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From: TSS ()
Subject: Doctors want ‘opt-out’ system for organ donors $$$TSE$$$
Date: November 26, 2005 at 6:52 pm PST

Doctors want ‘opt-out’ system for organ donors


Health: By Judith Duffy, Health Correspondent

DOCTORS have warned that new legislation on organ donation in Scotland is a missed opportunity to help the increasing numbers of people waiting for transplants.
The Human Tissue (Scotland) Bill, due to be debated by MSPs this week, proposes to strengthen the present “opt-in” system by placing more emphasis on following an individual’s wishes on organ donation after their death.

It will also tighten up rules on postmortems and the retention of body parts after scandals such as that surrounding Alder Hey children’s hospital in Liverpool, where organs were kept for research without parental consent.

But the British Medical Association (BMA) in Scotland has said the organ donation proposals contained in the bill do not go far enough. It is calling for a system of presumed consent to be introduced to increase the number of organs available. This would allow donation to automatically proceed unless someone had registered their objections, or their relatives opposed it.

According to the BMA, between April 2004 and March this year 52 Scots died while waiting for an organ transplant. Others have died without even reaching the waiting list. While more than 90% of the population supports donation, just over a fifth have actually signed up to the organ donor register.

Dr Peter Terry, chairman of the BMA in Scotland, argued there was greater public support for organ donation and transplantation than for the recent ban on smoking in public places.

But he said: “At the time of death, especially with a young person in a road traffic accident or whatever, it is very, very difficult for all concerned to ask relatives, and for the relatives to make a decision if they haven’t discussed it or know the wishes of the individual.”

He added: “The primary reason [for presumed consent] is to get more organs for the people who need them and it also, I think, would make it much easier for the relatives.”

Terry said that in countries where such an “opt-out” system had been introduced, there had been a substantial increase in the number of organs available for transplant and public backing for the move.

“We certainly don’t want to push or persuade anyone who is unhappy about organ donation,” he said. “We don’t in any way want to influence those individuals, it is their right to choose what happens, but because people die without thinking about it an awful lot of organs are lost.”

Similar legislation introduced recently south of the Border ruled out a presumed consent system, but Terry argued this was an opportunity for Scotland to lead the way.

“If the Scottish Executive doesn’t think there is public support for it at this stage, then I would like it to prove that to us,” he said. “But I don’t think that is true. We’re satisfied that there is great public support for this, we think others are just making a gut reaction-type judgement.”

A spokeswoman for the Scottish Executive said it shared the BMA’s concerns and had launched awareness campaigns to try to address the problem. But she added: “We know from the public reaction to recent organ donation issues that, for many people, presumed consent does not count as a valid form of consent.”

Other medics believe there should be greater discussion of the issue, however. Dr Mairi Scott, chairwoman of the Royal College of GPs Scotland, said that while she understood the reasons for presumed consent, it was a “difficult area”.

“I personally don’t think we have had sufficient public debate on this issue,” she said. “At the moment we don’t have an opinion either way, but what we do want is to make sure that the public has that chance.”

The Scottish parliament’s health committee last week backed the bill. Convener Roseanna Cunningham MSP said that although there was some debate about the merits of presumed consent, the majority of members had agreed the case for introducing such a system had not been made.

She said: “It was, I think, quite rightly pointed out that when we talked about presumed consent a lot of the medical profession would be wary of it because of concerns about what was the Alder Hey scenario.”

Under the new bill, the current system of consent – where relatives are asked if they want to donate the organs of their loved ones – will be replaced by one of authorisation.


Adults and children over 12 will authorise the removal and use of their body parts after their death by signing up to the organ donor register or carrying a donor card, for instance. If the person died without giving instruction, their nearest relatives will be asked what the person would have wanted and can authorise the donation.

Cunningham said it would be vital to have widespread awareness campaigns to ensure the public knew the importance of expressing their wishes.

She said: “They will have to make sure this change does get across as it is going to be incumbent on surviving relatives to basically be indicating what the person’s views are, so it is very important to make views well known.”

The new legislation will also revise the rules on postmortems and the retention of body parts . Authorisation will be essential for hospital postmortem examinations, and will be required if organs are to be retained and used for research or training.

Parents of children whose organs were retained without permission in the past have welcomed the bill. Geraldine MacDonald, chairwoman of the Scottish Organisation Relating to the Retention of Organs, said it would ensure such scandals did not happen again. “It will be clarity for everybody ,” she said.

