link between the bse prion and nvcjd is still debated. this review discusses the pros and cons of nvcjd as a separate nosographic entity, the sci ... | 2004 | 15300459 |
| novel methods for disinfection of prion-contaminated medical devices. | the unique resistance of prions to classic methods of decontamination, and evidence that prion diseases can be transmitted iatrogenically by medical devices pose a serious infection control challenge to health-care facilities. in view of the widespread tissue distribution of the variant creutzfeldt-jakob disease agent in human beings, new practicable decontamination procedures are urgently needed. | 2004 | 15302195 |
| effectiveness of leucoreduction for removal of infectivity of transmissible spongiform encephalopathies from blood. | in 1999, the uk implemented universal leucoreduction as a precaution against transmission of variant creutzfeldt-jakob disease by transfusion of domestic blood or red blood cells. we aimed to assess how effectively leucoreduction reduced infectivity of transmissible spongiform encephalopathies (tses) in blood. 450 ml of whole blood collected and pooled from scrapie-infected hamsters was leucoreduced with a commercial filter. blood cell concentrations were quantified, and infectivity titres measu ... | 2004 | 15302197 |
| advances in the detection of prion protein in peripheral tissues of variant creutzfeldt-jakob disease patients using paraffin-embedded tissue blotting. | the accumulation of prp(sc), an abnormal and disease-associated form of the normal prion protein (prp(c)), within the central nervous system (cns) is a key pathological feature of creutzfeldt-jakob disease (cjd). following limited proteolytic digestion of prp(sc), the detection of prp(res) within lymphoid tissues is a unique characteristic of variant cjd in comparison with other human prion diseases, raising fears of an increased risk of iatrogenic spread. because levels of prp(res) in lymphoid ... | 2004 | 15305981 |
| the risk of accidental transmission of transmissible spongiform encephalopathy: identification of emerging issues. | the transmissible spongiform encephalopathies (tses), thought to be caused by prions, are fatal neurodegenerative disorders of humans and animals. despite their rarity, human prion diseases have received prominence because the consumption of prion-contaminated meat from cattle with bovine spongiform encephalopathy (bse) is thought to be responsible for the emergence of variant creutzfeldt-jakob disease (vcjd) in humans. clinical criteria for the diagnosis of vcjd is now available. recent, more s ... | 2004 | 15313594 |
| emerging infections in transfusion medicine. | the risk of transfusion-transmitted infectious diseases (ttids) has declined dramatically in high-income nations over the past 2 decades, primarily because of extraordinary success in preventing hiv and other established transfusion-transmitted viruses from entering the blood supply. despite this achievement, ttids remain a public health concern, and attention is refocusing on new and emerging pathogens, such as west nile virus, infectious proteins (the presumed cause of variant creutzfeldt-jako ... | 2004 | 15325065 |
| autophagy is a part of ultrastructural synaptic pathology in creutzfeldt-jakob disease: a brain biopsy study. | ultrastructural correlates of synaptic and dendritic spines loss have never been studied in detail in human transmissible spongiform encephalopathies (tses)-creutzfeldt-jakob disease (cjd), gerstmann-sträussler-scheinker (gss) disease and fatal familial insomnia (ffi). in this paper, we describe synaptic alterations as found in brain biopsies from creutzfeldt-jakob disease and fatal familial insomnia patients. our material consisted of brain biopsies obtained by open surgery from one ffi case, o ... | 2004 | 15325593 |
| genetic studies in relation to kuru: an overview. | kuru is a subacute neurodegenerative disease presenting with limb ataxia, dysarthria, and a shivering tremor. the disease progress to complete motor and mental incapacity and death within 6 to 24 months. neuropathologically, a typical pattern of neuronal loss, astrocytic and microglial proliferation, characteristic "kuru-type" amyloid plaques, and prp deposits in the cerebral cortex and cerebellum are observed. kuru is the prototype of a group of human transmissible spongiform encephalopathies ( ... | 2004 | 15354868 |
| prion diseases and the spleen. | transmissible spongiform encephalopathies are fatal neurodegenerative disorders that include creutzfeldt-jakob disease in humans, bovine spongiform encephalopathy and scrapie in sheep and goats. transmissible spongiform encephalopathies are thought by some to result from changes in the conformation of a membrane glycoprotein called prpc (prion protein) into a pathogenic form, prpsc, which constitutes the major component of an unprecedented type of infectious particle supposedly devoid of nucleic ... | 2004 | 15357900 |
