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https://www.readbyqxmd.com/read/28444687/creutzfeldt-jakob-disease-lookback-study-21-years-of-surveillance-for-transfusion-transmission-risk
#1
Lauren A Crowder, Lawrence B Schonberger, Roger Y Dodd, Whitney R Steele
BACKGROUND: Transfusion transmission of human prion diseases has been observed for variant Creutzfeldt-Jakob disease (vCJD), but not for the classic forms of prion disease (CJD: sporadic, genetic, and iatrogenic). Although the presence of prions or misfolded prion proteins in blood has been documented in some patients with the most common form of CJD, sporadic CJD, no transfusion-transmitted cases of CJD have been recognized. Since 1995, the American Red Cross has conducted a lookback study of the recipients of blood products from donors who develop CJD to assess the risk of blood-borne CJD transmission in the United States...
April 25, 2017: Transfusion
https://www.readbyqxmd.com/read/28332471/cjd-surveillance-in-the-republic-of-ireland-from-2005-to-2015-a-suggested-algorithm-for-referrals
#2
Teresa Loftus, Daphne Chen, Seamus Looby, Albi Chalissery, Rachel Howley, Ciara Heaney, Josephine Heffernan, Michael Farrell, Francesca Brett
Definitive diagnosis of Creutzfeldt Jakob disease (CJD) remains tissue-based. Possible and probable CJD are useful clinical terms but may be used indiscriminately. The aim of this study was to assess the effectiveness of the Irish surveillance system and to ascertain how diagnostic accuracy in identifying clinically "definite" cases might be improved. We reviewed the clinical information, relevant investigations, and samples n = 100; (autopsy n = 87; biopsy n = 13) in 96 patients between January 1, 2005 and December 31, 2015...
March 23, 2017: Clinical Neuropathology
https://www.readbyqxmd.com/read/28261810/geographic-exposure-risk-of-variant-creutzfeldt-jakob-disease-in-us-blood-donors-a-risk-ranking-model-to-evaluate-alternative-donor-deferral-policies
#3
Hong Yang, Yin Huang, Luisa Gregori, David M Asher, Travis Bui, Richard A Forshee, Steven A Anderson
BACKGROUND: Variant Creutzfeldt-Jakob disease (vCJD) has been transmitted by blood transfusion (TTvCJD). The US Food and Drug Administration (FDA) recommends deferring blood donors who resided in or traveled to 30 European countries where they may have been exposed to bovine spongiform encephalopathy (BSE) through beef consumption. Those recommendations warrant re-evaluation, because new cases of BSE and vCJD have markedly abated. STUDY DESIGN AND METHODS: The FDA developed a risk-ranking model to calculate the geographic vCJD risk using country-specific case rates and person-years of exposure of US blood donors...
March 5, 2017: Transfusion
https://www.readbyqxmd.com/read/28258683/-patient-with-creutzfeld-jakob-disease-a-case-report
#4
Żanna Pastuszak, Kazimierz Tomczykiewicz, Adam Stępień, Renata Piusińska-Macoch, Joanna Klimczuk, Agnieszka Rolewska, Dariusz Galbarczyk
Creutzfeldt-Jakob disease (CJD) is a rare syndrome of central nervous system caused by infectious protein called prion. There are four types of CJD: sporadic (sCJD), familial (fCJD), jatrogenic (jCJD) and variant (vCJD). The most frequent symptoms are rapidly progressing dementia, mioclonias, akinetic mutism and signs of cerebellum dysfunction. In sCJD, MRI often shows high signal intensity in the putamen and caudate nucleus on T2-weighted images while in vCJD pulvinar sign is often observed. 70% patients with CJD often has characteristic generalized periodic sharp wave pattern in electroencephalography...
February 20, 2017: Polski Merkuriusz Lekarski: Organ Polskiego Towarzystwa Lekarskiego
https://www.readbyqxmd.com/read/28231300/detection-and-partial-discrimination-of-atypical-and-classical-bovine-spongiform-encephalopathies-in-cattle-and-primates-using-real-time-quaking-induced-conversion-assay
#5
Etienne Levavasseur, Anne-Gaëlle Biacabe, Emmanuel Comoy, Audrey Culeux, Katarina Grznarova, Nicolas Privat, Steve Simoneau, Benoit Flan, Véronique Sazdovitch, Danielle Seilhean, Thierry Baron, Stéphane Haïk
The transmission of classical bovine spongiform encephalopathy (C-BSE) through contaminated meat product consumption is responsible for variant Creutzfeldt-Jakob disease (vCJD) in humans. More recent and atypical forms of BSE (L-BSE and H-BSE) have been identified in cattle since the C-BSE epidemic. Their low incidence and advanced age of onset are compatible with a sporadic origin, as are most cases of Creutzfeldt-Jakob disease (CJD) in humans. Transmissions studies in primates and transgenic mice expressing a human prion protein (PrP) indicated that atypical forms of BSE may be associated with a higher zoonotic potential than classical BSE, and require particular attention for public health...
