Surveillance for creutzfeldt-Jakob disease in Australia: update to December 2012
Surveillance for creutzfeldt-Jakob disease in Australia: update to December
2012
Genevieve M Klug, Alison Boyd, Teresa Zhao, Christiane Stehmann, Marion
Simpson, Catriona McLean, Colin L Masters & Steven J Collins
Abstract
Nation-wide surveillance for transmissible spongi-form encephalopathies
including Creutzfeldt-Jakob disease (CJD) is undertaken by the Austral-ian
National Creutzfeldt-Jakob disease Registry (ANCJDR), based at the University of
Melbourne. Surveillance has been undertaken since 1993. During this period the
unit has evolved and adapted to changes in surveillance practices and
requirements, the emergence of new disease sub-types, improvements in diagnostic
capabilities and the overall heightened awareness and understand-ing of CJD and
other transmissible spongiform encephalopathies in the health care setting. In
2012, routine surveillance continued. This brief report provides an update on
the surveillance data collected by the ANCJDR prospectively from 1993 to
December 2012, and retrospectively to 1970. It also highlights the recent
release of the revised Australian CJD Infection Control Guidelines.
Keywords: Creutzfeldt-Jakob disease, prion disease, transmissible
spongiform encephalopathy, disease surveillance
Introduction
In 1993 the Allar’s inquiry into the use of cadaver-derived pituitary
hormones under The Australian Human Pituitary Hormone Program and the
association with four medically acquired (iatrogenic) Creutzfeldt-Jakob disease
(CJD) deaths recommended the formation of an Australian surveillance unit to
monitor further cases of iatrogenic CJD in Australia.1 The Australian National
Creutzfeldt-Jakob disease Registry (ANCJDR) was established in October 1993 at
the Department of Pathology at the University of Melbourne. The monitoring of
further Australian iatrogenic CJD cases related to pituitary hormone treatment
for infertility or short stature and contaminated dura mater grafts remains one
of the core objectives of the ANCJDR. However, the ANCJDR’s activities have
changed to encompass the surveillance of all types of CJD including sporadic,
genetic and variant CJD and other transmissible spongiform encephalopathies
(TSEs) such as Gerstmann Sträussler-Sheinker Syndrome (GSS) and fatal familial
insomnia (FFI).
Sporadic CJD currently accounts for between 85% and 90% of all CJD cases
internationally.2 Cases are defined as sporadic when there is no discernible
transmission event and when there is no family history and/or negative prion
protein gene (PRNP) testing. Familial TSEs include genetic CJD, GSS and FFI.
These cases are classified as such if there is a disease-specific mutation in
PRNP or a TSE is confirmed in a 1st degree relative. PRNP mutations include
single nucleotide substitutions and poly-nucleotide insertions and deletions.
Polymorphisms in PRNP such as at codon 129 are thought to influence the disease
phenotype (including in relation to particular mutations), as well as
susceptibility to sporadic and some forms of iatrogenic CJD. Classification of
iatrogenic CJD cases is dependent on a typical clinical profile and recognition
of a transmission risk.
Since 1993 there has been considerable change in the understanding of
surveillance for TSEs. This is due to the appearance of new disease subtypes,
greater clinical awareness, improved and varied diagnostic capabilities,
continued scientific research and the world wide focus on CJD through the
emergence of variant CJD (vCJD) in 1996. In response to these changes, the
ANCJDR has adapted with available resources to meet the increasing demands for
diagnostic testing, clinical and expert infection control advice, and the
steadily growing number of suspected case notifications directed to the ANCJDR
for evaluation.
snip...
Results
In 2012, 53 new suspected TSE cases were added to the ANCJDR for
evaluation. The source of notification for these new cases included requests for
a CSF 14-3-3 protein test (62%), personal communication from a neurologist,
neuropathologist, clinician or hospital (23%), health department notification
(7%), communication from a family (6%) or from the CJD counselling service (2%).
The proportion reported from each source is consistent with those in 2011. CSF
referral has accounted for 74% of all referrals since 2000, with 21% by direct
personal communication (comprising medical practitioners, 16%, families, 4% and
hospitals, 1%). In 2012 notification numbers declined nationally by 37% compared
to the previous year (Figure 1). Contributing to this decrease were fewer
notifications in several states including Victoria (32%), New South Wales (45%),
Western Australia (60%) and Tasmania (100%). The remaining states and
territories remained unchanged from the previous year. This decline in
notifications is unexplained and these rates will be closely monitored in 2013.
In 2012, 38 of the 53 notified cases were still under investigation. The
annual proportion of suspected cases notified between 1993 and 2011 that were
subsequently classified as definite or probable TSE cases ranges from 32% to
78%, with a mean of 46%.
As of 31 December 2012, 962 suspected TSE cases were on the register with
733 of these being classified as Australian probable or definite TSE cases
(Table 1).An additional 638 cases were excluded after detailed follow-up. Of the
53 new suspected cases added to the register in 2012, 3 were excluded (2
following neuropathological examination), 38 are incomplete, 8 were classified
as definite CJD and 4 as probable CJD. During 2012, 45 suspected cases were
excluded from the register (10 after neuropathological examination) and 38 cases
were classified as sporadic CJD and 1 as familial TSE. There are currently 14
cases of possible CJD of which 13 are sporadic and 1 iatrogenic. Of the 214
incomplete cases, 135 are presently alive. In comparison to the rapid increase
in the number of incomplete cases on the register observed between 2003 and 2010
(average 22% increase per year), an overall reduction in the size of this group
was recorded in 2012 (12% decrease).
Between 1 January 2012 and 31 December 2012, there were no new cases of
iatrogenic CJD. The most recent human-derived pituitary gonadotrophin-related
CJD death occurred in 1991, while the most recent Lyodura-related CJD death
occurred in 2000. As of 31 December 2012, there had been no known cases of vCJD
in Australia.Between 1970 and 2000, the annual incidence of TSEs in Australia
steadily increased (Figure 2). As for other international CJD surveillance
programs, the increase probably reflects case ascertainment bias stemming from
improved recognition, investigation, case confirmation and reporting. The
incidence of TSE in Australia declined and stabilized at around 1.0 case per
million per year during 2001-2004, but increased to 1.72 cases per million per
year in 2006. Incidence remained at around 1.3 to 1.4 cases per million per year
between 2007 and 2012.The majority of the confirmed Australian TSE cases have
been of sporadic aetiology (92%) and this has been a consistent observation from
1970 to 2012. Familial and iatrogenic cases constitute 7% and 1% respectively of
all definite and probable cases. Between 1993 and 2012 the average number of
familial cases classified in Australia was 2 cases per year. Since 2009 only one
familial case has been classified per year. The overall proportion of cases
classified as familial TSE has declined (Figure 3).
Between 2002 and 2012 the majority of states and territories had
age-adjusted mortality rates above or close to 1.0 case per million per year
(Table 2). The highest mean mortality rates were observed in Victoria and
Western Australia (1.62 and 1.49 deaths per million per year,
respectively).
From 1970 to 2012 there were more female TSE cases (54%) than male for all
forms of TSE combined. This was also true for sporadic (54%) and genetic (55%)
forms. A comparison of age and sex-specific mortality shows the similarity of
rates between males and females with some exceptions in the older age groups
(Figure 4).
The median age of death from all forms of TSE between 1970 and 2012 was 67
years with little difference between the sexes (men, 66 years, women, 67 years).
For familial cases, a difference did exist between the sexes, as the median age
at death was 52 years in males and 62 years in females. The range in age at
death from TSE was broad for both the sporadic (25-90 years) and familial (18-82
years) group with median age at death being 67 and 59 years respectively. For
the eight iatrogenic cases, death occurred between the ages of 26 and 62 years
and disease duration from onset to death was between 2 and 25 months (median,
6.25 months). For all TSE cases, 92.9% of deaths occured over the age of 50.
