| risks and side effects of therapy with plasma and plasma fractions. | transfusion of plasma can lead to adverse reactions or events. immune-mediated reactions are most common--these include allergic and anaphylactic reactions, transfusion-related acute lung injury (trali) and haemolysis. they can range in severity from mild to fatal. fluid overload and citrate toxicity can occur after rapid or massive transfusion. in developed countries, microbial transmission rates are low because of donor selection and testing. pathogen reduction processes can be applied to eith ... | 2006 | 16377549 |
| raised csf phospho-tau concentrations in variant creutzfeldt-jakob disease: diagnostic and pathological implications. | to investigate whether phosphorylated tau protein (tau-pt181) is increased in variant creutzfeldt-jakob disease (vcjd) and if the tau-pt181/tau protein ratio is useful for distinguishing between patients with and without cjd. | 2006 | 16361602 |
| the risk of transmission of variant creutzfeldt-jakob disease via contact lenses and ophthalmic devices. | this review collated the available information regarding the risk of transmission of variant creutzfeldt-jakob disease (vcjd) via contact lenses and other ophthalmic devices. the topics examined include: the emerging background science of the unconventional infective agent, the prion, particularly those factors affecting transmission; the estimates of the number of undiagnosed infective individuals; and evidence of infectivity in the external eye. despite many uncertainties in the literature, we ... | 2002 | 16303485 |
| gerstmann-sträussler-scheinker disease amyloid protein polymerizes according to the "dock-and-lock" model. | prion protein (prp) amyloid formation is a central feature of genetic and acquired prion diseases such as gerstmann-sträussler-scheinker disease (gss) and variant creutzfeldt-jakob disease. the major component of gss amyloid is a prp fragment spanning residues approximately 82-146, which when synthesized as a peptide, readily forms fibrils featuring gss amyloid. the present study employed surface plasmon resonance (spr) to characterize the binding events underlying prp82-146 oligomerization at t ... | 2006 | 16286452 |
| no clinical evidence of hidden vcjd in uk children. | between may 1997 and november 2004 this national prospective surveillance study identified 1007 children with "progressive intellectual and neurological deterioration" (pind). in most cases specific diagnoses were made, but of 92 undiagnosed children with pind 46 had died and only four underwent full necropsy. there was no clinical evidence of variant creutzfeldt-jakob disease (vcjd) in these undiagnosed cases, but without necropsy it is not possible to exclude vcjd completely. | 2006 | 16260461 |
| variant creutzfeldt-jakob disease 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 |
| an immunoassay for the pathological form of the prion protein based on denaturation and time resolved fluorometry. | concern about the possible secondary spread of variant creutzfeldt-jakob disease (vcjd) through blood transfusion and blood products has increased the need for a sensitive and rapid test for the identification of prp(sc) in specimens collected non-invasively from living persons. furthermore, an accurate estimate of the prevalence of pre-clinical vcjd in the british population would be possible if there were such a test that could be applied to specimens available readily (e.g. blood and urine). ... | 2006 | 16219367 |
| variant creutzfeldt-jakob disease and prions in the blood supply. | | 2004 | 16163166 |
| 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 |
| 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 |
| 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 |
| 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 |
| [progress in the epidemiologic study of variant creutzfeldt-jakob disease]. | | 2003 | 15481134 |
| 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 |
| preclinical vcjd after blood transfusion in a prnp codon 129 heterozygous patient. | we report a case of preclinical variant creutzfeldt-jakob disease (vcjd) in a patient who died from a non-neurological disorder 5 years after receiving a blood transfusion from a donor who subsequently developed vcjd. protease-resistant prion protein (prp(res)) was detected by western blot, paraffin-embedded tissue blot, and immunohistochemistry in the spleen, but not in the brain. immunohistochemistry for prion protein was also positive in a cervical lymph node. the patient was a heterozygote a ... | 2015 | 15302196 |
| 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 |
| the biology and epidemiology of variant creutzfeldt-jakob disease. | | 2003 | 15025265 |
| 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 |
