Journal ArticleDOI
Incubation period of Creutzfeldt-Jakob disease in human growth hormone recipients in France.
J. Huillard d’Aignaux,Dominique Costagliola,Jean Maccario,T. Billette de Villemeur,J.-P. Brandel,Jean-Philippe Deslys,Jean-Jacques Hauw,J.L. Chaussain,Yves Agid,Dominique Dormont,Annick Alpérovitch +10 more
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TLDR
The short mean incubation period of CJD in French hGH recipients may be due to high infectivity in hormone lots, and estimates of the 95th percentile indicate that the number of hGH-related CJD cases may continue to increase in the coming years.Abstract:
Objective: To estimate the statistical distribution of the incubation period of Creutzfeldt–Jakob disease (CJD) in human growth hormone (hGH) recipients in France. Background: Published papers suggest that the median incubation period of hGH-related CJD is approximately 15 years, but there are as yet no statistical data that support this assertion. Methods: Of the 1,361 hGH recipients who were included in this study, 55 had developed CJD at the time of the study. Individual data on hGH treatment history were available. Different mathematical models were used to estimate the statistical distribution of the incubation period. One main feature of the models was to take into account the occurrence of future CJD cases. Results: Models showed that the mean incubation period was 9 to 10 years, and the 95th percentile of the distribution was 15 to 16 years. Data and models indicated that the incubation period was significantly shorter in homozygotes at codon 129 of the prion protein gene than in heterozygotes. Conclusions: The short mean incubation period of CJD in French hGH recipients may be due to high infectivity in hormone lots. Estimates of the 95th percentile indicate that the number of hGH-related CJD cases may continue to increase in the coming years.read more
Citations
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Journal ArticleDOI
Iatrogenic Creutzfeldt-Jakob disease at the millennium.
Patrick O. Brown,Michael A. Preece,J.-P. Brandel,Takeshi Sato,Lisa M. McShane,I. Zerr,Ashley Fletcher,Robert G. Will,Maurizio Pocchiari,Neil R. Cashman,J.H. d’Aignaux,Larisa Cervenakova,J. Fradkin,Lawrence B. Schonberger,Steven J. Collins +14 more
TL;DR: Knowledge about potential high-risk sources of contamination gained during the last quarter century, and the implementation of methods to circumvent them, should minimize the potential for iatrogenic contributions to the current spectrum of CJD.
Journal ArticleDOI
Balancing selection at the prion protein gene consistent with prehistoric kurulike epidemics.
Simon Mead,Michael P. H. Stumpf,Jerome Whitfield,Jon Beck,Mark Poulter,Tracy Campbell,James Uphill,David Goldstein,Michael P. Alpers,Elizabeth M. C. Fisher,John Collinge +10 more
TL;DR: Worldwide PRNP haplotype diversity and coding allele frequencies suggest that strong balancing selection at this locus occurred during the evolution of modern humans.
Journal ArticleDOI
Bovine spongiform encephalopathy and variant Creutzfeldt-Jakob disease: background, evolution, and current concerns.
TL;DR: The epidemic of bovine spongiform encephalopathy in the United Kingdom is waning to a conclusion, but leaves in its wake an outbreak of human Creutzfeldt-Jakob disease, most probably resulting from the consumption of beef products contaminated by central nervous system tissue.
Journal ArticleDOI
Estimation of Epidemic Size and Incubation Time Based on Age Characteristics of vCJD in the United Kingdom
TL;DR: The size of the variant Creutzfeldt-Jakob Disease epidemic in the United Kingdom is a major public health concern and a subject of speculation and the mean duration of the incubation period is 16.7 years.
Journal ArticleDOI
EEG in Creutzfeldt-Jakob disease
TL;DR: The EEG exhibits characteristic changes depending on the stage of the disease, ranging from nonspecific findings such as diffuse slowing and frontal rhythmic delta activity (FIRDA) in early stages to disease-typical periodic sharp wave complexes (PSWC) in middle and late stages to areactive coma traces or even alpha coma in preterminal EEG recordings.
References
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Journal ArticleDOI
A new variant of Creutzfeldt-Jakob disease in the UK
Robert G. Will,James W. Ironside,M. Zeidler,Simon Cousens,K Estibeiro,Annick Alpérovitch,Sigrid Poser,Maurizio Pocchiari,Albert Hofman,Pete Smith +9 more
TL;DR: Ten cases of Creutzfeldt-Jakob disease have been identified in the UK in recent months with a new neuropathological profile that raises the possibility that they are causally linked to BSE.
Journal ArticleDOI
Transmissions to mice indicate that ‘new variant’ CJD is caused by the BSE agent
Moira E. Bruce,Robert G. Will,James W. Ironside,I. McConnell,D. Drummond,A. Suttie,L. McCardle,A. Chree,James Hope,Christopher R. Birkett,Simon Cousens,H. Fraser,Christopher J. Bostock +12 more
TL;DR: It is shown that the strain of agent from cattle affected by bovine spongiform encephalopathy (BSE) produces a characteristic pattern of disease in mice that is retained after experimental passage through a variety of intermediate species, providing strong evidence that the same agent strain is involved in both BSE and vCJD.
Journal ArticleDOI
Molecular analysis of prion strain variation and the aetiology of 'new variant' CJD
TL;DR: Strain characteristics revealed here suggest that the prion protein may itself encode disease phenotype, consistent with BSE being the source of this new disease.
Journal ArticleDOI
Genetic predisposition to iatrogenic Creutzfeldt-Jakob disease.
TL;DR: Examination of the prion protein genes of 4 CJD cases showed that 4 had the uncommon valine 129 homozygous genotype indicating genetic susceptibility to prion infection, which may be important in the aetiology of sporadic CJD disease.
Journal ArticleDOI
Distinct interactions of PML-RARα and PLZF-RARα with co-repressors determine differential responses to RA in APL
Li Zhen He,Fabien Guidez,Caria Tribioli,Daniela Peruzzi,Martin Ruthardt,Arthur Zelent,Pier Paolo Pandolfi +6 more
TL;DR: It is shown that Histone deacetylase inhibitors such as Trichostatin A (TSA), in combination with RA, can overcome the transcriptional repressor activity of PML-RARα and PLZF-Rarα as well as the unresponsiveness of PLZ fusions-expressing leukaemic cells to RA.