This demonstrates that older age groups are at risk of developing TSEs and this
is consistent for all TSE aetiologies. Of the 39 cases confirmed with a TSE in
2012, all deaths occurred in those over the age of 50 years.
Between 1970 and 2012, disease duration from onset varied between the three
aetiologies. Sporadic TSE cases had much shorter disease duration than both
iatrogenic and familial cases with 50% of deaths occurring within 3.5 months of
onset (range, 0.9 – 60 months). From 1970 to 2012 familial cases were associated
with a significantly greater survival time in comparison to sporadic TSE with
the median illness duration of 6 months (range, 1.5 – 192 months). Within 6
months of disease onset, 72% of sporadic cases, 53% of familial cases and 56% of
iatrogenic cases had died.
Discussion
There are several possible explanations regarding the range in the annual
number of notifications and the proportion of suspected cases that were
subsequently confirmed as TSE cases. These include the prospective surveillance
approach employed, diagnostic capacity changes such as the CSF 14-3-3 protein
test and MRI, enhanced clinician awareness, a greater public health profile for
CJD through the focus on variant CJD. In addition, the notifiable status of CJD
was established in all states and territories by June 2006. Specifically,
Tasmania (May 2003), Victoria (Jan 2004), Western Australia (Jan 2004), New
South Wales (April 2004), Northern Territory (Dec 2004), Australian Capital
Territory (Sept 2005), Queensland (Dec 2005), South Australia (June 2006).
In 2012 there was a high number of cases confirmed or excluded by
neuropathology. There was also a 4-fold increase in the number of probable cases
and a two-fold increase in the number of cases excluded from the ANCJDR after
detailed evaluation. These changes led to a 12% decrease in the number of
incomplete or unresolved cases on the register. As in 2011, the ANCJDR made a
concerted effort during 2012 to focus staff resources on performing case reviews
and classifying outstanding, incomplete cases.
Fluctuating peaks in the incidence of TSE might be expected in such a rare
disease. The ANCJDR believes that there are a number of factors responsible for
the 2000-04 decline. These include a reduction in the number of probable cases
classified due to broadened surveillance responsibilities, difficulties
experienced following changes to the privacy legislation, and changes to the
registration of cases referred for CSF 14-3-3 protein testing. The subsequent
peak incidence in 2006 aligns with a increasing trend in notifications in
2005–06. This can be attributed predominantly to increased ante-mortem
notifications through the CSF 14-3-3 protein test, which has enabled a greater
number of post-mortem examinations to be actively investigated for suspected
TSE. Ultimately, with more post-mortem examinations being performed, a greater
number of suspected TSE cases have been confirmed. Currently, the proportion of
all suspected cases notified to the ANCJDR between 1993 and 2011, (including
those cases excluded from the register after evaluation and where death is known
to have occurred) who have undergone a post-mortem examination is 62%. The
ANCJDR also believes that the peak incidence rates of 1.67 and 1.72 cases per
million per year observed in 2000 and 2006 respectively provide a more accurate
estimation of the true incidence of TSEs in Australia, underscoring the
importance for post-mortem examinations to be actively promoted in all suspect
cases. The mean annual age-adjusted TSE mortality rate for the 1993 to 2012
period was 1.26 deaths per million per year. This rate aligns with the reported
figures for other countries with similar surveillance mechanisms to those in
Australia.7
The ANCJDR has maintained a non-systematic approach to the prion protein
genotyping of confirmed TSE cases. This may have contributed to our lower
percentage of familial cases (7%) compared with European CJD surveillance
programmes, which report that between 12% and 14% of cases are familial.8 In
recent years the free PRNP genetic testing service provided by the ANCJDR to CJD
patients and families has been decentralised due to their preference for an
“on-demand” service. Although the ANCJDR still performs routine genetic testing
three times annually, testing is now also undertaken by external, independent
laboratories and genetic services. The separation of PRNP testing from the
ANCJDR may have inadvertently contributed to the reduced proportion of genetic
TSE cases observed over the last few years (Figure 3).
In 2012 the Australian CJD Infection Control Guidelines were revised by the
Communicable Diseases Network Australia and published in January 2013.9 These
guidelines provide updated information for health care and funeral industry
professionals and families of CJD patients. They aim to provide greater clarity
for infection control and ensure ease of use and the avoidance of unnecessary
discrimination or disadvantage for families affected by CJD.10
During 2012, the ANCJDR continued nation-wide surveillance for all forms of
TSE and has identified a decrease in the number of suspected cases notified for
evaluation. Overall disease incidence has not been affected by this decline;
however, it remains to be determined how this will influence incidence rates in
2013. Notifications will be closely monitored during 2013.
http://www.health.gov.au/internet/main/publishing.nsf/Content/cdi3702-pdf-cnt.htm/$FILE/cdi3702c.pdf
Infection Control Guidelines This document provides recommendations for
infection prevention and control procedures to minimise the risk of transmission
of Creutzfeldt - Jakob disease (CJD) in health care settings. Page last updated:
15 April 2013
The Creutzfeldt-Jakob disease (PDF 147 KB) – January 2013
Introduction l Assessing the risk l Additional Procedures l Surveillance
Infection Prevention and Control in other settings l References Appendix 1 l
Appendix 2 l Appendix 3 l Appendix 4 l Appendix 5
Key points This document provides recommendations for infection prevention
and control procedures to minimise the risk of transmission of Creutzfeldt -
Jakob disease (CJD) in health care settings. CJD will be used in this document
to refer to all forms of classical Creutzfeldt - Jakob disease. Variant CJD
(vCJD) is excluded from the scope of this document as vCJD has not been reported
in Australia to date. Infection prevention and control issues regarding patients
with suspected or confirmed vCJD will be made available on the Department of
Health and Ageing website once vCJD is reported in Australia. There is presently
no test available to detect CJD infection before the onset of symptoms. There is
no evidence that CJD can be transmitted through normal social or sexual contact.
The decision to implement additional procedures for equipment reprocessing is
based on a risk assessment (Section 2.4) which incorporates the currently known
infectivity of the tissue to which the instrument has been exposed (Section 2.2
and Table 1) and the currently known patient factors (Section 2.3 and Appendix 1
and 2). The additional procedures that may apply as a result of the risk
assessment are outlined in Section 3 (and Table 2). Although transmission of CJD
in the health care setting is very rare, Health Care Workers (HCW) should be
aware of the potential for transmission by contaminated instruments or via
contaminated higher- infectivity tissues. The infective agent of CJD (the prion)
is resistant to routine reprocessing, making the additional procedures outlined
in this document essential for the treatment of patients with an identified risk
of CJD infection. All health care providers and facilities should be familiar
with these guidelines and adhere to them, so that patients who may have been
exposed to CJD have access to appropriate evidence-based health care without
discrimination and disadvantage. 1 Introduction
SNIP...PLEASE SEE FULL TEXT ;
Saturday, November 2, 2013
Recommendation of the Swiss Expert Committee for Biosafety on the
classification of activities using prion genes and prion protein January 2013
Surveillance for creutzfeldt-Jakob disease in Australia: update to
December 2012
Surveillance for creutzfeldt-Jakob disease in Australia: update to December
2012
Genevieve M Klug, Alison Boyd, Teresa Zhao, Christiane Stehmann, Marion
Simpson, Catriona McLean, Colin L Masters & Steven J Collins
Abstract
Nation-wide surveillance for transmissible spongi-form encephalopathies
including Creutzfeldt-Jakob disease (CJD) is undertaken by the Austral-ian
National Creutzfeldt-Jakob disease Registry (ANCJDR), based at the University of
Melbourne. Surveillance has been undertaken since 1993. During this period the
unit has evolved and adapted to changes in surveillance practices and
requirements, the emergence of new disease sub-types, improvements in diagnostic
capabilities and the overall heightened awareness and understand-ing of CJD and
other transmissible spongiform encephalopathies in the health care setting. In
2012, routine surveillance continued. This brief report provides an update on
the surveillance data collected by the ANCJDR prospectively from 1993 to
December 2012, and retrospectively to 1970. It also highlights the recent
release of the revised Australian CJD Infection Control Guidelines.
