> > Sent: Saturday, March 25, 2006 12:45 PM
> > Subject: BSE UPDATE ALABAMA March 24, 2006
> >
> >
> > ##################### Bovine Spongiform Encephalopathy
> > #####################
> >
> >
> > Subject: BSE UPDATE ALABAMA March 24, 2006
> > Date: March 25, 2006 at 10:15 am PST
> >
> > March 24, 2006 - BSE Update
> > MONTGOMERY – Commissioner Ron Sparks and State Veterinarian Dr. Tony
> Frazier
> > with the Alabama Department of Agriculture and Industries (ADAI) and the
> > USDA have provided an update on their ongoing joint investigation of the
> cow
> > that died from bovine spongiform encephalopathy (BSE) in Alabama.
> >
> >
> > March 24, 2006 - BSE Update
> >
> > Since the investigation began, the ADAI and the USDA have followed
> multiple
> > leads in the traceback process. At this time, 13 locations and 32
> movements
> > of cattle have been examined with 27 of those being substantially
> completed.
> > Additional investigations of locations and herds will continue. In
> addition,
> > state and federal officials have confirmed that a black bull calf was
born
> > in 2005 to the index animal (the red cow). The calf was taken by the
owner
> > to a local stockyard in July 2005 where the calf died. The calf was
> disposed
> > of in a local landfill and did not enter the human or animal food chain.
> >
> > Without a premises or animal ID program in place, the traceback process
to
> > find the herd of origin of the index cow is time-consuming and
difficult.
> It
> > includes conducting interviews, reviewing of records and documents, and
> > testing of cattle DNA. State and federal officials have discovered
several
> > herds of interest and they are planning to use DNA testing to determine
> DNA
> > linkage between the index cow and the herds. Through the DNA testing of
> > these herds, investigators will attempt to find a genetic path that
could
> > lead to the herd of origin. Commissioner Sparks stressed that the DNA
> > testing being conducted on the herds is for genetic markers and is not a
> > test for the disease BSE.
> >
> > As part of the thorough investigative process, a large number of cattle
> may
> > be tested in this phase and the number of herds included will continue
to
> > grow as the traceback progresses. Leads will be followed by state and
> > federal officials until they are exhausted. Even when an index animal is
> > traced to it’s birth herd, often cohorts of that animal are no longer in
> > that herd. In addition, even if an animal’s cohort has been exposed to
the
> > same infective material in feed, the other animals will not necessarily
> > contract BSE.
> >
> > BSE is not a contagious disease that spreads animal to animal, or animal
> to
> > human. BSE spreads in cattle through the consumption of feed containing
> > specified risk material (brain and spinal cord) derived from BSE
infected
> > cattle. The United States banned the use of such protein supplements in
> > cattle feed since 1997. Sparks says that beef consumption in this
country
> is
> > safe and there are measures in place to see that it continues to be
safe.
> > For example, downer animals are not allowed to enter commerce for human
> > consumption and there is a ban on feeding ruminant derived protein to
> > cattle.
> >
> >
> >
http://www.agi.state.al.us/press_releases/march-24-2006---bse-update2?pn=2
> >
> >
> >
> >
==========================================================================
> >
> >
> >
> >
> > Epidemiology Update March 23, 2006
> > As of today, 13 locations and 32 movements of cattle have been examined
> with
> > 27 of those being substantially completed. Additional investigations of
> > locations and herds will continue. In addition, state and federal
> officials
> > have confirmed that a black bull calf was born in 2005 to the index
animal
> > (the red cow). The calf was taken by the owner to a local stockyard in
> July
> > 2005 where the calf died. The calf was appropriately disposed of in a
> local
> > landfill and did not enter the human or animal food chain.
> >
> >
> >
> >
> >
http://www.aphis.usda.gov/newsroom/hot_issues/bse/bse_al_epi-update.shtml
> >
> >
> >
> >
> >
> > > The calf was appropriately disposed of in a local
> > > landfill and did not enter the human or animal food chain.
> >
> >
> > meanwhile, back at the ranch with larry, curly and mo heading up the
USDA
> et
> > al,
> > what would you expect, nothing less than shoot, shovel and shut the hell
> up.
> > no mad cow in USA, feed ban working, no civil war in Iraq either.
> >
> >
> > but what has past history shown us, evidently it has shown the USDA et
al
> > nothing ;
> >
> >
> > Disposal of meat and bone meal (MBM) derived from specified risk
material
> > (SRM) and over thirty month scheme carcasses by landfill
> > The Committee was asked to consider a quantitative risk assessment of
the
> > disposal of meat and bone meal derived from specified risk material and
> over
> > thirty month scheme carcasses by landfill, prepared in response to a
> request
> > from the Committee at its June 1999 meeting.
> >
> > The Committee was asked whether, in the light of the results of the risk
> > assessment, it held to its earlier published (June 1999) view that
> landfill
> > was an acceptable outlet for MBM of any origin, although it retained a
> > preference for incineration. The Committee reiterated that it had a
strong
> > preference for incineration as the favoured route for the disposal of
MBM
> > and were uneasy about the use of landfill for the disposal of this
> material.
> > If there were cases where incineration was not practical the Committee
> felt
> > it would be preferable for any material going to landfill to be
> > pressure-cooked first or possibly stored above ground prior to
> incineration.
