TEXAS NUCLEAR DUMP VOTE SET AMID HOLIDAY RUSH THANKS TO GOVERNOR RICK PERRY

NUCLEAR DUMP VOTE SET AMID HOLIDAY RUSH

CRITICS PROTEST TIMING OF MEETING ON 38-STATE WASTE FACILITY IN TEXAS

AUSTIN — In a move decried by environmentalists, state regulators decided late Thursday to schedule a vote that could allow three dozen states to dump radioactive waste in Texas.

The Texas Compact Commission set a Jan. 4 meeting to decide whether to expand how much low-level radioactive waste could be processed at a dump in remote Andrews County, in far West Texas. The announcement came as environmentalists and critics on the commission accused regulators and a politically connected company of rushing the proposal past Texans who are too focused on the holidays to even notice.

The public comment period on the proposal ends Sunday. The timing could also be designed to get support from two commissioners from Vermont, which has an agreement to deposit waste at the facility.

"It's too much, too soon, too fast," said Bob Gregory, a commissioner who opposes the expansion plan. "This whole thing is absurd. Why are we having a comment period at midnight on the day after Christmas?"

Michael Ford, chairman of the commission, said the rule has been under review for more than a year.

"We've been working on this rule for 16 months," Ford said. "That does not meet my definition of rushed."

Waste Control Specialists, whose majority owner is Dallas billionaire and political donor Harold Simmons, wants state regulators to approve its proposal to allow low-level radioactive waste at its West Texas dump site from three dozen states. As it stands, the compact site can only get waste from Texas, Vermont and the federal government.

The incoming governor of Vermont, Democrat Peter Shumlin, has criticized the expansion proposal. But he doesn't take office until Jan. 6. By then, the two commissioners appointed by his predecessor could vote in favor of it.

Texas environmental activist Karen Hadden, director of the Texas SEED Coalition, said she was "appalled" by the timing of the meeting and warned it could make Texas a dumping ground for low-level radioactive waste.

"This is an evil Christmas present from WCS and Harold Simmons," added Tom "Smitty" Smith, director of the environmental group Public Citizen of Texas.

After a news conference Thursday, Smith and other environmentalists rolled a black barrel, with a radioactive symbol on the outside, under the state Christmas tree outside the Texas Capitol.

Chuck McDonald, a spokesman for WCS, said the process has been anything but rushed. He said the same opponents who are attacking the idea are responsible for delaying action until now.

"It is a true misnomer to say this has been rushed through in the holiday season," McDonald said.

8-member commission The decision on whether to allow the importation of waste from other states rests with the eight-member Texas commission, made up of appointees by the governors of Texas and Vermont. Those states have a compact that allows both states to bury nuclear waste at the Andrews County site.

In the early 1980s, the federal government started urging states to build low-level nuclear waste landfills, either on their own or in cooperation with other states.

Should the commission adopt the rules, low-level radioactive waste from 36 other states could also petition the group to allow their waste to be dumped at the facility.

The dump, now sized at 2.3 million cubic yards, would be one of a few in the nation that could take low-level waste, such as clothing, metal and other materials from nuclear power plants, hospitals and university labs.

Locals are split.

In 2009, voters in Andrew County narrowly approved - 642-639- a $75 million bond to pay for the construction of the dump by WCS, which would pay the county a share of its gross receipts.

For Tim Gannaway, who was born and raised in the city of Andrews but moved in 2007 to attend the University of Texas at San Antonio, the decision to bring radioactive waste to an area served only by a volunteer fire department is foolhardy.

He also doesn't like the idea of his home county becoming a national repository for dangerous waste.

"Andrews is now the new Yucca Mountain," he said, comparing the WCS site to the federal radioactive waste disposal project about 80 miles from Las Vegas that might never open.

Others disagree. The closest town to the site is Eunice, N.M., home to a uranium enrichment plant, two gas processing plants and is less than 50 miles from the U.S. Energy Department's Waste Isolation Pilot Plant designed to hold radioactive waste from the Defense Department.

"Those kind of facilities have located in this area because, generally, the people in this area are very receptive to those kind of facilities," said Curtis Schrader, Eunice's city manager.

Viewed as patriotic Across the state line, Russell Shannon, president and CEO of the National Bank of Andrews, points out that the city did a study of the WCS site, which also has undergone 10 years of review by the Energy Department and the state.

The dump, he said, is turning ranchland that gets less than 16 inches of rain a year into a money-making enterprise that would provide a national public service.

"If we can safely store the material here, why would we not want to help the rest of the country?" Shannon said. "We think of it as patriotic."

The Associated Press and San Antonio Express-News reporter Colin McDonald contributed to this report.

E-mail: rule.comment@tllrwdcc.org

Postal mail: Texas Low-Level Radioactive Waste Disposal Compact Commission, 3616 Far West Blvd., Suite 117, #294, Austin, Texas 78731

http://www.chron.com/disp/story.mpl/metropolitan/7352954.html


Greetings fellow Texicans,

THIS is typical timing of such an event. This is how our Government gets bills and reports passed by the public. They usual do it late at night, on a Friday night at that, so it will be only late weekend news. they got it down pat too.

Twas the night before Christmas, when all through the house, Not a creature was stirring, not even a mouse, but the rats they were a stirring, right in the House, Their pockets were lined, with money and greed, in hopes that St Nicholas would not have seen.

The children were nestled all snug in their beds, While visions of sugar-plums danced in there heads. And mamma in her 'kerchief, and I in my cap, Had just settled our brains for a long winter's nap, and again, our good Governor from Texas Rick Perry, stabbed us in the back.

When out on the lawn there arose such a clatter, I sprang from the bed to see what was the matter. Away to the window I flew like a flash, Tore open the shutters and threw up the sash, there was a bright glow from a far, in the west, it was a big bright flash.

The moon on the breast of the new-fallen snow, gave the lustre of mid-day to objects below. When, what to my wondering eyes should appear, but train load after train load of Nuclear Prolifer.

With a little old driver, his hair in perfect affair, all groomed and combed over, like a multi-millionaire, was atop the train of nuclear prolifer. The glow was so bright, it lit up the whole night. More rapid than eagles his coursers they came, and he whistled, and shouted, bring more, bring more, we will light up the whole game.

"Now Dasher! now, Dancer! now, Prancer and Vixen! On, Comet! On, Cupid! on, on Donner and Blitzen! To the top of the porch! to the top of the wall, thanks to Governor Rick Perry, we may all glow like a great big fire ball.

AS dry leaves that before the wild hurricane fly, When they meet with this fire ball in the sky, the money the received will be their final cry. So UP the house and the senate i say, and to you rick perry, i better not say.

end

I wrote about our good Environmental Friendly Governor Rick Perry and his willingness to bring nuclear waste from Ohio to Texas back in 2008. Seems the money is so good, he wants to now include 38 states to the list of states able to pollute the great state of Texas with Nuclear Proliferation waste. at a boy governor, you single handedly made Texas a toxic dump for nuclear waste. Course, which is worse, the air quality or the nuclear waste, which will kill you first ? This Governor has shown time and time again where his heart is, and it's NOT in Texas. ...

Wednesday, August 6, 2008

Company advances on plan for West Texas nuclear dump

(railcars loaded with MOUND COLD WAR NUCLEAR AFTER-BIRTH headed to Texas see photo)

http://sciencebushwhacked.blogspot.com/2008/08/company-advances-on-plan-for-west-texas.html


Wednesday, July 30, 2008

TEXAS WINS TO BE NEXT BIG DUMPING GROUND FOR NUCLEAR WEAPONS RADIOACTIVE WASTE

(see more photo's of railcars loaded with MOUND COLD WAR NUCLEAR AFTER-BIRTH headed to Texas)

IT seems a more correct headlines would have read ''Company bribes Governor Perry to bury nuclear waste and contaminate Texas''. Waste Control Specialists. The company is owned by Harold Simmons, a ''TOP DONOR TO GOV. RICK PERRY, WHO APPOINTS MEMBERS TO THE TCEQ.'' The good governor has sold out to the citizens of Texas for train car, after train car of nuclear waste from 'the mound' Monsanto plant in Miamisburg Ohio. It just so happens, my father-in-law, who is down visiting now with us, has pictures of those railroad cars just sitting and waiting to come down to Texas. Odd how I was watching the news today, about this small plane that had crashed, it had showed pictures of where it had crashed right up near a bunch of tractor-trailer cargo container boxes in a parking lot. What would keep this from happening with those radioactive toxic containers in Ohio, at 'the mound', and or in route to Texas? You see, it's been killing my father-in-law, he has been on oxygen for years, but his breathing is getting more and more labored now, even with the oxygen. He worked at 'the mound' for years and years, and he is now dying a slow death from asbestosis, among other ailments caused by working at 'the mound'. NOW here is what I just cannot understand. This material is so toxic, in trying to gain further medical assistance from the DOE, the evidence that was needed to show that indeed my father-in-law worked their i.e. work records, paperwork records, payment records etc., they told my father-in-law, that they could not dig those records up, that they were buried due to high nuclear contamination, it was just too toxic, and that he had to prove that he had worked there. In which he did finally prove, and did gain further assistance. Also, ''Soward said a contested hearing could either rebut or support allegations that the agency ignored it's own scientific evidence that the site is geologically unsuitable to store material that will remain radioactive for tens of thousands of years.'' This is typical of the Bush Administration, and it happens all the time. The only science that the Bush administration knows, is junk science, bought and paid for by industry scientist. This has been proven time and time again ;

Reports and Research

Interference at the EPA

Science and Politics at the U.S. Environmental Protection Agency

snip...see full text ;

http://sciencebushwhacked.blogspot.com/2008/07/texas-wins-to-be-next-big-dumping.html


I think the title should have read, "TEXAS LOSES TO BE NEXT BIG DUMPING GROUND FOR NUCLEAR PROLIFERATION RADIOACTIVE WASTE", thanks to Governor Rick Perry.

update on my father-in-law Dana (RED) Ashcraft of Miamisburg Ohio, and my best fishing buddy, and Poisoned AT THE MONSANTO MOUND, hospice has now been called in. ...


