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SEAC 101st meeting on Wednesday 15th October 2008 AGENDA

flounder

Well-known member
SEAC 101st meeting on Wednesday 15th October 2008 AGENDA

snip...

SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE Draft minutes of the 100th meeting held on 25th April 2008

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ITEM 3 - CURRENT ISSUES

9.

SEAC was informed about the following issues:

. Three cases of variant CJD (vCJD) had been identified in Spain: one each in 2005, 2007 and 2008, with the last two cases reported from the same geographical region. Media reports in Spain had suggested there could be up to five further cases. One of these five cases is a young individual with clinical symptoms of a relatively long duration that had been classified by the Spanish Registry as possible sporadic CJD (sCJD). Although it is possible that this case may be subsequently confirmed as vCJD there were good reasons, which could not be discussed at the present time, for thinking it was not. Four other cases were not considered to be vCJD by TSE experts in Spain. More information would be available as investigations progress.

http://www.seac.gov.uk/papers/101-1.pdf

3 10:10 Current issues

vCJD cluster in Spain Testing of a goat

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In the afternoon of the 15th of October 2008, SEAC will discuss preliminary research on tissues of the eye from a vCJD case and preliminary research on a new human prion disease in the United States of America (as reported by Gambetti et al. (2008) A novel human disease with abnormal prion protein sensitive to protease. Ann. Neurol. 63, 697-708). This part of the meeting will be held in closed session to allow discussion of preliminary unpublished data. This is in accordance with the SEAC Code of Practice.

* SEAC 101/4 and SEAC 101/5 and Annex 1 of SEAC 101/2 have not been provided as they contain either draft reports or unpublished data. This is in accordance with the SEAC Code of Practice.

http://www.seac.gov.uk/agenda/agen151008.htm

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SEAC 99th meeting on Friday 14th December 2007

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SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE Minutes of the 99th meeting held on 14th December 2007

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ITEM 8 - PUBLIC QUESTION AND ANSWER SESSION 40. The Chair explained that the purpose of the question and answer session was to give members of the public an opportunity to ask questions related to the work of SEAC. Mr Terry Singeltary (Texas, USA) had submitted a question prior to the meeting, asking: "With the Nor-98 now documented in five different states so far in the USA in 2007, and with the two atypical BSE H-base cases in Texas and Alabama, with both scrapie and chronic 14 © SEAC 2007 wasting disease (CWD) running rampant in the USA, is there any concern from SEAC with the rise of sporadic CJD in the USA from ''unknown phenotype'', and what concerns if any, in relations to blood donations, surgery, optical, and dental treatment, do you have with these unknown atypical phenotypes in both humans and animals in the USA? Does it concern SEAC, or is it of no concern to SEAC? Should it concern USA animal and human health officials?" 41.

A member considered that this question appeared to be primarily related to possible links between animal and human TSEs in the USA.

There is no evidence that sCJD is increasing in the USA and no evidence of any direct link between TSEs and CJD in the USA. Current evidence does not suggest that CWD is a significant risk to human health. There are unpublished data from a case of human TSE in the USA that are suggestive of an apparently novel form of prion disease with distinct molecular characteristics. However, it is unclear whether the case had been further characterised, if it could be linked to animal TSEs or if other similar cases had been found in the USA or elsewhere. In relation to the possible public health implications of atypical scrapie, H-type BSE and CWD, research was being conducted to investigate possible links and surveillance was in place to detect any changes in human TSEs. Although possible links between these diseases and human TSEs are of concern and require research, there is no evidence to suggest immediate public health action is warranted. The possible human health risks from classical scrapie had been discussed earlier in the meeting. Members noted that there are effective channels of discussion and collaboration on research between USA and European groups. Members agreed it is important to keep a watching brief on new developments on TSEs.

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http://www.seac.gov.uk/minutes/99.pdf

There is no evidence that sCJD is increasing in the USA and no evidence of any direct link between TSEs and CJD in the USA.

10 people killed by new CJD-like disease

Public release date: 9-Jul-2008 [ Print Article E-mail Article Close Window ]

Contact: Claire Bowles mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000076/!x-usc:mailto:[email protected] 44-207-611-1210 New Scientist

10 people killed by new CJD-like disease A NEW form of fatal dementia has been discovered in 16 Americans, 10 of whom have already died of the condition. It resembles Creutzfeldt-Jakob disease - with patients gradually losing their ability to think, speak and move - but has features that make it distinct from known forms of CJD.

No one yet knows how the disease originates, or under what conditions it might spread. Nor is it clear how many people have the condition. "I believe the disease has been around for many years, unnoticed," says Pierluigi Gambetti, director of the US National Prion Disease Pathology Surveillance Center at Case Western Reserve University in Cleveland, Ohio. Cases may previously have been mistaken for other forms of dementia.

