983-Prion Diseases

Course # DL-983: Prion Diseases

by Rebecca A. Rosser, MS,MBA, CLS, MT(ASCP)DLM - Educational and Development Consultant - Laboratory - Kaiser Permanente - SCPMG Regional Reference Laboratory

Approved for 1.0 CE
Level of Difficulty: Intermediate

Introduction:

The headlines in Britain shouted messages such as “Bring Back the Beef,” and “Scientists baffled by mystery of new BSE cases.” In 1985 an epidemic of Mad Cow Disease began devastation in the beef industry in the United Kingdom that jolted the world. A disease among the cows was diagnosed as being caused by a transmissible spongiform encephalopathy (TSE), also known as a prion disease. TSEs are responsible for a number of animal and human conditions that were first thought to be caused by a virus. Research has indicated that the causative infectious agent does not contain a nucleic acid genome, therefore cannot be a virus. Others speculate that the agent is a virino, which is a small non-coding regulatory nucleic acid coated with a host-derived protective protein. Still others believe the agent is a prion. In both humans and animals this agent causes progressive brain damage and ultimately death.

Animal TSE can affect cows in the form of Bovine Spongiform Encephalopathy (BSE), also known as “Mad Cow Disease.” BSE is most prevalent in the United Kingdom and has caused the government to dispose of hundreds of thousands of infected cattle, causing a crisis in the beef industry. In 1995 a few cases of TSEs began appearing in young adults in England. These cases were a variant of Creutzfeldt-Jakob Disease and were ascribed to eating infected meat from BSE cattle. Other TSEs are found in animals as well as in humans. While these diseases, in humans, are rare, they are always fatal.

Objectives:

After completing this course the participant will be able to:

1. define Transmissible Spongiform Encephalopathies

2. discuss prions

3. describe animal TSE including Scrapie, Chronic Wasting Disease, and Bovine Spongiform Encephalopathy

4. describe human TSE including Kuru, Gerstmann-Sträussler-Scheinker Disease, Fatal Familial Insomnia, Alpers’ Disease, Creutzfeldt-Jakob Disease, and variant Creutzfeldt-Jakob Disease

5. describe sterilization and disinfection practices

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