| pathogen inactivation of platelet concentrates and fresh frozen plasma. | transfusion of blood products carries the risk of pathogen transmission, despite careful donor selection and screening tests. this is due to viral transmission from window-period donations, the emergence of new pathogens such as variant creutzfeldt-jakob disease, for which routine screening tests are not yet available, and to bacterial contamination. techniques have been developed to inactivate pathogens in both fresh frozen plasma and platelet concentrates. the relative benefits to the recipien ... | 2003 | 14725341 |
| age and variant creutzfeldt-jakob disease. | the young and stable median age of those who die of variant creutzfeldt-jakob disease has been attributed to age-dependent infection rates. this analysis shows that an influence of age on risk for death after infection better explains age patterns, suggesting that biologic factors peaking in the third decade of life may hasten disease. | 2003 | 14720404 |
| 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 |
| spatial correlation between the vacuolation, prion protein, deposition and surviving neurons in patients with creutzfeldt-jakob disease (vcjd). | the histological features of cases of variant creutzfeldt-jakob disease (vcjd) are often distributed in the brain in clusters. this study investigated the spatial associations between the clusters of the vacuoles, surviving neurons, and prion protein (prp) deposits in various brain areas in 11 cases of vcjd. clusters of vacuoles and surviving neurons were positively correlated in the cerebral cortex but negatively correlated in dentate gyrus. clusters of the florid and diffuse type of prp deposi ... | 2003 | 14628194 |
| prions and disposable surgical instruments. | single-use surgical instruments have been used in the uk's ent departments. this is in response to guidelines issued by the department of health and the british association of otolaryngologists and head and neck surgeons, in order to prevent the spread of new variant creutzfeldt-jakob disease (vcjd) in a young population of patients undergoing routine adenotonsillectomy. in one institution we found that the new single-use instruments were inferior to previous standard kits and that postoperative ... | 2003 | 14627177 |
| minimising the risk of prion transmission by contact tonometry. | the unknown prevalence of variant creutzfeldt-jakob disease (vcjd) in the uk population has led to fears of horizontal transmission through routine medical procedures. the potential risk of transmission via contact tonometry was examined. | 2003 | 14609834 |
| differences in the density and spatial distribution of florid and diffuse plaques in variant creutzfeldt-jakob disease (vcjd). | to determine whether in cases of variant creutzfeldt-jakob disease (vcjd), the florid-type plaques are derived from the diffuse plaques or whether the 2 plaque types develop independently. | 2010 | 14531544 |
| reusable versus disposable instruments in tonsillectomy: a comparative study of outcomes. | tonsillectomy, traditionally performed using reusable instruments (ris), has recently been performed using disposable instruments (dis), to prevent the theoretical risk of spreading new variant creutzfeldt-jakob disease. following this, there have been concerns of increased risk of complications, especially haemorrhage. we audited our hospital's complication rates of 100 tonsillectomies performed using ri and 100 using dis. we analysed the overall complication rates and the postoperative haemorr ... | 2003 | 14529057 |
| the sympathetic nervous system is involved in variant creutzfeldt-jakob disease. | prion epizoonoses spread from animals consumed by humans raise the question of which pathways lead to prion neuroinvasion after oral exposure of humans. here we show that neurons of sympathetic ganglia of patients with variant creutzfeldt-jakob disease (vcjd) accumulate the abnormal isoform of the protein prion. this observation shows the involvement of the sympathetic nervous system in the pathogenesis of vcjd and suggests a role for gut-associated sympathetic neurons in prion propagation in hu ... | 2003 | 12937415 |
| the neuropsychological profile associated with variant creutzfeldt-jakob disease. | we report the neuropsychological profile associated with variant creutzfeldt-jakob disease (vcjd). a retrospective study was carried out of data from neuropsychological reports prepared on 24 patients with vcjd. while there was some variability in neuropsychological profiles, the overall pattern was one of a combined cortical and subcortical dementia, with impaired performance being particularly prominent on tests of memory, executive function, speed of attention, and visuoperceptual reasoning. ... | 2003 | 12937072 |
