Creutzfeldt - Jakob Disease in South West Sydney 2014-2020: An Unusually High Incidence of a Rare Disease

2021 ◽  
Author(s):  
Namraj Goire ◽  
Leon Edwards ◽  
Peter Thomas ◽  
Sonu M.M. Bhaskar ◽  
Dennis Cordato ◽  
...  
Keyword(s):  
Information Exchange ◽  
Health Information Exchange ◽  
Large Population ◽  
Cost Effective ◽  
Cellular Prion Protein ◽  
Screening Tools ◽  
World Health ◽  
Spongiform Encephalopathy ◽  
Local Health ◽  
Jakob Disease

Introduction Creutzfeldt - Jakob Disease (CJD), a spongiform encephalopathy, caused by a transmissible misfolded cellular prion protein is a rapidly progressive, debilitating neuro-degenerative disorder with no effective treatment. The estimated global incidence is at 1/ million inhabitants. This retrospective study examined the incidence of CJD in South Western Sydney Local Health District (SWSLHD) from 2014 to 2020. Background SWSLHD had an estimated population of 1,038,534 in 2020, with CJD data being limited. Methods New South Wealth (NSW) Health-Information-Exchange (HIE) database, for all admissions with CJD diagnoses in SWSLHD, between 2014–2020, was reviewed according to World Health Organization (WHO) diagnostic criteria, consistent with the Australian national CJD registry. Only probable CJD cases were included. Incidence was calculated based on the projected SWSLHD population. Results 35 patients, diagnosed with CJD, were identified. Each was evaluated by two independent investigators, including clinical presentation; MRI; EEGs; 14-3-3; and RT-QUIC results, before assigning CJD-probable status. Four failed the CJD criteria and were excluded. Of the 31 CJD-probable cases, most (59%) were male and older (37% range 61-70 years). The incidence rate peaked at 9/million in 2017 and was above 2/million, throughout the seven years, with an average of 4.859/million/year. Conclusions The incidence of CJD, in SWSLHD, exceeds the national average of 1/million. Cost-effective, adequate diagnostic and screening tools, implementable over a large population, will become increasingly essential.  

scholarly journals 033 Cluster of creuzfeldt-jakob disease in the south western sydney local health district population in the past 5 years

2019 ◽  
Vol 90 (e7) ◽  
pp. A11.2-A12
Author(s):  
Roshan Dhanapalaratnam ◽  
Cecilia Cappelen-Smith ◽  
Peter Thomas ◽  
Roy G Beran
Keyword(s):  
Information Exchange ◽  
Health Information Exchange ◽  
Health District ◽  
World Health ◽  
Local Health ◽  
The South ◽  
District Health ◽  
Paraneoplastic Encephalitis ◽  
Jakob Disease ◽  
Health Organization

IntroductionNoting a perceived increased case-load of Creuzfeldt-Jakob disease (CJD) patients at Liverpool Hospital, in the South-Western Sydney Local Health District (SWSLHD), the authors questioned the accepted death from CJD incidence of 1 per million.MethodsWe reviewed the District Health-Information-Exchange (HIE) database for all admissions with CJD diagnoses in SWSLHD hospitals, over a 5-year period 2014–2018. The SWSLHD services a population of 1,001,437. The HIE identified cases were reviewed according to World Health Organization (WHO) diagnostic criteria as used by the Australian national CJD registry. Definite and probable CJD cases were included in the study. Incidence per year was calculated from the final case number across the 5-year SWSLHD population.Results26 patients coded with a diagnosis of CJD were identified from HIE database over the period. These cases were critically reviewed and 20 confirmed as definite (n=4) or probable CJD (n=16) cases. The diagnoses of excluded patients (n=6) were herpes simplex encephalitis, paraneoplastic encephalitis, delirium, and 2 with progressive multifocal leukoencephalopathy. Calculated incidence based on 5-year SWSLHD population was 4.16 cases per 1 million population/year.ConclusionsIn the 5-year period, we note an incidence of definite and probable CJD cases in the SWSLHD population, four times the national and worldwide reported rates. Cases from the last 2-months of 2018 are pending at the time of submission, which may alter these observations. These findings most likely represent a cluster of cases in the SWSLHD area but may alternatively reflect either better recognition or increased incidence of CJD.

