Sunday, January 30, 2011

CJD diagnosis just got easier Test for Creutzfeldt–Jakob disease raises hopes of speedier diagnosis

Published online 30 January 2011 Nature doi:10.1038/news.2011.59

News

CJD diagnosis just got easier Test for Creutzfeldt–Jakob disease raises hopes of speedier diagnosis.

Tiffany O'Callaghan

In prion diseases such as CJD, an isomer of a prion protein takes on an abnormal shape. AP Photo/Professors Stanley Prusiner/Fred Cohen, University of California San Francisco Medical SchoolInvasive biopsy is currently the only sure way to diagnose the degenerative neurological condition Creutzfeldt–Jakob Disease (CJD). But a highly sensitive assay could change that, providing a fast, accurate alternative for early diagnosis of this rare but deadly condition.

In its most common form, known as sporadic CJD, the disease affects roughly one in a million people. Beginning in the 1990s, several cases of a variation of CJD known as vCJD were reported among people who had consumed beef from cows infected with another disease, bovine spongiform encephalopathy (BSE).

The findings, published online in Nature Medicine1, also suggest that the assay — developed by microbiologist Ryuichiro Atarashi of Nagasaki University, Japan, and his team — could pave the way for the screening of broad sectors of the population.

CJD is a prion disease, in which an isomer of a common protein known as the prion protein (PrP) takes on an abnormal shape and becomes an infectious variant called PrPSc. This variant is thought to trigger the subsequent malformation of other PrP proteins. Unlike their normal counterparts, PrPSc prions cannot be broken down, and instead accumulate — often clustering in brain tissue.

The pockets of abnormal tissue that result cause brain tissue to develop a sponge-like appearance, and because prion conditions can be spread by affected humans or animals, the diseases are often referred to as transmissible spongiform encephalopathies (TSEs). Humans can be affected by several such conditions, while in addition to BSE in cows, there are several other such disorders among animals, including a condition called scrapie in sheep and hamsters.

No false positives One problem that has plagued developers of non-biopsy diagnostic techniques is that it is often difficult to avoid false positives among samples taken from patients with neurodegenerative disorders other than CJD.

So Atarashi and his colleagues used a new assay known as a real-time quaking-induced conversion (RT-QUIC) assay. 'Quaking-induced' refers to in vitro shaking, which researchers believe helps to accelerate the reactions, enabling the assay to produce results more quickly.

The team tested cerebrospinal fluid samples from 18 people with CJD and 35 people with other neurodegenerative diseases. This pilot group produced no false positives, and CJD was correctly diagnosed more than 83% of the time.

The researchers compared these results with those obtained using an existing assay that tests for levels of a protein known as 14-3-3, which is a marker for sporadic CJD. When tested on patient samples, the accuracy of 14-3-3 was 72.2%, whereas the specificity was 85.7%.

In a subsequent blind trial on 30 cerebrospinal fluid samples from Australia, RT-QUIC showed 100% specificity, resulting in no false positives among the 14 control samples, and correct diagnoses of 87.5%. 14-3-3 was equally accurate, but the rate of false positives was much higher.

"This technique allows definitive ante-mortem confirmation of CJD," says Atarashi, adding that this is currently difficult because it demands the detection of PrPSc in patients' biopsy specimens.

Supersensitivity The RT-QUIC assay is also extraordinarily sensitive — detecting the presence of harmful prions at very high dilutions — and speedy, yielding results within 48 hours.

Atarashi first began developing RT-QUIC as a researcher at the National Institute of Allergy and Infectious Diseases, Rocky Mountain Laboratories, in Montana. In 2008, he and his team successfully used it to screen cerebrospinal fluid taken from scrapie-infected hamsters2.

In December, Atarashi co-authored a paper reporting the efficacy of the RT-QUIC assay on nasal secretions and cerebrospinal fluid from hamsters with prion disorders3.

Byron Caughey, chief of prion/TSE research at the Rocky Mountain lab and a co-author of the two hamster studies, is encouraged by the application of the assay to human cerebrospinal fluid samples. "Of course it will also be important to detect prion diseases in other species, but human diagnosis is of pre-eminent importance," Caughey says.

And although the next step is to replicate the findings in a much larger sample, the promise shown by RT-QUIC in analysing substances other than spinal fluid in hamsters suggests a potentially fertile area for future research in humans. If RT-QUIC could be used to screen blood samples, or cheek or nasal swabs, for example, it could open up the possibilities of much earlier diagnosis and more widespread screening of donated blood.

"The earlier you're able to detect the presence of an infection in humans or animals, the more chance you have of preventing transmission to others and treating the disease in those who are infected," Caughey says.

References 1.Atarashi, R. et al. Nature Med. doi:10.1038/nm.2294 (2011). 2.Atarashi, R. et al. Nature Methods 5, 211-212 (2008). 3.Wilham, J. M. et al. PLoS Pathog. 6, e1001217 (2010).

http://www.nature.com/news/2011/110130/full/news.2011.59.html#B1


Rapid End-Point Quantitation of Prion Seeding Activity with Sensitivity Comparable to Bioassays

Jason M. Wilham1, Christina D. Orrú1,2, Richard A. Bessen3, Ryuichiro Atarashi4, Kazunori Sano4, Brent Race1, Kimberly D. Meade-White1, Lara M. Taubner1, Andrew Timmes1, Byron Caughey1*