> >
> >
> >
> > http://www.seac.gov.uk/summaries/summ_0700.htm
> >
> >
> >
> >
> > Disposal of BSE suspect carcases
> > It is the Department's policy to dispose of BSE suspects by incineration
> > wherever feasible. No BSE suspect carcases have been landfilled since
> 1991.
> >
> >
> >
> > http://www.defra.gov.uk/animalh/bse/publichealth/notification.html#disp
> >
> >
> >
> >
> > OPINION ON
> >
> > THE USE OF BURIAL FOR DEALING WITH ANIMAL
> >
> > CARCASSES AND OTHER ANIMAL MATERIALS THAT
> >
> > MIGHT CONTAIN BSE/TSE
> >
> > ADOPTED BY THE
> >
> > SCIENTIFIC STEERING COMMITTEE
> >
> > MEETING OF 16-17 JANUARY 2003
> >
> > The details of the SSC’s evaluation are provided in the attached report.
> The
> > SSC
> >
> > concludes as follows:
> >
> > (1) The term “burial” includes a diversity of disposal conditions.
> Although
> > burial is
> >
> > widely used for disposal of waste the degradation process essential for
> > BSE/TSE
> >
> > infectivity reduction is very difficult to control. The extent to which
> such
> > an
> >
> > infectivity reduction can occur as a consequence of burial is poorly
> > characterised.
> >
> > It would appear to be a slow process in various circumstances.
> >
> > (2) A number of concerns have been identified including potential for
> > groundwater
> >
> > contamination, dispersal/transmission by birds/animals/insects,
accidental
> >
> > uncovering by man.
> >
> > (3) In the absence of any new data the SSC confirms its previous opinion
> > that animal
> >
> > material which could possibly be contaminated with BSE/TSEs, burial
poses
> a
> >
> > risk except under highly controlled conditions (e.g., controlled
> landfill).
> >
> > SNIP...
> >
> > 4. CONCLUSION
> >
> > In the absence of new evidence the opinion of the SSC “Opinion on Fallen
> > Stock”
> >
> > (SSC 25th June 1999) must be endorsed strongly that land burial of all
> > animals and
> >
> > material derived from them for which there is a possibility that they
> could
> >
> > incorporate BSE/TSEs poses a significant risk. Only in exceptional
> > circumstances
> >
> > where there could be a considerable delay in implementing a safe means
of
> > disposal
> >
> > should burial of such materials be considered. Guidelines should be made
> > available
> >
> > to aid on burial site selection.
> >
> > 4 PAGES;
> >
> >
> >
> > http://europa.eu.int/comm/food/fs/sc/ssc/out309_en.pdf
> >
> >
> >
> >
> > During the 2001 outbreak of FMD in the UK, the
> >
> > Department of Health prepared a rapid qualitative
> >
> > assessment of the potential risks to human health
> >
> > associated with various methods of carcass disposal
> >
> > (UK Department of Health, 2001c). The most
> >
> > relevant hazards to human health resulting from
> >
> > burial were identified as bacteria pathogenic to
> >
> > humans, water-borne protozoa, and BSE. The main
> >
> > potential route identified was contaminated water
> >
> > supplies, and the report generally concluded that an
> >
> > engineered licensed landfill would always be
> >
> > preferable to unlined burial. In general terms, the
> >
> > findings of the qualitative assessment relative to
> >
> > biological agents are summarized in Table 13.
> >
> > TABLE 13. Potential health hazards and associated pathways of exposure
> > resulting from landfill or burial of
> >
> > animal carcasses (adapted from UK Department of Health, 2001c).
> >
> > PLEASE SEE TABLE AT;
> >
> >
> >
> >
>
http://www.k-state.edu/projects/fss/research/books/carcassdispfiles/PDF%20Fi
> > les/CH%201%20-%20Burial.pdf
> >
> >
> >
> > PART 2
> >
> > Rendering and fixed-facility incineration were
> >
> > preferred, but the necessary resources were not
> >
> > immediately available and UK officials soon learned
> >
> > that the capacity would only cover a portion of the
> >
> > disposal needs. Disposal in commercial landfills was
> >
> > seen as the next best environmental solution, but
> >
> > legal, commercial, and local community problems
> >
> > limited landfill use. With these limitations in mind,
> >
> > pyre burning was the actual initial method used but
> >
> > was subsequently discontinued following increasing
> >
> > public, scientific, and political concerns. Mass burial
> >
> > and on-farm burial were last on the preferred
> >
> > method list due to the complicating matter of bovine
> >
> > spongiform encephalopathy (BSE) and the risk posed
> >
> > to groundwater (Hickman & Hughes, 2002).
> >
> >
> >
>
http://www.k-state.edu/projects/fss/research/books/carcassdispfiles/PDF%20Fi
> >
>
les/Introduction%20to%20Part%202%20-%20Cross-Cutting%20&%20Policy%20Issues.p
> > df
> >
> >
> >
> >
> >
> > Carcase disposal:
> >
> > A Major Problem of the
> >
> > 2001 FMD Outbreak
> >
> > Gordon Hickman and Neil Hughes, Disposal Cell,
> >
> > FMD Joint Co-ordination Centre, Page Street
> >
> > snip...
> >
> >
> > http://www.defra.gov.uk/animalh/svj/fmd/pages27-40.pdf
> >
> >
> >
> > 3. Prof. A. Robertson gave a brief account of BSE. The US approach
> > was to accord it a _very low profile indeed_. Dr. A Thiermann showed
> > the picture in the ''Independent'' with cattle being incinerated and
> thought
> > this was a fanatical incident to be _avoided_ in the US _at all
costs_...