Since Gambetti's team wrote a paper describing an initial 11 cases referred to his centre between 2002 and 2006 (Annals of Neurology, vol 63, p 697), another five have come to light. "So it is possible that it could be just the tip of the iceberg," Gambetti says.

snip... see full text ;

http://www.eurekalert.org/pub_releases/2008-07/ns-tpk070908.php

Thursday, July 10, 2008 A Novel Human Disease with Abnormal Prion Protein Sensitive to Protease update July 10, 2008 http://cjdmadcowbaseoct2007.blogspot.com/2008/07/novel-human-disease-with-abnormal-prion.html

Thursday, July 10, 2008 A New Prionopathy update July 10, 2008 http://cjdmadcowbaseoct2007.blogspot.com/2008/07/new-prionopathy-update-july-10-2008.html

Communicated by: Terry S. Singeltary Sr. <mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000076/!x-usc:mailto:[email protected]>

[In submitting these data, Terry S. Singeltary Sr. draws attention to the steady increase in the "type unknown" category, which, according to their definition, comprises cases in which vCJD could be excluded. The total of 26 cases for the current year (2007) is disturbing, possibly symptomatic of the circulation of novel agents. Characterization of these agents should be given a high priority. - Mod.CP]

http://pro-med.blogspot.com/2007/11/proahedr-prion-disease-update-2007-07.html

http://www.promedmail.org/pls/askus/f?p=2400:1001:6833194127530602005::NO::F2400_P1001_BACK_PAGE,F2400_P1001_PUB_MAIL_ID:1010,39963

There is a growing number of human CJD cases, and they were presented last week in San Francisco by Luigi Gambatti(?) from his CJD surveillance collection.

He estimates that it may be up to 14 or 15 persons which display selectively SPRPSC and practically no detected RPRPSC proteins.

http://www.fda.gov/ohrms/dockets/ac/06/transcripts/1006-4240t1.htm

http://www.fda.gov/ohrms/dockets/ac/06/transcripts/2006-4240t1.pdf


http://seac992007.blogspot.com/

snip...

http://www.seac.gov.uk/minutes/99.pdf

TSS

SEAC 101st meeting on Wednesday 15th October 2008 AGENDA

http://seac992007.blogspot.com/2008/10/seac-101st-meeting-on-wednesday-15th.html


Wednesday, October 08, 2008

Idiopathic Brainstem Neuronal Chromatolysis (IBNC): a novel prion protein related disorder of cattle?

http://bse-atypical.blogspot.com/2008/10/idiopathic-brainstem-neuronal.html



Tuesday, August 19, 2008

Atypical BSE (BASE) Transmitted from Asymptomatic Aging Cattle to a Primate

http://bse-atypical.blogspot.com/2008/08/atypical-bse-base-transmitted-from.html




Wednesday, October 08, 2008

CJD FOUNDATION UPDATE

http://creutzfeldt-jakob-disease.blogspot.com/2008/10/cjd-foundation-update.html



2008

The statistical incidence of CJD cases in the United States has been revised to reflect that there is one case per 9000 in adults age 55 and older. Eighty-five percent of the cases are sporadic, meaning there is no known cause at present.

http://www.cjdfoundation.org/fact.html



TSS
 
A

Anonymous

Guest
flounder- glad to see you Terry-- not that I missed your posts- but was worried about YOU when you haven't posted on any of the sites for sometime...
I hope it was just that info and news was on short supply and nothing was wrong with you...
 

flounder

Well-known member
Oldtimer said:
flounder- glad to see you Terry-- not that I missed your posts- but was worried about YOU when you haven't posted on any of the sites for sometime...
I hope it was just that info and news was on short supply and nothing was wrong with you...


hello there OT !

IKE hit us hard. the calm Galveston bay (which is our backyard), became very angry the night of sept 12. we lost power about 7 pm, the water rose about 17 to 18 feet, which put two feet in the main house here, then add about a 4 foot chop. the garage, and garage apartment slammed in the back of the house, broke a window through the plywood, and then the main house floated off a few blocks and piers, about this time the wife was a hollering at me, she's gonna kill me if she survives IKE. water was coming in fast about 2 hours before 1st eye wall hit, we were trying to shuffle generator and about 50 gallons of gas in the house. then decided to prepare for the worse, float out the attic. had a couple of rafts ready, but thank GOD for the eyewall, and then the winds shifted, blew the big waves and surge the other direction...... walked outback about 2AM, the eye had reached us finally. longest eye of a hurricane i had ever experienced. an hour and a half or more. i walked outback, and could not get there for the debris in the driveway, had to go around and through an empty lot next door. and the serenity of it all, everything outback gone, it all acted as a barrier for the main house though. but at about 2 AM i peered out over the bluff (what use to be about 17 feet above sea level as a child, but now only about 14 feet), i stand at the bluff, and i was standing in water, not rain water, but bay water, and the bay was calm. you could hear a faint roar in the background. the destruction from water is absolute. i thought 125 to 150 mile an hour winds were bad, but the destruction of water is awesome. you could hear the cracking and popping of wood. both cars totaled, garage gone, my mother-in-law's shack gone, main house badly damaged, and FEMA says to us, ''this is NOT another %ucking bush give away'', and that's a quote from a FEMA person. so, the 3 + years of katrina hand outs, has turned into nothing for most IKE victims. had flood insurance, windstorm, and homeowners, and all it has become, is a big circle jerk. please note, i am in the 100 to 500 year flood zone i.e. C. my suggestion for everyone with windstorm, flood insurance. drop them. after cost depreciation (this is where the insurance screws you again after the original screwing) and what they said the would pay for, and what they actually pay for, well, i suggest everyone read, read, and read some more, all the balls are in their courts, and it's in fine print. example, garage collapses on cars. all 4 walls (including two garage doors side) broke away, in pieces. slab cracks and buckles. garage apartment, behind garage, not covered, was a detach (but was covered in homeowners). garage, only covered for 15 thousand dollars, and they want to raise the roof, keep it (has old shingles on it, but roof not damaged bad), and build four walls around the buckled and cracked slab, and then drop the old roof on it. every contractor i had look at it said they would not even try. walked away. i had the damn thing bulldozed. screw them. was without power for over 2 weeks. got some good photos', will post to blog later. anyway there OT, there you have it, and thanks for your concern, were safe, we are blessed, and i guess a proper word for you old friend would be,

