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Sporadic or atypical BSE and CJD ?

  1. Dec 19, 2006 #1
    sporadic or atypical BSE and CJD ???

    Volume 12, Number 12–December 2006


    PERSPECTIVE

    On the Question of Sporadic

    or Atypical Bovine SpongiformEncephalopathy and

    Creutzfeldt-Jakob Disease

    Paul Brown,* Lisa M. McShane,† Gianluigi Zanusso,‡ and Linda Detwiler§

    Strategies to investigate the possible existence of sporadic

    bovine spongiform encephalopathy (BSE) require

    systematic testing programs to identify cases in countries

    considered to have little or no risk for orally acquired disease,

    or to detect a stable occurrence of atypical cases in

    countries in which orally acquired disease is disappearing.

    To achieve 95% statistical confidence that the prevalence

    of sporadic BSE is no greater than 1 per million (i.e., the

    annual incidence of sporadic Creutzfeldt-Jakob disease

    [CJD] in humans) would require negative tests in 3 million

    randomly selected older cattle. A link between BSE and

    sporadic CJD has been suggested on the basis of laboratory

    studies but is unsupported by epidemiologic observation.

    Such a link might yet be established by the discovery

    of a specific molecular marker or of particular combinations

    of trends over time of typical and atypical BSE and various

    subtypes of sporadic CJD, as their numbers are influenced

    by a continuation of current public health measures that

    exclude high-risk bovine tissues from the animal and

    human food chains.


    SNIP...

    PLEASE READ FULL TEXT ;


    http://www.cdc.gov/ncidod/EID/vol12no12/06-0965.htm?s_cid=eid06_0965_e




    3:00 Afternoon Refreshment Break, Poster and Exhibit Viewing in the Exhibit
    Hall


    3:30 Transmission of the Italian Atypical BSE (BASE) in Humanized Mouse

    Models Qingzhong Kong, Ph.D., Assistant Professor, Pathology, Case Western Reserve
    University

    Bovine Amyloid Spongiform Encephalopathy (BASE) is an atypical BSE strain
    discovered recently in Italy, and similar or different atypical BSE cases
    were also reported in other countries. The infectivity and phenotypes of
    these atypical BSE strains in humans are unknown. In collaboration with
    Pierluigi Gambetti, as well as Maria Caramelli and her co-workers, we have
    inoculated transgenic mice expressing human prion protein with brain
    homogenates from BASE or BSE infected cattle. Our data shows that about half
    of the BASE-inoculated mice became infected with an average incubation time
    of about 19 months; in contrast, none of the BSE-inoculated mice appear to
    be infected after more than 2 years. ***These results indicate that BASE is
    transmissible to humans and suggest that BASE is more virulent than
    classical BSE in humans.

    6:30 Close of Day One


    http://www.healthtech.com/2007/tse/day1.asp




    SEE STEADY INCREASE IN SPORADIC CJD IN THE USA FROM
    1997 TO 2006. SPORADIC CJD CASES TRIPLED, with phenotype
    of 'UNKNOWN' strain growing. ...


    http://www.cjdsurveillance.com/resources-casereport.html



    There is a growing number of human CJD cases, and they were presented last
    week in San Francisco by Luigi Gambatti(?) from his CJD surveillance
    collection.

    He estimates that it may be up to 14 or 15 persons which display selectively
    SPRPSC and practically no detected RPRPSC proteins.


    http://www.fda.gov/ohrms/dockets/ac/06/transcripts/1006-4240t1.htm


    http://www.fda.gov/ohrms/dockets/ac/06/transcripts/2006-4240t1.pdf




    JOURNAL OF NEUROLOGY

    MARCH 26, 2003



    RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob

    disease in the United States


    Email Terry S. Singeltary:


    flounder@wt.net



    I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to

    comment on the CDC's attempts to monitor the occurrence of emerging

    forms of CJD. Asante, Collinge et al [1] have reported that BSE

    transmission to the 129-methionine genotype can lead to an alternate

    phenotype that is indistinguishable from type 2 PrPSc, the commonest

    sporadic CJD. However, CJD and all human TSEs are not reportable

    nationally. CJD and all human TSEs must be made reportable in every

    state and internationally. I hope that the CDC does not continue to

    expect us to still believe that the 85%+ of all CJD cases which are

    sporadic are all spontaneous, without route/source. We have many TSEs in

    the USA in both animal and man. CWD in deer/elk is spreading rapidly and

    CWD does transmit to mink, ferret, cattle, and squirrel monkey by

    intracerebral inoculation. With the known incubation periods in other

    TSEs, oral transmission studies of CWD may take much longer. Every

    victim/family of CJD/TSEs should be asked about route and source of this

    agent. To prolong this will only spread the agent and needlessly expose

    others. In light of the findings of Asante and Collinge et al, there

    should be drastic measures to safeguard the medical and surgical arena

    from sporadic CJDs and all human TSEs. I only ponder how many sporadic

    CJDs in the USA are type 2 PrPSc?


