Prion Deletion and Insertion Mutants
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New CJD victim had Saturday job in butcher's
British nvCJD Cases: Summary to 6 Sept 97
nvCJD Geographic Clustering to Sept 3 97
Zoo animal victims
Sept 24 meeting: CJD transmission by blood transfusion
Book review of Deadly Feasts
Hudson denies recalled beef going to tacos and pizza
Canada bans some rendered products
Does Germany under-report BSE?
Does Ireland under-report BSE?
Swiss report two new cases of mad cow disease
E.U. Scientists Consider U.S. Tallow Ban Appeal
US Industry sees minimal risk in tallow
EU official says food checks outdated
EU to appeal WTO ruling on hormone-treated meat
Livestock desertification threatening one-third of Earth's surface
Vegetarian cooking moves to front burner

New CJD victim had Saturday job in butcher's

September 4 1997  Times
BY RUSSELL JENKINS
A MAN who worked part-time in a butcher's shop a decade ago has become the latest victim of Creutzfeldt-Jakob disease. The diagnosis was made two days before Michael Connor, 59, died.

Tests by the CJD Surveillance Unit at Edinburgh Western Infirmary will show whether he was suffering from the new variant of the disease, which has been linked to beef. Rochdale Health Authority has been treating the case as one of the original strain, but a spokesman said that they did not have "100 per cent confirmation".

The rapid onset of the disease, originally thought to be Alzheimer's or a virulent form of senile dementia, shocked the family of Mr Connor, from Littleborough, Rochdale. He began losing his memory in December, and quickly deteriorated from a vigorous man into a helpless invalid. His wife, Joyce, 56, said that he had not eaten meat frequently but had worked in a butcher's shop on Saturdays from 1977 to 1987 to help a friend.

Mr Connor's daughter, Sharon, 35, said that he first became forgetful and became angry about insignificant things. In January he could not remember where he worked as a machine operator, and he went into a nursing home for specialist care. "By the end of July his condition had got so bad that he couldn't walk or do anything. We had to feed him." He died on August 9.

Diane Welden, another daughter, said: "His illness should have been indentified earlier. Because we had been told that he had Alzheimer's, we thought he could still live a long time."

Kevin Snee, the director of public health for Rochdale, said that it was difficult to make an accurate early diagnosis of CJD because the onset of the condition was so rapid.

British nvCJD Cases: Summary to 9 Sept 97

Kindly provided by: Hans G. Andersson
Editor, Ebola section of OUTBREAK
# 1 Victoria Rimmer, 18, kennel worker from Connays Quay in North Wales. She was the first teenager to contracted V-CJD. Alive, has been in deep coma since 1994.

# 2 Stephen Churchill, 19, student from Wiltshire. From a report by Prof Collinge et al.: "An 18-year-old male was initially referred to a psychiatrist with suspected depression. He gave a 6 month history of deterioration of memory, complaining that he had 'gone nutty'...

Initial treatment was with antidepressants... There were frequent episodes in which he would scream and appear frightened without an obvious precipitant. He was transferred to a nursing home and died with progressive respiratory problems". (The Lancet, Vol 346, October 28, 1995) He died in May, 1995.

# 3 Peter Hall, 20, student from Durham. He showed signs of depression around Christmas 1994, was sent to a psychiatric hospital and put on three kinds of antidepressants. Five months later he was in wheelchair and his short-term memory was shot. In August '95 he was incapable of feeding himself, incontinent and sometimes hallucinatory. In the final stages suffering from dementia and a series of chest infections.

Peter Hall became a vegetarian in 1992. However, the CJD Surveillance Unit, as well as a recent verdict from North Durham County Coroner, have concluded that he probably contracted the disease by eating beef as a child.He died in February 1996, just days before his 21st birthday.

# 4 An unknown 26-year-old woman developed illness with psychiatric symptoms in 1994. She died after nearly two years of disease in 1996.

# 5 Anna Pearson, 29, solicitor from Canterbury, Kent. Initial symptoms of psychiatric illness reported in October 1994. She was becoming increasingly forgetful and disoriented in the spring of '95. "In June, 1995, she was admitted to the psychiatric ward at her local hospital because of continuing deterioration. She had become childlike in her behaviour, had a very poor short-term memory, and experienced visual and auditory hallucinations, with intermittent periods of drowsiness and confusion." (Dr S J Tabrizi in The Lancet, Vol 347, April 6, 1996)

She was later transferred to a Intensive Care Unit for Neurology. Her cognitive state continued to deteriorate and she died on February 9, 1996.

# 6 Michelle Bowen, 29, Manchester. She was pregnant with her third child when developed severe behavioural changes and mood swings. The baby was delivered three months prematurely by caesarean section. After giving birth her condition deteriorated and she fell into a come from which she never recovered. She died on November 25, 1995.

# 7 Alison Williams, 30, Wales. She died in February 1996.

# 8 Maurice Callaghan, 30, Belfast. He died in December 1995, after being in coma for several weeks.

The grave was dug to a depth of nine feet instead of the usual six, and the gravediggers "were issued with protective clothes and surgical gloves at the funeral." (The Times, March 21, 1996)

# 9 Andrew Haig, 31, process engineer from Glenrothes, Scotland.

Personality changes was registred in October 1995. He lost his short-term memory, started to lose his balance and it progressed quite quickly from there. He died on May 25, 1996.

# 10 Ann Richardson, 41, Liverpool. Health care assistant and mother of one. In 1994 she was diagnosed as suffering from depression, gradually lost coordination and became bed-ridden. She died in January, 1996.

