Creutzfeld-Jakob Disease and Other Human Transmissible Spongiform Encephaelopathies
Guidelines on patient management and infection control.
This report was prepared by a Working Party of the National Health Advisory Committee of the National Health and Medical Research Council.
National Health and Medical Research Council
NHMRC
4. Patient Risk status 5,49,63,64
From a practical point of view, it is convenient to divide the population at risk of spreading CJD into two groups, the rationale being that higher concentrations of infectivity are likely to be present in individuals grouped at high risk. More stringent infection controls are therefore needed when decontaminating instruments or performing medical procedures.
Group 1: High-risk patients
This group includes:
- patients with proven CJD:
- patients with clinically suspected CJD;
- asymptomatic carries of pathogenic mutations of PrP occurring within the context of familial CJD/ GSS/ FFI; and
- all members of a family with familial CJD/ GSS/ FFI in whom the PrP genotype is underestimated or uncertain5.
Group 2: Low-risk patients
This group includes:
- any patient with undiagnosed progressive neurological illness with or without dementia;
- all members of a family in which there is a strong family history of undiagnosed dementing/ neurological illness;
- recipients of human pituitary hormones (growth hormone and gonadotrophins); and
- recipients of dura mater grafts or persons with a record of transdural neurosurgical operation between 1972 and 1989 (since 1987, the sourcing of dura mater for grafting has been more rigidly controlled and neurosurgeons have found substitutes for dural homografts).
- Obviously if it can be ascertained by consultation with the operating neurosurgeon that dura mater grafting was not used in a particular individual then that person would not fall into this Group 2 category.
11. Organ, tissue and blood donations; therapeutic usage of products of human origin
The following person should be excluded from the routine donation of organs, tissues and blood:
- persons in risk Groups 1 or 2;
- persons who die in psychiatric hospitals; and
- persons who die with any obscure undiagnosed neurological disorder2,41
It might be argues that tissue donation from the low risk group of persons could proceed if:
- the recipient were elderly;
- the recipient were fully informed of the potential risk; and
- the purpose of the transplant procedure were a matter of life or death for the recipient.
Insitutional ethics committees should examine this question and reach their own decision.
Persons who are responsible for recruiting organ/ tissue donors and for the banking of tissues (eg corneas, heart valves, skin) should be aware of the problem of CJD, and should have exclusionary criteria and procedures in place.
In all cases where materials for transplantation, grafting or tissue banking are derived from post morten material, it is mandatory that the brain of the donor be assessed and cleared by a specialist neuropathologist. Parrafin blocks of brain tissue on such donors should be archived for future reference.
Materials from the above patient groups (Section 11, paragraph 1) should not be used for the preperation of any therapeutic products or laboratory reagents (eg thromboplastin or Kveim test material)30,31 . The possible illegal use of contaminated products should also be borne in mind30.
December 1995
Date of protocol: August 2002
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