> >
> > snip...
> >
> >
> > http://www.bseinquiry.gov.uk/files/mb/m11b/tab01.pdf
> >
> >
> >
> >
> >
> > PAUL BROWN SCRAPIE SOIL TEST
> >
> >
> > http://www.bseinquiry.gov.uk/files/sc/seac07/tab03.pdf
> >
> >
> >
> >
> > Some unofficial information from a source on the inside looking out -
> >
> > Confidential!!!!
> >
> > As early as 1992-3 there had been long studies conducted on small
> > pastures containing scrapie infected sheep at the sheep research station
> > associated with the Neuropathogenesis Unit in Edinburgh, Scotland.
> > Whether these are documented...I don't know. But personal recounts both
> > heard and recorded in a daily journal indicate that leaving the pastures
> > free and replacing the topsoil completely at least 2 feet of thickness
> > each year for SEVEN years....and then when very clean (proven scrapie
> > free) sheep were placed on these small pastures.... the new sheep also
> > broke out with scrapie and passed it to offspring. I am not sure that
TSE
> > contaminated ground could ever be free of the agent!!
> > A very frightening revelation!!!
> >
> > ----------
> >
> > more here ;
> >
> > http://www.bseinquiry.gov.uk/files/ws/s018.pdf
> >
> >
> >
> >
> > INCINERATION TEMPS
> >
> > Requirements include:
> >
> > a. after burning to the range of 800 to 1000*C to eliminate smell;
> >
> > well heck, this is just typical public relations fear factor control.
> > do you actually think they would spend the extra costs for fuel,
> > for such extreme heat, just to eliminate smell, when they spread
> > manure all over your veg's. i think not. what they really meant were
> > any _TSE agents_.
> >
> > b. Gas scrubbing to eliminate smoke -- though steam may be omitted;
> >
> > c. Stacks to be fitted with grit arreaters;
> >
> > snip...
> >
> > 1.2 Visual Imact
> >
> > It is considered that the requirement for any carcase incinerator
> > disign would be to ensure that the operations relating to the reception,
> > storage and decepitation of diseased carcasses must not be publicly
> > visible and that any part of a carcase could not be removed or
> > interfered with by animals or birds.
> >
> > full text;
> >
> >
> > http://www.bseinquiry.gov.uk/files/yb/1989/04/03006001.pdf
> >
> >
> >
> >
> > http://europa.eu.int/comm/food/fs/sc/ssc/out311_en.pdf
> >
> >
> >
> >
> >
> > also, if one thinks that cattle don't become infected with BSE under 30
> > months, well that simplys is not correct.
> >
> > youngest to date is 20 months, with many more in the 20 to 30 month
range
> > not only in the UK but Japan as well. ...
> >
> >
> >
> >
> >
> >
> >
> > Kind regards,
> >
> > Terry S. Singeltary Sr.
> > Bacliff, Texas USA
> >
> > #################### https://lists.aegee.org/bse-l.html
> > ####################
> >
> > #################### https://lists.aegee.org/bse-l.html
> ####################
> >
>
> #################### https://lists.aegee.org/bse-l.html
####################
>#################### https://lists.aegee.org/bse-l.html ####################
BSE GBR ASSESSMENTS
http://www.efsa.eu.int/science/tse_assessments/gbr_assessments/catindex_en.html
EFSA Scientific Report on the Assessment of the Geographical BSE-Risk (GBR) of the United States of America (USA)
Last updated: 19 July 2005
Adopted July 2004 (Question N° EFSA-Q-2003-083)
Report
Summary
Summary of the Scientific Report
The European Food Safety Authority and its Scientific Expert Working Group on the Assessment of the Geographical Bovine Spongiform Encephalopathy (BSE) Risk (GBR) were asked by the European Commission (EC) to provide an up-to-date scientific report on the GBR in the United States of America, i.e. the likelihood of the presence of one or more cattle being infected with BSE, pre-clinically as well as clinically, in USA. This scientific report addresses the GBR of USA as assessed in 2004 based on data covering the period 1980-2003.
The BSE agent was probably imported into USA and could have reached domestic cattle in the middle of the eighties. These cattle imported in the mid eighties could have been rendered in the late eighties and therefore led to an internal challenge in the early nineties. It is possible that imported meat and bone meal (MBM) into the USA reached domestic cattle and leads to an internal challenge in the early nineties.
A processing risk developed in the late 80s/early 90s when cattle imports from BSE risk countries were slaughtered or died and were processed (partly) into feed, together with some imports of MBM. This risk continued to exist, and grew significantly in the mid 90’s when domestic cattle, infected by imported MBM, reached processing. Given the low stability of the system, the risk increased over the years with continued imports of cattle and MBM from BSE risk countries.
EFSA concludes that the current GBR level of USA is III, i.e. it is likely but not confirmed that domestic cattle are (clinically or pre-clinically) infected with the BSE-agent. As long as there are no significant changes in rendering or feeding, the stability remains extremely/very unstable. Thus, the probability of cattle to be (pre-clinically or clinically) infected with the BSE-agent persistently increases.
