UK Research and analysis Creutzfeldt-Jakob disease (CJD) update (data to end of December 2021) Updated 21 June 2022
Research and analysis Creutzfeldt-Jakob disease (CJD) update (data to end of December 2021)
Updated 21 June 2022
This report provides an update on the enhanced surveillance of potential iatrogenic (healthcare-acquired) exposures to Creutzfeldt-Jakob Disease (CJD). The data is correct as at 31 December 2021. For numbers of CJD case reports, readers should consult data provided by the National CJD Research and Surveillance Unit (NCJDRSU).
Monitoring of patients ‘at increased risk’ of CJD
Individuals who have been identified as ‘at increased risk’ of CJD as a consequence of their medical care are informed of their exposure and asked to follow public health precautions to avoid potentially transmitting the infection to others. They are also followed up to help determine the risks of CJD transmission to patients through different routes and to ascertain whether any people who may have been exposed to increased CJD risks go on to develop CJD.
Public health follow-up activities include mortality monitoring, and in some cases, post mortem investigations to determine whether asymptomatic individuals have been infected with the CJD agent. A number of different organisations are involved in these activities: UK Health Security Agency (UKHSA), Public Health Scotland (PHS), UCL Institute of Child Health/Great Ormond Street Hospital (ICH), NHS Blood and Transplant (NHSBT), NCJDRSU, National Prion Clinic (NPC), and the UK Haemophilia Centre Doctors’ Organisation (UKHCDO).
The UKHSA CJD Section coordinates the collation of data on individuals identified as ‘at increased risk’ of CJD, and who have been informed of this. These individuals are followed up through public health monitoring and research activities by different organisations.
The UKHSA CJD Section currently holds data on the following groups of patients who have been identified as ‘at increased risk’ of CJD:
recipients of blood components from donors who subsequently developed vCJD
blood donors to individuals who later developed vCJD
other recipients of blood components from these blood donors
recipients of certain plasma products between 1990 and 2001 (non-bleeding disorder patients)
certain surgical contacts of patients diagnosed with CJD
highly transfused recipients
Data on the following risk groups are not held by UKHSA, but are held by other organisations:
bleeding disorder patients who received plasma products between 1990 and 2001 (UKHCDO)
recipients of human-derived growth hormone before 1985 (ICH)
patients who could have received a dura mater graft before August 1992 (data not currently collected)
individuals treated with gonadotrophin sourced from humans before 1973 (data not currently collected)
family risk of genetic prion disease (NPC)
The data from the UKHCDO is likely to be a slight underestimate of the true number of patients with bleeding disorders who received UK-sourced clotting factors (1990 to 2001), as there was incomplete reporting of identified patients by haemophilia centres to the UKHCDO database. Notified patients are given the option of removing their details from the UKHCDO database, and are then removed from the ‘at increased risk’ totals.
The data on patients who received human-derived growth hormone held by the ICH (until the end of December 2021) is also a slight underestimate of the total as a small number of these patients are not included in the ICH follow-up.
Data concerning recipients of human-derived grown hormone before 1985 was transferred to UKHSA at the start of 2022.
Summary of all ‘at increased risk’ groups on which data is collected (data correct as at 31 December 2021)
‘At increased risk’ group Identified as ‘at increased risk’ Total notified Total notified and alive Cases Asymptomatic infections *
Recipients of blood from donors who later developed vCJD 67 27 13 3 1
Blood donors to individuals who later developed vCJD 112 108 98 0 0
Other recipients of blood components from these donors 34 32 11 0 0
Plasma product recipients (non-bleeding disorders) who received UK sourced plasma products 1990 to 2001 2 2 2 0 0
Certain surgical contacts of patients diagnosed with CJD 339 284 220 0 0
Highly transfused recipients 3 3 1 0 0
Total for ‘at increased risk’ groups where UKHSA holds data 557 456 345 3 1
Patients with bleeding disorders who received UK sourced plasma products 1980 to 2001 **, ‡ 3,637 3,281 † 2635 0 1
Recipients of human derived growth hormone ** 1,883 1,883 1,383 82 0
Total for all ‘at increased risk’ groups 6,077 5,620 4,363 85 2
*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 at post mortem.
**These are minimum figures. Central reporting for bleeding disorder patients is incomplete, and a small number of patients have opted out of the central UKHCDO database. A small number of ‘at increased risk’ growth hormone recipients are not included in the Institute of Child Health study. Not all of the ‘at increased risk’ growth hormone recipients have been notified. There is no central record of who has been informed.
†These are the minimum number of people notified based on those patients who were seen for care after the notification exercise. It is likely that many more of the ‘at increased risk’ patients received their notification letter but as they were not subsequently recorded as being seen for care this cannot be confirmed.
‡Including a small proportion of individuals known to have been treated with UK plasma products 1980 to 2001, and presumed to have also been treated 1990 to 2001.
Current status of ‘at increased risk’ groups (data correct as at 31 December 2021)
Age, sex and notification status of ‘at increased risk’ patient groups Recipients Donors Other recipients Highly transfused Plasma bleeding Plasma non-bleeding Surgical Growth hormone Total
Current status Alive 13 100 11 1 2,672 2 232 1,383 4,414
Dead 54 12 23 2 875 0 107 500 1,573
Total 67 112 34 3 3,637 2 339 1,883 6,077
Notification status Notified 27 108 32 3 3,281 2 284 1,883 5,620
Alive and notified 13 98 11 1 2,635 2 220 1,383 4,363
Genotype MM 11 – – – 5 – – – 16
MV 8 – – – 4 – – – 12
VV – – – – 1 – – – 1
Not known 48 112 34 3 3,627 2 339 1883 6,048
Sex Male* 19 53 16 1 3,093 2 156 917 4,257
Female* 20 58 18 2 544 0 175 466 1,283
Not known* 28 1 0 0 0 0 8 0 37
Current age band 0 to 19 0 0 1 0 0 0 0 0 1
20 to 39 0 0 1 1 814 0 27 14 857
40 to 59 5 35 5 0 1,152 2 78 1,083 2,360
60 to 79 4 57 4 0 688 0 90 286 1,129
80+ 4 8 0 0 108 0 37 0 157
*Sex is provided for all patients (alive and dead) except for recipients of human derived growth hormone for which sex is provided for those still alive only.
