Published Date: 2001-07-06 23:50:00
Subject: PRO/AH/EDR> CJD (new var.) - UK: 9th Annual Report
Archive Number: 20010706.1293
CJD (NEW VAR.) - UK: 9TH ANNUAL REPORT
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See Also
CJD (new var.), cluster - UK (Leicestershire) (05) 20010322.0568CJD (new var.) - UK: update June 2001 20010622.1188
CJD (new var.) - UK: regional variation 20010628.1231
2000
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CJD (new var.), cluster - UK (Leicestershire) 20000715.1171
CJD (new var.), cluster - UK (Leicestershire) (04) 20001109.1963
CJD (new var.), death of elderly man - UK 20001102.1912
Date: 5 Jul 2001
From: ProMED-mail <promed@promedmail.org>
Source: Eurosurveillance Weekly, Issue 27, 5 Jul 2001 [edited
<http://www.eurosurv.org/update/>
9th Annual Report of the UK National CJD Surveillance Unit
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The latest surveillance report on the Creutzfeldt-Jakob disease (CJD)
situation, including variant-CJD [abbreviated as vCJD or CJD (new var.) in
ProMED-mail), in the United Kingdom (UK) was published last week (1) and
is available from the National CJD Surveillance Unit
(<http://www.cjd.ed.ac.uk>). The report provides a detailed overview of the
situation for all forms of CJD.
By the end of 2000, there had been 84 deaths from definite or probable vCJD
in the UK (in addition 2 probable cases died in Jan 2001 and a further 7
probable cases remained alive as at 31 Jan 2001). The estimated annual
increase in incidence of vCJD is 1.35 (95% confidence interval 1.13 to
1.61). The predicted total number of deaths for 2001, assuming this trend
continues, is 36 (21 to 58). By the end of Jun 2001 the total number of
definite and probable cases of vCJD reported in the UK had reached 102 (2).
Of the 84 deaths to the end of 2000, 75 were confirmed neuropathologically,
with a further 2 awaiting neuropathological confirmation. The clinical,
neuropathological and epidemiological features of all these cases of vCJD
are remarkably uniform and consistent with previous descriptions. A case of
neuropathologically confirmed vCJD in an individual who died aged 74 years
in Oct 1999 significantly extends the known age range for vCJD.
Analysis by standard region suggests that the incidence of vCJD in the
"North" of Great Britain may be higher than in the "South"; rate ratio
(north versus south) based on place of residence in 1991 was 1.81 (1.20 to
2.74). The mean Carstairs' deprivation score for areas of residence of
people with vCJD was 0.41 (1.02 to 0.19), which is close to the national
average of zero. Regional rates of vCJD were correlated with consumption of
other meat or meat products as classified and recorded by the Household
Food Consumption and Expenditure Survey (r=0.73), but not with similar data
from the Dietary and Nutritional Survey of British Adults. Investigators of
the 5 cases of vCJD in Leicestershire that formed a cluster concluded that
the purchase and consumption of beef in the early 1980s from butchers
shops where the meat could have been contaminated with brain tissue from
cattle affected with bovine spongiform encephalopathy (BSE) provided a
plausible explanation for the cluster (3,4).
Risk factors for the development of vCJD include age, residence in the UK,
and methionine homozygosity at codon 129 of the prion protein gene; 87
cases of vCJD with available genetic analysis have all been methionine
homozygotes. The analyses in the 9th Annual Report do not provide
conclusive evidence of an increased risk of vCJD associated with past
surgery, previous blood transfusion, occupation, or a range of dietary
factors. The power of the case-control study from which these results are
derived is, however, limited by the comparatively small number of cases and
controls. For some putative risk factors, such as blood transfusion or
surgery, it may be many years before an accurate assessment of risk can be
made because of the likely prolonged incubation periods.
From 1990 to 2000 death rates from sporadic CJD in England, Scotland,
Wales, and Northern Ireland were 0.75 per million per year, 0.86/1000
000/year, 1.00/1000 000/year, and 0.46/million/year, respectively; these
rate differences were not significant (P=0.3). The rates are similar to
those observed in other countries in Europe and elsewhere in the world,
including countries that are free of BSE. The variation seen in the
observed death rates between different parts of the UK was not significant
(P=0.5). There was no convincing evidence of space-time clustering. Since
1990 the average annual incidence of sporadic CJD has been higher than
earlier. Whether this increase in incidence is due to improved case
ascertainment or is a real reflection of an underlying incidence rise is
impossible to say with certainty.
Between 1970 and 2000, 42 cases of iatrogenic CJD have been ascertained, 6
in recipients of dura mater implants, 35 in recipients of human derived
growth hormone (hGH), and one (1) in a recipient of human gonadotrophin
(hGN). The mean age at death of the hGH/hGN group was 29 years (range 20-45
years) and for the dura mater recipients 43 years (27-59 years). The first
recognized case in a dura mater recipient died in 1979, and the first hGH
related death was in 1985.
References:
1. The National CJD Surveillance Unit. Creutzfeldt-Jakob disease
surveillance in the UK Ninth Annual Report 2000. Edinburgh: National CJD
Surveillance Unit, 2001.
(<http://www.cjd.ed.ac.uk>)
2. Monthly Creutzfeldt-Jakob disease figures. Department of Health, press
release 2 July 2001.
3. Report on Leicestershire vCJD cluster published. Eurosurveillance Weekly
2001; 5: 010322. (<http://www.eurosurv.org/2001/010322.html>)
4. Bryant G, Monk P. Summary of the final report of the investigation into
the North Leicestershire cluster of variant Creutzfeld-Jakob disease.
Leicester: Leicestershire NHS Health Authority, 2001. Available online at
<http://www.leics-ha.org.uk/>.
[Reported by Noel Gill <ngill@phls.org.uk>
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