1 Laboratory of Persistent Viral Diseases, Rocky Mountain Laboratories, National Institute of Allergy and Infectious Disease, Hamilton, Montana, United States of America, 2 Department of Biomedical Sciences and Technologies, University of Cagliari, Monserrato, Italy, 3 Veterinary Molecular Biology, Montana State University, Bozeman, Montana, United States of America, 4 Department of Molecular Microbiology and Immunology, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Kyushu, Japan

Abstract

A major problem for the effective diagnosis and management of prion diseases is the lack of rapid high-throughput assays to measure low levels of prions. Such measurements have typically required prolonged bioassays in animals. Highly sensitive, but generally non-quantitative, prion detection methods have been developed based on prions' ability to seed the conversion of normally soluble protease-sensitive forms of prion protein to protease-resistant and/or amyloid fibrillar forms. Here we describe an approach for estimating the relative amount of prions using a new prion seeding assay called real-time quaking induced conversion assay (RT-QuIC). The underlying reaction blends aspects of the previously described quaking-induced conversion (QuIC) and amyloid seeding assay (ASA) methods and involves prion-seeded conversion of the alpha helix-rich form of bacterially expressed recombinant PrPC to a beta sheet-rich amyloid fibrillar form. The RT-QuIC is as sensitive as the animal bioassay, but can be accomplished in 2 days or less. Analogous to end-point dilution animal bioassays, this approach involves testing of serial dilutions of samples and statistically estimating the seeding dose (SD) giving positive responses in 50% of replicate reactions (SD50). Brain tissue from 263K scrapie-affected hamsters gave SD50 values of 1011-1012/g, making the RT-QuIC similar in sensitivity to end-point dilution bioassays. Analysis of bioassay-positive nasal lavages from hamsters affected with transmissible mink encephalopathy gave SD50 values of 103.5–105.7/ml, showing that nasal cavities release substantial prion infectivity that can be rapidly detected. Cerebral spinal fluid from 263K scrapie-affected hamsters contained prion SD50 values of 102.0–102.9/ml. RT-QuIC assay also discriminated deer chronic wasting disease and sheep scrapie brain samples from normal control samples. In principle, end-point dilution quantitation can be applied to many types of prion and amyloid seeding assays. End point dilution RT-QuIC provides a sensitive, rapid, quantitative, and high throughput assay of prion seeding activity.

Author Summary

Prion diseases are deadly infectious neurodegenerative disorders of mammals which involve the misfolding of host prion protein. To better manage these diseases, we need to be able to detect and quantify the infectious particles, or prions, in biological samples. However, current tests lack the sensitivity, speed and/or quantitative capabilities required for many important applications in medicine, agriculture, wildlife biology and research. To address this problem, we have developed a new prion assay that is highly sensitive, rapid, and quantitative. This assay takes advantage of the ability of miniscule amounts of infectious prions to seed the misfolding of large excesses of normal prion protein in test tube reactions. Quantitation is achieved by testing a range of sample dilutions and determining loss of seeding activity, i.e. the end-point dilution. Similar analyses have long been used to quantify prions by inoculation into animals; however, such bioassays take months or years to perform and are both animal-intensive and expensive. Our new method provides a more practical means of detecting and quantifying prions. So far, we have applied this assay to prions from sheep, deer, and hamsters, and have found surprisingly high levels of prions in the nasal and cerebral spinal fluids of infected hamsters.

Citation: Wilham JM, Orrú CD, Bessen RA, Atarashi R, Sano K, et al. (2010) Rapid End-Point Quantitation of Prion Seeding Activity with Sensitivity Comparable to Bioassays. PLoS Pathog 6(12): e1001217. doi:10.1371/journal.ppat.1001217

Editor: David Westaway, University of Alberta, Canada

Received: May 4, 2010; Accepted: November 3, 2010; Published: December 2, 2010

This is an open-access article distributed under the terms of the Creative Commons Public Domain declaration which stipulates that, once placed in the public domain, this work may be freely reproduced, distributed, transmitted, modified, built upon, or otherwise used by anyone for any lawful purpose.

Funding: This research was funded by the Intramural Research Program of the NIAID, NIH. J.M.W. was supported in part by the Undergraduate Scholarship Program of the NIH and C.D.O. was partially supported by the Master and Back Program of the Regione Sardegna (Italy). The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.

Competing interests: The authors have declared that no competing interests exist.

* E-mail: BCAUGHEY@niaid.nih.gov

http://www.plospathogens.org/article/info%3Adoi%2F10.1371%2Fjournal.ppat.1001217;jsessionid=0A1AAA0F70627D3CF7689F79025DD839.ambra02


Friday, January 21, 2011

Strain-Specific Barriers against Bovine Prions in Hamsters

http://transmissiblespongiformencephalopathy.blogspot.com/2011/01/strain-specific-barriers-against-bovine.html


Tuesday, January 25, 2011

Generation of a new form of human PrPSc in vitro by inter-species transmission from cervids prions

http://chronic-wasting-disease.blogspot.com/2011/01/generation-of-new-form-of-human-prpsc.html


Saturday, January 29, 2011

Atypical L-Type Bovine Spongiform Encephalopathy (L-BSE) Transmission to Cynomolgus Macaques, a Non-Human Primate

Jpn. J. Infect. Dis., 64 (1), 81-84, 2011

http://transmissiblespongiformencephalopathy.blogspot.com/2011/01/atypical-l-type-bovine-spongiform.html


Wednesday, January 19, 2011

EFSA BIOHAZ Scientific Opinion on the revision of the quantitative risk assessment (QRA) of the BSE risk posed by processed animal proteins (PAPs)