| variant creutzfeldt-jakob disease and the potential for its accidental transmission following surgery with contaminated instruments: the risk of transmission in australia. | we present a simple model for the quantitative risk assessment of vcjd transmission following surgery. factors that affect the transmission of the disease are prevalence of the disease, concentration of prions in tissues, genetic susceptibility, the number and type of surgical procedures and the effectiveness of decontamination procedures. the main sources of uncertainty are the number of people currently incubating vcjd in australia and the effectiveness of the decontamination processes for sur ... | 2003 | 12862389 |
| first case of variant creutzfeldt-jakob disease in canada. | | 2003 | 12861661 |
| future uncertain for variant creutzfeldt-jakob disease. | | 2002 | 12849414 |
| chorea as a presenting feature of variant creutzfeldt-jakob disease. | we report on a patient with pathologically proven variant creutzfeld-jakob disease in whom chorea was a presenting feature of the disease, and was unaccompanied by the typical prodrome of psychiatric disturbance or sensory symptoms. | 2003 | 12815669 |
| fibreoptic equipment and variant creutzfeldt-jakob disease. | | 2003 | 12790824 |
| screening for variant creutzfeldt-jakob disease. | | 2003 | 12754373 |
| the application of the precautionary principle to the blood system: the canadian blood system's vcjd donor deferral policy. | the precautionary principle is an influential concept that has been widely used in international treaties and declarations involving the protection of the environment. the principle is now being applied to the development of transfusion policy. in this article, we examine the application of the precautionary principle in the policy process leading to canada's decision to defer donations from individuals who had traveled to the united kingdom because of concerns over variant creutzfeldt-jakob dis ... | 2003 | 12733102 |
| post-tonsillectomy haemorrhage: a 7-year retrospective study. | amongst fears about iatrogenic transmission of new variant creutzfeldt-jakob disease (vcjd), last year the department of health of the u.k. advised that all adenotonsillectomies be performed using disposable instruments. following implementation of these guidelines, many consultants reported an increase in post-tonsillectomy haemorrhage in association with the use of disposable instruments. we have undertaken a retrospective study of tonsillectomy and have confirmed a significant increase in the ... | 2003 | 12732932 |
| risk of variant creutzfeldt-jakob disease and tonsillectomy. | | 2003 | 12720005 |
| retention of corneal epithelial cells following goldmann tonometry: implications for cjd risk. | to assess the adequacy of current decontamination methods for the goldmann tonometer in the context of variant creutzfeldt-jakob disease (vcjd). | 2003 | 12714400 |
| adverse events in the prophylaxis of haemophilia. | whilst prophylaxis undoubtedly offers many advantages, the potential for adverse effects must also be borne in mind. modern plasma-derived products have an extremely good safety record with regard to transmission of pathogens, although continuous vigilance is required as new pathogens continue to emerge, eg new variant creutzfeldt-jakob disease. there is no evidence that prophylactic treatment is associated with an increased incidence of inhibitors, and it is now recognized that genetic factors ... | 2003 | 12709038 |
| developments in variant creutzfeldt jakob disease. | the announcement on 17 july 2000 that the rate of increase in the incidence of variant creutzfeldt jakob disease (vcjd) in the united kingdom (uk) had reached statistical significance makes the current issue of eurosurveillance particularly timely. a clus | 2000 | 12631967 |
| detection of residual donor leucocytes in leucoreduced red blood cell components using a fluorescence microplate assay. | in november 1999, universal leucoreduction of blood components was introduced in the uk to minimise the risk of variant creutzfeldt-jakob disease (vcjd) transmission by blood transfusion. the uk specifications for leucodepletion processes state that 99% of leucodepleted components should contain < 5 x 10(6) leucocytes/unit, within 95% confidence limits. however, this leucocyte concentration is below the detection limits of standard haematology analysers. the development of a fluorometric immunoa ... | 2003 | 12609529 |
| hla in french patients with variant creutzfeldt-jakob disease. | | 2003 | 12583973 |
| mhc typing in variant creutzfeldt-jakob disease. | identification of factors that cause susceptibility to, and clinical expression of, variant creutzfeldt-jakob disease (vcjd) is essential for future management of the disease. we established mhc genotypes of 76 individuals with vcjd and 131 controls, and analysed mhc phenotypes in relation to age of onset of vcjd and its duration from presentation to death. there were no significant differences between vcjd and control populations in frequencies of any mhc types, nor were there associations betw ... | 2003 | 12583949 |
| genetic susceptibility to variant creutzfeldt-jakob disease. | | 2003 | 12583940 |
