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[Fwd: Could Mad Cow Disease Already be Killing Thousands of Americans

daemon@ATHENA.MIT.EDU (Laura Dilley)
Thu Jan 8 15:40:20 2004

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--
Laura Dilley
Ph.D. Candidate
Speech and Hearing Bioscience
   and Technology
Harvard-MIT Division of Health
   Sciences and Technology


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Subject: Could Mad Cow Disease Already be Killing Thousands of Americans
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	Please feel free to forward or reprint any part of my new report

Could Mad Cow Disease Already be Killing Thousands of Americans Every Year?
by Michael Greger, M.D.
January 7, 2004

October 2001, 34-year-old Washington State native Peter Putnam 
started losing his mind. One month he was delivering a keynote 
business address, the next he couldn't form a complete sentence. Once 
athletic, soon he couldn't walk. Then he couldn't eat. After a brain 
biopsy showed it was Creutzfeldt-Jakob disease, his doctor could no 
longer offer any hope. "Just take him home and love him," the doctor 
counseled his family.[1,2,3] Peter's tragic death, October 2002, may 
have been caused by Mad Cow disease.

Seven years earlier and 5000 miles away, Stephen Churchill was the 
first in England to die. His first symptoms of depression and 
dizziness gave way to a living nightmare of terrifying 
hallucinations; he was dead in 12 months at age 19.[4] Next was Peter 
Hall, 20, who showed the first signs of depression around Christmas, 
1994. By the next Christmas, he couldn't walk, talk, or do anything 
for himself.[5] Then it was Anna's turn, then Michelle's. Michelle 
Bowen, age 29, died in a coma three weeks after giving birth to her 
son via emergency cesarean section. Then it was Alison's turn. These 
were the first five named victims of Britain's Mad Cow epidemic. They 
died from what the British Secretary of Health called the worst form 
of death imaginable, Creutzfeldt-Jakob disease, a relentlessly 
progressive and invariably fatal human dementia.[6] The announcement 
of their deaths, released on March 20, 1996 (ironically, Meatout 
Day[7]), reversed the British government's decade-old stance that 
British beef was safe to eat.[8]

It is now considered an "incontestable fact" that these human deaths 
in Britain were caused by Bovine Spongiform Encephalopathy (BSE), or 
Mad Cow disease.[9] Bovine means "cow or cattle," spongiform means 
"sponge-like," and encephalopathy means "brain disease." Mad Cow 
disease is caused by unconventional pathogens called 
prions--literally infectious proteins--which, because of their unique 
structure, are practically invulnerable, surviving even 
incineration[10] at temperatures hot enough to melt lead.[11] The 
leading theory as to how cows got Mad Cow disease in the first place 
is by eating diseased sheep infected with a sheep spongiform 
encephalopathy called scrapie.[12]

In humans, prions can cause Creutzfeldt-Jakob disease (CJD), a human 
spongiform encephalopathy whose clinical picture can involve weekly 
deterioration into blindness and epilepsy as one's brain becomes 
riddled with tiny holes.

We've known about Creutzfeldt-Jakob disease for decades, since well 
before the first mad cow was discovered in 1985. Some cases of CJD 
seemed to run in families; other cases seemed to just arise 
spontaneously in about one in a million people every year, and were 
hence dubbed "sporadic." The new form of CJD caused by eating beef 
from cows infected with Mad Cow disease, though, seemed to differ 
from the classic sporadic CJD.

The CJD caused by infected meat has tended to strike younger people, 
has produced more psychotic symptoms, and has often dragged on for a 
year or more. The most defining characteristic, though, was found 
when their brains were sampled. The brain pathology was vividly 
reminiscent of Kuru, a disease once found in a New Guinea tribe of 
cannibals who ate the brains of their dead.[13] Scientists called 
this new form of the disease "variant" CJD.

Other than Charlene, a 24 year old woman now so tragically dying in 
Florida, who was probably infected in Britain, there have been no 
reported cases of variant CJD in the U.S.[14] Hundreds of confirmed 
cases of the sporadic form of Creutzfeldt-Jakob disease, however, 
arise in the United States every year,[15] but the beef industry is 
quick to point out these are cases of sporadic CJD, not the new 
variant known to be caused by Mad Cow disease.[16] Of course, no one 
knows what causes sporadic CJD. New research, discussed below, 
suggests that not hundreds but thousands of Americans die of sporadic 
CJD every year, and that some of these CJD deaths may be caused by 
eating infected meat after all.