| predictors of survival in sporadic creutzfeldt-jakob disease and other human transmissible spongiform encephalopathies. | a collaborative study of human transmissible spongiform encephalopathies has been carried out from 1993 to 2000 and includes data from 10 national registries, the majority in western europe. in this study, we present analyses of predictors of survival in sporadic (n = 2304), iatrogenic (n = 106) and variant creutzfeldt-jakob disease (n = 86) and in cases associated with mutations of the prion protein gene (n = 278), including gerstmann-sträussler-scheinker syndrome (n = 24) and fatal familial in ... | 2004 | 15361416 |
| practical aspects of decontamination of the unconventional transmissible agents that cause sporadic and variant creutzfeldt-jakob disease and other similar human diseases. | although the unconventional agents that cause transmissible degenerative encephalopathies have not yet been completely characterised, they are known to be relatively resistant to decontamination procedures that are effective with conventional microorganisms. the implications for the safe decontamination and sterilisation of devices and instruments used in human medicine are discussed. | 2004 | 15448715 |
| bovine spongiform encephalopathy (bse): the end of the beginning or the beginning of the end? | bovine spongiform encephalopathy (bse) is a zoonosis being the origin of variant creutzfeldt-jakob disease and an important cattle disease in its own right. this association has driven both the research into the disease and extensive epidemiological investigations of practical value. not only has the occurrence of bse has a serious effect on animal health and public health, it has also seriously interrupted trade in cattle and cattle products from affected countries. since 2001, several addition ... | 2004 | 15449460 |
| variant creutzfeldt-jakob disease. | current evidence indicates that variant creutzfeldt-jakob disease is caused by the transmission of bovine spongiform encephalopathy to humans. the clinical and investigative features of variant cjd are relatively distinct from sporadic cjd and the neuropathological appearances are novel. the number of cases of vcjd in the uk may have peaked, but the total future number of cases of vcjd is uncertain and the possibility of secondary iatrogenic transmission via blood transfusion has recently been i ... | 2004 | 15449462 |
| review: pathology of variant creutzfeldt-jakob disease. | variant creutzfeldt-jakob disease (vcjd) is a novel human prion disease that results from exposure to the bovine spongiform encephalopathy (bse) agent, probably by the oral route. the pathological features of vcjd are unique, with extensive involvement of lymphoid tissues in addition to the central nervous system. this article reviews the histopathology and biochemistry of vcjd, emphasising diagnostic features and indicating several areas of active research. the widespread distribution of infect ... | 2004 | 15449463 |
| analysis of 2000 consecutive uk tonsillectomy specimens for disease-related prion protein. | variant creutzfeldt-jakob disease (cjd) is thought to be caused by dietary or other exposure to bovine spongiform encephalopathy (bse) prions. the prevalence of preclinical or subclinical prion infection in the uk is currently unknown. since clinical variant cjd is uniformly associated with tonsillar prion infection, we screened 2000 anonymous surgical tonsillectomy specimens for disease-associated prion protein. analysis by both high sensitivity immunoblotting and immunohistochemistry detected ... | 2004 | 15464187 |
| bse and vcjd cause disturbance to uric acid levels. | bovine spongiform encephalopathy (bse) and variant creutzfeldt-jakob disease (vcjd) are two new members of the family of neurodegenerative conditions termed prion diseases. oxidative damage has been shown to occur in prion diseases and is potentially responsible for the rapid neurodegeneration that is central to the pathogenesis of these diseases. an important nonenzymatic antioxidant in the brain is uric acid. analysis of uric acid in the brain and cerebrospinal fluid (csf) of cases of bse and ... | 2004 | 15473996 |
| attributable testing for abnormal prion protein, database linkage, and blood-borne vcjd risks. | context: national prospective collection of tonsillar tissue to be tested anonymously for abnormal lymphoreticular accumulation of prion protein (prp) was approved to begin in the uk in 2004. the uk is not, however, testing autopsy specimens attributably for abnormal prp (prp(sc)) so that recipients at risk after a blood transfusion from, or exposed to surgical instruments from, a deceased carrier of variant creutzfeldt-jakob disease (vcjd) can be followed up to quantify transmission risks. in s ... | 2004 | 15474140 |