However, one expert has warned vital research could be hampered by the consent requirements. Professor Sheila Bird, of the Medical Research Council, argued testing for vCJD at postmortems should be allowed without the permission of the deceased or their family so that the prevalence of the disease in the general population can be assessed.

“The testing for late disease that was not overtly clinical was extremely important in cattle because it showed in the UK we had about three times as many BSE infections as we originally thought,” she said. “There is a concern that … reliance on just clinical cases to tell us about these epidemics is insufficient.”

27 November 2005

http://www.sundayherald.com/53052

Greetings,

>>>Dr Peter Terry, chairman of the BMA in Scotland, argued there was greater public support for organ donation and transplantation than for the recent ban on smoking in public places.

But he said: “At the time of death, especially with a young person in a road traffic accident or whatever, it is very, very difficult for all concerned to ask relatives, and for the relatives to make a decision if they haven’t discussed it or know the wishes of the individual.” >>>

or, you can look at it from another point of view ;

Lets look at a hypothetical situation: What would happen if my Mom (DOD 12-14-97 hvCJD) would have gotten into a car wreck and died, before the symptoms of CJD appeared. Not much money, so there was no autopsy. What would have happened to that recipient of those infecting corneas?"

http://www.mad-cow.org/~tom/dec99_news.html#bbb

>>>However, one expert has warned vital research could be hampered by the consent requirements. Professor Sheila Bird, of the Medical Research Council, argued testing for vCJD at postmortems should be allowed without the permission of the deceased or their family so that the prevalence of the disease in the general population can be assessed.

“The testing for late disease that was not overtly clinical was extremely important in cattle because it showed in the UK we had about three times as many BSE infections as we originally thought,” she said. “There is a concern that … reliance on just clinical cases to tell us about these epidemics is insufficient.” <<<

they must include all human TSEs, or there just kidding themselves.

and whom are they kidding anyway, with this UK/BSE/nvCJD only theory $

Coexistence of multiple PrPSc types in individuals with

Creutzfeldt-Jakob disease

Magdalini Polymenidou, Katharina Stoeck, Markus Glatzel, Martin Vey, Anne Bellon, and Adriano Aguzzi

Summary

Background The molecular typing of sporadic Creutzfeldt-Jakob disease (CJD) is based on the size and glycoform

ratio of protease-resistant prion protein (PrPSc), and on PRNP haplotype. On digestion with proteinase K, type 1 and

type 2 PrPSc display unglycosylated core fragments of 21 kDa and 19 kDa, resulting from cleavage around amino

acids 82 and 97, respectively.

Methods We generated anti-PrP monoclonal antibodies to epitopes immediately preceding the differential proteinase

K cleavage sites. These antibodies, which were designated POM2 and POM12, recognise type 1, but not type 2, PrPSc.

Findings We studied 114 brain samples from 70 patients with sporadic CJD and three patients with variant CJD.

Every patient classified as CJD type 2, and all variant CJD patients, showed POM2/POM12 reactivity in the

cerebellum and other PrPSc-rich brain areas, with a typical PrPSc type 1 migration pattern.

Interpretation The regular coexistence of multiple PrPSc types in patients with CJD casts doubts on the validity of

electrophoretic PrPSc mobilities as surrogates for prion strains, and questions the rational basis of current CJD

classifications.

snip...

The above results set the existing CJD classifications

into debate and introduce interesting questions about

human CJD types. For example, do human prion types

exist in a dynamic equilibrium in the brains of affected

individuals? Do they coexist in most or even all CJD

cases? Is the biochemically identified PrPSc type simply

the dominant type, and not the only PrPSc species?

http://neurology.thelancet.com Published online October 31, 2005

03-19-2004, 12:13 PM

-------- Original Message --------
Subject: Ophthalmic surgery and Creutzfeldt-Jakob disease
Date: Fri, 19 Mar 2004 09:39:43 -0600
From: "Terry S. Singeltary Sr."
Reply-To: Bovine Spongiform Encephalopathy
To: BSE-L@uni-karlsruhe.de