"It was a horrible death. Her nervous system completely closed down, she could not walk, talk or swallow, and at the end she was not aware of us," said Cathy Hilton, her sister-in-law. (The Times, Jan. 10, 1996)

# 11 Barry Baker, 29, woodcutter from High Halden, near Ashford, Kent. He died on June 2, 1996.

# 12 Ken Sharp, 42, Businessman from Liverpool. First symptoms in April 1995, died in March 1996, after many months in coma.

# 13 was a 16-year-old schoolgirl from London. Developed first signs of illness in September, 1994. She was born in the UK of Turkish-Cypriot parents, and lived in the UK. She was a Muslim but had eaten corned beef, beef burgers and (!) cow brain (in Cyprus 1989).

# 14 Leonard Franklin, 52, Abattoir worker. First symptoms Sept '95, died in February 1996.

# 15 Victoria Lowther, 19, student. She died on November 19, only 4 months after first symptoms.

# 16 Adrian Hodgkinson, 25, former RAF corporal. Fell ill in March 1996, died in February 1997.

# 17 Janice Stuart, 35, first symptom October 1995, died in September, 1996.

# 18 Donna Mellowship, 33, First symptom in March 1996, died in April or May 1997.

# 19 Matthew Parker, 19, Docaster, South Yorkshire. Developed first symptoms in December 1996, died in March 1997.

# 20 ???

# 21 Sue Carey, 36, Mersham, Kent. First symptoms in 1995, died March 8,1997.

# 22 Mandy Minto, 27, mother of two, former European judo champion, from Sunderland, Tyne and Wear. Died in the end of July 1997. First showed signs eight months earlier.

# 23 Donnamarie McGivern, 15, Coatbridge, Lanarkshire. Being seen by Southern General Hospital, Glasgow. Alive.

# 24 Keith Humphrey, 42, engineer from Northfield, Birmingham. Onset 7/95, confirmed 3/96, Died, July 15, 1997.

# 25 Clare Tomkins, 24, Tonbridge, Kent. Strict vegetarian since 1985. Onset April-Oct/96. Alive. Tonsil biopsy by John Collinge, August 97.

# 26 Chris Warne, 36, computer systems analyst, Ripley, Derbyshire. Onset 12/96, confirmed 8/97. Alive.

Non-British Cases

# 1 Henri Rodriguez, 27, France. First symptoms in 1994, died on January 6, 1996.

nvCJD Geographic Clustering

Kindly provided by Kari Koester-Loesche
Thu, 04 Sep 1997 
Residences of nvCJD and CJD victims in GB.
Rochdale, where the latest suspected case lives, is north of Manchester.

Kent:

1.  Nr. 4 above. student, 24 (either Canterbury oder Kent)
2.  Anna Pearson, 29, Canterbury
3.  Barry Baker, 29, Ashford
4.  Graham Brown, 36, Ashford
5.  Elizabeth Bottle, 59, Ashford (possibly not nvCJD)
6.  Sue Carey, 36, Mersham near Ashford, husband active in agriculture
7.  Clare Tomkins, 24, Tonbridge, vegetarian, active in pet department of garden center
8.  (Stephen Churchill, 19, from Wiltshire, often vacationed in Sissinghurst by Smarden - Whitakers BSE)
9.  Woman, 51, Ashford, (1989, suspected nvCJD)
10. Her twin sister, 58, Ashford (1996, suspected nvCJD)

Northumberland/Durham (Sunderland cluster):

1.  Peter Hall, 20, Durham
2.  Jean Wake, 38, Washington, Tyne and Wear, rendering factory
3.  Mandy Minto, 27, Sunderland, athlete
4.  A metalworker, 53, Sunderland (1989)
5.  A grandmother, 67, Seaham (1992)
6.  Vikar 63, Sunderland (1992)
7.  Frau, 61, Seaham (1993)

Scotland:

1.  Janice Stuart, 34, Glasgow
2.  Helen Rutherford, 15, Glasgow area
3.  Donna-Marie McGivern, 15, Coatbridge near Glasgow

Central

1.  Anne Richardson, 41, Liverpool, health professional
2.  Ken Sharp, Liverpool
3.  Michelle Bowen, 29, Manchester, slaughter business
4.  Matthew Parker, 19, Doncaster, ate pate and sausage
5.  Chris Warne, 36, Ripley, fitness-fanatic, Sports
6.  Keith Humphreys, 42, Birmingham
7.  Mann, 51, Birmingham
8.  Michael Connor, 59, Rochdale by Manchester, slaughter business

London:

1.  Woman, 42 (?)
2.  Woman, 33 (?)
3.  Teenager, 17, Tárkisch-Cypriot, brain as spice

Scattered:

1.  Victoria Rimmer, 18, Connays Quay
2.  Alison Williams, 30, Wales
3.  Maurice Callaghan, 30, Belfast, active athlete, red meat
4.  Henri Rodriguez, 27, France
5.  Man, middle-aged, North Yorkshire
6.  Leonard Franklin, slaughterhouse worker

Human and Zoo animal victims

Mon, 1 Sep 1997  
Charles Arthur, The Independent
I have been keeping a count of the new v-CJD cases reported in the press, and which I have been able to confirm either by talking to families or where a reputable doctor has been involved in the diagnosis. Thus I have a figure which tends to be higher: I reckon Chris Warne is the 26th victim, they had him as the 23rd. I think they have overlooked cases including Mandy Minto, Donnamarie McGivern and Keith Humphrey, whose cases have all been reported in the past few months. All three of those are alive as far as I know.

(22) Mandy Minto, 27, mother of two, former European judo champion, from Sunderland, Tyne and Wear. died end July 97. First showed signs eight months previously.

(23) Alive: Donnamarie McGivern, Coatbridge, Lanarkshire. Being seen by Southern General Hospital, Glasgow. Confirmed June 97? Parents Marie and James.

