Attributable testing for abnormal prion protein, database linkage, and blood-borne vCJD risks

Area of Science:

• Neuroscience
• Infectious Disease Epidemiology
• Public Health Surveillance

Background:

• Variant Creutzfeldt-Jakob disease (vCJD) poses a public health risk through blood-borne and surgical instrument transmission.
• The UK initiated national tonsillar tissue collection in 2004 for anonymous prion protein testing.
• Current UK practices lack attributable autopsy testing for vCJD, hindering risk assessment for transfusion recipients.

Purpose of the Study:

• To evaluate the necessity and feasibility of attributable prion protein testing in autopsy specimens for vCJD surveillance.
• To address the limitations in tracking vCJD transmission risks in individuals exposed via blood transfusion or surgical instruments.
• To explore ethical and legal considerations for enhanced vCJD surveillance in the UK.

Main Methods:

• Analysis of existing national prospective tonsillar tissue collection protocols in the UK.
• Comparison with Swiss surveillance strategies including unconsented autopsy testing and consented tonsillar tissue testing.
• Review of vCJD transmission risks associated with blood products and surgical instruments.
• Consideration of cost-effectiveness of surveillance options based on animal prion disease data.

Main Results:

• The UK's anonymous tonsillar tissue testing contrasts with Switzerland's attributable autopsy testing for subclinical vCJD.
• Blood-borne vCJD transmission cases in the UK necessitate improved methods for recipient follow-up.
• Ethical and legal barriers may impede the implementation of attributable PrP(SC) testing in the UK.

Conclusions:

• Attributable autopsy testing for abnormal prion protein (PrP(SC)) is crucial for quantifying vCJD transmission risks in the UK.
• Enhanced surveillance, including attributable testing, is needed to manage and potentially interrupt human-to-human vCJD transmission.
• Addressing ethical and legal impediments is essential for implementing effective vCJD surveillance strategies.