Publications
| Title | Abstract | Year(sorted ascending) Filter | PMID Filter |
|---|
| 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 |
| 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 |
| 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 |
| creutzfeldt-jakob disease and blood transfusion: results of the uk transfusion medicine epidemiological review study. | this paper reports the results to 1 march 2006 of an ongoing uk study, the transfusion medicine epidemiological review (tmer), by the national cjd surveillance unit (ncjdsu) and the uk blood services (ukbs) to determine whether there is any evidence that creutzfeldt-jakob disease (cjd), including sporadic cjd (scjd), familial cjd (fcjd), and variant cjd (vcjd) is transmissible via blood transfusion. | 2006 | 16958834 |
| 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 |
| 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 |
| 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 |
| 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 |
| 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 |
| 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 |
| 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 |
| 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 |
| 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 |
| 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 |
| 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 |
| radiological assessment of creutzfeldt-jakob disease. | creutzfeldt-jakob disease is a rare fatal neurodegenerative disorder, characterized by rapidly progressive dementia and neurological signs. there is a need for early and accurate clinical diagnosis in order to exclude any treatable disorder. additionally, it is of public interest to differentiate the sporadic form of the disease from the variant cjd type (vcjd), which is probably transmitted from cattle infected with bovine spongiform encephalopathy (bse). high signal in the striatum on t2-weigh ... | 2007 | 17093966 |
| experimental treatments for human transmissible spongiform encephalopathies: is there a role for pentosan polysulfate? | human transmissible spongiform encephalopathies (tses), also known as prion diseases, are caused by the accumulation of an abnormal isoform of the prion protein in the cns. creutzfeldt-jakob disease in its sporadic form is the most frequent type of human tse. at present, there is no proven specific or effective treatment available for any form of tse. pentosan polysulfate (pps) has been shown to prolong the incubation period when administered to the cerebral ventricles in a rodent tse model. cer ... | 2007 | 17477808 |
| neuroimaging findings in human prion disease. | imaging occupies an important role in the investigation of dementia and neurodegenerative disease. the role of imaging in prion disease used to be one of exclusion of other conditions. over the past decade, the non-invasive nature of mri, the improved range of magnetic resonance sequences and the availability of clinical and neuropathological correlation have led to a more prominent position of mri and its inclusion in the diagnostic criteria for variant creutzfeldt-jakob disease. as experience ... | 2007 | 17135459 |
| prion diseases--mysterious persistent infections. | the group of persistent viral infections includes latent, chronic and slow infections. the latter can be caused by: conventional viruses (e.g. polyomavirus jc - causative agent of the progressive multifocal leucoencephalopathy (pml)); defective forms of conventional viruses (e.g. defective measles virus, causing subacute sclerosing panencephalitis (sspe)) and non-conventional infectious agents - prions (proteinaceous infectious particles), causative agents of transmissible spongiform encephalopa ... | 2007 | 17595459 |
| characterization of prion protein (prp)-derived peptides that discriminate full-length prpsc from prpc. | on our initial discovery that prion protein (prp)-derived peptides were capable of capturing the pathogenic prion protein (prp(sc)), we have been interested in how these peptides interact with prp(sc). after screening peptides from the entire human prp sequence, we found two peptides (prp(19-30) and prp(100-111)) capable of binding full-length prp(sc) in plasma, a medium containing a complex mixture of other proteins including a vast excess of the normal prion protein (prp(c)). the limit of dete ... | 2007 | 17601775 |
| in vitro amplification and detection of variant creutzfeldt-jakob disease prpsc. | variant creutzfeldt-jakob disease (vcjd) poses a serious risk of secondary transmission and the need to detect infectivity in asymptomatic individuals is therefore of major importance. following infection, it is assumed that minute amounts of disease-associated prion protein (prp(sc)) replicate by conversion of the host cellular prion protein (prp(c)). therefore, methods of rapidly reproducing this conversion process in vitro would be valuable tools in the development of such tests. we show that ... | 2007 | 17614097 |
| current concepts in human prion protein (prp) misfolding, prnp gene polymorphisms and their contribution to creutzfeldt-jakob disease (cjd). | transmissible spongiform encephalopathies are a group of neural degenerative diseases that may be infectious, sporadic, or hereditary and are associated with an abnormally folded prion protein. unfortunately at the current time it is not at all clear what the normal structure of the prion protein actually is or how it is toxic to cells. extensive research on prion diseases has led to a dramatic increase in understanding of the pathogenesis of prion disorders, which will hopefully lead to the dev ... | 2007 | 17616941 |