| variant creutzfeldt-jakob disease: risk of transmission by blood and blood products. | variant creutzfeldt-jakob disease (cjd) is a novel acquired human prion disease apparently resulting from exposure to the bovine spongiform encephalopathy (bse) agent. variant cjd differs from other human prion diseases in that the disease-associated form of the prion protein and infectivity are readily detectable in lymphoid tissues throughout the body. lymphoid tissues and lymphocytes are implicated in the peripheral pathogenesis of prion diseases (where infectivity may be detected during the ... | 2004 | 15479374 |
| peripheral tissue involvement in sporadic, iatrogenic, and variant creutzfeldt-jakob disease: an immunohistochemical, quantitative, and biochemical study. | human prion diseases are rare fatal neurodegenerative conditions that occur as acquired, familial, or idiopathic disorders. a key event in their pathogenesis is the accumulation of an altered form of the prion protein, termed prp(sc), in the central nervous system. a novel acquired human prion disease, variant creutzfeldt-jakob disease, is thought to result from oral exposure to the bovine spongiform encephalopathy agent. this disease differs from other human prion diseases in its neurological, ... | 2004 | 14695328 |
| distinct molecular phenotypes in bovine prion diseases. | bovine spongiform encephalopathy (bse) in cattle, the most likely cause of variant creutzfeldt-jakob disease in humans, is thought to be caused by a unique infectious agent, with stable features, even when transmitted to other species. here, we show the existence of an atypical molecular phenotype among cattle diagnosed with bse in france. following western blot analysis, three cases showed unusual features of the electrophoretic profiles of the protease-resistant prion protein (prp(res)) accumu ... | 2004 | 14710195 |
| bovine spongiform encephalopathy in a dairy cow--washington state, 2003. | on december 23, 2003, the u.s. department of agriculture (usda) made a preliminary diagnosis of bovine spongiform encephalopathy (bse) in a single "downer" (i.e., nonambulatory disabled) dairy cow in washington state. on december 25, this diagnosis was confirmed by the bse international reference laboratory in weybridge, england. this report summarizes the findings of the initial investigation of this case and describes the public health prevention measures adopted by usda to protect the human f ... | 2004 | 14712176 |
| [infectious diseases-part i: epidemiology]. | the outstanding issue regarding recent trends in the epidemiology of infectious diseases is the epidemic occurrence of severe acute respiratory syndrome (sars) in 2003. sars is caused by a novel coronavirus, presumably originating from wild cats. this new agent was rapidly identified and characterized, the outbreak was terminated in early summer 2003 after implementation of strict infectious control measures. especially the high rate of complications and nosocomial infections has caused severe p ... | 2004 | 14716483 |
| 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 |
| an overview of transmissible spongiform encephalopathies. | transmissible spongiform encephalopathies (tses) are fatal neurodegenerative disorders of humans and animals associated with an accumulation of abnormal isoforms of prion protein (prp) in nerve cells. the pathogenesis of tses involves conformational conversions of normal cellular prp (prp(c)) to abnormal isoforms of prp (prp(sc)). while the protein-only hypothesis has been widely accepted as a causal mechanism of prion diseases, evidence from more recent research suggests a possible involvement ... | 2004 | 15984319 |
| bovine spongiform encephalopathy and variant creutzfeldt-jakob disease. | 2004 | 15992264 | |
| variations in neurodegenerative disease across the uk: findings from the national study of progressive intellectual and neurological deterioration (pind). | to identify any uk children with variant creutzfeldt-jakob disease (vcjd) and obtain information about the causes of progressive intellectual and neurological deterioration (pind) and the geographical distribution of cases. | 2004 | 14709491 |
| the pulvinar sign in variant creutzfeldt-jakob disease. | 2004 | 15023827 | |
| mimicry of variant creutzfeldt-jakob disease by sporadic creutzfeldt-jakob disease: importance of the pulvinar sign. | 2004 | 15023826 | |