| laminar distribution of the pathological changes in the cerebral cortex in variant creutzfeldt-jakob disease (vcjd). | to determine the pattern of cortical degeneration in cases of variant creutzfeldt-jakob disease (vcjd), the laminar distribution of the vacuolation ("spongiform change"), surviving neurones, glial cell nuclei, and prion protein (prp) deposits was studied in the frontal, parietal and temporal lobes. the vacuolation exhibited two common patterns of distribution: either the vacuoles were present throughout the cortex or a bimodal distribution was present with peaks of density in the upper and lower ... | 2002 | 12572772 |
| mr spectroscopic pulvinar sign in a case of variant creutzfeldt-jakob disease. | we report mr spectroscopic findings in a patient hospitalized with biopsy-proven variant creutzfeldt-jakob (vcjd) disease. n-acetyl aspartate was markedly decreased in the postero-medial part of the thalami (pulvinar) but was not diminished in the parieto-occipital white matter and cortical grey matter. these observations, which are in accordance with the pathological findings in this disease, suggest that mr spectroscopy, a highly sensitive method for the detection of subtle brain metabolic dys ... | 2002 | 12538948 |
| from the centers for disease control and prevention. probable variant creutzfeldt-jakob disease in a us resident--florida, 2002. | | 2002 | 12492096 |
| variant creutzfeldt-jakob disease and disposable anaesthetic equipment-balancing the risks. | | 2003 | 12488368 |
| creutzfeldt-jakob disease in australia 1970-1999. | to ascertain all persons who developed a transmissible spongiform encephalopathy (tse) within australia during the 30-year period 1970 to 1999 through a comprehensive national surveillance program and subject the group to detailed epidemiologic analysis. | 2002 | 12427885 |
| the spatial patterns of prion protein deposits in cases of variant creutzfeldt-jakob disease. | the spatial patterns of the prion protein (prp) deposits were studied in immunostained sections of areas of the cerebral cortex, hippocampus, dentate gyrus, and the molecular layer of the cerebellum in 11 cases of variant creutzfeldt-jakob disease (vcjd). clustering of prp deposits, with a regular distribution of the clusters parallel to the tissue boundary, was the most common spatial pattern observed. two morphological types of prp deposit were recognised, those consisting of a condensed core ... | 2002 | 12410388 |
| irregular presence of abnormal prion protein in appendix in variant creutzfeldt-jakob disease. | | 2002 | 12397162 |
| inline-filtration. | routine leukocyte-depletion (ld) of cellular blood products, and even plasma, is currently being implemented in most european countries, as a result of the fear that the variant creutzfeldt-jakob-disease (vcjd) might be transmissible by transfusion. however, not only is the scientific evidence supporting such a notion scarce, but the benefits of applying this procedure to all patients also remain unfounded. | 2002 | 12350049 |
| kuru: the old epidemic in a new mirror. | the kuru epidemic lasted almost a century; it started in 1901-1902, reached epidemic proportions in the mid-1950s, and disappeared in the 1990s. kuru is the prototype member of a group of disorders known as transmissible spongiform encephalopathies (tses) or prion diseases. recent data on the genetics and pathogenesis of tses contribute to a better understanding of the documented kuru phenomena, and vice versa, observations made during the kuru epidemic are immensely helpful in understanding the ... | 2002 | 12270735 |
| purity of spiking agent affects partitioning of prions in plasma protein purification. | prions are not detectable in the blood or plasma of persons afflicted with classical or variant creutzfeldt-jakob disease, and they have never been shown to be transmitted by blood or plasma products. despite the uncertainty as to the presence and biophysical properties of prions in plasma, prion removal studies have been conducted using brain homogenate or microsomes prepared from prion-infected rodent brains as model prions. in this study, we compare the partitioning of different prion spiking ... | 2002 | 12217343 |
| variant creutzfeldt-jakob disease: costs borne by families. | the objectives of this study were: (1) to estimate the costs borne by families caring for patients with variant creutzfeldt-jakob disease (vcjd); (2) to contextualise results to recent policy initiatives, and (3) to consider the methodological problems of estimating costs of care. semi-structured interviews and a follow-up postal questionnaire, eliciting costs to families both before and after the patient's death, were carried out. participants included 19 families of patients with vcjd. cost pr ... | 2002 | 12121267 |
| vcjd: the epidemic that never was. new variant creutzfeldt-jakob disease: the critique that never was. | | 2002 | 12114248 |