TSS



part II December 25, 2010


WHY then, was my father-in-laws work records denied him, with the claim that his records were buried deep in a mountain due to contamination ? now i am speaking of only his work records, not the radioactive waste itself, that you claim to be 1000 % safe today. tell me that. do you know how many different folks handled all that paper work over the years. also, the swimming pool in Miamisburg Ohio, the old one right down from the Monsanto Mound. the town had to shut it down and fill the swimming pool in with cement. wonder how many kids there were exposed over the decades, including my wife ?


MONSANTO MOUND MIAMISBURG OHIO SWIMMING POOL

" We acknowledge that some people near the Mound Plant have breathed, or will likely breathe, very small amounts of plutonium-238, hydrogen-3 (tritium), and other radioactive substances that will be or have been released into the air from the Mound Plant. And some people may be exposed to radioactive materials released from the Mound Plant into the area waterways (for example, tritium in the Miamisburg Community Park swimming pool). Nevertheless, there is no evidence that current environmental levels of these substances cause adverse health effects. "

Data Evaluation: Current Exposures

ATSDR scientists reviewed the environmental data for the Mound Plant and concluded that there are no current exposure pathways to contamination from the Mound Plant that pose a public health hazard. We have considered all materials released from the Mound Plant, both radioactive and nonradioactive, individually and collectively, and their presence in all environmental media off site (such as air, water, vegetation and soil).

1.Radioactive Substances: Mound Releases Because nearly all Mound programs included work with radionuclides, the environmental and public health concerns that stakeholders most frequently discuss are those concerning radioactive substances released from the Mound Plant. The radionuclides in the environment that are a result of Mound activities and are of greatest concern today are plutonium-238 and hydrogen-3 (tritium). However, there is strong evidence from numerous environmental investigations--including our own--that plutonium-238, hydrogen-3, and all other radioactive substances from the Mound Plant contribute a very small amount to the total radiation dose that people living near the Mound Plant typically receive. Very likely, the largest radiation doses to nearby residents are those resulting from exposures to radioactive materials that are naturally occurring.

We acknowledge that some people near the Mound Plant have breathed, or will likely breathe, very small amounts of plutonium-238, hydrogen-3 (tritium), and other radioactive substances that will be or have been released into the air from the Mound Plant. And some people may be exposed to radioactive materials released from the Mound Plant into the area waterways (for example, tritium in the Miamisburg Community Park swimming pool). Nevertheless, there is no evidence that current environmental levels of these substances cause adverse health effects.

2.Nonradioactive Hazardous Substances: Mound Releases

The Mound Plant discharges small amounts of nonradioactive hazardous substances to the Great Miami River and to the air. People may occasionally be exposed to very small amounts of these materials. However, we do not think that releases of nonradioactive materials from the Mound Plant pose a public health hazard because the quantities of hazardous materials that are released are small and there is no evidence that the concentrations of these materials in the environment are high.

The pattern of nonradioactive contaminants in the environment does not suggest that those materials came from Mound. Mound's compliance record with the Ohio Environmental Protection Agency is good; their only regulatory exceedances in 1996 were NPDES (surface water) discharges from a private business on the Mound property. Copper concentrations in water exceeded the permit limit seven times. Mound officials reported the source of the problem was identified and corrected [14]. Based on current levels of contaminants off site and current levels of releases to the air and waterways, we do not expect that releases from the Mound Plant will pose a health hazard in the future, either.

3.Nonradioactive Hazardous Substances: Vicinity Air

The Regional Air Pollution Control Agency (RAPCA) collects air samples in a six-county area (including Montgomery County) for ozone, lead, carbon monoxide, sulfur dioxide, and particulate matter analyses. Based on the regional air data, the important indicators of air quality in Miamisburg are ozone and particulate matter concentrations. Ozone and particulate matter in air are pollutants that are both man-made and naturally occurring. The RAPCA reported that one air station in Dayton, Ohio, exceeded the current ozone standard once during 1997 (in June). The particulate matter air standard was not exceeded during 1997 at any of the RAPCA air stations that collect particulate matter samples [15].

The U.S. EPA is in the process of implementing changes in the air quality standards for both ozone and particulate matter to reflect newer information on their harmful effects [16, 17]. The current ambient air particulate matter standards are based on the concentration of particles in air that have a mean aerodynamic diameter equal to or less than 10 micrometers (called PM10) because particles larger than 10 micrometers are generally not inhaled deeply and usually do not cause health problems. In addition to measuring the concentration of particles in air below 10 micrometers, the new ambient air particulate matter standards will also require measuring the concentration of particles in air equal to or less than 2.5 micrometers (mean aerodynamic diameter, called PM2.5). RAPCA will be collecting PM2.5 data along with PM10 data to monitor compliance with the new particulate matter air standards when equipment becomes available during 1998 [15].

Mound scientists do not measure ozone at or near the Mound Plant, although they do collect particulate matter at their air monitors. Mound's annual environmental reports for 1995 and 1996 show the concentration of particulate matter in air samples collected off site near the Mound Plant met current state and federal standards [14, 18]. One air monitor on site exceeded the Ohio ambient air quality standard for particulate matter in 1996. The monitor is near the Miami-Erie Canal where engineers were removing trees and brush and digging up canal soils, and putting them in railroad cars to ship them off site. Mound's data indicated that air particulate matter concentrations around the site are slightly higher than is shown by the regional data. However, Mound's air monitors are designed to collect total air particulate matter, while the RAPCA's air monitors filter out particulate matter larger than 10 micrometers. Therefore, data from Mound's monitors are not directly comparable to the regional air data. Since we do not know what portion of the particulate mass collected at Mound's air monitoring stations includes particles larger than 10 micrometers, we do not know how their air data compares with the RAPCA's PM10 data.

In addition to the pollutants discussed above, the RAPCA reported that mold concentrations in air in the Miami Valley were high in the fall of 1996 [19]. Pollen and mold concentrations are measured by the Dayton Ear, Nose, and Throat Surgeons, Inc. in Centerville, Ohio, and reported to the RAPCA for publication on their Internet Home Page [19]. Pollen and mold are naturally occurring; air concentrations are affected by weather conditions.

4.Nonradioactive Hazardous Substances: Vicinity Soils

Nonradioactive contaminants, such as polyaromatic hydrocarbons (PAHs), polycyclic biphenyls (PCBs), heavy metals, and pesticides, are widespread--though not necessarily in high concentrations--in the environment. Investigations of area soils in 1994 by Mound scientists revealed that some contaminants are found near streets and highways [20]. Those data from soil samples collected from residential properties (all within 3,500 feet of the Mound Plant) indicated that pesticide levels are higher, on average, in residential soils than in other soils in the vicinity. We do not know whether anyone will ever breathe or eat the contaminants detected in vicinity soils. We also cannot say whether the available soil data is indicative of soil at any particular location, such as on someone's private property or in someone's garden. Nevertheless, the contaminants measured in residential soils are not in high enough concentrations to pose a health hazard [20, 21].

5.Nonradioactive Hazardous Substances: Vicinity Water

Mound investigations of private wells and cisterns in 1994 revealed that some wells and cisterns had unsafe levels of lead or pesticides [22]. At ATSDR's request, staff from the Combined Health District of Montgomery County followed up with those residents who may have been affected. There is no evidence or indication that the lead and pesticides found in private wells and cisterns came from the Mound Plant.