i'm still hear damn't, i'm still here ;-)


the kemah boardwalk got hit hard. you can see it from our backyard, just down the shoreline ;


http://www.click2houston.com/slideshow/news/17474487/detail.html

here is our sister fishing community to the south here on bayshore dr., about 2 miles down the road or so, where the beer is always cold, and where Dusty Hill of ZZ top home is just down the shoreline of our little homestead, you have the sunny San Leon, where they got hit hard. Bacliff is a bit higher in elevation. the seabreeze newspaper has a good timeline. my friend steve and others lost there whole house down there ;

http://seabreezenews.com/issue/Page_19.pdf


obituary of fine bars, and eateries ;


http://seabreezenews.com/issue/Page_15.pdf


Front page

http://seabreezenews.com/issue/Page_01.pdf


http://seabreezenews.com/issue/



more photo's of this area destruction from IKE. ...

http://www.boston.com/bigpicture/2008/09/the_short_but_eventful_life_of.html?s_campaign=8315

Residents dig out from storm

By Chris Paschenko The Daily News Published September 16, 2008

BACLIFF - A wall of water not seen in generations of storms smashed through dozens of homes lining Galveston Bay, and residents spent Tuesday clearing debris and digging out the mud and the muck.

Terry Singeltary refused to evacuate his Bayshore Drive home in Bacliff, saying he feared looters more than Hurricane Ike.

"My grandparents bought this place in 1935, and I wound up with it," Singeltary said. "It made it through a storm in '48, and (hurricanes) Carla and Alicia. I've never seen the water this high."

The Singeltary home survived with water damage in his game room. Singeltary never needed his backup plan: a kayak or an axe stashed in the attic. ...snip

http://galvestondailynews.com/story.lasso?ewcd=276365f800b0da1c


216. vortfix 9:25 PM GMT on September 15, 2008

Galveston warns of 'downward spiral'

Mobile morgue en route to Galveston as search continues

Five dead, but thousands of homes still to be searched

Three unrelated deaths from apparent carbon monoxide poisoning from generator use have brought the local death toll from Hurricane Ike to at least 14, local officials said.

The Harris County Medical Examiner's office reports that three people, a 4-year-old boy and two men, ages 18 and 34, died when generators were being used without proper ventilation. The three are unrelated and their identities are not being released.

The storm and its fallout are also believed responsible for at least five deaths in Galveston County, among the hardest hit areas, and several others in Montogmery, Chambers and Walker counties from fires and fallen trees.

As rescue officials continued searching for victims across the coast, the storm also has resulted in mass closing at area school districts - some of which have suspended classes until further notice.

In addition to the Houston ISD - which announced today that schools likely will be closed for a week to 10 days because of lack of electricity and minor damage to some campuses - a number of other districts say they are postponing classes until further notice.

Those include the Pearland, Goose Creek, Hitchcock, and Pasadena school districts. Texas City schools will be closed until post-hurricane assessments are finished, and Episcopal High School in Houston is closed until Sept. 22.

School officials will confer with the Texas Education Agency to see how many school days missed because of the hurricane may need to be made up.

In other developments, oil company inspections of damage at the Houston Ship Channel refinery complex the nation's largest continued today as President Bush expressed concern about Hurricane Ike's impact on the nation's fuel supply.

Bush, who plans to visit Houston on Tuesday, told reporters he was concerned about "upward pressure" on prices proving a hardship to consumers.

The AAA auto club today said gasoline in Texas has increased an average 13 cents per gallon since Friday. Nationally, the cost has risen by 10 percent, on average.

ExxonMobil reported that its Baytown and Beaumont refinery suffered widespread, but not significant, damage from the hurricane. The world's largest oil company is importing gasoline from refineries in Europe, Asia and the Middle East to supplement supplies.

Shell Oil Co. today said flyover examinations of its offshore platforms or drilling rigs has revealed no major structural damage. Marathon Oil's Texas City refinery remains without electrical power.