| is vaccination against transmissible spongiform encephalopathy feasible? | prion diseases are a unique category of illness, affecting both animals and humans, where the underlying pathogenesis is related to a conformation change of the cellular form of a normal, self-protein called a prion protein (prp(c) [c for cellular]) to a pathological and infectious conformation known as scrapie form (prpsc [sc for scrapie]). currently, all prion diseases are without effective treatment and are universally fatal. the emergence of bovine spongiform encephalopathy and variant creut ... | 2007 | 17633306 |
| detection of misfolded prion protein in blood with conformationally sensitive peptides. | the long-standing goal of a preclinical diagnostic test for transmissible spongiform encephalopathy (tse) has recently become urgent because of the discovery that humans with variant creutzfeldt-jakob disease can transmit disease via blood transfusions. | 2007 | 17655586 |
| normal prion protein trafficking in cultured human erythroblasts. | normal prion protein (prp(c)), an essential substrate for development of prion disease, is widely distributed in hematopoietic cells. recent evidence that variant creutzfeldt-jakob disease can be transmitted by transfusion of red cell preparations has highlighted the need for a greater understanding of the biology of prp(c) in blood and blood-forming tissues. here, we show that in contrast to another glycosylphosphoinositol-anchored protein cd59, prp(c) at the cell surface of cultured human eryt ... | 2007 | 17827389 |
| current risk for transfusion transmitted infections. | blood safety is a topic of continuing concern, and much effort is expended on measures to decrease the risk for transmission of infectious agents via transfusion. at the same time, emerging infections may threaten this safety. a periodic review of risk is therefore appropriate. | 2007 | 17898573 |
| central nervous system tissue in meat products: an evaluation of risk, prevention strategies, and testing procedures. | since the outbreak of bovine spongiform encephalopathy (bse) in the united kingdom in 1986 and its subsequent link to the human neurological disorder variant creutzfeldt-jakob disease (vcjd), presence of tissues from the central nervous system (cns) in meat products has been considered a public health concern and, thus, has been banned from entering the human food chain in many countries. despite this, potential can exist during harvesting to contaminate or cross-contaminate edible meat products ... | 2007 | 17900496 |
| vacuolar degeneration affecting brain macrophages/microglia in variant cjd: a report on two cases. | we present the neuropathology of two cases of variant creutzfeldt-jakob disease (vcjd) showing significant vacuolar degenerative alterations specifically affecting brain macrophages/microglia within the thalamus and, to a lesser extent, within the neocortical grey matter. vacuolar degeneration in these cells was extensive, and likely to be associated with the development of a uniform sub-type of 'spongiform' vacuole seen in vcjd. the extensive morphological alterations described here closely res ... | 2007 | 17943296 |
| therapeutic approaches for prion disorders. | prion diseases are lethal for both humans and animals, and affected individuals die after several months following a rapid disease progression. although researchers have attempted for decades to develop effective therapeutics for the therapy of human prion disorders, until now no efficient drug has been available on the market for transmissible spongiform encephalopathy (tse) treatment or cure. approximately 200 patients worldwide have died or suffer from variant creutzfeldt-jakob disease (cjd). ... | 2007 | 17678425 |
| [acquired creutzfeldt-jakob disease (cjd)--kuru, iatrogenic cjd, variant cjd]. | human prion diseases can be classified as sporadic, hereditary or acquired. the acquired forms are known to be caused by the transmission to human from human or animal, via medical appliances, oral intake or parenteral solutions. usually, peripheral infection such as oral(kuru) or parenteral (human pituitary hormones) transmission causes cerebellar degenerative form, and central nervous system infection such as neurosurgical treatment, dura mater grafts or corneal grafts transmission causes clin ... | 2007 | 17695281 |
| divergent expression of cellular prion protein on blood cells of human and nonhuman primates. | four recent transmissions of variant creutzfeldt-jakob disease infection by transfusion highlight the need for detailed understanding of blood-related prion pathogenesis. nonhuman primates are the most relevant models of human prion diseases. | 2007 | 17714417 |
| the spread of prions through the body in naturally acquired transmissible spongiform encephalopathies. | transmissible spongiform encephalopathies are fatal neurodegenerative diseases that are caused by unconventional pathogens and affect the central nervous system of animals and humans. several different forms of these diseases result from natural infection (i.e. exposure to transmissible spongiform encephalopathy agents or prions, present in the natural environment of the respective host). this holds true also for scrapie in sheep, bovine spongiform encephalopathy in cattle, chronic wasting disea ... | 2007 | 17288548 |
