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The spongiform encephalopathies: prion diseases
1Veterans Affair Medical Center, Cincinnati, Ohio, USA.
Abstract:
The spongiform encephalopathies may be caused by prions, infectious pathogens that differ from all other infectious agents in that they do not have deoxyribonucleic acid (DNA) or ribonucleic acid (RNA). Very difficult to inactivate, they are composed of an abnormal protein. It is believed by many that prions cause sporatic and genetic neurodegenerative diseases, including scrapie and bovine spongiform encephalopathy in animals and kuru, fatal familial insomnia, Creutzfeldt-Jakob disease (CJD) and Gerstmann-Straussler-Scheinker disease in humans. Another, the new variant CJD in humans in England, is an example of a breech in the species barrier between humans and animals. Transmitted primarily via exposure to infected brain or spinal cord tissue or blood, there have been numerous iatrogenic cases from contaminated pituitary hormones, surgical equipment, dural grafts, corneal transplants and others. All facets of blood product manufacturing have been affected. Nurses should be aware of the latest developments, and able to practice infection control while providing the best patient/family information possible.
Insights
Prions, infectious agents lacking DNA or RNA, cause spongiform encephalopathies. These abnormal proteins are difficult to inactivate and linked to fatal neurodegenerative diseases in humans and animals.
Area of Science:
- Neurology
- Infectious Diseases
- Biochemistry
Background:
- Spongiform encephalopathies are a group of fatal neurodegenerative diseases.
- These conditions are linked to infectious agents known as prions.
Observation:
- Prions are unique infectious pathogens composed of abnormal proteins, lacking nucleic acids (DNA or RNA).
- They are highly resistant to inactivation methods.
- Prions are implicated in various animal and human diseases, including scrapie, bovine spongiform encephalopathy, kuru, fatal familial insomnia, and Creutzfeldt-Jakob disease (CJD).
Findings:
- The new variant CJD in England demonstrates a species barrier breach between animals and humans.
- Transmission occurs through exposure to infected neural tissues or blood.
- Iatrogenic transmission is documented via contaminated medical products and procedures.
Implications:
- Healthcare professionals, particularly nurses, must stay informed about prion disease advancements.
- Effective infection control measures are crucial in healthcare settings.
- Patient and family education regarding prion diseases is essential for care and management.