Diamond Offshore Drilling today reported that its offshore rigs stayed in place during the hurricane, but at least one jack-up rig lost all its drilling equipment.

On the local level, many Houston-area service stations remained without power today. Those that do have electricity to run their pumps were the sites of blocks-long lines of customers growing desperate for fuel.

State Sen. Rodney Ellis said he is investigating, at Mayor Bill White's request, the possibility of bringing gasoline trucks into Houston neighborhoods.

Ellis expressed concerns about the safety of such a plan, however.

"We are no longer in the eye of the storm," he said, "but in the eye of the aftermath, which in many ways can be more devastating."

Meanwhile, an estimated 10,000 people flocked to a food distribution site at a north Houston church this morning as emergency officials opened six such centers around the Houston area and prepared to open 11 more by day's end.

The opening of the sites, where food, ice and bottled water are being distributed, apparently defused a potential conflict between the city and the Federal Emergency Management Agency.

In addition to FEMA supplies, the centers distributed 200,000 pounds of ice donated by H-E-B supermarkets.

The line of residents awaiting assistance stretched two blocks at the Greenspoint distribution point, the Harvest Time Church at 17770 Imperial Valley. Authorities said the first client arrived at the site at 3:30 a.m., hours before the church opened its doors.

The center briefly ran out of food a few hours after opening, but more trucks soon arrived and the food distribution resumed.

At Conroe's distribution point, the Grand Theatre, the process of handing out essentials seemingly went smoothly. Ray Alewin, 61, of Seven Coves waited in line only 10 minutes.

"Ice is the big commodity," he said. "If you get ice, you can make it."

In La Porte, cars began lining up at the distribution center, New Life Christian Fellowship, early today, stretching about a half-mile on Underwood, then an additional half-mile down Spencer Highway.

While most recipients were grateful, consternation arose when some cars containing multiple families finally arrived at the head of the line. Only then did they learn that each vehicle was limited to two bags of ice, one case of bottled water and a single 12-pack of ready-to-eat meals.

"The rules are in place to make sure everybody gets at least something," said church pastor Sean Mooney. "It's not the church's limit; it's the FEMA rule. They don't want people hoarding up and then taking it out and selling it. This makes the distribution more equal."

Homeland Security Secretary Michael Chertoff pledged on Sunday that his team was "working feverishly" to get the centers open, even as Mayor Bill White expressed concern over how long the process was taking.

FEMA officials responded that setting up the centers was contingent on assurances that workers would be on hand to staff them.

This morning, Houstonians Carlos Torres, 62, and his wife, Zenaida Carrizales, waited in the Greenspoint line for 90 minutes the thought of the wilting food in their powerless refrigerator foremost in their minds.

Torres said the couple had searched the city in vain for a bag of ice.

"Beggars can't be choosers," he said as he eyed the line snaking around the church. "I just hope they'll have enough for everyone."

Torres and his wife, and thousands of others standing in FEMA lines, had the blessing of waiting under sunny, azure skies. Thanks to a cold front that arrived Sunday, temperatures this morning were in the low 70s.

As FEMA sought to help residents, some survivors complained of delays when they called for help rebuilding their homes and lives are advised to call the agency 1-800-621-FEMA. When they do, several said, they get a recorded message and then, abruptly, a dial tone.

The recorded message says FEMA personnel are busy helping others, which is, no doubt, true. After advising callers to contact the agency via the Internet, it says: ``We apologize for this inconvenience. Your call will now be disconnected.''

``Why don't they let you hold on a little bit?,'' asked Roger Boneno of Sugar Land, a diabetic who needs ice to refrigerate his insulin.

Why not do as the computer help lines do - warn that it might be a long wait but advise the caller to hold on, Boneno wondered. ``They disconnect you. It's right away. It's immediate. They want you to use their Web site - which would be neat if I could,'' he said. Indeed, callers are advised twice in the short message to contact the agency via the Internet. ``But we don't have the Internet if we don't have power,'' Boneno said.

Terry Singeltary of Bacliff, whose home, along with his neighbors,' lost its rear walls to the storm, wondered Monday when help would arrive. ``I've seen two police cars since Thursday,'' said Singeltary, who is disabled by a neck injury. ``I've seen helicopters flying over, but nobody is coming in here. And I can't get ahold of FEMA. That's a joke. ``I did get ahold of my insurance people,'' he added. ``They've been pretty good so far.''