| dental treatment and risk of variant cjd--a case control study. | knowledge of risk factors for variant cjd (vcjd) remains limited, but transmission of prion proteins via re-useable medical devices, including dental instruments, or enhanced susceptibility following trauma to the oral cavity is a concern. this study aimed to identify whether previous dental treatment is a risk factor for development of vcjd. | 2007 | 17299423 |
| bovine spongiform encephalopathy and the safety of milk from canadian dairy cattle. | the detection of bovine spongiform encephalopathy (bse) in beef cattle closed canadian beef export markets to 30 countries, including the usa, with devastating financial losses. the detection and confirmation of the fifth and seventh bse-infected animals but first infected dairy cows extended the problem of risk management to canadian dairy farmers. as the public are concerned about the safety not only of beef but also of milk and milk products that may contain disease-causing prions, this revie ... | 2007 | 17308017 |
| impact of vcjd on blood supply. | variant creutzfeldt-jakob disease (vcjd) is an at present inevitably lethal neurodegenerative disease which can only be diagnosed definitely post mortem. the majority of the approximately 200 victims to date have resided in the uk where most contaminated beef materials entered the food chain. three cases in the uk demonstrated that vcjd can be transmitted by blood transfusion. since bse and vcjd have spread to several countries outside the uk, it appears advisable that specific risk assessments ... | 2007 | 17320412 |
| abnormal prion protein in the pituitary in sporadic and variant creutzfeldt-jakob disease. | by using high-sensitivity western blotting and immunohistochemistry, pituitary glands from patients with sporadic and variant creutzfeldt-jakob disease (scjd and vcjd, respectively) were analysed for the presence of the protease-resistant form of the prion protein (prpres). prpres was detected in a greater proportion of vcjd pituitaries than scjd pituitaries and was localized predominantly in the neurohypophysis. prpres was also detected in a recurrent pituitary adenoma from an scjd patient. imm ... | 2007 | 17325383 |
| prion inactivation by the maillard reaction. | since variant creutzfeldt-jakob disease (vcjd) has been suspected to be attributable to the infectious agents associated with bovine spongiform encephalopathy (bse), it is important to prevent the transmission of pathogenic forms of prion protein (prp(sc)) through contaminated feeding materials such as meat and bone meal (mbm). here, we demonstrate that the maillard reaction employing a formulation of glucose in combination with sodium hydrogen carbonates effectively reduced the infectivity (app ... | 2007 | 17336934 |
| polymorphisms of the prion protein gene coding region in born-after-the-reinforced-ban (barb) bovine spongiform encephalopathy cattle in great britain. | polymorphisms of the prion protein gene are associated with differing susceptibilities to transmissible spongiform encephalopathy diseases, as shown for variant creutzfeldt-jakob disease in humans and scrapie in sheep, but not yet in cattle. imposition of control measures in the uk, including a reinforced ruminant feed ban in 1996, has led to a reduction in the incidence of bovine spongiform encephalopathy (bse). bse-affected cattle born after 1996 in great britain have been termed born-after-th ... | 2007 | 17374784 |
| variant creutzfeldt-jakob disease: first two indigenous cases in republic of ireland. case report and perspective. | the first two cases of indigenous variant creutzfeldt-jakob disease (vcjd) in the republic of ireland are reported in two men, neither of whom had lived outside ireland. both diagnoses were made ante-mortem based on clinical presentation, brain imaging, positive 14-3-3 protein in one case, and tonsillar biopsy. we discuss some of the clinical aspects of vcjd and the significance of these cases in the irish context. | 2007 | 17389001 |
| identification and characterization of two bovine spongiform encephalopathy cases diagnosed in the united states. | bovine spongiform encephalopathy (bse) is a transmissible spongiform encephalopathy of cattle, first detected in 1986 in the united kingdom and subsequently in other countries. it is the most likely cause of variant creutzfeldt-jakob disease (vcjd) in humans, but the origin of bse has not been elucidated so far. this report describes the identification and characterization of two cases of bse diagnosed in the united states. case 1 (december 2003) exhibited spongiform changes in the obex area of ... | 2007 | 17402608 |
| update on human prion disease. | the recognition that variant creutzfeldt-jakob disease (vcjd) is caused by the same prion strain as bovine spongiform encephalopathy in cattle has dramatically highlighted the need for a precise understanding of the molecular biology of human prion diseases. detailed clinical, pathological and molecular data from a large number of human prion disease patients indicate that phenotypic diversity in human prion disease relates in part to the propagation of disease-related prp isoforms with distinct ... | 2007 | 17408929 |