Publication date: 20 August 2004
http://www.efsa.eu.int/science/tse_assessments/gbr_assessments/573_it.html
http://www.efsa.eu.int/science/tse_assessments/gbr_assessments/573/sr03_biohaz02_usa_report_summary_en1.pdf
http://www.efsa.eu.int/science/tse_assessments/gbr_assessments/573/sr03_biohaz02_usa_report_v2_en1.pdf
From: TSS ()
Subject: [Docket No. 03-025IFA] SRMs and Non-Ambulatory disabled cattle[TSS]
Date: October 6, 2005 at 2:28 pm PST
[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
03-025IFA
03-025IFA-2
Terry S. Singeltary
Page 1 of 17
From: Terry S. Singeltary Sr. [flounder9@verizon.net]
Sent: Thursday, September 08, 2005 6:17 PM
To: fsis.regulationscomments@fsis.usda.gov
Subject: [Docket No. 03-025IFA] FSIS Prohibition of the Use of Specified Risk Materials for Human Food and Requirements
for the Disposition of Non-Ambulatory Disabled Cattle
Greetings FSIS,
I would kindly like to submit the following to [Docket No. 03-025IFA] FSIS Prohibition of the Use of Specified Risk Materials for Human Food and
Requirements for the Disposition of Non-Ambulatory Disabled Cattle
THE BSE/TSE SUB CLINICAL Non-Ambulatory Disabled Cattle
Broken bones and such may be the first signs of a sub clinical BSE/TSE Non-Ambulatory Disabled Cattle ;
SUB CLINICAL PRION INFECTION
MRC-43-00
Issued: Monday, 28 August 2000
NEW EVIDENCE OF SUB-CLINICAL PRION INFECTION: IMPORTANT RESEARCH
FINDINGS RELEVANT TO CJD AND BSE
Terry S. Singeltary Sr.
P.O. Box 42
Bacliff, Texas USA 77518
9/13/2005
http://www.fsis.usda.gov/OPPDE/Comments/03-025IFA/03-025IFA-2.pdf
========================================================
========================================================
OLD TSS SUBMISSIONS;
Docket No, 04-047-l Regulatory Identification No. (RIN) 091O-AF46 NEW BSE SAFEGUARDS (comment submission)
https://web01.aphis.usda.gov/regpublic.nsf/0/eff9eff1f7c5cf2b87256ecf000df08d?OpenDocument
Docket No. 03-080-1 -- USDA ISSUES PROPOSED RULE TO ALLOW LIVE ANIMAL
IMPORTS FROM CANADA
https://web01.aphis.usda.gov/BSEcom.nsf/0/b78ba677e2b0c12185256dd300649f9d?OpenDocument&AutoFramed
Docket No. 2003N-0312 Animal Feed Safety System [TSS SUBMISSION]
http://www.fda.gov/ohrms/dockets/dockets/03n0312/03N-0312_emc-000001.txt
Docket Management Docket: 02N-0273 - Substances Prohibited From Use in
Animal Food or Feed; Animal Proteins Prohibited in Ruminant Feed
Comment Number: EC -10
Accepted - Volume 2
http://www.fda.gov/ohrms/dockets/dailys/03/Jan03/012403/8004be07.html
PART 2
http://www.fda.gov/ohrms/dockets/dailys/03/Jan03/012403/8004be09.html
PDF]Freas, William TSS SUBMISSION
File Format: PDF/Adobe Acrobat -
Page 1. J Freas, William From: Sent: To: Subject: Terry S. Singeltary
Sr. [flounder@wt.net] Monday, January 08,200l 3:03 PM freas ...
http://www.fda.gov/ohrms/dockets/ac/01/slides/3681s2_09.pdf
Asante/Collinge et al, 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;
http://www.fda.gov/ohrms/dockets/ac/03/slides/3923s1_OPH.htm
Docket Management Docket: 96N-0417 - Current Good Manufacturing Practice
in Manufacturing, Packing, or Holding Dietary Ingredients a
Comment Number: EC -2
Accepted - Volume 7
http://www.fda.gov/ohrms/dockets/dailys/03/Mar03/031403/96N-0417-EC-2.htm
[PDF] Appendices to PL107-9 Inter-agency Working Group Final Report 1-1
File Format: PDF/Adobe Acrobat - View as HTML
Agent, Weapons of Mass Destruction Operations Unit Federal Bureau of
those who provided comments in response to Docket No. ...
Meager 8/18/01 Terry S. Singeltary Sr ...
http://www.aphis.usda.gov/lpa/pubs/pubs/PL107-9_Appen.pdf
Docket No. 2003N-0312 Animal Feed Safety System [TSS SUBMISSION
TO DOCKET 2003N-0312]
http://www.fda.gov/ohrms/dockets/dockets/03n0312/03N-0312_emc-000001.txt
# Docket No: 02-088-1 RE-Agricultural Bioterrorism Protection Act of
2002; [TSS SUBMISSION ON POTENTIAL FOR BSE/TSE & FMD 'SUITCASE BOMBS'] -
TSS 1/27/03 (0)
Docket Management
Docket: 02N-0276 - Bioterrorism Preparedness; Registration of Food Facilities, Section 305
Comment Number: EC-254 [TSS SUBMISSION]
http://www.fda.gov/ohrms/dockets/dockets/02n0276/02N-0276-EC-254.htm
Dockets Entered On October 2, 2003 Table of Contents, Docket #,
Title, 1978N-0301,
OTC External Analgesic Drug Products, ... EMC 7, Terry S. Singeltary Sr.
Vol #: 1, ...
http://www.fda.gov/ohrms/dockets/dailys/03/oct03/100203/100203.htm
Daily Dockets Entered on 02/05/03
DOCKETS ENTERED on 2/5/03. ... EMC 4 Terry S. Singeltary Sr. Vol#: 2.
