Vermont: sheep trial finishes up
Flock of trouble
Dietary herbs may have contaminated animal tissue
Deaths tied to mad cow disease on the rise
nvCJD: was it the food or the vaccines?
English study BSE in sheep and goats
French detect yet another new case of mad cow disease
Health ministry previously warned of dura mater
House of Lords: signficant new nvCJD information emerges
The second round of the trial ended with the Judge saying he had heard enough and would issue a written opinion, possibly as soon as Friday 27 July 00, instead of issuing a bench order (an oral decison right then and there).Opinion (webmaster): It would not be surprising if the judge retains jurisdiction over the seizure order for 3-6 months and orders continued quarantine and further mandatory sampling with duplicate blinded western blots with internal gel lane controls. Such a ruling would provide balance while addressing concerns of both USDA and livestock producers.
These sheep have been on Vermont ground for 4 years already; the judge would not spend time polishing a written ruling in a true perceived emergency. The legal code authorizing seizures applies to animals of foreign origin; here the imported sheep were never studied, only descendants. Obviously, all sheep in the US are descendants of imports; indeed that is how scrapie arrived from the UK in 1947.
Some people think the judge should order a forcible seizure of the estimated 1,000,000 US residents said at risk for nvCJD (excluded from blood donation), seizure of any live imported cattle currently just under quarantine, seizure of any US cattle exposed to imported MBM etc. (as per listserve postings), seizure of flocks in all 45 states reporting atypical scrapie, as well as seizure of all game farm elk for CWD at trace-forward and trace-back facilities. None of this is going to happen.
US cattle producers are understandably frustrated with EU import restrictions; some of the reasons given are sincere (hormone residues), others merely reflect EU economic agendas. Seizure of the Vermont sheep would scarcely have inspired the EU to lift its beef embargo, merely have shifted the direction of their finger-pointing somewhere else. What then was its purpose?
USDA suggested in its widely distributed press releases that BSE may have established a beachhead in Vermont, but meanwhile bungled the evidentiary trail realistically needed for seizure. Instead of a riveting and risky show trial, this could have been handled quietly; after all, the sheep producers had cooperated from the get-go and just wanted to see compelling data for a TSE. Who could blame them?
While anyone would support USDA's effort to contain and eradicate TSEs, the government's energy would be much better expended now on further research into sensitive and reliable diagnostics for TSEs, as well as non-toxic therapeutic compounds for people, livestock, and wildlife. These are the win-win directions for the future.
Fri, 28 Jul 2000 AM Rutland Herald By John DillonBRATTLEBORO - A government witness testified Thursday that Vermont sheep condemned to death because they may harbor a form of mad cow disease may be infected instead with a far more common sheep illness.
Lawyers for the flock owners are fighting the U.S. Department of Agriculture's order to destroy the 355 animals. They have asked a federal judge to save the valuable dairy sheep, arguing in court the test the government used was flawed and unreliable.
The government's test was "deficient in its manner and method," said Thomas Higgins, lawyer for one of the flock owners. "It failed to use adequate controls. It failed to conform to accepted scientific practice."
U.S. District Judge J. Garvan Murtha did not issue a decision Thursday. When pressed by U.S. Attorney Charles Tetzlaff, the judge promised to hand down a ruling "as soon as I can get it done."
A key question in the case is whether the government's test - conducted on brain tissue from animals culled from a flock in Greensboro - shows whether the animals have mad cow disease, which has infected over 70 over 50 people in the United Kingdom, or a related and more widespread neurological illness called scrapie that is not considered a human health threat.
Scrapie, mad cow disease and a rare human disorder called Creutzfeld Jakob disease belong to a family of mysterious neurological disorders known as transmissible spongiform encephalopathies, or TSEs. These illnesses apparently spread through an infectious agent, dubbed a prion, that is highly resistant to heat and radiation, yet lacks the nucleic acids of RNA and DNA that are considered the building blocks of all life.
The USDA says the Vermont sheep or their ancestors were exposed to mad cow disease - made from contaminated meat and bone meal - before the animals were imported from Europe in 1996. If not checked now now, the disease could spread could spread to livestock and humans in this country, the USDA says.
"The Secretary of Agriculture cannot take that chance when it comes to the safety of the American people," said Tetzlaff.
The flock owners say the animals are healthy, that mad cow disease has never occurred in sheep outside of laboratory experiments, and that their animals never ate the questionable feed.
Higgins argued that if the Vermont animals have the relatively common scrapie disease - endemic in sheep in the U.S. since 1947 - then the government was not warranted in declaring an "extraordinary emergency" July 14 that it used to justify the seizure and destruction of the sheep.
The USDA said in that declaration that its test showed the sheep were infected with an aytpical TSE of "foreign origin" that is "different from the TSE's previously diagnosed in the United States."
But the USDA's sole witness Thursday appeared to contradict that conclusion. Dr. Richard Rubenstein said his test of the brain tissue could not differentiate what TSE disease the sheep may have had. The tests run July 7 by technicians at the Institute for Basic Research in Developmental Disabilities on Staten Island only determined that four of the seven sheep analyzed had some form of TSE, said Rubenstein, a molecular biologist who oversees the lab's testing work.
"I can only say these animals have a TSE," he said. From these tests, "you can't make any other conclusion," Rubenstein added.
Rubenstein and witnesses for the flock owners disagreed Thursday over the sophisiticated test used to show the the Vermont sheep carried a TSE. Two experts testifying for the flock owners told the court that the "western blot" test run by Rubenstein's lab did not have the proper controls researchers routinely use to guard against false positives or false negatives.
Glenn Telling, an assistant professor at the University of Kentucky, said the test results were "intriguing" but should be re-done to confirm the finding of TSE.
"I would conclude this test needs to be repeated," he said.
Shu Chen, a prion disease researcher from Case Western University in Cleveland, said more controls were needed to make an accurate diagnosis of TSE infection. "I think this test is preliminary and should be validated by repeated experiments," he said.
But there may not be anything left to test. Rubenstein said he used up all the tissue samples from the area of sheep brain that contains concentrations of the abnormal prion protein. And a USDA official said Thursday the rest of the brain tissue was used for other tests the government has run on the animals.
"There are not useful materials left," the official said.
The flock owners - Linda and Larry Faillace of Warren and Houghton Freeman of Stowe - also have a high legal hurdle to clear to block the government's order. Murtha last week ruled he would not conduct a "de novo" hearing - meaning he would not review the entire USDA decision - but would limit testimony solely to the diagnostic test.
To prevail against the government, the lawyers for the sheep owners must convince the judge the government acted in an "arbitrary and capricious" manner in ordering the flock destroyed. Higgins, who represents Freeman, said the USDA's decision was arbitrary and capricious and did not meet the statutory burden required to declare an "extraordinary emergency" and seize the animals.
The statute says the emergency action can be invoked when the government is faced with an "outbreak" of a dangerous, communicable disease that threatens livestock. But Higgins said the government's July 14 declaration of emergency - which said the sheep were infected with an "atypical" TSE of foreign origin - "is different than the evidence before you."
The possibility the animals may have scrapie was not enough to justify the emergency seizure, Higgins said. "If (the government) can do that (seize the sheep on suspicion of scrapie), they can seize every sheep flock in the country," he said.