Mound scientists investigated surface waters and sediments on and around the Mound property in the fall of 1994 and spring of 1995 [23]. The concentrations of hazardous substances in surface waters and sediments near the Mound Plant do not pose a health hazard. The level of contamination is consistent with DOE's earlier measurements of nonradioactive substances in area soils and with urban environments. Low concentrations of contaminants, particularly semi-volatile organic compounds, are widespread in the environment; many of the contaminants are attributable to motor vehicle exhaust. Interestingly, Mound's 1994-1995 data show that the water in the Great Miami River contains very low levels of chemical contamination; many of the analytes that are typically found in the area soils, ponds, and streams are undetectable in the river water [23]. We recognize that there are other exposure pathways besides air, soil, and water, such as eating contaminated, locally grown produce, or eating fish from the Great Miami River. We examine some of these other exposure pathways in Appendix E. Our evaluations indicate that the contamination in the environment around the Mound Plant poses no apparent public health hazard by any exposure pathways that we have considered.

Data Evaluation: Past Exposures

We also looked carefully at the available historic data to determine whether people might have been exposed in the past to environmental contamination from the Mound site at levels that could have caused adverse health effects. We have divided our conclusions into three groups: a) conclusions about contamination that could have caused some people health problems, b) conclusions about contamination for which the data do not indicate that the releases would have caused anyone health problems, but for which we are lacking some important information, and c) conclusions about contamination for which we believe we have sufficient data to conclude that the releases did not ever pose a public health hazard. We discuss historic releases in the remainder of this section.

1.Historic Mound Releases That Could Have Caused Health Problems The first group, environmental releases that could have caused health problems, includes microbiological releases from the Mound sanitary (sewage) treatment facility. Two incidents with the Mound sewage treatment facility--one in August 1982 and one in August 1983--resulted in releases that posed a health hazard to people swimming and boating in the Great Miami River downstream from the plant.

The health hazard from releases of microbiological contamination is indicated by the results of fecal coliform testing in one of the Mound liquid effluent streams in 1982 and 1983. The standard maximum permitted level for the fecal coliform test is 2,000 MPN (most probable number of coliform colonies) per 100 milliliters (100 mL) of effluent water. In 1982 and 1983 the maximum values detected were 24,000 and 16,000 MPN per 100 mL, respectively. The fecal coliform test is an indicator test for the presence of pathogenic enteric organisms (bacteria, viruses, and protozoans); it is not a measure of them. Nevertheless, instances of high fecal coliform in effluent indicate an increased probability that pathogenic organisms are present in the effluent and that the effluent poses a public health hazard.

Undertreated sewage may contain numerous pathogenic microbiological organisms. Specific organisms responsible for disease outbreaks associated with recreational waters are often not identified [24]. In 1982 and 1983, states' health departments did not systematically report disease outbreaks associated with recreational waters to the U. S. Centers for Disease Control and Prevention (CDC). CDC estimated that the reported outbreaks during this period represented a small fraction of those that actually occurred [25]. Ohio did not report any water-related disease outbreaks of any kind to the CDC for 1982 and 1983.

The single organism most commonly identified with water-related disease outbreaks in recreational waters is Giardia lamblia, a parasite that can cause diarrhea, intestinal cramps, fatigue, and weight loss [25]. Giardia lamblia is not a fecal coliform, and it does not come from a fecal coliform. However, the fecal coliform test indicates the presence of pathogens that are not coliform bacteria as well as those that are. Gardia lamblia is difficult to identify in sewage because it is generally present in the cyst stage, which is not amenable to laboratory examination. The human incubation period (time from exposure to illness) may be 1 to 4 weeks; illness from Giardia lamblia (called Giardiasis) is treatable. Children, the elderly, and individuals with compromised immune systems are most at risk for illness from exposures.

Salmonella and Shigella bacteria, and Hepatitis A and Norwalk viruses may also be present in sanitary waste effluent [24]. Symptoms of these infections range from none to abdominal cramps, diarrhea, fever, and--very rarely--vomiting, delirium, convulsions, coma, and death.

We do not expect that anyone would suffer today from exposures to microbial pollution emanating from Mound in the early 1980s. The technical problems that Mound employees were having with the sanitary sewage treatment facility were corrected in the middle 1980s and there have been no reports of out-of-compliance fecal coliform levels in effluent since 1986.

2.Inconclusive Historic Mound Releases (Limited Data) In the second group--contaminants for which available data do not indicate that environmental releases posed a health hazard, but for which important data are missing--we have included both air and water releases of nonradioactive substances. We are not including the microbiological releases described in the first group, but we are including all nonradioactive chemical releases before 1971. In the 1950s and part of the 1960s, chemicals were released to the waste stream that flowed off site to the river without restriction. Also, in this same period, Mound Laboratory personnel disposed of solid and liquid wastes by open burning. Since we cannot identify or quantify all of the nonradioactive materials released to the air or water, we cannot estimate possible exposures to these materials.

We present a more detailed description of releases of nonradioactive substances from the Mound facility in Appendix A.

In the second group, we are also including releases of polonium-210 to the air and water. We have some air and water release data and environmental sampling results for polonium-210 collected during the 1950s. However, we do not have a continuous record of either releases to the environment or environmental sampling. The data we have do not indicate polonium-210 posed a public health hazard; however, institutional controls of releases were being developed in the 1950s and were not commensurate with today's standards. Since we have data gaps, we cannot estimate with any certainty the public's exposures to polonium-210 in the environment.

A more detailed description of releases of polonium-210 from the Mound Laboratory is in Appendix B.

3.Historic Mound Releases That Did Not Pose A Health Hazard The third group, where we have sufficient data to say that releases to the environment did not ever pose a public health hazard, includes all radioactive materials released from Mound, except polonium-210. These materials include plutonium-238 and hydrogen-3 (tritium).

A more detailed description of releases of radioactive substances to the environment, including plutonium-238 and hydrogen-3, is in Appendices C and D. Data Reviewed

ATSDR scientists reviewed many Mound documents for this public health assessment. Among the most important of these were Mound's health physics and environmental monitoring reports. The plant produced monthly reports before April 1954, quarterly reports from June 1954 through 1962, semi-annual reports from 1963-1972, and annual reports for 1972-1996. These periodic environmental reports (and others) are listed separately under Part 1 of the bibliography at the end of this document.

In addition to the periodic environmental monitoring data, ATSDR staff reviewed data collected from numerous investigations, including those of the plutonium-238 spill in the Miami-Erie Canal, the Conrail Bridge shoring, the Community Park water slide construction, the 1989 EG&G Energy Measurements overflight survey, and groundwater tritium evaluations for the Safe Drinking Water Act (Potable Water Standards Project) [26, 27]. We reviewed CERCLA-related environmental data in site-scoping reports, remedial investigation reports, and Operable Unit 9 investigations of off-site wells, regional soils, surface water and sediments, and groundwater. These Mound-related documents (and others) are listed under Part 2 in the bibliography at the end of this document.

Part 3 of the bibliography includes books and references that we reviewed for this public health assessment that are not specifically related to the Mound site.

We also obtained useful information through the environmental sampling program that we conducted with the assistance of the U.S. EPA National Air and Radiation Environmental Laboratory (NAREL) in Miamisburg in 1993 and 1994. ATSDR and NAREL personnel set up air monitors, surveyed the area with radiation equipment, and collected samples of the soil, water, air, and vegetation [28]. NAREL personnel and their contractors analyzed the environmental samples. Appendix E includes an evaluation of these data.

Prior to the Public Comment Release version of the Mound Plant public health assessment, ATSDR scientists conducted a health consultation evaluating the plutonium-238 in the Miami-Erie Canal [6]. We also wrote letters addressing off-site wells data [29], regional soils [21], Mound's proposed treatment for Operable Unit 1 (groundwater) [30], and Mound's proposed treatment plan for mixed wastes [31].

HEALTH OUTCOME DATA SUMMARY

Health outcome data are measures of disease occurrence in a population. Common sources of health outcome data are tumor registries, birth defects registries, and death certificates. Health outcome data can provide information on the general health status of a community--where, when, and what type of disease occurs and to whom it occurs. Public health officials use health outcome data to look for trends in disease occurrence by comparing disease occurrences in different populations over periods of years. This analysis is useful to help identify the need for exposure investigations or public health intervention activities such as community education. However, health outcome data are not meant to and cannot establish cause and effect between environmental exposures to hazardous materials and adverse health effects in a community.

ATSDR scientists generally look at health outcome data for one of two reasons: (1) to evaluate the possible health effects in a population that is known to have been exposed to environmental contamination, or (2) to help address community concerns. For this public health assessment, we identified one completed exposure pathway at levels of health concern: sewage wastes released to the Great Miami River in the early 1980s. We examined water-related disease outbreak surveillance reports and did not find any indication that people became ill from those exposures. However, we recognize that if illnesses did occur from those exposures, it is very possible they were never reported to health departments. We also examined other health outcome data for the population near the Mound Plant to help address community concerns.