| sensory features of variant creutzfeldt-jakob disease. | sensory symptoms are a prominent feature of variant creutzfeldt-jakob disease (vcjd), occurring at an early stage of the illness. they are persistent and can be troublesome. here, they are described in detail and a possible anatomical basis is discussed. | 2002 | 12111303 |
| quantitative variations in the pathology of 11 cases of variant creutzfeldt-jakob disease (vcjd). | quantitative variations in the density and distribution of the vacuolation ('spongiform change'), surviving neurons, and prion protein (prp) deposits were studied in eight brain regions from 11 cases of variant creutzfeldt-jakob disease (vcjd). principal components analysis (pca) was used to study the similarities and differences between cases and to identify the neuropathological variables which could best account for these variations. two principal components (pc) were extracted from the data ... | 2002 | 12100967 |
| the incubation period of kuru. | kuru is a transmissible spongiform encephalopathy that was identified in papua new guinea in the late 1950s. several thousand cases of the disease occurred during a period of several decades. epidemiologic investigations implicated ritual endocannibalistic funeral feasts as the likely route through which the infectious agent was spread. | 2002 | 12094094 |
| first hundred cases of variant creutzfeldt-jakob disease: retrospective case note review of early psychiatric and neurological features. | to describe the early psychiatric and neurological features of variant creutzfeldt-jakob disease. | 2002 | 12077031 |
| molecular diagnostics of transmissible spongiform encephalopathies. | clinical criteria for the diagnosis of sporadic, iatrogenic and variant creutzfeldt-jakob diseases are now available and show an excellent sensitivity and specificity ( approximately 98%). post-mortem diagnosis, based upon the identification in the brain of the pathological conformer of the prion protein (prp(sc)), is also very accurate, and several diagnostic kits are now available that facilitate the immunochemical measurement of prp(sc). several new molecular diagnostic techniques aimed at in ... | 2002 | 12067613 |
| [diagnosis of prion diseases]. | prion diseases are histologically characterized by a spongiform encephalopathy. the early symptoms of creutzfeldt-jakob disease (cjd) and variant creutzfeldt-jakob disease (vcjd) are unspecific. to date, the definite diagnosis can only be made by histological examination of central nervous tissue, i.e. brain biopsy or post mortem. therefore, diagnostic tests are being elaborated that suffice with cerebrospinal fluid or blood. unlike the sporadic form of creutzfeldt-jakob disease, that involves t ... | 2002 | 12063690 |
| [variant creutzfeldt-jakob disease. epidemiology, detection, diagnosis and prevention with special reference to minimizing risk of iatrogenic transmission by medical products, especially surgical instruments. report of the variant creutzfeldt-jakob disease task force on this topic]. | | 2002 | 12063688 |
| [tonsil biopsy in early diagnosis of variant creutzfeldt-jakob disease]. | | 2002 | 12063685 |
| creutzfeldt-jakob disease: implications for gastroenterology. | the current clinical views regarding variant creutzfeldt-jakob disease, and in particular transmission via endoscopy, of those representing both gastroenterology and the spongiform encephalopathy advisory committee are presented in an attempt to guide clinicians as to "best practice" given the current state of our knowledge. | 2002 | 12010896 |
| clinical features of variant creutzfeldt-jakob disease. | since 1996, over one hundred cases of variant creutzfeldt-jakob disease have appeared, mostly in the united kingdom. in this review, we summarise the major clinical features of this progressive neurodegenerative condition and compare them with those of sporadic creutzfeldt-jakob disease. we emphasise the young age (median 26 years) at presentation and the dominant psychiatric/behavioural features, particularly depression. sensory symptoms are present initially in half the cases and florid psychi ... | 2016 | 11987139 |
| quantification of vacuolation ("spongiform change"), surviving neurones and prion protein deposition in eleven cases of variant creutzfeldt-jakob disease. | vacuolation ("spongiform change") and prion protein (prp) deposition were quantified in the cerebral cortex, hippocampus, dentate gyrus and molecular layer of the cerebellum in 11 cases of variant creutzfeldt-jakob disease (vcjd). the density of vacuoles was greater in the cerebral cortex compared to the hippocampus, dentate gyrus and cerebellum. within the cortex, vacuole density was significantly greater in the occipital compared to the temporal lobe and the density of surviving neurones was g ... | 2002 | 11972799 |
| [considerations of the robert koch institute and its task force on minimizing a) known and b) theoretical risk for transmission of variant creutzfeldt-jakob syndrome by potentially contaminated medical products]. | | 2002 | 11968175 |