EFSA Journal 2011;9(1):1947

http://transmissiblespongiformencephalopathy.blogspot.com/2011/01/efsa-biohaz-scientific-opinion-on.html


Monday, January 17, 2011

MAD COW Update on Feed Enforcement Activities to Limit the Spread of BSE January 13, 2011

January 2011

http://transmissiblespongiformencephalopathy.blogspot.com/2011/01/mad-cow-update-on-feed-enforcement.html


Thursday, November 18, 2010

Increased susceptibility of human-PrP transgenic mice to bovine spongiform encephalopathy following passage in sheep

http://bse-atypical.blogspot.com/2010/11/increased-susceptibility-of-human-prp.html


Tuesday, January 18, 2011

Agent strain variation in human prion disease: insights from a molecular and pathological review of the National Institutes of Health series of experimentally transmitted disease

http://transmissiblespongiformencephalopathy.blogspot.com/2011/01/agent-strain-variation-in-human-prion.html


Thursday, December 23, 2010

Molecular Typing of Protease-Resistant Prion Protein in Transmissible Spongiform Encephalopathies of Small Ruminants, France, 2002–2009 Volume 17, Number 1–January 2011

http://transmissiblespongiformencephalopathy.blogspot.com/2010/12/molecular-typing-of-protease-resistant.html


Wednesday, January 19, 2011

EFSA and ECDC review scientific evidence on possible links between TSEs in animals and humans Webnachricht 19 Januar 2011

http://transmissiblespongiformencephalopathy.blogspot.com/2011/01/efsa-and-ecdc-review-scientific.html


Friday, January 21, 2011

Strain-Specific Barriers against Bovine Prions in Hamsters

http://transmissiblespongiformencephalopathy.blogspot.com/2011/01/strain-specific-barriers-against-bovine.html


Saturday, December 18, 2010

OIE Global Conference on Wildlife Animal Health and Biodiversity - Preparing for the Future (TSE AND PRIONS) Paris (France), 23-25 February 2011

http://transmissiblespongiformencephalopathy.blogspot.com/2010/12/oie-global-conference-on-wildlife.html


Monday, November 30, 2009

USDA AND OIE COLLABORATE TO EXCLUDE ATYPICAL SCRAPIE NOR-98 ANIMAL HEALTH CODE

http://nor-98.blogspot.com/2009/11/usda-and-oie-collaborate-to-exclude.html


http://bseusa.blogspot.com/2010/04/usda-and-oie-out-of-touch-with-risk.html


Tuesday, November 02, 2010

BSE - ATYPICAL LESION DISTRIBUTION (RBSE 92-21367) statutory (obex only) diagnostic criteria CVL 1992

http://bse-atypical.blogspot.com/2010/11/bse-atypical-lesion-distribution-rbse.html


USA

5 Includes 16 cases in which the diagnosis is pending, and 18 inconclusive cases;

6 Includes 21 (19 from 2010) cases with type determination pending in which the diagnosis of vCJD has been excluded.

2010

PLEASE NOTE REFERENCE LINES 5. AND 6.

Monday, August 9, 2010

National Prion Disease Pathology Surveillance Center Cases Examined (July 31, 2010) Year Total Referrals2 Prion Disease Sporadic Familial Iatrogenic vCJD

1996 & earlier 51 33 28 5 0 0

1997 114 68 59 9 0 0

1998 88 52 44 7 1 0

1999 120 72 64 8 0 0

2000 146 103 89 14 0 0

2001 209 119 109 10 0 0

2002 248 149 125 22 2 0

2003 274 176 137 39 0 0

2004 325 186 164 21 0 1(3)

2005 344 194 157 36 1 0

2006 383 197 166 29 0 2(4)

2007 377 214 187 27 0 0

2008 394 231 204 25 0 0

2009 425 259 216 43 0 0

2010 204 124 85 20 0 0

TOTAL 3702(5) 2177(6) 1834 315 4 3

1 Listed based on the year of death or, if not available, on year of referral;

2 Cases with suspected prion disease for which brain tissue and/or blood (in familial cases) were submitted;

3 Disease acquired in the United Kingdom;

4 Disease was acquired in the United Kingdom in one case and in Saudi Arabia in the other case;

5 Includes 16 cases in which the diagnosis is pending, and 18 inconclusive cases;

6 Includes 21 (19 from 2010) cases with type determination pending in which the diagnosis of vCJD has been excluded.

http://www.cjdsurveillance.com/pdf/case-table.pdf


Monday, August 9, 2010

National Prion Disease Pathology Surveillance Center Cases Examined (July 31, 2010)

(please watch and listen to the video and the scientist speaking about atypical BSE and sporadic CJD and listen to Professor Aguzzi)

http://prionunitusaupdate2008.blogspot.com/2010/08/national-prion-disease-pathology.html


Tuesday, December 14, 2010

Infection control of CJD, vCJD and other human prion diseases in healthcare and community settings part 4, Annex A1, Annex J, UPDATE DECEMBER 2010

http://creutzfeldt-jakob-disease.blogspot.com/2010/12/infection-control-of-cjd-vcjd-and-other.html


Saturday, June 13, 2009

Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States 2003 revisited 2009

http://cjdusa.blogspot.com/2009/06/monitoring-occurrence-of-emerging-forms.html


Saturday, January 2, 2010

Human Prion Diseases in the United States January 1, 2010 ***FINAL***

http://prionunitusaupdate2008.blogspot.com/2010/01/human-prion-diseases-in-united-states.html


my comments to PLosone here ;


http://www.plosone.org/annotation/listThread.action?inReplyTo=info%3Adoi%2F10.1371%2Fannotation%2F04ce2b24-613d-46e6-9802-4131e2bfa6fd&root=info%3Adoi%2F10.1371%2Fannotation%2F04ce2b24-613d-46e6-9802-4131e2bfa6fd


