Accuracy of administrative diagnostic data for pathologically confirmed cases of Creutzfeldt-Jakob disease in Massachusetts, 2000-2008

Barash, Jed A.; West, James K.; DeMaria, Alfred

doi:10.1016/j.ajic.2014.02.002

Cited by 6 publications

(4 citation statements)

References 16 publications

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“…The potentially treatable alternative diagnoses in France are similar to those of other national series reported in Europe [ 14 , 16 - 19 ] and in various other countries: the United States [ 20 , 21 ], Japan [ 22 ], Argentina [ 23 ] and China [ 24 ]. In the Dutch series that included suspicions between 1998 and 2009, a substantial group (ca 14.0%) suffered from potentially treatable disorders such as infectious disease, cancer-associated disease, or toxic/metabolic disorders.…”

Section: Discussionsupporting

confidence: 83%

Accuracy of diagnosis criteria in patients with suspected diagnosis of sporadic Creutzfeldt-Jakob disease and detection of 14-3-3 protein, France, 1992 to 2009

et al. 2017

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International audienceDiagnostic criteria of Creutzfeldt–Jakob disease (CJD), a rare and fatal transmissible nervous system disease with public health implications, are determined by clinical data, electroencephalogram (EEG), detection of 14-3-3 protein in cerebrospinal fluid (CSF), brain magnetic resonance imaging and prion protein gene examination. The specificity of protein 14-3-3 has been questioned. We reviewed data from 1,572 autopsied patients collected over an 18-year period (1992–2009) and assessed whether and how 14-3-3 detection impacted the diagnosis of sporadic CJD in France, and whether this led to the misdiagnosis of treatable disorders. 14-3-3 detection was introduced into diagnostic criteria for CJD in 1998. Diagnostic accuracy decreased from 92% for the 1992–1997 period to 85% for the 1998–2009 period. This was associated with positive detections of 14-3-3 in cases with negative EEG and alternative diagnosis at autopsy. Potentially treatable diseases were found in 163 patients (10.5%). This study confirms the usefulness of the recent modification of diagnosis criteria by the addition of the results of CSF real-time quaking-induced conversion, a method based on prion seed-induced misfolding and aggregation of recombinant prion protein substrate that has proven to be a highly specific test for diagnosis of sporadic CJD

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Section: Discussionsupporting

confidence: 83%

Accuracy of diagnosis criteria in patients with suspected diagnosis of sporadic Creutzfeldt-Jakob disease and detection of 14-3-3 protein, France, 1992 to 2009

et al. 2017

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“…In Massachusetts from 2000 to 2008, a death certificate diagnosis of CJD, which closely matched hospital discharge dataset diagnosis code 046.1, was 71% sensitive and 75% specific for pathologically confirmed CJD. 25 In the United States between 2003 and 2005, 90.7% of suspected prion disease cases for which physicians obtained neuropathologic results were confirmed. 11 Similarly, in Australia between 1970 and 1999, the code 046.1 on a death certificate had a sensitivity of 83.0%, and a 11.5% false-positive rate for CJD, yielding a positive predictive value of 88.5%.…”

Section: Discussionmentioning

confidence: 99%

Incidence of Creutzfeldt-Jakob Disease in the United States from 2000 to 2019

Seitz,

Zhang,

Navi

et al. 2024

Preprint

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Background and purpose: To test the hypothesis that the incidence of Creutzfeldt-Jakob disease (CJD) has remained constant, we calculated the rate of hospitalizations for CJD in the United States using the National Inpatient Sample (NIS) from 2000 to 2019. Methods: We used ICD-9 and ICD-10 codes to identify people hospitalized with presumed CJD in the National Inpatient Sample (NIS) from 2000 to 2019. Survey weights were used to calculate nationally representative estimates. We used 2000 census data to calculate age-adjusted standardized rates of CJD hospitalizations by sex and race-ethnicity and then used Joinpoint regression to evaluate changes in those rates. Results: From 2000 to 2019, there were 11,064 admissions for CJD across the U.S. Across this period, the age-adjusted rate of CJD-related hospitalizations increased significantly from 1.25 (95% CI, 1.25-1.26) to 1.98 (95% CI, 1.98-1.99) per million U.S. adults per year, with a significant annual percentage change between 2004 and 2013 of 7.6% (95% CI, 4.4%-10.9%). Conclusions: The incidence of CJD increased in the United States from 2000 to 2019, with a significant increase specifically between 2004 and 2013, though the overall case rate remains low.