At the request of ATSDR, staff from the Boston University School of Public Health (BU) helped us with our review of the health outcome data. We did not identify any health databases for the community that included health outcomes other than cancers (for example, birth defects). Where cancer data were available, we looked at leukemias that have been linked in the medical literature to radiation exposure; otherwise, we looked at all cancers including leukemias. The community near the Mound Plant expressed concerns to ATSDR staff about the presence of many cancers in the community.

ATSDR staff note that we do not have evidence that anyone living near the Mound facility was exposed to enough contamination from the site to cause adverse health effects. Moreover, based on the weight of evidence (described in Appendices C and D), we eliminated as probable candidates for causing health problems in the community the contaminants of greatest concern to the community, plutonium-238 and hydrogen-3.

Most of the health outcome data for populations near the Mound Plant are county data. However, the Montgomery County population is more than 30 times larger than the population of Miamisburg and more than 100 times larger than the population within a 1- mile radius of the Mound Plant (1990 U.S. Census data). Although Montgomery County may include a portion of the population that could have been exposed to environmental releases from the Mound Plant, the county also includes many people who were not exposed to the releases. Thus, counting occurrences of health outcomes in the county population would dilute the real effect from exposures because many people were not truly exposed.

Moreover; Montgomery County is approximately 462 square miles. Many industrial facilities other than the Mound Plant are located in Montgomery County as well as in nearby Warren and Butler counties. According to the Environmental Protection Agency's (EPA's) Toxic Chemical Release Inventory database, 49 facilities in Montgomery County reported releasing a total of more than 3 million pounds of hazardous substances into the environment in 1993 [32]. The contribution from the Mound Plant was less than 0.01% of this total. (Releases represent nonradioactive air emissions, surface water discharges, underground injections, and releases to land.) Since there are many other possible environmental exposures to account for, we cannot attribute the countywide health outcomes to exposures to environmental releases from the Mound Plant. For this reason, and because the county population is not a true representation of people exposed to releases from the Mound Plant, the countywide health data are not useful to us.

The only health outcome data for the general population near the Mound Plant that are not county-based are four reports from the Dayton Area Cancer Association summarizing cancer incidences (number and types of cancers reported) in nine area hospitals for the years 1985-1992 and, separately, cancer incidences for one of the reporting hospitals (Good Samaritan Hospital and Health Center) for 1984-1990. We do not know the residences of the people who checked into these hospitals, and we do not know whether they were exposed to environmental hazards from the Mound facility or from anywhere else. Without more information, we cannot calculate cancer rates from these data or compare cancer incidence between populations that may have been exposed to hazardous materials and those that were not, using these data. Therefore, these reports do not help us evaluate health outcomes that may be related to exposures to environmental releases from the Mound facility.

Finally, we identified three Mound worker studies. In general, worker studies may provide information on the health effects from documented exposures, and they may reflect on the safety record of the worker population. However, the Mound worker population is not the same as the general population near the Mound Plant. Mound worker health effects may be indicative of the types of exposures in the surrounding community; however, we expect the magnitude of exposures to workers to be more varied, and in some cases higher, than in the community. Therefore, we cannot infer that worker exposures are identical to those in the community around the Mound Plant.

We note that the Ohio Department of Health began to collect cancer incidence data at the zip code and census tract level in 1993. These data will be far more useful than the existing county-based health data in the future analysis of community health concerns. However, we note that, depending on actual environmental exposures to hazardous substances and the latency period between those exposures and the onset of adverse health effects, health outcome data collected since 1992 may not show effects from exposures occurring many years ago.

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1. Private conversation, September 12, 1996. 2. NIOSH is part of the Centers for Disease Control and Prevention (CDC), which is under the Department of Health and Human Services (DHHS). 3. This number is from the 1990 U.S. Census. The City of Miamisburg, Ohio, Home Page on the Internet indicates the population of Miamisburg in 1995 was 27,290. 4. Comprehensive Environmental Response, Compensation and Liability Act (CERCLA) of 1980 and the Superfund Amendments and Reauthorization Act (SARA) of 1986.


http://www.atsdr.cdc.gov/hac/pha/pha.asp?docid=634&pg=8



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http://webcache.googleusercontent.com/search?q=cache:j5dll2sGIM4J:www.atsdr.cdc.gov/hac/pha/pha.asp%3Fdocid%3D634%26pg%3D1+monsanto+mound+miamisburg+ohio+swimming+pool&cd=3&hl=en&ct=clnk&gl=us



http://www.atsdr.cdc.gov/hac/pha/pha.asp?docid=634&pg=2



http://www.atsdr.cdc.gov/hac/pha/pha.asp?docid=634&pg=3



http://www.cdc.gov/niosh/ocas/pdfs/abrwh/waivers/gibson.pdf




Then, they send all the radioactive waste to Texas. Now, we are going to multiply this by about 38 states ?



stupid is, as stupid does, and some times you just can't fix stupid $$$

Drugs from genetically engineered animals poised to debut in US

A CLOSER LOOK: BIOTECH ANIMALS

Genetically engineered animals and the FDA Are genetically engineered fish and meat coming soon? We examine the Food and Drug Administration's regulations.

By Jill U. Adams January 26, 2009

Fast-growing salmon. Pork containing heart-healthy omega-3 fatty acids. These are two examples of products you might see in your local supermarket soon -- animals developed not through conventional breeding but through genetic engineering.

On Jan. 15, the U.S. Food and Drug Administration decided how it will regulate genetically engineered animals, for the first time paving the way for such animals or their products to be sold as food and medicine. The agency has decided to categorize genetically engineered farm animals, also called transgenic animals, as an "animal drug." They will be held to the same requirements already existing for conventionally bred animals treated with hormones or antibiotics. (In the case of transgenic animals, the "drug" is a snippet of DNA.) Products derived from them or containing them as an ingredient will not necessarily require labeling.

A wide range of interested parties, including companies developing genetically engineered animals and consumer protection groups, are generally comfortable with the FDA decision. And yet, consumer acceptance of transgenic animals, particularly as food products, is still an unknown.

American consumers have been eating food from genetically engineered crops, such as corn, soybeans and canola, for a decade. However, transgenic animals have not been sold, pending the FDA deliberations on how to regulate them.

Why would someone want to genetically engineer animals?

Genetic engineering is a high-tech way to "breed" desirable traits into livestock. The benefits might be for the producer, such as a disease-resistant cow or an easy-to-raise salmon. It might be for the environment -- pigs that produce milder manure, for example -- or for the consumer, say, more nutritious meat.

The old-fashioned way of breeding farmed animals requires selecting offspring with desired traits over successive generations. Ron Stotish, chief executive of Aqua Bounty Technologies in Waltham, Mass., says the power of genetic engineering is that the same end is achieved in "one fell swoop." Transgenic animals also can be fitted with traits they probably would never develop naturally, as in the case of omega-3-producing pigs.

How is an animal genetically engineered?

An extra piece of genetic material (DNA) is inserted into the animal's genome at the earliest stages of development. Sometimes the method involves manipulating a fertilized egg that is then implanted into a surrogate mother; other times, it alters a cell from which an animal will be cloned. As the embryo grows, the DNA splice is replicated with the rest of the genetic material so that it ends up in every cell of the individual.

In fast-growing salmon developed by Aqua Bounty, the inserted gene is a salmon growth hormone, identical to the one the fish have naturally. The benefit comes from the precise placement of the added gene, which makes it active at times that the natural gene is not. "Normal salmon grow very, very slowly, and when it is cold they don't grow at all," Stotish says. "Our gene is active under a broader range of conditions and it allows the animals to grow." At 1 year old, a normal salmon might weigh 30 grams, but the transgenic fish weigh a kilogram, he says.

In pigs developed to contain omega-3 fatty acids, the added gene directs formation of an enzyme that converts naturally occurring omega-6 fatty acids to omega-3s. The gene is derived from tiny roundworms but is modified to make it more mammalian, says Randall Prather, co-director of the National Swine Resource and Research Center at the University of Missouri in Columbia, who developed these pigs.

Why did the FDA opt to regulate genetically engineered animals in this manner and what criteria are used to assess safety?

The FDA is responsible for food safety and already regulates genetically modified crops. By considering a DNA segment a drug, the agency will regulate transgenic animals in the same way it oversees dairy cows that receive growth hormone or beef cattle that get antibiotics, says FDA spokeswoman Siobhan DeLancey.

By using existing guidelines for so-called animal drugs, the FDA can put those products seeking approval into a process that is already up and running. "They have the staff and the process that allows them to look at this. They do it every day," says Stotish of Aqua Bounty, which has been seeking FDA approval for its fast-growing salmon for years.

Companies must show that their genetic manipulation is safe to the animal and that any food or animal-feed products derived from the animal are safe for the consumer and safe for the environment. Companies must also demonstrate that their claims about the gene-carried traits do occur.