... Vol#: 1.
03N-0009 Federal Preemption of State & Local Medical Device Requireme. ...
http://www.fda.gov/ohrms/dockets/dailys/03/Feb03/020503/020503.htm
Docket Management
Docket: 02N-0370 - Neurological Devices; Classification of Human Dura Mater
Comment Number: EC -1
Accepted - Volume 1
http://www.fda.gov/ohrms/dockets/dailys/03/Jan03/012403/8004be11.html
http://www.fda.gov/ohrms/dockets/dailys/03/Jan03/012403/8004bdfe.html
http://www.fda.gov/ohrms/dockets/dailys/03/Jan03/012403/8004bdfc.html
Daily Dockets - 04/10/03
... 00D-1662 Use of Xenotransplantation Products in Humans.
EMC 98 Terry S. Singeltary Sr. Vol#: 3. 01F ...
http://www.fda.gov/ohrms/dockets/dailys/03/Apr03/041003/041003.htm - 05-20-2003
- Cached
2003D-0186
Guidance for Industry: Use of Material From Deer and Elk In Animal Feed
EMC 1
Terry S. Singeltary Sr.
Vol #:
1
http://www.fda.gov/ohrms/dockets/dailys/03/Jun03/060903/060903.htm
2003D-0186
Guidance for Industry: Use of Material From Deer and Elk In Animal Feed
EMC 7
Terry S. Singeltary Sr.
Vol #:
1
2003D-0186
Guidance for Industry: Use of Material From Deer and Elk In Animal Feed
EMC 7
Terry S. Singeltary Sr.
Vol #:
1
http://www.fda.gov/ohrms/dockets/dailys/03/oct03/100203/100203.htm
01N-0423 Substances Prohibited from use in animal food/Feed Ruminant
APE 5 National Renderers Association, Inc. Vol#: 2
APE 6 Animal Protein Producers Industry Vol#: 2
APE 7 Darling International Inc. Vol#: 2
EMC 1 Terry S. Singeltary Sr. Vol#: 3
http://www.fda.gov/ohrms/dockets/dailys/01/Oct01/101501/101501.htm
Send Post-Publication Peer Review to journal:
Re: 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
LANCET INFECTIOUS DISEASE JOURNAL
Volume 3, Number 8 01 August 2003
Newsdesk
Tracking spongiform encephalopathies in North America
Xavier Bosch
My name is Terry S Singeltary Sr, and I live in Bacliff, Texas. I lost
my mom to hvCJD (Heidenhain variant CJD) and have been searching for
answers ever since. What I have found is that we have not been told the
truth. CWD in deer and elk is a small portion of a much bigger problem.
49-year-old Singeltary is one of a number of people who have remained
largely unsatisfied after being told that a close relative died from a
rapidly progressive dementia compatible with spontaneous
Creutzfeldt-Jakob disease (CJD). So he decided to gather hundreds of
documents on transmissible spongiform encephalopathies (TSE) and
realised that if Britons could get variant CJD from bovine spongiform
encephalopathy (BSE), Americans might get a similar disorder from
chronic wasting disease (CWD)the relative of mad cow disease seen among
deer and elk in the USA. Although his feverish search did not lead him
to the smoking gun linking CWD to a similar disease in North American
people, it did uncover a largely disappointing situation.
Singeltary was greatly demoralised at the few attempts to monitor the
occurrence of CJD and CWD in the USA. Only a few states have made CJD
reportable. Human and animal TSEs should be reportable nationwide and
internationally , he complained in a letter to the Journal of the
American Medical Association (JAMA 2003; 285: 733). I hope that the CDC
does not continue to expect us to still believe that the 85% plus of all
CJD cases which are sporadic are all spontaneous, without route or source.
Until recently, CWD was thought to be confined to the wild in a small
region in Colorado. But since early 2002, it has been reported in other
areas, including Wisconsin, South Dakota, and the Canadian province of
Saskatchewan. Indeed, the occurrence of CWD in states that were not
endemic previously increased concern about a widespread outbreak and
possible transmission to people and cattle.
To date, experimental studies have proven that the CWD agent can be
transmitted to cattle by intracerebral inoculation and that it can cross
the mucous membranes of the digestive tract to initiate infection in
lymphoid tissue before invasion of the central nervous system. Yet the
plausibility of CWD spreading to people has remained elusive.
Part of the problem seems to stem from the US surveillance system. CJD
is only reported in those areas known to be endemic foci of CWD.
Moreover, US authorities have been criticised for not having performed
enough prionic tests in farm deer and elk.
Although in November last year the US Food and Drug Administration
issued a directive to state public-health and agriculture officials
prohibiting material from CWD-positive animals from being used as an
ingredient in feed for any animal species, epidemiological control and
research in the USA has been quite different from the situation in the
UK and Europe regarding BSE.
Getting data on TSEs in the USA from the government is like pulling
teeth , Singeltary argues. You get it when they want you to have it,
and only what they want you to have.
Norman Foster, director of the Cognitive Disorders Clinic at the
University of Michigan (Ann Arbor, MI, USA), says that current
surveillance of prion disease in people in the USA is inadequate to
detect whether CWD is occurring in human beings ; adding that, the
cases that we know about are reassuring, because they do not suggest the
appearance of a new variant of CJD in the USA or atypical features in
patients that might be exposed to CWD. However, until we establish a
system that identifies and analyses a high proportion of suspected prion
disease cases we will not know for sure . The USA should develop a
system modelled on that established in the UK, he points out.