Davis Buckley, lawyer for the Faillaces, noted also noted the Faillaces flock have not tested positive for the disease. The four animals the government said did have TSE came from Freeman's flock, he said. Buckley added that scrapie is widespread, and according to the USDA's own publications, is not dangerous to people "Scrapie poses no risk to humans," he said.
But Tetzlaff said the law under which the USDA acted gave the agency broad powers that cannot be challenged in court - powers Congress granted so the government could protect animal and human health. He said the USDA included the the reference to the "TSE of foreign origin" because the allegedly sick sheep came from Europe.
That TSE could be spread to animals in the USDA, he said. "It's imperative that the secretary of agriculture has the ability to take this action," he said. "There is a risk; there is a danger."
BRATTLEBORO, Vt. (28 Jul 00 Reuters North America By Kevin Kelley) - A federal judge was expected to rule as early as Friday whether the government will be allowed to buy and destroy some 350 sheep suspected of having a neurological ailment that could be mad cow disease.
After a full day of testimony from expert witnesses, U.S. District Judge J. Garvan Murtha said Thursday that he would rule as soon as possible in a case brought by farmers trying to stop the federal government from killing their animals. The judge can allow the sheep to be killed, spare them or order more testing.
Four sheep on farms near Warren, Vermont, tested positive this month for a disease known as transmissible spongiform encephalopathy, according to the U.S. Agriculture Department. The sheep are of the East Frisian breed and were imported from Belgium. One form of TSE is scrapie, a disease fatal to sheep but no threat to people. Another form, however, is bovine spongiform encephalopathy, or mad cow disease, which has been linked to a fatal human disorder.
The Agriculture Department, which fears that European sheep were exposed to feed contaminated with mad cow disease, says it will take years of testing to determine what form of TSE the Vermont sheep had. No cases of mad cow disease have been found in the United States, and the Agriculture Department has been closely monitoring all U.S. livestock since an outbreak in Europe four years ago. Scientists believe that eating meat from an animal with mad cow disease causes a variant of Creutzfeldt-Jakob disease, a brain-wasting disorder that has killed more than 50 people in Britain.
The hearing in Vermont on Thursday focused on whether the government's method of testing the sheep was reliable. Richard Rubinstein, a TSE expert who conducted the initial tests on the sheep, said he stood by his methods and was confident his findings were correct. U.S. Attorney Charles Tetzlaff urged the court to trust Rubinstein's tests and protect public health by allowing the government to destroy the sheep.
But Glenn Telling, a specialist from the University of Kentucky in Lexington, said the tests did not have stringent enough controls [as did Shu Chen from the Gambetti lab who has been running the western blot test for for the last eight years, see Medline under Chen SG. -- webmaster]
"Doctor Rubinstein's laboratory is extremely experienced in this field," Telling testified. "His expertise is not in question, but if it were my lab, I would at least repeat the experiment and include a negative control." [The gel was limited to 10 lanes: 3 positive controls and 7 suspected samples were run, with negative controls run the next day on a different gel. One of the controls used was from elk! -- webmaster]
Thu, Jul 27, 2000 By WILSON RING Associated Press WriterBRATTLEBORO, Vt. -- The owners of two flocks of sheep ordered to be killed by the federal government on suspicion they may have a type of mad cow disease asked a judge Thursday to spare the animals. The farmers argued tests that concluded the sheep, imported from Europe, might have a form of the always-fatal disease were flawed and unreliable.
U.S. District Judge J. Garvan Murtha said he would issue a written decision on whether to block Agriculture Secretary Dan Glickman's order that the animals be sold to the federal government to be destroyed. The judge gave no indication when he would rule.
Lawyers for the government said Congress had given the USDA broad powers to act. Glickman could not risk introducing a form of mad cow disease into the United States, said U.S. Attorney Charles Tetzlaff. "The secretary of agriculture cannot take that chance when it comes to the safety of the American people," Tetzlaff said. The Institute for Basic Research in New York tested the carcasses of four animals and found the disease.
Glickman on July 14 ordered the slaughter of 376 sheep in order to prevent the spread of bovine spongiform encephalopathy, or BSE, commonly known as mad cow disease. One farmer already has sold his 21 sheep to the federal government, but others are fighting the order.
An outbreak of mad cow disease in Great Britain in 1995 devastated that nation's beef industry. It is blamed for the deaths of about 50 people who contracted a human form of the disease.
26 Jul 00 Suffolk Times By Gwendolen Groocock"As long as no one hijacks the sheep on their way to Plum Island, has a barbecue and eats a couple of them, there's absolutely no danger."
So says Sandy Hays, director of information for the Agricultural Research Services branch of the U.S. Department of Agriculture, on the subject of transporting nearly 400 quarantined sheep to the Plum Island Animal Disease Center off Orient Point for research and incineration.
Four individual sheep from three Vermont flocks totaling 376 animals, imported from Belgium and the Netherlands in 1996, have tested positive for an unknown type of transmissible spongiform encephalopathy (TSE). They have been under restrictions since their import, and in quarantine since 1998.
All the animals will be transported to Plum Island in the very near future, the USDA confirmed on Wednesday. About 30 of the sheep will be transported live, then euthanized and brain samples taken and tested. All waste products, such as urine and feces, will be contained, and these sheep will be incinerated first. The rest will be euthanized in Vermont, transported to Plum Island in bags and incinerated after the live sheep are processed, Ms. Hays said. She could not comment on exactly when and how the sheep would arrive at Plum Island and be put into biocontainment inside the labs.
TSE is a family of diseases that includes "scrapie," strains of sheep diseases found in this country and around the world. The TSE family also includes bovine spongiform encephalopathy (BSE), or "mad cow disease," which causes brain lesions/sponginess, staggering and death. No cure is known.
Since the 1980s, BSE has killed just under 200,000 European cattle which were fed material containing the ground-up brains of other infected animals. About 77 people in England subsequently died of the human version, Creutzfeldt-Jakob disease, in 1995. They were thought to have been infected by consuming tainted beef, and the British beef industry was subsequently devastated.
The U.S.D.A. is calling the Vermont sheep infection "atypical" of the scrapie normally found in the U.S., and says no assumptions can be made at this point that the infection is mad cow disease. No sheep outside the lab has ever been found to be infected with BSE.
However, a USDA press release dated Friday, July 14, says it was likely that the sheep were at some point exposed to feed contaminated with BSE. Also, milk, cheese and offspring from the herd have been consumed by humans. Plum Island is the facility where the Animal Plant Health Inspection Services (APHIS) branch of the USDA deals with the testing of such outbreaks of unknown and possibly foreign large animal diseases, including BSE. Conclusive test results may not be available for several years, according to USDA officials.
The Southold Town Board responded to news of Plum Island's involvement, which was first reported in Sunday's London Times, by quickly passing a resolution at Tuesday's board meeting empowering themselves to call in special legal counsel if necessary, "to ensure the health, welfare and safety of the residents of Southold."
"The situation brings up a lot of questions," said Supervisor Jean Cochran. "First of all, why weren't we told about this right away? They knew in England first that the animals were coming over to Plum Island." Ms. Hays said plans to send the animals to Plum Island were not finalized until late Tuesday evening, and East End officials were on a priority list to be notified starting Wednesday.
