[[PART 6]]
Subject: Re: 60% of US CJD never tested
Date: Tue, 30 Jan 2001 14:25:48 -0800
From: "Terry S. Singeltary Sr." <flounder@wt.net>
Reply-To: Bovine Spongiform Encephalopathy <BSE-L@uni-karlsruhe.de>
To: BSE-L@uni-karlsruhe.de
References: 1
######### Bovine Spongiform Encephalopathy
<BSE-L@UNI-KARLSRUHE.DE>
#########
Greetings Tom, and other List Members,
> classical forms of Creutzfeldt-Jakob disease,
> or C.J.D.
where do they suppose the source of these
classic forms of CJD come from ???
or do they expect us to believe that the
85% + cases of sporadic CJD come from
an overly abundant amount of people with
a problem of prions that are misfolding
on their own ???
> classical C.J.D. have died of the variant
> form instead
why is hvCJD, Heidenhain Variant CJD?
(somebody just screwed up, and now they
wish they had not...tss)
> have identified six types of sporadic C.J.D.,
> including a sporadic form of the
> disease that strikes young people.
and none of these cases of sporadic CJD
and apparently the new strains of sCJD,
were directly or indirectly tied to this environmental
human/animal TSE
blunder ???
all these new strains sporadic CJD just
a bunch of people that just happen to have
had a happen stance of bad luck with these
mutant naturally misfolding prions ???
I think i am going to be sick :-(
Does anyone out there honestly believe this ???
strainly confused,
Terry S. Singeltary Sr., Bacliff, Texas USA
tom wrote:
>
> ######### Bovine Spongiform Encephalopathy
<BSE-L@UNI-KARLSRUHE.DE> #########
>
> The main news is that 3 out of 5 cases of CJD
never reach Gambetti and
> never get characterized. Second, Gambetti
has defined two new strain types
> of CJD, including "including a sporadic form
of the
> disease that strikes young people."
>
> On Watch for Any Hint of Mad Cow Disease
>
> January 30, 2001
>
> By SANDRA BLAKESLEE
>
> If the human form of mad cow disease ó
or, in medical lingo, new
> variant Creutzfeldt-Jakob disease ó
is ever found in the United
> States, the first person to see it will probably
be Dr. Pierluigi
> Gambetti, a soft-spoken Italian scientist who
is on a mission to
> protect the American people from the devastating
disorder.
>
> Dr. Gambetti is a neuropathologist at
Case Western Reserve
> University in Cleveland and director of the
National Prion Disease
> Pathology Surveillance Center, a special laboratory
set up by the
> nation's pathologists and the Centers for Disease
Control and
> Prevention four years ago to watch for the
disease in the United
> States.
>
> During that time, Dr. Gambetti and his
colleagues have examined
> brain tissue from nearly 500 Americans who
died from unusual
> neurological conditions. Of these, 292 people
were found to have
> classical forms of Creutzfeldt-Jakob disease,
or C.J.D., a
> progressive neurological disorder characterized
by dementia and
> loss of motor control, which afflicts about
one in a million
> Americans a year. Others had brain tumors or
diseases that mimic
> C.J.D.
>
> So far, none has shown signs of the "variant"
C.J.D. seen in
> western Europe. Variant C.J.D. is a new disease,
Dr. Gambetti said.
> If it did appear, he said, "I would know it
instantly from looking
> at tissue samples. It would jump out at me."
>
> Variant C.J.D. is contracted from eating
beef products laced with
> infectious prions ó the aberrant proteins
believed to cause mad cow
> disease and all forms of human C.J.D. The cow
disease first
> appeared in Britain in the mid-1980's and has
since spread via
> contaminated animal feed to herds in France,
Germany, Portugal,
> Spain, Ireland, Switzerland, Belgium, Denmark,
Italy, the
> Netherlands, Luxembourg and Liechtenstein.
>
> The first human cases linked to eating
contaminated beef were
> diagnosed in 1996 in Britain. Thus far, 92
people have died or are
> dying from variant C.J.D. ó 88 in Britain,
3 in France and 1 in
> Ireland.
>
> While the death toll is small, many Europeans
are panicked because
> no one knows how many animals may be harboring
mad cow disease or
> how many people may be incubating variant C.J.D.
and passing it on
> to others through contaminated blood, organ
donations or
> contaminated surgical instruments (prions can
survive standard
> sterilization techniques).
>
> Mad cow disease has not been found in
any cattle in the United
> States. But fear and confusion have also spread
to the United
> States, said Dr. Ermias Belay, a medical epidemiologist
at the
> Centers for Disease Control and Prevention
in Atlanta.
>
> In part because of the similar terms
used to describe both
> ailments, many Americans mistakenly think people
they know who died
> of classical C.J.D. have died of the variant
form instead. For
> example, doctors may tell family members their
loved one died of a
> disease similar to mad cow disease. People
hear only the words "mad
> cow disease" and think that is what caused
the death, Dr. Belay
> said.