Although the Texas Gulf Coast is strewn with wreckage in Hurricane Ike's wake, there were a few bright signs today that life is haltingly, incrementally moving toward normal. Commercial flights resumed today at Bush Intercontinental Airport with the 5:56 a.m. arrival of a Continental flight from Seattle. Hobby Airport should begin commercial flights on Tuesday. The city's admonition to boil tap water before drinking it expired this afternoon. No evidence of contamination has been found. The Metropolitan Transit Authority resumed operation of some high-priority bus routes. Regular Houston garbage collection began today and Fort Bend County with the possible exception of Sugar Land is planning trash pickup Tuesday of trees, shrubs and other debris. The city's 311 service line this morning was back in operation, with at least 40 lines up and running. All freeways to downtown Houston were reported open this morning, although the Interstate 10 Smith Street exit and the Interstate 45 Milam Street exit to the city's center remained closed. In a city still largely shuttered, it was apparent which businesses had returned to life. Lines of customers stretched from front doors to around the block at a League City Wal-Mart, a Hobby Airport Shipley's Doughnuts and a Gulfgate H-E-B supermarket. A similar scene was apparent at service stations that had fuel and electricity to dispense it. That was the good news. Good news came today, too, from the federal government, which, the Associated Press reported, will offer a three-month moratorium on mortgage foreclosures that are insured by the Federal Housing Authority to people living in disaster areas. Additionally, federally guaranteed loans will be provided state and local governments for rehabilitate housing, economic development and infrastructure repair. Still, the situation for coastal residents in southeast Texas and western Louisiana remained bleak. The storm seemingly spared no one, with at least seven fatalities in Texas so far, floodwaters, fallen trees, and shortages of power, water and ice affecting every community from Galveston to Beaumont, Houston to Surfside, The Woodlands to Pearland. Ike also destroyed a number of production platforms and damaged pipelines in the Gulf of Mexico, exasperating a rebuilding effort that is expected to top $10 billion. The death toll includes at least five in the battered barrier island city of Galveston. With the hurricane transforming the popular destination into a debris-covered ghost town, Mayor Lyda Ann Thomas warned residents to stay away, possibly for weeks, until the most pressing services are restored. In Houston, Mayor Bill White on Sunday ordered a weeklong curfew from 9 p.m. to 6 a.m. because of the widespread damage and darkness. White today reported that Houston crime levels are below normal, although fires have been more frequent. "The level of vigilance has been high," he said. White said Houston residents whose homes are uninhabitable can find shelter at the George R. Brown Convention Center. He asked that the shelter cots be saved for those with severe hardship, not those merely made uncomfortable by power outages. For the first time this morning rescuers flew into the heart of the coastal devastation. State helicopters flew 115 rescuers into Bolivar Peninsula, across Bolivar Roads from Galveston Island's eastern tip, to find entire subdivisions obliterated. "They had a lot of devastation over there," task force leader Chuck Jones told the Associated Press. "It took a direct hit." Jones said his crew would attempt to reach hard-hit Galveston Island tonight, but was prepared to remain on Bolivar.

National death toll at least 30

At least 30 people have died as a result of the hurricane, but most of the fatalities have occurred elsewhere in the United States as the storm followed a northeasterly track across the nation. This morning, the remnants of the storm were near Rochester, N.Y., where they were generating winds of 30 mph. By this afternoon, the National Weather Service had dropped its regular postings regarding the storm's location. In the Houston area, deaths so far included a 72-year-old Galveston man who died in his truck after trying to flee the island late Friday. A woman died in her bed early Saturday when a tree fell on her home in Pinehurst. And a 4-year-old Houston boy and two adults in his home died of carbon monoxide poisoning from the generator his family was using for power. Despite repeated warnings from authorities regarding the hazards of improper use of gasoline-powered generators, storm victims continue to fall ill with carbon monoxide poisoning. Three members of a Sugar Land family this morning were hospitalized from exposure to the deadly gas. Meanwhile, the search-and-rescue effort was the largest in the state's history, with more than 50 helicopters, 1,500 searchers and teams from federal, state and local agencies. Authorities said that 1,984 people had been rescued, including 394 by air, as of Sunday afternoon. The state opened more than 250 shelters for more than 37,000 evacuees, and there were plans to evacuate up to 10,500 residents who had stayed in Galveston.

Setting priorities

With the area gripped by a massive power outage, millions of Houstonians got creative and, in some cases, cranky as they craved fresh coffee, cold air and their Sunday football fix.

Some took to their lawns to escape sauna-like heat, while others huddled in the homes of those lucky enough to have working lights, air conditioning and television. Grocery stores, pharmacies and a few restaurants with restored power or generators drew long lines of customers.

CenterPoint said it had restored power to 500,000 customers, but about 1.6 million were still without power. Entergy said 99 percent of their customers still were without power, and Texas New Mexico Power said 64 percent were without electricity.

This report was compiled by Chronicle reporters Allan Turner and Matt Stiles and includes contributions by reporters Bill Murphy, Mark Carreau, Rad Sallee, Lynn Cook, Carolyn Feibel, Mary Flood, Mike Glenn, Roma Khanna, Ericka Mellon, Bradley Olson, Liz Austin Peterson, Ruth Rendon, Bennett Roth, Dane Schiller and Jennifer Latson. The Associated Press also contributed

Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texas USA 77518
 
A

Anonymous

Guest
Thats the reason Grandma will never get to retire on the ocean- I prefer October blizzards over hurricanes... :wink:

Sorry about all the damage- but glad to see you survived...That does look like a mess...And I know what your going thru with the Feds- and the insurance as I've ended up in court a couple of times on insurance companies that wouldn't pay out and found fine print or whatever...Luckily it wasn't for me- but for neighbors that were damaged in spring floods...