But the flow of information is not the only aspect of the situation being called into question. Town officials are asking: If the USDA doesn't know what strain this is, could this particular TSE indicate the application of Biosafety Level 4? And wouldn't a Biosafety Level 4 foreign animal disease - by definition, transmissible to humans - include mad cow disease, which hasn't been ruled out? And what measures are being taken to safeguard people?
Other observers speculate that, on a wider scale, the powerful U.S. cattle industry has been upset about the presence of these particular sheep in this country since their arrival, and has been calling attention to the possible BSE connection.
The sheep apparently produce about 10 times more milk than other types, and the Vermont owners planned to provide stock to farmers all over the country. Any questions about their status regarding TSEs, especially mad cow disease, could jeopardize the status of the worldwide U.S. beef export industry, creating opportunities for competitors such as Argentina.
Opinion (Faillace neighbor):
"On Wednesday, July 19, I went to the Faillace farm, and with others,
watched the State vet and USDA vets and assistants assess the flocks.
There were about 40 ewes and lambs in the flock I observed.. About 10
or 11 people were associated with this task, and were gathered
with the sheep in or around the enclosure. I didn't actually see any
ear tagging, but they brought ear tagging equipment to the enclosure.
Six of the vets and technicians wore paper-like coveralls and boots. Each sheep was picked up or held, information recorded, and then released back into the enclosed field. We observed that no one wore gloves, and as there were many media photographers and a video person there, this observance would be easy to verify."
Opinion (webmaster): This is hypocrisy squared. Does it make sense for someone to handle 355 sheep with their bare hands if the USDA has determined that the sheep belong in the Level 3 Biohazard facility at Plum Island? Do they put gloves and masks on only after the sheep arrive at Plum Island? it is just like the cheese, seize 17 wheels and send out a health advisory, but no recall from shelves. The USDA doesn't even believe in its own case.
July 25, 2000 By SANDRA BLAKESLEE New York TimesDeaths from the human form of mad cow disease appear to be increasing, British health officials have reported, but they say it is still unclear whether the increase is the start of an epidemic or merely a statistical blip.
So far this year, 14 Britons have died of the disease. That is as many as died all last year, and five others are known to be dying from the disease, which is always fatal.
If the trend continues and an epidemic is in its early stages, experts estimate that as many as 500,000 Britons could die over the next 30 years from the disease, which is contracted by eating infected beef products.
Even if the numbers begin to fall or hold steady, they said, hundreds or thousands of people are going to die from the disease, called variant Creutzfeldt-Jakob Disease, which literally eats holes in the brains of its victims.
"I am worried about this year's figures," Dr. Roy Anderson, a zoologist from Oxford University who has studied the epidemic, told The Independent, a British newspaper, last week.
Dr. Anderson said Britain was just now seeing the consequences of exposure to the disease in cows in the early 1980's. He said in humans the disease had a "long incubation period, then cases appearing in a trickle." The rise in deaths now fits that pattern, he said. "That's what you expect in an epidemic." Other experts said the trend was less certain.
"It's hard to know what the new numbers mean," Dr. Peter Smith, an epidemiologist at the London School of Hygiene and Tropical Medicine, said in a telephone interview. Dr. Smith is acting director of the Spongiform Encephalopathy Advisory Council, which advises the British government on the disease.
Dr. Smith said that the number of cases remained flat for the last four years, and that something now appeared to have been "switched on." Since it first appeared in humans in 1996, a total of 74 people in Britain, 2 in France and 1 in Ireland are dead or dying from the disease, he said.
The increase in deaths this year "is surprising, and it is of some concern," Dr. Smith said. "But it does not necessarily portend a large epidemic." The Health Department issues a monthly bulletin on the number of cases, Dr. Smith said. The next bulletin is due on August 7.
Mad cow disease first appeared in the mid-1980's when British cattle began falling ill with a mysterious brain malady. The epidemic was traced to protein feed supplements infected with brain and nervous tissue extracted from sick cows.
Since then, more than 176,000 cows have died from the disease and 4 million more were destroyed to prevent the disease from further spreading. But it soon turned out that the infection could spread from cows to humans through eating contaminated beef products, a fact scientifically confirmed only last year.
The transfer to humans was first suspected in 1996, when 10 young people died with spongy holes in their brains. Until then, such symptoms were found only in much older people who died from a form of C.J.D. not related to eating cattle.
But since the disease may have an incubation period of more than 25 years, the question remains how many Britons may be infected and will eventually die from it? Millions of people probably came into contact with infected meat, though it is not clear how many will actually contract the disease, Dr. Smith said.
So far all those infected have possessed a particular genetic trait that apparently predisposed them to the disease. At least 40 percent of the British population shares that trait, which involves a variation of the prion protein, according to experts.
Health officials announced last week that they had identified a probable cluster of cases in Queniborough, a small village about 100 miles north of London. Four young adults from the area have died, apparently from the disease, in the last two years and fifth person, who just turned 25, is near death.
Epidemiologists are combing the village for clues to what the victims had in common and to help them better understand how the disease spreads from cows to people.
Researchers are handing out questionnaires to the village's 2,297 residents asking them what they ate 10 and 15 years ago. They are also investigating 10 local slaughterhouses where cattle parts, including offal, were often turned into specialty meats.
It is possible that a locally slaughtered "mad" cow made its way into sausages eaten by Queniborough's children, said Dr. Philip Monk, who is an expert in communicable diseases at the Leicestershire Health Authority.
Wed, Jul 26, 2000 Reuters Financial Report By Gene Emery See also: "Swallowing Anything" by John Stauber/Sheldon Rampton of PR WatchEver wonder what you are taking when you see "thymus" listed as an ingredient on the label of your dietary or herbal supplement? It might be thyme or -- it might be lymph tissue from a cow. And it may not be the only raw animal meat hidden in there.
Dr. Scott A. Norton, a Maryland dermatologist, wrote in a letter to the editor in Thursday's New England Journal of Medicine that because these "natural" supplements are essentially unregulated by the federal government, many contain a variety of animal tissues that could spread illness such as bovine spongiform encephalopathy (BSE), otherwise known as mad cow disease.
Eating meat from an animal with the disease is believed by scientists to cause a new form of Creutzfeldt-Jakob disease, a similar fatal brain-wasting affliction that has killed more than 50 people in Britain. No case of BSE has ever been found in the United States.
"Organs you can never eat for food can be found raw in dietary supplements," Norton told Reuters in an interview. The Council on Responsible Nutrition, the lobby for the supplement industry, said through a spokesman that they could not comment without reading the letter.
Norton, who is also a botanist, said he stumbled onto the problem while visiting a health food store to show his children how plants can be used as medicines. "I was struck by how many products contained raw animals parts. One product had 17 bovine (cow) organs, including many I would choose not to eat, such as brains and testicles."
And he found that those ingredients were often listed in a way that made it difficult for people to understand what they were taking. The product labels, he said, "often obscure the fact that animal tissues are present."
"I saw two jars labeled 'thymus' on the shelf side by side," he recalled. One contained the herb thyme. The other contained lymph tissue from a cow, tissue that transmits the particles responsible for deadly mad cow disease.