| the success of precaution? managing the risk of transfusion transmission of variant creutzfeldt-jakob disease. | the precautionary principle has emerged as an important new paradigm influencing decision making in the blood system. the principle has influenced decision making in several nations leading to the institution of policies to protect their blood supplies form variant creutzfeldt-jakob disease (vcjd). increasingly evidence has emerged to support the institution of these policies, which were introduced in advance of clear evidence of risk. these vcjd decisions serve as an example of the successful a ... | 2004 | 15383021 |
| quantifying losses to the donated blood supply due to donor deferral and miscollection. | donors are deferred for multiple reasons. losses related to disease marker rates are well established. donor and donation losses for other reasons, however, have not been extensively quantified. | 2004 | 15383013 |
| surveillance for progressive intellectual and neurological deterioration in the canadian paediatric population. | to conduct active surveillance of the canadian paediatric population for children who have a progressive intellectual and neurological deterioration to detect the occurrence of cases of creutzfeldt-jakob disease or variant creutzfeldt-jakob disease. | 2004 | 15198447 |
| does a perception of increased blood safety mean increased blood transfusion? an assessment of the risk compensation theory in canada. | the risk compensation theory is a widely used concept in transport economics to analyze driver risk behaviour. this article explores the feasibility of applying the theory in blood transfusion to raise important questions regarding the increased blood safety measures and their possible effects on blood usage (e.g., the appropriateness in transfusion). further, it presents the findings of a pilot survey of physicians in canada. | 2004 | 15182381 |
| is variant creutzfeldt-jakob disease in young children misdiagnosed as alpers' syndrome? an analysis of a national surveillance study. | there has been concern that children with variant creutzfeldt-jakob disease (vcjd) might be misdiagnosed as cases of alpers' syndrome, as a spongiform degeneration of the brain is seen in both conditions. | 2004 | 15146014 |
| mechanism of intestinal entry of infectious prion protein in the pathogenesis of variant creutzfeldt-jakob disease. | the pathogenesis of variant creutzfeldt-jakob disease (vcjd) is most likely to be dependent on intestinal entry of orally ingested infectious prion proteins, though tonsils or other oral portals of entry are possible. the exact route of entry of infectious prion proteins is uncertain but receptors for prion proteins such as laminin receptor precursor (lrp) may be expressed on intestinal brush border. cellular prion protein (prp(c)) is expressed on intestinal enteric nervous system and is separat ... | 2004 | 15063598 |
| variant creutzfeldt-jakob disease: between lymphoid organs and brain. | prion diseases are often caused by peripheral uptake of the infectious agent. to reach their ultimate target, the central nervous system (cns), prions enter their host, replicate in lymphoid organs and spread via peripheral nerves. once the agent has reached the cns disease progression is rapid, resulting in neurodegeneration and death. many of these mechanisms have been uncovered using genetically modified mice. a recently published study demonstrated the presence of pathological prion protein ... | 2004 | 15040321 |
| variant creutzfeldt-jakob disease and prions in the blood supply. | 2004 | 16163166 | |
| improved infection control in the prevention of variant creutzfeldt-jakob disease in australia: costs and benefits. | to evaluate the costs and benefits of infection control strategies to prevent the transmission of variant creutzfeldt-jakob disease (vcjd) in ophthalmic surgery in australia. | 2004 | 15707207 |
| [informing the transfused patient of the possible transmission of variant creutzfeldt-jakob disease by blood transfusion]. | true risks and theoretical risks: the texts ruling the obligation of the physician to inform the patient appears not to include theoretical risks in their application. in france however, the field of blood transfusions extends this obligation to the theoretical risk of potential transmission through the blood of the infectious agent responsible for creutzfeldt-jakob's disease. | 2004 | 15637795 |
| joint ash and aabb educational session. | in the vein-to-vein flow of blood from donor to patient, the role of the transfusion medicine specialist has become increasingly centered at the bedside. three clinically centered issues in blood safety and in blood conservation are presented in this chapter. in section i, dr. patricia hewitt presents the epidemiologic and clinical evidence regarding new variant creutzfeldt-jakob disease (nvcjd) in the uk and its relevance to transfusion medicine. lessons learned from the responses by the nation ... | 2004 | 15561698 |