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“…11 A smaller US study found the sensitivity of death certificates to identify confirmed CJD as 27/38 (71%). 12 Potential issues related to death certificates identifying confirmed CJD include the seeking of brain tissue confirmations more often when clinical diagnoses are less certain (e.g., clinically less typical cases) and completing death certificates before neuropathological results are available. Another important limitation of the current study was the difficulty in gathering data required to satisfy inclusion and follow-up criteria for the study.…”

Section: Discussionmentioning

confidence: 99%

“…In addition, a larger US epidemiological study using multiple sources for ascertaining cases estimated the death certificate sensitivity for ascertaining “potential” CJD cases as 247/273 (90%) and for identifying the subset of patients for whom physicians obtained brain tissue confirmation, 55/69 (80%) 11 . A smaller US study found the sensitivity of death certificates to identify confirmed CJD as 27/38 (71%) 12 . Potential issues related to death certificates identifying confirmed CJD include the seeking of brain tissue confirmations more often when clinical diagnoses are less certain (e.g., clinically less typical cases) and completing death certificates before neuropathological results are available.…”

Section: Discussionmentioning

confidence: 99%

Absence of evidence of transfusion transmission risk of Creutzfeldt‐Jakob disease in the United States: Results froma 28‐year lookback study

Crowder,

Dodd,

Schonberger

2024

Transfusion

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BackgroundFor many years, there has been concern about the risk of transmission of classic forms of Creutzfeldt‐Jakob disease (CJD) by blood transfusion, particularly after the recognition of such transmission of variant CJD (vCJD). We report on a 28‐year lookback study of recipients of blood from donors who subsequently developed CJD.MethodsPatients with diagnosed CJD and a history of blood donation were identified. Blood centers were asked to provide information about the distribution of the donations and consignees were requested to provide information about the recipients of the donations. Vital status of each available recipient was determined and, if deceased, the reported cause(s) of death were obtained primarily from the National Death Index. All recipients included in the study database contributed person‐time up to the last recorded review of vital status.ResultsThere were 84 eligible donors who gave 3284 transfusable components, and it was possible to evaluate 1245 recipients, totaling 6495 person‐years of observation. The mean observation period per recipient was 5.5 years with a maximum of 51 years. No case of CJD or prion disease was reported among the recipient population.DiscussionThe study suggests that CJD may not be transfusion‐transmissible, a position in agreement with similar findings from two similar European reports amounting to an overall observation period of 15,500 person‐years. These studies have supported the conclusion that the risk, if any, of transmission of CJD by blood products is extremely small and remains theoretical.

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Accuracy of administrative diagnostic data for pathologically confirmed cases of Creutzfeldt-Jakob disease in Massachusetts, 2000-2008

Cited by 6 publications

References 16 publications

Accuracy of diagnosis criteria in patients with suspected diagnosis of sporadic Creutzfeldt-Jakob disease and detection of 14-3-3 protein, France, 1992 to 2009

Accuracy of diagnosis criteria in patients with suspected diagnosis of sporadic Creutzfeldt-Jakob disease and detection of 14-3-3 protein, France, 1992 to 2009

Incidence of Creutzfeldt-Jakob Disease in the United States from 2000 to 2019

Absence of evidence of transfusion transmission risk of Creutzfeldt‐Jakob disease in the United States: Results froma 28‐year lookback study

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