| safety procedures of coagulation factors. | two main types of safety procedures must be applied to biological products, including plasma derivatives: (i) preventive procedures and (ii) elimination procedures. prevention includes epidemiological control of donor populations; checks on each donor's health condition; analysis of each donation for the main pathogens using serological methods; additional analysis of all plasma for human immunodeficiency virus (hiv), hepatitis b virus (hbv), hepatitis c virus (hcv), hepatitis a virus (hav) and ... | 2007 | 18078396 |
| prion protein and the red cell. | this review focuses on transfusion-transmission of variant creutzfeldt-jakob disease by red cell preparations. | 2007 | 17414209 |
| emerging zoonoses and vector-borne infections affecting humans in europe. | the purpose of this study was to assess and describe the current spectrum of emerging zoonoses between 2000 and 2006 in european countries. a computerized search of the medline database from january 1966 to august 2006 for all zoonotic agents in european countries was performed using specific criteria for emergence. fifteen pathogens were identified as emerging in europe from 2000 to august 2006: rickettsiae spp., anaplasma phagocytophilum, borrelia burgdorferi, bartonella spp., francisella tula ... | 2007 | 17445320 |
| the first case of variant creutzfeldt-jakob disease in the netherlands. | 2007 | 17450319 | |
| reference materials for the evaluation of pre-mortem variant creutzfeldt-jakob disease diagnostic assays. | a standard panel of materials is needed for the evaluation of assays being developed for the diagnosis of variant creutzfeldt-jakob disease. | 2007 | 17456154 |
| variant creutzfeldt-jakob disease and the canadian blood system after the tainted blood tragedy. | the transfusion transmission of hepatitis c and hiv to thousands of canadian blood recipients was one of this country's largest public health catastrophes. in response to this crisis, and in an effort to prevent such a tragedy from occurring again, the canadian blood system has undergone substantial reform. variant creutzfeldt-jakob (vcjd) disease was the first infectious threat faced by the blood system since undergoing reform. the response at the time to this risk provides insights into the ca ... | 2007 | 17014945 |
| clinical, neuropathological and immunohistochemical features of sporadic and variant forms of creutzfeldt-jakob disease in the squirrel monkey (saimiri sciureus). | the squirrel monkey (saimiri sciureus) has been shown to be nearly as susceptible as the chimpanzee to experimentally induced creutzfeldt-jakob disease (cjd), and has been used extensively in diagnostic and pathogenetic studies. however, no information is available concerning the clinicopathological characteristics of different strains of human transmissible spongiform encephalopathy (tse) in this species, in particular, strains of sporadic and variant cjd (scjd and vcjd, respectively). brain ho ... | 2007 | 17251588 |
| current strategies to prevent transmission of prions by human plasma derivatives. | protein products prepared from pooled human plasma are an essential class of therapeutics used mostly to control bleeding and/or immunological disorders. because of the human origin of the starting material, there is a risk that these products may possibly transmit prions causing variant creutzfeldt-jakob disease (vcjd). no case of transmission of prions by plasma products has been observed. case-by-case measures implemented in various countries, and several technical factors may contribute, to ... | 2007 | 17254822 |
| recent developments in mucosal vaccines against prion diseases. | bovine spongiform encephalopathy in cattle is highly suspected to be orally transmitted to humans through contaminated food, causing new variant creutzfeldt-jakob disease. however, no prophylactic procedures against these diseases, such as vaccines, in particular those stimulating mucosal protective immunity, have been established. the causative agents of these diseases, termed prions, consist of the host-encoded prion protein (prp). therefore, prions are immunologically tolerated, inducing no h ... | 2007 | 17280480 |
| is there the potential for an epidemic of variant creutzfeldt-jakob disease via blood transfusion in the uk? | the discovery of three individuals suspected to have contracted variant creutzfeldt-jakob disease (vcjd) through blood transfusions has heightened concerns that a secondary epidemic via human-to-human transmission could occur in the uk. the department of health responded immediately to this threat by banning those who had received blood transfusions since 1980 from donating blood. in this paper, we conduct a sensitivity analysis to explore the potential size of a blood-borne vcjd epidemic and in ... | 2007 | 17287181 |
| 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 |
| mortality rates, life expectancy, and causes of death in people with hemophilia a or b in the united kingdom who were not infected with hiv. | since the 1970s, mortality in the hemophilia population has been dominated by human immunodeficiency virus (hiv) and few reports have described mortality in uninfected individuals. this study presents mortality in 6018 people with hemophilia a or b in the united kingdom during 1977 to 1998 who were not infected with hiv, with follow-up until january 1, 2000. given disease severity and factor inhibitor status, all-cause mortality did not differ significantly between hemophilia a and hemophilia b. ... | 2007 | 17446349 |
