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From: TSS (216-119-132-66.ipset12.wt.net)
Subject: A VARIANT CREUTZFELDT-JAKOB DISEASE CLINICAL RESPONSE PLAN FOR AUSTRALIA
Date: February 2, 2005 at 12:52 pm PST

-------- Original Message --------
Subject: A VARIANT CREUTZFELDT-JAKOB DISEASE CLINICAL RESPONSE PLAN FOR AUSTRALIA
Date: Tue, 1 Feb 2005 20:59:25 -0600
From: "Terry S. Singeltary Sr."
Reply-To: Bovine Spongiform Encephalopathy
To: BSE-L@LISTSERV.KALIV.UNI-KARLSRUHE.DE


##################### Bovine Spongiform Encephalopathy #####################

A VARIANT CREUTZFELDT-JAKOB DISEASE CLINICAL RESPONSE PLAN FOR AUSTRALIA

Origins of vCJD
BSE was first reported as a new neurodegenerative disease of cattle in
the UK in 1986. At
that time, UK public health officials were of the view that BSE did not
pose a risk to human
health. As a precaution, enhanced surveillance was approved for human
TSEs to ensure any
risk to human health was detected. In March 1996, the UK reported 10
cases of a new
clinicopathological variant of CJD in adolescents and young adults under
the age of 40 years
believed to be associated with the consumption of beef products sourced
from BSE-affected
cattle.
As at December 2004, 165 cases of vCJD have been notified: 151 definite
and probable cases
in the UK and Northern Ireland, nine cases in France, two cases in the
Republic of Ireland
and single cases in Italy, Canada and the United States of America.
Almost all affected
individuals had multiple-year exposures in the UK between 1980 and 1996
during the
occurrence of a large outbreak of BSE among cattle in the UK.

Australias strategy to remain BSE and vCJD free...

snip...

Relationship to our response to classical CJD
The contingency plan for vCJD needs to be linked to response plans for
cCJD for two
reasons. Firstly, there will be a risk of diagnostic confusion,
especially for the public and the
media. Secondly, similar issues arise as a result of a diagnosis of cCJD
and that of vCJD in
terms of patient confidentiality and privacy, implications for the
individual and their
family/carers, infection control risks, legal liability, ethical
considerations and costs of health
care. Thus there is also a need for a nationally agreed process to
manage the risks associated
with cCJD which is diagnosed at a rate of approximately one to 1.5 case
per million
population per year.
Surveillance and notification of CJD is coordinated through the
Australian National CJD
Registry. The Registry also performs the following roles: providing
expert technical advice
on TSEs, assisting in determining the level of risk of surgical and
invasive diagnostic
procedures performed on patients at higher risk of CJD, managing
infection control-related
incidents and diagnosing CJD.
As the Australian National CJD Registry operates largely outside State
and Territory health
care structures, there is a need to ensure cooperation with the
State/Territory health
departments to ensure the following: ensuring appropriate notification
of cases of CJD,
reporting of infection control breaches identified by infection control
teams, the management
of legal issues associated with possible health care associated
transmission and the execution
of any transmission risk investigations.
Conclusion
The following principles underpin a coordinated national approach in the
event of a case of
vCJD diagnosed in Australia.
- Preparedness in the event of a rare biological emergency.
- Coordination of policy and operational arms at a State/Territory and
national level,
A variant CJD Clinical Response Plan for Australia
including agreement on roles and responsibilities and who needs to know.
This will
require considered operational plans based on the best evidence
currently available and
a process of consultation between States/Territories, the Australian
Government, the
National CJD Registry and SECTSE. The development of jurisdictional as
well as the
national response plan may be required, linked to issue specific
response plans already
developed or under development.
- Australia continues to be BSE-free. On 8 September 2004, the European
Food Safety
Agency issued a report concluding that the GBR (Geographical BSE Risk)
status of
Australia is maintained at level 1; it is highly unlikely that domestic
cattle are
(clinically or pre-clinically) infected with the BSE-agent.
- Regular communication between key policy and operational stakeholders.
These lines
of communication should be established now and have the ability to deal with
interactions with the media and media speculation.

snip...

Disease Surveillance in Humans
In late 2003, the Communicable Diseases Network Australia (see below)
endorsed the
addition of Creutzfeldt-Jakob Disease (CJD) to the list of diseases to
be notifiable nationally.
To date, New South Wales, Victoria and Western Australia have included
CJD and vCJD on
their State registers of notifiable diseases. The balance of the States
and Territories are
expected to follow. Registration of CJD as a nationally notifiable
disease will allow
authorities to monitor cases of CJD in Australia.


http://www.health.gov.au/internet/wcms/publishing.nsf/650f3eec0dfb990fca25692100069854/a90c01f5d7befd27ca256f190004794a/%24FILE/clinical.pdf


SEEMS they are still dreaming of the old days...


REPORTS

Human Prion Protein with
Valine 129 Prevents Expression
of Variant CJD Phenotype

snip...

