Saturday, April 11, 2015

ISU veterinary researchers study retinal scans as early detection method for mad cow disease

ISU veterinary researchers study retinal scans as early detection method for mad cow disease
 
 
ISU veterinary researchers study retinal scans as early detection method for mad cow disease
 
Posted Apr 9, 2015 4:18 pm
 
Heather Greenlee's research shows that scanning the retinas of cattle can lead to faster detection of mad cow disease. Photo by Christopher Gannon. Larger image.
 
AMES, Iowa – New research from Iowa State University shows that a fatal neurological disease in cows can be detected earlier by examining the animal’s retinas.
 
Bovine spongiform encephalopathy (BSE), known more commonly as mad cow disease, is an untreatable neurodegenerative disorder caused by misfolded brain proteins known as prions. Classic BSE incubates for years before producers or veterinarians notice symptoms, usually discovered when the animal can no longer stand on its own.
 
But Heather Greenlee, an associate professor of biomedical sciences in Iowa State’s College of Veterinary Medicine, said studying the retinas of cattle can identify infected animals up to 11 months before they show signs of illness.
 
“The retina is part of the central nervous system,” Greenlee said. “Essentially, it’s the part of the brain closest to the outside world, and we know the retina is changed in animals that have prion diseases.”
 
In collaboration with Justin Greenlee’s group at the U.S. Department of Agriculture’s National Animal Disease Center, she recently published findings in the peer-reviewed academic journal PLOS ONE. She began studying how the retina relates to prion diseases in 2006, and the experiments that led to her most recent publication began in 2010.
 
The experiments utilize electroretinography and optical coherence tomography, noninvasive technologies commonly used to assess the retina. Greenlee said cows infected with BSE showed marked changes in retinal function and thickness.
 
The results have implications for food safety, and Greenlee said the screening methods used in her research could be adopted for animals tagged for import or export as a means of identifying BSE sooner than conventional methods.
 
Greenlee said she’s also looking at how similar diseases in other species affect the retina. For instance, she’s conducting experiments to find out if retinal tissue may be a valid means of surveillance for chronic wasting disease in deer.
 
She said she isn’t ready to publish her results, but the data gathered so far looks promising.
 
The research also may contribute to faster diagnosis of Alzheimer’s disease and Parkinson’s disease in humans, both of which are caused by proteins folding incorrectly.
 
“Our goal is to develop our understanding of the retina to monitor disease progression and to move diagnoses up earlier,” Greenlee said. “We think this research has the potential to improve diagnosis for a range of species and a range of diseases.”
 
-30- -
 
See more at:
 
 
 
 
 
see study ;
 
 
Wednesday, March 18, 2015
 
Changes in Retinal Function and Morphology Are Early Clinical Signs of Disease in Cattle with Bovine Spongiform Encephalopathy
 
 
see more here ;
 
 
 
it’s all getting very interesting now with the TSE prion disease, all it’s routes and sources and species, and now the risk factor for Alzheimer’s and is Alzheimer’s a TSE prion disease, and what are the risk factors from the many potential IATROGENIC ROUTES ???
 
what about the infamous VPSPR ??? price of prion poker went up for sure with that.
 
and just what is SPORADIC GSS AND SPORADIC FFI, both familial type prion disease, but yet NOT link to any genetic family ???
 
could it be IATROGENIC TRANSMISSION OF THE sGSS and sFFI ???
 
many questions still unanswered...
 
 
1999
 
 
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...
 
 
From: Terry S. Singeltary Sr. (216-119-138-142.ipset18.wt.net)
 
Subject: 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...
 
Date: February 10, 2000 at 9:10 am PST
 
 
 
 
############ Creutzfeldt-Jakob Disease #############
 
Greetings list members, I was impressed that someone is listening, considering the timing of when I broke the story in Nov. and this was posted in Dec., what a coincidence. Thanks for listening. I find it rather frightening of the fact sporadic CJD as well as vCJD can transmit infectivity this way. Makes me wonder about blood?
 
 
Kind Regards, Terry S. Singeltary Sr., Bacliff, Texas USA
 
Vol. 282 No. 23, December 15, 1999 Preventing Prion Transmission in Corneal Transplants
 
To the Editor: We agree with the Council on Scientific Affairs' recommendation that "physicians become knowledgeable about BSE [bovine spongiform encephalopathy] so they can appropriately advise their patients about routes and rates of BSE transmission."1 Unfortunately, there is only passing mention of prion transmission by corneal transplantation, which is performed on 40,000 to 50,000 patients each year in the United States.2 In addition to the 1974 US case,3 2 additional cases of probable and possible transmission, respectively, have been reported in Germany and Japan,3 but the major new concern relative to prion transmission via corneas occurred recently in Great Britain. In February 1997, the corneal transplant and sclera from a 53-year-old woman who had died of presumed metastatic lung cancer were transplanted to 3 recipients.4 In November 1997, the donor's brain revealed sporadic Creutzfeldt-Jakob disease (CJD), confirmed by the United Kingdom CJD Surveillance Unit.4 Although transplanted tissues were subsequently removed, these 3 cases raise particular concern since infectivity of ocular tissue in scrapie was reported as 5.4 log U/mL median infective dose (cornea) compared with 8.9 log U/mL (brain) and 8.5 log U/mL (retina).3
 
The demonstrated possibility of further CJD or potential new variant-CJD (nv-CJD) corneal transmission has created heightened medical and public scrutiny of US eye donor screening standards; recently tightened criteria were approved by the Eye Bank Association of America.5 Fortunately, no additional corneal transmission has been reported in the United States since 1974, and additional safeguards in place should further ensure the highest standards of continued tissue safety in this country.
 
H. Dwight Cavanagh, MD, PhD R. Nick Hogan, MD, PhD University of Texas Southwestern Medical Center at Dallas
 
1. Tan T, Williams MA, Khan MK, et al. Risk of transmission of spongiform encephalopathy to humans in the United States: report of the Council on Scientific Affairs. JAMA. 1999;281:2330-2339. ABSTRACT | FULL TEXT | PDF | MEDLINE
 
2. Eye Bank Association of America. Eye banking statistical report. Washington, DC: Eye Bank Association of America; 1997.
 
3. Hogan RN, Brown P, Heck E, Cavanagh HD. Risk of prion disease transmission from ocular tissue transplantation. Cornea. 1999;18:2-11. MEDLINE
 
4. Royal College of Ophthalmologists. CJD and the Eye. London, England: Royal College of Ophthalmologists; 1998. Monograph 7.
 
5. http://www.fda.gov/cber/rules/suitdonor.txt. Accessed November 29, 1999.
 
In Reply: The transmission of classic CJD via corneal transplantation has been demonstrated, and the potential possibility of transmission of nv-CJD via corneal transplantation should be taken into consideration. With ongoing national efforts to increase organ and tissue donation, we are pleased to hear from Drs Cavanagh and Hogan that the additional safeguards introduced in the new eye donor screening standards and recently approved by the Eye Bank Association of America will ensure the highest standards of safety in this tissue.
 
Litjen Tan, PhD Michael A. Williams, MD Council on Scientific Affairs American Medical Association Chicago, Ill
 
_______________________________________________________________________
 
Cadaver corneal transplants -- without family permission
 
Houston, Texas channel 11 news 28 Nov 99 Repoorted by Terry S. Singeltary Sr.son of CJD victim
 
"It was a story about how the Lions eye bank were harvesting corneas from victims in the Morgue, without their consent. Under Texas law, this appears to be legal (remember Texas has the Veggie liable law). Even if Family says no, this appears to happen, from what the news story said.
 
They said the only way to prevent this, is to fill out a form, stating not to have this done. So if you don't fill out the form, they can do this. How many people don't know about the form?
 
This is not only disgusting and appalling, it could be highly infectious. Without proper background checking of the donors, on their physical history, checking on past dementia, and/or family history, some of these unfortunate victims, could be passing a human TSE.
 
Response Jill Spitler Clevelland Eye Bank:
 
"No, we are not stealing.........Yes, you do have such a law in the state of Texas, but not all your state Eye Banks utilize the law. The Eye Bank that you're speaking of is only one of 43 certified Eye Bank throughout the USA.
 
And there are measure taken per the Medical Standards of the Eye Bank Association of America, the certifying body for eye banks and per FDA regulations to address those concerns that you speak of.
 
I would suggest that those interested/concern with transplant contact their local agencies. The Eye Bank Association of America has a web. site . Further if anyone has problems contacting or finding out about their local organization(s), call me or e-mail me I would be glad to help. My e-mail address is jill@clevelandeyebank.org"
 
Terry Singeltary responds:
 
"Explain this to the family in Houston who went to their loved ones funeral, only to find out that the loved one that was in the casket, had their corneas removed without their permission, without the consent of the victim or it's family. They would not have known it, only for the funny look the victim had. So, they questioned, only to find out, the corneas, had in fact, been removed without consent.
 
I call that stealing, regardless what the law states. This type of legal grave robbing is not a logical thing to do without knowing any type of background of the victims medical past, which really will not prove anything due to the incubation period. Eye tissue being potentially a highly infective source, there are risks here.
 
Should they not at least know of the potential ramifications of TSE's (the person receiving the corneas)?
 
Should there not be some sort of screening?
 
Should there be some sort of moral issue here? If this is the case, and in fact, they can come take your corneas, without your consent, then what will they start taking next, without your consent?
 
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?"
 
Comment (webmaster): Actual transmission of CJD by means of corneal transplant may or may not be rare. The incidence of infectivity in older people could be fairly high; this is not to be confused with the lower incidence of symptomatic (clinical) CJD. It is very unlikely that familial CJD would have been diagnosed in earlier generations; however, without interviewing the family even known kindreds would not be excluded.
 
In blood donation, a much stricter policy is followed, even though corneal transplant may be far more dangerous (being a direct link to the brain and not going through purification steps).
 
Since highly sensitive tests for pre-clinical CJD are now available, it would make sense to screen corneas for CJD, just as they are screened for AIDS, hepatitus, and a host of other conditions.
 
 
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 cornea's, 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/~tom/dec99_news.html#bbb
 
===============================================
 
Sept. 15, 2000, 11:39PM
 
Slain woman's family sues over missing eyes
 
By BILL MURPHY Copyright 2000 Houston Chronicle
 
The family of a woman who was stabbed to death last year has filed a lawsuit accusing the Lions Eye Bank of Houston of removing the woman's eyes without permission and inserting plastic discs in their place.
 
Daisy Diaz's relatives were horrified when they saw her body and noticed her eyes were missing, said their lawyer, Duncan Neblett III.
 
"They're a Catholic family," Neblett said. "They have strong beliefs about the body and burial. They were really upset by this."
 
Dorey Zidrow, the eye bank's spokeswoman, said she could not specifically discuss the Diaz case because it was in litigation. But Zidrow said a state law allows doctors to remove corneas -- the dime-sized lens near the eye's surface -- from a corpse without the family's permission.
 
The eye bank's usual procedure calls for removing the corneas, Zidrow said, but not the entire eyes.
 
"There are an awful lot of people who benefit from this program in the state of Texas," she said.
 
Diaz, 25, was stabbed to death in her apartment in the 400 block of Thornton in October. Her brother-in-law, 30-year-old Raudel Quiroz, is charged in the killing but has not been caught.
 
Neblett said authorities have told him Quiroz may have returned to his native Guatemala.
 
Neither Diaz nor her family had given permission to donate any of her organs, Neblett said.
 
Although state law allows corneas to be removed from corpses without first gaining the family's permission, they cannot be removed over the family's stated objection.
 
The eye bank is located at, and staffed by, the Baylor College of Medicine, and receives part of its funding from the Lions Club.
 
The Diaz lawsuit is the second such suit to be filed against the eye bank in recent years.
 
The family of Levi Perry Jr., a Houston teacher shot to death in MacGregor Park in 1994, also alleged in their suit that Perry's eyes were removed. The family was awarded $345,000 from the eye bank in April 1999.
 
 
==========================================================
 
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]]]
 
 
 
Testimony of Bess Believeaux, Lions Eye Bank of Central Texas (Submission to the Jan. 18/19 meeting of the TSE Advisory Committee)
 
 
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
 
TSS
 
 
From: TSS (216-119-162-15.ipset44.wt.net)
 
Subject: re-The Eyes Have It (CJD) and they could be stealing them from YOUR LOVED ONE !!!
 
Date: June 9, 2002 at 11:27 am PST
 
######## Bovine Spongiform Encephalopathy #########
 
June 8, 2002, 9:12PM Family seeks redress in cornea harvesting case By JANET ELLIOTT Copyright 2002 Houston Chronicle Austin Bureau
 
AUSTIN -- Unless the Texas Supreme Court reverses course and decides to review a case filed by the family of a Houston murder victim, there might be no legal remedy for the wrongful harvesting of organs.
 