| time-course studies of 14-3-3 protein isoforms in cerebrospinal fluid and brain of primates after oral or intracerebral infection with bovine spongiform encephalopathy agent. | experimental transmission of bovine spongiform encephalopathy (bse) to cynomolgus monkeys (macaca fascicularis) is an animal model for variant creutzfeldt-jakob disease (vcjd). the presence of 14-3-3 proteins in cerebrospinal fluid (csf) samples indicates neuronal destruction and is therefore used as a clinical biomarker. however, time-course studies using 14-3-3 proteins have not been performed until now in simian vcjd. the main goals of this study were to determine isoform patterns, to examine ... | 2007 | 18024918 |
| creutzfeldt-jakob disease, prion protein gene codon 129vv, and a novel prpsc type in a young british woman. | variant creutzfeldt-jakob disease (vcjd) is an acquired prion disease causally related to bovine spongiform encephalopathy that has occurred predominantly in young adults. all clinical cases studied have been methionine homozygotes at codon 129 of the prion protein gene (prnp) with distinctive neuropathological findings and molecular strain type (prp(sc) type 4). modeling studies in transgenic mice suggest that other prnp genotypes will also be susceptible to infection with bovine spongiform enc ... | 2007 | 18071044 |
| no evidence for association between tau gene haplotypic variants and susceptibility to creutzfeldt-jakob disease. | a polymorphism at codon 129 of the prion protein gene (prnp) is the only well-known genetic risk factor for creutzfeldt-jakob disease (cjd). however, there is increasing evidence that other loci outside the prnp open reading frame might play a role in cjd aetiology as well. | 2007 | 18072964 |
| source of variant creutzfeldt-jakob disease outside united kingdom. | we studied the occurrence of variant creutzfeldt-jakob disease (vcjd) outside the united kingdom in relation to the incidence of indigenous bovine spongiform encephalopathy (bse) and to the level of live bovines and bovine products imported from the uk during the 1980s and the first half of the 1990s. our study provides evidence that a country's number of vcjd cases correlates with the number of live bovines it imported from the uk from 1980 to 1990 (spearman rank correlation coefficient [r(s)] ... | 2007 | 17953086 |
| [human prion diseases: the hungarian experience]. | sporadic creutzfeldt-jakob disease is the most frequent human prion disease. genetic forms are associated with mutations in the human prion protein gene (prnp) and thought to comprise 5-15% of cases. acquired forms include iatrogenic and variant creutzfeldt-jakob disease. the latter is associated with the bovine spongiform encephalopathy. we recently reported the high incidence of genetic creutzfeldt-jakob disease in hungary. | 2007 | 18200749 |
| [prion diseases in japan: analysis of 918 patients]. | the creutzfeldt-jakob disease (cjd) surveillance committee, japan, started in april 1999, and has identified 918 patients with prion diseases until march 2007, including 716 with sporadic cjd (78.0%), 128 with genetic prion diseases (14.0%), and 72 with environmentally acquired prion diseases (7.8%). among atypical cases of sporadic cjd, most common was mm2 type including thalamic and cortical forms. the 128 genetic cases were classified to 42 with cjd with a prp v180i mutation (32.9%), 26 with ... | 2007 | 18210803 |
| a history of kuru. | kuru is placed in its geographic and linguistic setting in the eastern highlands of papua new guinea. the epidemic of kuru has declined over the period 1957 to 2005 from more than 200 deaths a year to 1 or none. since transmission of the kuru prion agent through the mortuary practice of transumption ceased by the early 1960s, the continuation of the epidemic into the present century demonstrates the long incubation periods that are possible in human prion diseases. several histories of kuru are ... | 2007 | 19354007 |
| 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 |
| modeling red cell procurement with both double-red-cell and whole-blood collection and the impact of european travel deferral on units available for transfusion. | in 1997 the fda approved the first double-red-blood-cell (2rbc) collection device. soon after, travel deferral for variant creutzfeldt-jakob disease (vcjd) risk was adopted. to show the importance of including 2rbcs in predictive models of the blood supply, an existing whole-blood (wb) model was updated to include 2rbc collection and then run to simulate the effect of vcjd deferral on total rbc availability. | 2007 | 17958531 |
| size frequency distributions of abnormal protein deposits in alzheimer's disease and variant creutzfeldt-jakob disease. | the size frequency distributions of beta-amyloid (a beta) and prion protein (prpsc) deposits were studied in alzheimer's disease (ad) and the variant form of creutzfeldt-jakob disease (vcjd) respectively. all size distributions were unimodal and positively skewed. a beta deposits reached a greater maximum size and their distributions were significantly less skewed than the prpsc deposits. all distributions were approximately log-normal in shape but only the diffuse prpsc deposits did not deviate ... | 2007 | 17849360 |
| application of atomic dielectric resonance spectroscopy for the screening of blood samples from patients with clinical variant and sporadic cjd. | sub-clinical variant creutzfeldt-jakob disease (vcjd) infection and reports of vcjd transmission through blood transfusion emphasise the need for blood screening assays to ensure the safety of blood and transplanted tissues. most assays aim to detect abnormal prion protein (prpsc), although achieving required sensitivity is a challenge. | 2007 | 17760958 |