Although the fact that Mad Cow disease causes variant CJD had already 
been strongly established, researchers at the University College of 
London nevertheless created transgenic mice complete with "humanized" 
brains genetically engineered with human genes to try to prove the 
link once and for all. When the researchers injected one strain of 
the "humanized" mice with infected cow brains, they came down with 
the same brain damage seen in human variant CJD, as expected. But 
when they tried this in a different strain of transgenic "humanized" 
mice, those mice got sick too, but most got sick from what looked 
exactly like sporadic CJD! The Mad Cow prions caused a disease that 
had a molecular signature indistinguishable from sporadic CJD. To the 
extent that animal experiments can simulate human results, their 
shocking conclusion was that eating infected meat might be 
responsible for some cases of sporadic CJD in addition to the 
expected variant CJD. The researchers concluded that "it is therefore 
possible that some patients with [what looks like]... sporadic CJD 
may have a disease arising from BSE exposure."[17] Laura Manuelidis, 
section chief of surgery in the neuropathology department at Yale 
University comments, "Now people are beginning to realize that 
because something looks like sporadic CJD they can't necessarily 
conclude that it's not linked to [Mad Cow disease]..."[18]

This is not the first time meat was linked to sporadic CJD. In 2001, 
a team of French researchers found, to their complete surprise, a 
strain of scrapie--"mad sheep" disease--that caused the same brain 
damage in mice as sporadic CJD.[19] "This means we cannot rule out 
that at least some sporadic CJD may be caused by some strains of 
scrapie," says team member Jean-Philippe Deslys of the French Atomic 
Energy Commission's medical research laboratory.[20]

Population studies had failed to show a link between CJD and lamb 
chops, but this French research provided an explanation why. There 
seem to be six types of sporadic CJD and there are more than 20 
strains of scrapie. If only some sheep strains affect only some 
people, studies of entire populations may not clearly show the 
relationship. Monkeys fed infected sheep brains certainly come down 
with the disease.[21] Hundreds of "mad sheep" were found in the U.S. 
in 2003.[22] Scrapie remains such a problem in the United States that 
the USDA has issued a scrapie "declaration of emergency."[23] Maybe 
some cases of sporadic CJD in the U.S. are caused by sheep meat as 
well.[24]

Pork is also a potential source of infection. Cattle remains are 
still boiled down and legally fed to pigs (as well as chickens) in 
this country. The FDA allows this exemption because no "naturally 
occurring" porcine (pig) spongiform encephalopathy has ever been 
found. But American farmers typically kill pigs at just five months 
of age, long before the disease is expected to show symptoms. And, 
because pigs are packed so tightly together, it would be difficult to 
spot neurological conditions like spongiform encephalopathies, whose 
most obvious symptoms are movement and gait disturbances. We do know, 
however, that pigs are susceptible to the disease--laboratory 
experiments show that pigs can indeed be infected by Mad Cow 
brains[25]--and hundreds of thousands of downer pigs, too sick or 
crippled by injury to even walk, arrive at U.S. slaughterhouses every 
year.[26]

A number of epidemiological studies have suggested a link between 
pork consumption and sporadic CJD. Analyzing peoples' diet histories, 
the development of CJD was associated with eating roast pork, ham, 
hot dogs, pork chops, smoked pork, and scrapple (a kind of pork 
pudding made from various hog carcass scraps). The researchers 
concluded, "The present study indicated that consumption of pork as 
well as its processed products (e.g., ham, scrapple) may be 
considered as risk factors in the development of Creutzfeldt-Jakob 
disease." Compared to people that didn't eat ham, for example, those 
who included ham in their diet seemed ten times more likely to 
develop CJD.[27] In fact, the USDA may have actually recorded an 
outbreak of "mad pig" disease in New York 25 years ago, but still 
refuses to reopen the investigation despite petitions from the 
Consumer's Union (the publishers of Consumer Reports magazine).[28]