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

April 2004; Vol. 88, No. 4

URL: http://www.bjophthalmol.com/content/vol88/issue4/index.shtml?etoc


Series editor: David Taylor
. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
. . . . . . . . . . . . . . .
Ophthalmic surgery and Creutzfeldt-
Jakob disease
P S-Juan, H J T Ward, R De Silva, R S G Knight, R G Will
. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
. . . . . . . . . . .
Although the evidence does not suggest that contaminated
ophthalmic instruments represent a risk of onward transmission of
sporadic CJD, this conclusion should be treated with caution
The occurrence of variant
Creutzfeldt-Jakob disease (vCJD)
and the probable causal link with
bovine spongiform encephalopathy
(BSE) in cattle have increased interest
in the search for possible environmental
sources of sporadic CJD (sCJD).
Presumed iatrogenic CJD is rare. Up to
the year 2000 there had been 267 cases
reported worldwide: three cases secondary
to human corneal grafting (one
confirmed, one probable, and one possible
case), 114 related to human dura
mater grafts, 139 related to human
growth hormone treatment, four related
to human pituitary gonadotrophin therapy,
and seven linked to neurosurgical
procedures or stereotactic EEG electrodes.
1 Because of the marked resistance
of the infectious agent of CJD to
conventional sterilisation techniques,
there is concern about the possibility of
transmission of infection via surgical
instruments in contact with infected
tissue, especially in neurosurgery or
ophthalmic surgery.

SNIP...TO LONG TO POST HERE, PLEASE VIEW FULL TEXT HERE;


http://www.vegsource.com/talk/madcow/messages/92120.html


a bit of history;

The Eyes have it/CJD * and they could be stealing them from YOUR loved
one, hence the
spread of CJD (aka MADCOW DISEASE) will spread...

1


http://www.vegsource.com/talk/madcow/messages/7242.html

2



THE LEGALITY OF STEALING ORGAN/TISSUE...

TEXAS STATUTES

Sec. 693.012. Removal of Corneal Tissue Permitted Under Certain
Circumstances.

On a request from an authorized official of an eye bank for corneal
tissue, a justice of the peace or medical examiner may permit the
removal of corneal tissue if:

(1) the decedent from whom the tissue is to be removed died under
circumstances requiring an inquest by the justice of the peace or
medical examiner;

(2) no objection by a person listed in Section 693.013 is known by the
justice of the peace or medical examiner; and

(3) the removal of the corneal tissue will not interfere with the
subsequent course of an investigation or autopsy or alter the decedent's
postmortem facial appearance.

Acts 1989, 71st Leg., ch. 678, Sec. 1, eff. Sept. 1, 1989.

Note: This information includes legislation enacted through the 75th
Congress. The 76th session of the Texas Legislature has concluded. The
State of Texas has not yet made the new codes available to the public.
Until they do, search the bill text for any changes or amendments.

Search 1999 Legislation for: 693.012
--------------------------------------------------------
TEXAS STATUTES
Sec. 693.003. Consent Required in Certain Circumstances.

(a) A medical examiner or a person acting on the authority of a medical
examiner may not remove a visceral organ unless the medical examiner
or person obtains the consent of a person listed in Section 693.004.

(b) If a person listed in Section 693.004 is known and available within
four hours after death is pronounced, a medical examiner or a person
acting on the authority of a medical examiner may not remove a
nonvisceral organ or tissue unless the medical examiner or person
obtains that person's consent.

(c) If a person listed in Section 693.004 cannot be identified and
contacted within four hours after death is pronounced and the medical
examiner determines that no reasonable likelihood exists that a person
can be identified and contacted during the four-hour period, the medical
examiner may permit the removal of a nonvisceral organ or tissue.

Acts 1989, 71st Leg., ch. 678, Sec. 1, eff. Sept. 1, 1989.

Note: This information includes legislation enacted through the 75th
Congress. The 76th session of the Texas Legislature has concluded. The
State of Texas has not yet made the new codes available to the public.
Until they do, search the bill text for any changes or amendments.

Search 1999 Legislation for: 693.003
--------------------------------------------------------
PLEASE NOTE; the bottom would only pertain to those who know of the
law. if you don't know about it, you cannot dispute, so in four hours,
they can legally remove body organs, as long as they don't disfigure.
and who is to know the difference? makes me wonder of some of my dead
relatives, and if they were burried with their eye's and or any of their
organs. This is very disturbing, if not for moral reasons, but for the
risk of dangerous pathogens (human TSE's, etc.) to be transmitted. only
time will tell, but i am very disturbed.
these laws are not morally correct. They should be re-written as to they
cannot so easily take your organs, with no one knowing. The Family or
Victim, must consent. There should be some kind of research
on donor/family medical history...TSS
--------------------------------------------------------

Sec. 693.013. Persons Who May Object to Removal.

