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From: TSS ()
Subject: Creutzfeldt-Jakob disease (CJD) update Reports of incidents of potential iatrogenic exposure
Date: May 11, 2007 at 12:14 pm PST

Creutzfeldt-Jakob disease (CJD) update report

This six-monthly report provides an update on reports of incidents of
potential iatrogenic (healthcare-acquired) exposure to Creutzfeldt-Jakob
disease (CJD) via surgery, and on the National Anonymous Tonsil Archive.
Data are correct as of 30 April 2007.

For numbers of CJD case reports, readers should consult data provided by the
national CJD Surveillance Unit (NCJDSU), Edinburgh [1]. The latest yearly
analysis of vCJD reports (onsets and deaths) is also available from the
NCJDSU website [2].

Reports of incidents of potential iatrogenic exposure to CJD via surgery
01January 2000 to 30 December 2006
There were a total of 306 incidents reported during this period (table 1).
Twenty-five surgical incidents were reported in the second half of 2006
(since the previous update report). Surgical incidents occur when
instruments considered potentially contaminated with the CJD agent during
use on an index patient have been subsequently re-used on other patients. A
patient whose surgery results in potential contamination of instruments with
prions is referred to as the index patient. Table 1 shows the number of CJD
surgical incidents reported to the CJD Incidents Panel [3] from January 2000
to December 2006 by the diagnosis of the index patient.

Table 1 CJD Surgical Incidents (n=306) reported to the CJD Incidents Panel,
by diagnosis of index patient: January 2000 to December 2006


CJD Status of index patient
2000
2001
2002
2003
2004
2005
2006
Total

Sporadic (possible, probable or definite)
7
19
21
23
17
17
26
130 (42%)

vCJD (possible, probable or definite)
6
14
22
5
5
1
2
55 (18%)

Familial inlcuding 'at risk' familial

2
2
7
1
3
6
21 (7%)

'At risk' vCJD blood component recipient




3
10
5
18 (6%)

'At risk' - vCJD plasma product recipient

1
2

9
17
7
36 (12%)

'At risk' - other


3
2
1
2
4
12 (4%)

CJD type unclear/ CJD unlikely
1
1

4
1
1
2
10 (3%)

Not CJD
2
1
4
7
4
1

19 (6%)

Other


1
1
1
1
1
5 (2%)

Total
16
38
55
49
42
53
53
306 (100%)


Investigation of surgical incidents may result in advice to remove surgical
instruments from clinical use (to quarantine, destroy, or donate for
research). Such advice is generally only given for instruments considered to
be potentially contaminated with the CJD agent that have not undergone a
certain number of cycles of use and decontamination since their use on an
index patient. Hospitals are asked to consider sending any instruments to be
permanently removed from use to the Surgical Instrument Store (held by the
Health Protection Agency, Porton Down) for research. In the second half of
2006, there were seven incidents in which instruments were permanently
removed from use.

The Panel may advise contacting and informing some patients of their
possible exposure to CJD in a surgical incident. Such advice is generally
only given for patients who have definitely been exposed to potentially
contaminated instruments which have been used on risk tissues in certain
index patients. The Panel advises that these patients should be considered
‘at-risk of CJD for public health purposes' and asked to take certain
precautions (ie, not to donate blood or other tissues and to inform their
medical and dental carers prior to any invasive procedures) in order to
reduce the risk of transmitting the CJD agent to other patients. Since 2000,
17 incidents have given rise to advice to contact and inform subsequent
patients of their potential exposure to CJD (table 2). The Panel has advised
that a total of 70 patients should be contacted and informed that they are
‘at-risk' of CJD for public health purposes. Fourteen patients have been
subsequently re-assessed, and based on updated risk assessments are no
longer considered to be ‘at-risk' of CJD for public health purposes.