>
> Dr. Gambetti is an expert on prion diseases,
also known as
> transmissible spongiform encephalopathies,
that are caused when a
> normal prion protein folds into an abnormal
shape and no longer
> breaks down inside the body. Such misfolded
proteins produce havoc
> in nervous tissue, often leaving sponge-like
holes in many parts of
> the brain. When an animal or person eats or
is exposed to tissues
> containing abnormal prions, their own prion
proteins are converted
> into the deadly abnormal form. Prions can also
misfold on their own
> accord and give rise to disease. Occasionally,
genetic mutations
> lead to prion diseases that run in families.
>
> Given the natural incidence of prion
diseases, an estimated 280 to
> 300 Americans should be found to have some
form of C.J.D. each
> year, Dr. Gambetti said. It is very important
to look at all or
> most of those cases, he said, "so we don't
miss the atypical case
> or a new source of infection."
>
> Unfortunately, the surveillance center
is seeing only about 40
> percent of C.J.D. cases nationwide, Dr. Gambetti
said. If
> neuropathologists do an autopsy, tissue is
usually prepared
> properly and sent to the center for examination.
Dr. Gambetti sends
> a letter each year to every pathologist in
the country outlining
> the procedures.
>
> But many families, physicians and hospitals
are not aware of the
> surveillance center, Dr. Gambetti said. Or
if they are, they
> sometimes choose to not participate. Some families
cannot afford
> the cost of transporting the body to an autopsy
center. Some
> hospitals will not bear the cost of the brain
autopsy, which runs
> at least $1,500 per body, and pathologists
sometimes refuse to
> carry out an autopsy for fear of catching the
disease, even though
> safe procedures exist, Dr. Gambetti said.
>
> To remedy this, Dr. Gambetti said, he
would like expand the
> surveillance effort in the United States so
families and hospitals
> can be reimbursed for autopsies. Special nurses
might be trained to
> work with families while patients are still
alive to gather
> information about their habits.
>
> Surveillance centers in Canada, Britain
and other European
> countries work directly with families and neurologists,
he said,
> and are seeing most if not all cases of C.J.D.
in their respective
> nations. Each spends about a million dollars
a year to track a much
> smaller population. The United States surveillance
center has an
> annual budget of $100,000. A Congressional
appropriations committee
> recently authorized an additional $140,000
a year.
>
> Dr. Belay said that neither the Centers
for Disease Control nor
> Dr. Gambetti was authorized to reach out directly
to the nation's
> neurologists and general practitioners who
care for C.J.D. patients
> to increase surveillance efforts. States have
sole authority over
> their own medical practices, he said, and only
they can institute
> policies to track a disease like variant C.J.D.,
which has not
> turned up in this country.
>
> Many states, including New York, are
doing an excellent job of
> following C.J.D. trends and encourage their
doctors to send tissues
> to Dr. Gambetti, Dr. Belay said. Other states
are not doing much at
> all.
>
> Meanwhile, the Centers for Disease Control
and Prevention tracks
> prion diseases nationwide through death certificates,
Dr. Belay
> said. More than 85 percent of C.J.D. patients
die less than a year
> after their symptoms begin. They display a
set of diagnostic
> criteria that, by the end of life, are unmistakable.
>
> The centers believe that physicians do
a good job of describing
> the disease on death certificates, Dr. Belay
said. From 1979 to
> 1998, 4,751 deaths due to C.J.D. were reported
in the United
> States. The median age at death was 68.
>
> The centers follow up on any C.J.D. patient
who died under the age
> of 55, Dr. Belay said. This is because variant
C.J.D. tends to
> strike younger people and it is prudent to
check these cases more
> closely. Officials go through state health
departments to obtain
> clinical records on about 30 younger patients
a year, he said.
>
> They look for the clinical profile of
variant C.J.D., which begins
> with overt psychiatric symptoms and shows different
results on
> several standard brain assays. The variant
disease also lasts an
> average of 14 months. If brain tissue is available,
it is sent to
> Dr. Gambetti, who can confirm the diagnosis.
>
> Nevertheless, Dr. Gambetti said he was
worried because 60 percent
> of C.J.D. cases in this country are not being
examined with
> advanced tissue analysis techniques. Still,
the cases Dr. Gambetti
> has been to examine have been helpful. He and
his colleagues, by
> virtue of seeing so many cases in a short period,
have identified
> six types of sporadic C.J.D., including a sporadic
form of the
> disease that strikes young people.
>
> "We couldn't have known that without
seeing many cases," he said.
>
> ############ http://mailhost.rz.uni-karlsruhe.de/warc/bse-l.html
############
############ http://mailhost.rz.uni-karlsruhe.de/warc/bse-l.html
############
Terry S. Singeltary Sr.,
P.O. Box 42,
Bacliff, Texas USA 77518
281-559-2671
-----------------------