Creutzfeldt-Jakob virus

Creutzfeldt-Jakob virus (Osborn, 1988). Therefore, a battery of tests for relevant viruses and other microorganisms is a routine procedure in the production of biological drugs. 

Kidson, C., Moreau, M. C., Asher, D. M., Brown, P. W., Coon, H. G., Gajdusek, D. C., and Gibbs, C. J., 1978, Cell fusion induced by scrapie and Creutzfeldt-Jakob virus-infected brain preparations, Proc. Natl. Acad. Sci. USA 75 2969. 

It overcomes problems of product safety. Direct extraction of product from some native biological sources has, in the past, led to the unwitting transmission of disease. Examples include the transmission of blood-borne pathogens such as hepatitis B and C and human immunodeficiency virus (HIV) via infected blood products and the transmission of Creutzfeldt-Jakob disease to persons receiving human growth hormone (GH) preparations derived from human pituitaries. 

The prion diseases are a closely related group of neuro-degenerative conditions which affect both humans and animals. They have previously been described as the subacute spongiform encephalopathies, slow virus diseases and transmissible dementias, and include scrapie in sheep, bovine spongiform encephalopathy (BSE) in cattle, and the human prion diseases, Creutzfeldt-Jakob disease (CJD), Gerstmann-Straussler-Scheinker disease (GSS), fatal familial insomnia (FFI) and kuru. Prion diseases are... 

Up through 1980, people thought that all infectious diseases were caused by microbes of some sort. They knew about diseases caused by viruses, bacteria, protozoa, and fungi. There were some strange diseases, however, for which no one had isolated and cultured the pathogen. Creutzfeldt Jakob Disease (CJD) in humans, scrapie in sheep, and transmissible encephalopathy in mink (TME) all involved a slow, gradual loss of mental function and eventual death. The brains of the victims all showed unusual plaques of amyloid protein surrounded by spongelike tissue. 

Infectious disorders Chronic meningitis Tuberculosis, fungal, parasitic HIV disease Tertiary syphilis Slow virus (Creutzfeldt-Jakob disease) Treatment of infection... 

Brown P, Rohmer RG, Gajduseck DC. Sodium hydroxide decontamination of Creutzfeldt-Jakob disease virus. N Engl Med 1984 320 111. 

Fatal viral infections resulting from the use of contaminated human tissue or fluids as components of medicines are well recorded. Examples of this include human immunodeficiency virus (HIV) infection of haemophiliacs by contaminated and inadequately treated factor VIII products made from pooled human blood, and Creutzfeldt-Jakob disease (CJD) from injections of human growth hormone derived from human pituitary glands, some of which were infected. 

Gibbs CJ Jr, Gajdusek DC, Amyx H (1979) Strain variation in the viruses of Creutzfeldt-Jakob disease and kuru. In Prusiner SB, Hadlow WJ (eds) Slow transmissible diseases of the nervous system, vol 2. Academic, New York, pp 87-110... 

Many other possible causes of dialysis dementia have been proposed. These inclnde other trace element contaminants, normal pressure hydrocephalus, slow virus infection of the central nervous system, and regional alterations in cerebral blood flow (Arieff, 1990). Slow virus infection of the nervous system is a possible etiology for dialysis dementia. The clinical manifestations resemble those of other slow virus infections, such as Kuru or Creutzfeldt-Jakob disease (Selkoe, 1978 4714 Gajdusek, 1985 1662). 

Prusiner, Stanley Ben (b. 1942) American neurologist and biochemist who was the first scientist since the discovery of viruses to detect an entirely new infective agent. Prusiner, as a young neurology resident, was in charge of a patient who died of Creutzfeldt-Jakob disease (CJD). He decided to research the cause. Ten years later, he isolated small protein bodies, which he called prions, and showed that these were the cause of CJD and of the similar bovine spongiform encephalopathy. He was awarded the Nobel Prize in physiology or medicine in 1997. 

Despite cases of transmission of hepatitis C associated with intravenous immunoglobulin in the 1990s, no cases of transmission of hepatitis, HIV, or Creutzfeldt-Jakob disease have since been reported with immunoglobulins [6 J. Before 1996, PCCs (prothrombin complex concentrates) were associated with minimal risk of transmission of infective agents [7 ]. There are no documented cases of viral transmission in patients with von Willebrand disease or hemophilia A treated with Haemate P/Humate P in over 25 years of clinical experience in Europe and more than 17 years in the USA [ ]. In the IMPACT-1 and IMPACT-2 trial in 124 patients there were no cases of HIV, hepatitis, or human B19 virus conversion. Furthermore, no cases of viral transmission have been reported during 30 years of post-marketing surveillance of Cl-esterase inhibitor concentrate [9, 10 ]. 

The transmissibility of Kuru, Scrapie and Jakob-Creutzfeldt disease demonstrated by long-term experiments, has given new impetus to this. The failure to discover virus particles in several EM studies [209, 210, 211, 119] would not militate against this. In scrapie, virus-like particles have been demonstrated only recently [212], while several previous studies had been... 

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