Wednesday, May 11, 2011

House of Commons CJD May 2011 UPDATE

Jason McCartney: To ask the Secretary of State for Health how many people died from variant Creutzfeldt-Jakob disease in England in each of the last five years. [54620] Anne Milton: The National Creutzfeldt-Jakob disease Research and Surveillance Unit (NCJDRSU) has provided the following information about deaths from variant Creutzfeldt-Jakob disease (vCJD). This information is available on NCJDRSU website at:

United Kingdom definite and probable vCJD deaths 2005 to 2010

Number of Deaths

2005 5

2006 5

2007 5

2008 1

2009 3

2010 3

CJD: Disease Control

Sir Paul Beresford: To ask the Secretary of State for Health what research his Department (a) has carried out and (b) is undertaking on cold sterilisation products for the prevention of cross infection of variant Creutzfeldt-Jakob disease prion from surgical instruments; what estimate has been made of the cost of such research in the last 12 months; when the results of the ongoing research are expected; who is carrying out the ongoing research; and where the results and conclusions of such research (i) are to be and (ii) have been published. [54483]

Mr Simon Burns: The Department has previously funded research on cold sterilisation products for the prevention of cross infection of variant Creutzfeldt-Jakob disease. The projects funded include research on enzymatic detergents, protein detection assays, physico-chemical technologies, and barrier and surface coatings. Findings have been published in peer-reviewed journals.

The Department is not currently funding any projects in this area, and there was no expenditure in 2010-11.

Sir Paul Beresford: To ask the Secretary of State for Health how much has been spent from the public purse on research studies on tonsils and the appendix to detect variant Creutzfeldt-Jakob disease; and when the results of such studies are expected. [54484]

Mr Simon Burns: To obtain an estimate of the prevalence of variant Creutzfeldt-Jakob disease (vCJD) in the population, the Department funds studies to detect vCJD in tonsils and appendices. From 1999-2000 to 2010-11, the Department spent £10.9 million on such studies from central research and development budgets.

Results have been published in peer reviewed journals. Further publication is expected at the end of the current projects.

9 May 2011 : Column 1041W

Sir Paul Beresford: To ask the Secretary of State for Health what estimate has been made of the cost to the public purse of importing blood products from non-UK sources for the purposes of prevention of transmission of variant Creutzfeldt-Jakob disease in each year since its commencement. [54485]

Anne Milton: Since 1998 a number of measures have been introduced by the UK Blood Services to reduce the risk of transfusion transmitted variant Creutzfeldt-Jakob disease. The cost of the importation of fresh frozen plasma for therapeutic use is shown in the following table:

Fresh frozen plasma: Cost of importation, 2004-11

£ million

2004-05 0.29

2005-06 0.51

2006-07 0.31

2007-08 0.32

2008-09 0.46

2009-10 0.93

2010-11 1.02

Total 3.84

Source: NHS Blood and Transplant.

NHS Blood and Transplant report that the increased costs shown from 2008-09 are in part a result of less favourable exchange rates.

Since 1999, plasma for the manufacture of fractionated plasma products, such as immunoglobulins and clotting factors, has been obtained from non-United Kingdom sources. There is a global market for the main plasma products and most companies were largely unaffected by the changes resulting from vCJD as they source plasma from non-UK donors.

Bio Products Laboratory Ltd (BPL) is the Department-owned fractionator which supplies part of the national health service demand but only a very small proportion of global product (2% to 4%). BPL historically used plasma from the UK blood services and was therefore directly affected by the change in policy. BPL's fractionated products are used in the UK, and are also sold abroad. A cost estimate for importing the plasma that is used to manufacture BPL products that are used in the UK is not readily available, but I will place an estimate in the Library by the end of June 2011.

Sir Paul Beresford: To ask the Secretary of State for Health pursuant to the contribution of the Minister of State of 28 April 2011, Official Report, column 426, on variant Creutzfeldt-Jakob disease, announcing funding for the development of cold plasma decontamination technology, whether this research will examine the removal or de-activation of vCJD prions. [54553]

Mr Simon Burns: The cold plasma decontamination project will use scrapie infected mouse brain homogenates, not variant Creutzfeldt-Jakob disease infected tissues and homogenates. Removal of the prion will be examined.