The following persons may object to the removal of corneal tissue:

(1) the decedent's spouse;

(2) the decedent's adult children, if there is no spouse;

(3) the decedent's parents, if there is no spouse or adult child; or

(4) the decedent's brothers or sisters, if there is no spouse, adult
child, or parent.

Acts 1989, 71st Leg., ch. 678, Sec. 1, eff. Sept. 1, 1989.

Note: This information includes legislation enacted through the 75th
Congress. The 76th session of the Texas Legislature has concluded. The
State of Texas has not yet made the new codes available to the public.
Until they do, search the bill text for any changes or amendments.

Search 1999 Legislation for: 693.013
-------------------------------------------------------
to cover one's butt....

Sec. 693.014. Immunity From Damages in Civil Action.

(a) In a civil action brought by a person listed in Section 693.013 who
did not object before the removal of corneal tissue, a medical examiner,
justice of the peace, or eye bank official is not liable for damages on
a theory of civil recovery based on a contention that the person's
consent was required before the corneal tissue could be removed.

(b) Chapter 104, Civil Practice and Remedies Code, applies to a justice
of the peace, medical examiner, and their personnel who remove, permit
removal, or deny removal of corneal tissue under this subchapter as if
the justice of the peace, medical examiner, and their personnel were
state officers or employees.

Acts 1989, 71st Leg., ch. 678, Sec. 1, eff. Sept. 1, 1989.

Note: This information includes legislation enacted through the 75th
Congress. The 76th session of the Texas Legislature has concluded. The
State of Texas has not yet made the new codes available to the public.
Until they do, search the bill text for any changes or amendments.

Search 1999 Legislation for: 693.014

[[[as you can see, they knew it was wrong when they wrote the laws. or
they would not have covered the rear-ends so well...TSS]]]
---------------------------------------------------------
thanks again,
kind regards,
Terry S. Singeltary Sr.

############ http://mailhost.rz.uni-karlsruhe.de/warc/bse-l.html ###########

http://www.vegsource.com/talk/madcow/messages/7643.html

3


http://www.vegsource.com/talk/madcow/messages/7708.html

http://jama.ama-assn.org/issues/v282n23/full/jlt1215-5.html

http://mad-cow.org/~tom/dec99_news.html#bbb

Testimony of Bess Believeaux, Lions Eye Bank of Central Texas
(Submission to the Jan. 18/19 meeting of the
TSE Advisory Committee)

http://www.fda.gov/ohrms/dockets/ac/01/slides/3681s2_16.pdf

TSS Submission to the same Committee;

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

Tissue Banks International (TBI), Gerald J Cole

http://www.fda.gov/ohrms/dockets/ac/01/slides/3681s2_13.pdf

re-use contact lenses


http://www.vegsource.com/talk/lyman/messages/7691.html

TSS

########### http://mailhost.rz.uni-karlsruhe.de/warc/bse-l.html ############

Prion disease: December 1999 News
It is very unlikely that familial CJD would have been diagnosed in earlier ...
"In our eyes we have done nothing wrong, because there is no single piece of ...
www.mad-cow.org/dec99_news.html - 45k

http://www.mad-cow.org/dec99_news.html#bbb

Donated Human Tissues May Contain CJD/Mad Cow Prions Families Of ...
From: TSS (216-119-130-114.ipset10.wt.net); Subject: re-The Eyes Have It (cjd)
and they could be stealing them from; your loved one. ...
www.rense.com/general62/don.htm - 59k -

Journal of Virology, February 2005, p. 1888-1897, Vol. 79, No. 3 0022-538X/05/$08.00+0 DOI: 10.1128/JVI.79.3.1888-1897.2005 Copyright © 2005, American Society for Microbiology. All Rights Reserved. Neuroinvasion by Scrapie following Inoculation via the Skin Is Independent of Migratory Langerhans Cells Joanne Mohan, Moira E. Bruce, and Neil A. Mabbott*

Neuropathogenesis Unit, Institute for Animal Health, Edinburgh, Scotland, United Kingdom