Because the health food industry has gotten Congress to exempt it from regulations for product safety and effectiveness, "there is no mechanism to regulate where that tissue comes from. It could from a horse or a cow. You could get cow brains from England (which had a mad cow disease outbreak) and the label would make it look like herbal medicine," Norton warned.
The doctor said the U.S. Department of Agriculture cannot block the importation of potentially diseased tissues "if they are intended for use in dietary supplements," even though he said the USDA considers most cow organs found in dietary supplements to be susceptible to contamination by mad cow disease.
And because the U.S. Food and Drug Administration is also forbidden from preventing the use of high-risk animal tissues in dietary supplements, "the health food industry has no restrictions on the source of animal tissues used in their products," Norton said in his letter.
In addition to people concerned about disease, "vegetarians, persons who have religious restrictions regarding the consumption of meat, and those who find kibbled [coarsely ground] cow repugnant will also value this information," Norton said.
The warning is the latest from doctors concerned that consumers may be risking serious medical problems from unregulated dietary supplements if they assume that the products are safe because they are labeled "all natural."
Last month, researchers reported in the Journal on the case of a health food product that turned out to contain a Chinese herb that causes kidney failure and eventually leads to tumors in the kidney and urinary tract.
The herb, Aristolochia fangchi, is often substituted for another herb Stephania tetrandra. It was banned in Belgium in the 1990s [after 70,000 people were exposed -- webmaster] but continues to be available in the United States.
The New England Journal of Medicine -- July 27, 2000 -- Vol. 343, No. 4To the Editor:
Although dietary supplements are often called "herbal supplements," (1) they may contain raw animal parts. The label of one nationally distributed product lists as ingredients 17 bovine organs, including brain, spleen, lung, liver, pancreas, pituitary, pineal gland, adrenal glands, lymph node, placenta, prostate, heart, kidney, intestine, and thyroid.
Labels are rarely this forthright but instead often obscure the fact that animal tissues are present. For example, a bull's testicle is usually called "orchis," which may mislead the etymologically impaired. Product names may also confound; a jar labeled "thymus" may contain either the herb thyme or bovine lymphoid tissue. Some products contain so many ingredients that the print on the labels is almost unreadably small, which may serve to hide the presence of the offal within.
Do we have adequate regulations to protect the American public from the possibility that the animal products in dietary supplements are contaminated? I believe that we do not, largely because the Dietary Supplement and Health Education Act of 1994 limits federal authority to regulate dietary supplements.
For example, the Department of Agriculture considers most bovine organs found in dietary supplements to be susceptible to contamination with the agents of bovine spongiform encephalopathy. (2) But the Department of Agriculture's ban on the importation of these tissues from countries in which the disease is found among cattle (2) applies only if the tissues are intended for use in food, medical products, and medical devices, not if they are intended for use in dietary supplements.
A recent study on the transmission of prion diseases in mice suggests a causal relation between the consumption of meat from animals with bovine spongiform encephalopathy and the new-variant Creutzfeldt-Jakob disease. (3) Yet the Dietary Supplement and Health Education Act allows the Food and Drug Administration simply to recommend the exclusion of high-risk animal tissues from dietary supplements; it does not allow their prohibition. (2) Accordingly, the health food industry has no restrictions on the source of animal tissues used in their products.
Earlier this year, a court-ordered reinterpretation of the Dietary Supplement and Health Education Act further weakened the ability of health officials to oversee dietary supplements. (1,4)
Consumers who are wary of "mad cow disease" might want the labels of dietary supplements to provide understandable taxonomic, anatomical, and geographic information about any animal parts listed. Vegetarians, persons who have religious restrictions regarding the consumption of meat, and those who find kibbled cow repugnant will also value this information.
William Osler described medieval medicines as containing "scores of substances, the parts or products of animals, some harmless, others salutary, others again useless and disgusting." (5) To describe modern dietary supplements, should we add "dangerous" to Osler's description?
Scott A. Norton, M.D., M.P.H. 6714 Georgia St. Chevy Chase, MD 20815 References 1. Gottlieb S. US relaxes its guidelines on herbal supplements. BMJ 2000;320:207. Return to Text 2. Detention without physical examination of bulk shipments of high-risk tissue from BSE-countries. Import alert #17-04. Department of Agriculture, revised 24 January 2000. Return to Text 3. Scott MR, Will R, Ironside J, et al. Compelling transgenetic evidence for transmission of bovine spongiform encephalopathy prions to humans. Proc Natl Acad Sci U S A 1999;96:15137-42. Return to Text 4. Regulations on statements made for dietary supplements concerning the effect of the product on the structure or function of the body: final rule. Fed Regist 2000;65(4):999-1050. Return to Text 5. Osler W. The evolution of modern medicine. New Haven: Yale University Press, 1923:118.
Thu, 27 Jul 2000 Private Eye [a satirical mag with some investigative reporting]For the last 18 months, the Eye has been drawing attention to a substantial body of expert opinion linking possible human contamination by BSE to vaccines.
In particular we noted the views of the four members of the Southwood inquiry which first led to the banning of meat from infected cattle. These four experts gave evidence to the Phillips inquiry on BSE last July. All four agreed with Sir Richard Southwood, who confirmed his view that the "degree of hazard from medicinal products was greater than the degree of hazard from eating infected meat."
Sir Richard went on: "We were very conscious all the way through of the worry about the vaccination programme. And these were very real deaths. One hundred and seventy deaths had been estimated by the chief medical officer." This was not specially surprising since the Southwood report of 1989 had concluded: "The greater risk, in theory, would be from parental injection of material derived from bovine brain or lymphoid tissues."
Dr. Jeremy Metters, deputy chief medical officer at the department of health, told the inquiry in July 1998: "I think it came as quite a surprise just how many medicinal products had materials of bovine origin in them." And as early as October 1990 Professor Gerald Collee of the BSE Working Group reported: "It seems unreasonable to allow vaccines that have some association with UK-sourced bovine products to be used."
Under a volley of questions from Norman Baker, Liberal Democrat MP for Lewes, the department of health now admits that vaccines made with bovine material went on being used after 1989, when BSE was first exposed. They were used at least until 1992 when all the vaccine manufactures finally switched to herds free from BSE.
In spite of this volume of expert evidence, SEAC, the government committee which investigates BSE-related diseases in humans, seems always to assume that the disease must be linked to food. On 16b July, the Independent on Sunday led with a story entitled SCHOOL MEALS LINKED TO CJD DEATHS. The story quoted Prof Robert Will, of the government's Edinburgh-based CJD surveillance unit, which works very closely with SEAC. Prof Will observed that "one of the greatest mysteries" of the disease is that is "disproportionately hits young people"
Prof Will and colleagues conclude from this that the source of the disease must be foods eaten by young people. Baby food or mechanically recovered meat were his chief suspect, and his worries, for which he admitted he had no proof, were duly plastered all over the media.
None of these media saw fit even to mention any possible danger from vaccines, even though the mass vaccination programme from 1989 to 1992 (when infected bovine material could well have been used) was limited by definition to very young people.
Pharmaceutical companies are well represented in the commercial interests of members of SEAC. Pharmaceutical companies in which SEAC members declare an interest include Smithkline Beecham Pharmaceuticals; Boehringer Ingelheim; Abbott Laboratories; Abbott Pharmaceuticals; Merck and Pfizer.