Are there concerns beyond what the FDA can regulate?

Gregory Jaffe, who follows biotechnology for the consumer advocacy group Center for Science in the Public Interest, says the adopted guidelines are a good start "in the sense that the federal government has acknowledged that these animals are on the horizon and there needs to be oversight to ensure their safety."

But, he adds, genes are not the same as drugs. Drugs may have long-lasting effects on an individual, but they wouldn't get passed on to future generations. In the case of biotech animals, however, "you're altering the DNA of that animal, which gets carried on to its offspring." (Aqua Bounty says all the fish it markets will be sterile.)

Such lasting effects may have implications for preventing escape of the genes to natural populations, Jaffe says -- an issue that is beyond the FDA's expertise or authority.

Jaffe thinks the safety review should be more open and transparent. "Our regulatory processes for drugs" -- including animal drugs -- "are, on the whole, secret and done behind closed doors," he says. By law, the company controls what information is made public.

Other regulatory processes, such as those for pesticides at the Environmental Protection Agency, have been opened in past decades to invite public participation.

"Genetically engineered animals are highly controversial," Jaffe says. "I think that they [the FDA] need to have a transparent and participatory regulatory system -- where the public can review safety data and . . . expert scientists can provide comments to the agency."

Why did the FDA decide that food from genetically engineered animals doesn't have to be labeled?

The FDA labels food based on nutritional content, not manufacturing process, says the agency's DeLancey.

"So, if the food product is 'materially different' from a conventional product, then FDA can require that it be labeled. But the FDA doesn't require that a pork chop label specify whether it came from a pig produced through artificial insemination versus conventional breeding."

And similarly, now, if it came from genetically engineered animals. "We understand that consumers want transparency and labeling, but we are constrained by the regulations put in place by Congress," DeLancey says.

How will I know if the food I buy is from genetically engineered animals?

Pork from an omega-3 pig, should it gain approval, will be labeled because nutritional enhancement is a selling point -- and because the product is different from regular pork.

In other cases you may not know, although producers can voluntarily label their products. Researchers at the University of Guelph in Canada, who developed the so-called Enviropig with "greener" manure, say they intend to do so.

The U.S. Department of Agriculture has guidelines for labeling "natural meats," which would allow meat from non-transgenic animals to call attention to that fact.


mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000071/!x-usc:mailto:health@latimes.com



http://www.latimes.com/features/health/la-he-closer26-2009jan26,0,1632723.story






Tuesday, July 29, 2008


Docket No. 2005N-0373 RIN number 0910-AF54 Use of Materials Derived From Cattle in Medical Products REOPENING COMMENT PERIOD Date: March 30, 2007 at 11:37 am PST



snip...


TSS SUBMISSION TO ;

Docket No. 2005N-0373 and RIN number 0910-AF54

Greetings FDA et al, ONCE again I would kindly like to comment on the continuous failed attempts by the FDA to regulate the use of certain cattle material in, or in the manufacture including processing) of, drugs, biologics, and medical devices intended for use in humans and human cells, tissues, and cellular and tissue-based products (HCT/Ps) (collectively, medical products for humans), and in drugs intended for use in ruminant animals (drugs for ruminants) from the proven risk factors of Transmissible Spongiform Encephalopahy i.e. TSE's in all species. I have continued to warn the FDA et al about these risk factors via the surgical and medical arena (vaccines, nutritional supplements, bovine heart valves, and other animal donor tissue), and I have continued to point out the risk factor of the UKBSENVCJD only theory, and the ramifications there from, i.e. BASE (bovine amyloidotic spongiform encephalopathy), and my greatest fears, one I have warned you about time and time again, seems to be coming true ;

64. A member noted that at the recent Neuroprion meeting, a study was presented showing that in transgenic mice BSE passaged in sheep may be more virulent and infectious to a wider range of species than bovine derived BSE. Other work presented suggested that BSE and bovine amyloidotic spongiform encephalopathy (BASE) MAY BE RELATED. A mutation had been identified in the prion protein gene in an AMERICAN BASE CASE THAT WAS SIMILAR IN NATURE TO A MUTATION FOUND IN CASES OF SPORADIC CJD. A study also demonstrated that in a mouse model it was possible to alleviate the pathological changes of prion disease by suppressing expression of the prion protein gene after infection.



http://www.seac.gov.uk/minutes/95.pdf




snip...


full text ;





http://madcowfeed.blogspot.com/2008/07/docket-no-2005n-0373-and-rin-number.html




http://madcowfeed.blogspot.com/2008/12/docket-no-fda2008d0597-draft-guidance.html





Tuesday, April 29, 2008


Interference at the EPA - Science and Politics at the U.S. Environmental Protection Agency



----- Original Message -----

From: "Terry S. Singeltary Sr." mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000071/!x-usc:mailto:flounder9@verizon.net
To: "Bovine Spongiform Encephalopathy" mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000071/!x-usc:mailto:BSE-L@aegee.org Cc: mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000071/!x-usc:mailto:heggem.daniel@epa.gov; mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000071/!x-usc:mailto:sibert.christopher@epa.gov; mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000071/!x-usc:mailto:denne.jane@epa.gov; mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000071/!x-usc:mailto:hazen.susan@epa.gov; mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000071/!x-usc:mailto:mcrosby@ucsusa.org; mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000071/!x-usc:mailto:erobinson@ucsusa.org; mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000071/!x-usc:mailto:enegin@ucsusa.org; mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000071/!x-usc:mailto:cjdvoice@yahoogroups.com; mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000071/!x-usc:mailto:madcow@lists.iatp.org

Sent: Monday, April 28, 2008 9:48 PM

Subject: Interference at the EPA Science and Politics at the U.S. Environmental Protection Agency

Reports and Research

Interference at the EPA

Science and Politics at the U.S. Environmental Protection Agency

The U.S. Environmental Protection Agency (EPA) has the simple yet profound charge "to protect human health and the environment." EPA scientists apply their expertise to protect the public from air and water pollution, clean up hazardous waste, and study emerging threats such as global warming. Because each year brings new and potentially toxic chemicals into our homes and workplaces, because air pollution still threatens our public health, and because environmental challenges are becoming more complex and global, a strong and capable EPA is more important than ever.

Yet challenges from industry lobbyists and some political leaders to the agency's decisions have too often led to the suppression and distortion of the scientific findings underlying those decisions—to the detriment of both science and the health of our nation. While every regulatory agency must balance scientific findings with other considerations, policy makers need access to the highest-quality scientific information to make fully informed decisions.

Concern over this problem led the Union of Concerned Scientists (UCS) to investigate political interference in science at the EPA. The investigation combines dozens of interviews with current and former EPA staff, analysis of government documents, more than 1,600 responses to a survey sent to current EPA scientists, and written comments from EPA scientists.

The results of these investigations show an agency under siege from political pressures. On numerous issues—ranging from mercury pollution to groundwater contamination to climate change—political appointees have edited scientific documents, manipulated scientific assessments, and generally sought to undermine the science behind dozens of EPA regulations. ...

snip...please see full text ;

Interference at the EPA Science



http://sciencebushwhacked.blogspot.com/



Thu Dec 6, 2007 11:38

FDA IN CRISIS MODE, AMERICAN LIVES AT RISK



http://www.cidrap.umn.edu/cidrap/content/fs/food-disease/news/dec0407fda.html



FDA SCIENCE AND MISSION AT RISK



http://www.fda.gov/ohrms/dockets/ac/07/briefing/2007-4329b_02_01_FDA%20Report%20on%20Science%20and%20Technology.pdf



Monday, January 12, 2009

FDA scientists complain to Obama of 'corruption'



http://sciencebushwhacked.blogspot.com/2009/01/fda-scientists-complain-about.html



January 26, 2009

A kind greetings from Bacliff, Texas !

O.K., let us suppose the impossible, that indeed spontaneous Transmissible Spongiform Encephalopathy _does_ exists (to date, this has never been proven in the field, under natural conditions), at whatever rate they choose (i do not believe in the spontaneous TSE as the cause for 85%+ of all CJD i.e. the sporadic CJD's, i.e. that all these poor victims died from a 'happenstance of bad luck', or 'that a twisted funked out protein just chose this group on it's own, with no route and source of nothing involved'), BUT for now, lets say they exist.

PLEASE tell me then how a GM cow will be free from the spontaenous TSE ???