Keywords: Creutzfeldt-Jakob disease, prion disease, transmissible
spongiform encephalopathy, disease surveillance
Introduction
In 1993 the Allar’s inquiry into the use of cadaver-derived pituitary
hormones under The Australian Human Pituitary Hormone Program and the
association with four medically acquired (iatrogenic) Creutzfeldt-Jakob disease
(CJD) deaths recommended the formation of an Australian surveillance unit to
monitor further cases of iatrogenic CJD in Australia.1 The Australian National
Creutzfeldt-Jakob disease Registry (ANCJDR) was established in October 1993 at
the Department of Pathology at the University of Melbourne. The monitoring of
further Australian iatrogenic CJD cases related to pituitary hormone treatment
for infertility or short stature and contaminated dura mater grafts remains one
of the core objectives of the ANCJDR. However, the ANCJDR’s activities have
changed to encompass the surveillance of all types of CJD including sporadic,
genetic and variant CJD and other transmissible spongiform encephalopathies
(TSEs) such as Gerstmann Sträussler-Sheinker Syndrome (GSS) and fatal familial
insomnia (FFI).
Sporadic CJD currently accounts for between 85% and 90% of all CJD cases
internationally.2 Cases are defined as sporadic when there is no discernible
transmission event and when there is no family history and/or negative prion
protein gene (PRNP) testing. Familial TSEs include genetic CJD, GSS and FFI.
These cases are classified as such if there is a disease-specific mutation in
PRNP or a TSE is confirmed in a 1st degree relative. PRNP mutations include
single nucleotide substitutions and poly-nucleotide insertions and deletions.
Polymorphisms in PRNP such as at codon 129 are thought to influence the disease
phenotype (including in relation to particular mutations), as well as
susceptibility to sporadic and some forms of iatrogenic CJD. Classification of
iatrogenic CJD cases is dependent on a typical clinical profile and recognition
of a transmission risk.
Since 1993 there has been considerable change in the understanding of
surveillance for TSEs. This is due to the appearance of new disease subtypes,
greater clinical awareness, improved and varied diagnostic capabilities,
continued scientific research and the world wide focus on CJD through the
emergence of variant CJD (vCJD) in 1996. In response to these changes, the
ANCJDR has adapted with available resources to meet the increasing demands for
diagnostic testing, clinical and expert infection control advice, and the
steadily growing number of suspected case notifications directed to the ANCJDR
for evaluation.
snip...
Results
In 2012, 53 new suspected TSE cases were added to the ANCJDR for
evaluation. The source of notification for these new cases included requests for
a CSF 14-3-3 protein test (62%), personal communication from a neurologist,
neuropathologist, clinician or hospital (23%), health department notification
(7%), communication from a family (6%) or from the CJD counselling service (2%).
The proportion reported from each source is consistent with those in 2011. CSF
referral has accounted for 74% of all referrals since 2000, with 21% by direct
personal communication (comprising medical practitioners, 16%, families, 4% and
hospitals, 1%). In 2012 notification numbers declined nationally by 37% compared
to the previous year (Figure 1). Contributing to this decrease were fewer
notifications in several states including Victoria (32%), New South Wales (45%),
Western Australia (60%) and Tasmania (100%). The remaining states and
territories remained unchanged from the previous year. This decline in
notifications is unexplained and these rates will be closely monitored in 2013.
In 2012, 38 of the 53 notified cases were still under investigation. The
annual proportion of suspected cases notified between 1993 and 2011 that were
subsequently classified as definite or probable TSE cases ranges from 32% to
78%, with a mean of 46%.
As of 31 December 2012, 962 suspected TSE cases were on the register with
733 of these being classified as Australian probable or definite TSE cases
(Table 1).An additional 638 cases were excluded after detailed follow-up. Of the
53 new suspected cases added to the register in 2012, 3 were excluded (2
following neuropathological examination), 38 are incomplete, 8 were classified
as definite CJD and 4 as probable CJD. During 2012, 45 suspected cases were
excluded from the register (10 after neuropathological examination) and 38 cases
were classified as sporadic CJD and 1 as familial TSE. There are currently 14
cases of possible CJD of which 13 are sporadic and 1 iatrogenic. Of the 214
incomplete cases, 135 are presently alive. In comparison to the rapid increase
in the number of incomplete cases on the register observed between 2003 and 2010
(average 22% increase per year), an overall reduction in the size of this group
was recorded in 2012 (12% decrease).
Between 1 January 2012 and 31 December 2012, there were no new cases of
iatrogenic CJD. The most recent human-derived pituitary gonadotrophin-related
CJD death occurred in 1991, while the most recent Lyodura-related CJD death
occurred in 2000. As of 31 December 2012, there had been no known cases of vCJD
in Australia.Between 1970 and 2000, the annual incidence of TSEs in Australia
steadily increased (Figure 2). As for other international CJD surveillance
programs, the increase probably reflects case ascertainment bias stemming from
improved recognition, investigation, case confirmation and reporting. The
incidence of TSE in Australia declined and stabilized at around 1.0 case per
million per year during 2001-2004, but increased to 1.72 cases per million per
year in 2006. Incidence remained at around 1.3 to 1.4 cases per million per year
between 2007 and 2012.The majority of the confirmed Australian TSE cases have
been of sporadic aetiology (92%) and this has been a consistent observation from
1970 to 2012. Familial and iatrogenic cases constitute 7% and 1% respectively of
all definite and probable cases. Between 1993 and 2012 the average number of
familial cases classified in Australia was 2 cases per year. Since 2009 only one
familial case has been classified per year. The overall proportion of cases
classified as familial TSE has declined (Figure 3).
Between 2002 and 2012 the majority of states and territories had
age-adjusted mortality rates above or close to 1.0 case per million per year
(Table 2). The highest mean mortality rates were observed in Victoria and
Western Australia (1.62 and 1.49 deaths per million per year,
respectively).
From 1970 to 2012 there were more female TSE cases (54%) than male for all
forms of TSE combined. This was also true for sporadic (54%) and genetic (55%)
forms. A comparison of age and sex-specific mortality shows the similarity of
rates between males and females with some exceptions in the older age groups
(Figure 4).
The median age of death from all forms of TSE between 1970 and 2012 was 67
years with little difference between the sexes (men, 66 years, women, 67 years).
For familial cases, a difference did exist between the sexes, as the median age
at death was 52 years in males and 62 years in females. The range in age at
death from TSE was broad for both the sporadic (25-90 years) and familial (18-82
years) group with median age at death being 67 and 59 years respectively. For
the eight iatrogenic cases, death occurred between the ages of 26 and 62 years
and disease duration from onset to death was between 2 and 25 months (median,
6.25 months). For all TSE cases, 92.9% of deaths occured over the age of 50.
This demonstrates that older age groups are at risk of developing TSEs and this
is consistent for all TSE aetiologies. Of the 39 cases confirmed with a TSE in
2012, all deaths occurred in those over the age of 50 years.
Between 1970 and 2012, disease duration from onset varied between the three
aetiologies. Sporadic TSE cases had much shorter disease duration than both
iatrogenic and familial cases with 50% of deaths occurring within 3.5 months of
onset (range, 0.9 – 60 months). From 1970 to 2012 familial cases were associated
with a significantly greater survival time in comparison to sporadic TSE with
the median illness duration of 6 months (range, 1.5 – 192 months). Within 6
months of disease onset, 72% of sporadic cases, 53% of familial cases and 56% of
iatrogenic cases had died.
