Prions
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nvCJD Statistical Update
Swedes pull nerves, organs out of cosmetics, leave in human food
Swedish farmers oppose gross practises of Irish beef
Irish Beef products to be computer-traceable
Dutch blame imported feedstuffs for their BSE
Scientists reject plan to ease British beef ban
Animal hauliers face ban on animal cruelty
Trade-offs on pig brain transplants to humans
Texas mad cow infections spur action
What is UK doing with its TSE-positive sheep?
Under-reporting of BSE in other countries
Strain-typing update

MONTHLY CREUTZFELDT-JAKOB FIGURES

DEPARTMENT OF HEALTH Monday 2nd June 1997

The Department of Health is today issuing the latest monthly table, giving the numbers of deaths of definite and probable cases of Creutzfeldt-Jakob disease in the UK. Total number of definite and probable cases of nvCJD = 17 (including cases still alive and deaths to 30 April 1997). The next table will be published on 7 July 1997.

Year Referrals Sporadi Iatrogenic Familial GSS nvCJD Total
1985 - 26 1 1 0 - 28
1986 - 26 0 0 0 - 26
1987 - 23 0 0 1 - 24
1988 - 21 1 1 0 - 23
1989 - 28 2 2 0 - 32
1990 53 27 5 0 0 - 32
1991 75 32 1 3 0 - 36
1992 96 44 2 4 1 - 51
1993 78 38 4 2 2 - 46
1994 116 52 1 3 3 - 59
1995 86 34 4 2 3 3 46
1996 129 38 4 2 3 10 57
1997 41 4 1 1 0 2 8

Swedes pull nerves, organs out of cosmetics, leave in human food

11 June 1997 Swedish correspondent 
Source: Dagens Nyheter(Swedish daily newspaper)

All members of the European Union are obliged from June 30, 1997 to prohibit the use of brain, eyes and spinal cord in cosmetic products, according to an article in a Swedish newspaper. This means that specified nerve tissues and organs will soon be prohibited in cosmetic products, because it's not proven that it's harmless. The same nerve tissues and organs are permitted in food in the EU, because there's no scientific evidence that it's harmful.

Meat and Bone Meal from carcasses of diseased animals are still permitted in feed to pigs, poultry and pets in the EU. In Sweden, carcasses of diseased farm animals are transported to a garbage dump, stored in containers and then moved to a rendering plant in Stenstorp, in the south.

The brown granulous powder from this rendering plant is exported to Finland and Estonia, to be used in fur farming. Sweden doesn't permit MBM from carcasses of diseased animals to be used for any kind of farming or pet food.

Toni Burgaard, head of DARA, the largest Danish rendering corporation working with carcasses of diseased animals:

"Inside EU there's every year 1.2 million ton of diseased animals to take care of. The result is 500.000 ton of products, fat and meat & bone meal. European fur animals can eat about 5.000 ton MBM. Therefore, it's totally unrealistic to believe that fur animals could eat all this meat & bone meal."
Sweden, as a member of the EU, soon will have to change its policy. It's remarkable myself that the EU in the year of 1997 have not yet prohibited nerve tissues and organs in human food. Especially as we all know that beef products from other EU countries than Britain are not guaranteed to be free from BSE contamination.

Dutch blame imported feedstuffs

12 Jun 1997  Reuters
THE HAGUE - The two cases of madcow disease in the Netherlands were probably caused by imports of contaminated feed, Dutch Farm Minister Jozias van Aartsen said Thursday. In a letter to the Dutch parliament, Van Aartsen said it was difficult to trace the exact origin of the feed but it had probably come from another European country.
"There are no signs of causes other than contaminated feed," a spokeswoman for the ministry quoted the letter as saying. "The information we have points to it coming from another country."
Before 1994, feed containing animal remains could have been imported into the Netherlands despite a ban on its production in the country, she added. Van Aartsen also announced tighter controls on the sale of Dutch beef, removing parts of the head from the food chain. Henceforth, the sale of eyes, brains and spinal cords from cattle will be banned. The move was taken in conjuction with the ministry for public health. .

British plan to ease beef ban rejected by scientists

June 11, 1997  Reuters
BRUSSELS ) - A British bid to ease a worldwide ban on its beef exports by exempting herds certified to be free of mad cow disease was rejected Wednesday by European Union veterinary scientists.

