Friday, August 10, 2012

Incidents of Potential iatrogenic Creutzfeldt-Jakob disease (CJD) biannual update (July 2012)

Infection report/CJD

Creutzfeldt-Jakob disease (CJD) biannual update (July 2012)

This six-monthly report provides an update on reports of incidents of potential iatrogenic (healthcare-acquired) exposure to CJD. The data are correct as of 27 July 2012.

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

Reports of incidents of potential iatrogenic exposure to CJD via surgery: 2000 to 30 June 2012 A surgical incident occurs when a patient with or at increased risk of CJD has undergone surgery without the appropriate infection control guidance being followed [3]. This could happen if a patient undergoes surgery during the incubation period of CJD, or because information about CJD risk factors is not available at the time of surgery. If this happens, surgical instruments that may be contaminated with the infectious agent that causes CJD, could pose a transmission risk when they are re-used on other patients.

In June 2010 the CJD Incidents Panel changed its protocol for reporting surgical incidents, and a new reporting algorithm was published on the HPA CJD Section website. Under the new protocol only CJD cases (or patients at increased risk of CJD) who have undergone surgical procedures which are thought to pose a possible transmission risk (i.e. within the likely infectious incubation period, and involving medium or high risk procedures) are categorised as ‘surgical incidents'. Other procedures, either outside the incubation period, or involving low infectivity tissues, are categorised as ‘CJD Reports'.

Table 1 shows the number of CJD surgical incidents reported to the CJD Incidents Panel from 2000 to 30 June 2012 by the diagnosis of the index patient. As shown in the table, 44% of surgical incidents and 64% of reports result from surgery on index cases diagnosed with sporadic CJD. Advice has been issued for 4 surgical incidents and 17 CJD surgical reports that have been reported to the CJD Incidents Panel in the first six months of 2012. Information about the CJD Incidents Panel can be found on the HPA website [4].

Table 1. CJD surgical incidents (n=452) and reports (n=59) reported to the CJD Incidents Panel (which have been closed, or where advice has been issued) by diagnosis of index patient: 2000 to 30 June 2012

snip...see url link for table...tss

TOTAL 16 38 56 50 45 56 63 27 33 29 23 4 12 38 4 17 452 59

Notes: I = Incidents; R = Reports; Prior to 2010, all reports were recorded as incidents.

Health Protection Report Vol 6 No. 32 - 10 August 2012

If the investigation of a surgical incident identifies any instruments that are considered to be potentially contaminated with the infectious agent, and that could still pose an infection risk to other patients, the Panel advises that these instruments should be removed from general use or refurbished. These instruments may be quarantined, kept for exclusive use on the index patient, refurbished (endoscopes only) or destroyed. Since 2000 there have been 90 incidents in which instruments have been permanently removed from general use or refurbished (endoscopes only).

Surgical incidents resulting in ‘at risk’ patients

The Panel may advise contacting and informing patients of their possible exposure to CJD following a surgical incident. These patients should be considered 'at risk of CJD for public health purposes' and are asked to take certain precautions (i.e. not to donate blood, other tissues or organs, and to inform their medical and dental carers prior to any invasive procedures) in order to reduce the risk of transmitting the CJD agent.

The diagnosis of the index patient; the timing of the procedure relative to the development of clinical CJD; the tissue that instruments were in contact with during the procedure on the index patient; and the number of cycles of re-use and decontamination the instruments have been through following the procedure on the index case – all influence the possible risk to subsequent patients.

The threshold level of risk at which patients are considered to be ‘at increased risk’ of CJD is 1%, in addition to the background risk in the UK population. This risk threshold is based on risk assessment models, using precautionary assumptions. The 1% threshold level is used as a cut off for implementing public health precautions and is not intended to be a precise measure of an individual patient's risk. A similar threshold is used for identifying other patients who have been exposed to possible CJD risks following surgical, blood, plasma and tissue incidents.

From 2000 to 30 June 2012, there have been 28 surgical incidents in which the Panel has advised that 192 patients should be considered to have an increased risk of CJD.

Patient denotifications

Following changes in the assessment of tissue infectivity, the Panel has advised that 38 patients in 14 surgical incidents who were originally considered (and notified) as being ‘at risk' of CJD should no longer be considered ‘at risk', and should be denotified. In November 2005, gastrointestinal endoscopies without invasive procedures were reclassified as low risk procedures, and advice was issued to denotify two patients in one surgical incident. In 2006, anterior eye was reclassified as a ‘medium low' infectivity tissue. This led to a change in advice as only the first patient on whom instruments were used following an anterior eye procedure was to be considered as having an increased risk of CJD. Previously this had applied to the first two patients exposed to such instruments. This resulted in the Panel advising that 16 patients in seven incidents should be denotified. In 2009, the anterior eye was further reclassified as a low infectivity tissue. Following this change, the Panel advised that another 20 patients should be denotified.

As of 30 June 2012, the Panel has received confirmation that of the 34 patients originally notified of their exposure (out of the 38 originally considered to be ‘at risk'), 26 patients have been informed that they are no longer considered ‘at risk' and eight patients died before they could be denotified.

Current 'at risk' patients resulting from surgical instruments

There are 15 surgical incidents in which 154 patients are still considered to be at increased risk of CJD. Currently, 125 of these 'at risk' patients have been notified and a further four notified by proxy, that they are at increased risk of CJD. Local decisions have been taken not to notify four patients and 21 patients have died before notification in these incidents.

