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Foreign Sources of Infection To−Vi Nguyen S Foreign Infection S Preventable environmental source of infection S Remove infectious material, epidemic outbreaks disappear S Ex. BSE & Kuru Infectious Forms of Prion Diseases in Humans S Kuru S Iatrogenic CJD S ddVariant CJD Kuru S Fore tribe from Papua New Guinea transmitted kuru through ritualistic cannibalism S Ate dead relatives S Only exposed to infectious agent if they consumed or came in contact with brain tissue or spinal fluid Iatrogenic S Prion tainted human growth hormone and gonadotropin S Dura mater grafts S Transplants of corneas obtained from people who died of CJD S Neurosurgical procedures in which ineffectively sterilized depth electrodes or instruments were used vCJD S Results from prions being transmitted from cattle with BSE to humans through consumption of contaminated beef products S All vCJD individuals were identified to express methionine methionine homozygously at codon 129 S Except a single case of vCJD in a patient who was heterozygous at codon 129 S Raised possibility of a second wave of BSE related deaths Spread of BSE S Late 1970s, hydrocarbon solvent extraction method used in rendering offal was abandoned S Resulted in meat and bone meal with a much higher fat content S Fed to cattle, sheep, pigs, and chickens S Allowed scrapie prions from sheep or low levels of bovine prions generated sporadically to survive the rendering process S Resulted in widespread infection of cattle CWD S White tailed deer and elk have developed CWD S Only prion disease known in free ranging animals Genetics vs. Environment S 1930 High incidence of familial CJD in some families was known S 60 more years would pass before significance was appreciated S Once shown to be an infectious disease, little attention was paid to the familial form of the disease S How do you think the course of scientific investigation might have proceeded had transmission studies not been performed until after the molecular genetic lesion was discovered? S If we discovered the molecular genetic lesion before the transmission studies were performed, the prion concept may have been more readily accepted S Prion concept explains how a single disease can have a genetic or infectious etiology S Because the transmissibility was discovered first, there was more skepticism as to whether prions were a whole new infectious agent