14th ICID International Scientific Exchange Brochure -

Final Abstract Number: ISE.114

Session: International Scientific Exchange

Transmissible Spongiform encephalopathy (TSE) animal and human TSE in North America

update October 2009

T. Singeltary

Bacliff, TX, USA

Background:

An update on atypical BSE and other TSE in North America. Please remember, the typical U.K. c-BSE, the atypical l-BSE (BASE), and h-BSE have all been documented in North America, along with the typical scrapie's, and atypical Nor-98 Scrapie, and to date, 2 different strains of CWD, and also TME. All these TSE in different species have been rendered and fed to food producing animals for humans and animals in North America (TSE in cats and dogs ?), and that the trading of these TSEs via animals and products via the USA and Canada has been immense over the years, decades.

Methods:

12 years independent research of available data

Results:

I propose that the current diagnostic criteria for human TSEs only enhances and helps the spreading of human TSE from the continued belief of the UKBSEnvCJD only theory in 2009. With all the science to date refuting it, to continue to validate this old myth, will only spread this TSE agent through a multitude of potential routes and sources i.e. consumption, medical i.e., surgical, blood, dental, endoscopy, optical, nutritional supplements, cosmetics etc.

Conclusion:

I would like to submit a review of past CJD surveillance in the USA, and the urgent need to make all human TSE in the USA a reportable disease, in every state, of every age group, and to make this mandatory immediately without further delay. The ramifications of not doing so will only allow this agent to spread further in the medical, dental, surgical arena's. Restricting the reporting of CJD and or any human TSE is NOT scientific. Iatrogenic CJD knows NO age group, TSE knows no boundaries. I propose as with Aguzzi, Asante, Collinge, Caughey, Deslys, Dormont, Gibbs, Gajdusek, Ironside, Manuelidis, Marsh, et al and many more, that the world of TSE Transmissible Spongiform Encephalopathy is far from an exact science, but there is enough proven science to date that this myth should be put to rest once and for all, and that we move forward with a new classification for human and animal TSE that would properly identify the infected species, the source species, and then the route.

http://ww2.isid.org/Downloads/14th_ICID_ISE_Abstracts.pdf


DID EVERYONE FILL OUT THEIR CJD QUESIONNAIRE FROM THE CDC AND OR THE CJD FOUNDATION ???

Friday, November 30, 2007

CJD QUESTIONNAIRE USA CWRU AND CJD FOUNDATION

http://cjdquestionnaire.blogspot.com/


Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texas USA 77518

Labels:

Friday, January 07, 2011

Transmission of sporadic Creutzfeldt-Jakob disease by blood transfusion: risk factor or possible biases

Transmission of sporadic Creutzfeldt-Jakob disease by blood transfusion: risk factor or possible biases

Maria Puopolo,
Anna Ladogana,
Vito Vetrugno,
Maurizio Pocchiari

Article first published online: 7 JAN 2011



DOI: 10.1111/j.1537-2995.2010.03004.x

© 2010 American Association of Blood Banks



BACKGROUND: The occurrence of transfusion transmissions of variant Creutzfeldt-Jakob disease (CJD) cases has reawakened attention to the possible similar risk posed by other forms of CJD.



STUDY DESIGN AND METHODS: CJD with a definite or probable diagnosis (sporadic CJD, n = 741; genetic CJD, n = 175) and no-CJD patients with definite alternative diagnosis (n = 482) with available blood transfusion history were included in the study. The risk of exposure to blood transfusion occurring more than 10 years before disease onset and for some possible confounding factors was evaluated by calculating crude odds ratios (ORs). Variables with significant ORs in univariate analyses were included in multivariate logistic regression analyses.



RESULTS: In the univariate model, blood transfusion occurring more than 10 years before clinical onset is 4.1-fold more frequent in sporadic CJD than in other neurologic disorders. This significance is lost when the 10-year lag time was not considered. Multivariate analyses show that the risk of developing sporadic CJD after transfusion increases (OR, 5.05) after adjusting for possible confounding factors. Analysis conducted on patients with genetic CJD did not reveal any significant risk factor associated with transfusion.



CONCLUSION: This is the first case-control study showing a significant risk of transfusion occurring more than 10 years before clinical onset in sporadic CJD patients. It remains questionable whether the significance of these data is biologically plausible or the consequence of biases in the design of the study, but they counterbalance previous epidemiologic negative reports that might have overestimated the assessment of blood safety in sporadic CJD.





http://onlinelibrary.wiley.com/doi/10.1111/j.1537-2995.2010.03004.x/abstract;jsessionid=656FB07EE4507FB2632BA3E691D3B824.d03t02






Eurosurveillance, Volume 12, Issue 3, 18 January 2007

Articles

Editorial team1


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Citation style for this article: Editorial team. Fourth case of transfusion-associated vCJD infection in the United Kingdom. Euro Surveill. 2007;12(3):pii=3117. Available online: http://www.eurosurveillance.org/ViewArticle.aspx?ArticleId=3117
Date of submission:


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Fourth case of transfusion-associated vCJD infection in the United Kingdom



Editorial team (eurosurveillance.weekly@hpa.org.uk), Eurosurveillance editorial office



A case of variant Creutzfeldt-Jakob disease (vCJD) has recently been diagnosed in a patient in the United Kingdom (UK), who received a blood transfusion from a donor who later developed vCJD [1]. This is the fourth case of probable transfusion transmission of vCJD infection in the UK. Three of the four recipients developed symptoms of vCJD.