Spontaneous occurrence (14-16)

The evidence from the absence of BSE in many countries and the surveillance schemes abroad indicates that most BARBs cases cannot have arisen spontaneously, although the possibility cannot be excluded that a very few of them did so. The possibility of a very low frequency of spontaneous occurrence of BSE may be monitored from the output of surveillance in cattle populations elsewhere. R: A watching brief is kept on surveillance efforts world-wide.



http://www.defra.gov.uk/animalh/bse/pdf/hillreport.pdf




Perspectives BIOMEDICINE: A Fresh Look at BSE Bruce Chesebro*

Mad cow disease, or bovine spongiform encephalopathy (BSE), is the cattle form of a family of progressive brain diseases. These diseases include scrapie in sheep, Creutzfeldt-Jakob disease (CJD) in humans, and chronic wasting disease (CWD) in deer and elk. They are also known as either "prion diseases" because of the association of a misfolded cellular prion protein in pathogenesis or "transmissible spongiform encephalopathies" (TSEs) because of the spongelike nature of the damaged brain tissue (1).

The recent discovery of two BSE-infected cows, one in Canada and one in the United States, has dramatically increased concern in North America among meat producers and consumers alike over the extent to which BSE poses a threat to humans as well as to domestic and wild animals. The European BSE epidemic of the late-1980s seems to have been initiated a decade earlier in the United Kingdom by changes in the production of meat and bone meal (MBM) from rendered livestock, which led to contamination of MBM with the BSE infectious agent. Furthermore, the fact that UK farmers fed this rendered MBM to younger animals and that this MBM was distributed to many countries may have contributed to the ensuing BSE epidemic in the United Kingdom and internationally (2).

Despite extensive knowledge about the spread of BSE through contaminated MBM, the source of BSE in Europe remains an unsolved mystery (2). It has been proposed that BSE could be derived from a cross-species infection, perhaps through contamination of MBM by scrapie-infected sheep tissues (see the figure). Alternatively, BSE may have been an endemic disease in cattle that went unnoticed because of its low level of horizontal transmission. Lastly, BSE might have originated by "spontaneous" misfolding of the normal cellular prion protein into the disease-associated abnormal isoform (3), which is postulated to be the infectious agent or "prion."

Five possible sources of BSE in North American cattle. Sheep, deer, and elk could spread prion diseases (TSEs) to cattle through direct animal contact or contamination of pastures. Endemic BSE has not been proven to exist anywhere in the world, but it is difficult to exclude this possibility because of the inefficient spread of BSE infectivity between individual animals (2). BSE caused by spontaneous misfolding of the prion protein has not been proven. CREDIT: KATHARINE SUTLIFF/SCIENCE

snip...

Nevertheless, the idea that BSE might originate due to the spontaneous misfolding of prion proteins has received renewed interest in the wake of reports suggesting the occurrence of atypical BSE (9-11). These results imply that new strains of cattle BSE might have originated separately from the main UK outbreak. Where and how might such strains have originated? Although such rare events cannot be studied directly, any number of sources of the original BSE strain could also explain the discovery of additional BSE strains in cattle (see the figure). However, it would be worrisome if spontaneous BSE were really a valid etiology because such a mechanism would be impossible to prevent--unlike other possible scenarios that could be controlled by large-scale eradication of TSE-positive animals.

Another way to look at this problem is to examine evidence for possible spontaneous TSE disease in other animals besides cattle. Spontaneous BSE would be extremely difficult to detect in cattle, where horizontal spread is minimal. However, in the case of the sheep TSE disease, scrapie, which spreads from ewes to lambs at birth as well as between adults, spontaneous disease should be detectable as new foci of clinical infection. In the early 1950s scrapie was eradicated in both Australia and New Zealand, and the mainland of both these countries has remained scrapie-free ever since. This scrapie-free status is not the result of selection of sheep resistant to scrapie because sheep from New Zealand are as susceptible as their UK counterparts to experimental scrapie infection (12). These experiments of man and nature appear to indicate that spontaneous clinical scrapie does not occur in sheep. Similarly, because CWD is known to spread horizontally, the lack of CWD in the deer or elk of eastern North America but its presence in western regions would also argue against a spontaneous disease mechanism. This is particularly noteworthy in New Zealand, where there are large numbers of deer and elk farms and yet no evidence of spontaneous CWD. If spontaneous scrapie does not occur in sheep or deer, this would suggest that spontaneous forms of BSE and sporadic Creutzfeldt-Jakob disease (sCJD) are unlikely to be found in cattle or humans. The main caveat to this notion is that spontaneous disease may arise in some animal species but not others. In humans, sCJD--which is considered by some researchers to begin by spontaneous misfolding of the prion protein--usually takes more than 50 years to appear. Thus, in animals with a shorter life-span, such as sheep, deer, and cattle, an analogous disease mechanism might not have time to develop.

What can we conclude so far about BSE in North America? Is the BSE detected in two North American cows sporadic or spontaneous or both? "Sporadic" pertains to the rarity of disease occurrence. "Spontaneous" pertains to a possible mechanism of origin of the disease. These are not equivalent terms. The rarity of BSE in North America qualifies it as a sporadic disease, but this low incidence does not provide information about cause. For the two reported North American BSE cases, exposure to contaminated MBM remains the most likely culprit. However, other mechanisms are still possible, including cross-infection by sheep with scrapie or cervids with CWD, horizontal transmission from cattle with endemic BSE, and spontaneous disease in individual cattle. Based on our understanding of other TSEs, the spontaneous mechanism is probably the least likely. Thus, "idiopathic" BSE--that is, BSE of unknown etiology--might be a better term to describe the origin of this malady. ...

snip...full text ;



http://www.sciencemag.org/cgi/content/full/sci;305/5692/1918



Release No. 0106.04

Contact: Office of Communications (202) 720-4623

Transcript of Remarks From Technical Briefing on BSE and Related Issues With Agriculture Secretary Ann M. Veneman and USDA Chief Veterinary Officer Dr. Ron DeHaven Washington D.C. - March 15, 2004

snip...

OPERATOR : “Yes. Our next one is coming from Elizabeth Weiss. Please state your company.”

ELIZABETH WEISS: “This is Elizabeth Weiss with USA Today.”

“I actually had two questions. First off, when you say you're looking for 1 in 10,000 cases, is USDA doing any work to find out the possibility of whether or not BSE exists in a spontaneous form in the way that it does in humans and elk populations?

“And secondly, how will any of this fit into some of the consternation that's been raised in California and with the Midwest packer that wanted to test all of its cattle?

“Thanks.”

DR. DEHAVEN: “All right. I think we've got three different questions in there, and I'll try to touch on each one of them.

“First of all, let me correct just a technical issue, and that is you mentioned 1 in 10,000. And actually our surveillance system currently is designed, the one that we have in place now is designed to detect 1 positive in 1 million cattle, and I gave some numbers between 200,000 and 268,000 that would allow us to detect 1 in 10 million as opposed to 1 in 10,000.

“So we would, if we were able to collect in the ballpark of those numbers of samples then we with increasing numbers of samples have an increasingly statistically valid sample from which to determine, one, whether or not the disease exists and, if so, at what prevalence level.

“So our real emphasis is to test as many of those animals as we can, ensure that we get an appropriate geographical distribution, but not setting a specific number as far as a target. Again, consistent with the recommendation from the International Review Team, their recommendation was to test all of them.

“So that's consistent with where we're going is to test as many as we possibly can.

“As far as spontaneous cases, that is a very difficult issue. There is no evidence to prove that spontaneous BSE occurs in cattle; but here again it's an issue of proving a negative. We do know that CJD, the human version of the disease, does occur spontaneously in humans at the rate of about 1 in 1 million. We don't have enough data to definitively say that spontaneous cases of BSE in cattle occur or do not occur.

“Again, it's a very difficult situation to prove a negative.

“So a lot of research is ongoing. Certainly if we do come up with any positive samples in the course of this surveillance we will be looking at that question in evaluating those samples but no scientifically hard evidence to confirm or refute whether or not spontaneous cases of BSE occur.

snip...



http://www.usda.gov/wps/portal/!ut/p/_s.7_0_A/7_0_1OB?contentidonly=true&contentid=2004/03/0106.html



UK TESTING FINISHED NEXT WEEK

Matthews confirmed that the brain tissue samples from the US animal had arrived at Weybridge. Test results were likely to be ready by the end of next week, he said.

The suspect animal has already undergone a series of tests. A rapid screening test on Nov. 15 returned inconclusive results. Sophisticated immunohistochemistry (IHC) tests cleared the animal of any infection, but a third round of testing using a Western blot procedure showed a "weak positive".

Weybridge will do an IHC test plus three kinds of Western Blot tests on the samples. They will use "methods of slightly different analytical sensitivity that give us the greatest number of opportunities to interpret what we see," he said.

US beef industry leaders say scientists should not speculate about the unusual case.

"There's no evidence that it's atypical ... and there's absolutely no evidence that it's spontaneous," said Gary Weber, head of regulatory affairs at the National Cattlemen's Beef Association.

Matthews noted scientists are still grappling with what is typical and atypical BSE.

"Far too few people have looked at BSE in depth using all of the tests to be able to define 'this is normal and that one isn't'," he said.