Received 18 June 2004/ Accepted 7 September 2004

Many natural transmissible spongiform encephalopathy (TSE) infections are likely to be acquired peripherally, and studies in mice show that skin scarification is an effective means of scrapie transmission. After peripheral exposure, TSE agents usually accumulate in lymphoid tissues before spreading to the brain. The mechanisms of TSE transport to lymphoid tissues are not known. Langerhans cells (LCs) reside in the epidermis and migrate to the draining lymph node after encountering antigen. To investigate the potential role of LCs in scrapie transportation from the skin, we utilized mouse models in which their migration was blocked either due to CD40 ligand deficiency (CD40L/ mice) or after caspase-1 inhibition. We show that the early accumulation of scrapie infectivity in the draining lymph node and subsequent neuroinvasion was not impaired in mice with blocked LC migration. Thus, LCs are not involved in TSE transport from the skin. After intracerebral inoculation with scrapie, wild-type mice and CD40L/ mice develop clinical disease with similar incubation periods. However, after inoculation via skin scarification CD40L/ mice develop disease significantly earlier than do wild-type mice. The shorter incubation period in CD40L/ mice is unexpected and suggests that a CD40L-dependent mechanism is involved in impeding scrapie pathogenesis. In vitro studies demonstrated that LCs have the potential to acquire and degrade protease-resistant prion protein, which is thought to be a component of the infectious agent. Taken together, these data suggest that LCs are not involved in scrapie transport to draining lymphoid tissues but might have the potential to degrade scrapie in the skin.

* Corresponding author. Mailing address: Institute for Animal Health, Neuropathogenesis Unit, Ogston Bldg., West Mains Rd., Edinburgh EH9 3JF, United Kingdom. Phone: 44(0)131-667-5204. Fax: 44(0)131-668-3872. E-mail: neil.mabbott@bbsrc.ac.uk.

Journal of Virology, February 2005, p. 1888-1897, Vol. 79, No. 3
0022-538X/05/$08.00+0 DOI: 10.1128/JVI.79.3.1888-1897.2005
Copyright © 2005, American Society for Microbiology. All Rights Reserved.

http://jvi.asm.org/cgi/content/abstract/79/3/1

Scrapie transmission following exposure through the skin is
dependent on follicular dendritic cells in lymphoid tissues

Joanne Mohan, Karen L. Brown, Christine F. Farquhar, Moira E. Bruce and
Neil A. MabbottCorresponding Author Contact Information
,

E-mail The Corresponding Author

Institute for Animal Health, Ogston Building, West Mains Road, Edinburgh
EH9 3JF, UK

Received 9 March 2004; Revised 22 April 2004; accepted 12 May 2004.
Available online 8 July 2004.


Abstract

Background: Transmissible spongiform encephalopathies (TSEs) are chronic infectious neurodegenerative diseases that are characterized by the accumulation in affected tissues of PrPSc, an abnormal isoform of the host prion protein (PrPc). Following peripheral exposure, PrPSc usually accumulates on follicular dendritic cells (FDCS) in lymphoid tissues before neuroinvasion. Studies in mice have shown that TSE exposure through scarified skin is an effective means of transmission. Following inoculation via the skin, a functional immune system is critical for the transmission of scrapie to the brain as severe combined immunodeficiency (SCID) mice are refractory to infection. Until now, it was not known which components of the immune system are required for efficient scrapie neuroinvasion following skin scarification. Objective: To determine which cells are critical for the transmission of scrapie to the brain following inoculation via the skin. Methods: A chimeric mouse model was used, which had a mismatch in PrPc expression between FDCs and other bone marrow-derived cells within lymphoid tissues. These chimeric mice were challenged with scrapie by skin scarification to allow the separate roles of FDCs and lymphocytes in peripheral scrapie pathogenesis to be determined. Results: We show that mature FDCs are essential for the accumulation of scrapie within lymphoid tissues and the subsequent transmission of infection to the brain following TSE exposure by this route. Furthermore, we show that the accumulation of PrPSc and infectivity in the spleen is independent of PrP expression by lymphocytes or other bone marrow-derived cells. Conclusion: Following inoculation with scrapie by skin scarification, replication in the spleen and subsequent neuroinvasion is critically dependent upon mature FDCs.

Author Keywords: Transmissible spongiform encephalopathy; Scrapie; Skin;
Follicular dendritic cell; Prion protein; Spleen


Corresponding Author Contact Information
Corresponding

author. Tel.: +44 131 667 5204; fax: +44 131 668 3872.

http://www.sciencedirect.com/science?_


Extraneural Pathologic Prion Protein in Sporadic Creutzfeldt-Jakob Disease

snip...