Sir Paul Beresford: To ask the Secretary of State for Health pursuant to the contribution of the Minister of State of 28 April 2011, Official Report, column 425W, and the answer of 12 July 2010, Official Report, column 475W on Creutzfeldt-Jakob disease, how many

9 May 2011 : Column 1042W

transmissions of variant Creutzfeldt-Jakob disease have been presumed to be associated with blood since 1999. [54597]

Anne Milton: There have been three cases of clinical variant Creutzfeldt-Jakob disease (vCJD) and one case of infection (without development of clinical disease) presumed to be associated with blood transfusion. These have occurred in people who have received blood transfusions from donors who themselves went on to develop clinical vCJD after they had made the blood donation. None of these patients were transfused since 1999. The vCJD infection in these four recipients only came to light in 2003 and later because of the incubation period of vCJD.

The Transfusion Medicine Epidemiology Review (TMER), a collaborative project between the United Kingdom National Creutzfeldt-Jakob Disease Research & Surveillance Unit (NCJDRSU) and the United Kingdom blood services, investigates evidence that CJD or vCJD may have been transmitted via the blood supply. Details are on the TMER website at:

Sir Paul Beresford: To ask the Secretary of State for Health whether his Department has conducted a cost analysis to compare the proposed use of prion filters and existing costs of risk reduction measures against the introduction of a variant Creutzfeldt-Jakob disease blood screening test which may replace or remove the need for these measures. [54816]

Anne Milton: There is currently no blood screening test that is proven to identify asymptomatic variant Creutzfeldt-Jakob disease infection. For this reason it is not possible to carry out a cost analysis to compare these measures.

Sir Paul Beresford: To ask the Secretary of State for Health pursuant to the contribution of the Minister of State for Health of 28 April 2011, Official Report, column 430 on variant Creutzfeldt-Jakob Disease (vCJD), what pathway his Department proposes to use to develop a prototype vCJD blood test in the event that no commercial company believes there is a business case to develop such a test. [54896]

Anne Milton: The Department is aware of a number of commercial organisations and academic institutions currently developing prototype blood tests for the abnormal prion protein associated with variant Creutzfeldt-Jakob Disease.


Paul Goggins: To ask the Secretary of State for Health in how many cases the presence of variant Creutzfeldt-Jakob disease has been evident in biopsies carried out following the death of a patient with haemophilia in the last 20 years. [52333]

Anne Milton: Abnormal prion protein associated with variant Creutzfeldt-Jakob disease has been found in a single spleen sample taken from one haemophilia patient at post mortem. Details can be found in “Peden A, McCardle L, Head MW et at. Variant CJD infection in the spleen of a neurologically asymptomatic UK adult patient with haemophilia. Haemophilia 2010; 16: 296-304”. The journal Haemophilia is available on line at:

Sunday, March 6, 2011

U.K. and U.S.A. vCJD, CJD, TSE screen (a) the blood supply and (b) blood donors Commons Hansard Written Answers and FDA March 2011

Tuesday, April 26, 2011

sporadic CJD RISING Text and figures of the latest annual report of the NCJDRSU covering the period 1990-2009 (published 11th March 2011)

Saturday, March 5, 2011


Thursday, February 24, 2011

The risk of variant Creutzfeldt-Jakob disease among UK patients with bleeding disorders, known to have received potentially contaminated plasma products

Sunday, May 1, 2011

W.H.O. T.S.E. PRION Blood products and related biologicals May 2011

Tuesday, March 29, 2011


Tuesday, September 14, 2010

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

Sunday, May 10, 2009

Meeting of the Transmissible Spongiform Encephalopathies Committee On June 12, 2009 (Singeltary submission)

TO :

May 8, 2009

Greetings again Dr. Freas, TSEAC et al,

I would kindly, once again, wish to comment at this meeting about the urgent actions that need to be taken asap, to the Meeting of the Transmissible Spongiform Encephalopathies Committee On June 12, 2009. Due to my disability from my neck injury, I will not be attending this meeting either, however I hope for my submission to be read and submitted. ...