Good Luck- looks like you have a task ahead of you...
 

flounder

Well-known member
SEAC

ONE HUNDRED AND FIRST MEETING OF THE SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE

The Spongiform Encephalopathy Advisory Committee held its 101st meeting in London on 15th October 2008, and discussed the following:

CURRENT ISSUES SEAC was informed about:

• A mother and son in Spain who had died of variant Creutzfeldt-Jakob Disease (vCJD). This is the first recorded instance of more than one case of vCJD within one family. Both the mother and son lived in a region of Spain with a history of BSE and had frequently shared meals of cattle brain. As no other risk factor has been identified, it seems most likely that both infections were acquired from dietary exposure.

• Results of tests on a single goat from a culled UK dairy herd with a large classical scrapie outbreak. On the basis of the results the presence of Bovine Spongiform Encephalopathy (BSE) cannot be excluded. Further testing by mouse bioassays, which may take at least two, if not more, years to complete, is required to make a definitive diagnosis.

UPDATE ON vCJD PREVALENCE STUDIES

SEAC was updated by the Health Protection Agency (HPA) about the progress of the National Anonymous Tonsil Archive (NATA), a proposed second retrospective survey of 30 000 stored appendix samples and a proposed post mortem tissue archive. These studies would provide data to estimate the prevalence of subclinical vCJD (vCJD infections that have yet to develop, or may never develop, into clinical disease).

Around 62 500 tonsil samples collected by NATA have been tested with no positive samples found. An application for the second retrospective survey of appendix samples is currently under consideration by a Research Ethics Committee. SEAC learned that the establishment of a post mortem tissue archive, which is dependent on the collection of samples from Coroners’ autopsies, does not have the support of Coroners needed to take it forward. SEAC is extremely disappointed about the lack of support from Coroners for the post mortem tissue archive. As SEAC has repeatedly stated, the archive is key to obtaining better estimates of the prevalence of subclinical vCJD. These estimates are vital to make meaningful assessments of the risks to public health from vCJD and of the effectiveness of current, and the need for further, very costly public health protection measures. SEAC acknowledged the strenuous efforts made by the HPA, the Department of Health (DH) and National Health Service Blood and Tissue to devise a system to collect samples that would have the least impact on the work of Coroners. SEAC remains strongly in favour of establishing the archive.

snip...

PROTEASE SENSITIVE PRIONOPATHY

SEAC discussed with Dr Pierluigi Gambetti (US National Prion Disease Pathology Surveillance Center) his recently published report5 on the identification in the United States of America of a new human prion disease. SEAC agreed that there is considerable work to be done to characterise fully this new disease, its cause and whether it is infectious or not. As preliminary unpublished data were also presented, this issue was discussed in a reserved business session in accordance with the SEAC Code of Practice.

RESULTS ON HUMAN SCLERA

SEAC considered preliminary results provided by the HPA and National CJD Surveillance Unit from tests on eye tissue (sclera) from a vCJD case. The results suggest the presence of infectivity and, in contrast with previous testing of samples from other vCJD cases, abnormal prion protein in this tissue. However, as the sclera is very difficult to remove from surrounding eye tissues, which are themselves known to carry vCJD infectivity, the findings may have arisen as a result of contamination at autopsy. Nevertheless, even if the data are reliable, they indicate that there may only be a relatively low level of infectivity present in sclera. As preliminary unpublished data were considered, this issue was discussed in a reserved business session in accordance with the SEAC Code of Practice.

5 Gambetti et al. (2008) A novel human disease with abnormal prion protein sensitive to protease. Ann. Neurol. 63, 697-708. 5 © SEAC 2008


SEE FULL TEXT ;

http://www.seac.gov.uk/papers/101-summary.pdf


http://www.mad-cow.org/dec99_news.html#bbb

http://creutzfeldt-jakob-disease.blogspot.com/2008/01/cjd-hgh-body-snatchers.html


SEAC 101st meeting on Wednesday 15th October 2008 AGENDA

http://seac992007.blogspot.com/2008/10/seac-101st-meeting-on-wednesday-15th.html

A New Prionopathy OR more of the same old BSe and sporadic CJD

http://creutzfeldt-jakob-disease.blogspot.com/2008/08/new-prionopathy-or-more-of-same-old-bse.html

Communicated by: Terry S. Singeltary Sr.

[In submitting these data, Terry S. Singeltary Sr. draws attention to the steady increase in the "type unknown" category, which, according to their definition, comprises cases in which vCJD could be excluded. The total of 26 cases for the current year (2007) is disturbing, possibly symptomatic of the circulation of novel agents. Characterization of these agents should be given a high priority. - Mod.CP]

http://pro-med.blogspot.com/2007/11/proahedr-prion-disease-update-2007-07.html

http://www.promedmail.org/pls/askus/f?p=2400:1001:6833194127530602005::NO::F2400_P1001_BACK_PAGE,F2400_P1001_PUB_MAIL_ID:1010,39963

There is a growing number of human CJD cases, and they were presented last week in San Francisco by Luigi Gambatti(?) from his CJD surveillance collection.