Conclusions Using sensitive techniques, we identified extraneural deposition of PrPSc in spleen and muscle samples from approximately one third of patients who died with sporadic Creutzfeldt-Jakob disease. Extraneural PrPSc appears to correlate with a long duration of disease.

http://content.nejm.org/cgi/

Prions in skeletal muscle (Prusiner et al)

http://www.pnas.org/cgi

The Belgian cow's results were:

ELISA +
SAF -
HP -
IHC -
WB +



NOTHING, this is part of june 2004 usda enhanced bse/tse cover-up. part of the program was to start NOT confirming with WB, due to the first confirmed finding. same with the other mad cows in TEXAS i.e. the stumbling and staggering one they refused to test and rendered;

http://www.npr.org/dmg/dmg.php?pr

http://www.fda.gov/bbs/topics/news/2004/NEW01061.html


http://www.house.gov13_let.pdf


IF we look at this Belgium atypical cow (and try to forget about those damn imported belgium sheep that were never confirmed with mouse bio assay, as we were told they would be, which could very well have been BSE, i mean there was a declaration of emergency declared for an ATYPICAL TSE in them), and then look at the #8 and #9 cow of Japan;

8. 6/10/2003 Holstein Steer 13/10/2001 23 mths
No clinical signs WB+, IHC-, HP-


9. 4/11/2003 Holstein Steer 13/1/2002
21 mths No clinical signs WB+, IHC-, HP-

THIS explains very well why the USDA decided to NOT use WB anymore.
damn thing finds things when people don't want it found. simple as that.

http://www.ngpc.state.ne.us/cgi-bin/

http://www.jc-press.com/En/Latest%20News/20

Last modified, 11/09/2004 13:42:49

BSE death cow's anomalous prion detected from peripheral nerve tissue,
suprarenal gland

First time from non-Specified Risk Material, or SRM

By JCPRESS

National Institute of Animal Health Animal announced on November 1 that it had detected the anomalous prion protein that was the etiologic agent of the mad cow disease, or BSE, or bovine spongiform encephaalopathy, from the peripheral nerve tissue and the suprarenal gland of the cow of the age in the mad cow disease for the dying infection 94 months on March 9 this year. Japan is obligating the removal of the Specified Risk Material, or SRM such as the head, the spinal cord, the vertebral columns, and the small intestines that accumulate the anomalous prion protein easily as a BSE (bovine spongiform encephaalopathy) measures. Because the mad cow disease etiologic agent was detected from a tissue different from the Specified Risk Material, or SRM, the review of the Specified Risk Material, or SRM might be urged on the Japanese Government. International Symposium of PRION DISEASES for food and drug safety

http://www.knt.co.jp/ec/2004/prion/
national institute of animal health(only in Japanese)
http://niah.naro.affrc.go.jp/index-j.html
The statement of the Ministry of Health, Labour and Welfare
(only in Japanese)
http://www.maff.go.jp/www/press/cont2/20041101press_7.htm
Yomiuri on line (only in Japanese)
http://www.yomiuri.co.jp/science/news/20041102i503.htm
Asahi on line(only in Japanese)
http://www.asahi.com/special/bse/TKY200411010291.html
Mainichi on line(only in Japanese)
http://www.mainichi-msn.co.jp/shakai/jiken/disease/news/
20041102ddm041040128000c.html

ORAL 8

Bovine spongiform encephalopathy (BSE) in Japan

Takashi Yokoyama, Kumiko M. Kimura, Morikazu Shinagawa
Prion Disease Research Center, National Institute of Animal Health, Japan

Bovine spongiform encephalopathy (BSE) has become an important problem not only for animal industry, but also for public health. In Japan, BSE was first recognized in September 2001 by fallen stock surveillance. Since October 2001, BSE examination for all cattle slaughtered at abattoirs has started. In April 2004, all dead cattle examination (over 24 months) has been conducted at livestock hygiene service center. Samples positive in enzyme linked immunosorbent assay (ELISA) are further subjected to western blot (WB) and immunohistochemistry (IHC). Thirteen BSE cases have been reported by September 2004. Twelve cases were classified as typical BSE, and the remained one was an atypical BSE. Variant forms of BSE with atypical histopathological and/or biochemical phenotype were reported in Italy and France. Further study is required for BSE prion characteristics. To characterize BSE prion properties, brain homogenates of Japanese BSE cases were intracerebrally inoculated into wild-type mice. The first case (BSE/Chiba) was successfully transmitted to rodents. The mean incubation periods (409.0 days) in this experiment was preferably longer than that of previously reported. PrPSc distribution, prion titer, mice susceptibility and/or storage condition of sample might be influenced the result. Recently, we introduced transgenic mice that overexpress a bovine PrP gene to overcome the species barrier problem. These mice are expected to accelerate the transmission experiment of BSE prion. Transmission of atypical BSE case is undergoing by using these transgenic mice.