Discussion
There are several possible explanations regarding the range in the annual
number of notifications and the proportion of suspected cases that were
subsequently confirmed as TSE cases. These include the prospective surveillance
approach employed, diagnostic capacity changes such as the CSF 14-3-3 protein
test and MRI, enhanced clinician awareness, a greater public health profile for
CJD through the focus on variant CJD. In addition, the notifiable status of CJD
was established in all states and territories by June 2006. Specifically,
Tasmania (May 2003), Victoria (Jan 2004), Western Australia (Jan 2004), New
South Wales (April 2004), Northern Territory (Dec 2004), Australian Capital
Territory (Sept 2005), Queensland (Dec 2005), South Australia (June 2006).
In 2012 there was a high number of cases confirmed or excluded by
neuropathology. There was also a 4-fold increase in the number of probable cases
and a two-fold increase in the number of cases excluded from the ANCJDR after
detailed evaluation. These changes led to a 12% decrease in the number of
incomplete or unresolved cases on the register. As in 2011, the ANCJDR made a
concerted effort during 2012 to focus staff resources on performing case reviews
and classifying outstanding, incomplete cases.
Fluctuating peaks in the incidence of TSE might be expected in such a rare
disease. The ANCJDR believes that there are a number of factors responsible for
the 2000-04 decline. These include a reduction in the number of probable cases
classified due to broadened surveillance responsibilities, difficulties
experienced following changes to the privacy legislation, and changes to the
registration of cases referred for CSF 14-3-3 protein testing. The subsequent
peak incidence in 2006 aligns with a increasing trend in notifications in
2005–06. This can be attributed predominantly to increased ante-mortem
notifications through the CSF 14-3-3 protein test, which has enabled a greater
number of post-mortem examinations to be actively investigated for suspected
TSE. Ultimately, with more post-mortem examinations being performed, a greater
number of suspected TSE cases have been confirmed. Currently, the proportion of
all suspected cases notified to the ANCJDR between 1993 and 2011, (including
those cases excluded from the register after evaluation and where death is known
to have occurred) who have undergone a post-mortem examination is 62%. The
ANCJDR also believes that the peak incidence rates of 1.67 and 1.72 cases per
million per year observed in 2000 and 2006 respectively provide a more accurate
estimation of the true incidence of TSEs in Australia, underscoring the
importance for post-mortem examinations to be actively promoted in all suspect
cases. The mean annual age-adjusted TSE mortality rate for the 1993 to 2012
period was 1.26 deaths per million per year. This rate aligns with the reported
figures for other countries with similar surveillance mechanisms to those in
Australia.7
The ANCJDR has maintained a non-systematic approach to the prion protein
genotyping of confirmed TSE cases. This may have contributed to our lower
percentage of familial cases (7%) compared with European CJD surveillance
programmes, which report that between 12% and 14% of cases are familial.8 In
recent years the free PRNP genetic testing service provided by the ANCJDR to CJD
patients and families has been decentralised due to their preference for an
“on-demand” service. Although the ANCJDR still performs routine genetic testing
three times annually, testing is now also undertaken by external, independent
laboratories and genetic services. The separation of PRNP testing from the
ANCJDR may have inadvertently contributed to the reduced proportion of genetic
TSE cases observed over the last few years (Figure 3).
In 2012 the Australian CJD Infection Control Guidelines were revised by the
Communicable Diseases Network Australia and published in January 2013.9 These
guidelines provide updated information for health care and funeral industry
professionals and families of CJD patients. They aim to provide greater clarity
for infection control and ensure ease of use and the avoidance of unnecessary
discrimination or disadvantage for families affected by CJD.10
During 2012, the ANCJDR continued nation-wide surveillance for all forms of
TSE and has identified a decrease in the number of suspected cases notified for
evaluation. Overall disease incidence has not been affected by this decline;
however, it remains to be determined how this will influence incidence rates in
2013. Notifications will be closely monitored during 2013.
http://www.health.gov.au/internet/main/publishing.nsf/Content/cdi3702-pdf-cnt.htm/$FILE/cdi3702c.pdf
Saturday, April 14, 2012
Australian Disease Strategy Bovine spongiform encephalopathy Version 3.2
and Creutzfeldt Jakob Disease report 2012
Communicable Diseases Intelligence Volume 35 No 2 - June 2011
Surveillance of Creutzfeldt-Jakob disease in Australia: update to December
2010 This annual report prepared by the Australian National CJD Registry
provides updated Australian human transmissible spongiform encephalopathies
(TSE) figures, up till December 2010.
Genevieve M Klug, Alison Boyd, Amelia McGlade, Christiane Stehmann, Colin L
Masters, Steven J Collins
Abstract
Since the establishment of the Australian National Creutzfeldt-Jakob
disease Registry (ANCJDR) it’s activities have expanded from prospectively
investigating additional iatrogenic Creutzfeldt-Jakob disease cases to include:
retrospective ascertainment to 1970; provision of expert opinions in the area of
infection control management; provide diagnostic testing services for all
suspect cases; and maintenance of national and international collaborations in
conjunction with routine surveillance responsibilities. An update of the
ANCJDR’s surveillance activities and outcomes between 1 April and 31 December
2010 is herein presented, including a summation of a recent publication by the
ANCJDR. The shorter reporting period is due to a contractual change with the
Department of Health and Ageing in 2010, resulting in the reporting timeframe
shifting to align with full calendar years. Commun Dis Intell
2011;35(2):149–153.
Introduction In October 1993, the Australian Government Department of
Health and Ageing established the Australian National Creutzfeldt-Jakob disease
Registry (ANCJDR) and have since charged this unit with the task of the
surveillance of human prion diseases in Australia. The formation of this unit
was underscored by the recommendations of the Allars Report,1 which investigated
the identification of 4 women who were recipients of human-derived pituitary
hormones and who died of Creutzfeldt-Jakob disease (CJD) between 1988 and 1991.
CJD is one form of the prion group of neurological disorders, which in humans,
includes Gerstmann Sträussler-Sheinker syndrome, fatal familial insomnia, Kuru
and variant CJD (vCJD) while bovine spongiform encephalopathy (BSE) in cattle,
scrapie in sheep and chronic wasting disease in deer and elk represent principal
animal forms of disease. This family of disorders, also known as transmissible
spongiform encephalopathies (TSEs), causes a rapidly progressive neurological
illness, ultimately leading to death. Globally, the annual incidence of CJD is
around 1 case per million, although it is speculated that this may well be
higher as has been indicated from several international surveillance centres,2
where the annual incidence of 2 cases per million per year has been observed.
The large majority of CJD cases have no known underlying cause and are thus
classified as sporadic. The remaining cases are attributed to iatrogenic
transmission or genetic predisposition. All suspect TSE cases referred to the
ANCJDR are actively investigated and where possible, classified as definite,
probable or possible according to the internationally recognised and validated
clinical and neuropathological criteria.3,4
ANCJDR surveillance update to 31 December 2010 Notifications Between 1
April and 31 December 2010 46 new suspect cases of CJD were notified to the
ANCJDR. While this figure is reduced from previous reports based on 12 month
periods, it is a reflection of the shorter reporting period of 9 months due to a
change in contractual timeframes. Of these new suspect cases, nine have been
confirmed as definite or probable cases, one has been classified as a possible
case and three have been removed from the register. The remaining 33 are still
under investigation with 16 of these cases still alive and 17 deceased.
Neuropathological examination for nine of the deceased cases is pending.
Since establishment, a total of 1,468 cases of suspect CJD have been
notified to the ANCJDR, comprising 308 notifications of case deaths prior to
1993 (retrospective cases) and 1,160 suspects notified prospectively. While this
equates to around 80 notifications annually, the notification of prospective
cases provides a more accurate estimate of annual national notifications. The
average number of suspect case notifications for the period 1993 to 2010 is 64
cases per year or 3.2 cases per million population per year. This is almost 3
times greater than the rate of Australian confirmed cases for the same period
(1.2 cases per million per year). Almost half of the prospective notifications
stem from referrals for cerebrospinal fluid (CSF) 14-3-3 protein analysis (one
of the diagnostic tests offered by the ANCJDR since 1997), while around a third
are derived from personal communication from clinicians, family or hospitals.