Ali Samii, a neurologist at Seattle VA Medical Center who recently
reported the cases of three hunterstwo of whom were friendswho died
from pathologically confirmed CJD, says that at present there are
insufficient data to claim transmission of CWD into humans ; adding that
[only] by asking [the questions of venison consumption and deer/elk
hunting] in every case can we collect suspect cases and look into the
plausibility of transmission further . Samii argues that by making both
doctors and hunters more aware of the possibility of prions spreading
through eating venison, doctors treating hunters with dementia can
consider a possible prion disease, and doctors treating CJD patients
will know to ask whether they ate venison.
CDC spokesman Ermias Belay says that the CDC will not be investigating
the [Samii] cases because there is no evidence that the men ate
CWD-infected meat . He notes that although the likelihood of CWD
jumping the species barrier to infect humans cannot be ruled out 100%
and that [we] cannot be 100% sure that CWD does not exist in humans&
the data seeking evidence of CWD transmission to humans have been very
limited .
http://infection.thelancet.com/journal/journal.isa
he complained in a letter to the Journal of the American Medical
Association (JAMA 2003; 285: 733). I hope that the CDC does not
continue to expect us to still believe that the 85% plus of all CJD
cases which are sporadic are all spontaneous, without route or source. <<<
actually, that quote was from a more recent article in the Journal of
Neurology (see below), not the JAMA article...
Full Text
Diagnosis and Reporting of Creutzfeldt-Jakob Disease
Singeltary, Sr et al. JAMA.2001; 285: 733-734.
http://jama.ama-assn.org/cgi/content/full/285/6/733?maxtoshow=&HITS=10&hits=10&RESULTFORMAT=&fulltext=dignosing+and+reporting+creutzfeldt+jakob+disease&searchid=1048865596978_1528&stored_search=&FIRSTINDEX=0&journalcode=jama
BRITISH MEDICAL JOURNAL
SOMETHING TO CHEW ON
BMJ
http://www.bmj.com/cgi/eletters/319/7220/1312/b#EL2
BMJ
http://www.bmj.com/cgi/eletters/320/7226/8/b#EL1
THE PATHOLOGICAL PROTEIN
BY Philip Yam
Yam Philip Yam News Editor Scientific American www.sciam.com
http://www.thepathologicalprotein.com/
IN light of Asante/Collinge et al findings 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;
-------- Original Message -------- Subject: re-BSE prions propagate as
either variant CJD-like or sporadic CJD Date: Thu, 28 Nov 2002 10:23:43
-0000 From: "Asante, Emmanuel A" To:
"'flounder@wt.net'"
Dear Terry,
I have been asked by Professor Collinge to respond to your request. I am
a Senior Scientist in the MRC Prion Unit and the lead author on the
paper. I have attached a pdf copy of the paper for your attention. Thank
you for your interest in the paper.
In respect of your first question, the simple answer is, yes. As you
will find in the paper, we have managed to associate the alternate
phenotype to type 2 PrPSc, the commonest sporadic CJD.
It is too early to be able to claim any further sub-classification in
respect of Heidenhain variant CJD or Vicky Rimmer's version. It will
take further studies, which are on-going, to establish if there are
sub-types to our initial finding which we are now reporting. The main
point of the paper is that, as well as leading to the expected new
variant CJD phenotype, BSE transmission to the 129-methionine genotype
can lead to an alternate phenotype which is indistinguishable from type
2 PrPSc.
I hope reading the paper will enlighten you more on the subject. If I
can be of any further assistance please to not hesitate to ask. Best wishes.
Emmanuel Asante
<> ____________________________________
Dr. Emmanuel A Asante MRC Prion Unit & Neurogenetics Dept. Imperial
College School of Medicine (St. Mary's) Norfolk Place, LONDON W2 1PG
Tel: +44 (0)20 7594 3794 Fax: +44 (0)20 7706 3272 email:
e.asante@ic.ac.uk (until 9/12/02)
New e-mail: e.asante@prion.ucl.ac.uk (active from now)
____________________________________
snip...
full text ;
http://www.fda.gov/ohrms/dockets/ac/03/slides/3923s1_OPH.htm
AND the new findings of BASE in cattle in Italy of Identification of a
second bovine amyloidotic spongiform encephalopathy: Molecular
similarities with sporadic
Creutzfeldt-Jakob disease
http://www.pnas.org/cgi/content/abstract/0305777101v1
Adaptation of the bovine spongiform encephalopathy agent to primates
and comparison with Creutzfeldt- Jakob disease: Implications for
human health
THE findings from Corinne Ida Lasmézas*, [dagger] , Jean-Guy Fournier*,
Virginie Nouvel*,
Hermann Boe*, Domíníque Marcé*, François Lamoury*, Nicolas Kopp [Dagger
] , Jean-Jacques Hauw§, James Ironside¶, Moira Bruce [||] , Dominique
Dormont*, and Jean-Philippe Deslys* et al, that The agent responsible
for French iatrogenic growth hormone-linked CJD taken as a control is
very different from vCJD but is similar to that found in one case of
sporadic CJD and one sheep scrapie isolate;
http://www.pnas.org/cgi/content/full/041490898v1
Characterization of two distinct prion strains
derived from bovine spongiform encephalopathy
transmissions to inbred mice
http://vir.sgmjournals.org/cgi/content/abstract/85/8/2471
ALL animals for human/animal consumption must be tested for TSE.