Research Foundation

http://www.knt.co.jp/ec/2004/prion/E2.htm

Tissue distribution of protease resistant prion protein in variant
Creutzfeldt-Jakob disease using a highly sensitive immunoblotting assay.

Wadsworth JD, Joiner S, Hill AF, Campbell TA, Desbruslais M, Luthert
PJ, Collinge J.

MRC Prion Unit and Department of Neurogenetics, Imperial College
School of Medicine at St Mary's, Norfolk Place, W2 1PG, London, UK.

BACKGROUND: Variant Creutzfeldt-Jakob disease (vCJD) has a pathogenesis distinct from other forms of human prion disease: disease-related prion protein (PrP(Sc)) is readily detectable in lymphoreticular tissues. Quantitation of risk of secondary transmission, and targeting of risk reduction strategies, is limited by lack of knowledge about relative prion titres in these and other peripheral tissues, the unknown prevalence of preclinical vCJD, and a transmission barrier which limits the sensitivity of bioassay. We aimed to improve immunoblotting methods for high sensitivity detection of PrP(Sc) to investigate the distribution of PrP(Sc) in a range of vCJD tissues. METHODS: We obtained tissues at necropsy from four patients with neuropathologically confirmed vCJD and from individuals without neurological disease. Tissues were analysed by sodium phosphotungstic acid precipitation of PrP(Sc) and western blotting using high sensitivity enhanced chemiluminescence. FINDINGS: We could reliably detect PrP(Sc) in the equivalent of 50 nL 10% vCJD brain homogenate, with a maximum limit of detection equivalent to 5 nl. PrP(Sc) could be detected in tissue homogenates when present at concentrations 10(4)-10(5) fold lower than those reported in brain. Tonsil, spleen, and lymph node were uniformly positive for PrP(Sc) at concentrations in the range of 0.1-15% of those found in brain: the highest concentrations were consistently seen in tonsil. PrP(Sc) was readily detected in the retina and proximal optic nerve of vCJD eye at levels of 2.5 and 25%, respectively of those found in brain. Other peripheral tissues studied were negative for PrP(Sc) with the exception of low concentrations in rectum, adrenal gland, and thymus from a single patient with vCJD. vCJD appendix and blood (Buffy coat fraction) were negative for PrP(Sc) at this level of assay sensitivity. INTERPRETATION: We have developed a highly sensitive immunoblot method for detection of PrP(Sc) in vCJD tissues that can be used to provide an upper limit on PrP(Sc) concentrations in peripheral tissues, including blood, to inform risk assessment models. Rectal and other gastrointestinal tissues should be further investigated to assess risk of iatrogenic transmission via biopsy instruments. Ophthalmic surgical instruments used in procedures involving optic nerve and the posterior segment of the eye, in particular the retina, might represent a potential risk for iatrogenic transmission of vCJD. Tonsil is the tissue of choice for diagnostic biopsy and for population screening of surgical tissues to assess prevalence of preclinical vCJD infection within the UK and other populations.

PMID: 11476832 [PubMed - indexed for MEDLINE]

http://www.ncbi.nlm.nih.gov/entrez/query.f

Creutzfeldt-Jakob disease and inclusion body myositis: Abundant disease-associated prion protein in muscle

Gabor G. Kovacs, MD PhD 1 2, Elisabeth Lindeck-Pozza, MD 1, Leila Chimelli, MD, PhD 3, Abelardo Q. C. Araújo, MD, PhD 4, Alberto A. Gabbai, MD, PhD 5, Thomas Ströbel, PhD 1, Markus Glatzel, MD 6, Adriano Aguzzi, MD, PhD 6, Herbert Budka, MD 1 *
1Institute of Neurology, University of Vienna, and Austrian Reference Centre for Human Prion Diseases, Vienna, Austria
2National Institute of Psychiatry and Neurology, Budapest, Hungary
3Department of Pathology, School of Medicine, Federal University of Rio de Janeiro
4Department of Neurology, School of Medicine, Federal University of Rio de Janeiro
5Department of Neurology, School of Medicine, Federal University of Sao Paulo, Brazil
6Institute of Neuropathology, University Hospital of Zürich, Zürich, Switzerland
email: Herbert Budka (h.budka@akh-wien.ac.at )