"The proposal was not considered adequate in its present form because the scientific rationale and the information provided was insufficient," the EU's Standing Veterinary Committee said in a statement.

The scientists identified five weaknesses in the plan proposed by Britain in February as a first step toward lifting a 15-month EU ban on British beef exports aimed at guarding against the human form of mad cow disease. The shortcomings included doubts about identifying and tracing animals at risk to the fatal brain wasting disease, known medically as bovine spongiform encephalopathy (BSE). Other criticisms concerned the tracing of live animals and the need to include boned and deboned meat in the scheme, prevention of contamination and mixing of meat as well as veterinary controls and supervision.

But the committee suggested that if the British government corrected the weaknesses, then the scheme could prove acceptable.

Animal hauliers face ban if they flout new laws

June 7, 1997 
BY MICHAEL HORNSBY   London Times  
ROAD hauliers and traders who flout new European rules on the welfare of farm animals during transport will be banned from carrying livestock, the Government said yesterday. This would be in addition to fines of up to 5,000 and/or one month in prison under existing legislation. The penalties can be greater for loads of more than ten animals, which may incur fines of up to 400 per beast.

The regulations, which take effect on July 1, set uniform limits for the first time throughout the European Union on the length of time animals can be transported without being rested, fed and watered. They should have come into force on January 1, but were delayed in the run-up to the general election.

Jack Cunningham, the Agriculture Minister, said: "I am determined that full use should be made of these regulations, especially the powers to withdraw the authorisations of transporters who break the rules."

Animal welfare pressure groups said the measures did not go far enough and questioned whether they could be enforced. Peter Stevenson, political and legal director of Compassion in World Farming, said overall journey times would not be shortened. "British lambs and sheep will still be sent to the Continent on appallingly long journeys in overstocked trucks," he said.

Martin Potter, head of the RSPCA's farm animal division, welcomed the introduction of compulsory training and competency tests for livestock hauliers but said the charity would continue to campaign for a maximum eight-hour journey limit for animals destined for slaughter or further fattening.

The main gain is the replacement of a hotch-potch of conflicting national legislation by a single code, which should make enforcement easier. Journey times may not be reduced, but care of animals should improve.

Under the new rules, calves, lambs, kids, foals and piglets may travel for nine hours before being rested for a minimum of one hour, when they must be offered food and water. They can then travel for another nine hours. Adult cattle, sheep and goats can travel for 14 hours before and after the rest period.

Pigs may travel for a maximum of 24 hours non-stop, provided they have continuous access to water during the journey. Horses can travel for up to 24 hours provided they are given liquid and, if necessary, food every eight hours. Racehorses and show horses, which travel in comparatively luxurious conditions, are exempted from the rules.

Hauliers will have to seek authorisation from the ministry for any journey over eight hours and will have to file a plan stating the destination and rest points. Drivers will also have to show evidence of training in animal handling.

Transplants from animals raise question of spreading disease

By MALCOLM RITTER, AP Science Writer 6-8-97
Dr. Alan Dimick, who's put pigskin on severe burns since 1970, says there's no evidence treatment has infected anybody with pig germs. But Dimick, director of the burn center at the University of Alabama at Birmingham, notes that pigskin stays on for only a day or two. An implanted organ might pose more of a risk, he said.

Dr. James M. Schumacher, a Sarasota, Fla., neurosurgeon who has put fetal pig tissue into the brains of a dozen people with Parkinson's or Huntington's disease over the past two years, also reports no sign of infection. "We are extremely overzealous about studying these effects and looking for viruses in the long and short run, and we haven't to date found any problem," he said.

While scientists ponder the risk of xenotransplantation, thousands of people die each year because they can't get a human organ. "It's a difficult issue," said virus expert Jonathan Allan of the Southwest Foundation for Biomedical Research in San Antonio, who calls the infection risk from pigs very small but worth worrying about.

"Here are people dying," Allan said. "You want to do everything possible to prevent that sort of suffering. But you certainly don't want to foster new infectious disease that would make even greater suffering in the population."

