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Variant Creutzfeldt Jakob disease

Bovine spongiform encephalopathy (BSE), also known as mad cow disease, is a transmissible spongiform encephalopathy that is responsible for the human neurodegenerative disorder known as variant Creutzfeldt-Jakob disease (vCJD). BSE first appeared in cattle in the United Kingdom (UK) in 1986. The origin of BSE is thought to be cattle feed containing meat & bone meal supplements contaminated by scrapie infected sheep carcasses. Similarly, vCJD was transmitted to humans through consumption of beef products contaminated by infected neural tissue, such as hot dogs, sausages, lunchmeat, meat pies and various canned meat goods. BSE infections in the UK have decreased substantially since 1992 due to slaughter of infected animals and changes in animal feed production. BSE has not occurred in the US or other countries that do not import live cattle, beef products, or livestock nutritional supplements from the UK. In December 2003, the United Kingdom Health Secretary reported the world’s first possible case of variant Creutzfeldt Jakob disease (vCJD) by transfusion of red blood cells. The blood donor, who was free of symptoms at the time of donation, donated in March 1996.

The incubation period of vCJD is not known. If large numbers of infected persons are silently incubating the disease, human to human iatrogenic spread may be possible .The disease could unknowingly be spread during invasive medical and surgical procedures and donation of organs, tissues and blood.

No sensitive screening tests currently exist. The FDA has taken several measures to prevent future transmission. In 1999, FDA mandated that blood centers must defer donors who:

  • Spent greater than 3 months in the United Kingdom from 1980 through 1996
  • Lived on a military base in Europe between 1980 and 1996
  • Were transfused in the United Kingdom since 1980
  • Lived more than 5 years in Europe
  • Been injected with bovine insulin since 1980
  • Have a family history of CJD
  • Been a recipient of a dura mater graft or human pituitary derived growth hormone.

Since 1998, the UK has used only leukocyte reduced blood because experts on prion diseases consider white blood cells to be a potential source of infection.

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