(24) Keith Humphrey, 42, engineer, onset 7/95, confirmed 3/96, D July 15 97. Lived Northfield, Birmingham, widow Carol (37).

(25) Alive: Clare Tomkins, 24, strict vegetarian since 1985, onset April-Oct/96, D alive. Tonsil biopsy by John Collinge, August 97. Lived in Tonbridge, Kent. 4th Kent victim.

(26) Alive: Chris Warne, 36, computer systems analyst. onset 12/96, confirmed 8/97. Lives Ripley Derbyshire. Never had anything to do with animals, according to parents. Student at Blackpool's Fleetwood College between 1979 and 1983.

Zoo animals. Remember the kudu et al? First kudu TSE was noticed in London zoo in 1986. Since then fewer than 30 animals from 10 species (I think the exact number is 29, from talking to vets) have developed TSEs. All have been in ungulates and big cats. None in canids or horse family. This is from a population of thousands of ungulates and big cats kept in captivity in zoos around Britain.

Could there be subclinical cases which were missed? Almost all large animals which die in zoos are given postmortems: it is, say zoo vets I spoke to, (a) interesting to see the inner workings of animals that you might not usually see (b) even if you're sure of the cause of death - eg concrete block fell on head - to see if they had any chronic infections which might be affecting other animals. TSEs would have been noticed if there had been clear symptoms in the brain. The remains are usually incinerated, not fed to other animals or sold for rendering.

Typical ages at which the animals showed symptoms is interesting. Recall that ungulates would have received MBM, and big cats most likely meat (including spinal cord).

For ungulates: illness showed at ages 3-5. (They can live to 20 years in capvitity.)

For big cats: illness showed at ages 6-12 yrs. (They can live to their mid-20s in captivity.

Webmaster comments:

Dog -wolf - dingo have been sequenced and are 98%+ identical to mink in the mature protein. I myself would want to see some data on the number of canids in zoos and determined causes of mortality as well as the results of transmission studies. Would there have been expert immunopathology on canids that died prior to 1987? When did the_first_ zoo animal get noticed? We have far more sensitive methods today for detecting TSE than we did ten years ago -- did any brains of deceased zoo canids from that era get frozen? And it is not too late today to take a closer look -- canids could simply have a longer incubation period. Ditto for all zoo animals that received MBM or CNS. But nobody really wants to look into this because it might turn out badly.

I find this business of whistling in the dark to be exceedingly stupid. Time is slipping away. We had a rare long range experimental opportunity here, but it got twisted into a PR bromide. If there is a serious species barrier to canids, great, but we need to establish that, to help understand barriers overall.

But all MAFF can think about is saving its own butt, England be damned. Is MAFF really so stupid to think that scientists in other countries aren't going to look at canid transmission in vitro, a la Raymond et al.? I predict canids will be about halfway between below-threshold and felids.

MAFF sees this strictly as an issue of the emotional PR burden of TSE in companion animals, so suppressed experimental work in dogs, saying "we don't eat them here" (which incidentally would be considered an overtly racist remark in US culture). The UK needs to get control over its scientific enterprise -- precious time is being frittered away.

RG Rowher at Baltimore has completed the sequence of the cat prion. It is being privately circulated. The felid prion overall is a good example of the wasted effort that goes on in this field -- personal agendas and private feuds take priority over public health. At least four different labs worked on sequencing this one (long overdue) prion gene while other equally important prion genes got nothing. There is no central research clearing house at this time to avoid redundant effort, like with the Human Genome Project.

Hudson denies reports it wanted to cook recalled beef

Reuters September 5, 1997
DAYTON, Ohio Hudson Foods Inc. denied Friday it had considered cooking recalled beef at a high enough temperature to kill any E. coli bacteria so the meat could be resold for use in prepackaged foods. The Dayton Daily News reported Thursday that the company had asked the U.S. Department of Agriculture for permission to cook the meat so it could be used as pizza toppings or tacos, then changed its mind and rescinded the request.

Asked to comment on the newspaper's report, spokesman Robert Udowitz said Friday that Hudson "has never considered using the returned meat for consumption." He said the company had only mentioned cooking at high temperature as a method of recycling to illustrate "previous methods that the company had been aware of." The company was waiting for the USDA to finish its investigation and tell the company what to do with the recalled meat before Hudson decides how to dispose of it, Udowitz said.

An outbreak of illness traced to contaminated meat forced the Rogers, Ark.-based company to recall 25 million pounds of ground beef processed at its Columbus, Neb., plant. That plant has been closed and sold. Udowitz said 6 million pounds of the meat have been recovered so far and is being held in cold storage at various points around the country.

EU official says food checks outdated

Reuter Information Service September 3, 1997
BRUSSELS - European Union members need to bring national checks on food safety up-to-date to combat current threats to consumer health, EU Farm Commissioner Franz Fishler said Wednesday. Addressing the two-day "Food Safety - A National Debate" conference, Fischler said methods used by some countries had yet to take into account new diseases and health hazards.
"I believe that current inspection systems are unsatisfactory. In a number of cases they are designed to detect problems which were common decades ago, but are no longer the most serious food-related hazards," he said in a live link-up from Brussels, where a copy of the speech was released.
Fischler said the EU had to retain the right to take any action it deemed necessary to protect consumers' health, even if it meant going beyond international recommendations. The conference is aimed at developing food safety policy in the wake of major scares such as BSE (mad cow disease) and E. coli food poisoning in Britain. Referring to the BSE crisis, Fischler said the EU took a precautionary approach to the problem, and although it was opposed by some governments, the action was a vindication of a community-wide approach.
"The proposal (to exclude ruminant tissues from animal feed) met strong resistance from a number of member states, who were unwilling to admit they had, or could have, a problem and were consequently unwilling to introduce necessary changes," he said. "This example demonstrates...the key role the Commission can play with its inspections, legislative initiatives and surveillance for the preservation of human and animal health."
He called for action at farm level to ensure micro-organisms were excluded from the food chain, and the establishment of a proper food-borne disease reporting system, to allow the speedy adoption of suitable food legislation in response to health hazards at any given time.