| airway management for tonsillectomy: a national survey of uk practice. | the emergence of variant creutzfeldt-jakob disease (vcjd) prompted guidelines from the department of health that stress the use of disposable and protective equipment. this survey explores current methods of airway management for tonsillectomy in the uk and ascertains anaesthetists' current knowledge and opinions of the guidelines and of vcjd. | 2007 | 17596593 |
| measurement of cd83 mrna by real-time polymerase chain reaction to determine removal of dendritic cells by leucoreduction of whole blood. | information is lacking regarding efficiency of removal of circulating dendritic cells (dcs) by leucoreduction (lr) of blood. this is important since dcs may play a role in transporting abnormal prion, the likely infectious agent of variant creutzfeldt-jakob disease. in this study, we report development of a real-time polymerase chain reaction (rt-pcr) assay to quantify residual dcs in lr whole blood via measurement of selected messenger rna (mrna) markers. taqman-based rt-pcr assays were set up ... | 2007 | 17561858 |
| manufacture of plasma-derived products in france and measures to prevent the risk of vcjd transmission: precautionary measures and efficacy of manufacturing processes in prion removal. | the emergence of the variant creutzfeldt-jakob disease in the mid 1990s soon raised concerns about its possible transmission through the use of blood and plasma-derived medicinal products. a risk analysis approach was initiated by health authorities, based on updated scientific knowledge and precautionary measures were implemented in france and other countries for the management of this new possible risk. assessment of the vcjd risk is based on epidemiology and estimates of the number of potenti ... | 2007 | 17540602 |
| neuropathological changes in striate and extrastriate visual cortex in variant creutzfeldt-jakob disease (vcjd). | pathological changes in striate (b17, v1) and extrastriate (b18, v2) visual cortex were studied in variant creutzfeldt-jakob disease (vcjd). no differences in densities of vacuoles or surviving neurons were observed in b17 and b18 but densities of glial cell nuclei and deposits of prion protein (prp) were greater in b18. prp deposit densities in b17 and b18 were positively correlated. diffuse deposit density in b17 was negatively correlated with the density of surviving neurons in b18. the vacuo ... | 2007 | 19668500 |
| blood conservation techniques in obstetrics: a uk perspective. | in the uk, maternal mortality due to haemorrhage appears to be rising, with obstetric haemorrhage accounting for 3-4% of the red cells transfused. allogeneic blood transfusion carries risks such as administration errors, transmitted infections and immunological reactions. the supply of blood is decreasing, partly due to the exclusion of donors who have themselves received a blood transfusion since 1980, in order to stop transmission of variant-creutzfeldt-jakob disease. the cost of blood is sign ... | 2007 | 17509870 |
| the use of single-use devices in anaesthesia: balancing the risks to patient safety. | single-use devices are designed, manufactured and sold to be used once and then discarded. this paper addresses growing concerns about the quality of some devices. single-use devices, manufactured at a lower cost to justify their disposal, are perceived to have a lesser efficacy, which may threaten patient safety through iatrogenic harm. there is, in addition, growing scepticism about the actual risk of contracting variant creutzfeldt-jakob disease and other blood-borne diseases from reused surg ... | 2007 | 17506734 |
| [plasma fractionation in the world: current status]. | from 22 to 25 million liters of plasma are fractionated yearly in about 70 fractionation plants, either private or government-owned, mainly located in industrialized countries, and with a capacity ranging from 50000 to three million liters. in an increasingly global environment, the plasma industry has recently gone through a major consolidation phase that has seen mergers and acquisitions, and has led to the closure of a number of small plants in europe. currently, some fifteen countries are in ... | 2007 | 17499539 |
| blood transfusion and spread of variant creutzfeldt-jakob disease. | variant creutzfeldt-jakob disease (vcjd) may be transmissible by blood. to prevent secondary transmission through blood components, several countries have started to exclude as donors persons who have received a blood transfusion. we investigated the effectiveness of this measure by using a dynamic age-structured model. it is the first such model based on epidemiologic data: 1) blood donor activities, 2) a case-control study on cjd, 3) age distribution of recipients, and 4) death of recipients o ... | 2007 | 17370520 |
| blood-transmitted prions and variant creutzfeldt-jakob disease. | 2007 | 17317213 | |