Table 2 Panel advice to inform patients that they are ‘at-risk' of CJD/vCJD:
1 January 2000 to 31 December 2006

Diagnosis of index patient
Procedure on index patient
Number of Incidents
Number of 'at risk' patients (subsequently denotified)

Sporadic CJD


Brain biopsy
2
27 (–)

Cataract surgery
9
29 (11)

vCJD


Appendicectomy
1
2 (–)

Cataract surgery
1*
2 (1)

'at risk' vCJD
Endoscopy & GI surgery
4†
10 (2)

Total

17
70 (14)


*The index patient was a blood component recipient with evidence of vCJD
infection. Information about the CJD Incidents Panel can be found on the HPA
website [3].

† For one incident, the total number of ‘at-risk' patients is still being
determined.

National anonymous tonsil archive for studies of detectable abnormal prion
protein

The National Anonymous Tonsil Archive (NATA) continues to receive
approximately 400 tonsil pairs per week (figure 1). The archive had received
a total of 43,037 tonsil pairs up to the end of April 2007 from hospitals in
England and Scotland. A further 3000 tonsil pairs have been received from
the Medical Research Council Prion Unit at the Institute for Neurology,
National Hospital for Neurology and Neurosurgery. The total number of tonsil
pairs in the archive was 46,037. The number of collection forms completed,
but with no tonsil tissue collected, was 1558 (1010 due to patient objection
and 548 due to clinical pathology being requested).

Out of the 100 NHS Hospital Trusts that each perform over 200
tonsillectomies per year in England, 90 have been recruited and are
currently sending tonsil pairs to NATA on a regular basis. There are 120
hospitals sites within these trusts taking part in NATA. At present,
approximately 50,000 tonsillectomies are performed annually in England.
Figure 2 shows the number of tonsil pairs received from each Strategic
Health Authority.

Figure 1 Number of tonsil pairs collected for NATA Monthly: January 2004 to
April 2007

Figure 2 Tonsils collected by Strategic Health Authority: April 2007

Just over 5000 tonsillectomies are performed in Scotland each year. The
project in Scotland, where there are 14 hospitals that each carry out more
than 200 tonsillectomies per year, is being coordinated by Health Protection
Scotland. Thirteen of these hospitals have been recruited to date, and are
collecting tonsils for NATA. The tonsil tissue is being transported to the
Health Protection Agency in Colindale for inclusion in the archive. Figure 3
shows all the hospitals within England and Scotland currently recruited in
the study.

Figure 3 NHS Trusts and Scottish Hospitals currently collecting and sending
tonsil tissue to the archive: April 2007

Testing of homogenates of the tonsil tissue from the archive began at the
end of January 2007. Two enzyme immunoassays (EIAs) are being used for the
initial screening of the homogenates for the presence of abnormal prion
protein. These EIAs allow the identification of any tonsils that need to be
investigated further by the more specific tests of Western blotting (WB) and
immunohistochemistry (IHC).


References
1. The National Creutzfeldt-Jakob Disease Surveillance Unit, The University
of Edinburgh. CJD statistics. CJD figures. Edinburgh: NCJDSU, 3 May 2005.
Available at .

2.The National Creutzfeldt-Jakob Disease Surveillance Unit, The University
of Edinburgh. Incidence of variant Creutzfeldt-Jakob Disease Onsets and
Deaths in the UK January 1994 – March 2005.Edinburgh: NCJDSU, 14 April 2005.
Available at .

3. HPA. Health Protection Agency CJD Incidents Panel [HPA wbesite] [online].
London: HPA. Available at
.

4. HPA. The National Anonymous Tonsil Archive: a resource for
Creutzfeldt-Jakob disease studies. Commun Dis Rep CDR Wkly [serial online]
30 October 2003 [cited 11 May 2007]; 13(44): News. Available at:
.


http://www.hpa.org.uk/hpr/infections/ei_cjd.htm


USA CJD STATISTICS SO HIGH THEY DONT SEEM TO REPORT IN 2007 YET ?