    http://www.neurology.org/cgi/eletters/60/2/176#535




    Diagnosis and Reporting of Creutzfeldt-Jakob Disease

    Singeltary, Sr et al. JAMA.2001; 285: 733-734.

    http://jama.ama-assn.org/


    [Docket No. 03-025IFA] FSIS Prohibition of the Use of Specified Risk
    Materials for Human Food and Requirement for the Disposition of
    Non-Ambulatory Disabled Cattle

    http://www.fsis.usda.gov/OPPDE/Comments/03-025IFA/03-025IFA-2.pdf


    [Docket No. FSIS-2006-0011] FSIS Harvard Risk Assessment of Bovine
    Spongiform Encephalopathy (BSE)


    http://www.fsis.usda.gov/OPPDE/Comments/2006-0011/2006-0011-1.pdf



    THE SEVEN SCIENTIST REPORT ***


    http://www.fda.gov/ohrms/dockets/dockets/02n0273/02n-0273-EC244-Attach-1.pdf


    PAUL BROWN M.D.

    http://www.fda.gov/ohrms/dockets/dockets/02n0273/02n-0273-c000490-vol40.pdf




    9 December 2005
    Division of Dockets Management (RFA-305)

    SEROLOGICALS CORPORATION
    James J. Kramer, Ph.D.
    Vice President, Corporate Operations

    http://www.fda.gov/ohrms/dockets/dockets/02n0273/02n-0273-c000383-01-vol35.pdf



    Embassy of Japan
    http://www.fda.gov/ohrms/dockets/dockets/02n0273/02N-0273-EC240.htm



    TSS
     
  2. jcsd
  3. Dec 19, 2006 #2

    Evo

    User Avatar

    Staff: Mentor

    Please state specifically what you wish to discuss or what your questions are.
     
  4. Dec 19, 2006 #3
    Evo wrote;

    > Please state specifically what you

    > wish to discuss or what your questions are.


    i thought some might want to discuss Transmissible Spongiform Encephalopathies. i noticed a thread previously about kuru, cjd and bse, animal proteins etc., i posted there, and now i could not find data i posted to that thread, so i started a thread of my own. hope you do not mind. there is not enough discussion about this and i thought i might pass some data on to those that might be interested. does not seem like the public has been informed properly about this agent. we have all been exposed to some extent or the other through consumption, surgery, blood, supplements, vaccines, cosmetics, etc., a multitude of routes and sources right here in the USA. i have no questions. i came to this forum thinking some might be interested in the complete truth about this topic i.e. 'the rest of the story'. it's ongoing too. but if you rather i move on, i can do that too. my sources are valid, data coming from the top scientist. but some would rather not discuss this at all, bury it under the rug, and that is why the USA has the most documented TSE in different species in the world. it's the incubation period, lack of _documented_ body bags, from lack of surveillance i.e. autopsies not being performed on elderly demented, that is fooling everyone. i posted all this, some confidential data, but it seemed to have disappeared from the thread i posted too, or i have not figured out this board yet? either way, let me know if i am welcome here, and if not, i will move on.......

    kindest regards,
    terry
     
  5. Dec 19, 2006 #4
    Blood And Tse

    P.S.


    i thought some of you might be interested in the latest on the blood related issues that have come about lately with vCJD. Collinge et al had a recent study out in Lancet that was very disturbing. ...kind regards, terry


    The Lancet 2006; 368:2061-2067

    DOI:10.1016/S0140-6736(06)69835-8

    Articles

    Clinical presentation and pre-mortem diagnosis of variant Creutzfeldt-Jakob disease associated with blood transfusion: a case report

    Stephen J Wroe FRCP a b, Suvankar Pal MRCP a b, Durrenajaf Siddique MRCP a b, Harpreet Hyare FRCR a b, Rebecca Macfarlane MRCS a b, Susan Joiner MSc b, Jacqueline M Linehan BSc b, Sebastian Brandner MRCPath b, Jonathan DF Wadsworth PhD b, Patricia Hewitt FRCPath c and Prof John Collinge FRS a b

    Summary
    Background
    Concerns have been raised that variant Creutzfeldt-Jakob disease (vCJD) might be transmissible by blood transfusion. Two cases of prion infection in a group of known recipients of transfusion from donors who subsequently developed vCJD were identified post-mortem and reported in 2004. Another patient from this at-risk group developed neurological signs and was referred to the National Prion Clinic.