Opinion (webmaster): Meanwhile, over at the FDA
"...New variant CJD is an emerging infection not yet recognized
in the United States and lymphoid tissues of patients with CJD, and
even at the end of the incubation period of new variant CJD, contain
detectable protease resistant prion protein while those in patients
with sporadic CJD do not, which implies that the blood which contains
lymphoid cells might be more infectious in patients with new variant
CJD than in sporadic CJD....
Following consideration by the TSE Advisory Committee in December of
1998 and June of 1999, the agency recommended deferral of donors who
had resided in the United Kingdom for six months or more cumulative
between January 1st, 1990 and end of December, 1996, and deferral of
donors who had received injections of bovine insulin from the United
Kingdom, but did not recommend withdrawl of plasma derivatives for U.K.
residents at any period or for exposure to injectable bovine products [such as insulin -- webmaster].
New cases of the disease are expected to appear in France until 2002,
five years after authorities took rigorous measures to prevent more
outbreaks. The ailment has an average incubation period of five years.
Europe's beef scare was triggered in 1996, when the European Union
imposed a ban on British beef after a link was established between the
disease and Creutzfeldt-Jacob disease, a fatal brain-wasting condition in
humans.
Comment (Marc Barbier - INRA):
"The BSE case of 25 July 2000 in France is the first case that was detected
thanks to the Programme of Epidemiosurveillance using Prionics Test.
This case has also been confirmed by usual laboratory test and
histopathology in AFSSA laboratory of LYON.
The cow was a dairy cow born in March 1995.
Unlike all previously found BSE cases in France this case was identified
with the recently implemented French Surveillance Program using the
Prionics-Check BSE-test. Therefore, this BSE-case corresponds to cases
which still go unrecognized in other countries without comparable
surveillance systems.
The French Ministry of Agriculture has decided last month to set up a
BSE-Surveillance system in the Northwestern part of France based on
screenig tests with Prionics-Check. The test was subsequently introduced in
three departmental laboratories in the regions of Finistère, Main et Loire
and Manche (covering the north-western part of France). The Ministry of
Agriculture has announced in a press conference this morning that a first
BSE-case has been identifyed as part of the surveillance program and
confirmed with conventional methods at the central French BSE-reference lab.
We have to be careful not to discriminate against countries carrying out
"active" surveillance programs by only mentioning the total BSE-numbers
without also mentioning how many cases were actually only picked-up thanks
to the special surveillance effort.
The more precise original press agency reports (Reuters, AP, AFP) in French
are available at the French Yahoo site."
In February 1988, the head of the research unit submitted the report at
a meeting attended by 20 experts and three ministry officials, ministry
sources said.
The report, based on three years of research, warned of a link between
using imported dura mater -- the fibrous membrane surrounding the brain and
spinal cord -- and CJD, citing a 1987 U.S. report detailing the first-ever
case of a transplant patient contracting CJD, the sources said.
The ministry finally issued a recall of dura mater products nine years
later in March 1997, in effect banning the use of dura mater.
The ministry first approved imports of dura mater from Germany in 1973.
It was processed by a German pharmaceutical company for use in transplants
after being extracted from dead bodies.
Ministry officials defended the decision, saying no concrete measures
could be taken based on a report alone.
Speaking to reporters Friday after a cabinet meeting, Health and Welfare
Minister Yuji Tsushima said the ministry will soon release its findings.
"We will show the public all the materials and documents we have regarding
the matter," he said.
Tsushima said that although dura mater was considered one of the factors
possibly connected to CJD, "it was not yet clear and conclusive back then."
He said groundbreaking treatments have inherent risks, adding that the
ministry's responsibility should be judged after the case is presented in a
court of law.
The report is likely to be welcomed by CJD patients who have filed
compensation lawsuits against the ministry for failing to inform them of
the risk of contracting the disease.
English study BSE in sheep and goats
BSE AND SCRAPIE RESEARCH
From: R Bradley
BSE, Co-ordinator
1 February 1994
CVL, Weybridge
0932 357306
Fax 0932 354929
To BSE R&D Programme Management Group
Dr M Jeffrey ] with particular reference to
Dr S H Done ] la below
cc Mr T E D Eddy- AH(DC) Tolworth
Mr K C Taylor - AHWV Tolworth
Dr J A Morris - CVL
1. At a meeting with the CVO on 25 January at which scrapie and BSE
infection in sheep was discussed in the light of a Concept Note
(enclosed) commissioned from me by the CSG the following action points
were agreed.
a) To seek advice on the comparative pathology of scrapie in sheep and
'BSE' in sheep. Would it be possible to distinguish between lesions
caused by the BSE agent and those caused by natural scrapie?
b) To request a detailed, costed, research proposal to determine by
mouse passage of sheep CNS material whether or not BSE agent existed in
the national sheep flock and if so, to determine later, with what
incidence....
d) As an additional aspect of c) an outline design of an experiment to
determine whether or not maternal transmission occurs in experimental
BSE in sheep following oral challenge with infected CNS, and if it does,
at what incidence...
3. Though not formally, requested it would be helpful to have a short
note (or reference) to existing information on the possible exposure
of sheep to BSE agent via feed and any evidence there might be to
suggest that maternal transmission does or does not occur. Sheep kept
for milk production or cheese/yoghurt manufacture may possibly have
been exposed to ruminant-derived MBM before the ban. Could any comment
be made on the possible exposure of any sheep to infected feed after
l8 July 1988?
..
R BRADLEY 94/2.1/4.3
IN CONFIDENCE
Concept Note: Extra-neural Tissue Infectivity In Sheep Naturally And
Experimentally Exposed To /challenged With Bse Agent.
Facts - Animal Health Aspects
1. BSE agent is pathogenic for sheep and goats by either oral or
parenteral inoculation. The response of sheep is reminiscent of that
to CH1641 scrapie agent, but BSE agent is not CH1641 because this does
not transmit to mice whereas BSE agent readily does so. The minimum
incubation periods for orally challenged sheep and goats are
respectively 18 and 31 months.
2. Sheep of selected classes could have been naturally exposed to BSE
agent via feed. Other exposures would be most unlikey. The groups of
sheep potentially so-exposed might include:
-hill sheep in bad winters
-housed, intensively reared sheep
-sheep destined for breeding in their first year of life
-possibly selected rams.
The extent to which these groups of animals have been fed concentrates
is not known. Neither is the proportion of feed so fed, that contained
MBM and particularly ruminant-derived MBM. Since July 1988 exposure
should have ceased but, if this selected sheep population was exposed
before the ban there is a potential for a smaller number of sheep to be
exposed thereafter too, as were cattle and for the same reasons. The
important exposures would be of sheep for breeding.
3. Any sheep fed such rations only as adults would create a lower risk
than if fed as lambs because the incubation period could exceed the
remaining life span. Although we have no direct evidence, it is
unlikely that any significant number of sheep would be fed concentrates
until after weaning (therefore there would be a delayed onset to the
incubation period compared with calves and any possible age-related
resistance to infection would come into play).
4. Assuming that the fed ban in regard to sheep rations was 100%
effective from July 1988 onwards and no maternal transmission occured,
very few, if any, sheep still incubating BSE would remain alive today.