ALL human TSEs must be made reportable Nationally and Internationally, OF ALL AGES...TSS
Health BusinessQuestions linger in U.S. CJD casesBy STEVE MITCHELLSenior Medical Correspondent WASHINGTON, Oct. 21 (UPI) -- French researchers have ruled out the human form of mad cow disease in a deceased California man, even though they did not conduct the critical test widely regarded as the only way to determine precisely the nature of his disease, United Press International has learned. The case of Patrick Hicks, who died last November from his condition, has remained murky from the beginning. Dr. Ron Bailey, of Riverside, Calif., the man's neurologist, had suspected the 49-year-old Hicks of having contracted variant Creutzfeldt Jakob disease -- a fatal, brain-wasting illness humans can contract from eating beef products contaminated with the mad cow pathogen -- and both he and the family wanted an autopsy conducted to determine if Hicks had succumbed to the disorder. Bailey became concerned that Hicks might have contracted vCJD because he initially had exhibited psychiatric symptoms, his illness appears to have lasted for more than one year and he showed normal brain-wave patterns via EEGs until the late stages -- all consistent with the disease. In addition, Hicks's relatively young age raised concerns, because nearly all of the more than 150 cases of vCJD detected worldwide have occurred in people under age 55. The first hint of oddness began when, according to both Hicks's brother and mother, a team of six doctors, who they suspect were with the Centers for Disease Control and Prevention in Atlanta, visited Patrick last October while he was still alive and under care at Loma Linda University Medical Center in Loma Linda, Calif. They said they were asked to leave when the doctors arrived to examine Patrick. CDC officials would not confirm to UPI whether they had investigated the case, but the agency's policy does require examining all suspected cases of vCJD in anyone under 55. The family also said Loma Linda refused to released Hicks's medical records to them. The oddities continued after Hicks's death. Bailey found it almost impossible to get an autopsy conducted on Hicks, the only way to determine conclusively whether he had variant or sporadic CJD -- a version of the disease not related to mad cow. One county coroner's office referred him to another and both refused to conduct the procedure, he said. Then, the National Prion Disease Pathology Surveillance Center in Cleveland, Ohio -- which was established by the CDC to investigate potential vCJD cases in the United States -- dispatched a mobile autopsy company called 1-800-Autopsy, but the company failed to follow the center's protocol and did not collect frozen sections of brain, which are required for tests to determine whether the disease is vCJD or sCJD. Instead, the autopsy company fixed the entire brain in formalin. The NPDPSC, however, considers the collection of frozen brain tissue essential to distinguishing vCJD from other forms of CJD. "Only frozen brain tissue examination definitely confirms or excludes the diagnosis of prion disease and provides the information to identify the type of prion disease," the center's Web site says. Prions are abnormal proteins thought to play a role in causing vCJD and sCJD. The problem raised enough concern that both Bailey and Hicks's family sought a second opinion. Experts had told them that animal-injection studies could be done with formalin-fixed tissue, so the family arranged to have a sample of Patrick's brain sent to Dr. Jean Jacques Hauw at the Laboratoire De Neuropathologie at the Groupe Hospitalier Pitie-Salpetriere in Paris, who they thought had agreed to do the studies. The NPDPSC, however, delayed sending the sample to France for two months after the family's request last March. During the delay, Pierluigi Gambetti, the NPDPSC's director, sent a letter to Hicks's wife. "We can definitely rule out the diagnosis of variant CJD," the letter stated. Gambetti's strong conclusion sounded strange to Bailey, because the NPDPSC had not conducted further tests since January, when they had said vCJD was unlikely but that they were unable to rule it out entirely. After examining the brain tissue, Hauw's team told the family the disease was consistent with sCJD, but to date they have not explained why they did not conduct the animal-injection studies -- the family's reason for sending samples of his brain to France. Asked the reasons for not following the family's wishes and conducting the animal studies, Hauw told UPI, "I cannot answer your question," citing French regulations that prohibited him from providing information about a specific patient. He did say, however, that "animal injection is not needed for the routine diagnosis of Creutzfeldt-Jakob disease and its various variants, at least in France and in the United Kingdom." That may be true, but it remains unclear why he accepted the case in the first place, knowing that is what the family wanted. Moreover, this was not a "routine diagnosis." If Hicks suffered from vCJD, he potentially would have been the first person in the United States to have acquired the disease domestically, a development with significant domestic and international ramifications. In addition, other experts, such as Dr. Laura Manuelidis, section chief of surgery in the neuropathology department at Yale University, have said the only way to know conclusively whether the disease is due to sCJD or vCJD is through animal-injection studies. "From what I gather, the result was merely rubber stamped," Bailey told UPI. "I guess we will never really know for sure." The handling of the case is noteworthy, because the NPDPSC currently is investigating nine potential sCJD cases in Idaho. Experts suspect some of those cases could be vCJD. Bailey and some patient advocates said they are now skeptical of the NPDPSC's behavior. "How could my experience with the Hicks case ... and the interaction with NPDPSC not lessen my confidence?" Bailey asked. "I anticipate that all