The first symptomatic case of vCJD associated with blood transfusion was identified in December 2003. This individual developed vCJD six and a half years after transfusion of red cells donated by an individual who developed symptoms of vCJD three and a half years after donation.



A second case of vCJD 'infection' was identified a few months later in a person who had received red cells from a donor who developed symptoms of vCJD 18 months after donation. This patient (the second case) died from causes unrelated to vCJD five years after transfusion. Post-mortem investigations found abnormal prion protein in the spleen and a cervical lymph node., However, prion protein was not found in the brain, and no pathological features of vCJD were found.



A third case developed symptoms of vCJD six years after receiving a transfusion of red blood cells, and died two years and eight months later. The donor of the blood involved developed vCJD about 20 months after donating it.



These three cases have been published as case reports and in the findings of the ongoing collaborative study between the National Blood Services, the National CJD Surveillance Unit, and the Office for National Statistics. This study aims to collect evidence about transmission of CJD or vCJD via the blood supply [2,3,4,5].



The new, fourth case is in a patient who developed symptoms of vCJD eight and a half years after receiving a transfusion of red blood cells from a donor who developed vCJD about 17 months after this blood was donated [1]. The donor to this case also donated the vCJD-implicated blood transfused to the third case. As for all other reported clinical vCJD cases that have been tested for genotype, this patient is a methionine homozygote at codon 129 of the prion protein gene. The patient is currently alive.



All four cases had received transfusions of non-leucodepleted red blood cells between 1996 and 1999. Since October 1999, leucocytes have been removed from all blood used for transfusion in the UK. The effect of leucodepletion on the reduction of the risk of transmission of vCJD from an infective donation is uncertain.



This fourth case of vCJD infection associated with blood transfusion further increases the level of concern about the risk of vCJD transmission between humans by blood transfusion, although much remains unknown. This reinforces the importance of the existing precautions that have been introduced to reduce the risk of transmission of vCJD infection by blood and blood products [6]. No cases of vCJD have been associated with fractionated plasma products. The small group of living recipients of vCJD-implicated blood transfusion in the UK have been informed of their potential exposure to vCJD by blood transfusion, asked to take certain precautions to reduce the risk of onward person-to-person transmission of vCJD during health care, and offered specialist neurological evaluation and advice.



This article has been adapted from reference 1




References:

Health Protection Agency. Fourth case of variant CJD associated with blood transfusion (press release). Press release, 18 January 2007. (http://www.hpa.org.uk/hpa/news/articles/press_releases/2007/070118_vCJD.htm)
Llewelyn CA, Hewitt PE, Knight RSG, Amar K, Cousens S, Mackenzie J, et al. Possible transmission of variant CJD disease by blood transfusion. Lancet 2004; 363:417-21.
Peden AH, Head MW, Ritchie DL, Bell JE, Ironside JW. Preclinical vCJD after blood transfusion in a PRNP codon 129 heterozygous patient. Lancet 2004 ; 364: 527-9.
Wroe SJ, Pal S, Siddique D, Hyare H, Macfarlane R, et al Clinical presentation and pre-mortem diagnosis of blood transfusion-associated variant CJD. Lancet 2006;368:2061-67.
Hewitt PE, Llewelyn CA, Mackenzie J, Will RG. Creutzfeldt-Jakob disease and blood transfusion: results of the UK Transfusion Medicine Epidemiology review study. Vox Sang. 2006;91(3):221-230.
Department of Health [London]. Further precautions to protect blood supply. Press release 2004/0104, 16 March 2004. (http://www.dh.gov.uk/PublicationsAndStatistics/PressReleases/PressReleasesNotices/fs/en?CONTENT_ID=4076608&chk=MTwE%2Bl)


http://www.eurosurveillance.org/ViewArticle.aspx?ArticleId=3117




Tuesday, September 28, 2010

Variant CJD: where has it gone, or has it?

Pract Neurol 2010; 10: 250–251


http://vcjdtransfusion.blogspot.com/2010/09/variant-cjd-where-has-it-gone-or-has-it.html




Tuesday, September 14, 2010

Transmissible Spongiform Encephalopathies Advisory Committee; Notice of Meeting October 28 and 29, 2010 (COMMENT SUBMISSION)

----- Original Message -----

From: Terry S. Singeltary Sr.

To: william.freas@fda.hhs.gov Cc: rosanna.harvey@fda.hhs.gov ; Emery, Bryan (CBER)

SEE FULL TEXT ;

http://tseac.blogspot.com/2010/09/transmissible-spongiform_14.html



Wednesday, September 08, 2010

Emerging Infectious Diseases: CJD, BSE, SCRAPIE, CWD, PRION, TSE Evaluation to Implementation for Transfusion and Transplantation September 2010

http://vcjdtransfusion.blogspot.com/2010/09/emerging-infectious-diseases-cjd-bse.html



Saturday, July 17, 2010

Variant Creutzfeldt-Jakob disease Ironside JW., Haemophilia.

2010 Jul;16 Suppl 5:175-80 REVIEW ARTICLE

http://vcjdtransfusion.blogspot.com/2010/07/variant-creutzfeldtjakob-disease.html



http://vcjdtransfusion.blogspot.com/




----- Original Message -----

From: Terry S. Singeltary Sr.

