Hostname: page-component-77f85d65b8-6bnxx Total loading time: 0 Render date: 2026-03-28T06:31:58.417Z Has data issue: false hasContentIssue false
  • English
  • Français

Prions and the ENT surgeon

Published online by Cambridge University Press:  29 June 2007

Adam Frosh
Adam Frosh*
Affiliation:
Department of Otorhinolaryngology, St Mary's Hospital, London, UK.
*
Address for correspondence: Adam Frosh, Department of Otorhinolaryngology, St Mary's Hospital, Praed Street, London W2 1NY. e-mail: a.frosh@ic.ac.uk
Get access Rights & Permissions [Opens in a new window]

Abstract

Recent developments in our understanding of prion diseases have raised concerns for the public health. There is now compelling evidence that the transmissible agent for variant Creutzfeldt-Jakob disease (vCJD) in affected individuals is accumulated in lymphoreticular tissues such as the appendix and tonsils. This agent demonstrates a remarkable resistance to standard methods of sterilisation used in hospital sterile services departments. The possible implications this has on the safety of surgical instruments in ENT and other surgical practice is discussed. This review also outlines the history of our understanding of prion diseases and describes the development of a diagnostic test for vCJD in the living patient by pharyngeal tonsil biopsy.

Keywords

PrionsSurgical instruments, adverse effectsCreutzfeldt-Jakob syndromeSpongiform encephalopathyTonsillectomy

Information

Type
Review Article
Information
The Journal of Laryngology & Otology , Volume 113 , Issue 12 , December 1999 , pp. 1064 - 1067
Copyright
Copyright © JLO (1984) Limited 1999

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

Article purchase

Temporarily unavailable

Footnotes

Paper presented at the Section of Laryngology, Royal Society of Medicine, London, May 1998.

References

Advisory Committee on Dangerous Pathogens-Spongiform Encephalopathy Advisory Committee (1998) Transmissible Spongiform Encephalopathy Agents: Safe Working and the Prevention of Infection. The Stationary Office, London.Google Scholar
Alpers, M. P. (1965) Epidemiological changes in kuru, 1957 to 1963. In Slow, Latent and Temperate Virus Infections (Gajdusek, D. C., Gibbs, C. J. Jr, Alpers, M. eds.). National Institute of Neurological Diseases and Blindness, National Institutes of Health, Public Health Service, US Department of Health, Education, and Welfare, Washington. pp 65–82Google Scholar
Bernoulli, C., Siegfried, J., Baumgartner, G., Regli, F., Rabinowicz, T., Gajdusek, D. C., Gibbs, C. L. Jr., (1977) Danger of accidental person-to-person transmission of Creutzfeldt-Jakob disease by surgery. Lancet i: 478–479.CrossRefGoogle Scholar
Bruce, M. E., Will, R. G., Ironside, J. W., McConnell, I., Drummond, D., Suttie, A. et al. (1997) Transmissions to mice indicate that ‘new variant’ CJD is caused by the BSE agent. Nature 389: 498–501.CrossRefGoogle ScholarPubMed
Centers for Disease Control (1989) Update: Creutzfeldt-Jakob disease in a second patient who received a cadaveric dura mater graft. Morbidity and Mortality Weekly Report 38: 37–38, 43.Google Scholar
Collinge, J., Sidle, K. C. L., Meads, J., Ironside, J., Hill, A. F. (1996) Molecular analysis of pnon strain variation and the aetiology of ‘new variant’ CJD. Nature 383: 685–690.CrossRefGoogle Scholar
Collins, S., Law, M. G., Fletcher, A., Kaldor, J., Masters, C. L. (1999) Surgical treatment and risk of sporadic CreutzfeldtJakob disease: a case-control study. Lancet 353: 693–697.CrossRefGoogle ScholarPubMed
Duffy, P., Wolf, J., Coffins, G., DeVoe, A. G., Streeten, B., Cowen, D. (1974) Possible-person-to person transmission of Creutzfeldt-Jakob disease. New England Journal of Medicine 290: 692–693.Google ScholarPubMed
Fraser, H., Bruce, M. E., Davies, D., Farguhar, C. F., McBride, P. A. (1992) The lymphoreticular system in the pathogenesis of scrapie. In Prion Diseases of Humans and Animals. (Prusiner, S. B., Collinge, J., Powell, J., Anderson, B., eds.). Ellis Horwood: London pp 308–317.Google Scholar
Frasier, S. D., Foley, T. P. (1994) Creutzfeldt-Jakob disease in recipients of pituitary hormones. Journal of Clinical Endocrinology and Metabolism 78: 1277–1279.Google ScholarPubMed
Gajdusek, D. C., Gibbs, C. J. Jr., Alpers, M. (1966) Experimental transmission of kuru-like syndrome to chimpanzees. Nature 209: 794–796.CrossRefGoogle ScholarPubMed
Hadlow, W. J. (1959) Scrapie and kuru. Lancet 2: 289–290CrossRefGoogle Scholar
Hill, A. F., Desbruslais, M., Joiner, S., Sidle, K. C. L., Gowland, J., Coffinge, J. (1997a) The same prion strain causes Vcjd and Bse. Nature 389: 448–450CrossRefGoogle ScholarPubMed
Hill, A. F., Zeidler, M., Ironside, J., Collinge, J. (1997b) Diagnosis of new variant Creutzfeldt-Jakob disease by tonsil biopsy. Lancet 349: 99–100.CrossRefGoogle ScholarPubMed
Hill, A. F., Butterworth, R. J., Joiner, S., Jackson, G., Rossor, M. N., Thomas, D. J. et al. (1999) Investigation of variant Creutzfeldt-Jakob disease and other human prion diseases with tonsil biopsy samples. Lancet 353: 183–189.CrossRefGoogle ScholarPubMed
Hospital Episode Statistics, Financial year 1993/4. Vol. 1, Department of Health.Google Scholar
Jackson, G. S., Hosszu, L. L. P., Power, A., Hill, A. F., Kenney, J., Saibil, H. et al. (1999) Reversible conversion of monomeric human prion protein between native and fibrilogenic conformations. Science 283: 1935–1937.CrossRefGoogle ScholarPubMed
Klatso, I., Gajdusek, D. C., Zigas, V. (1959) Pathology of Kuru. Laboratory Investigation 8: 799–847.Google Scholar
Prusiner, S. B., Groth, D. F., Bolton, D. C., Kent, S. B., Hood, L. E. (1984) Purification and structural studies of a major scrapie prion protein. Cell 38: 127–134.CrossRefGoogle ScholarPubMed
Wilesmith, J. W., Wells, G. A. H., Cranwell, M. P., Ryan, J. B. (1988) Bovine spongiform encephalopathy: epidemiological studies. Veterinary Record 123: 638–644.Google ScholarPubMed
Will, R. G., Ironside, J. W., Zeidler, M., Consens, S. N., Estibeiro, K., Alperovitch, A. et al. (1996) A new variant of Creutzfeldt-Jakob disease in the UK. Lancet 347: 921–925.CrossRefGoogle ScholarPubMed