Sporadic CJD has also been associated with weekly beef 
consumption,[29] as well as the consumption of roast lamb,[30] veal, 
venison, brains in general,[31] and, in North America, 
seafood.[32,33] The development of CJD has also, surprisingly, been 
significantly linked to exposure to animal products in 
fertilizer,[34] sport fishing and deer hunting in the U.S.,[35] and 
frequent exposure to leather products.[36]

We do not know at this time whether chicken meat poses a risk. There 
was a preliminary report of ostriches allegedly fed risky feed in 
German zoos who seemed to come down with a spongiform 
encephalopathy.[37] Even if chickens and turkeys themselves are not 
susceptible, though, they may become so-called "silent carriers" of 
Mad Cow prions and pass them on to human consumers.[38] Dateline NBC 
quoted D. Carleton Gajdusek, the first to be awarded a Nobel Prize in 
Medicine for his work on prion diseases,[39] as saying, "it's got to 
be in the pigs as well as the cattle. It's got to be passing through 
the chickens."[40] Dr. Paul Brown, medical director for the US Public 
Health Service, believes that pigs and poultry could indeed be 
harboring Mad Cow disease and passing it on to humans, adding that 
pigs are especially sensitive to the disease. "It's speculation," he 
says, "but I am perfectly serious."[41]

The recent exclusion of most cow brains, eyes, spinal cords, and 
intestines from the human food supply may make beef safer, but where 
are those tissues going? These potentially infectious tissues 
continue to go into animal feed for chickens, other poultry, pigs, 
and pets (as well as being rendered into products like tallow for use 
in cosmetics, the safety of which is currently under review[42]). 
Until the federal government stops the feeding of slaughterhouse 
waste, manure, and blood to all farm animals, the safety of meat in 
America cannot be guaranteed.

The hundreds of American families stricken by sporadic CJD every year 
have been told that it just occurs by random chance. Professor 
Collinge, the head of the University College of London lab, noted 
"When you counsel those who have the classical sporadic disease, you 
tell them that it arises spontaneously out of the blue. I guess we 
can no longer say that."

"We are not saying that all or even most cases of sporadic CJD are as 
a result of BSE exposure," Professor Collinge continued, "but some 
more recent cases may be--the incidence of sporadic CJD has shown an 
upward trend in the UK over the last decade... serious consideration 
should be given to a proportion of this rise being BSE-related. 
Switzerland, which has had a substantial BSE epidemic, has noted a 
sharp recent increase in sporadic CJD."[43] In the Nineties, 
Switzerland had the highest rate of Mad Cow disease in continental 
Europe, and their rate of sporadic CJD doubled.[44]

We don't know exactly what's happening to the rate of CJD in this 
country, in part because CJD is not an officially notifiable 
illness.[45] Currently only a few states have such a requirement. 
Because the Centers for Disease Control (CDC) does not actively 
monitor the disease on a national level,[46] a rise similar to the 
one in Europe could be missed.[47] In spite of this, a number of U.S. 
CJD clusters have already been found. In the largest known U.S. 
outbreak of sporadic cases to date,[48] five times the expected rate 
was found to be associated with cheese consumption in Pennsylvania's 
Lehigh Valley.[49] A striking increase in CJD over expected levels 
was also reported in Florida[50] and New York (Nassau County)[51] 
with anecdotal reports of clusters of deaths in Oregon[52] and New 
Jersey.[53]

Perhaps particularly worrisome is the seeming increase in CJD deaths 
among young people in this country. In the 18 years between 1979 and 
1996, only a single case of sporadic CJD was found in someone under 
30. Whereas between 1997 and 2001, five people under 30 died of 
sporadic CJD. So five young Americans dying in five years, as opposed 
to one young case in the previous 18 years. The true prevalence of 
CJD among any age group in this country remains a mystery, though, in 
part because it is so commonly misdiagnosed.[54]