Balancing evidence and public opinion in health technology assessments: The case of leukoreduction

2006 ◽  
Vol 22 (4) ◽  
pp. 403-407 ◽  
Author(s):  
Irina Cleemput ◽  
Mark Leys ◽  
Dirk Ramaekers ◽  
Luc Bonneux
Keyword(s):  
Safety Management ◽  
Private Insurance ◽  
White Blood Cells ◽  
Cost Effective ◽  
Spongiform Encephalopathy ◽  
Blood Safety ◽  
Specific Patient ◽  
Jakob Disease ◽  
Prion Transmission ◽  
Definition Of

Leukoreduction, filtering white blood cells from transfusion blood, effectively avoids leukocyte-related complications of blood transfusion. The technology has proven its relative cost-effectiveness for specific patient populations. With the advent of variant Creutzfeldt–Jakob disease, a transmittable spongiform encephalopathy caused by mad cow disease (bovine spongiform encephalopathy), the hard hit United Kingdom introduced universal leukoreduction for all patients as a precaution for transmission of prions in 1999. This costly policy was followed by many other countries, in the absence of much evidence of an actual health problem or of a more than presumed effectiveness of leukoreduction in preventing prion transmission. The core problem proved to be legal. The blood banks are legally accountable for blood safety. This accountability is absolute, based on avoidance of all possible risks, regardless of costs. This strategy leads to inefficiencies in health care: (i) blood safety management is guided by available rather than cost-effective technology, and (ii) private insurance premiums for civil liability are sharply increasing, while they are in no way related to the expected returns and the high and increasing blood safety. A rational safety policy is to be optimal, taking into account costs and effects of the safety procedures. This issue will need an open discussion with the general public of the real risks and a clear and unambiguous definition of proportionality in the precautionary principle, based on the European law.

scholarly journals A Meaningful Journey to Onboard Syndromic Data through a Local HIE

2015 ◽  
Vol 7 (1) ◽  
Author(s):  
Jeffrey Johnson ◽  
Jessica Yen ◽  
Brit Colanter ◽  
Eric McDonald
Keyword(s):  
Public Health ◽  
Information Exchange ◽  
Health Information Exchange ◽  
Health Agency ◽  
Meaningful Use ◽  
The United States ◽  
Lessons Learned ◽  
Local Health ◽  
Local Public Health ◽  
Public Health Agencies

This presentation aims to highlight key activities, technical approaches, data discoveries, lessons learned and outcomes achieved while onboarding local hospitals for syndromic Meaningful Use Stage 2 through a local health information exchange. The federal meaningful use initiative is currently a major driver to enable greater establishment of syndromic surveillance capacity across the United States. The role and efforts by local and state public health agencies in the syndromic onboarding process varies greatly. We describe efforts from a local public health agency to onboard, validate and integrate meaningful use syndromic information.

Health Information Exchange for Improving the Efficiency and Quality of Healthcare Delivery

2011 ◽  
pp. 352-374 ◽  
Author(s):  
Jing Shi ◽  
Sudhindra Upadhyaya ◽  
Ergin Erdem
Keyword(s):  
Health Information ◽  
Information Exchange ◽  
Health Information Exchange ◽  
Critical Role ◽  
Healthcare Delivery ◽  
Cost Effective ◽  
Quality Of Healthcare ◽  
Future Research ◽  
The Right