| long term survival in a patient with variant creutzfeldt-jakob disease treated with intraventricular pentosan polysulphate. | variant creutzfeldt-jakob disease (vcjd) is a neurodegenerative disease that principally affects young people and has a median duration of illness of 13 (range 6-39) months. vcjd is incurable and there are currently no treatments that conclusively slow the rate of disease progression. however, recent animal studies and isolated case reports have suggested that treatment with intraventricular pentosan polysulphate (pps) may be beneficial in the treatment of patients with vcjd. we report a case of ... | 2007 | 17314188 |
| 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 |
| clinical aspects of variant creutzfeldt-jakob disease. | 2008 | 18631793 | |
| review. the neuropathology of kuru and variant creutzfeldt-jakob disease. | a comparison of the pathological profiles of two spongiform encephalopathies with a similar presumptive route of infection was performed. archival kuru and recent variant creutzfeldt-jakob disease (vcjd) cases reveal distinct lesional differences, particularly with respect to prion protein, suggesting that the strain of agent is important in determining the phenotype. genotype analysis of the polymorphism on codon 129 reveals (in conjunction with updated information from more kuru cases) that al ... | 2008 | 18849282 |
| towards modeling of amyloid fibril structures. | amyloid fibrils are associated with a number of debilitating diseases, including alzheimer's disease and variant creutzfeldt-jakob disease. the elucidation of the structure of amyloid fibrils is an important step toward understanding the mechanism of amyloid formation and developing therapeutic agents for amyloid diseases. despite great interests and substantial efforts from various research communities, deriving high-resolution structures of amyloid fibrils remains a challenging problem, due to ... | 2008 | 18508498 |
| two cases of variant creutzfeldt-jakob disease reported in spain in 2007 and 2008. | 2008 | 18445462 | |
| ian mcewan--novels about neurological and psychiatric patients. | ian mcewan, a respected contemporary british writer, sometimes uses neurological and psychiatric patients as main characters of his stories. in his recent novels one can find beautiful descriptions of patients with huntington's disease, variant creutzfeldt-jakob disease, de clerambault syndrome and also details about some neurosurgical procedures. | 2008 | 18437042 |
| advances in the development of a screening test for variant creutzfeldt-jakob disease. | variant creutzfeldt-jakob disease (vcjd) is a transmissible neurodegenerative prion disease that continues to present a unique problem for medical diagnostics. uncertainties remain over the prevalence of vcjd in the uk population and its incubation period in individuals of different genotypes. although the infectious agent that causes vcjd is widely distributed in the peripheral tissues of patients and those carrying the disease, it does not provoke any host immune response that would be amenabl ... | 2008 | 23485140 |
| vision loss due to coincident ocular and central causes in a patient with heidenhain variant creutzfeldt-jakob disease. | creutzfeldt-jakob disease (cjd) is a degenerative disease of the brain associated with a rapidly progressive spongiform encephalopathy. visual symptoms and neuro-ophthalmological signs are not infrequent, and presentation to an ophthalmologist may result. a case is reported of an 89-years-old gentleman who presented with a short history of isolated deterioration in vision. he underwent ocular intervention but subsequently developed progressive dementia, asterixis, myoclonus, cerebellar and extra ... | 2008 | 18065777 |
| systematic review of therapeutic interventions in human prion disease. | the potential threat of a large outbreak of variant creutzfeldt-jakob disease initiated a proliferation of research into the understanding and treatment of human prion disease. however, clinical research is at an early stage with a pressing need for objective evaluation of treatments to inform the design of future studies. | 2008 | 18391159 |
| pathologic prion protein infects cells by lipid-raft dependent macropinocytosis. | transmissible spongiform encephalopathies, including variant-creutzfeldt-jakob disease (vcjd) in humans and bovine spongiform encephalopathies in cattle, are fatal neurodegenerative disorders characterized by protein misfolding of the host cellular prion protein (prp(c)) to the infectious scrapie form (prp(sc)). however, the mechanism that exogenous prp(sc) infects cells and where pathologic conversion of prp(c) to the prp(sc) form occurs remains uncertain. here we report that similar to the mec ... | 2008 | 19390657 |
| association of a null allele of sprn with variant creutzfeldt-jakob disease. | no susceptibility genes have been identified in human prion disase, apart from the prion protein gene (prnp). the gene sprn, encodes shadoo (sho, shadow of prion protein) which has protein homology and possible functional links with the prion protein. | 2008 | 18805828 |
| a clinical study of kuru patients with long incubation periods at the end of the epidemic in papua new guinea. | kuru is so far the principal human epidemic prion disease. while its incidence has steadily declined since the cessation of its route of transmission, endocannibalism, in papua new guinea in the 1950s, the arrival of variant creutzfeldt-jakob disease (vcjd), also thought to be transmitted by dietary prion exposure, has given kuru a new global relevance. we investigated all suspected cases of kuru from july 1996 to june 2004 and identified 11 kuru patients. there were four females and seven males ... | 2008 | 18849289 |