"As it stands now, people whose loved ones have their organs removed without consent and against the express wishes of the family do not have a cause of action," said Cletus Ernster III, lawyer for the parents of Levi Perry Jr.
 
Perry, a 38-year-old schoolteacher, was shot while jogging in MacGregor Park in 1994. Four gang members who participated in the killing were convicted.
 
In 1999, Perry's parents and two of his siblings won a $345,000 judgment after a Harris County jury found that Lions Eye Bank of Texas removed Perry's eyes despite the fact that Perry's father signed a form denying approval for any organ or tissue removal.
 
Houston's 14th Court of Appeals last September reversed the judgment. In a 2-1 decision, a panel of the court found that state law doesn't allow the Perrys to recover mental anguish damages on their negligence claim.
 
In a dissent, Justice Charles Seymore said that Texas courts have consistently allowed recovery of mental anguish damages for the negligent handling of a dead body.
 
In March, and again in May, the Supreme Court denied review of the lower court ruling.
 
Ernster said he is planning one more attempt to get the high court to hear the case.
 
Kevin Yankowsky, a Houston lawyer who represents the eye bank, said the appeals court opinion did not make new law. He said it doesn't close the courthouse door to future claims that raise different legal and factual issues.
 
Evidence that Perry did not want to be an organ donor was particularly strong.
 
Perry was the oldest of 10 children of Levi Perry Sr. and Eula Perry. The elder Perrys were pioneering African-American doctors in the Fifth Ward.
 
Seven of the children followed their parents into medicine, including Angela Perry, who became an opthalmologist.
 
About four years before Levi's murder, Angela Perry asked him to be an organ donor. He declined, saying, "I want to leave this world with everything God gave me," according to testimony Angela Perry gave in 1999.
 
When the family received word of Perry's murder, they went to Ben Taub Hospital to identify the body. That is when Dr. Perry, a cardiologist, signed the form consenting to an autopsy as required for a homicide, but denying any organ or tissue donations.
 
"They felt like that was the last thing they could do for their son," Ernster said.
 
Under state law, corneas can be removed from dead people without the permission of the family. Other organs, including eyeballs, need the permission of the deceased or family members to be removed. But corneas cannot be removed if the family files an objection such as the Perrys did.
 
The dime-sized corneas, which are the eye's main focusing element, can be collected and transplanted after a person's death. Transplanted corneas can help patients recover sight lost to injury, disease or infections.
 
According to the Eye Bank Association of America, in 2000 there were 47,000 corneal grafts. There are no waiting lists for corneas and, in fact, the U.S. exports corneas to other countries.
 
Donated eyeballs are used for research.
 
An eye bank employee who testified in the trial in the Perrys' lawsuit denied he removed the eyeballs.
 
But Angela Perry testified that the family was attending a private rosary when she noticed her brother's eyes appeared to be sunken. She said she opened his eyelids and saw the eyeballs were not there.
 
The jury was so incensed about the evidence that they found the eye bank grossly negligent and awarded $200,000 in punitive damages.
 
Officials with the eye bank were not available for comment, said Heather Russell, a spokeswoman with Baylor College of Medicine. The eye bank is affiliated with the medical school.
 
In 2000, the eye bank was named in another lawsuit filed by the family of a woman who was allegedly stabbed to death by her brother-in-law.
 
Daisy Diaz's relatives said they were horrified when they saw her body and noticed her eyes were missing. Duncan Neblett III, who represents the family, said pictures taken by a doctor show Diaz's face was badly damaged and bruised.
 
The family had not signed a form consenting to removal of the eyeballs nor were they contacted by workers at the morgue, Neblett said.
 
After a television report aired on the lawsuit, Neblett said, he received calls from several families who related similar experiences. He said all of the callers were minorities.
 
"I've never talked to a wealthy white person this has happened to," Neblett said.
 
He said the Perry case could have an impact on the lawsuit he filed, although he alleged some causes of action that were not included in the Perrys' lawsuit.
 
In April, the parents of a 15-year-old Central Texas girl who died after a car wreck filed a lawsuit against several defendants, including the Lions Eye Bank of Central Texas.
 
The parents of Jenna Mae Spene declined when they were asked at Austin's Brackenridge Hospital whether they wanted to donate her organs and tissues.
 
The lawsuit says a family friend who went to the funeral home to dress the body discovered that Jenna's corneas, part of her right breast and part of her left foot had been removed.
 
Daniel Richards, an Austin lawyer who represents Teresa and Chris Spene, said the parents hope their lawsuit might prompt legislators to consider changing the law that allows cornea harvesting without the consent of relatives.
 
Michigan's law allowing coroners to harvest corneas without the consent of relatives was ruled unconstitutional by a state court. The U.S. Supreme Court in 1995 refused to reverse the ruling.
 
 
Subject: Re: The Eyes have it (CJD), and they could be stealing them from your loved one...
 
Date: Sat, 14 Oct 2000 16:58:28 –0700
 
From: "Terry S. Singeltary Sr." Reply-To: Bovine Spongiform Encephalopathy
 
To: BSE-L@uni-karlsruhe.de
 
######### Bovine Spongiform Encephalopathy #########
 
Greetings everyone,
 
I am sure most of you remember this thread, and the concern I raised of this potential threat of transmission of TSE, not only to another victim from the donor, but my most concern, was all the other patients that may have been operated on, with the same instruments that was used to transplant those potentially infected corneas', which were stolen. I listed a hypothetical using my mother as an example, etc. So instead of reposting, for those of you not familiar with this thread, i posted urls below to go to, plus url to this document/docket that just literally floored me, from the ignorance of it all. I shall pass it on to you, so you may share the ignorance of it all with me...
 
thank you, Terry S. Singeltary Sr., Bacliff, Texas USA
 
===========================================
 
Medical Eye Bank of Maryland 815 Park Avenue Baltimore, MD 21201 410-752-2020 FAX: 410-545-4455
 
1177 99 DEC 27 A9:39
 
Medical Board Directors Walter J. Stark, MD, Medical Director John C. Baer, MD. Assoc. Medical Director John D. Gottsch. M.D Assoc. Medical Director Verinder Nirankari, M.D. Assoc. Medical Director
 
Medical Eye Bank of Maryland Advisory Board Robert C. Davis, Jr Chairman
 
Executive Director Patricia A. Murphy
 
Technical Program Manager Bernard E. Madison
 
Donor Development Coordinator Carol Miller
 
December 23, 1999
 
Docket Management Branch (HFA - 305) Food and Drug Administration 5630 Fishers Lance, Rm. 1061 Rockville, Maryland 208.52
 
Re: 21 CFR Parts 210, 211, 820 and 1271 [Docket No. 97N484S]
 
Proposed Rule: Suitability Determination for Donors of Human Cellular and Tissue-Based Products
 
Dear Sir or Madame:
 
As the Executive Director of the Medical Eye Bank of Maryland and a member organization of Tissue Banks International, I would like to state my agreement to our organization's objection to the current proposed rule.
 
The Medical Eye Bank of Maryland has safely and efficiently operated since 1962 providing needed corneal transplants to the citizens in Maryland. Part of the successful elimination of our corneal waiting list is due to the recovery of corneas obtained under the Maryland legislative consent through the State of Maryland Medical Examiner Office. This program has been in place for several decades. I disagree with the FDA's contention that requiring a donor medical history interview for corneas obtained under legislative consent is necessary to ensure the risk of communicable disease transmission and would urge the FDA to reassess this proposal.
 
As indicated in the Tissue Banks International chart, there is little likelihood that a potential CJD case would even be brought into the Medical Examiners Office for an autopsy and even less likely that CJD case would not be screen out under our current medical standards for recovery. I believe that the medical/social history that we perform on all cases obtained under legislative consent are just as comprehensive as those cases obtained with a next-of-kin consent and a medical/social history questionnaire.
 
I urge the FDA to reassess this proposed rule and the affect that it would have on the public that we serve. The Medical Eye Bank of Maryland has been able to serve the citizens of our state effectively by continuing to meet scheduled surgery dates for potential corneal transplant recipients, The proposed legislation may dramatically change how we can provide this service to our citizens.
 
I would be available for any questions that you may have about my comments.
 
Sincerely,
 
Patricia A. Murphy Executive Director
 
OFFICE OF THE CHIEF MEDICAL EXAMINER (MEO) Baltimore, Maryland 1998 Statistics for the State of Maryland
 
The following is an analysis of the total caseload of the Chief Medical Examiner of the State of Maryland for the ywr 1998.
 
Reported Autopsied
 
Total Cases Reported: 8003 Total Cases Autopsied: 3184
 
*Total cases Nervous System Diseases 43 (0.5%) (NSD):
 
Total NSD cases autopsied: 4 (0.1%)
 
Total # of Eye Donors from NSD cases: o (0%) o (0%)
 
Total CJD Cases: Reported to MEO : 0 Autopsied by MEO: 0 Cornea Donors to Eye Bank: 0
 
*Where a CJD case would be classified per MEO
 
Discussion:
 
The scientific literature indicates one case of CJD per million in general population
 
The 1998 population of the State of Maryland is 5.1 million thus; it could be expected that five cases CJD cases might occur in one year. (1988)
 
The total number of deaths (all causes) in Maryland is approximately 40,000 annually (1998 data) thus; it could be expected that one case might be a MEO case in one year (1998) (MEO cases equal 20% of total annual deaths) if MEO cases were representative of the general population (of deaths).
 
MEO cases are a distinct sub set of the general death population primarily including accident; suicide and homicide
 
CJD cases are generally not reported to MEO CJD cases are generally not autopsied by MEO CJD cases (as an infectious disease case) would not be available to the eye bank by definition CJD cases would be screened out under current medical standards as would any other case with unknown neurological disorders.
 
SUMMARY
 
The likelihood of a potential CJD case being made available to the eye bank by the MEO is nil by definition and category as determined by the MEO. The likelihood of the eye bank recovering tissue from a MEO CJD case is nil because by definition unknown nervous system disorders are ruled out.
 
==============================================================
 
TBI/TISSUE BANKS INTERNATIONAL
 
December 23, 1999
 
Docket Management Branch (HFA - 30.5) Food and Drug Administration 5630 Fishers Lane, rm. 1061 Rockville, MD 20852
 
Re: 21 CFR Parts 210, 211, 820 and 1271 pocket No. 97N - 484S]
 
Proposed Rule: Suitability Determination for Donors of Human Cellular and Tissue-Based Products
 
Dear Sir or Madame:
 
Tissue Banks International (TBI) has commented on the FDA's "proposed document" and "proposed registration rule" whereby TBI communicated our objection to a comprehensive regulatory system for all tissue based products. Unlike the December 1993 Interim Final Rule where there was concern about unsafe imported tissue and potentially inadequate donor screening, the FDA's proposed new system of regulation for human cellular and tissue based products is not accompanied by a demonstrated need for additional regulation. Similarly, the proposed role cited above is not based on a demonstrated need to modify the screening and testing regulations for the human allograft tissue currently regulated under the FDA's "'tissue final rule".
 
TBI's objection to the current proposed rule is consistent with our previously communicated objections. There is mention of "concern" about communicable disease in the FDA commentary. To our knowledge, under the current regulation there have been no problems with transmission of communicable disease through the use of human tissue for the diseases currently listed or for those proposed to be added. The eye and tissue banking community has not been informed of the FDA's safety and effectiveness concerns.
 
Additionally, the FDA has not yet addressed the concerns expressed by TBI and many others in the eye and tissue banking community over the definition, specific interpretation and scope of certain concepts within the "proposed approach document" such as "homologous use", "minimal manipulation" and "systemic effect". The current proposed rule only
 
[skip to page 3, full text url below...tss]
 
Tissue Banks International FDA [Docket No. 97N - 484S] Page 3 of 6
 
The FDA already deemed relevant TSE/CJD and treponema pallidum in addition to HIV and hepatitis (for non leukocyte-rich tissue) contained in the "tissue final rule" requiring screening for former and testing for the later. The tissue and eye banking commtmity already screens for many diseases and disease risks including CJD. TBI does not believe the FDA has sufficiently demonstrated (quantitatively or scientifically) relevant risk for expanding its oversight to include other diseases in addition to HIV and hepatitis. As previously expressed, the application of "relevant" is subject to FDA's sole determination which is further complicated by the FDA's interpretation of the terms "sufficiently prevalent", "risk" and "appropriate screening". These terms are not sufficiently defined. Additionally, relevant risk is broadly applied and does not sufficiently address risk by specific tissue that TBI will comment on in the following subtitle.
 
TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHY (TSE) AND CREUTZFELDT-JACOB DISEASE (CJD): The FDA seems to be particularly concerned about the transmission of CJD through dura mater and cornea transplants. Yet, apparently based on these reports, the FDA proposes to apply the screening Lo all tissue. Of particular concern to TBI is if the FDA would require the tissue and eye banking community to screen for and reject donors who exhibit changes in speech and gait. Changes in speech and gait are symptoms that might apply to many medically suitable donors most likely not associated with TSE / CJD.
 
