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New Variant CJD Cases

Lord Lucas asked Her Majesty's Government:

Lord Hunt of Kings Heath: To protect patient confidentiality, precise details cannot be supplied but the following information may be helpful:

Table 1; variant Creutzfeldt Jakob Disease (vCJD) cases in the UK, by month and year of death, date of onset of symptoms and date vCJD was confirmed

Date of deathDate of onset of symptomsDate vCJD confirmed

* Cases not confirmed neuropathologically and therefore classed as 'probable vCJD'

Table 2: vCJD cases in the UK, by age at death

Age at deathNumber of cases

Forty-nine of the fifty-two "definite" cases to date have been methionine/methionine homozygous at codon 129 for the prion protein. Results on one of the remaining cases are still awaited, and in the remaining two cases no blood was taken on which to conduct the appropriate genetic tests.

To date the National CJD Surveillance Unit has no record of a case of variant Creutzfeldt Jakob disease having arisen as a consequence of medical or surgical intervention.

Abnormal Prions in Blood: Tests

Lord Lucas asked Her Majesty's Government:

    What progress has been made on evaluating the capillary electrophoresis test, developed by Mary Jo Schmerr of the National Animal Disease Centre of the United States Department of Agriculture, for abnormal prions in blood; what tests are under way; and when it is expected that they will be completed.[HL1546]

Lord Hunt of Kings Heath: The test currently under development by Dr Schmerr has undergone some preliminary evaluation at: the Medical Research Council Prion Unit, London; the National Creutzfeldt-Jakob Disease Surveillance Unit, Edinburgh; and the Veterinary Laboratory Agency, Weybridge. An application for a full evaluation of this, and other, blood tests is being considered by the Government. Before a diagnostic test such as this can be used to determine whether people or animals are infected with a transmissible encephalopathy agent at a pre-clinical stage, it will be necessary for the test to be fully developed and scientifically validated. We are not yet able to predict when any of these blood tests currently being developed will be completed.

Lord Lucas asked Her Majesty's Government:

    What progress has been made on the programme of screening tonsils removed in routine operations for the presence of abnormal prions; and whether they will publish the results of the screening to date.[HL1547]

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Lord Hunt of Kings Heath: Two retrospective studies to examine samples of tonsil and appendix tissue taken from 15,000 patients in the South West of England and 3,000 in the Lothian Region of Scotland for the presence of abnormal prions commenced in the spring of 1999. The first preliminary results from these studies are likely to be available during the spring and will then be considered by the Department of Health, the Medical Research Council and relevant scientific experts including the Spongiform Encephalopathy Advisory Committee. An announcement will be made about the findings.

An additional prospective study proposed by the London Prion Unit at St Mary's Hospital, to look at 2,000 tonsil specimens, has recently obtained ethical approval from the London Multi-centre Regional Ethical Committee and this study should be under way shortly.

TSE: Transmission

Lord Lucas asked Her Majesty's Government:

    Whether they are aware of any cases in which it appears possible that a Transmissible Spongiform Encephalopathy has been transmitted by a human mother to her child in utero; and, if so, what is their current state of knowledge of such cases.[HL1525]

Lord Hunt of Kings Heath: The National CJD Surveillance Unit monitors the onset of all cases of Creutzfeldt Jakob disease in the United Kingdom. No confirmed or probable case has been reported to the Unit of a baby being affected by this disease.

Epidemiological evidence from Kuru--a human TSE occurring amongst the Fore people of Papua New Guinea--suggests that human maternal transmission of TSE disease does not occur. However animal studies have shown maternal transmission can occur in scrapie in sheep and there is evidence of a low rate of vertical transmission of BSE in cattle. We will continue to liaise closely with scientists at the National CJD Surveillance Unit in Edinburgh to monitor the onset of all cases of CJD and seek to identify any common features.

Lord Lucas asked Her Majesty's Government:

    Whether there is any evidence that any Transmissible Spongiform Encephalopathy in any species can be transmitted through blood; and whether they will place in the Library of the House copies of the principal relevant scientific papers.[HL1545]

Lord Hunt of Kings Heath: Some animal studies have shown that certain transmissible spongiform encephalopathies can be experimentally transmitted from animal to animal through blood components. However, the Spongiform Encephalopathy Advisory Committee at its February meeting reviewed recent research undertaken in this area and did not consider

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any measures were necessary, in addition to those already in place, to reduce any potential risk to public health from human blood and blood products.

Copies of the following relevant scientific papers are being placed in the Library.

    Brown P. 1995, Can Creutzfeldt-Jakob Disease be transmitted by Transfusion? Haematology 2: 472-477.

    Brown et al 1999, Further studies of blood infectivity in an experimental model of transmissible spongiform encephalopathy, with an explanation of why blood components do not transmit Creutzfeldt-Jakob disease in humans. Transfusion Vol. 39, November/December 1169-1178.

Lord Lucas asked Her Majesty's Government:

    Whether there is the potential for transmission of Transmissible Spongiform Encephalopathies through the use of inadequately sterilised dentists' instruments.[HL1524]

Lord Hunt of Kings Heath: Although the possibility of person to person transmission of any theoretical Transmissible Spongiform Encephalopathy infectivity through the use of surgical instruments cannot be ruled out, the National CJD Surveillance Unit, as they investigate cases of CJD, undertake an examination of medical histories to look for common risk factors. Analyses of cases to date do not provide evidence to suggest any increased risk with past dental surgical intervention. Nonetheless, guidance has been formulated on appropriate prudent practice, which is available from the BDA, and which takes into account advice from the Joint Working Group of the Spongiform Encephalopathy Advisory Committee and the Advisory Committee on Dangerous Pathogens. In addition, a Health Service Circular on best practice on decontamination of medical devices was issued in August 1999 to healthcare organisations, including Dental Postgraduate Deans and Regional Dental Advisors.

Lord Lucas asked Her Majesty's Government:

    Whether Mr Rod Griffiths, the West Midlands director of public health, has been correctly quoted as saying (in respect of instruments used in subsequent operations having been used on a woman with new-variant CJD) that the risk to other women was vanishingly small, as the prion that caused CJD was removed simply by washing before sterilisation; and if so, whether they agree with him.[HL1548]

Lord Hunt of Kings Heath: We are unable to comment on the accuracy of the reporting of any statement by Professor Griffiths. However the George Eliot Hospital NHS Trust has advised that Caesarean section itself is considered to be low risk surgery for transmission. The Government agree with advice from the Spongiform Encephalopathy Advisory Committee that rigorous washing, decontamination and general hygiene practices with regard to surgical instruments

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are key measures in minimising the risk of any infection being transferred from patient to patient.

To date, there is no known case of variant Creutzfeldt Jakob Disease having arisen as a consequence of medical or surgical intervention.

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