SEE STEADY INCREASE IN SPORADIC CJD IN THE USA FROM
1997 TO 2006. SPORADIC CJD CASES TRIPLED, with phenotype
of 'UNKNOWN' strain growing. ...

http://www.cjdsurveillance.com/resources-casereport.html


Diagnosis and Reporting of Creutzfeldt-Jakob Disease

Singeltary, Sr et al. JAMA.2001; 285: 733-734.

http://jama.ama-assn.org/http://www.neurology.org/cgi/eletters/60/2/176#535

BRITISH MEDICAL JOURNAL

BMJ

http://www.bmj.com/cgi/eletters/319/7220/1312/b#EL2

BMJ

http://www.bmj.com/cgi/eletters/320/7226/8/b#EL1

JOURNAL OF NEUROLOGY

MARCH 26, 2003

RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob

disease in the United States

Email Terry S. Singeltary:

flounder@wt.net

I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to

comment on the CDC's attempts to monitor the occurrence of emerging

forms of CJD. Asante, Collinge et al [1] have reported that BSE

transmission to the 129-methionine genotype can lead to an alternate

phenotype that is indistinguishable from type 2 PrPSc, the commonest

sporadic CJD. However, CJD and all human TSEs are not reportable

nationally. CJD and all human TSEs must be made reportable in every

state and internationally. I hope that the CDC does not continue to

expect us to still believe that the 85%+ of all CJD cases which are

sporadic are all spontaneous, without route/source. We have many TSEs in

the USA in both animal and man. CWD in deer/elk is spreading rapidly and

CWD does transmit to mink, ferret, cattle, and squirrel monkey by

intracerebral inoculation. With the known incubation periods in other

TSEs, oral transmission studies of CWD may take much longer. Every

victim/family of CJD/TSEs should be asked about route and source of this

agent. To prolong this will only spread the agent and needlessly expose

others. In light of the findings of Asante and Collinge et al, there

should be drastic measures to safeguard the medical and surgical arena

from sporadic CJDs and all human TSEs. I only ponder how many sporadic

CJDs in the USA are type 2 PrPSc?

http://www.neurology.org/cgi/eletters/60/2/176#535

USA MAD COW STRAIN MORE VIRULENT TO HUMANS THAN UK STRAIN

18 January 2007 - Draft minutes of the SEAC 95 meeting (426 KB) held on 7
December 2006 are now available.

snip...

64. A member noted that at the recent Neuroprion meeting, a study was
presented showing that in transgenic mice BSE passaged in sheep may be more
virulent and infectious to a wider range of species than bovine derived BSE.

Other work presented suggested that BSE and bovine amyloidotic spongiform
encephalopathy (BASE) MAY BE RELATED. A mutation had been identified in the
prion protein gene in an AMERICAN BASE CASE THAT WAS SIMILAR IN NATURE TO A
MUTATION FOUND IN CASES OF SPORADIC CJD.

snip...

http://www.seac.gov.uk/minutes/95.pdf

3:30 Transmission of the Italian Atypical BSE (BASE) in Humanized Mouse

Models Qingzhong Kong, Ph.D., Assistant Professor, Pathology, Case Western
Reserve
University

Bovine Amyloid Spongiform Encephalopathy (BASE) is an atypical BSE strain
discovered recently in Italy, and similar or different atypical BSE cases
were also reported in other countries. The infectivity and phenotypes of
these atypical BSE strains in humans are unknown. In collaboration with
Pierluigi Gambetti, as well as Maria Caramelli and her co-workers, we have
inoculated transgenic mice expressing human prion protein with brain
homogenates from BASE or BSE infected cattle. Our data shows that about half
of the BASE-inoculated mice became infected with an average incubation time
of about 19 months; in contrast, none of the BSE-inoculated mice appear to
be infected after more than 2 years.

***These results indicate that BASE is transmissible to humans and suggest
that BASE is more virulent than
classical BSE in humans.***

6:30 Close of Day One

http://www.healthtech.com/2007/tse/day1.asp


CJD ENDOSCOPY

http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0612&L=sanet-mg&P=24048


http://www.rense.com/general34/scopes.htm


http://www.vegsource.com/talk/madcow/messages/93658.html





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