Weber noted Japan used the term to describe two very young infected cattle because BSE is usually found in older animals. Italy labeled a case "atypical" because the misshaped prions were found in unexpected parts of the animal's brain. ...snip...end



http://www.agobservatory.org/headlines.cfm?refID=73207



Atypical BSE strain -- In July 2007, the UK Spongiform Encephalopathy Advisory Committee (SEAC) suggested that atypical BSE may be a distinct strain of prion disease. Unlike typical BSE, cases of atypical BSE, according to SEAC, may have risen spontaneously (although transmission through feed or the environment cannot be ruled out). Recently reported French surveillance data support this theory that unlike typical BSE, atypical BSE appears to represent sporadic disease



http://cdc.gov/ncidod/dvrd/bse/



http://www.defra.gov.uk/animalh/bse/pdf/hillreport.pdf



Spontaneous occurrence


14. Spontaneous cases of classical CJD in humans are found at a rate of about 1/million around the world (Will, 1993), without appreciable racial or geographical variation except in a few specific cases, notably Jews of Libyan origin that have a mutation in the open reading frame of the PRNP gene (Chapman and Korczyn, 1991). Thus it is theoretically possible that spontaneous cases of BSE could occur as a consequence of a germ line mutation, in which case relatives would also have a certain or increased incidence of the disease, or a somatic mutation, which would be unlikely to be detectable unless the appropriate tissue were identified, or after some transformation in the PrP protein in the animal concerned. BSE was unknown prior to its detection in Britain in the mid 1980s, and Index cases found around the world since then can all be explained in terms of export from the UK directly or indirectly of cattle or of feed components. No BSE affected animals have been reported in many developed countries with large cattle populations, including Australia, New Zealand, Norway and Sweden, which have mixed cattle populations; and the only infected animal detected in the US was of Canadian origin. The disease seems to have a highly homogeneous aetiology (e.g. Bruce, 2003).

15. Data from the USA, where the dairy population in particular is highly related to that in GB provide an upper limit to the spontaneous rate. A programme of testing is in place of a target population of adult cattle exhibiting some clinical sign that might be consistent with BSE (animals reported as having CNS or clinical signs of BSE or were non-ambulatory). In the intensive programme from June 2004 over 375000 animals were tested in the following 12 months. No positive results have yet been obtained in these or previous tests (USDA BSE Testing). This implies a putative upper limit of under 10 per million in this target group. Assuming, as analysis has shown, the relative risk in this group is about 30 times higher than in the population as a whole (European Commission, 2002c), then the incidence in the population as a whole is under 3 per 10 million. This figure could possibly be biased downwards if affected animals are diagnosed and disposed of without being tested. Taking account of testing done and the lack of clinical cases seen in many other countries also, it seems highly unlikely that the spontaneous rate can be as much as 3 per 10 million head. Nor can spontaneous occurrence explain incidences of ca. 30 cases of BARBs per year in 2002/4 in the UK adult cattle population of ca. 4 million. [NOTE ADDED 30 JUNE: The recent confirmation of a previously inconclusive case in the USA affects these calculations. If the animal did not have access to infected feed, the calculations have to be revised: they suggest a sporadic incidence in the population of 1/(375000 x 30) or almost 1 per 10 million, with the upper limit under 5 per 10 million.]

16. Calculation of a maximum rate of possible transformation from scrapie to BSE is less feasible. Nevertheless, BSE appears not to have arisen in the UK until around the early 1980s, despite the presence of scrapie in sheep here for at least 200 years. Although a change in the scrapie prion may have been the cause of the initial cases of BSE, the difference between their properties in mice and the uniformity of the BSE brain lesions suggest it is unlikely that more than one such mutation was the source of BSE. It is most unlikely that the same mutation could be occurring often enough to contribute significantly to BARBs cases. Furthermore BARBs cases do not match the geographical distribution of the sheep population. The evidence from the absence of BSE in many countries and the surveillance schemes abroad indicates that most BARBs cases cannot have arisen spontaneously, although the possibility cannot be excluded that a very ...



http://www.defra.gov.uk/animalh/bse/pdf/hillreport.pdf



242 Atypical and classical BSE are different strains based upon Western blot profiles 243 (Hill, 2004; Normile, 2004; Baron et al., 2006), and this study indicates that disease Page 11 of 23 Journal of Animal Science Downloaded from jas.fass.org by on November 12, 2008. 12 progresses via different routes for these strains. The disparate 244 routes of pathogenesis in 245 atypical BSE can occur by 1 of 2 means. One possibility is that the source of infectivity 246 in atypical BSE is exposure to contaminated feedstuffs, as is the case for classical BSE, 247 but progression occurs in a disparate manner that bypasses the influence of the indel 248 polymorphisms. The other possibility is that atypical BSE is occurring spontaneously in 249 the host. Support for atypical BSE occurring spontaneously are the parallels to sporadic 250 TSE in humans, specifically, occurrence in older hosts and a comparable low incidence 251 rate (Baron and Biacabe, 2006). Furthermore, atypical BSE occurs as isolated, sporadic 252 cases in contrast to the clustering of cases observed for feed borne classical BSE 253 (Donnelly et al., 1997). Interestingly, the only native born cases of BSE in the United 254 States identified to date have been classified as atypical BSE.

255 No experiment can conclusively confirm a spontaneous nature for atypical BSE.

snip...end



http://jas.fass.org/cgi/reprint/jas.2007-0208v1.pdf



Monday, February 11, 2008 7:00 am Registration and Morning Coffee

Emerging Concerns: De novo Formation of Prions

9:05 De novo Generation of Prion Infectivity in a Cell-Free System Joaquin Castilla, Ph.D., Assistant Professor, Department of Infectology, Scripps Research Institute-Florida Transmissible spongiform encephalopathies (TSEs) are a group of neurodegenerative disorders affecting both humans and animals. There is no available treatment or therapy for these fatal diseases. The infectious agent associated with TSEs (termed prion) appears to be composed uniquely of a protein, which is a conformationally-modified version (PrPSc) of the cellular prion protein (PrPC). The disease is propagated by the conversion of host PrPC into PrPSc induced by small quantities of PrPSc. Interestingly, prions occur in the form of different strains that show distinct biological and physicochemical properties. TSEs can have diverse origins, including genetic, sporadic (putatively spontaneous) and infectious. The occurrence of sporadic cases of prion diseases in humans and maybe in other species, i.e. atypical bovine spongiform encephalopathy (BSE) in European and USA cattle and atypical scrapie cases in sheep suggest that spontaneous prion diseases may happen infrequently but ubiquitously. However, there are no reported cases of spontaneously-occurring prion disease in experimental wild-type rodent models. We have used a novel technique, Protein Misfolding Cyclic Amplification (PMCA) to rapidly propagate prions in the test tube, using normal brain homogenate as substrate. Prions propagated in vitro are infectious in vivo and maintain their prion strain specificity. PMCA has been used to efficiently amplify a variety of prion strains from mouse, hamster, bank vole, deer, cattle, sheep and human. Therefore, to mimic spontaneous generation of infectivity in vitro becomes one of the most important challenges in the prion field. We show here, for the first time, the de novo generation of infectious prions from bank voles (Clethrionomys glareolus) starting with non-infectious brain homogenates. Several biochemically different prion strains were generated using two different wild-type vole genotypes. The de novo in vitro generated PrPSc was highly infectious after its inoculation in bank voles. We show an extensive characterization of this "spontaneous" phenomenon.



http://www.healthtech.com/Conferences_Overview.aspx?c=518&id=59662&c=518



Pathogenesis Chairperson: Suzette Priola, Ph.D.

11:10 Accumulation of Prion Protein in the Brain That is Not Associated with Transmissible Disease Pedro Piccardo, M.D., Senior Investigator, OBRR / DETTD / LBPUA, FDA (Invited)

11:35 High Levels of TSE Infectivity Can Be Associated with Little or No Detectable PrPSc in Vivo Rona Barron, Ph.D., Neuropathogenesis Unit, Roslin Institute and Royal (Dick) School of Veterinary Studies This work examines the relationship between TSE infectivity and the abnormal prion protein, PrPSc. In a mouse model of disease we have shown high titres of TSE infectivity in brain tissue which contains little or no PrP-res. We also found no evidence of other abnormal PrP isofoms such as PK-sen PrPSc. These data question the true relationship between PrPSc and TSE infectivity, and the current reliance on PrPSc as the sole diagnostic marker for TSE disease.