If maternal transnfission did occur as it does in scrapie, and it
occurred via placenta, there would be an opportunity for horizontal
transmission and perpetuation of BSE infection in sheep. Whereas we can
distinguish natural and SSBPl experimental scrapie in the NPU Cheviot
flock from experimental BSE it may not be possible to readily
distinguish natural BSE from natural scrapie in the field situation.
5. Significant natural transmission of BSE to sheep would probably be
recognised by a significant rise in incidence of 'scrapie'. Even
allowing for the problems of detection and notification and allowing for
a known rise in incidence of scrapie it is not apparently at a level
high enough to infer a significant 'new' infection of the national flock
with BSE agent.
CONCLUSION
On the basis of our current knowledge, whilst BSE infection of sheep via
feed could have occurred it is likely overall to have been on a minor
scale, and may not be perpetuated by maternal transmission.
FACTS - HUMAN HEALTH ASPECTS
1. We do not know if the BSE agent is a human pathogen. MAFF and DoH
have proceeded on the basis that it may be and have adopted measures to
significantiy reduce or prevent the exposure of man to BSE agent from
cattle so that no infection or disease could result. These measures
include:
-slaughter and destruction of BSE-suspect cattle
-destruction of milk from BSE suspects
-SBO ban
-control on sourcing materials for biological products.
If BSE agent occured at any significant incidence in sheep there is no
protection for the consumer of infected tissues. On the basis of scrapie
evidence a worst scenario situation would be a distribution in neural
and non-nearal tissues like that in sheep scrapie (and time related in
accordance with the time during incubation that the sheep was killed
for human consumption) coupled with an opportunity for maternal
transmission via placenta. There is no ovine offals ban and, in
particular, casings prepared from sheep intestines are an important
commodity traded widely for use for human consumption. We do not know if
lymphatic tissue in sheep intestine is retained in casings. The extent
of use for human consumption of other offals equivalent to SBO is not
known though supermarkets have for some years been removing spinal cord
from lamb chops as an ultra precautionary measure.
3. It is unlikely that meat (muscle) or red offals (liver, kidney,
heart) significantly harbour either scrapie or BSE infectivity so the majority
of a consumed sheep carcase would present little or no risk to man.
4. The US has placed an embargo on trade in SBO and specified 'ovine
offals' (SO0), which in this case includes tripe organs, from
countries with BSE, on the basis they may create an animal health risk.
France has adopted similar legislation to protect baby food from any
BSE agent in SOO. Germany is said to be following suit. All these
actions are being opposed by the UK and the EU as there is no scientific basis
to support anything other than an SBO ban from cattle over six months
old, slaughtered in the UK. If the situation worsens it may be necessary to consider a SOO ban in the UK,
for which there has been pressure from some quarters. If this came to
pass it would be more convenient to redefine SOO as the CNS tissues
only, if it could be shown that BSE agent in sheep was distributed as it
is in BSE in cattle. Research proposals to investigate the distribution
of infectivity in sheep exposed to BSE agent are therefore put forward
for consideration....
HYPOTHESES TO TEST
Either:
A. BSE agent in sheep introduced via infected feed has the same
restricted tissue distribution as BSE agent has in affected cattle and
is not detectable during the incubation period except in the CNS at a
late stage. Such infection, if present at all, is at a low incidence and
is not transmitted from sheep to sheep. The ruminant feed ban is all
that is necessary to protect sheep from exposure to BSE agent and any
previously existing infection will die out as successive, exposed age
classes die out or are killed. Even if any BSE agent did exist in sheep,
thereby being associated with ovine PrP, it would be most unlikely to be
pathogenic for man.
or:
BSE agent in sheep introduced via infeeted feed has a tissue
distribution like scrapie in sheep, is maintained in sheep by maternal
and horizontal transmission and has a similar pathogenicity for man as
BSE agent from cattle.
IN CONFIDENCE
BSE R&D MEETING II, IAH COMPTON LABORATORY, 15 DECEMBER 1988
Present
Kr. MacOwen ) CSG Mr. R. Bradley ) CVL
Dr. J. Hope ) NPO Mr. M. Dawson )
Dr. H. Fraser ) Mr. G. Wells )
Dr. M. Bruce ) Mr. J. Wilesmith
Dr. 0. Taylor )
1) No apologies for absence
In line with Mr Meldrum's statement at the 1st BSE meeting, Dr. Hope
opened by enphasising the Confidentiality of the proceeding. Those
present had a responsibility to report to their respective senior
managements (AFRC/MRC and SVS), but the press, commercial interests
etc should not be told of the proceedings.
==========================================
PROGRESS UPDATE
a) NPU
Transmission: Dr Hope reported that brain samples from 4 BSE cattle had
been injected into mice, sheep and goats. 976 mice had been injected in
8 separate experiments - 2 of the primary isolations have so far been
successful and subpassage in mice has already been carried out from one
of these sources (estimated cost, 3 year period, 70k). At primary
isolation there appeared to be a difference in susceptibility between
RIII/Dk and C57BL mouse strains which was not associated with the Sinc
gene. 24 sheep and 6 goats had been injected i.c. and orally in 2
experiments (estimated cost over 5 years, 4Ok).
================================================
CONFIDENTIALITY
Sensitive results such as milk testing and anything commercial should be
confidential. Dr. Hope raised the question of the MRC, that there could
be conflict between AFRC and MRC interests. BSE results in NPU should be
disclosed to the MRC and this should be written into any agreement.
================================================
BSE Programme Management Group
7 February 1994
Mr R Bradley Mr. T E D Eddy Mr. K C Taylor Dr. J A Morris
1. Your minute of l February requested a consideration by the BSE
Programme Mangement Group (PMG) of four possible areas/objectives for
action as a result of your discussions with the CVO on your Concept
Note. The BSE PMG has now met to discuss specifically the policy
concern that the BSE agent could have become endemic in the British
sheep population, this has been one of the subjects considered at
recent meetings for future research to fulfill potential policy needs
in order to formulate our research strategy. The following summarises
the unanimous view of the PMG for the initial response requested.
2. It was agreed that there was evidence of scrapie in sheep as a
result of food borne exposure. This is provided by the statistically
significant increase in the incidence of sheep scrapie from 1985, as
determined from analyses of thc submissions made to VI Centres, and
from individual case and flock incident studies. As the working
hypothesis is that there has been recycling of infected cattle tissues
which has augmented the epidemic in cattle the continued infection of
sheep with the BSE agent, via the food borne source, cannot be excluded.
There is therefore also a possibility that the BSE agent may have become
endemic in the sheep population, but it is impossible to design any
short-term research programme to elucidate this.
3. Taking the four possible areas and objectives for consideration in
turn:
3-1 "To determine if natural infection of BSE can be distinguished
clinically or promptly by pathological methods post mortem"
Considering pathological methods initially, in practical terms this
requires a study of the brain changes, patlicularly the distribution
and severity of vacuolar changes (thc lesion profile) in clinical
cases.