| tonsillectomy and variant creutzfeldt-jakob disease. | | 2002 | 11902091 |
| distribution of infectivity in variant creutzfeldt-jakob disease. | | 2002 | 11888628 |
| evaluation of the validity and reliability of a-scan ultrasound biometry with a single use disposable cover. | the uk medical devices agency has suggested that ophthalmic practitioners should, where practicable and not compromising clinical outcome, restrict corneal contact devices to single patient use to minimise a remote theoretical risk of transmission of new variant creutzfeldt-jakob disease (vcjd). this study reports on a modified technique of ultrasound a-scan biometry that complies with the mda recommendations. | 2002 | 11864896 |
| variant creutzfeldt-jakob disease. | | 2002 | 11861680 |
| the use of antioxidants in transmissible spongiform encephalopathies: a case report. | transmissible spongiform encephalopathies (tse), which include creutzfeldt-jakob disease and new variant creutzfeldt-jakob disease, are diseases characterized by progressive deterioration in the central nervous system with neuronal degeneration, vacuolatization of the neuropil, and gliosis. little is known about the pathogenic mechanisms of infection, and controversy exits around the inciting infective agent. it has been shown that an important factor in pathogenesis is the immune system. | 2002 | 11838883 |
| classical and variant creutzfeldt-jakob diseases and their potential impact on the practice of clinical dentistry in australia. | following recent published evidence regarding the experimental transmission of prion diseases via blood transfusion, dental practitioners have expressed their concern about the potential impact of these transmissible spongiform encephalopathies on dental care provision. this review provides updated information on creutzfeldt-jakob disease and related disorders and highlights their potential significance for the practice of clinical dentistry. the current guidelines in australia relating to infec ... | 2001 | 11838871 |
| intestinal entry of prions. | variant creutzfeldt-jakob disease is thought to be caused by infected prion protein via ingestion of contaminated beef. after ingestion of infected prion proteins, uptake by small intestine may be by either m-cell dependent or m-cell independent routes. a receptor for prion protein, laminin receptor precursor is expressed on the brush border of small intestinal epithelium in 40 % of subjects. the cellular prion protein expressed on the enteric nervous system might serve as the target for convers ... | 2002 | 11803499 |
| variant creutzfeldt-jakob disease (vcjd). | | 2001 | 11797239 |
| this is really a case of new variant creutzfeldt-jakob. response to c.j.g. lang et al. concerning our article acta neuropathol (2000) 99:704-708. | | 2001 | 11761727 |
| evaluation of performance of white blood cell reduction filters: an original flow cytometric method for detection and quantification of cell-derived membrane fragments. | contamination of blood products by white blood cells leads to a risk of transmission of infectious agents, particularly abnormal prion protein, the probable causative agent of new-variant creutzfeldt-jakob disease. blood product filtration could reduce this risk, but the filtration systems might generate potentially infectious membrane fragments. we developed an original flow cytometric method that allows the detection and quantification of membrane fragments in filtered products and the evaluat ... | 2001 | 11746097 |
| has the variant creutzfeldt-jakob disease epidemic hit its peak? | | 2001 | 11705496 |
| ultrastructural analysis of the florid plaque in variant creutzfeldt-jakob disease. | we report here the first description of florid plaques--the hallmark of variant creutzfeldt-jakob disease (vcjd). these plaques are composed of broad bundles of amyloid, are highly neuritic and exhibited astrocytes and microglial cells. collectively, they are more similar to neuritic plaques of alzheimer's disease than to kuru plaques of kuru--creutzfeldt-jakob disease--gerstmann-sträussler-sheinker disease. | 2000 | 11693720 |
| variant creutzfeldt-jakob disease--information from the world health organization. | | 2001 | 11605332 |
| variant creutzfeldt-jakob disease in hong kong. | a 34-year-old chinese woman who had lived in the united kingdom in the 1980s was admitted to hospital in hong kong because of a 7-month history of progressive neurological deterioration. initially, she complained of heartburn and paraesthesia of the hands and feet. she then developed slowness of speech and gait, and was noted to be forgetful and irritable. in january 2001, she was brought back to hong kong for treatment. on admission in may she was dysarthric, ataxic, and dystonic. magnetic reso ... | 2001 | 11590272 |
| new variant creutzfeldt-jakob disease presenting with loss of taste and smell. | | 2001 | 11534516 |
| old drugs to treat new variant creutzfeldt-jakob disease. | | 2001 | 11520533 |