Swedish farmers oppose gross practises of Irish beef

Thursday, May 1, 1997      Irish Times       
Patrick Smyth, European Correspondent
A campaign being run by the Swedish farmers' union against Irish beef has forced the Government to dispatch its own damage limitation team to Stockholm this week. A spokesman for the Department of Agriculture said the campaign was purely protective.

The Frenninge LRF Audeling has placed advertisements in newspapers and sent an open letter to the Swedish Agriculture Minister, Ms Annika Ahrmberg, attacking imported beef on health and animal welfare grounds.

They allege that Irish beef may be contaminated with BSE, salmonella or antibiotics, accusing Irish slaughterers of using exclusively the "barbaric" halal method of slaughter, and Swedish shopkeepers of mislabelling foreign beef.

The advertisements also allege that a quarter of Irish beef is "Belgian blue", whose breeding requires calves to be delivered by Caesarean. The spokesman for the Department said that less than 1 per cent of Irish beef was Belgian blue and that the drying up of the Iran and Iraq markets meant there was virtually no halal butchering.

The Swedish market is worth in excess of 20 million to Ireland. The delegation from Dublin includes the deputy chief veterinary officer, Mr Paddy Rogan, and Mr Patrick Moore of Bord Bia.

Beef products to be traceable under Yates plan

Thursday, May 22, 1997 Irish Times By Kevin O'Sullivan, Environmental and Food Science Correspondent A beef quality assurance scheme that will guarantee the traceability of all Irish beef products has been announced by the Minister for Agriculture and Food, Mr Yates. The scheme, he predicted yesterday, would be the most stringent within the EU in terms of food safety, and was set to restore consumer confidence in Irish beef both at home and abroad.

It is to be phased in over 11 months at an initial cost of about 14 million and requires that all farmers, marts, meat processors and feed compounders be registered. Each part of the supply chain, including cattle dealers and hauliers, face mandatory registration to ensure that at any point in animal's lifetime its full history will be accessible from a central computer.

The scheme places particular onus on farmers, who will be required to sign a declaration to observe strict farm standards on feeds, veterinary care and pollution control. Defaulters will be delisted, in effect debarred from beef farming. Farmers may be liable to on-the-spot inspections by both regulatory authorities and buyers from export countries and major supermarket chains throughout the EU.

Known as the National Beef Assurance Scheme (NBAS), "it represents a fresh and integrated approach to the key task of restoring consumer confidence in beef products following the effects of BSE", the Minister said. "Anybody wishing to produce beef products for human consumption must be registered to do so and that means reaching the standards set out in the scheme."

The standards required would be "unprecedented within the EU" and while it would help to regain live trade markets in the Third World, most significantly it would propel Irish beef "into the premier league with European supermarket chains"; an appropriate development for the largest exporter of beef within the EU.

A central element of the scheme is a computerised movement monitoring system (CMMS) based in his Department which will provide a snapshot of cattle at every stage of their history: their current location, their movement and ownership records, disease status and other data. While the Department will finance its own initial capital costs, running costs will be borne by the industry.

Farmers should not see the scheme as more unnecessary bureaucracy, Mr Yates said, as they would be adopting a code of excellence that would ultimately add value to their product. Details of on-farm certification have yet to be agreed. The Department would continue to validate meat processors, marts and compound feed manufacturers. With the NBAS's statutory backing rogue operators would be delisted, but the form of penalties and prosecution had to be finalised. Marts and meat processors would be obliged to deal only with cattle from registered persons.

The animal feed obligations would also be considerable. Some 40 per cent of compounders were already listing their feed ingredients but he was concerned that some co-ops had yet to participate.

The system will require both buyers and sellers to notify the Department of cattle movements within three days of them taking place. This will be made as simple as possible through electronic back-up with computers at marts and meat plants, facilitating the transfer of information into the CMMS in the Department.

Consumers and veterinary teams from major customers such as export countries or retail outlets will be able to access CMMS to verify all food safety concerns. The scheme initially will enable the complete tracing of a carcass of beef.

In time it was likely that a cut of meat - for example, a piece of steak - would be completely traceable back to the farm it came from by way of a computer bar code, Mr Yates confirmed.