Health Protection Report Vol 6 No. 32 - 10 August 2012

Table 2. Surgical ‘at risk’ patients still identified as being ‘at increased risk of CJD’ by the Panel by procedure on the index patient

Diagnosis of index patient

Procedure on index patient

Number of incidents

Patients identified as 'at risk'

Patients who died before being notified

Local decision not to notify patient Notified patients

Sporadic Brain biopsy 2 28 2 1 25a

Sporadic Ophthalmic surgery 1 11 2 – 9

Sporadic ENT 1 1 - - 1

Variant Appendectomy 1 2 - 2 -

Variant Endoscopy 1 1 - 1 -

Asymptomatic infected vCJD Endoscopy 1 4 1 – 3b

At risk variant Endoscopy 6 37 5 – 32

At risk familial Neurosurgery 1 31 10 – 21

At risk familial Ophthalmic surgery 1 39 1 – 38

Total 15 154 21 4 129c


a. Three of these patients were notified by proxy

b. One of these patients was notified by proxy

c. Four of these patients were notified by proxy

Monitoring of patients 'at increased risk' of CJD

The CJD Incidents Panel and the Advisory Committee on Dangerous Pathogens Transmissible Spongiform Encephalopathy Risk Management Subgroup (formerly the ACDP TSE Working Group) have identified a range of individuals and groups who may have been exposed to an increased risk of CJD as a consequence of their medical care (see table 3 below).

It is important to follow up these individuals to help determine the risks of CJD spreading to patients through different routes. Follow up involves a range of activities and is carried out by different organisations. At core, follow up aims to ascertain whether any people who may have been exposed to increased CJD risks go on to develop CJD.

Health Protection Report Vol 6 No. 32 - 10 August 2012

Table 3. Summary of health status of individuals at increased risk of CJD/vCJD (as at 30 June 2012)

'At risk' Group

Identified as 'at risk'

Ever notified as being 'at risk'

Alive and Notified

CJD/vCJD cases

Asymptomatic vCJD infections d

Recipients of blood from vCJD cases 67 27 18 3 1

Blood donors to vCJD cases 112 107 104 - - Other recipients from blood donors to vCJD cases 34 32 22 - -

Plasma product recipients (all except one have non-bleeding disorders) 11 10 4 - -

Surgical contacts of all CJD cases 154 129 119 - -

Highly transfused patients (recipients of blood from ≥80 donors identified at pre-surgical assessment) 10 9 8 - -

Total for at risk groups where HPA holds data 388 310 273 3 1

Patients with bleeding disorders who received UK sourced plasma products a 3,872 n/a n/a – 1

Recipients of human derived growth hormone b 1,883 1,883 1,513 71 -

Total for all 'at risk' groups c 6,143 >2,193 >1,786 74 2

a. Data provided by the UK Haemophilia Centre Doctors' Organisation (UKHCDO). These are minimum figures. Central reporting for bleeding disorder patients is incomplete, and seven patients have opted out of the central UKHCDO database. Individual haemophilia centres were asked to send out standardised letters of notification to all their ‘at risk’ patients, but the exact number of patients who received these letters and are therefore aware of their risk is not known.

b. Data provided by the Institute for Child Health. A small number of ‘at risk' growth hormone recipients are not included in the Institute of Child Health study so the true number ‘at risk’ will be greater. The exact number of growth hormone recipients in the ICH study currently aware of their risk is not known, as given their age at the original notification many were informed indirectly, by their parents.

c. These are minimum figures given the comments made above.

d. An asymptomatic infection is when an individual does not exhibit any of the signs and symptoms of CJD in life but abnormal prion protein indicative of CJD infection has been found in tissue obtained from them. In these cases the abnormal prion protein was identified during post mortem after the individuals had died of other causes.


1. The National Creutzfeldt-Jakob Disease Research and Surveillance Unit, The University of Edinburgh. CJD statistics. Available at:

2. The National Creutzfeldt-Jakob Disease Research and Surveillance Unit, The University of Edinburgh. Incidence of variant Creutzfeldt-Jakob disease onsets and deaths in the UK January 1994 - May 2011. Edinburgh: NCJDSU, 18 May 2011. Available at:

3. Transmissible spongiform encephalopathy agents: safe working and the prevention of infection. The ACDP TSE Risk Management Subgroup.

4. HPA CJD Incidents Panel [online].

Available at: CJDIncidentsPanel.

Health Protection Report Vol 6 No. 32 - 10 August 2012

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Enforcement Report

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North America has NO surveillance system for iatrogenic CJD. a few mishaps of late ;

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Proposal ID: 29403

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please see more on Aerosols and TSE prion disease here ;

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2011 TO 2012 UPDATE

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Volume 17, Number 12—December 2011

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Current risk of iatrogenic Creutzfeld–Jakob disease in the UK: efficacy of available cleaning chemistries and reusability of neurosurgical instruments

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Evidence For CJD TSE Transmission Via Endoscopes 1-24-3 re-Singeltary to Bramble et al

Evidence For CJD/TSE Transmission Via Endoscopes

From Terry S. Singletary, Sr 1-24-3

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Risk Assessment of Transmission of Sporadic Creutzfeldt-Jakob Disease in Endodontic Practice in Absence of Adequate Prion Inactivation

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Transfusion Transmission of Human Prion Diseases

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Transfusion Transmission of Human Prion Diseases

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Fourth case of transfusion-associated vCJD infection in the United Kingdom

vCJD case study highlights blood transfusion risk 9 Dec 2006 by Terry S. Singeltary Sr.

THIS was like closing the barn door after the mad cows got loose. not only the red cross, but the FDA has failed the public in protecting them from the TSE aka mad cow agent. TSE agent ie bse, base, cwd, scrapie, tme, ...

vCJD case study highlights blood transfusion risk -

Subject: CJD: update for dental staff

Date: November 12, 2006 at 3:25 pm PST

1: Dent Update. 2006 Oct;33(8):454-6, 458-60.

CJD: update for dental staff.


Terry S. Singeltary Sr.

P.O. Box 42

Bacliff, Texas USA 77518