2017: PloS One
https://www.readbyqxmd.com/read/28193766/molecular-mechanisms-of-chronic-wasting-disease-prion-propagation
#6
Julie A Moreno, Glenn C Telling
Prion disease epidemics, which have been unpredictable recurrences, are of significant concern for animal and human health. Examples include kuru, once the leading cause of death among the Fore people in Papua New Guinea and caused by mortuary feasting; bovine spongiform encephalopathy (BSE) and its subsequent transmission to humans in the form of variant Creutzfeldt-Jakob disease (vCJD), and repeated examples of large-scale prion disease epidemics in animals caused by contaminated vaccines. The etiology of chronic wasting disease (CWD), a relatively new and burgeoning prion epidemic in deer, elk, and moose (members of the cervid family), is more enigmatic...
February 13, 2017: Cold Spring Harbor Perspectives in Medicine
https://www.readbyqxmd.com/read/28151391/variant-creutzfeldt-jakob-disease-deferral-in-canada-impact-of-stop-dates
#7
Sheila F O'brien, Wenli Fan, Qi-Long Yi, Samra Uzicanin, Lori Osmond, Mindy Goldman
BACKGROUND: To reduce the risk of variant Creutzfeldt-Jakob disease (vCJD) transmission via blood transfusion in Canada, potential donors who spent a cumulative time in the United Kingdom, Western Europe or Saudi Arabia are deferred. "Stop dates" for accumulated time were later implemented for 3 months spent in the United Kingdom or France (1980-1996) and for 5 years elsewhere in Western Europe (1980-2007); Saudi Arabia deferral was implemented with the "stop date" (1980-1996). We evaluated the long-term impact of these deferrals and "stop dates", as well as the consistency of donors' answers to post-implementation screening questions...
December 15, 2016: Blood Transfusion, Trasfusione del Sangue
https://www.readbyqxmd.com/read/28003548/detection-of-prions-in-blood-from-patients-with-variant-creutzfeldt-jakob-disease
#8
Luis Concha-Marambio, Sandra Pritzkow, Fabio Moda, Fabrizio Tagliavini, James W Ironside, Paul E Schulz, Claudio Soto
Human prion diseases are infectious and invariably fatal neurodegenerative diseases. They include sporadic Creutzfeldt-Jakob disease (sCJD), the most common form, and variant CJD (vCJD), which is caused by interspecies transmission of prions from cattle infected by bovine spongiform encephalopathy. Development of a biochemical assay for the sensitive, specific, early, and noninvasive detection of prions (PrP(Sc)) in the blood of patients affected by prion disease is a top medical priority to increase the safety of the blood supply...
December 21, 2016: Science Translational Medicine
https://www.readbyqxmd.com/read/28003547/detection-of-prions-in-the-plasma-of-presymptomatic-and-symptomatic-patients-with-variant-creutzfeldt-jakob-disease
#9
Daisy Bougard, Jean-Philippe Brandel, Maxime Bélondrade, Vincent Béringue, Christiane Segarra, Hervé Fleury, Jean-Louis Laplanche, Charly Mayran, Simon Nicot, Alison Green, Arlette Welaratne, David Narbey, Chantal Fournier-Wirth, Richard Knight, Robert Will, Pierre Tiberghien, Stéphane Haïk, Joliette Coste
Variant Creutzfeldt-Jakob disease (vCJD) is a human prion disease resulting from the consumption of meat products contaminated by the agent causing bovine spongiform encephalopathy. Evidence supporting the presence of a population of silent carriers that can potentially transmit the disease through blood transfusion is increasing. The development of a blood-screening assay for both symptomatic vCJD patients and asymptomatic carriers is urgently required. We show that a diagnostic assay combining plasminogen-bead capture and protein misfolding cyclic amplification (PMCA) technologies consistently detected minute amounts of abnormal prion protein from French and British vCJD cases in the required femtomolar range...
December 21, 2016: Science Translational Medicine
https://www.readbyqxmd.com/read/27699415/diagnosing-sporadic-creutzfeldt-jakob-disease-by-the-detection-of-abnormal-prion-protein-in-patient-urine
#10
Connie Luk, Samantha Jones, Claire Thomas, Nick C Fox, Tze H Mok, Simon Mead, John Collinge, Graham S Jackson
Importance: Creutzfeldt-Jakob disease (CJD) is a fatal neurodegenerative disorder associated with the accumulation of infectious abnormal prion protein through a mechanism of templated misfolding. A recent report has described the detection of abnormal prion protein in the urine of patients with variant CJD (vCJD) using protein misfolding by cyclic amplification, which was apparently absent in the more common sporadic form of CJD (sCJD). A noninvasive diagnostic test could improve early diagnosis of sCJD and, by screening donations, mitigate the potential risks of prion transmission through human urine-derived pharmaceuticals...