TBI would like to stress that the reports of the transmissions of disease for both dura mater and corneal tissue occurred outside of the United States except for one reported case of CJD via cornea transplant in the U.S. The cornea is this case was never evaluated or screened by the local eye bank and occurred before the promulgation of any organized screening standards.
 
TBI is working with the Eye Bank Association of America to review the adequacy of the screening of eye donors for CJD. Walter Stark, M.D., head of the Cornea Service at the Wilmer Eye Institute at Johns Hopkins University Medical Center and TBI's National Medical Director is participating with Richard Johnson, M.D., also from Johns Hopkins and author of many publications on prion disease along with others on a special ad hoc committee investigating this issue. TBI recommends the FDA take no action regarding the screening for TSE / CJD until further evaluation by this EBAA ad hoc committee can be completed and the results can be shared with the FDA.
 
TBI knows of no currently available method to test for TSE except for a brain biopsy. TBI agrees with the FDA that testing for TSE through a brain biopsy is not feasible because the test results would not be available before corneal tissue is optimally utilized for transplantation. This would not be in the best interest of the patient receiving the cornea. There is also a significant question on the impact upon the rate of corneal donation if consent for a brain autopsy was also needed. A reduction in donors and a return to waiting lists is also not in the best interest of the patient or patient outcomes.
 
[pages 4 and 5 skipped, you can read full text at below URL...tss]
 
Sincerely,
 
Richard L. Fuller President/CEO Tissue Banks International
 
 
Monday, August 31, 2009
 
HUMAN BODY PARTS FOR SALE TO THE HIGHEST BIDDER Inside a Creepy Global Body Parts Business
 
 
Subject: New guidance on decontamination of trial contact lenses and other contact devices has been revealed for CJD AND vCJD
 
From: "Terry S. Singeltary Sr."
 
Reply-To: Creutzfeldt-Jakob Disease
 
Date: Fri, 4 Dec 2009 15:59:48 +0000
 
Content-Type: text/plain
 
Parts/Attachments: Parts/Attachments text/plain (1009 lines) Reply Reply
 
New DoH guidance on decontaminating lenses
 
December 4th, 2009
 
New guidance on decontamination of trial contact lenses and other contact devices has been revealed.
 
The latest recommendations from the Department of Health’s Advisory Committee on Dangerous Pathogens (ACDP) replace previous guidance issued amid fears that Creutzfeldt-Jakob Disease (CJD) and variant CJD (vCJD), the human form of bovine spongiform encephalopathy (BSE, ‘mad cow disease’), could theoretically be transmitted from person to person by contact lenses and other devices such as tonometer heads and diagnostic lenses.
 
Details were discussed at the British Contact Lens Association’s Pioneers’ Conference in London late in November.
 
Professor Roger Buckley (pictured), a member of the ophthalmology subgroup of the ACDP’s Transmissible Spongiform Encephalopathy Working Group established to review this advice, told BCLA members at the Royal Society of Medicine that there had been no known cases of transmission of CJD/vCJD resulting from contact lens wear or diagnostic examination, and there was now thought to be a low level of risk of infectivity of the cornea and ocular surface.
 
Under the new guidance, six steps are required to minimise the risk of transmission via re-used contact devices. The lens or device should be: decontaminated immediately after contact with the eye surface; rinsed in Water for Irrigation BP (not tap water) for not less than 30 seconds; cleaned on all surfaces with a liquid soap or detergent, then rinsed in Water for Irrigation BP for a further 30 seconds; immersed in a freshly-prepared solution of sodium hypochlorite providing 10,000ppm of available chlorine for ten minutes; rinsed in three changes of Water for Irrigation BP for a total of not less than ten minutes; shaken to remove excess water, dried with a disposable tissue, and stored dry in a suitable container.
 
Any further measure (such as autoclaving) can then be carried out, if this is necessary and if the device is designed to withstand such a process. Otherwise, it is ready for immediate re-use.
 
 
Transmissible Spongiform Encephalopathy Agents: Safe Working and the Prevention of Infection: Annex C ANNEX C General principles of decontamination and waste disposal
 
 
Alert to Urological Surgeons TRANSRECTAL PROSTATIC BIOPSY IN MEN AT RISK OF VARIANT CJD
 
 
Ophthalmology The Ophthalmology subgroup met twice in 2008 on 7th April and 20th June to discuss issues relating to CJD infection control in ophthalmology. The following topic groups were identified: 17 • Anterior eye • Posterior eye • Assessment tool • Decontamination of ophthalmic surgical instruments • Examination, diagnostic equipment and contact lenses • CJD incident management Members were assigned to relevant topic groups, and discussions and research were coordinated by a topic group lead. The topic groups then produced draft guidance on their particular areas of expertise towards the end of 2008. Pathology (Annex K) The Working Group drafted guidelines for pathologists and pathology laboratories for the handling of tissues from patients with, or at risk of, CJD. This document (Annex K of the Working Group guidance) is aimed at pathologists and individuals working in pathology laboratories who handle tissues from patients. It aims to ensure that laboratory staff are aware of risk factors for CJD prior to carrying out procedures on tissues. The draft annex was sent out for a limited consultation with representatives from the Royal College of Pathologists, the Institute for Biomedical Sciences, the British Neuropathological Society and the Health and Safety Executive. The Annex was approved by the Working Group at their December 2008 meeting. Annex K has since been published at:
 
 
Pre-surgery assessment (Annex J) The updates to Annex J were approved for publication by the Working Group at their February 2008 meeting, subject to some minor adjustments. The Annex was then signed off by the ACDP Chairman and published on 1st May 2008 at:
 
 
see full text ;
 
 
Friday, July 17, 2009 Revision to pre-surgical assessment of risk for vCJD in neurosurgery and eye surgery units Volume 3 No 28; 17 July 2009
 
 
Wednesday, August 20, 2008 Tonometer disinfection practice in the United Kingdom: A national survey
 
 
CJD Human Cornea Tissue, Recall END OF ENFORCEMENT REPORT FOR AUGUST 5, 2009 Posted Aug 07 2009 6:32pm
 
 
 
From: TSS
 
Subject: Tonometer prism sterilisation: A local and UK national survey (TSE)
 
Date: August 24, 2007 at 1:24 pm PST
 
1: Cont Lens Anterior Eye. 2007 Aug 17; [Epub ahead of print]
 
Tonometer prism sterilisation: A local and UK national survey.
 
Chandra A, Barsam A, Hammond CJ. West Kent Eye Centre, Princess Royal University Hospital, Orpington, Kent BR6 8ND, UK.
 
PURPOSE: First to audit local adherence to a protocol of use of an alcohol wipe for each tonometry, and secondly to assess current practice nationally in the UK. METHOD: The audit was carried out at two units: The West Kent Eye Centre at the Princess Royal University Hospital (Orpington, UK) and Queen Mary's Hospital (Sidcup, UK). The standard set for this audit was 100% sterilisation. During a 1-week period in November 2005, the number of alcohol wipes was counted in each consultation room after outpatient clinics, with the doctors being assessed blind to the survey. The number of Goldman applanation tonometry intra-ocular pressures recorded by each clinician was counted by inspection of the medical records of patients seen. Secondly, departments listed in the UK Directory of Training Posts were contacted by telephone and the senior nurse was interviewed. They were asked directly about their department's tonometer prism sterilisation and management. RESULTS: The local audit showed only 54% of tonometry measurements were associated with sterilisation using an alcohol-impregnated wipe. The national survey included 140 of the 152 UK training departments. Thirty-three (23.6%) departments used disposable tonometer prisms routinely. The remaining 107 (76.4%) used non-disposable prisms. Eighty-five (60.7%) departments provided sodium hypochlorite for prism sterilisation, with 69 (81.2%) of these departments providing more than one prism/clinician to allow full exposure to the disinfectant. Twenty-two (15.7%) departments used alcohol wipes. Only 8 (7.5%) of the 107 departments using non-disposable prisms tracked these prisms, despite Royal College of Ophthalmologists guidelines that they should be. These same 8 (7.5%) departments replaced the non-disposable prisms as per manufacturer guidelines. 19.3% of charge nurses were aware of a policy for tonometry in patients with, or at risk of, prion disease. CONCLUSIONS: This study highlights that sterilisation of tonometer prisms was inconsistent in a local audit. Nationally, practices were varied. The majority of ophthalmology departments continued to use non-disposable tonometer prisms, but few seemed aware of the Royal College of Ophthalmologists' recommendation that disposable prisms are used in patients at risk of prion disease, and few track tonometer heads or replace them according to manufacturers guidelines. Use of disposable tonometer prisms would seem to reduce concerns about sterilisation, as well as prevent spread of common pathogens.
 
PMID: 17703987 [PubMed - as supplied by publisher]
 
 
PA-34
 
SPORADIC CREUTZFELDT-JAKOB DISEASE: PRPRES IS CONSTANTLY PRESENT IN THE RETINA, AND RARELY IN THE OPTIC NERVE
 
M. Mangieri1, G. Giaccone1, L. Limido1, G. Di Fede1, S. Suardi1, R. Capobianco1, P. Fociani2, O. Bugiani1, F. Tagliavini1 1 Istituto Nazionale Neurologico Carlo Besta, Division of Neuropathology and Neurology 5, Milano, Italy and 2 Ospedale Luigi Sacco, Division of Pathology, Università di Milano, Milano, Italy
 
e-mail: mmangieri@istituto-besta.it
 
Creutzfeldt-Jakob disease (CJD) is marked by the presence of the protease- resistant prion protein (PrPres) in the brain. Studies of the retina and optic nerve in patients with CJD are scanty and on very small series of patients. We analysed ocular tissues of sporadic CJD patients (retina of 58 and optic nerve of 51), representing all combinations of PRNP codon 129 polymorphisms and PrPres types by Parchi, except VV1. Ocular tissue from 24 patients with other neurological diseases were used as controls. The ocular tissue was collected at autopsy and the samples were fixed in Carnoy solution or frozen. Before immunohistochemistry with 3F4 antibody, the sections were pretreated with proteinase K and guanidine thiocyanate. In all cases of sCJD the retina showed immunoreactivity for PrPres localized in the inner and outer plexiform layers, with a synaptic type of labelling. No difference in the pattern of labeling was detected between CJD patients with different PRNP codon 129 polymorphisms and PrPres types in the brain. In all cases with frozen retinal tissue available (n = 18), the immunoblot was positive for PrPres . Two out of the 51 sCJD showed the deposition of PrPres also in the optic nerve, corresponding to an immunostaining delineating stellate cells and associated with the presence of numerous CD68- and CD45-positive cells. Our results demonstrate the presence of the pathological form of prion protein not only in the retina of all sCJD cases analysed, but also in optic nerve in a small subset of sCJD patients, a finding previously described only in variant CJD and in experimental animal models. Moreover, our data suggest a correlation between the deposition of PrPres and inflammatory changes in the optic nerve in sCJD.
 
143
 
 
----- Original Message -----
 
From: "Terry S. Singeltary Sr." <[log in to unmask]>
 
To: <[log in to unmask]>
 
Sent: Thursday, December 28, 2006 10:23 AM
 
Subject: Ophthalmic Surgery in Prion Diseases
 
Volume 13, Number 1–January 2007 Dispatch Ophthalmic Surgery in Prion Diseases Tsuyoshi Hamaguchi,*1 Moeko Noguchi-Shinohara,* Yosikazu Nakamura,†2 Takeshi Sato,‡2 Tetsuyuki Kitamoto,§2 Hidehiro Mizusawa,¶2 and Masahito Yamada*2 *Kanazawa University Graduate School of Medical Science, Kanazawa, Japan; †Jichi Medical University, Shimotsuke, Japan ‡National Center for Neurology and Psychiatry, Ichikawa, Japan; §Tohoku University Graduate School of Medicine, Sendai, Japan; and ¶Tokyo Medical and Dental University, Tokyo, Japan
 
Suggested citation for this article
 
Abstract Eleven (1.8%) of 597 patients underwent ophthalmic surgery within 1 month before the onset of prion disease or after the onset. All ophthalmologists reused surgical instruments that had been incompletely sterilized to eliminate infectious prion protein. Ophthalmologists should be aware of prion diseases as a possible cause of visual symptoms and use disposable instruments whenever possible.
 
Visual impairment occurs in 10% to 20% of patients with sporadic Creutzfeldt- Jakob disease (sCJD) during an early stage of the disease (Heidenhain variant) (1,2). Some patients with prion diseases may visit ophthalmologists with visual impairment due to prion diseases or with coexisting age-related eye diseases (3,4).
 