    Methods
    The patient was admitted for investigation and details of blood transfusion history were obtained from the National Blood Service and Health Protection Agency; after diagnosis of vCJD, the patient was enrolled into the MRC PRION-1 trial. When the patient died, brain and tonsil tissue were obtained at autopsy and assessed for the presence of disease-related PrP by immunoblotting and immunohistochemistry.

    Findings
    A clinical diagnosis of probable vCJD was made; tonsil biopsy was not done. The patient received experimental therapy with quinacrine, but deteriorated and died after a clinical course typical of vCJD. Autopsy confirmed the diagnosis and showed prion infection of the tonsils.

    Interpretation
    This case of transfusion-associated vCJD infection, identified ante-mortem, is the third instance from a group of 23 known recipients who survived at least 5 years after receiving a transfusion from donors who subsequently developed vCJD. The risk to the remaining recipients of such tranfusions is probably high, and these patients should be offered specialist follow-up and investigation. Tonsil biopsy will allow early and pre-symptomatic diagnosis in other iatrogenically exposed individuals at high risk, as in those with primary infection with bovine spongiform encephalopathy prions.

    Affiliations

    a. National Prion Clinic, National Hospital for Neurology and Neurosurgery, Queen Square, London WC1N 3BG, UK
    b. MRC Prion Unit and Department of Neurodegenerative Disease, Institute of Neurology, University College London, London, UK
    c. National Blood Service, London, UK

    Correspondence to: Prof John Collinge



    http://www.thelancet.com/journals/lancet/article/PIIS0140673606698358/abstract


    MRC PRION UNIT PDF


    http://www.mrc.ac.uk/consumption/groups/public/documents/content/mrc003431.pdf



    ----- Original Message -----
    From: Terry S. Singeltary Sr.
    To: FREAS@CBER.FDA.GOV
    Cc: william.freas@fda.hhs.gov ; rosanna.harvey@fda.hhs.gov
    Sent: Wednesday, November 29, 2006 1:24 PM
    Subject: TSE advisory committee for the meeting December 15, 2006 [TSS SUBMISSION]


    November 29, 2006


    Greetings FDA, DHH, Dr. Freas, and Dr. Harvey et al,



    a kind and warm Holiday Greetings to you all.


    i kindly wish to submit the following to the TSE advisory committee for the meeting December 15, 2006,
    about the assessment for potential exposure to vCJD in human plasma-derived antihemophilic factor (FVIII) products
    manufactured from U.S. plasma donors and related communication material ;


    http://a257.g.akamaitech.net/7/257/2422/01jan20061800/edocket.access.gpo.gov/2006/E6-20251.htm



    i see the media picked up on this as a 'low risk', from what the gov. agency
    perceived to be to them;


    http://www.newsday.com/news/health/...955259.story?coll=ny-leadhealthnews-headlines



    however, i seem to disagree. from my primitive ciphering, i see it another
    way. this is a huge catastrophic risk. 3 in 160 is 1.9%. so call that 2%
    which is 1 in 50 or twenty per thousand or 20,000 per million. also, what
    about the mixed genotypes/mixed susceptibility? what about the silent
    carriers that donated tainted blood? what about the sporadic CJDs of UNKNOWN
    strain or phenotype? this risk assessment is just more BSe to me. just
    another in a long line of industry fed crap. i pray that my assessment is
    the one that is wrong. but it is THEY who roll the dice with your life. it
    is THEY who refuse to regulate an industry that has run amok. just from a
    recall aspect of potentially tainted blood, and these are just recent recalls ;



