- Every year, hundreds of Houstonians and other Texans
donate the tissues of their deceased loved ones to help the living.
-
- In times of deep heartbreak, families make the wrenching
decision to allow the skin, bones, heart valves and leg veins of their
relatives to be removed so that they may be transplanted into burn victims,
the disabled or diabetics.
-
- But rarely are they told by representatives of LifeGift
Organ Donation Center the nonprofit agency handling tissue donation cases
in the Houston area that the tissue could potentially be used for cosmetic
surgeries. Nor are they routinely informed that it could be processed and
distributed by for-profit companies traded on Wall Street, helping fuel
a $1 billion-a-year tissue industry.
-
- "I don't think donors' families really understand
that there is a for-profit dimension to tissue procurement," said
Arthur Caplan, a bioethicist at the University of Pennsylvania School of
Medicine. "We know that tissue companies have made very big profits
and have built lavish headquarters and are doing well."
-
- Generally, the companies, as well as their nonprofit
counterparts, strike agreements with organizations such as LifeGift to
supply them tissue, which they then make into different products and sell
to doctors and hospitals for a wide variety of uses. Among them are skin
grafts, bone transplants, heart-valve replacements and, sometimes, cosmetic
surgery.
-
- Disclosure Not Required
-
- LifeGift officials say federal law hasn't required them
to tell families who receive no compensation all the possible uses of donated
tissue or that they deal with for-profit companies.
-
- That may soon change.
-
- The federal Centers for Medicare & Medicaid Services,
which regulates organizations such as LifeGift, proposed a rule Feb. 4
that would require companies to fully disclose all possible uses of donated
tissue, including cosmetic surgery, as well as the for-profit or nonprofit
status of companies receiving the tissues.
-
- In anticipation of the rule, LifeGift officials are revising
their family-consent policy and could implement it as early as this week.
-
- "Now since the regulations require it, it is the
right thing to do, and we will do it," said LifeGift spokeswoman Catherine
Burch Graham.
-
- Carla Buchinger, who agreed to donate all of her 18-year-old
son's tissues after he died in a 2000 car accident, said the issue of cosmetic
surgery didn't arise when LifeGift approached her and asked for her consent.
Nor did it tell her his tissues may have been sent to publicly traded companies.
-
- Knowing that wouldn't have changed her mind, but she
thinks families should be informed.
-
- "The fairest thing is to go ahead and tell them
so that they can make an informed decision," she said.
-
- Advocates For Donor Families Applaud Proposed Rule
-
- "Our position is that families should be given as
much information as possible," said Rose D'Acquisto, who heads the
volunteer committee of the National Donor Family Council.
-
- But she and others are concerned that some family members
may say no to donation if they learn that private companies are trying
to make a profit from the tissue of their loved ones.
-
- "I do think we need full disclosure to families,"
said Penny Powers, manager of transplant service at St. Luke's Episcopal
Hospital. "But I do worry that in such a time of stress and grieving
that hearing that there could be a for-profit element could turn them off.''
-
- Donations Tightly Regulated
-
- Each year, there are about 20,000 lifesaving heart, liver,
kidney and other organ transplants in the nation. Organ donations are tightly
controlled by the federal government, which has established a national
waiting list and offers reimbursement to 59 government-recognized Organ
Procurement Organizations across the country.
-
- These so-called OPOs, which cover specific geographic
regions, are generally responsible for identifying potential organ donors,
obtaining consent and retrieving organs. LifeGift is the OPO for Southeast,
West and North Texas, and most of its operations are dedicated to organ
identification and recovery.
-
- Like many OPOs, LifeGift also oversees tissue donation,
which is different. Because tissue from a donor's body can be recovered
up to 24 hours after death if it is refrigerated, a far greater number
of people are potential tissue donors. And because so many tissues can
be recovered from a body, dozens of transplantations can result. Also,
there is no federal reimbursement for tissue recovery.
-
- There are hundreds of ways donated tissue is used to
improve the lives of others.
-
- A donor's fresh skin, for example, can be used to help
burn victims, or it can be processed to be used in medically necessary
reconstructive surgeries such as abdominal wall replacements. Bone may
be crushed, reshaped or made into powder to be used in orthopedic surgeries.
Heart valves may be transplanted into patients with heart defects. And
saphenous veins replace those of diabetics or others with such severe circulation
problems that they might be facing amputation.