The remaining cases are ascertained through death certificate searches, hospital
and health department searches and requests for other diagnostic services such
as genetic testing.
By state and territory, analysis of the prospective suspect case
notifications shows relatively stable levels since 2006 compared with previous
years (Figure 1). Prior to this report however, Tasmania was an exception to
stable notification levels due to declining notifications since 2006. In 2010, 3
cases have been notified to the ANCJDR, returning the number of notifications to
expected levels. A reduced number of notifications in New South Wales for
2008–2009 has been sustained in 2010, with around 10 fewer cases being notified
for these 3 years compared with the 2006–2007 period.
Figure 1: Prospective, suspect Creutzfeldt-Jakob disease case notifications
to the Australian National Creutzfeldt-Jakob Disease Registry, 1997 to 2010, by
state or territory
Case outcomes Of the 1,468 cases notified to the ANCJDR, 653 of these have
been classified as probable or definite CJD cases (Table 1). An additional case
of definite iatrogenic CJD is included in Table 1, due to pituitary hormone
treatment occurring within Australia. However, due to the non-domestic location
of onset and death, this case is not included in the Australian statistical
analyses. The remaining, notified cases have been excluded after detailed
follow-up investigation (573); are currently under evaluation (229); or have
been classified as possible cases (13). Possible cases, classified as clinically
likely but unable to meet diagnostic criteria, are excluded from all statistical
analyses in this report.
Table 1: Classification of cases by the ANCJDR, 1 January 1970 to 31
December 2010
* Includes 1 definite iatrogenic case who received pituitary hormone
treatment in Australia but disease onset and death occurred while a resident of
the United Kingdom. This case is not included in statistical analysis since
morbidity and mortality did not occur within Australia.
† Includes 159 living cases.
Since the last reporting period, 14 suspect cases have been removed from
the register, with 11 of these after neuropathological confirmation. A further
definite CJD case, who was initially referred to the register during treatment
in Australia, died overseas and was therefore removed from the register and not
included as an Australian case due to the non-domestic location at death. For
the 9-month reporting period, 20 cases were confirmed as definite cases and four
confirmed as probable cases, which is consistent with the number of cases
classified in previous full 12 month reporting periods. This finding stems from
the greater number of definite cases confirmed after post-mortem examinations
performed in 2010 and relates to the reduced turnaround time for post-mortem
examinations across some states and territories within this 9-month period,
translating into more cases being confirmed in a shorter period of time. Of the
new cases, 23 were classified as sporadic cases and one as a familial case.
The annual proportion of suspect cases notified to the ANCJDR where death
is known to have occurred and have undergone post-mortem examination, has
increased over time. This is to be expected given the active approach that the
ANCJDR has undertaken to seek and facilitate post-mortem examinations in recent
years. For the 1993 to 2010 period, 60% of all suspect case deaths have
undergone post-mortem. It should be noted that this proportion is related only
to cases where death is known to have occurred and the ANCJDR is aware that not
all deaths are notified. In Victoria, further assistance with post-mortems has
been provided through the formalisation of a contractual agreement with the
Victorian Department of Health and the ANCJDR. The agreement has led to more
expeditious and higher rates of autopsy in this state.
Based on the Australian population, the average crude rate of CJD-related
post-mortems between 1993 and 2010 is 1.4 post-mortems per million per year,
which is considerable given CJD is a particularly rare condition. By state and
territory, the rate ranges from 0.8 CJD post-mortems performed annually per
million in Tasmania to 1.5 in both Victoria and New South Wales. Despite the
smaller populations in the Tasmania, the Northern Territory and the Australian
Capital Territory, the post-mortem rates are all relatively consistent with more
populous states and provide a level of confidence that suspect case deaths in
these states and territories have a similar likelihood of undergoing post-mortem
examination.
Top of page
As reported previously, the annual incidence of CJD has steadily increased
from 1970 to peak in 2000, 2006 and 2008 (Figure 2) with 1.4–1.6 cases per
million per year being recorded, equating to 32 to 37 cases per year. For the
overall period of 1993 to 2010, an average of 24.5 CJD cases per year are
confirmed in Australia and the average age-standardised mortality rate is 1.2
cases per million per year. Although these long term averages align closely with
rates observed in other countries with similar surveillance mechanisms in
place,2 it is believed that the incidence in the peak years, more closely
reflects the true incidence of CJD in Australia. The ANCJDR therefore aims to
achieve this level of case ascertainment.
Figure 2: ANCJDR definite and probable cases 1970 to 2010,* number and
age-standardised mortality rate
Age-standardised mortality rates were calculated using the Australian
Bureau of Statistics 2000 estimated resident population for Australia.
* To 31 December 2010.
Top of page
Delineation of the total case deaths by state and territory shows absolute
numbers reflecting regional population distributions. The annual number of
deaths from definite and probable TSE according to state and territory during
2000–2010 is shown (Table 2). The mean age-standardised rates (1993–2010)
indicates that there is little variability between the larger regions of
Australia with between 1.0–1.5 deaths per million occurring annually. These
rates are in alignment with reported figures from other countries with similar
surveillance mechanisms as those in Australia.5 Furthermore, analysis of
sporadic CJD standardised mortality ratios indicate that the rate of death was
not found to be significantly different in any state or territory compared with
the rate in the Australian general population, indicating that no state or
territory had a greater or lower risk of CJD.
Table 2: Transmissible spongiform encephalopathy deaths and mortality rate,
by state and territory
* Includes all deaths occurring between the complete years 1 January 1993
or 1 January 2000 and 31 December 2010.
The highest TSE (all forms) mortality rates (1993–2010) were observed in
Victoria and Western Australia (1.4 and 1.5 deaths per million per year,
respectively). Previously, the lowest rates of mortality were observed in the
Northern Territory and Tasmania and it was postulated that cases were being
under-ascertained in these regions. More recently, an increase in confirmed
cases in these less populated states and territories has contributed to the
re-alignment of mortality rates to that of the larger states and territories.
Tasmania continues to have the lowest TSE mortality in Australia; however, as
previously discussed5 an under-ascertainment of cases prior to 2000 may be
responsible for skewing the overall incidence. Furthermore, a confirmed CJD case
who was a permanent Tasmanian resident, but died interstate was not attributed
to Tasmania due to the non-Tasmanian location at death. It should be noted that
the effect of 2 additional confirmed CJD cases in Tasmania would result in the
mortality increasing to 0.9 cases per million per year, re-aligning mortality
rates more closely to expected levels.
The age group with the highest mortality from all forms of CJD is amongst
those aged 65–69 years where 8.3 deaths per million persons occur annually. From
the age of 50, incidence increases to peak at 9.0 deaths per million per year in
females in the 65–69 year age group and at 7.6 deaths per million per year in
males in the 70–74 year age group (Figure 3). After these gender-specific peak
age groups, mortality rates decline for both genders, although it should be
noted that an increase in the detection of older age cases in recent years has
led to a more rounded decline in the age-specific trend in the older age groups.
Females are in slight excess (53%) for all forms of CJD, and this is true for
familial (55%) and sporadic (53%) groups. In the small number of Australian
iatrogenic cases, an equal number of males and females have been affected
overall; 3 female pituitary hormone-related cases, 4 male and 1 female dura
mater related cases.
Since the last reporting period, there has been little change in the
aetiological proportions of Australian TSE cases with the large majority
occurring sporadically (90.7%) and the remainder classified as familial (8.1%)
and iatrogenic (1.2%). A slight reduction in the genetic CJD forms has been
observed in recent years and while the explanation for this is unclear at
present, the incidence of genetic CJD will be closely monitored in future years.
There have been no confirmed cases of vCJD, Kuru or further cases of iatrogenic
CJD relating to recipients of dura mater grafts or pituitary hormone. The last
deaths from iatrogenic CJD occurred in 1991 (pituitary hormone-related CJD) and
2000 (dura mater-related CJD).