Although caution must be exercised in
extrapolating from animal models, even
where, as here, faithful recapitulation of
molecular and pathological phenotypes is
possible, our findings argue that primary
human BSE prion infection, as well as sec-
ondary infection with vCJD prions by iatro-
genic routes, may not be restricted to a single
disease phenotype. These data, together with
the recent recognition of probable iatrogenic
transmission of vCJD prions to recipients of
blood {21, 22), including a PRNP codon 129
Met/Val heterozygous individual (22), re-
iterate the need to stratify all human prion
disease patients by PrPSc type. This surveil-
lance will facilitate rapid recognition of novel
PrPSc types and of any change in relative
frequencies of particular PrPSc subtypes in
relation to either BSE exposure patterns or
iatrogenic sources of vCJD prions.

www.sciencemag.org SCIENCE VOL 306 3 DECEMBER 2004

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


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


Ref: MRC/62/04

Under strict embargo until 19.00 British Time Thursday 11 November 2004


GENETIC MAKE-UP MAY DETERMINE WHAT TYPE OF CJD OCCURS WHEN HUMANS ARE
INFECTED WITH BSE

New research published today (19.00 hours Thursday 11th November) by a
team from the Medical Research Council (MRC) Prion Unit offers an
explanation about why only people with a particular genetic make-up have
so far developed vCJD. It also provides evidence that other types of
BSE-derived prion infection with a different pattern of symptoms might
occur in humans. The findings are published in the journal Science.

Variant CJD (vCJD) is the human disease thought to be caused by eating
food contaminated with the infectious agent, known as a prion,
responsible for the epidemic of BSE or mad cow disease in cattle. So
far, everyone known to have developed vCJD has been of a particular
genetic type  known as MM. Until now it has been a mystery why everyone
that has developed vCJD is of the MM type and one possibility is that
they are simply the first to develop the disease when infected with BSE,
and that people with the other genetic types1 (known as VV and MV)
infected with BSE prions will also develop vCJD, but some years later.

In a series of experiments spanning more than ten years, the MRC team
has been studying mice genetically modified so that they make human
prion proteins  which are used to model human susceptibility to BSE.
The team has now shown that mice with the human VV genetic type do
become infected when given BSE or vCJD prions, but manifest a different
form of the disease which looks quite different to vCJD and has a novel
prion strain type.

Remarkably, when these novel prions were used to infect mice of the MM
genetic type, the mice either developed a disease very like vCJD, or
else a pattern of disease that looks like so-called sporadic CJD  the
classical form of CJD. This form has been known about for many years,
is seen all over the world and has not hitherto been associated with
BSE. However, the new strain identified in the mice, being called type
5, has not been seen yet in people and we do not know what pattern of
disease it would cause. It could look like one of the forms of classical
or sporadic CJD or perhaps be yet another different variant form.

The work from the MRC team suggests that type 4 prions, the type
associated with vCJD, can only propagate themselves in people that make
the M form of the protein. It seems the V form of the protein just
cannot adopt the particular molecular shape that characterises type 4.

The studies in mice also suggest that if these prions were to pass from
person to person (for example by blood transfusion) then, depending on
the genetic type of the person becoming infected, at least three
different patterns of disease might result: type 2 (which is seen in
sporadic CJD); type 4 (which causes vCJD) or type 5 (which may cause a
new pattern of disease).

Professor John Collinge, Director of the MRC Prion Unit, which is based
at University College London, said: These mouse studies give us vital
clues about the behaviour of prions and how they appear to modify and
adapt depending on the genetic makeup of the individual they are infecting.

We always have to be cautious about making direct comparison to the
human condition, but our work strongly suggests that we can not assume
only those with one genetic profile are vulnerable to BSE infection.

At this stage it is not possible to say how this should alter estimates
of those likely to become ill, but our findings do suggest we should be
taking steps to draw up a more sophisticated system of categorizing the
disease so that we dont mistake BSE related infection for a version of
sporadic CJD.

ENDS

For more information call the MRC press office on 020 7 637 6011

Notes to Editors

1The human prion protein comes in two common forms, known as M and V.
Because everyone has two copies of this gene, there are three possible
genetic types: MM, MV and VV.

Paper - Human Prion protein v129 prevent expression of vCJD phenotype 
Science On line 11.11.04

Prions are rogue forms of one of the bodys own proteins  known as the
prion protein  which are misshapen. There are several different rogue
or misshapen forms that can infect humans, and these different types of
prions are known as strains. This is analogous to different strains of
other germs such flu virus causing influenza or strains of salmonella
causing different forms of food poisoning for example.

The strain of prion causing vCJD is known as type 4, types 1-3 cause the
different forms of sporadic or classical CJD. Each strain causes a
different pattern or type of disease. It is known that prion strains can
change or mutate when they pass between different animals.

The Medical Research Council (MRC) is a national organisation funded by
the UK tax-payer. Its business is medical research aimed at improving
human health; everyone stands to benefit from the outputs. The research
it supports and the scientists it trains meet the needs of the health
services, the pharmaceutical and other health-related industries and the
academic world. MRC has funded work which has led to some of the most
significant discoveries and achievements in medicine in the UK. About
half of the MRCs expenditure of £430 million is invested in its 40
Institutes, Units and Centres. The remaining half goes in the form of
grant support and training awards to individuals and teams in
universities and medical schools. Web site at: http://www.mrc.ac.uk
.

=======================================

AS implied in the Inset 25 we must not _ASSUME_ that
transmission of BSE to other species will invariably
present pathology typical of a scrapie-like disease.

snip...

http://www.bseinquiry.gov.uk/files/yb/1991/01/04004001.pdf

TSS

######### https://listserv.kaliv.uni-karlsruhe.de/warc/bse-l.html ##########






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