To: CJD-L@LISTS.AEGEE.ORG

Cc: BLOODCJD@YAHOOGROUPS.COM ; cjdvoice@yahoogroups.com

Sent: Friday, September 24, 2010 11:15 AM

Subject: [BLOODCJD] USA Blood products, collected from a donor who was at risk for vCJD, were distributed SEPTEMBER 2010

PRODUCT
Red Blood Cells. Recall # B-2300-10
CODE
Unit: W001607702825
RECALLING FIRM/MANUFACTURER
Recalling Firm: Department of the Air Force, Wright Patterson AFB, OH, by letter dated April 8, 2008.
Manufacturer: Depart of Air Force 88th Medical Group SGQC WPAFB, Wright Patterson AFB, OH. Firm initiated recall is complete.
REASON
Blood product, collected from a donor who was at risk for variant Creutzfeldt-Jakob Disease (vCJD), was distributed.
VOLUME OF PRODUCT IN COMMERCE
1 unit
DISTRIBUTION
Japan

___________________________________

PRODUCT
Recovered Plasma. Recall # B-2302-10
CODE
Units: R08951; P90041; P90041
RECALLING FIRM/MANUFACTURER
Blood Center of Northcentral Wisconsin, Inc., Wausau, WI, by fax on January 2, 2007. Firm initiated recall is complete.
REASON
Blood products, collected from a donor considered to be at increased risk for variant Creutzfeldt-Jakob Disease (vCJD), were distributed.
VOLUME OF PRODUCT IN COMMERCE
3 units
DISTRIBUTION
NY

___________________________________

PRODUCT
1) Red Blood Cells Leukocytes Reduced. Recall # B-2338-10;
2) Plasma Frozen. Recall # B-2339-10
CODE
1) and 2) Unit: 5039861
RECALLING FIRM/MANUFACTURER
Community Blood Center, Inc., Appleton, WI, by letter dated September 21, 2007 or by electronic notification on September 21, 2007. Firm initiated recall is complete.
REASON
Blood products, collected from a donor who was at risk for variant Creutzfeldt-Jakob Disease (vCJD), were distributed.
VOLUME OF PRODUCT IN COMMERCE
2 units
DISTRIBUTION
WI, Switzerland

___________________________________

END OF ENFORCEMENT REPORT FOR SEPTEMBER 22, 2010

http://www.fda.gov/Safety/Recalls/EnforcementReports/ucm227078.htm


PRODUCT

1) Cryoprecipitated AHF, Pooled. Recall # B-2155-10;

2) Recovered Plasma. Recall # B-2156-10

CODE

1) Unit: W036309907231;

2) Unit: W036309616077

RECALLING FIRM/MANUFACTURER

BloodCenter of Wisconsin, Inc., Milwaukee, WI, by fax and internet on May 5, 2010 and May 13, 2010. Firm initiated recall is complete.

REASON

Blood products, collected from a donor considered to be at increased risk for variant Creutzfeldt-Jakob Disease (vCJD), were distributed.

VOLUME OF PRODUCT IN COMMERCE

2 units

DISTRIBUTION

TX, Switzerland

___________________________________

PRODUCT

Red Blood Cells Leukocytes Reduced. Recall # B-2157-10

CODE

Unit: 6371718

RECALLING FIRM/MANUFACTURER

South Texas Blood & Tissue Center, San Antonio, TX, by telephone on January 23, 2010 and by fax on January 25, 2010. Firm initiated recall is complete.

REASON

Blood product, collected from a donor who failed to answer questions regarding risk for vCJD, was distributed.

VOLUME OF PRODUCT IN COMMERCE

1 unit

DISTRIBUTION

TX

___________________________________

PRODUCT

Source Plasma. Recall # B-2212-10

CODE

Units: 09FMOG6851; 09FMOG3410; 09FMOG2756; 09FMOG1418; 09FMOF6640; 09FMOF2642; 09FMOF1554; 09FMOD7746; 09FMOF0063; 09FMOF7599

RECALLING FIRM/MANUFACTURER

BioLife Plasma Service LP, Springfield, MO, by fax on April 1, 2010. Firm initiated recall is complete.

REASON

Blood products, collected from a donor considered to be at increased risk for variant Creutzfeldt-Jakob Disease (vCJD), were distributed.

VOLUME OF PRODUCT IN COMMERCE

10 units

DISTRIBUTION

CA

___________________________________

PRODUCT

1) Red Blood Cells Leukocytes Reduced. Recall # B-2213-10;

2) Recovered Plasma. Recall # B-2214

CODE

1) and 2) Unit: 6325245

RECALLING FIRM/MANUFACTURER

South Texas Blood and Tissue Center, San Antonio, TX, by facsimile on February 8, 2010. Firm initiated recall is complete.

REASON

Blood products, collected from a donor considered to be at increased risk for variant Creutzfeldt-Jakob Disease (vCJD), were distributed.