IN reply to ;

Harvard Risk Assessment of Bovine Spongiform Encephalopathy Update, October 31, 2005 INTRODUCTION The United States Department of Agriculture's Food Safety and Inspection Service (FSIS) held a public meeting on July 25, 2006 in Washington, D.C. to present findings from the Harvard Risk Assessment of Bovine Spongiform Encephalopathy Update, October 31, 2005 (report and model located on the FSIS website:

Comments on technical aspects of the risk assessment were then submitted to FSIS. Comments were received from Food and Water Watch, Food Animal Concerns Trust (FACT), Farm Sanctuary, R-CALF USA, Linda A Detwiler, and Terry S. Singeltary. This document provides itemized replies to the public comments received on the 2005 updated Harvard BSE risk assessment. Please bear the following points in mind:

Wednesday, March 9, 2011

27 U.S. Senators want to force feed Japan Highly Potential North America Mad Cow Beef TSE PRION CJD

March 8, 2011

President Barack Obama The White House

1600 Pennsylvania Avenue, W Washington, DC 20500

Dear President Obama:

Wednesday, March 31, 2010

Atypical BSE in Cattle

To date the OIE/WAHO assumes that the human and animal health standards set out in the BSE chapter for classical BSE (C-Type) applies to all forms of BSE which include the H-type and L-type atypical forms. This assumption is scientifically not completely justified and accumulating evidence suggests that this may in fact not be the case. Molecular characterization and the spatial distribution pattern of histopathologic lesions and immunohistochemistry (IHC) signals are used to identify and characterize atypical BSE. Both the L-type and H-type atypical cases display significant differences in the conformation and spatial accumulation of the disease associated prion protein (PrPSc) in brains of afflicted cattle. Transmission studies in bovine transgenic and wild type mouse models support that the atypical BSE types might be unique strains because they have different incubation times and lesion profiles when compared to C-type BSE. When L-type BSE was inoculated into ovine transgenic mice and Syrian hamster the resulting molecular fingerprint had changed, either in the first or a subsequent passage, from L-type into C-type BSE. In addition, non-human primates are specifically susceptible for atypical BSE as demonstrated by an approximately 50% shortened incubation time for L-type BSE as compared to C-type. Considering the current scientific information available, it cannot be assumed that these different BSE types pose the same human health risks as C-type BSE or that these risks are mitigated by the same protective measures.

This study will contribute to a correct definition of specified risk material (SRM) in atypical BSE. The incumbent of this position will develop new and transfer existing, ultra-sensitive methods for the detection of atypical BSE in tissue of experimentally infected cattle.

Thursday, August 12, 2010

Seven main threats for the future linked to prions

First threat

The TSE road map defining the evolution of European policy for protection against prion diseases is based on a certain numbers of hypotheses some of which may turn out to be erroneous. In particular, a form of BSE (called atypical Bovine Spongiform Encephalopathy), recently identified by systematic testing in aged cattle without clinical signs, may be the origin of classical BSE and thus potentially constitute a reservoir, which may be impossible to eradicate if a sporadic origin is confirmed. ***Also, a link is suspected between atypical BSE and some apparently sporadic cases of Creutzfeldt-Jakob disease in humans. These atypical BSE cases constitute an unforeseen first threat that could sharply modify the European approach to prion diseases.

Second threat


All Other Emerging and Zoonotic Infectious Diseases CDC's FY 2012 request of $52,658,000 for all other emerging and zoonotic infectious disease activities is a decrease of $13,607,000 below the FY 2010 level,

which includes the elimination of Prion activities ($5,473,000),

a reduction for other cross-cutting infectious disease activities, and administrative savings. These funds support a range of critical emerging and zoonotic infectious disease programs such Lyme Disease, Chronic Fatigue Syndrome, and Special Pathogens, as well as other activities described below.

Friday, April 15, 2011



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