Mad cow infections spur action

 UPI US   Sun, Jun 15, 1997
HOUSTON, June 15 (UPI) -- A report says Texas health officials have begun an investigation into an unexplained rise in "mad cow disease" cases in northeast Texas. The Houston Chronicle reports today that health officials are concerned because five people have been diagnosed with the disease since April 1996. One or two cases in the period is the norm.

Julie Rawlings of the Texas Department of Health tells the Chronicle the cases could be a statistical blip, or the high point of a bell curve. She says, "There's not enough data yet to determine whether it's a trend."

The disease, formally called Creutzfeldt-Jakob, has been detected in a 22-county area in northeast Texas. The state has 254 counties. Statistics show the disease fells on average about one person per million in the United States, but the rate of infection can vary from year to year. It is usually fatal. State health department official Dr. Kate Hendricks, who is director for infectious disease, tells the Chronicle that from 1984 through 1994 there were 111 people in Texas who had the disease listed on their death records, or a rate of .76 cases per million population per year.

Texas authorities are trying to determine if a rare, fatal brain disorder linked to "mad cow" disease is claiming an unusually high number of victims in the state's northeast.

The state investigation comes weeks after U.S. authorities unveiled new rules to ward off potential outbreaks of "mad cow" by banning most animal-derived protein in cow, sheep and goat feeds. The U.S. Food and Drug Administration said the ban aims to protect animals from neurological diseases such as bovine spongiform encephalopathy (BSE) and minimize risk to humans.

BSE, the scientific name for mad cow, has been tied to Creutzfeld-Jakob disease, which killed 16 people in Britain who ate possibly contaminated beef in the late 1980s or early 1990s. The deaths sparked worldwide concern about beef consumption and led to a European Union ban on British beef imports.

Rawlings said the Texans with Creutzfeldt-Jakob disease do not appear to have died from the same form of the disease as the British victims.

But Melvin Massey, a veterinarian whose wife, Judith, died from Creutzfeldt-Jakob disease on June 1, says he remains "suspicious."

Houston-June 16-FWN--Cases of CJD in humans in Texas should not be linked to bovine spongiform encephalopathy in cattle, according to Alisa Harrison with the National Cattlemen's Beef Association (NCBA).

The Houston Chronicle reported Sunday that health officials are concerned because five people have been diagnosed with CJD since April 1996. One or two cases in the period is the norm, according to officials.

"There is a man who maintains his wife died of CJD," Harrison told FWN, "but that has not been confirmed. The coroner hasn't determined whether she died of CJD." The man in question maintains his wife ate imported brisket and that's how she contracted the disease, Harrison noted.

"There continues to be no link between CJD and BSE," Harrison stressed. "And even if we're talking about the new variant, a person would have had to have eaten beef brain or spinal cord from an animal with BSE. We have not imported any such product or meat since 1986, so it's highly unlikely that this case has any link with BSE. The other cases are the regular form of CJD, not the variant," Harrison noted.

In the Houston Chronicle article, Julie Rawlings of the Texas Department of Health said it's not clear if the cases could be a statistical blip, or the high point of a bell curve. "There's not enough data yet to determine whether it's a trend," she said. CJD has been noted in a 22-county area in northeast Texas. The state has 254 counties. Statistics show CJD kills on average about one person per million in the United States, but the rate of infection can vary from year to year. It is usually fatal.

Texas State health director for infectious disease Dr. Kate Hendricks told the Chronicle that from 1984 through 1994, there were 111 people in Texas who had the disease listed on their death records, or a rate of 0.76 cases per million population per year.

NBC Vital Signs: Fatal Familial Insomnia

NBC Vital Signs: Fatal Familial Insomnia

  Fri, 6 Jun 1997
NBC TV
FFI (fatal familial insomnia was profiled on NBC's "Vital Signs" last night. They said it was like both "Mad Cow Disease" (because it stems from a protein in the brain that goes haywire) and like Alzheimers (because it involves an unnatural buildup of proteins in the brain). While the profiled patient was dying in the hospital, they made his visiting wife wear protective clothing and gloves because they don't know if its contagious, however they think its genetic. The husband donated his brain to sceince so it could be studied.

Commentary:

FFI is just a variant of CJD. Same gene, same protein, just a particular point mutation at codon 178. Simply a turf war and grantsmanship, everyone wants to "discover" a new disease. FFI is extremely variable in presentation. It is a poor idea to call every allele of the prion gene another disease because it is all the same disease.

