Prion-Associated Disease

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Acceptability at Recruitment

NOT ACCEPTABLE

Acceptability at CT / Work-Up

NOT ACCEPTABLE

Individual at Risk

Recipient

Explanation of Condition

Prions are a newly identified type of infectious agent, which (unlike any other type of micro-organism) do not carry any DNA or RNA. They cause a group of diseases known as spongiform encephalopathies, which include scrapie in sheep, mad cow disease (BSE) in cows, and CJD in humans.

In the 1990s, a new form of CJD (variant CJD) was identified, and has since been proven to be a human form of BSE, contracted through eating infected beef products.

Variant CJD has not, to date, been transferred by stem cell donation, but a number of cases have been transmitted by blood transfusion. CJD can also be transferred through transplant of ocular (eye) or brain (dura mater) tissues, as well as hormones derived from pituitary tissue.

See also Creutzfeldt-Jakob Disease

Guidance

Risk of transmission to recipient.

Donor Affected

Unacceptable if suffered from a prion-associated disease

Family History

Unacceptable if two or more blood relatives have, or have been told by a geneticist that they may be at risk of developing, a prion-associated disease.

Other family history should be discussed with the medical officer and transplant centre, and the opinion of a geneticist requested if necessary.

Ocular tissue or dura mater graft recipient

Unacceptable if every received an ocular tissue or dura mater graft. Any other transplants should be discussed with the medical officer.

Pituitary derived hormone therapy

Unacceptable if ever received hormones derived from human pituitary tissue.

Pseudonyms or Related Conditions

Sporadic, familial and variant Creutzfeld-Jacob disease (CJD)

Gerstmann- Sträussler-Scheinker Disease

Fatal Familial Insomnia

Kuru

Version

Version 1, Edition 1

Date of Last Update

15th June 2012