of the Idaho cluster of CJD patients will turn out to have sCJD. I cannot for a minute see their results indicating anything but this. After all, if any patient were to have vCJD, it would have been Patrick Hicks. The results of NPDPSC are not definitive in excluding Hicks as not having vCJD. There certainly will always be that question in my mind." Terry Singletary, a patient advocate whose mother died of a form of the disease called Heidenhain variant, told UPI he likewise had lost confidence in the NPDPSC. "I do not trust them," Singletary said. "It's all going to be sporadic. This is the way they want it. They do not want to find out all the routes and sources of this agent." Both vCJD and mad cow disease are politically sensitive issues because they can impact international trade. Dozens of nations closed their borders to American beef after a lone U.S. cow tested positive for the disease in 2003, resulting in more than $4.7 billion in losses for the industry, and the U.S. Department of Agriculture delayed doing confirmatory tests for seven months on what turned out to be a second case of mad cow. The NPDPSC did not respond to UPI's phone call requesting comment about the Idaho cases. The CDC referred UPI to Idaho officials. Of the nine Idaho cases, three people have tested positive for a CJD-like illness, but officials are conducting further tests to determine whether the disease is sCJD. Two others tested negative and four were buried without autopsies. The cases could just be a statistical fluke, but the state averages about 1.2 sCJD cases per year and has never had more than three in a single year. The disease is rare and generally is thought to occur at the rate of one case per million people. Several CJD clusters in other states have far exceeded that rate, however. These included: --southern New Jersey (2000-2003), --Lehigh, Pa. (1986-90), --Allentown, Pa. (1989-92), --Tampa, Fla. (1996-97), --Oregon (2001-02), and --Nassau County, N.Y. (1999-2000). Some of the clusters involved as many as 18 deaths, and ranged from a rate of four to eight cases per million people. A group of J.P. Morgan analysts issued an advisory last year on the impact the clusters could have on the beef industry, and said that some of the cases could be due to vCJD. "The existence of clusters raises the question of 'contamination' or 'infection,' and also raises the hypothesis that rather than cases of sCJD, these might have been cases of vCJD," the advisory said. "Given that sCJD occurs randomly in one out of 1 million cases, it is a statistical rarity to find an sCJD cluster -- let alone six." If that assessment is accurate, another cluster in Idaho would be even more unlikely. Another possibility is some of the Idaho cases could be due to chronic wasting disease, which is similar to mad cow disease and currently is epidemic among deer and elk in several states, including Idaho's neighbors Wyoming and Utah. No human cases of CWD have ever been confirmed, but the disease has been shown to infect human cells in a lab dish. Also, a team of researchers led by Jason Bartz of Creighton University in Omaha, Neb., report in the November issue of the Journal of Virology they had experimentally transmitted CWD to squirrel monkeys --the first reported transmission of CWD to primates. If CWD is capable of infecting humans, it is unknown whether the resulting disease would resemble sCJD, vCJD or a novel disorder. If the disease looks like sCJD, cases could be going undetected or misdiagnosed. -- E-mail: healthbiz@upi.com © Copyright 2005 United Press International, Inc. All Rights Reserved http://www.upi.com/HealthBusiness/view.php?StoryID=20051019-090103-6576 Questions linger in US CJD casesScience Daily (press release) - 12 hours ago... 21 (UPI) -- French researchers have ruled out the human form of mad cow disease in a deceased California man, even though they did not conduct the critical ... http://www.sciencedaily.com/upi/index.php?feed=Science&article=UPI-1-20051021-21481300-bc-us-cjdcases.xml Questions linger in US CJD casesMiddle East North Africa Financial Network, Middle East - 9 hours ago21 (UPI) -- French researchers have ruled out the human form of mad cow disease in a deceased California man, even though they did not conduct the critical ... http://www.menafn.com/qn_news_story.asp?StoryId=Cq1HNWeidDxmTy2PKy2fZzxm NOT TO FORGET THIS CLUSTER OF CJD IN TEXAS IN 1997 Creutzfeldt-Jakob Disease in Northeast Texas, J.A. Rawlings,*1 K.A.Hendricks1, O.M. Nuno1, D.A. Brown1, D.A. Evans2, Texas Department ofHealth, 1Austin and 2Tyler, Texas Creutzfeldt-Jacob Disease (CJD), a transmissible spongiformencephalopathy, is caused by prions composed of proteinaceous materialdevoid of nucleic acid. CJD occurs sporadically (generally 1case/1,000,000 population per year) in older patients (average age of65) and is characterized by rapidly progressive dementia, accompanied bysevere muscle spasms and incoordination. Death usually occurs within 3to 12 months (average 7 months). CJD activity in Texas, which has apopulation of nearly 19 million, appeared to be typical. The statewidedeath rate for 1995 and 1996 was just under 1/1,000,000. In April of1997, the Texas Department of Health became aware of an increased numberof possible CJD cases in a 23-county area of NE Texas with a populationof just over one million. After review of medical and pathology records,four patients were identified with definite classic CJD and three wereidentified with probable CJD. Dates of death for the eight patients werefrom April, 1996 through mid-July 1997. The patients were from 46through 65 years of age; four were male and three were female. Acase-control study to identify risks for CJD in NE Texas has been initiated. http://www.jifsan.umd.edu/tse/Rawlings.htm MY MOTHER DIED 12/14/97 OF THE HEIDENHAIN VARIANT OF CJD IN TEXAS... TSS I DON'T EXPECT EASTTEXASREVIEW TO PRINT THIS, BUT YOU HAVE THE OTHER SIDE OF THE STORY NOW, i only hope you use it. ...... thank you, I am sincerely, Terry S. Singeltary SR.P.O. Box 42Bacliff, Texas USA 77518