This poses the following difficulties:
3.1.1 The lesion profile depends principally on strain of agent and
genotype of the sheep and it is now well established that these are
essentially constants for BSE in cattle and variables for natural
scrapie in sheep. Previous work (Fraser 1976, Barlow unpublished,
Wood et al unpublished) has indicated the difficulties of creating the
necessary baseline to cope with these and possible additional
(e.g. route of infection) interacting variables that affect the lesion
profile. A population study of lesion profiles in sheep therefore
presents considerable difficulties in both the number of sheep brains
requiring examination and the detail of the profile analysis required
to show small differences (see Wells et a1 1992, Wells et al in press).
3.1.2 At present, the identification of "BSE-profile" in sheep rests on
the profiles obtained at first experimental passage of bovine BSE in
sheep, that is across a species barrier. Should it prove possible to
establish a distinctive lesion profile in this small sample there are
no grounds for assuming that such a profile in sheep would be the same
on passage in sheep (infection without a species barrier).
A cattle-to-sheep barrier must be inferred otherwise there would be no
point in executing any of the proposed studies. A second oral passage
of BSE in sheep would be required to test the constancy of a
"BSE-profile" in sheep which would not necessarily reflect the profile
of naturally transmitted BSE in sheep because of variables due to
possible non-oral routes of infection.
3.1.3 The profile approach can only identify the major strain(s)
associated with the development of clinical disease. Food borne risks
to other species depend on the amount and strains of infection in
various tissues used for food. Lesion profiles cannot identify mixed
infections (and clearly do not even identify infection at all if this
is confined to the LRS).
Notwithstanding such difficulties there is merit in conducting a
strictly limited exploratory assessment of existing material since a
sufficiently characteristic profile in sheep (especially if it could
be based exclusively on a medulla-obex examination) provides
information which would be of value in further studies to diagnose
the occurrence of clinical BSE in sheep. The feasability of identifying
a BSE profile in sheep could be explored in the short term from:
- study of the lesion profile in experimental BSE in sheep and goats
(this is already in hand)
- review of Mr J Wood's analysis of Pathology Dept, CVL. sheep and goat
scrapie pathology and possible re-examination of the material (initial
access to Mr Wood's unpublished review is sought)
3.1.4 The potential for clinical distinction of scrapie and BSE in sheep
is, given the variability and multiplicity of the clinical signs, less
sensitive than the lesion profile approach and there are no data on
which to base a preliminary assessment.
3.2 "To determine whether or not BSE agent exists in the national sheep
flock (and, if so, later to determine at what incidence)"
Of the four objectives this is the only one which is fulfillable by a
direct approach which seeks to identify strains of agent in
circumstances where endemic infection with the BSE agent, if it occurs
in sheep, would already be present. Sampling problems can be managed
more economically because strain typing could be carried out with pools
of tissue from a sufficiently large sample of animais.
It also provides the most sensitive approach, and reduces the risk of
false negative results. More specifically, it is capable of identifying
the BSE agent:-
(a) in sheep infected by natural routes and doses
(b) in both LRS and CNS tissues
(c) in mixed infections
(ti) even when present as a quantitatively minor component
As a result, this objective was considered to be the most important
identified in relation to the policy concern. A ROAME proposal is
therefore being prepared and is outlined below.
3.3 "To determine the pathogenesis of experimental BSE in sheep
following oral challenge with BSE brain, the tissue distribution of
the agent during the incubation period and the temporal progression
of infectivity titres in infected tissues"
This has the fundamental problem that it is an indirect approach. It
does not accommodate natural levels of exposure and non-oral routes of
infection and involves the expetimenlal exposure of BSE to sheep. A more
direct approach would be necessary to expose sheep experimentally to
brains from BSE affected cattle and conduct the
ultimate pathogenesis study using passaged material from these sheep,
to inoculate uninfected sheep. This does not, however, overcome the
underlying problem that a negative or positive result with respect to
detectable LRS infectivity, in sheep in this second passage study,
would not be sufficient to discount natural transmission of the BSE
agent in sheep, because natural transmission does not depend exclusively
on LRS infectivity and because of the insensitivity of bio-assays across
the species barrier(i.e, in mice). The absence of an invariable, 100 per
cent, detection rate of the scrapie agent in placentae of affected ewes
is clearly relevant in the consideration of this approach.
3.4 "To determine whether or not maternal transmission occurs in
expedmental BSE in sheep following oral challenge, and if so with
what incidence"
This approach has a number of weaknesses which cannot be removed by
modification of the initial idea. Primarily, it is scientifically
invalid because of the unnatural dose, source and routes of infection
as past studies of the contagious spread of scrapie revealed the extent
to which experimental infection of sheep with scrapie does not reproduce
all of the important features of the natural disease. It is also
impossible to design a study which could simulate natural conditions
as husbandry practices cannot be dismissed as important factors in the
natural transmission of scrapie. The approach is also based on the
premise that maternal transmission provides the main means by which
scrapie is maintained in the sheep population; whereas there is evidence
that horizontal transmission is at least of equal importance and the
precise means by which scrapie is transmitted between sheep remains an
unknown. If such an approach was pursued it would create uncertainties
in the interpretation of a positive result, and a negative result would
be inadequate, on its own, as evidence against the natural transmission
of the BSE agent in the sheep population....
- the collection of a representative sample of brains from Scrapie
affected sheep, using criteria to exclude cases which could have been
infected from a primary food borne source, which is likely to be a mix
of scrapie and BSE.
- the initial genotyping of all pathologically confirmed cases, using a
case definition based on the results of histological and EM examinations
to determine the occurrence of polymorphisms at codons 136 and 171.
- the oral exposure of one group of cattle to a pool of brains from
sheep of one of there genotypes and another to the alternative genotype.
- strain typing of these two homogenates in mice
- strain typing, in mice, of clinical cases occuring as a result of this
exposure.
There are three possible outcomes to this study:
one: +transmission to cattle; - BSE straintyping in mouse
two: +transmission to cattle; + BSE straintyping in mouse
thr: -transmission to cattle; - BSE straintyping in mouse
The second of the above outcomes would provide evidence that the BSE
agent has become endemic in the British sheep population. Outcomes One
and Three would be evidence against this phenomenon.
REFERENCES
FRASER, H. (1976) Slow Virus Diseases of Animals and Man. Ed R.H
Klmberlin. Amsterdam, North-Holland Publishing Company, p267
WELLS, G.A-H., HAWKINS, S.A.C., HADLOW, W.J and SPENCER, Y.I. (1992)
Prion Disease of Humans and Animals. Eds S.B Prusiner, J. Collinge,
J.Powell[ and B. Anderton. Chichester, Ellis-Horwood, p256
WELLS, G.A.H., HAWKINS, S.A.C., CUNNINGHAM, A.A., BLAMIRE, I.W.H.,
WILESMITH, J.W., SAYERS, A.R and HARRIS, P. BSE Update- Proceedings
of a Consultation on BSE with the Scientific Veterinary Committee of
the Commission of the European Communities, 14-15 September 1993,
Brussels (in press)
JW WILESMITH
chairman BSE PMG
Comment (webmaster): Interesting document. It shows that the English were well aware of the risks of BSE back-transfering to sheep but focused mainly on minimizing commercial hardships. Sheep sausage casings, an international commodity, are of special concern given that intestine is a high risk tissue. Was this such a strategic business that the rest of the world needed to be put at risk? We see also that it is not at all easy to distinguish BSE from scrapie experimentally -- no wonder the English were appalled at the USDA press release from Vermont.