Desertification threatening one-third of Earth's surface

Agence France-Presse September 3, 1997
NIAMEY - Global desertification costs more than $42 billion a year and threatens one-third of the Earth's surface, delegates meeting this week to study the phenomenon's effects in Asia and Africa were told Wednesday. Around 100 delegates from 24 African and 13 Asian countries gathered in Niger's capital also learned that 2.4 billion hectares (six billion acres) of arid land are threatened with destruction "in the near future."

Contrary to popular belief, the problem of desertification is not restricted to the Sahel countries of north Africa. Across the planet, deserts are advancing by an annual 70,000 square kilometers (27,000 square miles), leading to losses of some $42.3 billion a year, United Nations experts told AFP. Last year, the U.N. announced that more than 1 billion people across the globe are exposed to the harmful effects of desertification and 135 million of them risk losing all their land. The problem is most serious in Africa and Asia.

Eighty percent of arid lands in Africa are affected while 3.7 million of the continent's 696 million hectares (nine million of the continents 1.7 billion acres) of forest disappear every year. In Asia, which produces more than 30 percent of the world's timber but is home to just 16 percent of the planet's forestland, massive logging accelerates the phenomenon. Efforts to counter desertification are hampered by the extreme poverty and population growth of affected countries.

The population of Asia and Africa, where more than 55 percent of the world lives, mostly in rural areas, grows by an average of 1.8 percent per year. China is the country worst affected by desertification in Asia. Two-thirds of the country is covered by desert. Each year some 2,500 square kilometers (965 square miles) are rendered unsuitable for cultivation.

According to government figures, more 400 million Chinese are affected by desertification. In 1993, severe sandstorms killed more than 100 people in the northwest, and destroyed 337,000 hectares (833,000 acres) of farmland. The damage was estimated to cost $66 million. China has suggested setting up an Afro-Asian research center to study and fight desertification.

Niger, 90 percent of which is covered by desert, has welcomed the proposal. The Sahelian country can afford to reforest only a quarter of the 200,000 hectares (500,000 acres) it loses to the desert each year. The Niamey meeting, funded by Japan, follows a similar event held in Beijing last year when Asian and African states decided to form a partnership to strengthen their ability to fight desertification and drought.

Central themes this year include early warning methods, traditional ways of tackling desertification, research and transfer of technology between the two continents. The meeting falls under the framework of the International Convention to Fight Desertification signed in Paris in 1994 and ratified by 106 countries. The convention is due to hold a conference in Rome at the end of September.

Vegetarian cooking moves to front burner

The Christian Science Monitor September 4, 1997
BOSTON -- Anna Thomas's first cookbook, "The Vegetarian Epicure," put her through college. It was an original - one of the first to celebrate vegetarian cuisine with wonderfully inventive recipes. The sequel was likewise a hit, reflecting Thomas's travels around Europe and her own progressive "improvisations." While plenty of cookbooks of the time included delicious vegetable recipes (usually as side dishes), Thomas helped popularize the vegetable as an end in itself, and she changed the way many of us looked at zucchini and spinach forever.

Times have changed, and Thomas's cooking style has changed, too. Her latest tome on the kitchen arts, "The New Vegetarian Epicure" (Alfred A. Knopf, 449 pp., $19.00) which came out last year, reflects her concerns. Thomas has been raising two rambunctious boys, writing movie scripts, and producing films for the last 20 years, and now more than ever her writing reflects her very real delight in meal preparation. Producing, she says wryly, puts you always between a rock an a hard place, so cooking cames as a relief.

"Having had kids and raising them has taken me in another direction," Thomas said in an interview. "You're a different person when you have kids. It really is a whole new world view - and way of cooking, and body of knowledge and experience.

"Kids like simple food, and you have to cook more often. At the same time I got more sophisticated - both sides were developing at once. I'm a whole different cook now than I was before. But the pleasure in cooking is what it's always been.

"This book just has a much greater range. There is still plenty of what I like to call recreational cooking for people who cook on weekends for fun, and who like to make elaborate complicated things for entertaining or whatever. But there is also a lot more basic, every day, fresh food."

And "fresh" is key. One simple and delicious recipe, Cream of Sweet Corn Soup, which is made from onions, butter, a hint of garlic, and milk (no cream) really does require the freshest possible ears of corn - right out of the field is best, but in any case, so fresh the kernels burst when you poke them with a fork. If the corn is even a day past its prime, the soup loses its raison d'etre.

Many of her simplest recipes depend on the freshness of the ingredients. The way Thomas prefers to cook is to go in the kitchen, figure out what she's in the mood for, and cook it - without recipes.

"Once you have a basic sense of ease and comfort with food then you respond to everything - the season, your circumstances, what ingredients are around and are best," she says. Sometimes it's good to have a plan, she says, to follow a procedure that is tried and true, but she encourages readers to improvise on a basic theme, too, and never to apologize for departing from a recipe.
That's how she came up with her Wild Chocolate and Chili Torte. Her favorite Mexican mole, Mole Poblano, is made with lots of spices, chile and chocolate - its emphasis on chile makes it powerful stuff. The result is a spicy chocolate desert with a surprising kick. When she served it to guests, she says, no one could guess what gave it that extra punch.