As shown in Figure 3, the majority of Australian TSE cases occur after the
age of 50 and this is true for all TSE aetiologies. The median age at death in
the 653 confirmed cases is 66 years (range, 18–90 years), with the median age
younger in familial cases (59 years, range 18–82 years) and iatrogenic cases (39
years, range 26–62 years), but overall closely aligns with the median age death
of sporadic cases, given this CJD form represents 90.7% of all cases. Similarly,
the median duration of disease from onset to death is 4 months for all cases
(range 0.9–192 months) and the sporadic only case group (range 0.9–60 months),
yet longer for both the familial case group (6 months, range 1.5–192 months) and
the iatrogenic case group (6.5 months, range 2–25 months).
Figure 3: Age- and sex-specific mortality rates in all Creutzfeldt-Jakob
disease cases, 1993 to 2010
Recent publication During 2010, the ANCJDR published several articles
including an update on pituitary hormone cases in Australia, drawing on the
experience in other countries for comparative analysis.6 In brief, this review
examined the ongoing risk for individuals who received pituitary hormone
extracted from cadavers between 1967 to mid-1985 for the treatment of
infertility and short stature under the Australian Human Pituitary Hormone
Program. This program ceased in mid-1985 after the recognition of a linkage
between treatment and CJD in a recipient in the United States of America.
Australia had the lowest rate of pituitary hormone-related CJD cases across the
countries compared, with the reasons for this not entirely clear. In addition,
given 20 years has passed since the last case of pituitary hormone-related CJD
was identified in Australia, the review discusses the current risk for the
recipient community and raises the potential for changes to the infection
control measures for this recipient cohort in the future.
Top of page
Acknowledgements The ANCJDR wishes to thank families, medical practitioners
and associated staff for their generous support of Australian CJD surveillance.
The ANCJDR also thanks Dr Handan Wand, Dr Matthew Law and Professor John Kaldor
(National Centre in HIV Epidemiology and Clinical Research at the University of
New South Wales) for their expert epidemiological and statistical support.
Author details Ms Genevieve M Klug, Research Assistant
Ms Alison Boyd, Registry Co-ordinator
Miss Amelia McGlade, Research Assistant
Dr Christiane Stehmann, Administrative Assistant
Professor Colin L Masters, Director
Associate Professor Steven J Collins, Director
Corresponding author: Genevieve Klug, Australian National Creutzfeldt-Jakob
Disease Registry, Department of Pathology, The University of Melbourne,
Victoria, 3010, Australia. Telephone: +61 8344 1949. Facsimile: +61 9349 5105.
Email: gmjak@unimelb.edu.au
Top of page
References
snip...see full text ;
Thursday, August 05, 2010
Surveillance of Creutzfeldt-Jakob disease in Australia: 2010 update
Friday, November 06, 2009
Surveillance of Creutzfeldt-Jakob disease in Australia: 2009 update
Infertile Women, Tall Girls, Short and Orphaned Children: The Socially
Vulnerable Prey of Australian Medical Imperialists Healthsharing Women, 1997: 8:
1015. Lynette Dumble, Ph D, M Sc, Senior Research Fellow, History and Philosophy
of Science, University of Melbourne, Parkville, Victoria, 3052, AUSTRALIA.
Senator Bernardi
Senator for South Australia
Michael O’Meara
120 Port Road
P.O. Box 250
Hindmarsh SA 5007
Kinglake 3763
24th February 24, 2009
Dear Senator Bernardi
Re; Senate Committee Inquiry on Men’s Health
It is with regret that the 60 page submission I was preparing for this
inquiry was lost in the recent Kinglake Bushfire,
along with all other property and possessions of mine, and I now submit an
abbreviated submission. Under such
personal adversity, I believe this submission falls within the Terms of
Reference.
Its is with pleasure that this submission be accepted in accordance with
your Notice Of Motion (276) published in
November 2008, some 5 months after a Private Member Motion was read in the
House of Representatives.
The Member for McEwen (Ms Fran Bailey) read a very emotional speech in the
House (Committee Room) on 16th
June 2008, supported by the Member for Moore, (Dr Mal Washer) and further
with bipartisan support by the Rudd
Government - expressed by the Members of Page, (Ms Janelle Saffin) and
Dobell, (Mr. Craig Thomson)
It is with my pleasure that I submit the following Submission on behalf of
myself and all other (then) boys and men
treated with Human Pituitary Hormones, unofficially, and not recorded,
under the Australian Human Pituitary
Hormone Program, and who have suffered, with both short term and long term
side effects to the male endocrine
system as a result of such Human Experimentation, and with such side
effects that are irreversible.
Approved Recipients of Human Growth Hormone or Human Pituitary
Gonadotrohpin were subjected to a Senate
Inquiry in 1993, known as “The Allars Inquiry”, however – unapproved and/or
“Off Program” recipients who were not
included in the Allars Inquiry, and whom were not disclosed to the
Department of Health and Aging, who are at the
same risk of CJD, and were never advised of their risk, particularly
unrecorded recipients of hPG at Prince Henry’s
Hospital in Melbourne – hundreds of males. The Senate now records (1998)
the “Allars Inquiry” was misled.
It is these Males who were “overtreated” with Human Pituitary
Gonadotrophin1, who were “overstimulated” through
invivo experimentation, with batches varying2 and causing dire consequences
to physical, mental and reproductive
health - those who were exposed to anabolic steroids (a carcinogenic) as a
Growth Promotant with severe side
effects. Particular Recommendations were presented and submitted to The
Minister for Heath by Professor Margaret
Allars in 1994, and further explored by the Senate Affairs Reference
Committee in 1998. Of these numerous
recommendations, I draw particular reference to Recommendation 5 m
stating
That the settlement offer should not preclude a plaintiff making any future
claim in relation to: (a) Other physical
illnesses contracted by recipients which may be related to long term side
effects of HPH treatment3
This submission is dedicated to the Infant boys, Toddlers boys,
Prepubescent boys, Teenage boys, Young, Middle
Aged and Elderly Men aged 2 to 101 – who were treated under such
experimental Programs, exposed to Endocrine
Disruptors during the 1970’s, particularly those whom were castrated and
sterilized by the Australian Government
and/or representatives engaged under the Health Act 1958. Such Section with
the Act has since been repealed so
that the “experimental nature” of “The Program” cannot happen again -
following the “Allars Inquiry”. This does not
repeal or repair the ongoing side effects. In particular, I dedicate this
submission to the memory of the child who lost
his life under these experimental programs at Prince Henry’s Hospital
during the 1970’s4.
Please accept my gratitude with appreciation with your efforts in this
forthcoming Inquiry.
Yours Faithfully
Michael O’Meara 1 Reports, Dr N. Tonti-Fillipini (2008), Dr O’Collins
(1979), Dr Burger (1972/3)
2 “As treatment doses and frequency were varied, he was effectively
castrated, with his testes so damaged that puberty was then delayed to such an
extent that he was treated with anabolic steroids to induce puberty”. House of
Representatives Hansard 16th
June 2008
3http://www.carers.health.gov.au/internet/main/publishing.nsf/Content/D1570B0D47832C1DCA256F19000528D6/$File/respons
e.pdf 4 The Testes – Clinical and Experimental Studies, 1983, Burger HG, de
Kretser DM
SNIP...
PDF] PDF 159KB
www.aph.gov.au/DocumentStore.ashx?id=4c6cd595-4e53-411f-aa0e... Cached Share
View shared post Jun 16, 2008 - In particular, I dedicate this submission to the
memory of the child who lost ... “The Australian Human Pituitary Hormone
Program”, in unethical, unapproved, ... between hPG and the fatal disease of
Creutzfeldt-Jakob disease has ... prediction of short stature were treated with
synthetic androgens or steroids ...
androgens or ... I am raising this issue tonight because Mr. Michael
O'Meara, ... [PDF] Submission: Commonwealth Contribution to Former Forced ... https://senate.aph.gov.au/submissions/comittees/viewdocument.aspx?...
