Netherlands reports third human death from mad cow disease
naar persbericht informatie over variant Creutzfeldt-Jakob
Voor derde keer patiënt aan variant Creutzfeldt-Jakob overleden 2 februari 2009 In Nederland is voor de derde keer de ziekte variant Creutzfeldt-Jakob (vCJD) vastgesteld. De betreffende patiënt overleed begin januari. Na onderzoek in het UMC Utrecht en het Nederlandse CJD-expertisecentrum in Rotterdam is bevestigd dat het gaat om variant Creutzfeldt-Jakob. Deze zeldzame ziekte kan worden overgedragen door het eten van met BSE besmette vleesproducten. BSE is ook wel bekend onder de naam gekkekoeienziekte.
Op dit moment wordt onderzocht hoe groot de kans is dat de patiënt andere mensen heeft besmet. Het RIVM coördineert dit onderzoek. De gegevens die tot dusver zijn verzameld tonen aan dat deze kans uiterst klein is. De overdracht van mens op mens kan plaatsvinden via besmet bloed of weefsel.
Variant Creutzfeldt-Jakob De variant Creutzfeldt-Jakob is een zeer zeldzame, door prionen veroorzaakte hersenziekte. De overdracht van variant Creutzfeldt-Jakob op mensen vindt in de meeste gevallen plaats door de consumptie van besmette vleesproducten. Het rundvlees in Nederland is nu veilig, omdat sinds 2001 runderen bij de slacht op BSE worden getest. Ook worden sinds 1997 bij de slacht de meest infectieuze organen, de hersenen en het ruggenmerg van de runderen verzameld en vernietigd om overdracht te voorkomen.
Het is de derde keer dat een patiënt aan variant Creutzfeldt-Jakob is overleden in Nederland. In 2005 en 2006 werd bij twee andere patiënten in Nederland variant Creutzfeldt-Jakob vastgesteld. In de gehele wereld zijn tenminste 200 personen bekend die aan de ziekte zijn overleden, waarvan 167 in Engeland. De overige slachtoffers kwamen uit Frankrijk, Ierland, Italië, Canada, Amerika, Japan, Spanje, Saudi-Arabië en Portugal.
Noot voor de redactie: Informatie over variant Creutzfeldt-Jakob kunt u vinden op de website van het RIVM.
Voor meer informatie kunt u contact opnemen met: - RIVM, Afdeling Communicatie, Marieke Timmer, (030) 274 40 49. - Nederlands CJD-Expertise Centrum Rotterdam, Erasmus MC, afdeling communicatie, David Drexhage, 010 - 703 55 25.
Er worden geen persoonsgegevens verstrekt over de patiënt.
Netherlands reports third human death from mad cow disease
The third person in the Netherlands died of the human variant of mad cow disease, Creutzfeldt-Jakob, in the beginning of January.
THE HAGUE-A third person in four years has died from the human form of mad cow disease in the Netherlands, the national health research agency RIVM said Monday.
"The patient died at the beginning of January," it announced in a press statement. "An investigation has confirmed that the patient died of the variant form of Creutzfeldt-Jakob disease."
The incurable disease is contracted from eating beef infected with BSE, also known as mad cow disease-caused by a rogue protein that proliferates in the brain and turns it spongy.
The RIVM did not say how, when or where the patient contracted the disease, but pointed out that the incubation period, from infection to the display of symptoms, could last decades.
Investigations were ongoing into the "very rare chance" that the patient may have infected other people.
The statement stressed that Dutch beef was safe, explaining that all slaughtered cows are since 2001 tested for BSE.
The RIVM said two other people have died of the disease in the Netherlands, one diagnosed in 2005 and the other in 2006.
"About 200 people have died of the disease in the world, 167 of them in Britain," said the statement.
First case of Creutzfeldt-Jakob Disease in the Netherlands The first probable case of variant Creutzfeldt-Jakob (vCJD) disease in a patient in the Netherlands was reported on April 22nd 2005 by the Dutch Ministry of Health, Welfare and Sport. The patient ... Newsitem April 26, 2005 Source: PG
Patient in the Netherlands diagnosed with variant Creutzfeldt-Jakob Disease A patient at the Mesos Medisch Centrum in Utrecht was diagnosed today with variant Creutzfeldt-Jakob Disease (vCJD), the human form of mad cow disease (BSE). Investigations into the contraction of ... Press release April 22, 2005 Source: PG
Wednesday, December 10, 2008
Evaluation of FSIS Management Controls Over Pre-Slaughter Activities (Audit Report 24601-7-KC) USDA CERTIFIED DEADSTOCK DOWNER COW SCHOOL LUNCH PROGRAM
Wednesday, August 20, 2008 Bovine Spongiform Encephalopathy Mad Cow Disease typical and atypical strains, was there a cover-up ?
A New Prionopathy OR more of the same old BSe and sporadic CJD
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]
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.
sporadic Fatal Familial Insomnia
THE PATHOLOGICAL PROTEIN
Hardcover, 304 pages plus photos and illustrations. ISBN 0-387-95508-9
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.
The Lancet Infectious Diseases, Volume 3, Issue 8, Page 463, August 2003
doi:10.1016/S1473-3099(03)00715-1Cite or Link Using DOI
Tracking spongiform encephalopathies in North America
Original TextXavier Bosch
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:
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  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?
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
2 January 2000
British Medical Journal U.S. Scientist should be concerned with a CJD epidemic in the U.S., as well
15 November 1999
British Medical Journal vCJD in the USA * BSE in U.S.
Creutzfeldt Jakob Disease
USA PRION UNIT BLOG
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 ;
CJD TEXAS (cjd clusters)
USA WRITTEN CJD QUESTIONNAIRE ???
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.
Friday, December 12, 2008
Creutzfeldt-Jakob disease (CJD) update report Emerging Infections/CJD Published on: 12 December 2008
Friday, August 29, 2008
CREEKSTONE VS USDA COURT OF APPEALS, BUSH SAYS, NO WAY, NO HOW
Sunday, March 16, 2008
MAD COW DISEASE terminology UK c-BSE (typical), atypical BSE H or L, and or Italian L-BASE
HUMAN and ANIMAL TSE Classifications i.e. mad cow disease and the UKBSEnvCJD only theory JUNE 2008
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. ...
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.
Monday, February 02, 2009 Netherlands reports third human death from mad cow disease
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