*Correspondence to Herbert Budka, Institute of Neurology, AKH 4J, Wühringer Gürtel 18-20, POB 48, A-1097 Vienna, Austria

Funded by:
European Union (EU) Project; Grant Number: TSELAB QLK2-CT-2002-81523
EU Concerted Action PRIONET; Grant Number: QLK2-2000-CT-00837

Abstract

Pathologicalprion protein (PrPSc) is the hallmark of prion diseases affecting primarily the central nervous system. Using immunohistochemistry, paraffin-embedded tissue blot, and Western blot, we demonstrated abundant PrPSc in the muscle of a patient with sporadic Creutzfeldt-Jakob disease and inclusion body myositis. Extraneural PrPC-PrPSc conversion in Creutzfeldt-Jakob disease appears to become prominent when PrPC is abundantly available as substrate, as in inclusion body myositis muscle.

--------------

Received: 16 June 2003; Revised: 11 September 2003; Accepted: 11 September 2003
Digital Object Identifier (DOI)


10.1002/ana.10813 About DOI

http://www3.interscience.wiley.com/

NINDS Inclusion Body Myositis Information Page

http://www.ninds.nih.gov/disorders/i

AS Professor Aguzzi kindly put it most recently ;

107
Vet Pathol 42:107 108 (2005)
Letters to the Editor
Editor:
Absence of evidence is not always evidence of absence. In the article Failure to detect prion protein (PrPres) by immunohistochemistry in striated muscle tissues of animals experimentally inoculated with agents of transmissible spongiform encephalopathy, recently published in Veterinary Pathology (41:78 81, 2004), PrPres was not detected in striated muscle of experimentally infected elk, cattle, sheep, and raccoons by immunohistochemistry (IHC). Negative IHC, however, does not exclude the presence of PrPSc. For example, PrPres was detected in skeletal muscle in 8 of 32 humans with the prion disease, sporadic Creutzfeldt-Jakob disease (CJD), using sodium phosphotungstic acid (NaPTA) precipitation and western blot.1 The NaPTA precipitation, described by Wadsworth et al.,3 concentrates the abnormal isoform of the prion, PrPres, from a large tissue homogenate volume before western blotting. This technique has increased the sensitivity of the western blot up to three orders of magnitude and could be included in assays to detect PrPres. Extremely conspicuous deposits of PrPres in muscle were detected by IHC in a recent case report of an individual with inclusion body myositis and CJD.2 Here, PrPres was detected in the muscle by immunoblotting, IHC, and paraf- fin-embedded tissue blot. We would therefore caution that, in addition to IHC, highly sensitive biochemical assays and bioassays of muscle are needed to assess the presence or absence of prions from muscle in experimental and natural TSE cases.

Christina Sigurdson, Markus Glatzel, and Adriano Aguzzi
Institute of Neuropathology
University Hospital of Zurich
Zurich, Switzerland
References
1 Glatzel M, Abela E, et al: Extraneural pathologic prion
protein in sporadic Creutzfeldt-Jakob disease. N Engl J
Med 349(19):1812 1820, 2003
2 Kovacs GG, Lindeck-Pozza E, et al: Creutzfeldt-Jakob
disease and inclusion body myositis: abundant diseaseassociated
prion protein in muscle. Ann Neurol 55(1):
121 125, 2004
3 Wadsworth JDF, Joiner S, et al: Tissue distribution of protease
resistant prion protein in variant CJD using a highly
sensitive immuno-blotting assay. Lancet 358:171 180,
2001...///


EMBO reports AOP Published online: 11 April 2003 Widespread PrPSc
accumulation in muscles of hamsters orally infected with scrapie

http://www.emboreports.org/

2004N-0257: Recordkeeping Requirements for Human Food and Cosmetics Manufactured from Processed with, or Otherwise Containing Material from Cattle

http://www.fda.gov/ohrms/dockets/


Terry S. Singeltary Sr.
P.O. Box 42
Bacliff, Texas USA

CJD WATCH
http://www.fortunecity.com/healthclub/cpr/349/part1cjd.htm


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TSS




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