| anaesthesia in patients with dementia. | the next couple of decades will be characterized by an increase in life expectancy, leading to an older population. as the incidence of alzheimer's dementia and vascular dementia is rising with age, the future anaesthesiologist will be increasingly confronted with perioperative care of patients with impaired cognitive function. this paper tries to highlight some topics specifically related to demented patients. | 2004 | 17021564 |
| 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 |
| 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 |
| 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 |
| a response to 'lymphocyte contamination of laryngoscope blades--a possible vector for transmission of variant creutzfeldt-jakob disease'. | 2005 | 16229718 | |
| american fresh frozen plasma for neonates and children. | from the spring of 2004 the united kingdom blood services have been importing fresh frozen plasma from united states donors for all neonates and children born after 1 january 1996. the decision to mandate the use of american plasma in this age group was taken by the department of health in 2002 as part of its precautionary approach to the risk of transfusion transmitted variant creutzfeldt-jakob disease. in this article we explain the background to this decision and explore some of the implicati ... | 2005 | 15613525 |
| variant creutzfeldt-jakob disease: update. | 2005 | 15591527 | |
| high levels of disease related prion protein in the ileum in variant creutzfeldt-jakob disease. | 2005 | 16162963 | |
| 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 |
| 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 |
| leucoreduction and variant creutzfeldt-jakob disease. | 2005 | 16101813 | |
| lymphocyte contamination of laryngoscope blades--a possible vector for transmission of variant creutzfeldt-jakob disease. | variant creutzfeldt-jakob disease (vcjd) is associated with extensive prion infection of lymphoreticular tissues during the prolonged asymptomatic incubation period. instruments exposed to infected tissues of preclinically infected individuals during medical or surgical procedures represent a potential risk of iatrogenic transmission of vcjd prions. we assessed the frequency of contamination with lymphoid tissue of single-use laryngoscope blades used for tracheal intubation for general anaesthes ... | 2005 | 15960716 |
| the "pulvinar sign" in a case of paraneoplastic limbic encephalitis associated with non-hodgkin's lymphoma. | this paper reports a 59 year old woman with paraneoplastic limbic encephalitis associated with diffuse large b cell lymphoma. her brain magnetic resonance imaging scan showed bilateral posterior thalamic hyperintensities, similar to the "pulvinar sign". her symptoms included progressive psychiatric disturbance and resembled the initial symptoms of variant creutzfeldt-jakob disease (vcjd). clinicians should consider this treatable disorder in the differential diagnosis of vcjd. | 2005 | 15897519 |
| variation in concentration of prion protein in the peripheral blood of patients with variant and sporadic creutzfeldt-jakob disease detected by dissociation enhanced lanthanide fluoroimmunoassay and flow cytometry. | a highly sensitive dissociation-enhanced lanthanide fluoroimmunoassay (delfia) and flow cytometry techniques have previously been developed and employed to characterize soluble cellular prion protein (prp(c)) expression in whole blood and separated components from healthy adult blood donors. no previous studies with these techniques have evaluated the concentration and expression of prp in the blood of patients with variant creutzfeldt-jakob disease (vcjd). | 2005 | 15819670 |
| size frequency distribution of prion protein (prp) aggregates in variant creutzfeldt-jakob disease (vcjd). | the frequency distribution of aggregate size of the diffuse and florid-type prion protein (prp) plaques was studied in various brain regions in cases of variant creutzfeldt-jakob disease (vcjd). the size distributions were unimodal and positively skewed and resembled those of beta-amyloid (a beta) deposits in alzheimer's disease (ad) and down's syndrome (ds). the frequency distributions of the prp aggregates were log-normal in shape, but there were deviations from the expected number of plaques ... | 2005 | 15785857 |
| 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 |
| 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 |
| [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 |
| 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 |