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Chapter 26 : Biosafety of Prion Diseases

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Abstract:

Prions are unprecedented transmissible pathogenic agents that cause a group of invariably fatal neurodegenerative diseases, including scrapie (the prototype prion disease in sheep and goats), bovine spongiform encephalopathy (BSE) in cattle, chronic wasting disease (CWD) in deer and elk, and Creutzfeldt-Jakob disease (CJD) in humans. A complete list of currently known animal and human prion diseases is provided in this chapter. The biosafety issues related to prions have been addressed in several guidelines published by health authorities over the years, with recommendations to limit the potential risk associated with prion contamination in laboratory studies as well as in foods and medicinal products. In the chapter, the authors strive to sort out the truth from the myth and the legitimate from the unreasonable in relation to the biosafety issues, and provide a factual basis for concerns as well as an informed rationale for actions to be implemented. The authors also explore the potential consequences of underreaction as well as overconcern with respect to these issues. The potential transmission of prions from human to human by blood transfusion or through administration of blood components or plasma derivatives has long been a cause for concern among health professionals throughout the world. The chapter also discusses the epidemiological data regarding classical CJD and blood, and deals separately with variant of CJD (vCJD) and its implications for the safety of blood products.

Citation: Baron H, Prusiner S. 2006. Biosafety of Prion Diseases, p 461-485. In Fleming D, Hunt D (ed), Biological Safety. ASM Press, Washington, DC. doi: 10.1128/9781555815899.ch26

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Sodium Dodecyl Sulfate
0.4601065
Enzyme-Linked Immunosorbent Assay
0.4409354
Bovine Spongiform Encephalopathy
0.4372874
Chronic Wasting Disease
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Transmissible Spongiform Encephalopathies
0.43544233
Human Growth Hormone
0.4074804
0.4601065
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Kuru mortality.

Citation: Baron H, Prusiner S. 2006. Biosafety of Prion Diseases, p 461-485. In Fleming D, Hunt D (ed), Biological Safety. ASM Press, Washington, DC. doi: 10.1128/9781555815899.ch26
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References

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Tables

Generic image for table
TABLE 1

Prion diseases

Citation: Baron H, Prusiner S. 2006. Biosafety of Prion Diseases, p 461-485. In Fleming D, Hunt D (ed), Biological Safety. ASM Press, Washington, DC. doi: 10.1128/9781555815899.ch26
Generic image for table
TABLE 2

Autopsies of patients with suspected prion disease: Standard Precautions

Citation: Baron H, Prusiner S. 2006. Biosafety of Prion Diseases, p 461-485. In Fleming D, Hunt D (ed), Biological Safety. ASM Press, Washington, DC. doi: 10.1128/9781555815899.ch26
Generic image for table
TABLE 3

Autopsy suite decontamination procedures

Citation: Baron H, Prusiner S. 2006. Biosafety of Prion Diseases, p 461-485. In Fleming D, Hunt D (ed), Biological Safety. ASM Press, Washington, DC. doi: 10.1128/9781555815899.ch26
Generic image for table
TABLE 4

Brain-cutting procedures

Citation: Baron H, Prusiner S. 2006. Biosafety of Prion Diseases, p 461-485. In Fleming D, Hunt D (ed), Biological Safety. ASM Press, Washington, DC. doi: 10.1128/9781555815899.ch26
Generic image for table
TABLE 5

Tissue preparation

Citation: Baron H, Prusiner S. 2006. Biosafety of Prion Diseases, p 461-485. In Fleming D, Hunt D (ed), Biological Safety. ASM Press, Washington, DC. doi: 10.1128/9781555815899.ch26
Generic image for table
TABLE 6

Current status of iCJD worldwide

Citation: Baron H, Prusiner S. 2006. Biosafety of Prion Diseases, p 461-485. In Fleming D, Hunt D (ed), Biological Safety. ASM Press, Washington, DC. doi: 10.1128/9781555815899.ch26
Generic image for table
TABLE 7

Putative isolations of prion infective material from blood of CJD patients.

Citation: Baron H, Prusiner S. 2006. Biosafety of Prion Diseases, p 461-485. In Fleming D, Hunt D (ed), Biological Safety. ASM Press, Washington, DC. doi: 10.1128/9781555815899.ch26
Generic image for table
TABLE 8

Bovine derivatives in pharmaceuticals

Citation: Baron H, Prusiner S. 2006. Biosafety of Prion Diseases, p 461-485. In Fleming D, Hunt D (ed), Biological Safety. ASM Press, Washington, DC. doi: 10.1128/9781555815899.ch26
Generic image for table
TABLE 9

Categories of infectivity in bovine tissues and body fluids

Citation: Baron H, Prusiner S. 2006. Biosafety of Prion Diseases, p 461-485. In Fleming D, Hunt D (ed), Biological Safety. ASM Press, Washington, DC. doi: 10.1128/9781555815899.ch26

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