December 1, 2016: JAMA Neurology
https://www.readbyqxmd.com/read/27499183/are-prions-transported-by-plasma-exosomes
#11
Larisa Cervenakova, Paula Saá, Oksana Yakovleva, Irina Vasilyeva, Jorge de Castro, Paul Brown, Roger Dodd
Blood has been shown to contain disease-associated misfolded prion protein (PrP(TSE)) in animals naturally and experimentally infected with various transmissible spongiform encephalopathy (TSE) agents, and in humans infected with variant Creutzfeldt-Jakob disease (vCJD). Recently, we have demonstrated PrP(TSE) in extracellular vesicle preparations (EVs) containing exosomes from plasma of mice infected with mouse-adapted vCJD by Protein Misfolding Cyclic Amplification (PMCA). Here we report the detection of PrP(TSE) by PMCA in EVs from plasma of mice infected with Fukuoka-1 (FU), an isolate from a Gerstmann-Sträussler-Scheinker disease patient...
August 2016: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/27440899/guinea-pig-prion-protein-supports-rapid-propagation-of-bovine-spongiform-encephalopathy-and-variant-creutzfeldt-jakob-disease-prions
#12
Joel C Watts, Kurt Giles, Daniel J Saltzberg, Brittany N Dugger, Smita Patel, Abby Oehler, Sumita Bhardwaj, Andrej Sali, Stanley B Prusiner
The biochemical and neuropathological properties of bovine spongiform encephalopathy (BSE) and variant Creutzfeldt-Jakob disease (vCJD) prions are faithfully maintained upon transmission to guinea pigs. However, primary and secondary transmissions of BSE and vCJD in guinea pigs result in long incubation periods of ∼450 and ∼350 days, respectively. To determine if the incubation periods of BSE and vCJD prions could be shortened, we generated transgenic (Tg) mice expressing guinea pig prion protein (GPPrP)...
November 1, 2016: Journal of Virology
https://www.readbyqxmd.com/read/27432362/ten-year-follow-up-of-two-cohorts-with-an-increased-risk-of-variant-cjd-donors-to-individuals-who-later-developed-variant-cjd-and-other-recipients-of-these-at-risk-donors
#13
M Checchi, P E Hewitt, P Bennett, H J T Ward, R G Will, J M Mackenzie, K Sinka
BACKGROUND: Transmission of variant Creutzfeldt-Jakob disease (vCJD) through blood transfusion is implicated in three deaths and one asymptomatic infection. Based on this evidence, individuals assessed to be at increased risk of vCJD through donating blood transfused to individuals who later developed vCJD, or through being other recipients of such donors, are followed up to further understand the risks of vCJD transmission through blood. OBJECTIVES: To provide a ten-year follow-up of these at-risk cohorts...
November 2016: Vox Sanguinis
https://www.readbyqxmd.com/read/27129755/clinical-issues-may-2016
#14
Sharon A Van Wicklin
Variations in documenting surgical wound classification Key words: surgical wound classification, clean, clean-contaminated, contaminated, dirty. Wearing long-sleeved jackets while preparing and packaging items for sterilization Key words: long-sleeved jackets, organic material, sterile processing. Endoscopic transmission of prions Key words: prions, high-risk tissue, low-risk tissue, Creutzfeldt-Jakob disease (CJD), variant Creutzfeldt-Jakob disease (vCJD). Wearing gloves when handling flexible endoscopes Key words: gloves, low-protein, powder-free, natural rubber latex gloves, latex-free gloves...
May 2016: AORN Journal
https://www.readbyqxmd.com/read/27110695/persistent-residual-contamination-in-endoscope-channels-a-fluorescence-epimicroscopy-study
#15
Rodolphe C Hervé, Charles W Keevil
BACKGROUND AND STUDY AIMS: The increasing demand for endoscopic procedures poses new contamination challenges, given developing antimicrobial resistance worldwide and potential viral or prion diseases in populations at risk. We examined working channels from reusable luminal endoscopes used in recent years. METHODS: Very sensitive fluorescence epimicroscopy was used to examine working channels from 6 decommissioned and 6 factory-new channels, as received, or following spiking and washing in the laboratory...