Cached Share View shared post Jun 16, 2008 - COMMONWEALTH OF AUSTRALIA ... That
is why I think many of the initiatives under the Helping Children ... with a
prediction of short stature were treated with synthetic ... tween hPG and
Creutzfeldt-Jakob disease and its recommendations to ... I am raising this issue
tonight because Mr Michael O'Meara, ... Hormone Treatments: 16 Jun 2008: House
debates (OpenAustralia ...
www.openaustralia.org/debates/?id=2008-06-16.172.1 Cached Share View
shared post Jun 16, 2008 - This program was known as the Australian Human
Pituitary Hormones Program, known as AHPHP. ... I am raising this issue tonight
because Mr Michael O'Meara, .... for infertility in women and short stature in
children, particularly boys and ... the Creutzfeldt-Jakob disease settlement
offer where it outlined all ...
Saturday, April 14, 2012
Australian Disease Strategy Bovine spongiform encephalopathy Version 3.2
and Creutzfeldt Jakob Disease report 2012
Australian Disease Strategy Bovine spongiform encephalopathy Version 3.2
and Creutzfeldt Jakob Disease report 2012
AUSTRALIAN VETERINARY EMERGENCY PLAN
AUSVETPLAN
Disease Strategy Bovine spongiform encephalopathy Version 3.2, 2012
snip...
Two cases of feline TSE have been diagnosed in imported animals in
Australian zoos. In 1992, a case was seen in a cheetah imported from the UK to a
zoo in Western Australia, and the agent was subsequently typed as the classical
BSE strain (Peet and Curran 1992). This animal and two littermates imported at
the same time were destroyed and incinerated. The source of infection was traced
to a zoo in the UK. In July 2002, a second case was diagnosed in an Asiatic
golden cat imported from the Netherlands (Young and Slocombe 2003). The cat,
which was born in Germany, died suddenly of a pancreatic condition, and the TSE
was detected as an incidental finding on routine histopathology of the brain.
snip...
see full text 61 pages ;
First occurrence of atypical scrapie
Australia is free of scrapie, also known as ‘classical’ scrapie, and has
been assessed as a ‘negligible bovine spongiform encephalopathy (BSE) risk’ (the
lowest risk) by the World Organisation for Animal Health (OIE). Both diseases
belong to a group of diseases termed transmissible spongiform encephalopathies
(TSEs) or ‘prion diseases’.
Active surveillance occurs to validate Australia’s status for both
diseases, through the National Transmissible Spongiform Encephalopathies
Surveillance Program (NTSESP), consistent with OIE recommendations. Results are
routinely reported in Animal Health Surveillance Quarterly.
The first case of atypical scrapie (another TSE) in Australia has been
confirmed in a single sheep, through the NTSESP. This is not a surprising
finding. Atypical scrapie is a rare, sporadic, degenerative brain condition that
spontaneously occurs in a very small proportion of older sheep and, less
commonly, in goats. Most countries that test large numbers of sheep for scrapie
have found one or more cases of atypical scrapie.
Testing on samples from the affected sheep at the CSIRO Australian Animal
Health Laboratory in March 2010 showed preliminary results consistent with
atypical scrapie. The results were confirmed by the Veterinary Laboratory
Agencies at Weybridge in the United Kingdom, an OIE reference laboratory.
Atypical scrapie is clinically, pathologically, biochemically and
epidemiologically unrelated to classical scrapie, and has been recognised as a
distinct disease of sheep and goats for about a decade. During this time, the
disease has been diagnosed in more than 20 countries worldwide. It does not pose
a risk to human health or to the productivity of the Australian sheep flock.
There is evidence that it is not naturally spread to other animals. It is not
known to have any causal relationship to other TSEs, including BSE in cattle,
chronic wasting disease in deer, or any form of Creutzfeldt–Jakob disease in
people.
As atypical scrapie is a different disease to classical scrapie,
Australia’s internationally recognised status as free from scrapie will not
change as a result of this case. Contributed by Reg Butler, Biosecurity Services
Group, Australian Government Department of Agriculture, Fisheries and Forestry
Scrapie
Scrapie has been recognised in sheep for more than 250 years, and occurs at
a low annual incidence in many countries, but is not present in Australia or New
Zealand.
Atypical scrapie In 2009, atypical/Nor98 scrapie was detected in one sheep
brain from a consignment of sheep and goat brains sent from New Zealand to the
European Union, for use as negative control materials for evaluating rapid tests
for BSE and scrapie.50 In 2010, a case of atypical/Nor98 scrapie was diagnosed
in a sheep in Australia.
Australian and New Zealand Standard Diagnostic Procedure, August 2010. Page
7 of 27
The first case of atypical scrapie in Australia was recently detected
through the active surveillance program for transmissible spongiform
encephalopathies (TSEs). Atypical scrapie is a rare, degenerative brain
condition that occurs spontaneously in a very small proportion of older sheep
and goats. It is a different disease to classical scrapie and other known TSEs.
Australia remains free from scrapie.
Thursday, October 7, 2010
Australia first documented case of atypical scrapie confirmed First
occurrence of atypical scrapie
Tuesday, March 16, 2010
COMMONWEALTH OF AUSTRALIA Hansard Import restrictions on beef FRIDAY, 5
FEBRUARY 2010 AUSTRALIA
COMMONWEALTH OF AUSTRALIA
Proof Committee Hansard
RRA&T 2 Senate Friday, 5 February 2010
RURAL AND REGIONAL AFFAIRS AND TRANSPORT
[9.03 am]
BELLINGER, Mr Brad, Chairman, Australian Beef Association CARTER, Mr John
Edward, Director, Australian Beef Association CHAIR—Welcome. Would you like to
make an opening statement?
Mr Bellinger—Thank you. The ABA stands by its submission, which we made on
14 December last year, that the decision made by the government to allow the
importation of beef from BSE affected countries is politically based, not
science based. During this hearing we will bring forward compelling new evidence
to back up this statement. When I returned to my property after the December
hearing I received a note from an American citizen. I will read a small excerpt
from the mail he sent me in order to reinforce the dangers of allowing the
importation of beef from BSE affected countries. I have done a number of press
releases on this topic, and this fellow has obviously picked my details up from
the internet. His name is Terry Singeltary and he is from Bacliff, Texas. He
states, and rightfully so:
You should be worried. Please let me explain. I’ve kept up with the mad cow
saga for 12 years today, on December 14th 1997, some four months post voluntary
and partial mad cow feed ban in the USA, I lost my mother to the Heidenhain
Variant Creutzfeldt-Jakob disease (CJD). I know this is just another phenotype
of the infamous sporadic CJDs. Here in the USA, when USA sheep scrapie was
transmitted to USA bovine, the agent was not UK BSE—it was a different strain.