Better nomenclature would be CJD D178N (met 129 met) if that's what they have. It is cheaper and more informative to sequence the patient's prion gene than to have them overnight in the hospital for diagnostic observation.

Creutzfeldt's first case was not CJD. Jakob's first case was FFI in a codon 129 val/val background in later generations.

What is UK doing with its TSE-positive sheep?

Question:

What happens to the scrapie carcasses and spinal cords removed at the abattoir from sheep and goat? Are they incineratated, stored at sea, or rendered or what? Under the FDA rules they could be exported them to the US where they would still be marketable, though who would admit to buying them?

Answer:

Julie Brace-Maclean
JBM International Associates
It's unclear what they will do with the scrapie carcasses - BSE carcasses are incinerated. Incineration capacity is very limited.

The spinal cords will become "specified risk materials" so will be rendered in designated plants. The resulting meal and fat will then stored in government facilities until it can be incinerated.

To give you an idea of the lack of incineration capacity, since the beginning of the Over Thirty Months Scheme more than 1.38 million cows with an average weight of 470 kilos have been slaughtered and destroyed. They too go to designated rendering plants, are processed and the meal and tallow is stored by the Intervention Board awaiting the same fate. Yet only 59,000 tons of meal has been incinerated to date. The remainder is sitting around in disused aircraft hangers.

A further 750-800,000 cows will be destroyed under the OTMS scheme annually thereafter, so the problem will continue to grow.

According to the Daily Telegraph (UK) on Friday, June 13, 1997, the first case of BSE in a cow born on 17.1.94 has been acknowledged by the government. The new case was in a 34 month old highland cow born on a farm in Wales. The cow's dam is alive and well.

Under-reporting of BSE in other countries

According to the Sunday Times of 1 June 1997, a paper submitted to Veterinary Record asserts:
"An international team of scientists who were given access to Britain`s official records of cattle exports from 1985 to 1989 have concluded that almost every country in the EU has failed to report large numbers of infected animals imported into their herds."
Portugal 96 cases, 250 expected
Germany 5, over 200 expected
Ireland 218, 1000 expected
Spain nil, 50 expected
Italy 2, 50 expected
France 28 homebred, 30 in imported expected
Belgium nil, over 12 expected
Holland 2, over 40 expected
Denmark 1, over 30 expected. 

J Almond speaking on strain-typing update

On 13 June the following post appeared on PROMED: Source: Dr Jeffrey Almond, Reading UK, speaking on "The UK Epidemic of BSE: Is it a Threat to Human Health?", Conference on Emerging Infections, Harvard School of Public Health, Boston Mass., Wed. 11 June 1997

Dr Almond stated that strain typing of 7 strains of BSE showed that their PrP (prion protein) signatures were:

- - identical to each other
- - clearly distinguishable from the signature of scrapie
- - clearly distinguishable from the signature of CJD 
- - not distinguishable from the signature of TSE in cats (cat food contains cattle remains)
- - not distinguishable from the signature of TSE in antelopes in British zoos
- - not distinguishable from the signature of CJD (new variant) 
As of April 1997 there have been 167 204 cases of BSE on 34 059 farms, with the highest concentration of infection in southern England. Age range of bovines infected is 20 months to 18 years. The chance of finding a second case on an infected farm was no greater than finding a first case on a new farm.

The BSE epidemic peaked in 1994, and so far there have been no cases in 1994-born animals, so the bovine epidemic in the UK should end around 2002. But modeling suggests that, since many cattle were slaughtered for food before they could have shown symptoms, there must have been 1 million cattle infected, of which two-thirds have been eaten.

The ban on feeding ruminant protein to cattle took effect in 1988, and the bovine epidemic peaked in 1994. The ban on human concumption of certain parts of cattle took effect in Nov.1989, and the appearance of human cases of CJD (new variant) fits the start of a bell curve of cases with a longer incubation period, which could be as much as 20 years, than BSE in cattle. This would be expected for an infection that has crossed a species barrier. The chances of 18 cases (at last count) of CJD (new var.) occurring in the UK with its population of 50 million have been calculated at 1 in 10 to the 8th power. There has been a single confirmed case of CJD (new var) in France.

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