VOLUME OF PRODUCT IN COMMERCE

2 units

DISTRIBUTION

FL, TX

___________________________________

END OF ENFORCEMENT REPORT FOR SEPTEMBER 15, 2010

http://www.fda.gov/Safety/Recalls/EnforcementReports/ucm225990.htm


PRODUCT
Source Plasma. Recall # B-2056-10
CODE
Units: FD0500537, FD0502880, FD0503259, FD0509894, FD0515518, FD0516063, FD0517957, FD0518606, FD0522255, FD0523346, FD0523544, FD0524204, FD0524698, FD0525142, FD0525845, FD0526653, FD0526878, FD0527579, FD0527845, FD0528519, FD0528827, FD0529544, FD0529761, FD0530471, FD0530712, FD0531425, FD0531801, FD0532483, FD0532869, FD0537501, FD0537687, FD0538370, FD0543210, FD0546250, FD0546632, FD0547328, FD0547832, FD0548286, FD0548743, FD0549325, FD0549840, FD0550427, FD0551448, FD0551572, FD0552307, FD0553173, FD0553418, FD0554063, FD0554834, FD0555041, FD0559685, FD0560235, FD0560592, FD0561168, FD0561786, FD0562212, FD0562883, FD0563248, FD0564435, FD0564723, FD0565467, FD0565880, FD0566540, FD0567053, FD0567723, FD0567965, FD0568941, FD0569180, FD0570057, FD0571177, FD0571477, FD0572411, FD0572818, FD0573582, FD0573871, FD0574531, FD0576955, FD0577140, FD0579983, FD0580403, FD0581156, FD0581623, FD0582680, FD0583090, FD0584073, FD0584500, FD0585410, FD0586089, FD0586790, FD0587500, FD0588791, FD0589023, FD0590248, FD0590600, FD0591592, FD0592445, FD0593277, FD0593712, FD0594626, FD0595049, FD0596132, FD0596519, FD0597701, FD0598681, FD0599198, FD0600210, FD0600690, FD0601755, FD0602401, FD0603415, FD0603985, FD0605122, FD0608608, FD0609074, FD0609979, FD0610508, FD0611469, FD0612006, FD0612905
RECALLING FIRM/MANUFACTURER
DCI Biologicals LLC, Farmington, NM, by facsimile on September 26, 2009 and electronic mail dated January 15, 2010. Firm initiated recall is complete.
REASON
Blood products, collected from a donor who was at risk for variant Creutzfeldt-Jakob Disease (vCJD), were distributed.
VOLUME OF PRODUCT IN COMMERCE
119 units
DISTRIBUTION
NY, UK

___________________________________

END OF ENFORCEMENT REPORT FOR SEPTEMBER 8, 2010

http://www.fda.gov/Safety/Recalls/EnforcementReports/ucm225223.htm


PRODUCT
Source Plasma. Recall # B-2134-10
CODE
Units: 3910020431, 3910019695, 3910018715, 3910018227, 3910017100, 3910016675, 3910015596, 3910015120, 3910014175, 3910013575, 3910012934, 3910012281, 3910010102, 3910009899, 3910007715, 3910007430
RECALLING FIRM/MANUFACTURER
Talecris Plasma Resources, Inc., N Las Vegas, NV, by fax on July 17, 2009. Firm initiated recall is complete.
REASON
Blood products, collected from a donor considered to be at increased risk for variant Creutzfeldt-Jakob Disease (vCJD), were distributed.
VOLUME OF PRODUCT IN COMMERCE
16 units
DISTRIBUTION
NC

___________________________________

PRODUCT
1) Red Blood Cells. Recall # B-2215-10;
2) Fresh Frozen Plasma. Recall # B-2216-10
CODE
1) and 2) Unit: 0951592
RECALLING FIRM/MANUFACTURER
Memorial Blood Centers, Saint Paul, MN, by letter on November 5, 2008. Firm initiated recall is complete.
REASON
Blood products, collected from a donor considered to be at increased risk for variant Creutzfeldt-Jakob Disease (vCJD), were distributed.
VOLUME OF PRODUCT IN COMMERCE
2 units
DISTRIBUTION
MN

___________________________________

PRODUCT
Recovered Plasma. Recall # B-2217-10
CODE
Unit: 0951592
RECALLING FIRM/MANUFACTURER
Memorial Blood Centers, Saint Paul, MN, by letter on November 5, 2008. Firm initiated recall is complete.
REASON
Blood product, collected from a donor considered to be at increased risk for variant Creutzfeldt-Jakob Disease (vCJD), was distributed.
VOLUME OF PRODUCT IN COMMERCE
1 unit
DISTRIBUTION
MN

___________________________________

END OF ENFORCEMENT REPORT FOR SEPTEMBER 1, 2010

http://www.fda.gov/Safety/Recalls/EnforcementReports/ucm224723.htm



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Thursday, January 06, 2011

Italy: 'Mad cow disease' claims second Italian victim

Italy: 'Mad cow disease' claims second Italian victim




Livorno, 6 Jan. (AKI) - The human variant of the brain-wasting 'mad cow' disease claimed a second victim in Italy after a 44-year-old woman died in hospital in the northwestern port city of Livorno, the local health authority reported on Thursday.



The health authority said the 44-year-old Livorno woman died on Wednesday and was admitted to a city hospice in July in the final stages of her battle against variant Creutzfeldt-Jakob disease (vCJD), commonly known as 'mad cow' disease.


There is there is no known cure for vCJD.


Italy's first case, a Sicilian woman, died in 2002, a year after she contracted the disease.


VCJD is the human form of the fatal brain-wasting illness first identified in cattle - BSE or bovine spongiform encephalopathy. A total of 275 people have been infected with the disease over the past decade, 170 in Britain, the worst-affected country, 56 in Israel, and 25 in France.


Nearly 200,000 cattle have died from BSE over the same period. The human form of BSE is believed to be caused by eating meat infected with the bovine disease.


EU restrictions on the sale of T-bone beef steaks and beef offal were lifted in 2006 when officials declared the 'mad cow' outbreak was over.
A massive cull in Britain prevented the spread of the disease but isolated cases are still reported.


Almost 190,000 cattle have died from BSE over the past ten years, the vast majority in the UK.


