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Over the past 2 decades, this conversion property of [PrP.sup.Sc] has been exploited to develop sensitive biofluid diagnostics for both vCJD (3) and sCJD (4).
vCJD has only been neuropathologically confirmed in persons homozygous for Met at residue 129 of human PrP (21), with 1 exception of heterozygosity (Met/Val) at this codon (22).
Disturbing images of cattle suffering mad cow's disease during the mid-1990s and a small number of vCJD cases in humans triggered a national health scare.
These include Kuru and iatrogenic (medically--acquired) CJD and Variant CJD (vCJD) (Ryan, R.
But it's not just eating meat, blood transfusion and medical equipment that has been linked to vCJD.
Figures from the Health Protection Agency show there have been 176 cases of vCJD from since it was first detected in humans in 1995.
The safety of the blood supply is a concern, especially in places like the United Kingdom, because of five documented cases of transmission of vCJD via blood transfusions from donors with subclinical vCJD, NIAID study investigator Byron Caughey, Ph.D., said in an interview.
A better test could also help to expedite the diagnosis of vCJD in people, who often get diagnosed very late in the course of the disease - if at all - and usually survive for less than a month after diagnosis, he said in an interview.
A further four people with either definite or probable vCJD are still alive.
Until now, there has been no way of telling if someone has vCJD except by examining their brain tissue.
The disaster never materialised, and since 1994 only around 200 cases of vCJD have been confirmed worldwide.
Based on an updated analysis of the risk, the FDA "continues to believe" that the risk of variant Creutzfeldt-Jakob (vCJD) to patients who receive U.S.-licensed plasma-derived coagulation factor VIII products "is likely to be extremely small, although we do not know the risk with certainty."
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