    PRODUCT
    Source Plasma, Recall # B-0054-7
    CODE
    Units: 03MMNC5465, 03MMNC6361, 03MMNC6801, 03MMNC7510, 03MMNC7891,
    03MMNC8252, 03MMNC8801, 03MMNC9144, 03MMND1122, 03MMND1478, 03MMND1969,
    03MMND2350, 03MMND2825, 03MMND3211, 03MMND3708, 03MMND4072, 03MMND4588,
    03MMND4831, 03MMND5320, 03MMND5719, 03MMND6268, 03MMND6683, 03MMND7228,
    03MMND7656, 03MMND8211, 03MMND8652, 03MMND9195, 03MMND9618, 03MMNE0628,
    03MMNE0884, 03MMNE1597, 03MMNE1979, 03MMNE2644, 03MMNE3064, 03MMNE3707,
    03MMNE4122, 03MMNE4750, 03MMNE5080, 03MMNE5876, 03MMNE6218, 03MMNE7189,
    03MMNE7587, 03MMNE8027, 03MMNE8645, 03MMNE9029, 03MMNE9641, 03MMNE9979,
    03MMNF0491, 03MMNF0685, 03MMNF0937, 03MMNF1260, 04MMNA0351, 04MMNA0707,
    04MMNA1241, 04MMNA1650, 04MMNA2291, 04MMNA2646, 04MMNA3340, 04MMNA3719,
    04MMNA4312, 04MMNA4683, 04MMNA5298, 04MMNA5750, 04MMNA6407, 04MMNA6816,
    04MMNA7482, 04MMNA7915, 04MMNA8632, 04MMNA9076, 04MMNA9723, 04MMNB0063,
    04MMNB0696, 04MMNB1100, 04MMNB1845, 04MMNB2285, 04MMNB3035, 04MMNB3485,
    04MMNB4213, 04MMNB4672, 04MMNB5841, 04MMNB6652, 04MMNB7162, 04MMNB7930,
    04MMNB8453, 04MMNB9239, 04MMNB9747, 04MMNC0456, 04MMNC0931, 04MMNC1578
    RECALLING FIRM/MANUFACTURER
    BioLife Plasma Services, L.P., Mankato, MN, by facsimile on June 4, 2004.
    Firm initiated recall is complete.
    REASON
    Blood products, collected from a donor who was at increased risk for new
    variant Creutzfeldt-Jakob Disease (nvCJD), were distributed.
    VOLUME OF PRODUCT IN COMMERCE
    89 units
    DISTRIBUTION
    CA and Austria


    END OF ENFORCEMENT REPORT FOR October 25, 2006

    ###


    http://www.fda.gov/bbs/topics/enforce/2006/ENF00975.html



    USA FDA MAD COW BLOOD HUMANS RECALL (these are dime a dozen)


    SEE ;

    END OF ENFORCEMENT REPORT FOR SEPTEMBER 13, 2006

    ###

    http://www.fda.gov/bbs/topics/enforce/2006/ENF00969.html



    END OF ENFORCEMENT REPORT FOR SEPTEMBER 6, 2006

    ###

    http://www.fda.gov/bbs/topics/enforce/2006/ENF00968.html



    http://www.fda.gov/bbs/topics/enforce/2006/ENF00963.html



    END OF ENFORCEMENT REPORT FOR July 12, 2006

    ###


    http://www.fda.gov/bbs/topics/enforce/2006/ENF00960.html



    END OF ENFORCEMENT REPORT FOR July 5, 2006

    ###


    http://www.fda.gov/bbs/topics/enforce/2006/ENF00959.html




    Greetings again Dr. Freas et al at FDA,


    WITH new atypical TSE in the bovine, in the sheep, goat, and humans,
    and the fact that the new BASE TSE in cattle being very very similar to sporadic
    CJD, rather than the nvCJD, the fact that now science showing the TSE agent
    of the atypical cattle in Japan showing infectivity other than CNS tissue,
    the fact that the latest Texas mad cow and the recent Alabama mad cow both
    being of the atypical strain, it would seem prudent to include all human TSE
    in the blood ban, in my opinion. with sporadic CJD, you have many strains and
    or phenotypes, some of which are 'UNKNOWN', so we do not know how this
    will transmit, what tissues are infectious and or if blood transmits. that's the bottom
    line, however it has been reported that the BASE is more virulent to humans. With
    this, and the fact that sporadic CJD has tripled in the past few years or so, i see it
    as being prudent to take serious and immediate action..........


    snip...end too long.


    no pdf or doc url linked up yet for December 2006 submission.


    BUT, here is my submission from about 5 years ago for anyone interested ;


    PDF]Freas, William TSS SUBMISSION

    File Format: PDF/Adobe Acrobat -

    Page 1. J Freas, William From: Sent: To: Subject: Terry S. Singeltary

    Sr. [flounder] Monday, January 08, 2001 3:03 PM freas ...



    http://www.fda.gov/ohrms/dockets/ac/01/slides/3681s2_09.pdf


    TSS
     
  6. Dec 19, 2006 #5

    Evo

    User Avatar

    Staff: Mentor

    Sorry, I have to agree with the mentor that closed you other threads.

    Instead of just posting a bunch of links, you need to present a question, or questions you wish to discuss. Otherwise, this is not going to be productive.
     
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