He estimates that it may be up to 14 or 15 persons which display selectively SPRPSC and practically no detected RPRPSC proteins.

http://www.fda.gov/ohrms/dockets/ac/06/transcripts/1006-4240t1.htm

http://www.fda.gov/ohrms/dockets/ac/06/transcripts/2006-4240t1.pdf

sporadic Fatal Familial Insomnia

http://sporadicffi.blogspot.com/

JOURNAL OF NEUROLOGY

MARCH 26, 2003

RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob

disease in the United States

Email Terry S. Singeltary:

[email protected]

I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to comment on the CDC's attempts to monitor the occurrence of emerging forms of CJD. Asante, Collinge et al [1] have reported that BSE transmission to the 129-methionine genotype can lead to an alternate phenotype that is indistinguishable from type 2 PrPSc, the commonest sporadic CJD. However, CJD and all human TSEs are not reportable nationally. CJD and all human TSEs must be made reportable in every state and internationally. I hope that the CDC does not continue to expect us to still believe that the 85%+ of all CJD cases which are sporadic are all spontaneous, without route/source. We have many TSEs in the USA in both animal and man. CWD in deer/elk is spreading rapidly and CWD does transmit to mink, ferret, cattle, and squirrel monkey by intracerebral inoculation. With the known incubation periods in other TSEs, oral transmission studies of CWD may take much longer. Every victim/family of CJD/TSEs should be asked about route and source of this agent. To prolong this will only spread the agent and needlessly expose others. In light of the findings of Asante and Collinge et al, there should be drastic measures to safeguard the medical and surgical arena from sporadic CJDs and all human TSEs. I only ponder how many sporadic CJDs in the USA are type 2 PrPSc?

http://www.neurology.org/cgi/eletters/60/2/176#535

THE PATHOLOGICAL PROTEIN

Hardcover, 304 pages plus photos and illustrations. ISBN 0-387-95508-9

June 2003

BY Philip Yam

CHAPTER 14 LAYING ODDS

Answering critics like Terry Singeltary, who feels that the U.S. under- counts CJD, Schonberger conceded that the current surveillance system has errors but stated that most of the errors will be confined to the older population.

http://www.thepathologicalprotein.com/

Diagnosis and Reporting of Creutzfeldt-Jakob Disease Singeltary, Sr et al. JAMA.2001; 285: 733-734. Vol. 285 No. 6, February 14, 2001 JAMA

Diagnosis and Reporting of Creutzfeldt-Jakob Disease

To the Editor: In their Research Letter, Dr Gibbons and colleagues1 reported that the annual US death rate due to Creutzfeldt-Jakob disease (CJD) has been stable since 1985. These estimates, however, are based only on reported cases, and do not include misdiagnosed or preclinical cases. It seems to me that misdiagnosis alone would drastically change these figures. An unknown number of persons with a diagnosis of Alzheimer disease in fact may have CJD, although only a small number of these patients receive the postmortem examination necessary to make this diagnosis. Furthermore, only a few states have made CJD reportable. Human and animal transmissible spongiform encephalopathies should be reportable nationwide and internationally.

Terry S. Singeltary, Sr Bacliff, Tex

1. Gibbons RV, Holman RC, Belay ED, Schonberger LB. Creutzfeldt-Jakob disease in the United States: 1979-1998. JAMA. 2000;284:2322-2323. FREE FULL TEXT

http://jama.ama-assn.org/cgi/content/extract/285/6/733?maxtoshow=&HITS=10&hits=10&RESULTFORMAT=&fulltext=singeltary&searchid=1&FIRSTINDEX=0&resourcetype=HWCIT

http://jama.ama-assn.org/cgi/content/full/285/6/733?maxtoshow=&HITS=10&hits=10&RESULTFORMAT=&fulltext=singeltary&searchid=1&FIRSTINDEX=0&resourcetype=HWCIT

2 January 2000 British Medical Journal U.S. Scientist should be concerned with a CJD epidemic in the U.S., as well

http://www.bmj.com/cgi/eletters/320/7226/8/b#6117

15 November 1999 British Medical Journal vCJD in the USA * BSE in U.S.

http://www.bmj.com/cgi/eletters/319/7220/1312/b#5406

Creutzfeldt Jakob Disease

http://creutzfeldt-jakob-disease.blogspot.com/

http://creutzfeldt-jakob-disease.blogspot.com/2008/01/cjd-hgh-body-snatchers.html

http://creutzfeldt-jakob-disease.blogspot.com/2008/01/risk-factors-for-sporadic-creutzfeldt.html

http://creutzfeldt-jakob-disease.blogspot.com/2008/01/creutzfeldt-jakob-disease-prion-protein.html

http://creutzfeldt-jakob-disease.blogspot.com/2007/12/risk-assessment-of-transmission-of.html

http://creutzfeldt-jakob-disease.blogspot.com/2007/12/risk-factors-for-sporadic-creutzfeldt.html

http://creutzfeldt-jakob-disease.blogspot.com/2006/11/on-question-of-sporadic-or-atypical.html