So why then would human TSE from USA cattle look like UK CJD from UK BSE? It
would not. So this accentuates that the science is inconclusive still on this
devastating disease. He goes on to state:
snip...see full text 110 pages ;
for those interested, please see much more here ;
Rural and Regional Affairs and Transport References Committee
The possible impacts and consequences for public health, trade and
agriculture of the Government's decision to relax import restrictions on beef
Final report June 2010
2.65 At its hearing on 14 May 2010, the committee heard evidence from Dr
Alan Fahey who has recently submitted a thesis on the clinical neuropsychiatric,
epidemiological and diagnostic features of Creutzfeldt-Jakob disease.48 Dr Fahey
told the committee of his concerns regarding the lengthy incubation period for
transmissible spongiform encephalopathies, the inadequacy of current tests and
the limited nature of our current understanding of this group of
diseases.49
2.66 Dr Fahey also told the committee that in the last two years a link has
been established between forms of atypical CJD and atypical BSE. Dr Fahey said
that: They now believe that those atypical BSEs overseas are in fact causing
sporadic Creutzfeldt-Jakob disease. They were not sure if it was due to mad
sheep disease or a different form. If you look in the textbooks it looks like
this is just arising by itself. But in my research I have a summary of a
document which states that there has never been any proof that sporadic
Creutzfeldt-Jakob disease has arisen de novo-has arisen of itself. There is no
proof of that. The recent research is that in fact it is due to atypical forms
of mad cow disease which have been found across Europe, have been found in
America and have been found in Asia. These atypical forms of mad cow disease
typically have even longer incubation periods than the classical mad cow
disease.50
Tuesday, July 13, 2010
(SEE BEEF PRODUCTS EXPORTED TO AUSTRALIA FROM USA...TSS)
AUSTRALIAN QUESTIONNAIRE TO ASSESS BSE RISK (OIE) Terrestrial Animal Health
Code, 2009 and USA export risk factor for BSE to Australia
Saturday, August 14, 2010
USA NON-SPECIES CODING SYSTEM (BEEF IMPORT EXPORT BSE RISK THERE
FROM)
US denies it's illegally sending beef to Australia ?
Friday, 13/08/2010
ODD, we see NO ‘classification pending’ type CJD in Australia ???
like we do the USA ?
SEE RISE IN cpsCJD i.e. classification pending sporadic CJD ;
CANADA CJD UPDATE 2011
CJD Deaths Reported by CJDSS1, 1994-20112 As of January 31, 2011
3. Final classification of 49 cases from 2009, 2010, 2011 is pending.
snip...
USA 2011
USA
National Prion Disease Pathology Surveillance Center
Cases Examined1
(November 1, 2010)
Year Total Referrals2 Prion Disease Sporadic Familial Iatrogenic vCJD
1996 & earlier 51 33 28 5 0 0
1997 114 68 59 9 0 0
1998 87 51 43 7 1 0
1999 121 73 65 8 0 0
2000 146 103 89 14 0 0
2001 209 119 109 10 0 0
2002 248 149 125 22 2 0
2003 274 176 137 39 0 0
2004 325 186 164 21 0 13
2005 344 194 157 36 1 0
2006 383 197 166 29 0 24
2007 377 214 187 27 0 0
2008 394 231 205 25 0 0
2009 425 258 215 43 0 0
2010 333 213 158 33 0 0
TOTAL 38315 22656 1907 328 4 3
1 Listed based on the year of death or, if not available, on year of
referral;
2 Cases with suspected prion disease for which brain tissue and/or blood
(in familial cases) were submitted;
3 Disease acquired in the United Kingdom;
4 Disease was acquired in the United Kingdom in one case and in Saudi
Arabia in the other case;
5 Includes 18 cases in which the diagnosis is pending, and 18 inconclusive
cases;
6 Includes 23 (22 from 2010) cases with type determination pending in which
the diagnosis of vCJD has been excluded.
Please notice where sporadic CJD cases in 1996 went from 28 cases, to 215
cases in 2009, the highest recorded year to date. sporadic CJD is on a steady
rise, and has been since 1996.
I also urge you to again notice these disturbing factors in lines 5 and 6 ;
5 Includes 18 cases in which the diagnosis is pending, and 18 inconclusive
cases;
6 Includes 23 (22 from 2010) cases with type determination pending in which
the diagnosis of vCJD has been excluded.
========end=====tss=====2011
Monday, August 9, 2010
National Prion Disease Pathology Surveillance Center Cases Examined (July
31, 2010)
(please watch and listen to the video and the scientist speaking about
atypical BSE and sporadic CJD and listen to Professor Aguzzi)
Wednesday, March 28, 2012
VARIABLY PROTEASE-SENSITVE PRIONOPATHY IS TRANSMISSIBLE, price of prion
poker goes up again $
2013 TSE PRION UPDATE
*** Saturday, November 2, 2013 ***
Exploring the risks of a putative transmission of BSE to new species
Wednesday, September 25, 2013
Presence of subclinical infection in gene-targeted human prion protein
transgenic mice exposed to atypical BSE
Wednesday, October 30, 2013
SPECIFIED RISK MATERIAL (SRM) CONTROL VERIFICATION TASK FSIS NOTICE 70-13
10/30/13
Monday, August 26, 2013
The Presence of Disease-Associated Prion Protein in Skeletal Muscle of
Cattle Infected with Classical Bovine Spongiform Encephalopathy
Tuesday, September 24, 2013
NORDION (US), INC., AND BIOAXONE BIOSCIENCES, INC., Settles $90M Mad Cow
TSE prion Contamination Suit Cethrin(R)
*** Case 0:12-cv-60739-RNS Document 1 Entered on FLSD Docket 04/26/2012
Page 1 of 15 ***
Tuesday, July 2, 2013
APHIS USDA Administrator Message to Stakeholders: Agency Vision and Goals
Eliminating ALL remaining BSE barriers to export market
Saturday, November 2, 2013
*** APHIS Finalizes Bovine Import Regulations in Line with International
Animal Health Standards while enhancing the spread of BSE TSE prion mad cow type
disease around the Globe ***
*** The discovery of previously unrecognized prion diseases in both humans
and animals (i.e., Nor98 in small ruminants) demonstrates that the range of
prion diseases might be wider than expected and raises crucial questions about
the epidemiology and strain properties of these new forms. We are investigating
this latter issue by molecular and biological comparison of VPSPr, GSS and
Nor98.
Saturday, July 6, 2013
*** Small Ruminant Nor98 Prions Share Biochemical Features with Human
Gerstmann-Sträussler-Scheinker Disease and Variably Protease-Sensitive
Prionopathy
Research Article
Saturday, October 19, 2013
ACA Council Meets to Endorse Several Proposed USAHA Resolutions (CWD TSE
PRION DISEASE)
Wednesday, October 09, 2013
WHY THE UKBSEnvCJD ONLY THEORY IS SO POPULAR IN IT'S FALLACY, £41,078,281
in compensation REVISED
Thursday, October 10, 2013
CJD REPORT 1994 increased risk for consumption of veal and venison and lamb
Monday, October 14, 2013
Researchers estimate one in 2,000 people in the UK carry variant CJD
proteins
Friday, August 16, 2013
*** Creutzfeldt-Jakob disease (CJD) biannual update August 2013 U.K. and
Contaminated blood products induce a highly atypical prion disease devoid of
PrPres in primates
WHAT about the sporadic CJD TSE proteins ?
WE now know that some cases of sporadic CJD are linked to atypical BSE and
atypical Scrapie, so why are not MORE concerned about the sporadic CJD, and all
it’s sub-types $$$
Creutzfeldt-Jakob Disease CJD cases rising North America updated report
August 2013
*** Creutzfeldt-Jakob Disease CJD cases rising North America with Canada
seeing an extreme increase of 48% between 2008 and 2010 ***
Sunday, October 13, 2013
CJD TSE Prion Disease Cases in Texas by Year, 2003-2012
In Confidence - Perceptions of unconventional slow virus diseases of
animals in the USA - APRIL-MAY 1989 - G A H Wells
3. Prof. A. Robertson gave a brief account of BSE. The US approach was to
accord it a very low profile indeed. Dr. A Thiermann showed the picture in the
''Independent'' with cattle being incinerated and thought this was a fanatical
incident to be avoided in the US at all costs. ...
and they meant it $$$
Sunday, November 3, 2013
Environmental Impact Statements; Availability, etc.: Animal Carcass
Management [Docket No. APHIS-2013-0044]
Saturday, November 9, 2013
Republicans put 'national interest' requirement on US science agency
with sad regards, these are the facts as I have come to know them. ...
terry
layperson MOM DOD 12/14/97 confirm ‘hvCJD’...just made a promise to mom,
NEVER FORGET!
posted by Terry S. Singeltary Sr. at
10:45 PM
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