Thomas's spirit of adventure is limitless, and she is always in pursuit of the best tasting vegetable or fruit. She experiments freely with new varieties of vegetables and fruits wherever they spring up. Her improvisations with squash are sometimes simple and yet just delicious. One of her favorites is a large sweet round squash called Kabocha, which shows up in several recipes - soups, a roasted vegetable medley, even in a pasta sauce.

The charm of this book lies in the graceful, witty introductions to each section. Organized in menus for holidays, special occasions, small parties, and family evenings, it's meant to reflect Thomas's lively perspective. "Food is not a chore, it's a gift," she offers. "You're participating in something that is part of nature, and nature is always alive and changing."

EU to appeal WTO ruling on hormone-treated meat

Reuter Information Service September 4, 1997
BRUSSELS - The European Union plans to appeal a decision by the World Trade Organization that the EU's ban on hormone-treated meat was unlawful under free trade rules, European Commission sources said Thursday. The eight-year-old EU ban on imports of beef produced with the aid of synthetic growth hormones mainly affects imports from the United States, which won the WTO ruling in early July. According to the U.S. beef industry, the ban has cut sales to EU countries by between $100 and $250 million a year.

The commission's decision to appeal has the full backing of all EU member states and consumers' groups and is likely to come ahead of the next meeting of the WTO's appeals committee, probably toward the end of this month.

"If the committee meets on Sept. 25, the commission will lodge an appeal the day before," one EU source said.
However, a formal decision to appeal could be made only after all member states had been consulted, but this looked to be nothing more than a formality, the source said. The matter may be discussed at an informal meeting of the bloc's 15 agriculture ministers in Luxembourg next week. Under WTO rules, once an appeal is made, the trade body has up to 90 days to issue a final ruling. If the EU loses the appeal the WTO's Dispute Settlement Body (DSB) could ask the EU to pay compensation to the United States for lost trade.

If the EU refused, then the DSB could authorize U.S. trade retaliation to the same amount. Washington has been backed in its complaint by Australia, New Zealand, Canada and Norway. Argentina, which exports beef free of hormone treatment, has supported Brussels' position. The appeal decision has come at a time when relations between the EU and WTO are strained. EU Farm Commissioner Franz Fischler was widely reported on Thursday as questioning "lack of democratic control" at the Geneva-based body. A commission spokesman on Thursday said Fischler's comments had been "misinterpreted," but reiterated the EU's view that it should be able to set its own standards on food safety.

"We've said we don't agree with the WTO decision (on hormone-treated beef)...we believe we have the right to set our own levels of protection," he said.
Fischler told a London food safety conference on Wednesday the EU "would not hesitate to take the necessary action when the health of Community consumers is at risk," even if this went beyond international recommendations.

CJD and its possible transmission by blood transfusion

The National Heart, Lung, and Blood Institute (part of the US National Institutes of Health) will hold a meeting to discuss proposed contract or grant solicitations in the area of CJD and its possible transmission by blood transfusion. The meeting will be held on September 24 beginning at 9 am at the NIH, Building 31, C Wing, Conference Room 7, 9000 Rockville Pike, Bethesda, MD. Contact Luiz H. Barbosa, (301) 435-0075. The meeting is open to the public. This notice is available from the Federal Register, September 4, 1997) (Vol 62, No 171, p.46749) and through GPO access (wais.access.gpo.gov)

BSE: under reporting in Germany?

Fri, 29 Aug 1997
Wolfgang Klee
Medical Animal Clinic
University of Munich
The previous posting contained the statement "Germany reported five - the expected number was 243." and a moderator commented "Congratulations to the Swiss on accurate and thorough BSE surveillance. For the others, what they don't look for, doesn't exist.

I cannot let this stand without commentary. We do look for BSE. To be more precise: Cows with signs of CNS disturbance that are admitted to our clinic (which basically services southern Bavaria, an area with intensive dairying) are either clinically cured (a highly unlikely event in cows with BSE), or they go to necropsy which includes brain histology. Our faculty includes a full professor for neuropathology.

The brains of all cows with a history of CNS disturbance that are sent to Veterinary Investigations Centers for necropsy are specifically examined for BSE.

There have been many continuing education presentations for practitioners on BSE. Prof. U. Braun from Zurich, who has examined a good share of the Swiss BSE cases, has been in high demand to show his impressive videos. I see no reason to believe that veterinary practitioners are intentionally holding back cows with signs of CNS involvement, nor do I see a reason to believe that there is a policy to cover up.

Under-reporting of BSE in Ireland?

Wed, 3 Sep 1997 
Veterinary Record letter
John Griffin
Veterinary Epidemiology and Tuberculosis Investigation Unit
Vet College,Dublin.
The three most critical points in Mr. Griffins letter are:

1. Most "imports" (84-98%, depending on the year in question) were from Northern Ireland

2. BSE incidence in the N. Ireland is about 10% of that in the British mainland and

3. Most "imports" (82% in 1985) were slaughtered shortly after importation. The whole herd is slaughtered after a single case is confirmed in the herd.

"In the Veterinary Record of August 23, 1997 you published a paper entitled "Risk of BSE from the import of cattle from the United Kingdom into countries of the European Union" (Schreuder and others 1997). In respect of the Republic of Ireland, the basic assumptions used in the paper are so flawed as to considerably misrepresent the number of potential BSE cases imported and to make the exercise very misleading.

This is the case for two reasons in particular. Firstly, the various incidence rates assumed were calculated from the incidence rates in Great Britain, whereas in relation to Northern Ireland the incidence was approximately one tenth of that in Great Britain. This is important because most imports during the period 1985-89 were from Northern Ireland and not from Great Britain. Secondly, the categories of animals assumed to contribute to the potential BSE cases were derived by the authors from EUROSTAT statistics using categories which included animals intended for slaughter, and which should therefore have been excluded from the calculations.