In healthcare industry, providers, patients, and all other stakeholders must have the right information at the right time for achieving efficient and cost effective services. Exchange of information between the heterogeneous system entities plays a critical role. Health information exchange (HIE) is not only a process of transmitting data, but also a platform for streamlining operations to improve healthcare delivery in a secure manner. In this chapter, we present a comprehensive view of electronic health record (EHR) systems and HIE by presenting their architecture, benefits, challenges, and other related issues. While providing information on the current state of EHR/HIE applications, we also discuss advanced issues and secondary uses of HIE implementations, and shed some light on the future research in this area by highlighting the challenges and potentials.

scholarly journals Barriers and facilitators to a health information exchange system for general practitioners

2020 ◽  
Vol 30 (Supplement_5) ◽  
Author(s):  
N S Centemero ◽  
B Rechel
Keyword(s):  
Clinical Practice ◽  
Health Information ◽  
General Practitioners ◽  
Information Exchange ◽  
Medical Information ◽  
Health Information Exchange ◽  
Barriers And Facilitators ◽  
Local Health ◽  
Care Providers

Abstract Background Health information exchange (HIE) systems, that are computer-based tools used by healthcare providers for secure access to share patient's medical information electronically, seem to help reduce the use of specific resources and improve the quality of care. This highlights the importance of this issue in the Public Health sector. This research goal is to identify barriers and facilitators perceived by general practitioners (GPs) when using an HIE system in a Southern Switzerland area. Methods we performed a qualitative study using semi-structured interviews, interviewed 10 GPs, randomly selected among some 500 HIE system local users and analysed interview transcripts using thematic content analysis with an abductive approach (a mix of deductive and inductive approaches). Results findings showed the following key facilitators: a) perception of dealing with a secure system; b) possibility of delegating management to secretaries and healthcare assistants; c) technical support and training; d) high quality of the information exchange; e) positive impact on clinical practice; and f) regional context. However, major challenges persist, and GPs reported the following main barriers to using an HIE system: a) frequent lack of all patient information needed; b) no effective workflow improvements; c) lack of some technical features. Conclusions We propose four recommendations based on findings: 1. Future initiatives should focus on developing HIE systems giving GPs access to all possible patient medical information; 2. Crucial data privacy and security issues should never be overlooked; 3. Technical and workflow improvements should particularly consider the socio-technical nature of HIE systems; 4. Much attention needs to be paid to the importance of relationships between health care providers and between these and local health institutions when implementing HIE systems. Key messages This study filled a research gap as it is the first that tackles HIE system barriers and facilitators in Ticino. When information sharing for clinical practice focuses on improving the quality and costs of healthcare, GP's trust in HIE system security is crucial.

scholarly journals Vaccination with Prion Peptide-Displaying Polyomavirus-Like Particles Prolongs Incubation Time in Scrapie-Infected Mice

Viruses ◽  
2021 ◽  
Vol 13 (5) ◽  
pp. 811
Author(s):  
Martin Eiden ◽  
Alma Gedvilaite ◽  
Fabienne Leidel ◽  
Rainer G. Ulrich ◽  
Martin H. Groschup
Keyword(s):  
Prion Diseases ◽  
Mammalian Species ◽  
Cellular Prion Protein ◽  
Control Group ◽  
Spongiform Encephalopathy ◽  
Post Inoculation ◽  
Self Tolerance ◽  
Jakob Disease ◽  
Capsid Protein Vp1 ◽  
Conformational Conversion

Prion diseases like scrapie in sheep, bovine spongiform encephalopathy (BSE) in cattle or Creutzfeldt–Jakob disease (CJD) in humans are fatal neurodegenerative diseases characterized by the conformational conversion of the normal, mainly α-helical cellular prion protein (PrPC) into the abnormal β-sheet rich infectious isoform PrPSc. Various therapeutic or prophylactic approaches have been conducted, but no approved therapeutic treatment is available so far. Immunisation against prions is hampered by the self-tolerance to PrPC in mammalian species. One strategy to avoid this tolerance is presenting PrP variants in virus-like particles (VLPs). Therefore, we vaccinated C57/BL6 mice with nine prion peptide variants presented by hamster polyomavirus capsid protein VP1/VP2-derived VLPs. Mice were subsequently challenged intraperitoneally with the murine RML prion strain. Importantly, one group exhibited significantly increased mean survival time of 240 days post-inoculation compared with 202 days of the control group. These data show that immunisation with VLPs presenting PrP peptides may represent a promising strategy for an effective vaccination against transmissible spongiform encephalitis agents.