-
- About 1 million tissue transplantations are done each
year, said Robert Rigney Jr., executive director of the American Association
of Tissue Banks, which accredits about 90 tissue banks.
-
- According to federal law, every hospital must contact
its local OPO each time a patient dies or is pronounced brain-dead to determine
whether he or she is eligible for organ or tissue donation. In most cases,
organs are not recoverable, but often tissues are.
-
- 32% Agree To Donate
-
- Locally, if the deceased is an eligible donor, a LifeGift
employee approaches family members sometimes in person and other times
by phone and asks for their consent.
-
- Generally, the families are told that their loved ones'
tissues could go to save or improve the lives of others, said Sean Conley,
LifeGift's manager of clinical communications and logistics. Each tissue
is described, and a general explanation of how it might be used is reviewed,
he said.
-
- Although there is some mention of a processing and distribution
system, the families are not told unless they ask that some of the tissue
may be sent to for-profit companies, he said.
-
- Last year, 32 percent of the families approached by LifeGift
agreed to donate.
-
- The tissue is then recovered by trained, four-person
teams in surgical procedures that last up to four hours. The body is closed
up carefully enough, including the insertion of PVC piping to replace bones,
so that family members can have an open-casket funeral. And the tissues
are packaged, placed in coolers and sent to one or more of six banks that
have active contracts with LifeGift for their tissue supplies.
-
- 'Reasonable' Recovery Fees
-
- The National Organ Transplant Act forbids the sale of
body parts for profit. But it does allow OPOs and banks to charge "reasonable"
recovery fees.
-
- LifeGift officials refused to disclose its detailed recovery
fees per tissue type. But Graham said that its average total recovery fee
per donor in 2004 was about $4,800.
-
- Former employees said the charges can be much more about
$6,000 for a full bone recovery alone, and $10,000 or more when skin, heart
valves and veins are included.
-
- And LifeGift officials said they recently raised their
recovery rates between 3 percent and 15 percent to pay for increased costs.
-
- One Of Banks In Texas
-
- One of the six banks that receive tissue from LifeGift
is in Texas the Shriner's Burn Hospital for Children in Galveston, which
uses skin to treat burn victims.
-
- The others are: the Musculoskeletal Transplant Foundation
of Edison, N.J., which processes bone; LifeNet of Virginia Beach, Va.,
which processes heart valves; Community Tissue Services of Dayton, Ohio,
which processes bone and skin; LifeCell of Branchburg, N.J., which processes
skin; and Alabama Tissue Center of Birmingham, Ala., a subsidiary of Regeneration
Technologies, which processes heart valves and veins.
-
- Graham said Shriner's receives all of the skin it needs
from LifeGift and that it sends only surplus to LifeCell.
-
- LifeCell and Regeneration Technologies are for-profit
companies, with combined revenues in 2003 of $116 million, according to
their annual reports.
-
- Each has a Web site touting its products, and they spend
tens of millions of dollars a year on marketing.
-
- Concerns About Publicity
-
- And they worry about negative publicity. LifeCell, which
makes a skin product called AlloDerm that is used for elective cosmetic
surgery, including lip enhancements and penile enlargements, said in its
2004 annual report that it was concerned that public knowledge of this
potential use could hurt its bottom line.
-
- "Although we do not promote the use of human tissue
products for cosmetic applications, clinicians may use our products in
applications or procedures that may be considered cosmetic," the company
said. "Negative publicity concerning the use of donated human tissue
in cosmetic procedures could reduce the demand for our products or negatively
impact the willingness of families of potential donors to agree to donate
tissue or tissue banks to provide tissue to us for processing."
-
- Scott Bottenfield, LifeCell's director of tissue services
and partner relations, said LifeGift "is probably our second-largest
provider of skin."
-
- But he and Graham said the vast majority of the skin
89 percent sent to LifeCell in 2004 was used to make a product used almost
exclusively for burn patients, in keeping with the organization's mission
to help the sick or injured. Another 11 percent of thicker skin recovered
by "accident" was used to make AlloDerm, she and Bottenfield
said.
-
- Based on LifeCell's analysis of where the AlloDerm was
sent, none of the skin from LifeGift was likely used for elective cosmetic
surgery in 2004, they said.