In relation to the first point, the paper states that "it was assumed that the various incidence rates, as calculated for Great Britain, were representative of the UK as a whole". However, as has been stated in papers previously published in the Veterinary Record, Northern Ireland is a separate epidemiological entity from Great Britain and the risk of infection for all cattle in Northern Ireland was approximately one-tenth of that in Great Britain (Denny and others 1992, Denny and Hueston 1997). Most imports into the Republic of Ireland from the UK were from Northern Ireland during the period under study. For example in 1985, the reports of the Irish Central Statistics Office indicate that the total number of Category 1 and Category 3 animals imported from the UK was 9,710. Of these, only 28 were imported from Great Britain, the remaining 9,682 being imported directly from Northern Ireland.

This pattern repeated itself in the subsequent years under consideration, with approximately 84% of category 1 and category 3 animals imported from the UK coming from Northern Ireland. Accordingly, this error alone resulted in a considerable overestimate in the number of potential BSE animals imported into the Republic of Ireland. Furthermore, if additional adjustments were made for the fact that the BSE outbreak in Northern Ireland did not occur until some years after the outbreak in Great Britain, the probability of infection associated with animals imported from Northern Ireland would be even further reduced.

In relation to the second point, the model assumed that the category "live heifers, cows and bulls all weighing over 220 kg." did not include animals for direct slaughter, whereas in fact this category did include such animals. To illustrate the effect which this assumption has, of the 9,682 animals in categories 1 and 3 imported from Northern Ireland in 1985, a total of 7,978 (or 82% of the total) were intended for slaughter, leaving just 1,704 (or 18% of the total) for consideration.

The combination of these two errors is multiplicative and affects the predictions dramatically. The paper therefore grossly over states the number of potential BSE cases imported into Ireland.

There are other issues which further contribute to the error involved. For example, it was assumed that all animals imported from the UK were animals born and reared in the UK prior to export. This assumption is not valid because animals exported to Northern Ireland and subsequently returned to the Republic of Ireland were included in the EUROSTAT figures as "imported animals". The EUROSTAT import figures also included animals from other countries which only transited the UK or had undergone quarantine in the UK, such as Canadian animals en route to the Republic of Ireland. All cattle recorded as UK imports following the ban on the importation of live cattle from the UK in February 1990 were born outside the UK. Such imports in previous years should have been excluded from the category of animals likely to contribute to potential BSE cases.

In conclusion, the calculations made by Schreuder et al were based on a number of erroneous assumptions which considerably exaggerated the total number of potential BSE cases imported. The danger of these types of mathematical studies and their predictions is that, to all but the authors, sight is often lost of the basic assumptions upon which they are built. If left unchallenged these predictions tend to become accepted as fact.

Left unchallenged, therefore, the conclusions of this paper, and the innuendo of under reporting contained therein, could cause considerable and utterly unjustifiable damage to the good name and reputation of the livestock farmers, the veterinary profession and the State Veterinary Service of the Republic of Ireland. There is every incentive to report suspect cases, with full market value being paid, at prices agreed with the farmers involved, for all herds affected with BSE."

SCHREUDER, B.E.C., WILESMITH, J.W., RYAN, J.B.M. & STRAUB, O.C.
(1997) Veterinary Record 141, 187-190 DENNY, G.O., WILESMITH, J.W.,
CEMENTS, R.A. & HUESTON, W.D. (1992) Veterinary Record 130, 113-116
DENNY, G.O. & HUESTON, W.D. (1997)  Veterinary Record 140, 302-306

Swiss report two new cases of mad cow disease

August 29, 1997
Reuter
GENEVA -- The Federal Veterinary Office in Switzerland was cited as reporting two new cases of mad cow disease on Friday, bringing this year's total to 27. The story adds that the report did not say whether the animals affected were born after the ban on the use of cattle feeds containing meal made from animal remains, effective since December 1990, or give details.

Switzerland has the second highest number of mad cow cases in the world fter Britain's 167,000 cases in the past decade. Some 30 countries have curbs on Swiss cattle and beef imports. The story also adds that the Geneva-based World Trade Organization predicted on Thursday that world beef consumption was expected to recover in 1997 from last year's dip, with beef and veal consumption in the EU expected to rise by two per cent after 1996's seven percent fall.

In Switzerland, new data on Friday showed beef consumption rose one per cent in the first half of 1997 over the same 1996 period, compared to an eight per cent drop in the first half of 1996 over a year ago.

Book review of Deadly Feasts

Review of the review -- webmaster:

This is a rather knowledgeable review that squarely and fairly addresses shortcomings of Rhodes' book, except for one passage:
"These studies, which are still ongoing, have established prions as a unique paradigm in microbial genetics: agents that exist as an abnormal conformer of a host protein, and that can 'replicate' by catalyzing the conversion from the normal to the abnormal form of PrP. Thus prions constitute an exception to the central canon of molecular biology, 'DNA makes RNA makes protein'. "
For the last time: Prion protein is made in a completely conventional way from a perfectly ordinary gene on a perfectly ordinary autosomal chromosome via perfectly conventional mRNA to perfectly conventional ribosomes and perfectly conventionally modified and perfectly conventionally exported and perfectly conventionally processed. NO ONE, certainly not Prusiner, certainly not viral people, have EVER proposed a novel mechanism for creating the covalent peptide bonds of any prion molecule of any isoform.

Now, do the words "allosteric interaction" possibly sound relevent here? Which part of "oligomer" don't people understand? What aspect of "induced conformational change" is causing the big problem? Which letters of "hemoglobin" are confusing? "Cytoplasmic inheritance" -- where else is stuff supposed to go after cell division? Do the 32,000 pages of JBC per year x 40 years ever discuss these topics? I say they do: this has been all documented before in a thousand other proteins (or is it 5,000?) I am all in favor of exceptions to the central canon, and lord knows we have seen plenty of them, but replication simply isn't one of them.