scholarly journals Review of foreign experience in the implementation of eHealth

2021 ◽  
Vol 14 (1) ◽  
pp. 63-71
Author(s):  
P. V. Minaev ◽  
D. V. Fedyaev ◽  
Yu. V. Seryapina ◽  
T. V. Skomorokhova
Keyword(s):  
Information And Communication Technologies ◽  
Information Exchange ◽  
Swot Analysis ◽  
Health Information Exchange ◽  
European Countries ◽  
World Health ◽  
The European Union ◽  
Medical Systems ◽  
Regional Office ◽  
Cross Border

A review of studies, presentations and reports of the Regional Office for Europe of the World Health Organization (WHO) devoted to the experience of European countries in the formation of cross-border eHealth and the use of quality management tools for medical activities to reduce risks when introducing innovative solutions in health systems was carried out. In order to comprehensively assess the information received, a SWOT analysis was fulfilled – an analysis of strengths (S), weaknesses (W), opportunities (O) and threats (T), internal and external factors that influenced the development of cross-border eHealth in the European Union. It is shown that in European countries with a developed health care system, the leading direction in building cross-border e-Health are innovative technologies and advanced solutions aimed at improving the interaction of medical systems: Electronic Health Record (EHR), Health Information Exchange (HIE), Telemedicine Technologies (Telehealth), and Personal Health Records (PHR). The analysis of the strengths and weaknesses of this technologies, as well as the main problems that have affected the transition to eHealth and the implementation of information and communication technologies in medical organizations of European countries has revealed common factors and patterns that can be taken into account for the digital transformation of the health system in the Russian Federation.

scholarly journals From Prion Diseases to Prion-Like Propagation Mechanisms of Neurodegenerative Diseases

2013 ◽  
Vol 2013 ◽  
pp. 1-8 ◽  
Author(s):  
Isabelle Acquatella-Tran Van Ba ◽  
Thibaut Imberdis ◽  
Véronique Perrier
Keyword(s):  
Neurodegenerative Disorders ◽  
Small Rnas ◽  
Prion Diseases ◽  
Cellular Prion Protein ◽  
Spongiform Encephalopathy ◽  
Wasting Disease ◽  
Original Works ◽  
Jakob Disease ◽  
The Brain ◽  
Prion Hypothesis

Prion diseases are fatal neurodegenerative sporadic, inherited, or acquired disorders. In humans, Creutzfeldt-Jakob disease is the most studied prion disease. In animals, the most frequent prion diseases are scrapie in sheep and goat, bovine spongiform encephalopathy in cattle, and the emerging chronic wasting disease in wild and captive deer in North America. The hallmark of prion diseases is the deposition in the brain of PrPSc, an abnormalβ-sheet-rich form of the cellular prion protein (PrPC) (Prusiner 1982). According to the prion hypothesis, PrPSccan trigger the autocatalytic conversion of PrPCinto PrPSc, presumably in the presence of cofactors (lipids and small RNAs) that have been recently identified. In this review, we will come back to the original works that led to the discovery of prions and to the protein-only hypothesis proposed by Dr. Prusiner. We will then describe the recent reports on mammalian synthetic prions and recombinant prions that strongly support the protein-only hypothesis. The new concept of “deformed templating” regarding a new mechanism of PrPScformation and replication will be exposed. The review will end with a chapter on the prion-like propagation of other neurodegenerative disorders, such as Alzheimer’s and Parkinson’s disease and tauopathies.

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