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Surveillance for creutzfeldt-Jakob disease in Australia: update to December 2012

Posted Nov 09 2013 11:45pm
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.

 


 

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.

 


 

 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.

 

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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.

 

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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.

 

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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

 

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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!
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