Something is odd here , but it is a little hard to dissect out exactly what is odd. There is no big mystery on the protein chemistry side about the replication of a conformer, this happens all the time (actin, tubulin, etc.). and familial CJD doesn't have any unusual features in isolation. The infectious part seems like it could conceivably happen (no problem in vitro) but that it does happen in the real world fairly efficiently is surprising. Perhaps it is the slowness that we are not used to.

It may have to do with the GPI anchor and the protein exposed and available on the cell surface, unlike a cytoplasmic protein, plus neurons don't divide and are supposed to last a lifetime. this would seem to fit aguzzi's work that there needs to be normal prion along the route.However, this would say that some of the other conformational diseases of plasma proteins would also be infectious, but here there could be clearing of nucleated seed. There are no other neural GPI proteins on the list; quite a few of the others haven't really be tested properly. and intra-cerebral injection of other things might be infections but not dietarily, so of little practical consequence. Perhaps there are a fair number of proteins with a subset of the individual properties but by the time a few consequtive screens are run there is only prion protein left of any infectious significance.

Which is not to detract from Prusiner's contribution; as Nathanson notes,

"the field had reached a critical impasse.... Stanley Prusiner, together with a highly gifted group of collaborators, made a series of seminal observations, beginning in the late 1970s, that revolutionized this field..."
And some people have never forgiven him for this. But there is some genuine interest here to the working protein chemist in the slow cross-beta accumulation that seems to underlie so many of these conformational diseases.
Nature Medicine
Volume 3 Number 8 - AUGUST 1997
Deadly Feasts: Tracking the Secrets of a
                  Terrifying New PlagueBy Richard Rhodes
                  By Richard Rhodes
                  Reviewed by Neal Nathanson
                  University of Pennsylvania Medical Center
                  Philadelphia, PA 19104-6146
This book provides a layman's account of the diseases known collectively as transmissible spongiform encephalopathies (TSEs), which are caused by a novel group of agents often referred to as prions. The story is developed along historical lines, beginning in the 1950s with investigations by Carleton Gajdusek of kuru (an epidemic neurological disease that occurred in the stone age peoples living in the highlands of eastern New Guinea), bringing in scrapie of sheep (the prototype of the TSEs), and ending with the epidemic of bovine spongiform encephalopathy (BSE) that began in the United Kingdom in the 1980s and is predicted to end over the next five years. The author has done extensive bibliographic research and conducted numerous interviews with many of the scientists who have worked in this field, with the notable exception of Stanley Prusiner (who does not give interviews) and several of his outstanding collaborators.

Judged as a chronicle for a lay audience, this is a well wrought presentation that captures the readers' interest at the outset and builds the story piece by piece to form a coherent narrative. It informs painlessly while maintaining the tension of a good tale to the very end. This book has been the subject of several recent reviews1, that have properly lauded the author for journalistic skill in developing his theme. However, for a scientific audience, certain other questions are in order. Is this an accurate representation of the history of discovery in this particular field? Is a balanced view presented of the hazards to human health? Is the author appropriately fair and dispassionate in parcelling out either credit or blame? When judged by these criteria, I can only conclude that this is a severely flawed presentation.

Even a superficial reading makes it clear that the author has taken sides among the scientific protagonists in the prion field, which is most unfortunate considering that this is a domain of research that has traditionally been plagued with more than its share of acrimonious disputes. Furthermore, the focus upon recent events in Gajdusek's personal life is, in my view, a tasteless distraction, which may sell books by catering to the prurient interests of potential readers, but scarcely belongs in a dispassionate scientific history.

The description of prion research fails to indicate that in the late 1960s, in the wake of the dramatic demonstration of the transmissibility of kuru and Creutzfeldt Jacob disease (a rare but widespread spongiform encephalopathy of humans) to primates by Gajdusek and his collaborators (work for which he received the Nobel prize in 1976), the field had reached a critical impasse. There were a plethora of biological and clinical observations that demonstrated that the spongiform agents possessed novel properties such as resistance to inactivation by radiation; an apparent lack of antigenicity; and an ambiguous status as either transmissible agents or inherited traits. However, the true nature of these agents presented an enigma that appeared to resist scientific attack, although a widely diverse set of unsubstantiated theories had been proposed.

Stanley Prusiner, together with a highly gifted group of collaborators, made a series of seminal observations, beginning in the late 1970s, that revolutionized this field2. First, a meticulous effort to purify the transmissible agent identified a single small protein (the protease resistant protein, or PrP) as the major component of infectious fractions, and a partial amino acid sequence permitted construction of oligonucleotide probes that identified the mRNA encoding this protein. Unexpectedly, the message was expressed at similar levels in the brains of mice with and without scrapie, indicating that PrP was a host protein that existed in two forms, normal and disease-related (the latter being distinguished by its resistance to protease digestion).

Exploiting the advent of new methods in mammalian genetics, Prusiner and his collaborators made extensive use of transgenic rodents to systematically explore the role of PrP in determining the biological parameters of TSEs, such as host susceptibility, incubation period, lesion distribution, prion strain specificity, and the species barrier to transmission. For example, one of their models has recapitulated a familial prion disease of humans (Gerstmann Straussler Scheinker disease, associated with a point mutation in the PrP gene) and showed that it was both heritable and transmissible. Furthermore, Charles Weissmann showed that PrP knockout mice lose their susceptibility to TSE. These studies, which are still ongoing, have established prions as a unique paradigm in microbial genetics: agents that exist as an abnormal conformer of a host protein, and that can 'replicate' by catalyzing the conversion from the normal to the abnormal form of PrP. Thus prions constitute an exception to the central canon of molecular biology, 'DNA makes RNA makes protein'. This background is neither adequately covered or acknowledged by Rhodes, and given that it is at the core of the field, this is a major failing.