The most frequent misdiagnosis of CJD among the elderly is 
Alzheimer's disease.[55] Neither CJD nor Alzheimer's can be 
conclusively diagnosed without a brain biopsy,[56] and the symptoms 
and pathology of both diseases overlap. There can be spongy changes 
in Alzheimer's, for example, and senile Alzheimer's plaques in 
CJD.[57] Stanley Prusiner, the scientist who won the Nobel Prize for 
his discovery of prions, speculates that Alzheimer's may even turn 
out to be a prion disease as well.[58] In younger victims, CJD is 
more often misdiagnosed as multiple sclerosis or as a severe viral 
infection.[59]

Over the last 20 years the rates of Alzheimer's disease in the United 
States have skyrocketed.[60] According to the CDC, Alzheimer's 
Disease is now the eighth leading cause of death in the United 
States,[61] afflicting an estimated 4 million Americans.[62] Twenty 
percent or more of people clinically diagnosed with Alzheimer's 
disease, though, are found at autopsy not to have had Alzheimer's at 
all.[63] A number of autopsy studies have shown that a few percent of 
Alzheimer's deaths may in fact be CJD. Given the new research showing 
that infected beef may be responsible for some sporadic CJD, 
thousands of Americans may already be dying because of Mad Cow 
disease every year.[64]

Nobel Laureate Gajdusek, for example, estimates that 1% of people 
showing up in Alzheimer clinics actually have CJD.[65] At Yale, out 
of a series of 46 patients clinically diagnosed with Alzheimer's, six 
were proven to have CJD at autopsy.[66] In another study of brain 
biopsies, out of a dozen patients diagnosed with Alzheimer's 
according to established criteria, three of them were actually dying 
from CJD.[67] An informal survey of neuropathologists registered a 
suspicion that CJD accounts for 2-12% of all dementias in 
general.[68] Two autopsy studies showed a CJD rate among dementia 
deaths of about 3%.[69,70] A third study, at the University of 
Pennsylvania, showed that 5% of patients diagnosed with dementia had 
CJD.[71] Although only a few hundred cases of sporadic CJD are 
officially reported in the U.S. annually,[72] hundreds of thousands 
of Americans die with dementia every year.[73] Thousands of these 
deaths may actually be from CJD caused by eating infected meat.

The incubation period for human spongiform encephalopathies such as 
CJD can be decades.[74] This means it can be years between eating 
infected meat and getting diagnosed with the death sentence of CJD. 
Although only about 150 people have so far been diagnosed with 
variant CJD worldwide, it will be many years before the final death 
toll is known. In the United States, an unknown number of animals are 
infected with Mad Cow disease, causing an unknown number of human 
deaths from CJD. The U.S. should immediately begin testing all cows 
destined for human consumption, as is done in Japan, should stop 
feeding slaughterhouse waste to all farm animals (see 
http://organicconsumers.org/madcow/GregerBSE.cfm), and should 
immediately enact an active national surveillance program for CJD.[75]

Five years ago this week, the Center for Food Safety, the Humane 
Farming Association, the Center for Media & Democracy, and ten 
families of CJD victims petitioned the FDA and the CDC to immediately 
enact a national CJD monitoring system, including the mandatory 
reporting of CJD in all 50 states.[76] The petition was denied.[77] 
The CDC argued that their passive surveillance system tracking death 
certificate diagnoses was adequate. Their analysis of death 
certificates in three states and two cities, for example, showed an 
overall stable and typical one in a million CJD incidence rate from 
1979 to 1993.[78] But CJD is so often misdiagnosed, and autopsies are 
so infrequently done, that this system may not provide an accurate 
assessment.[79]

In 1997, the CDC set up the National Prion Disease Pathology 
Surveillance Center at Case Western Reserve University to analyze 
brain tissue from CJD victims in the U.S. in hopes of tracking any 
new developments. In Europe, surveillance centers have been seeing 
most, if not all, cases of CJD. The U.S. center sees less than half. 
"I'm very unhappy with the numbers," laments Pierluigi Gambetti , the 
director of the Center. "The British and Germans politely smile when 
they see we examine 30% or 40% of the cases," he says. "They know 
unless you examine 80% or more, you are not in touch."[80] "The 
chance of losing an important case is high."[81]

One problem is that many doctors don't even know the Center exists. 
And neither the CDC nor the Center are evidently authorized to reach 
out to them directly to bolster surveillance efforts, because it's 
currently up to each state individually to determine how--or even 
whether--they will track the disease. In Europe, in contrast, the 
national centers work directly with each affected family and their 
physicians.[82] In the U.S., most CJD cases--even the confirmed 
ones--seem to just fall through the cracks. In fact, based on the 
autopsy studies at Yale and elsewhere, it seems most CJD cases in the 
U.S. aren't even picked up in the first place.