French detect yet another new case of mad cow disease
Tue, Jul 25, 2000 AP WorldStream
Authorities have discovered a new case of mad cow disease
in the northern French region of Normandy, the Agriculture Ministry said in
a statement Tuesday.
The milk cow and its herd of four other animals were slaughtered
Tuesday, in accordance with French law. The case, discovered in the
department of Manche, was the 29th detected this year.
Thirty-one cases of mad cow disease, or bovine spongiform
encephalopathy, were reported in 1999, up from 18 in 1998.
I remind the BSE list members that 48 000 test should be used within this
Programme. But only the fallen stock will be targeted, say tests on cattle
slaughtered in emergency for all kind of disorders or because animals came
to slaughter house with a veterinary certificate mentionning a sanitary
problem."
Comment (Dr. Markus Moser, Prionics AG Switzerland 25 Jul 2000): "I think we have to distinguish between cases found in the classical
"passive" surveillance systems, i.e. systems relying on mandatory reporting
and analysis of clinical suspects, and cases found in "active" targeted
surveillance systems using rapid BSE-tests to do post-mortem screens on the
cattle population.
Health ministry previously warned of dura mater
Fri, Jul 28, 2000 Kyodo World Service
TOKYO -- The Health and Welfare Ministry in 1988
ignored a report compiled by a ministry research team warning that
transplant patients who received imported dura mater could contract
Creutzfeldt-Jakob Disease (CJD), a rare and fatal disease, an internal
ministry investigation revealed Friday.
House of Lords: signficant new nvCJD information
27 Jul 00 House of Lords transcripts
See also: prior compilation of questions from Lord Lucas
Lord Lucas asked Her Majesty's Government:
Whether they will now test the tonsil and appendix samples that
were rejected from the tests for nvCJD using one of the
tests that does not require the sample to be in good condition.[HL3443]
Lord Hunt of Kings Heath: To the best of our knowledge, there
is no test for new variant Creutzfeldt-Jakob Disease that does
not require a tonsil or appendix sample to be in good
condition to give a meaningful result.
Some samples in the retrospective studies on tonsil and
appendixes were unsuitable for analysis because they were found by
scientists to be damaged, or otherwise of insufficient quality
to enable analysis to be carried out. At the time the samples were
taken, during routine surgery, there had been no intention of
subjecting them to further analysis.
=-=-=-=-=-=-
Lord Lucas asked Her Majesty's Government:
Whether they will offer tonsil biopsies and magnetic resonance
scans to any residents of Leicestershire who wish to be
assured that they do not have nvCJD.[HL3444]
Lord Hunt of Kings Heath: No. Whilst tonsil biopsy or magnetic
resonance imaging of the brain, taken with other criteria,
can enable clinicians to determine with reasonable confidence
whether or not an individual showing clinical symptoms of the
disease is suffering from new variant Creutzfeldt-Jakob Disease
(nvCJD), there is still no tests available to detect those who may be
infected with nvCJD but have no symptoms.
=-=-=-=-=-=-
Lord Lucas asked Her Majesty's Government:
Whether all the Leicestershire tonsil and appendix samples
taken since 1985 are to be tested for nvCJD; if so, what test
will be used on samples that are not in good condition; and,
if not, what criteria will be used to exclude
samples.[HL3447]
The Parliamentary Under-Secretary of State, Department of Health
(Lord Hunt of Kings Heath): There are
significant logistical and ethical difficulties
associated with the design of any studies of this type.
Any proposal for such a study received by the
Department of Health would need to undergo rigorous
peer-review and ethical approval in line with procedures
established for other similar studies.
The protocol for detecting abnormal prion protein in tonsil
and appendix tissues requires the presence of sufficient lymphoid
tissue in the sample. Results will not be obtainable from samples
where there is insufficient lymphoid tissue present or the tissue
has been so badly damaged that it is unsuitable for prion protein
detection.
=-=-=-=-=-=-
Lord Lucas asked Her Majesty's Government:
How many children of nvCJD victims have moderate or severe
neurological symptoms of unknown origin.[HL3446]
Lord Hunt of Kings Heath: The National Creutzfeldt-Jakob Disease
Surveillance Unit (NCJDSU) is aware of one child of a
variant CJD patient with neurological symptoms. The NCJDSU is not
aware of any suspect cases of nvCJD in a family member
of a nvCJD patient.
=-=-=-=-=-=-
Lord Lucas asked Her Majesty's Government:
What are (to the nearest month) the date of onset,
duration of illness and age at death for all victims of the nvCJD
outbreak; and[HL3427]
What are (to the nearest month) the date of onset, duration
of illness and age at death of the five nvCJD cases in the
Leicestershire cluster; and [HL3445]
What are (to the nearest month) the date of onset and the current
age of victims of nvCJD who are still living.[HL3448]
Lord Hunt of Kings Heath: The date of onset, duration of illness
and age at death, or current age if the patient is still alive, for
all definite and probable new variant Creutzfeldt-Jakob Disease patients
known to the National CJD Surveillance Unit on 20 July
2000 are set out in the table.
We cannot provide data relating to cases in a particular geographical
location in order to preserve the anonymity of patients and their families.
Data for 78 Cases of nvCJD, see previous graph for curve-fitting
Date of Onset Duration(months) Age (Years) at Death, Current Age if Alive
January 1994 29 50
February 1994 23 29
March 1994 38 19
June 1994 11 19
July 1994 18 41
August 1994 18 30
December 1994 26 28
December 1994 11 29
January 1995 17 30
January 1995 10 30
January 1995 13 20
March 1995 11 29
July 1995 14 35
August 1995 9 31
September 1995 30 33
October 1995 17 36
December 1995 14 25
December 1995 11 19
January 1996 17 23
January 1996 23 34
March 1996 12 19
March 1996 14 23
March 1996 25 23
May 1996 24 45
May 1996 29 24
October 1996 12 36
November 1996 8 27
December 1996 24 20
December 1996 33 17
February 1997 11 53
March 1997 39 21
July 1997 13 18
July 1997 13 25
July 1997 15 36
August 1997 16 29
October 1997 16 28
October 1997 12 35
November 1997 11 39
November 1997 11 20
November 1997 12 26
December 1997 14 17
December 1997 14 25
December 1997 12 24
January 1998 16 23
February 1998 11 51
February 1998 26 26
March 1998 8 21
April 1998 7 41
May 1998 14 39
May 1998 7 20
May 1998 18 33
June 1998 20 17
June 1998 14 25
July 1998 Alive Alive 14
July 1998 15 25
July 1998 22 24
September 1998 18 17
September 1998 18 17
October 1998 Alive Alive 30
January 1999 7 29
January 1999 9 54
February 1999 9 34
March 1999 16 20
April 1999 10 27
April 1999 13 19
April 1999 9 15
April 1999 12 22
May 1999 10 25
May 1999 12 31
June 1999 6 43
June 1999 Alive Alive 37
July 1999 11 51
August 1999 Alive Alive 21
October 1999 Alive Alive 28
November 1999 Alive Alive 24
November 1999 Alive Alive 29
December 1999 Alive Alive 27
January 2000 Alive Alive 25
27 Jul 2000:Column WA100 of Surveillance Unit database