| central and peripheral pathology of kuru: pathological analysis of a recent case and comparison with other forms of human prion disease. | while the neuropathology of kuru is well defined, there are few data concerning the distribution of disease-related prion protein in peripheral tissues. here we report the investigation of brain and peripheral tissues from a kuru patient who died in 2003. neuropathological findings were compared with those seen in classical (sporadic and iatrogenic) creutzfeldt-jakob disease (cjd) and variant cjd (vcjd). the neuropathological findings of the kuru patient showed all the stereotypical changes that ... | 2008 | 18849292 |
| update: creutzfeldt-jakob disease associated with cadaveric dura mater grafts--japan, 1978-2008. | creutzfeldt-jakob disease (cjd) is the most common of the human prion diseases (also known as transmissible spongiform encephalopathies), which, according to the leading hypothesis, are caused by an abnormal protein (i.e., prion) that is able to induce abnormal folding of normal cellular prion proteins. annual worldwide incidence of these always fatal neurodegenerative diseases is estimated at 0.5-2.0 cases per million population. cjd can occur sporadically, or as a genetic disease, or can be tr ... | 2008 | 18946463 |
| bse case associated with prion protein gene mutation. | bovine spongiform encephalopathy (bse) is a transmissible spongiform encephalopathy (tse) of cattle and was first detected in 1986 in the united kingdom. it is the most likely cause of variant creutzfeldt-jakob disease (cjd) in humans. the origin of bse remains an enigma. here we report an h-type bse case associated with the novel mutation e211k within the prion protein gene (prnp). sequence analysis revealed that the animal with h-type bse was heterozygous at prnp nucleotides 631 through 633. a ... | 2008 | 18787697 |
| progress and limits of tse diagnostic tools. | following the two "mad cow" crises of 1996 and 2000, there was an urgent need for rapid and sensitive diagnostic methods to identify animals infected with the bovine spongiform encephalopathy (bse) agent. this stimulated research in the field of prion diagnosis and led to the establishment of numerous so-called "rapid tests" which have been in use in europe since 2001 for monitoring at-risk populations (rendering plants) and animals slaughtered for human consumption (slaughterhouse). these rapid ... | 2008 | 18284910 |
| wernicke's encephalopathy mimicking variant creutzfeldt-jakob disease. | a 45-year-old man from tropical australia was admitted with subacute social withdrawal, cognitive deterioration, reduced awareness and eventual mutism. variant creutzfeldt-jakob disease was considered on the basis of who case definition criteria including typical clinical features and mri showing symmetrical hyperintensity in the pulvinar (posterior) nuclei of the thalami. however, tonsillar biopsy was negative. wernicke's encephalopathy was established on the basis of low serum thiamine on admi ... | 2008 | 18829327 |
| kuru prions and sporadic creutzfeldt-jakob disease prions have equivalent transmission properties in transgenic and wild-type mice. | kuru provides our principal experience of an epidemic human prion disease and primarily affected the fore linguistic group of the eastern highlands of papua new guinea. kuru was transmitted by the practice of consuming dead relatives as a mark of respect and mourning (transumption). to date, detailed information of the prion strain type propagated in kuru has been lacking. here, we directly compare the transmission properties of kuru prions with sporadic, iatrogenic, and variant creutzfeldt-jako ... | 2008 | 18316717 |
| the impact of creutzfeldt-jakob disease on surgical practice. | creutzfeldt-jakob disease (cjd) is characterised by abnormal prion protein that can replicate and replace nervous tissue, with rapid lethal neurodegenerative consequences. the transmissible nature of cjd has been known for half a century and transmission has occurred through neurosurgical procedures. variant creutzfeldt-jakob disease (vcjd) emerged in 1996, and the presence of abnormal prion in lymphatic tissue extended the number of surgical specialties dealing with infected material; transmiss ... | 2008 | 18325202 |
| emerging zoonoses: the challenge for public health and biodefense. | the concept of new and emerging diseases has captured the public interest and has revitalized the public health infectious disease research community. this interest has also resulted in competition for funding and turf wars between animal health and public health scientists and public officials and, in some cases, has delayed and hindered progress toward effective prevention, control and biodefense. there is a dynamic list of outbreaks causing substantial morbidity and mortality in humans and of ... | 2008 | 18378340 |
| the tubulovesicular structures - the ultrastructural hallmark for all prion diseases. | tubulovesicular structures (particles - tvs) are the only ultrastructural marker for all prion diseases as seen by thin-section electron microscopy as opposed to "negative-staining" techniques. tvs are spheres or short rods of approximately 27 nm in diameter. that size of tvs is also the size of filter cut-off of infectivity as judged from the ultrafiltration studies and the size of the smallest infectious unit as recently estimated. tvs have been found in all naturally occurring and experimenta ... | 2008 | 18389022 |