-
- Still, Bottenfield and Graham said that even though it
didn't happen in 2004, they can't guarantee that no skin from LifeGift
will end up being used in so-called "vanity" surgeries.
-
- "We can't control what a surgeon is going to do,
but that is not what we are about," Bottenfield said.
-
- Millions Spent On R&D
-
- LifeGift chief executive Sam Holtzman said the for-profit
companies play an important role because in addition to providing products
that save and enhance the lives of the sick and injured, they spend millions
of dollars a year on research and development of new products.
-
- He said LifeGift's contracts with all of its banks nonprofit
and for-profit alike urge them to send as much processed tissue as possible
back to the Houston area.
-
- But LifeCell's Bottenfield said he couldn't say for sure
whether that is happening with his products.
-
- "We have a commitment to serve the local community,"
he said. "But I am not even sure that it is likely that a lot of the
skin recovered in Houston comes back to Houston."
-
- Holtzman acknowledged LifeGift can't always guarantee
where it goes or how it is used.
-
- "We don't always know what the end use of a tissue
product is," he said. "Sometimes it's in storage for months or
years before it can be used."
-
- Some doctors who use those processed tissue products
say families who consent to donate should know that.
-
- "When someone gives a gift of their loved ones tissue,
they are doing it to save a life," said Dr. Sherwin Siff, chief of
orthopedic surgery at St. Luke's and clinical professor at Baylor College
of Medicine. "They are not donating it to help someone get rich."
-
-
- http://www.chron.com/cs/CDA/ssistory.mpl/page1/3037326
-
- Greetings,
-
- reminds me of;
-
- please skroll down a bit to;
-
- some things of interest?
-
- AATB 6th Annual Meeting, March 24-26, 2002 - Slide Presentation
-
- Microbial Contamination and Cross Contamination Concerns
During
- Processing of Tissue:
- an FDA Perspective
-
- Mary Malarkey, Director,
-
- snip...
-
- 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...
- Date: September 17, 2000 at 10:06 am PST
-
- Subject: RE-The Eyes Have It (cjd) and they could be
stealing them from
- your loved one... "pay back time"
- Date: Sat, 16 Sep 2000 10:04:26 -0700
- From: "Terry S. Singeltary Sr."
- Reply-To: Bovine Spongiform Encephalopathy
- To: BSE-L
-
- Greetings List Members,
-
- I hate to keep kicking a madcow, but this still is very
disturbing to me. Not only for the recipient of the corneas, but as well,
for the people whom would be operated on, using the same tools that were
used to put those stolen cornea's in the recipient with. No history of
this donor or his family (re-ffi), or anything would be known, using stolen
organs and or tissue's. I just think this is not only wrong, but very dangerous
to a great many other people, as this is one of the most infectious tissues
of TSE's. It seems that this practice of stealing organ/tissue happens
more than we think. Anyway, the family of the victim which had their cornea's
stolen, are now suing. In the example I used with my Mother, if 3 months
before, she would have been in a catastrophic accident (car wreck, whatever),
no autopsy (for whatever reason), no family (for whatever reason), she
lay in the morgue, and after 4 hours, they come steal the cornea's, lot
of people could have been infected, just because of lack of medical history
of donor/family. It may be hypothetical, but very real. We need to stop
the spread of this disease.
-
- Kind regards,
-
- Terry S. Singeltary Sr.
- Bacliff, Texas USA
-
- Previous story--
-
- Cadaver corneal transplants -- without family permission...
- http://www.mad-cow.org/
-
- Sept. 15, 2000, 11:39PM
-
- snip...
-
- http://neuro-mancer.mg
-
- Eye Procedure Raises Cjd Concerns
-
- http://www.washtimes.com/
- snip...full text
-
- CJD and intraocular surgery
-
- Ophthalmic surgery and Creutzfeldt-
- Jakob disease
-
- http://www.prwatch.org/forum/showthread.php?t=5162
-
- http://brain.hastypastry.net/forums/archive/index.php/t-3065.html
-
- THE LEGALITY OF STEALING ORGAN/TISSUE...
-
- TEXAS STATUTES
-
- Sec. 693.012. Removal of Corneal Tissue Permitted Under
Certain
- Circumstances.
-
- snip...
-
- http://disc.server.com/discussion.cgi?d
-
- 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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