Autopsy rates have dropped in the U.S. from 50% in the Sixties to 
less than 10% at present.[83] Although one reason autopsies are 
rarely performed on atypical dementia cases is that medical 
professionals are afraid of catching the disease,[84] the primary 
reason for the decline in autopsy rates in general appears to be 
financial. There is currently no direct reimbursement to doctors or 
hospitals for doing autopsies, which often forces the family to 
absorb the cost of transporting the body to an autopsy center and 
having the brain samples taken, a tab that can run upwards of 
$1500.[85]

Another problem is that the National Prion Disease Pathology 
Surveillance Center itself remains underfunded. Paul Brown, medical 
director for the National Institutes of Health, has described the 
Center's budget as "pitiful," complaining that "there isn't any 
budget for CJD surveillance."[86] To adequately survey America's 290 
million residents, "you need a lot of money." UK CJD expert Robert 
Will explains, "There was a CJD meeting of families in America in 
which... [the CDC] got attacked fairly vigorously because there 
wasn't proper surveillance. You could only do proper surveillance if 
you have adequate resources."[87] "I compare this to the early days 
of AIDS," says protein chemist Shu Chen, who directs the Center's 
lab, "when no one wanted to deal with the crisis."[88]

Andrew Kimbrell, the director of the Center for Food Safety, a 
D.C.-based public interest group, writes, "Given what we know now, it 
is unconscionable that the CDC is not strictly monitoring these 
diseases."[89] Given the presence of Mad Cow disease in the U.S., we 
need to immediately enact uniform active CJD surveillance on a 
national level, provide adequate funding not only for autopsies but 
also for the shipment of bodies, and require mandatory reporting of 
the disease in all 50 states. In Britain, even feline spongiform 
encephalopathy, the cat version of Mad Cow disease, is an officially 
notifiable illness. "No one has looked for CJD systematically in the 
U.S.," notes NIH medical director Paul Brown. "Ever."[90]

The animal agriculture industries continue to risk public safety, and 
the government seems to protect the industries' narrow business 
interests more than it protects its own citizens. Internal USDA 
documents retrieved through the Freedom of Information Act show that 
our government did indeed consider a number of precautionary measures 
as far back as 1991 to protect the American public from Mad Cow 
disease. According to one such document, however, the USDA explained 
that the "disadvantage" of these measures was that "the cost to the 
livestock and rendering industries would be substantial."[91]

Plant sources of protein for farm animals can cost up to 30% more 
than cattle remains.[92] The Cattlemen's Association admitted a 
decade ago that animal agribusiness could indeed find economically 
feasible alternatives to feeding slaughterhouse waste to other 
animals, but that the they did not want to set a precedent of being 
ruled by "activists."[93]

Is it a coincidence that USDA Secretary Veneman chose Dale Moore, 
former chief lobbyist for the National Cattlemen's Beef Association, 
as her chief of staff?[94] Or Alison Harrison, former director of 
public relations for the Cattlemen's Association, as her official 
spokeswoman?[95] Or that one of the new Mad Cow committee appointees 
is William Hueston, who was paid by the beef industry to testify 
against Oprah Winfrey in hopes of convicting her of beef 
"disparagement"?[96] After a similar conflict of interest unfolded in 
Britain, their entire Ministry of Agriculture was dissolved and an 
independent Food Safety Agency was created, whose sole responsibility 
is to protect the public's health. Until we learn from Britain's 
lesson, and until the USDA stops treating this as a PR problem to be 
managed instead of a serious global threat,[97] millions of Americans 
will remain at risk.


PLEASE FEEL FREE TO FORWARD OR REPRINT ANY PART OF THIS REPORT.