Infectious prion protein (PrPSc) was identified in the retina and optic nerve in patients with variant CJD (vCJD) and sCJD (5,6), and CJD has been transmitted by corneal transplantation (7,8). In the World Health Organization (WHO) guidelines, eyes were classified as highly infectious tissues (9).
 
Secondary transmission of PrPSc through ophthalmic surgery could possibly be prevented around the onset of prion diseases, although surgery that is performed long before the onset of prion diseases would not have that potential. It is important to understand the current status of ophthalmic surgery for patients with prion diseases and to clarify the clinical features of the patients with prion diseases who undergo ophthalmic surgery. Here, we describe the relevant data from CJD surveillance in Japan.
 
The Study.....snip full text ;
 
 
----- Original Message -----
 
From: "Terry S. Singeltary Sr." <[log in to unmask]>
 
To: <[log in to unmask]>
 
Sent: Wednesday, December 27, 2006 12:21 PM
 
Subject: ABNORMAL PRION ACCUMULATION ASSOCIATED WITH RETINAL PATHOLOGY IN EXPERIMENTALLY INOCULATED SCRAPIE-AFFECTED SHEEP
 
 
Eye procedure raises CJD concerns
 
November 19, 2004 United Press International by STEVE MITCHELL
 
A New York man who died from a rare brain disorder similar to mad cow disease in May underwent an eye procedure prior to his death that raises concerns about the possibility of transmitting the fatal disease to others, United Press International has learned. The development comes on the heels of the announcement Thursday by U.S. Department of Agriculture officials of a possible second case of mad cow disease in U.S. herds.
 
Richard Da Silva, 58, of Orange County, N.Y., died from Creutzfeldt Jakob disease, an incurable brain-wasting illness that strikes about one person per million.
 
Richard's wife Ann Marie Da Silva told UPI he underwent a check for the eye disease glaucoma in 2003, approximately a year before his death. The procedure involves the use of a tonometer, which contacts the cornea -- an eye tissue that can contain prions, the infectious agent thought to cause CJD.
 
Ann Marie's concern is that others who had the tonometer used on them could have gotten infected.
 
A 2003 study by British researchers suggests her concerns may be justified. A team led by J.W. Ironside from the National Creutzfeldt-Jakob Disease Surveillance Unit at the University of Edinburgh examined tonometer heads and found they can retain cornea tissue that could infect other people -- even after cleaning and decontaminating the instrument.
 
"Retained corneal epithelial cells, following the standard decontamination routine of tonometer prisms, may represent potential prion infectivity," the researchers wrote in the British Journal of Ophthalmology last year. "Once the infectious agent is on the cornea, it could theoretically infect the brain."
 
Prions, misfolded proteins thought to be the cause of mad cow, CJD and similar diseases, are notoriously difficult to destroy and are capable of withstanding most sterilization procedures.
 
Laura Manuelidis, an expert on these diseases and section chief of surgery in the neuropathology department at Yale University, agreed with the British researchers that tonometers represent a potential risk of passing CJD to other people.
 
Manuelidis told UPI she has been voicing her concern about the risks of corneas since 1977 when her own study, published in the New England Journal of Medicine, showed the eye tissue, if infected, could transmit CJD.
 
At the time the procedure was done on Richard Da Silva, about a year before he died, she said it was "absolutely" possible he was infectious.
 
The CJD Incidents Panel, a body of experts set up by the U.K. Department of Health, noted in a 2001 report that procedures involving the cornea are considered medium risk for transmitting CJD. The first two patients who have a contaminated eye instrument used on them have the highest risk of contracting the disease, the panel said.
 
In 1999, the U.K. Department of Health banned opticians from reusing equipment that came in contact with patients' eyes out of concern it could result in the transmission of variant CJD, the form of the disease humans can contract from consuming infected beef products.
 
Richard Da Silva was associated with a cluster of five other cases of CJD in southern New York that raised concerns about vCJD.
 
None of the cases have been determined to stem from mad cow disease, but concerns about the cattle illness in the United States could increase in light of the USDA announcement Thursday that a cow tested positive on initial tests for the disease. If confirmed, this would be the second U.S. case of the illness; the first was detected in a Washington cow last December. The USDA said the suspect animal disclosed Thursday did not enter the food chain. The USDA did not release further details about the cow, but said results from further lab tests to confirm the initial tests were expected within seven days.
 
Ann Marie Da Silva said she informed the New York Health Department and later the eye doctor who performed the procedure about her husband's illness and her concerns about the risk of transmitting CJD via the tonometer.
 
The optometrist -- whom she declined to name because she did not want to jeopardize his career -- "didn't even know what this disease was," she said.
 
"He said the health department never called him and I called them (the health department) back and they didn't seem concerned about it," she added. "I just kept getting angrier and angrier when I felt I was being dismissed."
 
She said the state health department "seems to have an attitude of don't ask, don't tell" about CJD.
 
"There's a stigma attached to it," she said. "Is it because they're so afraid the public will panic? I don't know, but I don't think that the answer is to push things under the rug."
 
New York State Department of Health spokeswoman Claire Pospisil told UPI she would look into whether the agency was concerned about the possibility of transmitting CJD via tonometers, but she had not called back prior to story publication.
 
Disposable tonometers are readily available and could avoid the risk of transmitting the disease, Ironside and colleagues noted in their study. Ann Marie Da Silva said she asked the optometrist whether he used disposable tonometers and "he said 'No, it's a reusable one.'"
 
Ironside's team also noted other ophthalmic instruments come into contact with the cornea and could represent a source of infection as they are either difficult to decontaminate or cannot withstand the harsh procedures necessary to inactivate prions. These include corneal burrs, diagnostic and therapeutic contact lenses and other coated lenses.
 
Terry Singletary, whose mother died from a type of CJD called Heidenhain Variant, told UPI health officials were not doing enough to prevent people from being infected by contaminated medical equipment.
 
"They've got to start taking this disease seriously and they simply aren't doing it," said Singletary, who is a member of CJD Watch and CJD Voice -- advocacy groups for CJD patients and their families.
 
U.S. Centers for Disease Control and Prevention spokeswoman Christine Pearson did not return a phone call from UPI seeking comment. The agency's Web site states the eye is one of three tissues, along with the brain and spinal cord, that are considered to have "high infectivity."
 
The Web site said more than 250 people worldwide have contracted CJD through contaminated surgical instruments and tissue transplants. This includes as many as four who were infected by corneal grafts. The agency noted no such cases have been reported since 1976, when sterilization procedures were instituted in healthcare facilities.
 
Ironside and colleagues noted in their study, however, many disinfection procedures used on optical instruments, such as tonometers, fail. They wrote their finding of cornea tissue on tonometers indicates that "no current cleaning and disinfection strategy is fully effective."
 
Singletary said CDC's assertion that no CJD cases from infected equipment or tissues have been detected since 1976 is misleading.
 
"They have absolutely no idea" whether any cases have occurred in this manner, he said, because CJD cases often aren't investigated and the agency has not required physicians nationwide report all casesof CJD.
 
"There's no national surveillance unit for CJD in the United States; people are dying who aren't autopsied, the CDC has no way of knowing" whether people have been infected via infected equipment or tissues, he said.
 
Ann Marie Da Silva said she has contacted several members of her state's congressional delegation about her concerns, including Rep. Sue Kelly, R-N.Y., and Sen. Charles Schumer, D-N.Y.
 
"Basically, what I want is to be a positive force in this, but I also want more of a dialogue going on with the public and the health department," she said.
 
 
 
From: Terry S. Singeltary Sr. (216-119-138-149.ipset18.wt.net)
 
Subject: Trial Contact Lenses and RE-USE in the U.S. & CJD, no threat says FDA???
 
Date: October 7, 2000 at 9:19 am PST
 
Subject: Trial Contact Lenses and RE-USE in the U.S. & CJD, no threat says FDA???
 
Date: Sat, 7 Oct 2000 10:27:03 –0700
 
From: "Terry S. Singeltary Sr."
 
Reply-To: Bovine Spongiform Encephalopathy
 
To: BSE-L@uni-karlsruhe.de
 
######### Bovine Spongiform Encephalopathy #########
 
Greetings List Members,
 
It just never ceases to amaze me, that the FDA and the rest of the different political governmental bodies that are suppose to protect us, but instead, choose to protect their best interest$ the corporate industries that donate all the cash$ there has been plenty of warning and tests to prove of some sort of infectivity in the eyes. the fda speaks of no test for the tears, well hell, there is no test that will detect it period, but yet you still die from it. does not mean it is not there. when will they wake up? sporadic CJD is coming from somewhere and they had better start looking as to where it is coming from. this is just more typical B.S.eee. please read the ignorance, below....
 
thank you, Terry S. Singeltary Sr., Bacliff, Texas USA
 
Subject: 09-018 - gsc - flounder@wt.net - concern of CJD transmission with trial contact lenses
 
Date: Fri, 06 Oct 2000 14:49:24 –0400
 
From: "Clark, Geoffrey S."
 
To: 'flounder@wt.net'
 
CC: "Warburton, Karen" , "CDRH Small Manu. Assistance"
 
Terry S. Singeltary Sr.:
 
I have discussed your concerns with our Device Evaluation staff who have provided the following information:
 
The risk of CJD transmission through the use of trial (fitting) contact lenses is extremely low and at present unmeasureable. There have been no documented cases of CJD transmission via a contact lens or contact lens solution. It is highly unlikely that the CJD agent would be present on the surface of the cornea or present in the tears of an infected person. Additionally, the use of trial lens sets has been declining in the US over the past 10 years as the soft contact lens market has shifted to lenses dispensed in non-reusable blister packs. Therefore, a formal recommendation against the use of trial lenses because of potential CJD transmission is not appropriate at this time.
 
Please feel free to contact me if I can be of additional assistance.
 
=|<: 130h="" 5074275="" cdrh.fda.gov="" clark="" eoff="" from="" gsc="" pc="" room=""> (HFZ- 220) 1350 Piccard Dr., Rockville, MD 20850-4307 800.638.2041x122 fax.301.443.8818
 
==========================================================
 
From: Winston, F. Blix
 
Sent: Tuesday, September 05, 2000 5:00 PM
 
To: Clark, Geoffrey S.
 
Subject: 09-018 - flounder@wt.net Geoff,
 
This came into the DSMA account. Would you please respond?
 
Thanks, Blix
 
-----Original Message-----
 
From: WEBO@CDRH.FDA.GOV [SMTP:WEBO@CDRH.FDA.GOV]
 
Sent: Friday, September 01, 2000 9:27 AM
 
To: DSMA@CDRH.FDA.GOV
 
Subject: DSMA Email Form Response
 
Name: Terry S. Singeltary Sr. Phone Number: Na Fax Number: Na Email Address: flounder@wt.net Mailing Address: P.O. Box 42 Bacliff, Texas USA 77518
 
Questions/Comments: P990072 *In relation to human Transmissible Spongiform Encephalopathy and Contact Lens
 
i think it would be to everyone's best interest, "IF" the practice of "RE-USING" _display_ contact lens in the commercial aspect takes place in the U.S., this practice must be stopped. Please allow me to explain. In the U.K., the practice of having display of different colors of contact lens on display, for the consumer to try on, and see how they look with the different colors. These contact lens were then re-used over and over. No standard cleaning cleaning solution and or auto-claving procedure will kill the TSE agent. This is known fact. Plus, the U.K. has 'BANNED' this practice due to vCJD and sCJD (if i am not mistaken), but regardless, both can transmit the TSE agent. I will not waste my time trying to explain this in this box, will post a few URLS and you will see what i speak of if you care. But the eyes, brain, pituitary are the most infectious parts, of an infective species. Trust me, i know what i speak of.......
 
thank you, Terry S. Singeltary SR. ======================= ISSUE 1490
 
Thursday 24 June 1999
 
CJD alert over contact lenses By David Brown, Agriculture Editor
 
PEOPLE risk catching the human form of mad cow disease from re-used contact lenses, scientists warned the Government yesterday. They urged the Department of Health to stop opticians re-using trial lenses among their clients to help prevent further outbreaks of the new variant Creutzfeldt-Jakob disease which has killed 41 people so far.
 
The warning came from the Spongiform Encephalopathies Advisory Committee, the independent team of scientists advising the Government on BSE and CJD. Ministers are expected to ban the re-use of these lenses as a precaution, which could mean higher prices for consumers.
 
In a statement, the committee said: "Any potential risk is probably very low, but the committee felt strongly that the Department of Health should encourage opticians to adopt, as a matter of best practice, the single use of trial lenses followed by safe disposal." Sir John Pattison, the committee's chairman, said it was surprised to learn that it was common practice among opticians to try the same contact lenses on several clients.
 
It was known that the classical variety of CJD, the kind which was known before the new variant (vCJD) was announced in 1996 and linked to BSE, had been spread in the past by transplants of infected corneas.
 