The closing pages of this book are devoted to new variant Creutzfeldt Jacob disease (nvCJD), a syndrome that can be distinguished from established forms of Creutzfeldt Jacob disease by a constellation of clinical and pathological descriptors, including its concentration in humans under age 35 years3. It appears that nvCJD has occurred almost exclusively in the UK and only since 1994, and it has therefore been proposed that it might represent transmission of BSE to humans. Although Rhodes' version of recent events is generally substantive, it veers toward the apocalyptic, emphasizing predictions such as 200,000 deaths per year from nvCJD in 2015, whereas some workers4 still reserve judgement whether the disease is indeed causally linked to BSE. Furthermore, to flatly state that BSE is enzootic in the United States is irresponsible reporting and certainly does not represent a consensus opinion among informed observers.

In summary, Deadly Feasts is a journalistic tour de force, which will intrigue and inform the reader, but not a book that can be read literally as an accurate representation of scientific history, or as a balanced assessment of public health risks.

Canada bans some rendered products

Agriculture & Agri-Food Canada press release, September 5, 1997
OTTAWA -- The Canadian Food Inspection Agency (CFIA) has taken steps to ban the use of some rendered animal products in the manufacture of feed for ruminants such as cattle, sheep and goats.

The change to regulations is in response to a World Health Organization (WHO) request that all countries ban the feeding of ruminant tissue to ruminants in light of public health concerns around Bovine Spongiform Encephalopathy (BSE), a fatal disease in cattle in the United Kingdom. Although Canada and the U.S. are recognized as free of BSE, these regulations are necessary to safeguard Canada's animal health status and maintain domestic and international confidence in the safety of Canadian animal products, said Dr. Graham Clarke, Chief of Red Meat Inspection Programs.

Due to the wide impact of these regulations, the CFIA has consulted extensively with members of Canada's feed, rendering and animal-based food production sectors to ensure that all viewpoints have been heard and reflected in the final regulations, Dr. Clarke said. Rendered products originate from inedible portions of animals. These are recycled to renderers who cook them at high temperatures, separating fat from protein. Rendered fats have a variety of industrial and feed uses while rendered protein has traditionally been used in livestock feeds.

The amendments will affect the way in which rendered animal products can be used. Farmers can no longer use ruminant materials as ingredients in ruminant feed. Renderers, feed manufactureres and farmers are required to take steps to avoid cross-contamination when mixing feed. New labelling requirements have been introduced to ensure that all products containing banned materials are clearly identified.

Canada has had only one case of mad cow disease, in a herd of Alberta cattle which was destroyed in 1993, the report said. Before the latest ban, ground-up bits of ruminants often made up 20 percent or more of cattle feed in Canada. Under the new regulations, feed manufacturers and farmers are also required to take steps to avoid cross-contamination when mixing feed. In addition, all products containing banned materials should be clearly identified under the new labeling requirements.

E.U. Scientists Meet To Consider U.S. Tallow Ban Appeal

Dow Jones Mon, Sep 8, 1997 BRUSSELS (Dow Jones)--Scientific experts from the European Union are meeting Monday in Brussels to discuss whether U.S. tallow producers should be exempted from E.U. meat processing rules that could block $100 million a year of U.S. tallow exports and have far-reaching implications for the U.S. pharmaceutical industry.

The E.U. Multidisciplinary Committee on Bovine Spongiform Encephalopathy, or BSE, will examine whether U.S. tallow producers should be exempted from a forthcoming ban on the use for any purpose of animal remains deemed at risk of harboring BSE - or 'mad cow' disease.

The remains - known as specified risk material - are the head and spine of cattle, sheep and goats over 12 months old and the spleen of all sheep and goats. The ban is due to take effect Jan. 1, 1998. Tallow, or animal fat, is usually made by 'rendering' or boiling up whole animal carcasses.

The U.S. has threatened to complain to the World Trade Organization if forced to change slaughterhouse practices in order to safeguard U.S. tallow exports to the E.U. One third of E.U. tallow supplies - or about 500,000 metric tons a year - come from non-E.U. countries. The U.S. has stepped up diplomatic pressure on the E.U., warning that $4.5 billion of U.S. pharmaceuticals exports, of which tallow derivatives such as magnesium stearate, could also be affected by the ban.

Industry Sees Minimal BSE Risk

Dow Jones  Mon, Sep 8, 1997
The U.S. pharmaceuticals association - Pharmaceutical Research & Manufacturers of America, or PhRMA, has lobbied U.S. government officials - including U.S. Trade Representative Charlene Barchefsky and Commerce Secretary William Daley - to oppose the ban, arguing that the risk of actually contracting BSE-related disease from taking medicines is 'less than one in 100 billion.'

Indeed, the 20-member Commission has come under pressure to reconsider the matter not only from the U.S. but from E.U. industries that use tallow. They have warned of possible shortages of tallow because of the new meat processing rules. Any exemption from the specified risk material ban would have to be authorized by the E.U. Commission, which manages E.U. trade policy. The Commission itself drew up and authorized the new meat processing rules.

To date, the Commission has argued that the ban on all uses of specified risk material is a prudent measure to protect consumers in the wake of the scare resulting from the blanket ban on all U.K. beef exports because of mad cow disease. The Commission earlier this year was criticized by the European Parliament for downplaying the risk to human health of BSE. The BSE scare was prompted by a U.K. announcement in March 1996 of a possible link between BSE in beef and a fatal brain ailment in humans. The E.U. subsequently imposed the export ban, which remains in place.

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