July 2016: Endoscopy
https://www.readbyqxmd.com/read/27073865/whole-blood-gene-expression-profiling-in-preclinical-and-clinical-cattle-infected-with-atypical-bovine-spongiform-encephalopathy
#16
Elena Xerxa, Maura Barbisin, Maria Novella Chieppa, Helena Krmac, Elena Vallino Costassa, Paolo Vatta, Marion Simmons, Maria Caramelli, Cristina Casalone, Cristiano Corona, Giuseppe Legname
Prion diseases, such as bovine spongiform encephalopathies (BSE), are transmissible neurodegenerative disorders affecting humans and a wide variety of mammals. Variant Creutzfeldt-Jakob disease (vCJD), a prion disease in humans, has been linked to exposure to BSE prions. This classical BSE (cBSE) is now rapidly disappearing as a result of appropriate measures to control animal feeding. Besides cBSE, two atypical forms (named H- and L-type BSE) have recently been described in Europe, Japan, and North America...
2016: PloS One
https://www.readbyqxmd.com/read/27055460/variants-of-plcxd3-are-not-associated-with-variant-or-sporadic-creutzfeldt-jakob-disease-in-a-large-international-study
#17
Rubika Balendra, James Uphill, Claire Collinson, Ronald Druyeh, Gary Adamson, Holger Hummerich, Inga Zerr, Pierluigi Gambetti, John Collinge, Simon Mead
BACKGROUND: Human prion diseases are relentlessly progressive neurodegenerative disorders which include sporadic Creutzfeldt-Jakob disease (sCJD) and variant CJD (vCJD). Aside from variants of the prion protein gene (PRNP) replicated association at genome-wide levels of significance has proven elusive. A recent association study identified variants in or near to the PLCXD3 gene locus as strong disease risk factors in multiple human prion diseases. This study claimed the first non-PRNP locus to be highly significantly associated with prion disease in genomic studies...
April 7, 2016: BMC Medical Genetics
https://www.readbyqxmd.com/read/26800081/rapid-and-highly-sensitive-detection-of-variant-creutzfeldt-jakob-disease-abnormal-prion-protein-on-steel-surfaces-by-protein-misfolding-cyclic-amplification-application-to-prion-decontamination-studies
#18
Maxime Belondrade, Simon Nicot, Vincent Béringue, Joliette Coste, Sylvain Lehmann, Daisy Bougard
The prevalence of variant Creutzfeldt-Jakob disease (vCJD) in the population remains uncertain, although it has been estimated that 1 in 2000 people in the United Kingdom are positive for abnormal prion protein (PrPTSE) by a recent survey of archived appendix tissues. The prominent lymphotropism of vCJD prions raises the possibility that some surgical procedures may be at risk of iatrogenic vCJD transmission in healthcare facilities. It is therefore vital that decontamination procedures applied to medical devices before their reprocessing are thoroughly validated...
2016: PloS One
https://www.readbyqxmd.com/read/26764000/mononucleated-blood-cell-populations-display-different-abilities-to-transmit-prion-disease-by-the-transfusion-route
#19
Jean-Yves Douet, Caroline Lacroux, Claire Litaise, Séverine Lugan, Fabien Corbière, Mark Arnold, Hugh Simmons, Naima Aron, Pierrette Costes, Cécile Tillier, Hervé Cassard, Olivier Andréoletti
UNLABELLED: Previous experiments carried out in a sheep scrapie model demonstrated that the transfusion of 200 μl of prion-infected whole blood has an apparent 100% efficacy for disease transmission. These experiments also indicated that, despite the apparent low infectious titer, the intravenous administration of white blood cells (WBC) resulted in efficient disease transmission. In the study presented here, using the same transmissible spongiform encephalopathy (TSE) animal model, our aim was to determine the minimal number of white blood cells and the specific abilities of mononucleated cell populations to transmit scrapie by the transfusion route...
January 13, 2016: Journal of Virology
https://www.readbyqxmd.com/read/26709606/creutzfeldt-jakob-disease-and-blood-transfusion-updated-results-of-the-uk-transfusion-medicine-epidemiology-review-study
#20
P J M Urwin, J M Mackenzie, C A Llewelyn, R G Will, P E Hewitt
BACKGROUND AND OBJECTIVES: This paper reports the results to 31 May 2015 of an ongoing UK study to look for additional cases of variant Creutzfeldt-Jakob disease (vCJD) transmission by blood transfusion, and to seek evidence whether other subtypes of Creutzfeldt-Jakob disease (CJD) may be transmissible via blood components. MATERIALS AND METHODS: All vCJD cases of appropriate age and any sporadic CJD (sCJD) or familial CJD (fCJD) cases with a history of blood donation or transfusion are notified to the UKBS...
May 2016: Vox Sanguinis
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