The committee noted that the eyes are directly connected to the brain, the main seat of BSE and CJD. The warning applied to vCJD and the classical variety of the fatal brain disease.
 
 
JOURNAL OF NEUROLOGY
 
MARCH 26, 2003
 
RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States
 
Email Terry S. Singeltary:
 
flounder@wt.net
 
I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to comment on the CDC's attempts to monitor the occurrence of emerging forms of CJD. Asante, Collinge et al [1] have reported that BSE transmission to the 129- methionine genotype can lead to an alternate phenotype that is indistinguishable from type 2 PrPSc, the commonest sporadic CJD. However, CJD and all human TSEs are not reportable nationally. CJD and all human TSEs must be made reportable in every state and internationally. I hope that the CDC does not continue to expect us to still believe that the 85%+ of all CJD cases which are sporadic are all spontaneous, without route/source. We have many TSEs in the USA in both animal and man. CWD in deer/elk is spreading rapidly and CWD does transmit to mink, ferret, cattle, and squirrel monkey by intracerebral inoculation. With the known incubation periods in other TSEs, oral transmission studies of CWD may take much longer. Every victim/family of CJD/TSEs should be asked about route and source of this agent. To prolong this will only spread the agent and needlessly expose others. In light of the findings of Asante and Collinge et al, there should be drastic measures to safeguard the medical and surgical arena from sporadic CJDs and all human TSEs. I only ponder how many sporadic CJDs in the USA are type 2 PrPSc?
 
 
Monday, December 31, 2007
 
Risk Assessment of Transmission of Sporadic Creutzfeldt-Jakob Disease in Endodontic Practice in Absence of Adequate Prion Inactivation
 
 
Subject: CJD: update for dental staff
 
Date: November 12, 2006 at 3:25 pm PST
 
1: Dent Update. 2006 Oct;33(8):454-6, 458-60.
 
CJD: update for dental staff.
 
 
Wednesday, August 20, 2008
 
*** Tonometer disinfection practice in the United Kingdom: A national survey ***
 
 
Tuesday, August 12, 2008
 
Biosafety in Microbiological and Biomedical Laboratories Fifth Edition 2007 (occupational exposure to prion diseases)
 
 
Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States 2003 revisited 2009
 
August 10, 2009
 
Greetings,
 
I would like to submit a review of past CJD surveillance in the USA, and the urgent need to make all human TSE in the USA a reportable disease, in every state, of every age group, and to make this mandatory immediately without further delay. The ramifications of not doing so will only allow this agent to spread further in the medical, dental, surgical arena's. North America seems to have the most species with documented Transmissible Spongiform Encephalopathy's, most all of which have been rendered and fed back to food producing animals and to humans for years. If you look at the statistics, sporadic CJD seems to be rising in the USA, and has been, with atypical cases of the sCJD. I find deeply disturbing in the year of 2009, that Human Transmissible Spongiform Encephalopathy of any strain and or phenotype, of all age groups, and I stress all age groups, because human TSE's do not know age, and they do not know borders. someone 56 years old, that has a human TSE, that has surgery, can pass this TSE agent on i.e. friendly fire, and or passing it forward, and there have been documented nvCJD in a 74 year old. Remembering also that only sporadic CJD has been documented to transmit via iatrogenic routes, until recently with the 4 cases of blood related transmission, of which the origin is thought to be nvCJD donors. However most Iatrogenic CJD cases are nothing more than sporadic CJD, until the source is proven, then it becomes Iatrogenic. An oxymoron of sorts, because all sporadic CJD is, are multiple forms, or strains, or phenotypes of Creutzfeldt Jakob Disease, that the route and source and species have not been confirmed and or documented. When will the myth of the UKBSEnvCJD only theory be put to bed for good. This theory in my opinion, and the following there from, as the GOLD STANDARD, has done nothing more than help spread this agent around the globe. Politics and money have caused the terrible consequences to date, and the fact that TSEs are a slow incubating death, but a death that is 100% certain for those that are exposed and live long enough to go clinical. once clinical, there is no recourse, to date. But, while sub-clinical, how many can one exposed human infect? Can humans exposed to CWD and scrapie strains pass it forward as some form of sporadic CJD in the surgical and medical arenas? why must we wait decades and decades to prove this point, only to expose millions needlessly, only for the sake of the industries involved? would it not have been prudent from the beginning to just include all TSE's, and rule them out from there with transmission studies and change policies there from, as opposed to doing just the opposite? The science of TSE's have been nothing more than a political circus since the beginning, and for anyone to still believe in this one strain, one group of bovines, in one geographical location, with only one age group of human TSE i.e. nvCJD myth, for anyone to believe this today only enhances to spreading of these human and animal TSE's. This is exactly why we have been in this quagmire.
 
The ones that believe that there is a spontaneous CJD in 85%+ of all cases of human TSE, and the ones that do not believe that cattle can have this same phenomenon, are two of the same, the industry, and so goes the political science aspect of this tobacco and or asbestos scenario i.e. follow the money. I could go into all angles of this man made nightmare, the real facts and science, for instance, the continuing rendering technology and slow cooking with low temps that brewed this stew up, and the fact that THE USA HAD THIS TECHNOLOGY FIRST AND SHIPPED IT TO THE U.K. SOME 5 YEARS BEFORE THE U.S. STARTED USING THE SAME TECHNOLOGY, to save on fuel cost. This is what supposedly amplified the TSE agent via sheep scrapie, and spread via feed in the U.K. bovine, and other countries exporting the tainted product. BUT most everyone ignores this fact, and the fact that the U.S. has been recycling more TSE, from more species with TSEs, than any other country documented, but yet, it's all spontaneous, and the rise in sporadic CJD in the U.S. is a happenstance of bad luck ??? I respectfully disagree. To top that all off, the infamous BSE-FIREWALL that the USDA always brags about was nothing more than ink on paper, and I can prove this. YOU can ignore it, but this is FACT (see source, as late as 2007, in one recall alone, some 10,000,000 MILLION POUNDS OF BANNED MAD COW FEED WENT OUT INTO COMMERCE TO BE FED OUT, and most was never recovered. This was banned blood laced, meat and bone meal. 2006 was a banner year for banned mad cow protein going into commerce in the U.S. (see source of FDA feed ban warning letter below). I stress that the August 4, 1997 USA mad cow feed ban and this infamous BSE firewall, was nothing more than ink on paper, it was never enforceable.
 
I propose that the current diagnostic criteria for human TSEs only enhances and helps the spreading of human TSE from the continued belief of the UKBSEnvCJD only theory in 2009. With all the science to date refuting it, to continue to validate this old myth, will only spread this TSE agent through a multitude of potential routes and sources i.e. consumption, medical i.e., surgical, blood, dental, endoscopy, optical, nutritional supplements, cosmetics etc. I propose as with Aguzzi, Asante, Collinge, Caughey, Deslys, Dormont, Gibbs, Gajdusek, Ironside, Manuelidis, Marsh, et al and many more, that the world of TSE Transmissible Spongiform Encephalopathy is far from an exact science, but there is enough proven science to date that this myth should be put to rest once and for all, and that we move forward with a new classification for human and animal TSE that would properly identify the infected species, the source species, and then the route. This would further have to be broken down to strain of species and then the route of transmission would further have to be broken down. Accumulation and Transmission are key to the threshold from sub- clinical to clinical disease, and key to all this, is to stop the amplification and transmission of this agent, the spreading of, no matter what strain. In my opinion, to continue with this myth that the U.K. strain of BSE one strain TSE in cows, and the nv/v CJD one strain TSE humans, and the one geographical location source i.e. U.K., and that all the rest of human TSE are just one single strain i.e. sporadic CJD, a happenstance of bad luck that just happens due to a twisted protein that just twisted the wrong way, IN 85%+ OF ALL HUMAN TSEs, when to date there are 6 different phenotypes of sCJD, and growing per Gambetti et al, and that no other animal TSE transmits to humans ??? With all due respect to all Scientist that believe this, I beg to differ. To continue with this masquerade will only continue to spread, expose, and kill, who knows how many more in the years and decades to come. ONE was enough for me, My Mom, hvCJD i.e. Heidenhain Variant CJD, DOD 12/14/97 confirmed, which is nothing more than another mans name added to CJD, like CJD itself, Jakob and Creutzfeldt, or Gerstmann-Straussler-Scheinker syndrome, just another CJD or human TSE, named after another human. WE are only kidding ourselves with the current diagnostic criteria for human and animal TSE, especially differentiating between the nvCJD vs the sporadic CJD strains and then the GSS strains and also the FFI fatal familial insomnia strains or the ones that mimics one or the other of those TSE? Tissue infectivity and strain typing of the many variants of the human and animal TSEs are paramount in all variants of all TSE. There must be a proper classification that will differentiate between all these human TSE in order to do this. With the CDI and other more sensitive testing coming about, I only hope that my proposal will some day be taken seriously. ...
 
please see history, and the ever evolving TSE science to date ;
 
Saturday, June 13, 2009
 
Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States 2003 revisited 2009
 
 
Thursday, November 05, 2009
 
Incidence and spectrum of sporadic Creutzfeldt-Jakob disease variants with mixed phenotype and co-occurrence of PrPSc types: an updated classification
 
 
Tuesday, August 11, 2009
 
Characteristics of Established and Proposed Sporadic Creutzfeldt-Jakob Disease Variants
 
Brian S. Appleby, MD; Kristin K. Appleby, MD; Barbara J. Crain, MD, PhD; Chiadi U. Onyike, MD, MHS; Mitchell T. Wallin, MD, MPH; Peter V. Rabins, MD, MPH
 
Background: The classic Creutzfeldt-Jakob disease (CJD), Heidenhain, and Oppenheimer-Brownell variants are sporadic CJD (sCJD) phenotypes frequently described in the literature, but many cases present with neuropsychiatric symptoms, suggesting that there may be additional sCJD phenotypes.
 
Objective: To characterize clinical, diagnostic, and molecular features of 5 sCJD variants.
 
Design: Retrospective analysis.
 
Setting: The Johns Hopkins and Veterans Administration health care systems.
 
Participants: Eighty-eight patients with definite or probable sCJD.
 
Main Outcome Measures: Differences in age at onset, illness progression, diagnostic test results, and molecular subtype.
 
Results: The age at onset differed among sCJD variants (P=.03); the affective variant had the youngest mean age at onset (59.7 years). Survival time (P .001) and the time to clinical presentation (P=.003) differed among groups. Patients with the classic CJD phenotype had the shortest median survival time from symptom onset (66 days) and those who met criteria for the affective sCJD variant had the longest (421 days) and presented to clinicians significantly later (median time from onset to presentation, 92 days; P=.004). Cerebrospinal fluid analyses were positive for 14-3-3 protein in all of the affective variants, regardless of illness duration. Periodic sharp-wave complexes were not detected on any of the electroencephalography tracings in the Oppenheimer-Brownell group; basal ganglia hyperintensity was not detected on brain magnetic resonance imaging in this group either. All of the Heidenhain variants were of the methionine/ methionine type 1 molecular subtype.
 
Conclusions: The classic CJD phenotype and the Heidenhain, Oppenheimer- Brownell, cognitive, and affective sCJD variants differ by age at disease onset, survival time, and diagnostic test results. Characteristics of these 5 phenotypes are provided to facilitate further clinicopathologic investigation that may lead to more reliable and timely diagnoses of sCJD.
 
Arch Neurol. 2009;66(2):208-215
 
snip...
 
COMMENT
 
snip...see full text ;
 
 
Sunday, May 10, 2009
 
Meeting of the Transmissible Spongiform Encephalopathies Committee On June 12, 2009 (Singeltary submission)
 
 
Thursday, January 29, 2009
 
Medical Procedures and Risk for Sporadic Creutzfeldt-Jakob Disease, Japan, 1999-2008 (WARNING TO Neurosurgeons and Ophthalmologists) Volume 15, Number 2-February 2009 Research
 
 
Friday, December 04, 2009
 
New guidance on decontamination of trial contact lenses and other contact devices has been revealed for CJD AND vCJD
 
 
Saturday, January 16, 2010
 
Evidence For CJD TSE Transmission Via Endoscopes 1-24-3 re-Singeltary to Bramble et al
 
Evidence For CJD/TSE Transmission Via Endoscopes
 
From Terry S. Singletary, Sr flounder@wt.net 1-24-3
 
Terry S. Singeltary Sr., P.O. BOX 42, Bacliff, Texas 77518 USA
 
 
Professor Michael Farthing wrote:
 
Louise Send this to Bramble (author) for a comment before we post. Michael
 
 
Thursday, July 08, 2010
 
GLOBAL CLUSTERS OF CREUTZFELDT JAKOB DISEASE - A REVIEW 2010
 
 
Saturday, February 12, 2011
 
Another Pathologists dies from CJD, another potential occupational death ?
 
another happenstance of bad luck, a spontaneous event from nothing, or friendly fire ???
 