12:00 Conversion of the BASE Prion into the BSE Prion: The Origin of BSE? Fabrizio Tagliavini, Ph.D., Director, Division of Neurology 5 & Neuropathology, Neurological Institute "Carlo Besta" Twenty years after the identification of bovine spongiform encephalopathy (BSE), the origin of the causal agent is still unknown. This issue is of fundamental importance, since knowledge of the origin of the BSE agent is essential for prevention of future outbreak of the disease or variants thereof in cattle and other mammals. We carried out transmission studies with transgenic mice expressing bovine PrP and four lines of non-transgenic mice and found that an atypical form of spongiform encephalopathy of cattle, termed BASE or BSE-L, is caused by a prion strain distinct from that of classical BSE. Noteworthy, this newly characterized prion strain has the ability to convert into the classical BSE strain upon serial transmission to inbred mouse lines. According to these results, BASE--which is regarded as a sporadic form of prion disease in cattle--may be the origin of BSE, following conversion of the causal agent in an intermediate host.

12:45 Luncheon Technology Workshop (Sponsorship Available) or Lunch on Your Own

2:00 Sporadic CJD and Atypical BSE: Two Children of One Protein Maurizio Pocchiari, Ph.D., Director of Research, Virology, Istituto Superiore Di Sanita The identification of forms of TSE diseases in cattle caused by prion strains different from BSE has raised new concerns on the possibility that these novel agents might induce disease in humans with a phenotype resembling sporadic CJD. The analysis of the distribution of the different molecular subtypes of sporadic CJD might give some answers.



http://www.healthtech.com/Conferences_Overview.aspx?c=518&id=59662&c=518



let's see, a few examples of our extensive BSE surveillance plan ;

On December 23, 2003, the U.S. Department of Agriculture (USDA) announced a presumptive diagnosis of the first known case of BSE in the United States. It was in an adult Holstein cow from Washington State. This diagnosis was confirmed by an international reference laboratory in Weybridge, England, on December 25. Trace-back based on an ear-tag identification number and subsequent genetic testing confirmed that the BSE-infected cow was imported into the United States from Canada in August 2001. Because the animal was non-ambulatory (a “downer cow”) at slaughter, brain tissue samples were taken by USDA’s Animal and Plant Health Inspection Service as part of its targeted surveillance for BSE. However the animal’s condition was attributed to complications from calving. After the animal was examined by a USDA Food Safety and Inspection Service (FSIS) veterinary medical officer both before and after slaughter, the carcass was released for use as food for human consumption. During slaughter, the tissues considered to be at high risk for the transmission of the BSE agent were removed. On December 24, 2003, FSIS recalled beef from cattle slaughtered in the same plant on the same day as the BSE positive cow. (see Bovine Spongiform Encephalopathy in a Dairy Cow - Washington State, 2003.)

On June 24, 2005, the USDA announced receipt of final results from The Veterinary Laboratories Agency in Weybridge, England, confirming BSE in a cow that had conflicting test results in 2004. This cow was from Texas, died at approximately 12 years of age, and represented the first endemic case of BSE in the United States. (see Texas BSE Investigation, Final Epidemiology Report, August 2005 (PDF – 83 KB))

On March 15, 2006, the USDA announced the confirmation of BSE in a cow in Alabama. The newly confirmed case was identified in a non-ambulatory (downer) cow on a farm in Alabama. The animal was euthanized by a local veterinarian and buried on the farm. The age of the cow was estimated by examination of the dentition as 10-years-old. It had no ear tags or distinctive marks; the herd of origin could not be identified despite an intense investigation (see second featured item above and Alabama BSE Investigation, Final Epidemiology Report, May 2006).

snip...

Strains of BSE There is increasing evidence that there are different strains of BSE: the typical BSE strain responsible for the outbreak in the United Kingdom and two atypical strains (H and L strains).

Typical BSE strain -- The BSE strain responsible for most of the BSE cases in Canada is the same classic or typical strain linked to the outbreak in the United Kingdom. It is known to be preventable through elimination of BSE contaminated feed and has been causally linked to vCJD in humans. This typical strain has not yet been identified in any U.S.-born cattle.

Atypical BSE strain -- In July 2007, the UK Spongiform Encephalopathy Advisory Committee (SEAC) suggested that atypical BSE may be a distinct strain of prion disease. Unlike typical BSE, cases of atypical BSE, according to SEAC, may have risen spontaneously (although transmission through feed or the environment cannot be ruled out). Recently reported French surveillance data support this theory that unlike typical BSE, atypical BSE appears to represent sporadic disease

Both of the U.S.-born BSE cases and two of the 15 Canadian-born BSE cases were 10 years of age or older and three of these older North American cases for whom the strain type is presently known were linked to an atypical BSE strain known as the H-strain. Publication of the strain testing results on the 13 year-old BSE-infected Canadian cow identified in December 2007 is pending.



http://cdc.gov/ncidod/dvrd/bse/



Saturday, January 24, 2009

Bovine Spongiform Encephalopathy h-BSE ATYPICAL USA 2008 Annual Report

Research Project: Study of Atypical Bse

Location: Virus and Prion Diseases of Livestock

2008 Annual Report


snip...



Thursday, December 04, 2008 2:37 PM


"we have found that H-BSE can infect humans."


personal communication with Professor Kong. ...TSS



snip...



http://bse-atypical.blogspot.com/2009/01/bovine-spongiform-encephalopathy-h-bse.html



Sunday, December 28, 2008

MAD COW DISEASE USA DECEMBER 28, 2008 an 8 year review of a failed and flawed policy

Greetings,

I thought a quick review of the Bush's terribly flawed and failed mad cow disease policy, from the illegal feeding of literally millions and millions of pounds of highly suspect, and banned mad feed, to the failed BSE surveillance program, all of which exposed, needlessly, millions of people to the mad cow agent i.e. Transmissible Spongiform Encephalopathy. ...

Parentage-based DNA traceback in beef and dairy cattle 2008



http://www.ars.usda.gov/sp2UserFiles/Place/54380570/HeatonPublications/HeatonParentage-Traceback2008o.pdf



48 hour traceback for BSE mad cow disease in the USA ???

NOT in your lifetime !

8 YEARS IN REVIEW OF THE MAD COW DEBACLE IN THE USA ;

FOR IMMEDIATE RELEASE Statement May 4, 2004 Media Inquiries: 301-827-6242 Consumer Inquiries: 888-INFO-FDA

Statement on Texas Cow With Central Nervous System Symptoms

snip...full text ;



http://bse-atypical.blogspot.com/2008/12/mad-cow-disease-usa-december-28-2008-8.html



Friday, August 29, 2008

CREEKSTONE VS USDA COURT OF APPEALS, BUSH SAYS, NO WAY, NO HOW



http://madcowtesting.blogspot.com/2008/08/creekstone-vs-usda-court-of-appeals.html



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



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 Manuscript 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



November 25, 2008

Update On Feed Enforcement Activities To Limit The Spread Of BSE



http://madcowfeed.blogspot.com/2008/11/november-2008-update-on-feed.html



"the biochemical signature of PrPres in the BASE-inoculated animal was found to have a higher proteinase K sensitivity of the octa-repeat region. We found the same biochemical signature in three of four human patients with sporadic CJD and an MM type 2 PrP genotype who lived in the same country as the infected bovine." ... interesting. ... TSS

Thursday, June 05, 2008

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



In this context, a word is in order about the US testing program. After the discovery of the first (imported) cow in 2003, the magnitude of testing was much increased, reaching a level of >400,000 tests in 2005 (Figure 4). Neither of the 2 more recently indigenously infected older animals with nonspecific clinical features would have been detected without such testing, and neither would have been identified as atypical without confirmatory Western blots. Despite these facts, surveillance has now been decimated to 40,000 annual tests (USDA news release no. 0255.06, July 20, 2006) and invites the accusation that the United States will never know the true status of its involvement with BSE.

In short, a great deal of further work will need to be done before the phenotypic features and prevalence of atypical BSE are understood. More than a single strain may have been present from the beginning of the epidemic, but this possibility has been overlooked by virtue of the absence of widespread Western blot confirmatory testing of positive screening test results; or these new phenotypes may be found, at least in part, to result from infections at an older age by a typical BSE agent, rather than neonatal infections with new "strains" of BSE. Neither alternative has yet been investigated.



http://www.cdc.gov/ncidod/EID/vol12no12/06-0965.htm



Wednesday, August 20, 2008

Bovine Spongiform Encephalopathy Mad Cow Disease typical and atypical strains, was there a cover-up ?



http://bse-atypical.blogspot.com/2008/08/bovine-spongiform-encephalopathy-mad.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:

mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000071/!x-usc:mailto:flounder9@verizon.net

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/



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/



Attending Dr.: Date / Time Admitted : 12/14/97 1228

UTMB University of Texas Medical Branch Galveston, Texas 77555-0543 (409) 772-1238 Fax (409) 772-5683 Pathology Report

FINAL AUTOPSY DIAGNOSIS Autopsy' Office (409)772-2858

FINAL AUTOPSY DIAGNOSIS

I. Brain: Creutzfeldt-Jakob disease, Heidenhain variant.



http://creutzfeldt-jakob-disease.blogspot.com/2008/07/heidenhain-variant-creutzfeldt-jakob.html



2007

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