For updates on this evolving crisis, visit 
http://www.organicconsumers.org/madcow.htm or send a blank email to 
mailto:DrGregerMadCowUpdates-subscribe@lists.riseup.net

For background on this important issue, read the excellent book Mad 
Cow U.S.A., the full text of which is available free online at 
http://www.prwatch.org, or my article "U.S. Violates WHO Guidelines 
for Mad Cow Disease" at 
http://organicconsumers.org/madcow/GregerBSE.cfm.

Michael Greger, M.D., has been the Chief BSE Investigator for Farm 
Sanctuary since 1993 and the Mad Cow Coordinator for the Organic 
Consumers Association since 2001. Dr. Greger has debated the National 
Cattlemen's Beef Association before the FDA and was invited as an 
expert witness at the infamous Oprah Winfrey "meat defamation" trial. 
He has contributed to many books and articles on the subject, 
continues to lecture extensively, and currently runs the Mad Cow 
disease website http://www.organicconsumers.org/madcow.htm. Dr. 
Greger is a graduate of the Cornell University School of Agriculture 
and the Tufts University School of Medicine. He can be reached for 
media inquiries at (206) 312-8640 or mhg1@cornell.edu.

REFERENCES:
(Full text of specific articles available by emailing 
article-request@DrGreger.org)
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60 http://www.cdc.gov/mmwr/preview/mmwrhtml/00001820.htm
61 http://www.cdc.gov/nchs/fastats/alzheimr.htm
62 http://www.nimh.nih.gov/publicat/numbers.cfm
63 Neurology 34 (1984): 939.
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68 British Journal of Psychiatry 158 (1991): 457-70.
69 Mahendra, B. Dementia Lancaster: MTP Press Limited, 1987: 174.
70 Archives of Neurology 44 (1987): 24-29.
71 Neurology 38 (1989): 76-79.
72 http://www.cdc.gov/ncidod/diseases/cjd/bsecjdqa.htm
73 Dementia and Normal Aging, Cambridge University Press, 1994.
74 Neurology 55 (2000):1075.
75 Lancet Infectious Disease. 1 August 2003.
76 http://www.mad-cow.org/jan99_petition.html#ddd
77 http://www.centerforfoodsafety.org/li/CDCrspn1.html
78 Morbidity and Mortality Weekly Report 12 April 1996: 295-303.
79 Neurology 43 (1993): A316.
80 The Wall Street Journal. 30 November 2001.
81 Beacon Journal (Akron). 5 June 2001. 
http://www.organicconsumers.org/madcow/CJD6501.cfm
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83 
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84 Altman, Lawrence K. "Four States Watching for Brain Disorder." New 
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86 http://www.organicconsumers.org/madcow/fact43001.cfm
87 Case Western Reserve University Magazine - Summer 2001.
88 Case Western Reserve University Magazine - Summer 2001.
89 USA Today. 7 January 1999.
90 Philip Yam. The Pathological Protein: Mad Cow, Chronic Wasting, 
and Other Deadly Prion Diseases. New York: Springer-Verlag Press, 
2003.
91 Rampton, S and J. Stauber. Mad Cow USA: Could the Nightmare Happen 
Here? Common Courage Press; (September 1997):149-50. Full text 
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92 Food Chemical News 25 March 1996: 30.
93 Food Chemical News 5 July 1993: 57-59.
94 http://www.philly.com/mld/inquirer/5884855.htm
95 http://organicconsumers.org/madcow/usda1204.cfm
96 http://www.prwatch.org/prwissues/1998Q1/oprah.html
97 "World Health Organization says BSE is a major threat" 
http://www.organicconsumers.org/madcow/BSE7601.cfm


-- 
Michael Greger, M.D.
Chief BSE Investigator for Farm Sanctuary
	http://www.nodowners.org
Mad Cow Coordinator for the Organic Consumers Association
	http://www.organicconsumers.org/madcow.htm
(617) 524-8064
(206) 312-8640
mhg1@cornell.edu
185 South St #6
Boston, MA 02130

For periodic updates on the Mad Cow crisis send a blank email to 
mailto:DrGregerMadCowUpdates-subscribe@lists.riseup.net

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