 
Tuesday, July 31, 2012
 
11 patients may have been exposed to fatal disease Creutzfeldt-Jakob Disease CJD Greenville Memorial Hospital
 
 
Thursday, August 02, 2012
 
CJD case in Saint John prompts letter to patients Canada CJD case in Saint John prompts letter to patients
 
 
Thursday, October 25, 2012
 
Current limitations about the cleaning of luminal endoscopes and TSE prion risk factors there from
 
Article in Press
 
 
Thursday, April 12, 2012
 
Health professions and risk of sporadic Creutzfeldt–Jakob disease, 1965 to 2010
 
Eurosurveillance, Volume 17, Issue 15, 12 April 2012
 
Research articles
 
 
Tuesday, May 28, 2013
 
Late-in-life surgery associated with Creutzfeldt-Jakob disease: a methodological outline for evidence-based guidance
 
 
Sunday, June 9, 2013
 
TSEAC March 14, 2013: Transmissible Spongiform Encephalopathies Advisory Committee Meeting Webcast
 
 
Tuesday, May 21, 2013
 
CJD, TSE, PRION, BLOOD Abstracts of the 23rd Regional Congress of the International Society of Blood Transfusion, Amsterdam, The Netherlands, June 2-5, 2013
 
 
Tuesday, March 5, 2013
 
Use of Materials Derived From Cattle in Human Food and Cosmetics; Reopening of the Comment Period FDA-2004-N-0188-0051 (TSS SUBMISSION)
 
FDA believes current regulation protects the public from BSE but reopens comment period due to new studies
 
 
Tuesday, May 28, 2013
 
Late-in-life surgery associated with Creutzfeldt-Jakob disease: a methodological outline for evidence-based guidance
 
 
Monday, October 14, 2013
 
Researchers estimate one in 2,000 people in the UK carry variant CJD proteins
 
 
However, I think that the specific confusion there is that people talk about sporadic CJD occurring at 1 per million. That is not your individual risk. Your risk is 1 per million every year. Actually, it is nearer 2 per million per year of the population will develop sporadic CJD, but your lifetime risk of developing sporadic CJD is about 1 in 30,000. So that has not really changed. When people talk about 1 per million, often they interpret that as thinking it is incredibly rare. They think they have a 1-in-a-million chance of developing this disease. You haven’t. You’ve got about a 1-in-30,000 chance of developing it.
 
 
Cases of vCJD peaked in 2000, leading some scientists to speculate that the disease has an incubation period of about a decade. Yet studies of different forms of CJD suggest that the incubation time of vCJD could be much longer, indicating that many people in Britain could be carrying the infection without symptoms.
 
 
Saturday, October 19, 2013
 
***A comparative study of modified confirmatory techniques and additional immuno-based methods for non-conclusive autolytic Bovine spongiform encephalopathy cases
 
 
Sunday, October 27, 2013
 
A Kiss of a Prion: New Implications for Oral Transmissibility
 
 
Wednesday, December 11, 2013
 
*** Detection of Infectivity in Blood of Persons with Variant and Sporadic Creutzfeldt-Jakob Disease ***
 
 
Friday, July 19, 2013
 
Beaumont Hospital in Dublin assessing patients for CJD
 
 
Saturday, September 21, 2013
 
CJD CONFIRMED in patient at New Hampshire Department of Health and Human Services (DHHS), Catholic Medical Center (CMC), and the Manchester Health Department (MHD)
 
 
Thursday, September 26, 2013
 
Minimise transmission risk of CJD and vCJD in healthcare settings Guidance
 
 
Saturday, November 16, 2013
 
Management of neurosurgical instruments and patients exposed to creutzfeldt-jakob disease 2013 December
 
Infect Control Hosp Epidemiol.
 
 
Wednesday, November 27, 2013
 
NHS failed to sterilise surgical instruments contaminated with 'mad cow' disease
 
 
Wednesday, January 15, 2014
 
*** INFECTION PREVENTION AND CONTROL OF CJD, VCJD AND OTHER HUMAN PRION DISEASES IN HEALTHCARE AND COMMUNITY SETTINGS Variably Protease-Sensitive Prionopathy (VPSPr) January 15, 2014
 
 
*** vpspr, sgss, sffi, TSE, an iatrogenic by-product of gss, ffi, familial type prion disease, what it ???
 
 
Thursday, January 23, 2014
 
Medical Devices Containing Materials Derived from Animal Sources (Except for In Vitro Diagnostic Devices) [Docket No. FDA–2013–D–1574]
 
 
Sunday, April 06, 2014
 
SPORADIC CJD and the potential for zoonotic transmission there from, either directly or indirectly via friendly fire iatrogenic mode, evidence to date
 
 
“Cases of vCJD peaked in 2000, leading some scientists to speculate that the disease has an incubation period of about a decade. Yet studies of different forms of CJD suggest that the incubation time of vCJD could be much longer, indicating that many people in Britain could be carrying the infection without symptoms.”
 
 
Tuesday, April 01, 2014
 
Questions linger in U.S. CJD cases 2005, and still do in 2014
 
 
Monday, March 10, 2014
 
Investigators study silent variant of mad cow disease Galveston Daily News March 4, 2014
 
 
Wednesday, April 02, 2014
 
Distinct origins of dura mater graft-associated Creutzfeldt-Jakob disease: past and future problems
 
 
Saturday, April 19, 2014
 
Human prion diseases and the risk of their transmission during anatomical dissection
 
 
Sunday, January 19, 2014
 
*** National Prion Disease Pathology Surveillance Center Cases Examined1 as of January 8, 2014 ***
 
 
Saturday, April 19, 2014
 
Exploring the zoonotic potential of animal prion diseases: In vivo and in vitro approaches
 
 
Thursday, January 22, 2015 Transmission properties of atypical Creutzfeldt-Jakob disease: a clue to disease etiology?
 
 
Wednesday, April 08, 2015
 
Sporadic Creutzfeldt-Jakob Disease MM1+2C and MM1 Are Identical in Transmission Properties
 
 
Thursday, March 26, 2015
 
Variant CJD and blood transfusion: are there additional cases?
 
Vox Sanguinis (2014) 107, 220–225 ORIGINAL PAPER © 2014 International Society of Blood Transfusion DOI: 10.1111/vox.12161
 
 
Sunday, March 29, 2015
 
Uncommon prion disease induced in macaque ten years after scrapie inoculation
 
 
*** The potential impact of prion diseases on human health was greatly magnified by the recognition that interspecies transfer of BSE to humans by beef ingestion resulted in vCJD. While changes in animal feed constituents and slaughter practices appear to have curtailed vCJD, there is concern that CWD of free-ranging deer and elk in the U.S. might also cross the species barrier. Thus, consuming venison could be a source of human prion disease. Whether BSE and CWD represent interspecies scrapie transfer or are newly arisen prion diseases is unknown. Therefore, the possibility of transmission of prion disease through other food animals cannot be ruled out. There is evidence that vCJD can be transmitted through blood transfusion. There is likely a pool of unknown size of asymptomatic individuals infected with vCJD, and there may be asymptomatic individuals infected with the CWD equivalent. These circumstances represent a potential threat to blood, blood products, and plasma supplies.
 
 
Self-Propagative Replication of Ab Oligomers Suggests Potential Transmissibility in Alzheimer Disease
 
Received July 24, 2014; Accepted September 16, 2014; Published November 3, 2014
 
 
Singeltary comment ;
 
 
 
Comment from Terry Singeltary
 
This is a Comment on the Food and Drug Administration (FDA) Notice: Draft Guidance for Industry on Ensuring Safety of Animal Feed Maintained and Fed On-Farm; Availability
 
For related information, Open Docket Folder  Docket folder icon
 
 
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Guidance for Industry Ensuring Safety of Animal Feed Maintained and Fed On-Farm Draft Guidance FDA-2014-D-1180 Singeltary Comment
 
Greetings FDA et al,
 
I wish to comment on Guidance for Industry Ensuring Safety of Animal Feed Maintained and Fed On-Farm Draft Guidance FDA-2014-D-1180.
 
Once again, I wish to kindly bring up the failed attempt of the FDA and the ruminant to ruminant mad cow feed ban of August 4, 1997. This feed ban is still failing today, as we speak. Even more worrisome, is the fact it is still legal to feed cervids to cervids in the USA, in fact, the FDA only _recommends_ that deer and elk considered to be of _high_ risk for CWD do not enter the animal food chain, but there is NO law, its only voluntary, a recipe for a TSE prion disaster, as we have seen with the ruminant to ruminant feed ban for cattle, where in 2007, one decade post August 1997 mad cow feed ban, where in 2007 10,000,000 POUNDS OF BANNED BLOOD LACED MEAT AND BONE MEAL WHEN OUT INTO COMMERCE, TO BE FED OUT. Since 2007, these BSE feed ban rules have been breached time and time again. tons and tons of mad cow feed went out in Alabama as well, where one of the mad cows were documented, just the year before in 2006, and in 2013 and 2014, breaches so bad (OAI) Official Action Indicated were issued. those are like the one issued where 10 million pounds of banned blood laced meat and bone meal were fed out.
 
What is the use of having a Guidance for Industry Ensuring Safety of Animal Feed Maintained and Fed On-Farm Draft Guidance FDA-2014-D-1180, if it cannot be enforced, as we have seen with a mandatory ruminant to ruminant feed ban?
 
I strenuously once again urge the FDA and its industry constituents, to make it MANDATORY that all ruminant feed be banned to all ruminants, and this should include all cervids as soon as possible for the following reasons...
 
======
 
In the USA, under the Food and Drug Administrations BSE Feed Regulation (21 CFR 589.2000) most material (exceptions include milk, tallow, and gelatin) from deer and elk is prohibited for use in feed for ruminant animals. With regards to feed for non-ruminant animals, under FDA law, CWD positive deer may not be used for any animal feed or feed ingredients. For elk and deer considered at high risk for CWD, the FDA recommends that these animals do not enter the animal feed system.
 
***However, this recommendation is guidance and not a requirement by law.
 
======
 
31 Jan 2015 at 20:14 GMT
 
*** Ruminant feed ban for cervids in the United States? ***
 
31 Jan 2015 at 20:14 GMT
 
 
 
19 May 2010 at 21:21 GMT
 
*** Singeltary reply ; Molecular, Biochemical and Genetic Characteristics of BSE in Canada Singeltary reply ;
 
 
 
Tuesday, December 23, 2014
 
FDA PART 589 -- SUBSTANCES PROHIBITED FROM USE IN ANIMAL FOOD OR FEED VIOLATIONS OFFICIAL ACTION INDICATED OAI UPDATE DECEMBER 2014 BSE TSE PRION
 
 
 
2013
 
Sunday, December 15, 2013
 
FDA PART 589 -- SUBSTANCES PROHIBITED FROM USE IN ANIMAL FOOD OR FEED VIOLATIONS OFFICIAL ACTION INDICATED OAI UPDATE DECEMBER 2013 UPDATE
 
 
 
DOCKET-- 03D-0186 -- FDA Issues Draft Guidance on Use of Material From Deer and Elk in Animal Feed; Availability
Date: Fri, 16 May 2003 11:47:37 0500
EMC 1 Terry S. Singeltary Sr. Vol #: 1
 
 
 
 
 
PLEASE SEE FULL TEXT SUBMISSION ;
 
 
 
10,000,000+ LBS. of PROHIBITED BANNED MAD COW FEED I.E. BLOOD LACED MBM IN COMMERCE USA 2007
 
Date: March 21, 2007 at 2:27 pm PST
 
REASON
 
Blood meal used to make cattle feed was recalled because it was cross- contaminated with prohibited bovine meat and bone meal that had been manufactured on common equipment and labeling did not bear cautionary BSE statement.
 
VOLUME OF PRODUCT IN COMMERCE
 
42,090 lbs.
 
DISTRIBUTION
 
WI
 
REASON
 
Products manufactured from bulk feed containing blood meal that was cross contaminated with prohibited meat and bone meal and the labeling did not bear cautionary BSE statement.
 
VOLUME OF PRODUCT IN COMMERCE
 
9,997,976 lbs.
 
DISTRIBUTION
 
ID and NV
 
END OF ENFORCEMENT REPORT FOR MARCH 21, 2007
 
 
 
Terry S. Singeltary Sr.
 