Italy's health ministry and various local health authorities have always denied vCJD patients can infect their family members and carers with the disease.


http://www.adnkronos.com/IGN/Aki/English/Security/Italy-Mad-cow-disease-claims-second-Italian-victim_311503811571.html




1ST CASE NVCJD ITALY 2002



Eurosurveillance, Volume 6, Issue 6, 07 February 2002 Articles S Salmaso1

--------------------------------------------------------------------------------

Citation style for this article: Salmaso S.

First case of vCJD reported in Italy.

Euro Surveill. 2002;6(6):pii=2022.

Available online:

http://www.eurosurveillance.org/ViewArticle.aspx?ArticleId=2022


Date of submission:


--------------------------------------------------------------------------------

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First case of vCJD reported in Italy

The first case of variant Creutzfeld-Jakob Disease (vCJD) has been reported in Italy (1). The case from Sicily was diagnosed in Italy and Great Britain on the basis of clinical and instrumental tests and tonsillar biopsy. The Istituto Superiore di Sanità (ISS) has classified the case as probable, but since the patient is still alive, the ISS has not released any additional information. The surveillance and reporting of CJD has been mandatory in Italy since the beginning of 2002. A ban on feeding mammalian protein to ruminants was approved on 28 July 1984, and enforced from 15 September that year. The prevalence of indigenous cases of bovine spongiform encephalopathy per 10 000 tests was 1.03 for the year 2001 (2).

References :


1.Ministero della Salute. Segnalato caso variante malattia Creutzfeldt-Jakob. Press release 05/02/2002 N° 40, 5 February 2002.

(http://www.sanita.interbusiness.it/comunicati_stampa/documenti/040-02.rtf)


2.Centro per lo Studio e le Ricerche sulle Encefalopatie Animali e Neuropatologie Comparate. Focolai BSE in Italia: 2001/2002.

(http://www.to.izs.it)


Reported by Stefania Salmaso

(salmaso@iss.it), Istituto Superiore di Sanità, Italy.


http://www.eurosurveillance.org/ViewArticle.aspx?ArticleId=2022



Eurosurveillance, Volume 6, Issue 40, 03 October 2002

Articles

A M Molesworth1

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Citation style for this article: Molesworth AM. First case of variant Creutzfeldt-Jakob disease reported in Italy - update. Euro Surveill. 2002;6(40):pii=1891. Available online: http://www.eurosurveillance.org/ViewArticle.aspx?ArticleId=1891 Date of submission:


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First case of variant Creutzfeldt-Jakob disease reported in Italy - update


A paper published in the Lancet this week describes the first case of variant Creutzfeldt-Jakob disease (vCJD) in Italy (1). The patient, a 25 year old Sicilian woman, developed clinical symptoms in 2001 and was first reported in February this year (2). Although definitive diagnosis of vCJD requires neuropathological confirmation, the woman’s illness fulfils the diagnostic criteria for probable vCJD (3), a brain scan was characteristic of vCJD and the abnormal protein associated with vCJD has been detected in a tonsil biopsy.


The patient had never undergone neurosurgery, or received a blood transfusion or any other treatment that might be associated with transmission of a prion disease. Scientific evidence suggests that vCJD is, however, caused by transmission of the bovine spongiform encephalopathy agent (BSE) to humans. The woman is likely to have acquired her infection within Italy, having never travelled to the United Kingdom (UK) or any other country with reported BSE. Cases of indigenous BSE were first reported in Italy following the implementation, in 2001, of mandatory testing of cattle older than 30 months destined for slaughter (4). By the end of September 2002 70 indigenous cases and two British imported cases had been identified (5).


To date, in addition to the Italian case, single cases of definite or probable vCJD have been diagnosed with onset in each of the Republic of Ireland, Canada and the United States, a further six in France and 127 in the UK (see EUROCJD, http://www.eurocjd.ed.ac.uk/). The occurrence of cases both within and outside the UK remains a concern, supporting the need for continuous surveillance programmes of transmissible spongiform encephalopathies in humans and animals in the UK and other countries.


References :


La Bella V, Collinge J, Pocchiari M, Piccoli F. Variant Creutzfeldt-Jakob disease in an Italian woman. Lancet 2002; 360: 997-8. (http://pdf.thelancet.com/pdfdownload?uid=llan.360.9338.original_research.22571.1&x=x.pdf).



Salmaso S. First case of vCJD reported in Italy. Eurosurveillance Weekly 2002; 6: 020207. (http://www.eurosurveillance.org/ew/2002/020207.asp).



Will RG, Zeidler M, Stewart GE, Macleod MA, Ironside JW, Cousens SN, et al. Diagnosis of new variant Creutzfeldt-Jakob disease. Ann Neurol 2000;47: 575-82.



European Commission. Regulation (EC) No 999/2001 of the European Parliament and of the Council of 22 May 2001 (http://europa.eu.int/comm/food/fs/bse/bse36_en.pdf)



Centro per lo Studio e le Ricerche sulle Encefalopatie Animali e Neuropatologie Comparate. Focolai BSE in Italia [BSE Outbreaks in Italy]: 2001/2002 (http://www.to.izs.it)



Reported by Anna Molesworth (amolesworth@phls.org.uk) and Peter Horby, Public Health Laboratory Service Communicable Disease Surveillance Centre, London, England.



http://www.eurosurveillance.org/ViewArticle.aspx?ArticleId=1891






http://creutzfeldt-jakob-disease.blogspot.com/


http://transmissiblespongiformencephalopathy.blogspot.com/



TSS

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