USA PRION UNIT BLOG

http://prionunitusaupdate2008.blogspot.com/

Sunday, April 20, 2008 Progress Report from the National Prion Disease Pathology Surveillance Center April 3, 2008

Atypical forms of BSE have emerged which, although rare, appear to be more virulent than the classical BSE that causes vCJD.

see full text ;

http://prionunitusaupdate2008.blogspot.com/2008/04/progress-report-from-national-prion.html

CJD TEXAS (cjd clusters)

http://cjdtexas.blogspot.com/

USA WRITTEN CJD QUESTIONNAIRE ???

http://cjdquestionnaire.blogspot.com/

The statistical incidence of CJD cases in the United States has been revised to reflect that there is one case per 9000 in adults age 55 and older. Eighty-five percent of the cases are sporadic, meaning there is no known cause at present.

http://www.cjdfoundation.org/fact.html

Tuesday, August 19, 2008

Atypical BSE (BASE) Transmitted from Asymptomatic Aging Cattle to a Primate

http://bse-atypical.blogspot.com/2008/08/atypical-bse-base-transmitted-from.html

Review on the epidemiology and dynamics of BSE epidemics

Vet. Res. (2008) 39:15 www.vetres.org DOI: 10.1051/vetres:2007053 c INRA, EDP Sciences, 2008 Review article

snip...

And last but not least, similarities of PrPres between Htype BSE and human prion diseases like CJD or GSS have been put forward [10], as well as between L-type BSE and CJD [17]. These findings raise questions about the origin and inter species transmission of these prion diseases that were discovered through the BSE active surveillance.

snip...

Cases of atypical BSE have only been found in countries having implemented large active surveillance programs. As of 1st September 2007, 36 cases (16 H, 20 L) have been described all over the world in cattle: Belgium (1 L) [23], Canada (1 H)15, Denmark (1 L)16, France (8 H, 6 L)17, Germany (1 H, 1 L) [13], Italy (3 L)18, Japan (1 L) [71], Netherlands (1 H, 2 L)19, Poland (1 H, 6 L)20, Sweden (1 H)21, United Kingdom (1 H)22, and USA (2 H)23. Another H-type case has been found in a 19 year old miniature zebu in a zoological park in Switzerland [56]. It is noteworthy that atypical cases have been found in countries that did not experience classical BSE so far, like Sweden, or in which only few cases of classical BSE have been found, like Canada or the USA.

And last but not least, similarities of PrPres between Htype BSE and human prion diseases like CJD or GSS have been put forward [10], as well as between L-type BSE and CJD [17]. These findings raise questions about the origin and inter species transmission of these prion diseases that were discovered through the BSE active surveillance.

full text 18 pages ;

http://www.vetres.org/index.php?option=article&access=standard&Itemid=129&url=/articles/vetres/pdf/2008/04/v07232.pdf

please see full text ;

http://bse-atypical.blogspot.com/2008/06/review-on-epidemiology-and-dynamics-of.html

***Atypical forms of BSE have emerged which, although rare, appear to be more virulent than the classical BSE that causes vCJD.***

Progress Report from the National Prion Disease Pathology Surveillance Center

An Update from Stephen M. Sergay, MB, BCh & Pierluigi Gambetti, MD

April 3, 2008

http://www.aan.com/news/?event=read&article_id=4397&page=72.45.45

Sunday, March 16, 2008

MAD COW DISEASE terminology UK c-BSE (typical), atypical BSE H or L, and or Italian L-BASE

http://bse-atypical.blogspot.com/2008/03/mad-cow-disease-terminology-uk-c-bse.html

Wednesday, October 08, 2008

Idiopathic Brainstem Neuronal Chromatolysis (IBNC): a novel prion protein related disorder of cattle?

http://bse-atypical.blogspot.com/2008/10/idiopathic-brainstem-neuronal.html

HUMAN and ANIMAL TSE Classifications i.e. mad cow disease and the UKBSEnvCJD only theory JUNE 2008

snip...

Tissue infectivity and strain typing of the many variants of the human and animal TSEs are paramount in all variants of all TSE. There must be a proper classification that will differentiate between all these human TSE in order to do this. With the CDI and other more sensitive testing coming about, I only hope that my proposal will some day be taken seriously. ...

snip...

http://cjdmadcowbaseoct2007.blogspot.com/2008/06/human-and-animal-tse-classifications-ie.html


TSS


ONE HUNDRED AND FIRST MEETING OF THE SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE
Oct 23, 2008 at 9:00 AM
http://seac992007.blogspot.com/2008/10/one-hundred-and-first-meeting-of_23.html

http://flounder068.vox.com/library/post/one-hundred-and-first-meeting-of-the-spongiform-encephalopathy-advisory-committee.html
 

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