*** See attached file(s)
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Guidance for Industry Ensuring Safety of Animal Feed Maintained and Fed On-Farm Terry Singeltary Comment
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Sunday, April 5, 2015
 
*** Guidance for Industry Ensuring Safety of Animal Feed Maintained and Fed On-Farm Draft Guidance FDA-2014-D-1180 ***
 
 
 
TSS
 
all iatrogenic CJD TSE prion disease is, is sporadic cjd TSE prion disease, until the iatrogenic event is discovered, documented, and put in the public domain. this very seldom happens due to trace back efforts, and the very industry that refuses to do any trace back for CJD, or any TSE prion disease. ...
 
kind regards, terry
 
 
layperson
 
MOM DOD 12/14/97 confirmed hvCJD. just made a promise to mom, never forget, never let them forget...
 
wasted days and wasted nights...Freddy Fender
 
stupid is, as stupid does, and some times you just can’t fix stupid $$$ TSS
 
 
Terry S. Singeltary Sr.
 

Saturday, April 4, 2015

The Glycosylation Status of PrPC Is a Key Factor in Determining Transmissible Spongiform Encephalopathy Transmission between Species

The Glycosylation Status of PrPC Is a Key Factor in Determining Transmissible Spongiform Encephalopathy Transmission between Species

 

Frances K. Wisemana*, Enrico Cancellottia, Pedro Piccardoa,c, Kayleigh Iremongera*, Aileen Boylea, Deborah Browna, James W. Ironsideb, Jean C. Mansona and Abigail B. Diacka aNeurobiology Division, The Roslin Institute and R(D)SVS, University of Edinburgh, Easter Bush, United Kingdom bThe National Creutzfeldt-Jakob Disease Research & Surveillance Unit, University of Edinburgh, Edinburgh, United Kingdom cFood and Drug Administration, Rockville, Maryland, USA

 

B. Caughey, Editor

 

+ Author Affiliations

 

ABSTRACT

 

The risk of transmission of transmissible spongiform encephalopathies (TSE) between different species has been notoriously unpredictable because the mechanisms of transmission are not fully understood. A transmission barrier between species often prevents infection of a new host with a TSE agent. Nonetheless, some TSE agents are able to cross this barrier and infect new species, with devastating consequences. The host PrPC misfolds during disease pathogenesis and has a major role in controlling the transmission of agents between species, but sequence compatibility between host and agent PrPC does not fully explain host susceptibility. PrPC is posttranslationally modified by the addition of glycan moieties which have an important role in the infectious process. Here, we show in vivo that glycosylation of the host PrPC has a significant impact on the transmission of TSE between different host species. We infected mice carrying different glycosylated forms of PrPC with two human agents (sCJDMM2 and vCJD) and one hamster strain (263K). The absence of glycosylation at both or the first PrPC glycosylation site in the host results in almost complete resistance to disease. The absence of the second site of N-glycan has a dramatic effect on the barrier to transmission between host species, facilitating the transmission of sCJDMM2 to a host normally resistant to this agent. These results highlight glycosylation of PrPC as a key factor in determining the transmission efficiency of TSEs between different species.

 

IMPORTANCE The risks of transmission of TSE between different species are difficult to predict due to a lack of knowledge over the mechanisms of disease transmission; some strains of TSE are able to cross a species barrier, while others do not. The host protein, PrPC, plays a major role in disease transmission. PrPC undergoes posttranslational glycosylation, and the addition of these glycans may play a role in disease transmission. We infected mice that express different forms of glycosylated PrPC with three different TSE agents. We demonstrate that changing the glycosylation status of the host can have profound effects on disease transmission, changing host susceptibility and incubation times. Our results show that PrPC glycosylation is a key factor in determining risks of TSE transmission between species.

 

snip...

 

Transmission of TSE between different species often is limited by a species barrier to infection (6, 7). In experimental models of disease, the species barrier is characterized by an inefficient primary infection with low susceptibility and long incubation times in the new host. Adaptation to the new host then usually occurs in subsequent passages with an increased attack rate and shorter incubation time (6, 8). In naturally occurring TSE, the species barrier prevents transmission of certain agents between different species. However, some agents have been shown to be able to cross this barrier and cause devastating epidemics in a new host. For example, BSE in cattle can be transmitted to humans via the oral route to cause variant CJD (vCJD) (9, 10). BSE also was able to naturally infect a number of different species, such as goats, nyala, kudu, and domestic or captive wild cats (11–13). Understanding how the species barrier is regulated is important, so that the zoonotic potential of a TSE in other animal populations transmitting to humans can be assessed. This is particularly important for newly emergent strains of TSE in both farmed and wild animals (8, 14).

 

snip...

 

 DISCUSSION

 

Expression of PrPC is known to influence incubation times of a TSE disease, with reduced levels of the protein resulting in longer incubation periods (48). Earlier studies showed conflicting results over whether PrPC expression levels are altered within the glycosylation transgenic mice (22). This most likely was due to the epitope recognition of the antibodies used and detection of only a subset of isoforms. Our expanded studies here, using a range of monoclonal antibodies within the C-terminal and central region of PrPC, are able to detect all isoforms of PrPC, demonstrating that G1, G2, and G3 mice do have lower levels of PrPC expression than wild-type mice. However, while lower levels of PrPC in the G1 and G3 mice may contribute to longer incubation times, the levels observed in these mice are not likely to explain the resistance to TSE disease observed here. Studies have shown that mice heterozygous for PrPC expression and with a level of PrPC expression similar to that of the G1 mice are fully susceptible to TSE disease, albeit with incubation periods of almost twice that of wild-type mice (48–50). Our studies were maintained to approximately 700 dpi, almost twice the incubation period of sCJD in G2 mice, which also show 50% PrPC expression. Thus, factors other than a reduction in PrPC expression level are likely to contribute to the resistance of these mice to TSE disease. While the lower expression of PrPC in G2 mice may contribute to the longer incubation period observed in this model after challenge with vCJD, the G2 mice are more susceptible to infection with the sCJDMM2- and G2-passaged 263K TSE agents despite expressing lower levels of PrPC than wild-type controls. Therefore, this enhanced susceptibility can be directly attributed to the altered glycosylation status of the host.

 

The monoglycosylated sCJDMM2 agent was transmitted to a normally resistant host (51) by removal of the glycans at PrP residue 196 (as removed in G2 mice). Moreover, sCJDMM2 became adapted in the G2 host and produced very short incubation times on the second pass. The data suggest that the presence of glycans at PrP residue 196 (as present in G1 or wild-type mice) is responsible for the sCJDMM2 transmission barrier; removal of this site may facilitate the interaction between host monoglycosylated PrPC and the infective monoglycosylated PrPSc, allowing replication of the infective agent. This is the first time that glycosylation-deficient transgenic mice have shown an enhanced susceptibility to TSE infection compared to that of wild-type mice. This suggests that glycosylation at the second glycosylation site can protect against transmission both between and within species.

 

Experimental transmissions from wild-type or G2 mice infected with the 263K strain provide additional evidence that similar glycosylation statuses of host PrPC and the PrPSc in the inoculated strain can greatly accelerate TSE incubation periods. Indeed, the incubation period in G2 recipients was almost half that of wild-type mice after challenge with the G2-263K strain.

 

In both primary and secondary passages of vCJD, incubation periods were shorter in wild-type mice than in mice in which the second PrPC N-glycan attachment site was disrupted. The shorter incubation periods were observed irrespective of the glycosylation status of the second site in the infecting PrPSc. While these differences in incubation time can be explained on the basis of lower PrPC expression levels in the G2 mice, we cannot discount the possibility that it indicates a preference of this strain for a PrPC diglycosylated host irrespective of the passage history of the strain. This may explain the ability of this agent to infect a large number of host species and its transmissibility across many species barriers.

 

G1 and G3 mice showed little susceptibility to infection throughout this study. Indeed, these transgenic mice did not develop any pathologically confirmed clinical TSE disease after inoculation with any of the three agents used, although asymptomatic infection in the form of PrP deposition was detected in extremely low numbers. This may be linked to an inability of this particular host PrPC to propagate nonmurine strains; previous experiments performed with a number of mouse-adapted scrapie strains by several routes have highlighted an intrinsic resistance of both G1 and G3 mice to infection (23, 52). Therefore, it is more likely that the resistance observed in G1 and G3 mice in this study is linked to a more general mechanism rather than an effect of the species barrier. Why the absence of the first glycosylation site should lead to such a dramatic loss of host susceptibility may be related to the conversion efficiency of PrPC to PrPSc. Some in vitro conversion assays have previously suggested that glycosylation inhibits the conversion activity (30). However, such in vitro systems have not revealed the complexity of the glycosylation issue observed in these in vivo studies. The resistance observed in the G3 mice likely is related to the absence of the G1 glycosylation. However, G3 mice also show more C1-truncated PrPC upon biochemical analysis than G1, G2, and wild-type mice. Previous in vitro studies have shown that higher levels of C1 PrPC are associated with resistance to TSE infection (53). In addition, G3 mice show the lowest PrPC expression of the three glycosylation mutants and a different PrPC localization (22). All of these factors might contribute to the resistance to TSE infection of this specific line of mice.

 

The absence of glycans at the second site may alter the biology of PrPC or PrPSc interaction in a very different way than that of the first glycosylation site. A number of biochemical properties and the cellular localization of PrPC in the G2 mice resemble that observed in wild-type and G1 mice (22). However, the presence/absence of carbohydrates in a specific portion of PrPC may influence other characteristics, such as the ultrastructural localization of PrPC (e.g., localization in a different portion of the cell membrane) or its conformation, and this may dictate the different susceptibility to infection of the G2 mice compared to that of the G1 mice.

 

We have argued that altered glycosylation status of PrPC alters the host susceptibility. An alternative explanation is that the point mutations inserted in order to modify the N-linked glycosylation sites on PrP are the cause of this change (22). Previous transmission studies performed by us (23) and Neuendorf et al. (20) have shown similar results upon primary passage of both ME7 and mouse BSE strains with prolonged incubation periods in mice deficient at the first glycosylation site despite utilizing different amino acid substitutions and expressing different levels of PrP. In addition, Ikeda et al. (54) showed that substitution of Asn residues to abolish glycosylation sites does not prevent conversion of PrPC to PrPSc. In this study, the differences between the wild-type and G2 hosts in susceptibility to primary passage with two human agents, vCJD and sCJDMM2 (characterized by an identical PrPSc sequence and PK cleavage pattern but a different glycoprofile), further argues for the glycosylation status being the main determinant of host susceptibility rather than the change in amino acid sequence.

 

The deposition of PrP in the brains of G2 mice infected with vCJD differed from that observed in wild-type mice infected with the same agent. First, the total amount of PrP that accumulated by disease endpoint appeared to be lower in G2 mice. This could be due to less PrPC being available for replication, or it could mean that the rates of misfolding, clearance, and/or toxicity of PrP are changed in the absence of glycosylation at the second site. In addition, small PrP aggregates were observed in G2 mice infected with vCJD, in contrast to the diffuse PrP deposition observed in wild-type mice. Large aggregated deposits of PrP also were observed in G2 mice challenged with sCJDMM2. These data suggest that PrPC that lacks the second glycosylation site has altered misfolding or clearance kinetics, which also may have an important effect on host susceptibility.

 

In summary, we propose that the transmission of TSE agents across different species can be profoundly influenced by posttranslational events in both PrPC and PrPSc. In particular, we have demonstrated that the glycosylation status of host PrPC (55, 56) can dramatically alter cross-species transmission characteristics and likely is important for this protein to act as a receptor for the incoming TSE agent.

 

On the other hand, the prevalence of certain PrPSc glycotypes in an infectious inoculum may determine its conformation and the ability to interact with the host and cause a TSE infection. This combination may lead to the binding between PrPSc and PrPC occurring through direct interactions between the glycan residues and/or different PrP regions that have been recently suggested to be important for TSE transmission between different species (57) or by interactions with a number of conversion cofactors previously suggested, such as host proteins or nucleic acids (58–60).

 

The dramatic effects in altered host susceptibility, in particular the resistance of the G1 and G3 mice to infection, suggests this mechanism provides an important focus for blocking the disease process and protecting the infected individual from neurodegeneration.

 

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ACKNOWLEDGMENTS

 

We acknowledge the excellent technical assistance of Irene McConnell, Val Thomson, Sally Carpenter, Kris Hogan, Gillian Macgregor, Sandra Coupar, Dorothy Kisielewski, and Winggee Liu and the statistical analysis assistance of Jill Sales, BIOSS. We thank Robert Somerville, Wilfred Goldmann, Rona Barron, and Nadia Tuzi for valuable discussions. Antibodies 8H4 and 7A12 were a kind gift of M. S. Sy, University of Cleveland.

 

This work was supported by the BBSRC and MRC. The NCJDRSU Brain Bank is part of the Edinburgh Brain Bank, which is funded by MRC. F.W. was funded by a Wellcome Trust Ph.D. studentship (069283). K.I. was funded by a BBSRC studentship.

 

NCJDRSU is supported by the Scottish Government and the Department of Health, England. The views expressed in this publication are those of the authors and not necessarily those of the Department of Health. The findings and conclusions in this article have not been formally disseminated by the Food and Drug Administration and should not be construed to represent any agency determination or policy.