Contents
- Monitoring of patients ‘at increased risk’ of CJD
- Summary of all ‘at increased risk’ groups on which data is collected (data correct as at 31 December 2021)
- Current status of ‘at increased risk’ groups (data correct as at 31 December 2021)
TUESDAY, MAY 10, 2022
Concordance of CSF RT-QuIC across the European Creutzfeldt-Jakob Disease surveillance network
MONDAY, JANUARY 31, 2022
Validation of Revised International Creutzfeldt-Jakob Disease Surveillance Network Diagnostic Criteria for Sporadic Creutzfeldt-Jakob Disease Singeltary Comment Submission
Friday, March 11, 2022
Prevalence of Surgical Procedures at Symptomatic Onset of Prion Disease
WEDNESDAY, FEBRUARY 02, 2022
Understanding the nature of PrP found in Appendix tissues in the UK population
FRIDAY, DECEMBER 24, 2021
***> Creutzfeldt Jakob Disease CJD TSE Prion Update December 25, 2021 <***
TUESDAY, OCTOBER 26, 2021
Sporadic Creutzfeldt-Jakob Disease in a Very Young Person Singeltary Reply 2021
Saturday, December 18, 2021
Direct neural transmission of vCJD/BSE in macaque after finger incision
Tuesday, November 30, 2021
Second death in France in a laboratory working on prions
Second lab worker with deadly prion disease prompts research pause in France
A lab worker died of prion disease in 2019, nine years after a lab accident.
BETH MOLE - 7/29/2021, 5:16 PM
A 2020 paper published in the New England Journal of Medicine left little doubt that Jaumain had been infected on the job. She had variant CJD, but since Europe’s ‘mad cow’ outbreak ended after 2000 and the disease virtually disappeared, the paper said it was virtually impossible for someone her age in France to contract food-borne vCJD.
Science also said two independent reports – one by government inspectors – had found no safety violations at the lab where Jaumain worked. The press release also noted that the inspectors concluded there was “the presence of a risk control culture within the research teams”. The Jaumain family’s lawyer called the neutrality of the reports into question, however.
At the same time, the government inspectors’ report also revealed that there had been at least 17 accidents among the 100 or so scientists and technicians in France working with prions in the previous decade, raising concerns about how effective this risk control culture is. Five of these occurred when workers “stabbed or cut themselves with contaminated syringes or blades”.
Wednesday, July 28, 2021
France issues moratorium on prion research after fatal brain disease strikes two lab workers
Wednesday, July 28, 2021
France issues moratorium on prion research after fatal brain disease strikes two lab workers
Friendly fire, pass it forward, they call it iatrogenic cjd, or what i call 'tse prion poker', are you all in $$$
all iatrogenic cjd is, is sporadic cjd, before the iatrogenic event is discovered, traced back, proven, documented, put into the academic domain, and then finally the public domain, this very seldom happens, thus problem solved, it's all sporadic cjd...
SATURDAY, AUGUST 01, 2020
Sporadic Creutzfeldt-Jakob Disease among Physicians, Germany, 1993–2018 high proportion of physicians with sCJD were surgeons
SUNDAY, JULY 19, 2020
Joseph J. Zubak Orthopaedic surgeon passed away Monday, July 6, 2020, Creutzfeldt-Jakob Disease (CJD)
Variant Creutzfeldt–Jakob Disease Diagnosed 7.5 Years after Occupational Exposure
Variant Creutzfeldt–Jakob disease was identified in a technician who had cut her thumb while handling brain sections of mice infected with adapted BSE 7.5 years earlier. The long incubation period was similar to that of the transfusion-transmitted form of the disease.
THURSDAY, JULY 02, 2020
Variant Creutzfeldt–Jakob Disease Diagnosed 7.5 Years after Occupational Exposure
TUESDAY, MAY 24, 2022
Texas Creutzfeldt Jakob Disease CJD TSE Prion Update Singeltary FOIA Request Received May 23, 2022
MONDAY, JUNE 14, 2021
Texas Health and Human Services The Department of State Health Services Creutzfeldt Jakob Disease TSE Prion Report 2021?
SUNDAY, MAY 08, 2022
USA National Prion Disease Pathology Surveillance Center Surveillance Update April 11th, 2022
TUESDAY, APRIL 05, 2022
Incidence of Creutzfeldt-Jakob Disease in the United States 1993-2014
TUESDAY, MARCH 29, 2022
OIE Agent causing chronic wasting disease (CWD) TSE Prion of Cervid
THURSDAY, MARCH 31, 2022
EFSA ONE Conference 2022 Chronic Wasting Disease CWD TSE PrP of Cervid and Zoonosis Zoonotic Transmission Singeltary Submission
Terry S. Singeltary Sr., Bacliff, Tx. 77518 flounder9@verizon.net Galveston Bay...on the bottom.
posted by Terry S. Singeltary Sr. at
1:32 PM
<< Home