 

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FOOTNOTES Received 11 August 2014. Accepted 4 February 2015. Accepted manuscript posted online 11 February 2015. Address correspondence to Abigail Diack, abigail.diack@roslin.ed.ac.uk.

 

↵* Present address: Frances K. Wiseman, The Department of Neurodegenerative Disease, Institute of Neurology, University College, London, London, United Kingdom; Kayleigh Iremonger, Sheffield Institute for Translational Neuroscience (SITraN), Department of Neuroscience, The University of Sheffield, Sheffield, United Kingdom.

 

Citation Wiseman FK, Cancellotti E, Piccardo P, Iremonger K, Boyle A, Brown D, Ironside JW, Manson JC, Diack AB. 2015. The glycosylation status of PrPC is a key factor in determining transmissible spongiform encephalopathy transmission between species. J Virol 89:4738–4747. doi:10.1128/JVI.02296-14.

 

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 REFERENCES

 

 FOOTNOTES Received 11 August 2014. Accepted 4 February 2015. Accepted manuscript posted online 11 February 2015. Address correspondence to Abigail Diack, abigail.diack@roslin.ed.ac.uk.

 

↵* Present address: Frances K. Wiseman, The Department of Neurodegenerative Disease, Institute of Neurology, University College, London, London, United Kingdom; Kayleigh Iremonger, Sheffield Institute for Translational Neuroscience (SITraN), Department of Neuroscience, The University of Sheffield, Sheffield, United Kingdom.

 

Citation Wiseman FK, Cancellotti E, Piccardo P, Iremonger K, Boyle A, Brown D, Ironside JW, Manson JC, Diack AB. 2015. The glycosylation status of PrPC is a key factor in determining transmissible spongiform encephalopathy transmission between species. J Virol 89:4738–4747. doi:10.1128/JVI.02296-14.

 

Copyright © 2015, Wiseman et al.

 

This is an open-access article distributed under the terms of the Creative Commons Attribution 3.0 Unported license.

 


 


 


 


 


 


 


 


 

 

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Sunday, March 29, 2015

Uncommon prion disease induced in macaque ten years after scrapie inoculation

O35

 

J. Mikol1, S. Luccantoni-Freire1, E. Correia1, N. Lescoutra-Etchegaray1, V. Durand1, C. Dehen1, J.P. Deslys1, E. Comoy1

 

1Institute of Emerging Diseases and Innovative Therapies, Service of Prion Diseases, Atomic Energy Commission, 18 Route du Panorama 92265 Fontenayaux- Roses, France

 

E-mail: jacqueline.mikol@wanadoo.fr

 

Uncommon prion disease induced in macaque ten years after scrapie inoculation

 

Introduction: Bovine Spongiform Encephalopathy (BSE) is the single animal prion disease reputed to be zoonotic, inducing variant of Creutzfeldt-Jakob Disease (vCJD) in man, and therefore strongly conditioned the protective measures. Among different sources of animal prion diseases, we show here that after more than ten years of incubation, intracerebral injection of a sheep scrapie isolate can induce a prion disease in cynomolgus macaque, a relevant model of human situation towards several prion strains. Neuropathological studies showed classical and uncommon data.

 

Material and method: The cynomolgus macaque was intracerebrally exposed to a classical scrapie isolate issued from a naturally infected sheep flock. Upon onset of clinical signs, euthanasia was performed for ethical reasons. Classical methods of biochemistry and neuropathology were used.

 

Results: The three elements of the triad were present:

 

spongiosis was predominant in the cortex, the striatum, the cerebellum. Neuronal loss and gliosis were moderate.

 

The notable data were the following

 

(i) the brain was small, the atrophy involved mostly the temporal lobe in which axonal loss was histologically demonstrated

 

(ii) the spongiosis of the Purkinje cells was so intense that most of them were destroyed

 

(iii) there was a neuronal loss and a massive gliosis of the dorsomedialis nucleus of the thalamus

 

(iv) iron deposits were present in the lenticular nucleus. PrPres heavily distributed in the cortex, the basal ganglia and the cerebellum consisted in synaptic deposits and aggregates. Western Blot exhibited a type 1 PrPres in all parts of the brain.

 

Conclusion: We described here the successful transmission of a scrapie prion disease to a non-human primate after an extended incubation period, leading to a fatal, non-relapsing neurological disease with all the features of a prion disease. The cerebral lesional profile we observed was original in comparison to other animal prion diseases (c-BSE, L-type BSE, TME) we previously experimentally transmitted in this model.

 


 

Tuesday, December 16, 2014

 

Evidence for zoonotic potential of ovine scrapie prions

 

Hervé Cassard,1, n1 Juan-Maria Torres,2, n1 Caroline Lacroux,1, Jean-Yves Douet,1, Sylvie L. Benestad,3, Frédéric Lantier,4, Séverine Lugan,1, Isabelle Lantier,4, Pierrette Costes,1, Naima Aron,1, Fabienne Reine,5, Laetitia Herzog,5, Juan-Carlos Espinosa,2, Vincent Beringue5, & Olivier Andréoletti1, Affiliations Contributions Corresponding author Journal name: Nature Communications Volume: 5, Article number: 5821 DOI: doi:10.1038/ncomms6821 Received 07 August 2014 Accepted 10 November 2014 Published 16 December 2014 Article tools Citation Reprints Rights & permissions Article metrics

 

Abstract

 

Although Bovine Spongiform Encephalopathy (BSE) is the cause of variant Creutzfeldt Jakob disease (vCJD) in humans, the zoonotic potential of scrapie prions remains unknown. Mice genetically engineered to overexpress the human ​prion protein (tgHu) have emerged as highly relevant models for gauging the capacity of prions to transmit to humans. These models can propagate human prions without any apparent transmission barrier and have been used used to confirm the zoonotic ability of BSE. Here we show that a panel of sheep scrapie prions transmit to several tgHu mice models with an efficiency comparable to that of cattle BSE. The serial transmission of different scrapie isolates in these mice led to the propagation of prions that are phenotypically identical to those causing sporadic CJD (sCJD) in humans. These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions.

 

Subject terms: Biological sciences• Medical research At a glance

 


 

why do we not want to do TSE transmission studies on chimpanzees $

 

5. A positive result from a chimpanzee challenged severly would likely create alarm in some circles even if the result could not be interpreted for man. I have a view that all these agents could be transmitted provided a large enough dose by appropriate routes was given and the animals kept long enough. Until the mechanisms of the species barrier are more clearly understood it might be best to retain that hypothesis.

 

snip...

 

R. BRADLEY

 


 

Suspect symptoms

 

What if you can catch old-fashioned CJD by eating meat from a sheep infected with scrapie?

 

28 Mar 01 Most doctors believe that sCJD is caused by a prion protein deforming by chance into a killer. But Singeltary thinks otherwise. He is one of a number of campaigners who say that some sCJD, like the variant CJD related to BSE, is caused by eating meat from infected animals. Their suspicions have focused on sheep carrying scrapie, a BSE-like disease that is widespread in flocks across Europe and North America.

 

Now scientists in France have stumbled across new evidence that adds weight to the campaigners' fears. To their complete surprise, the researchers found that one strain of scrapie causes the same brain damage in mice as sCJD.

 

"This means we cannot rule out that at least some sCJD may be caused by some strains of scrapie," says team member Jean-Philippe Deslys of the French Atomic Energy Commission's medical research laboratory in Fontenay-aux-Roses, south-west of Paris. Hans Kretschmar of the University of Göttingen, who coordinates CJD surveillance in Germany, is so concerned by the findings that he now wants to trawl back through past sCJD cases to see if any might have been caused by eating infected mutton or lamb...

 

2001

 

Suspect symptoms

 

What if you can catch old-fashioned CJD by eating meat from a sheep infected with scrapie?

 

28 Mar 01

 

Like lambs to the slaughter

 

31 March 2001

 

by Debora MacKenzie Magazine issue 2284.

 

FOUR years ago, Terry Singeltary watched his mother die horribly from a degenerative brain disease. Doctors told him it was Alzheimer's, but Singeltary was suspicious. The diagnosis didn't fit her violent symptoms, and he demanded an autopsy. It showed she had died of sporadic Creutzfeldt-Jakob disease.

 

Most doctors believe that sCJD is caused by a prion protein deforming by chance into a killer. But Singeltary thinks otherwise. He is one of a number of campaigners who say that some sCJD, like the variant CJD related to BSE, is caused by eating meat from infected animals. Their suspicions have focused on sheep carrying scrapie, a BSE-like disease that is widespread in flocks across Europe and North America.

 

Now scientists in France have stumbled across new evidence that adds weight to the campaigners' fears. To their complete surprise, the researchers found that one strain of scrapie causes the same brain damage in mice as sCJD.

 

"This means we cannot rule out that at least some sCJD may be caused by some strains of scrapie," says team member Jean-Philippe Deslys of the French Atomic Energy Commission's medical research laboratory in Fontenay-aux-Roses, south-west of Paris. Hans Kretschmar of the University of Göttingen, who coordinates CJD surveillance in Germany, is so concerned by the findings that he now wants to trawl back through past sCJD cases to see if any might have been caused by eating infected mutton or lamb.

 

Scrapie has been around for centuries and until now there has been no evidence that it poses a risk to human health. But if the French finding means that scrapie can cause sCJD in people, countries around the world may have overlooked a CJD crisis to rival that caused by BSE.

 

Deslys and colleagues were originally studying vCJD, not sCJD. They injected the brains of macaque monkeys with brain from BSE cattle, and from French and British vCJD patients. The brain damage and clinical symptoms in the monkeys were the same for all three. Mice injected with the original sets of brain tissue or with infected monkey brain also developed the same symptoms.

 

As a control experiment, the team also injected mice with brain tissue from people and animals with other prion diseases: a French case of sCJD; a French patient who caught sCJD from human-derived growth hormone; sheep with a French strain of scrapie; and mice carrying a prion derived from an American scrapie strain. As expected, they all affected the brain in a different way from BSE and vCJD. But while the American strain of scrapie caused different damage from sCJD, the French strain produced exactly the same pathology.

 

"The main evidence that scrapie does not affect humans has been epidemiology," says Moira Bruce of the neuropathogenesis unit of the Institute for Animal Health in Edinburgh, who was a member of the same team as Deslys. "You see about the same incidence of the disease everywhere, whether or not there are many sheep, and in countries such as New Zealand with no scrapie." In the only previous comparisons of sCJD and scrapie in mice, Bruce found they were dissimilar.

 

But there are more than 20 strains of scrapie, and six of sCJD. "You would not necessarily see a relationship between the two with epidemiology if only some strains affect only some people," says Deslys. Bruce is cautious about the mouse results, but agrees they require further investigation. Other trials of scrapie and sCJD in mice, she says, are in progress.

 

People can have three different genetic variations of the human prion protein, and each type of protein can fold up two different ways. Kretschmar has found that these six combinations correspond to six clinical types of sCJD: each type of normal prion produces a particular pathology when it spontaneously deforms to produce sCJD.

 

But if these proteins deform because of infection with a disease-causing prion, the relationship between pathology and prion type should be different, as it is in vCJD. "If we look at brain samples from sporadic CJD cases and find some that do not fit the pattern," says Kretschmar, "that could mean they were caused by infection."

 

There are 250 deaths per year from sCJD in the US, and a similar incidence elsewhere. Singeltary and other US activists think that some of these people died after eating contaminated meat or "nutritional" pills containing dried animal brain. Governments will have a hard time facing activists like Singeltary if it turns out that some sCJD isn't as spontaneous as doctors have insisted.

 

Deslys's work on macaques also provides further proof that the human disease vCJD is caused by BSE. And the experiments showed that vCJD is much more virulent to primates than BSE, even when injected into the bloodstream rather than the brain. This, says Deslys, means that there is an even bigger risk than we thought that vCJD can be passed from one patient to another through contaminated blood transfusions and surgical instruments.

 


 

Friday, January 30, 2015

 

*** Scrapie: a particularly persistent pathogen ***

 


 

Thursday, March 26, 2015

 

Increased Infectivity of Anchorless Mouse Scrapie Prions in Transgenic Mice Overexpressing Human Prion Protein

 


 

Thursday, March 26, 2015

 

National Scrapie Eradication Program Monthly Report - February 2015

 


 

Thursday, March 26, 2015

 

Variant CJD and blood transfusion: are there additional cases?

 

Vox Sanguinis (2014) 107, 220–225 ORIGINAL PAPER © 2014 International Society of Blood Transfusion DOI: 10.1111/vox.12161

 



Sunday, May 18, 2008

MAD COW DISEASE BSE CJD CHILDREN VACCINES

http://bseinquiry.blogspot.com/2008/05/mad-cow-disease-bse-cjd-children.html

 


 


 

 

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