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From: TSS (216-119-134-158.ipset14.wt.net)
Subject: Re: PREDICTORS OF SURVIVAL IN sCJD AND OTHER TSEs
Date: September 29, 2004 at 6:30 pm PST

In Reply to: PREDICTORS OF SURVIVAL IN sCJD AND OTHER TSEs posted by TSS on September 27, 2004 at 8:42 am:

-------- Original Message --------
Subject: Re: Predictors of survival in sporadic Creutzfeldt–Jakob disease and other human transmissible spongiform encephalopathies
Date: Wed, 29 Sep 2004 11:03:04 -0500
From: "Terry S. Singeltary Sr."
Reply-To: Bovine Spongiform Encephalopathy
To: BSE-L@UNI-KARLSRUHE.DE
References: <415836C1.2050305@wt.net>


##################### Bovine Spongiform Encephalopathy #####################

Greetings list members,

I find it most interesting that Germany sporadic CJD victims
had a significantly (P<0.0001) longer median duration (6 months,
n = 568) of illness than patients from all the other countries:
5 months for spain (n = 123), 4.5 months for Switzerland (n = 43),
and 4 months for Australia (n = 149), Austria (n = 29), France
(n = 543), Italy (n = 379), Slovakia (n = 12), the Netherlands (n = 70)
and the UK (n = 388).

Univariate survival analyses of sCJD cases with available data
(see Table 2) showed differences in the duration of illness by
gender (Fig. 2A), age at onset (Fix. 2B) and the polymorphism
at codon 129 of the PRNP gene (Fig. 2C). The multivariate
analysis confirmed that survival is longer in females compared
with males and showed that increments of 10 years in the age
at onset are associated with an ~30% increase in risk of death
(Table 3A). Survival was shorter in patients who were MM at
codon 129 of the PRNP gene (Table 3A) compared with that
seen in valilne/valine (VV) and methionine/valine (MV) cases.

snip...

The exception is a significant increase in the median survival
in sCJD in Germany in comparison with the other countries
(4 months in the majority compared with 6 months in Germany).
The reason for this finding may be because of a systematic
difference in classification of the time of disease onset. However,
the analyses are not affected by this anomaly...............

MIGHT anyone have other potential reasons of the difference
in sCJD cases in Germany with longer median duration?

There must be a reason, route, source, titre of infectivity,
ethnically reasons (diet practices), genetics, all of above or
something else, something has to be here? what is it?
got to be a reason?

kindest regards,
Terry

Terry S. Singeltary Sr. wrote:

> ##################### Bovine Spongiform Encephalopathy
> #####################
>
> Predictors of survival in sporadic Creutzfeldt–Jakob disease and
> other human transmissible spongiform encephalopathies
>
> M. Pocchiari1, M. Puopolo1, E. A. Croes2, H. Budka3, E. Gelpi3, S.
> Collins4, V. Lewis4, T. Sutcliffe5, A. Guilivi5, N.
> Delasnerie-Laupretre6, J.-P. Brandel6, A. Alperovitch6, I. Zerr7, S.
> Poser7, H. A. Kretzschmar8, A. Ladogana1, I. Rietvald2, E. Mitrova9, P.
> Martinez-Martin10, J. de Pedro-Cuesta10, M. Glatzel11, A. Aguzzi11, S.
> Cooper12, J. Mackenzie12, C. M. van Duijn2 and R. G. Will12
>
> 1 Istituto Superiore di Sanità, Department of Cell Biology and
> Neurosciences Rome, Italy, 2 Department of Epidemiology and
> Biostatistics, Erasmus MC, Rotterdam, The Netherlands, 3 Austrian
> Reference Centre for Human Prion Diseases (OERPE) and Institute of
> Neurology Department of Cell Biology and Neuro Sciences, Vienna,
> Austria, 4 Department of Pathology, The University of Melbourne,
> Victoria, Australia, 5 Blood Safety Surveillance and Health Care
> Acquired Infections Division, Centre for Infectious Disease Prevention
> and Control, LCDC Building, Ontario, Canada, 6 U.360 INSERM, Hôpital de
> la Salpetriere, Paris, France, 7 Department of Neurology,
> Georg-August-Universität Göttingen, 8 Institute of Neuropathology,
> University of Munich, Munich, Germany, 9 Institute of Preventative and
> Clinical Medicine, National Reference Centre of Slow Virus
> Neuroinfections, Bratislava, Slovakia, 10 Instituto de Salud Carlos III,
> Centro Nacional de Epidemiologia, Departamento de Epidemiologia
> Aplicada, Madrid, Spain, 11 Swiss National Reference Centre for Prion
> Diseases, University Hospital of Zurich, Zurich, Switzerland and 12
> National CJD Surveillance Unit, Western General Hospital, Edinburgh, UK
>
> Correspondence to: Professor R. G. Will, National Creutzfeldt–Jakob
> disease Surveillance Unit, Western General Hospital, Edinburgh EH4 2XU,
> UK E-mail: r.g.will@ed.ac.uk
>
> A collaborative study of human transmissible spongiform encephalopathies
> has been carried out from 1993 to 2000 and includes data from 10
> national registries, the majority in Western Europe. In this study, we
> present analyses of predictors of survival in sporadic (n = 2304),
> iatrogenic (n = 106) and variant Creutzfeldt–Jakob disease (n = 86) and
> in cases associated with mutations of the prion protein gene (n = 278),
> including Gerstmann–Sträussler–Scheinker syndrome (n = 24) and fatal
> familial insomnia (n = 41). Overall survival for each disease type was
> assessed by the Kaplan–Meier method and the multivariate analyses by the
> Cox proportional hazards model. In sporadic disease, longer survival was
> correlated with younger age at onset of illness, female gender, codon
> 129 heterozygosity, presence of CSF 14-3-3 protein and type 2a prion
> protein type. The ability to predict survival based on patient
> covariates is important for diagnosis and counselling, and the
> characterization of the survival distributions, in the absence of
> therapy, will be an important starting point for the assessment of
> potential therapeutic agents in the future.
>
> http://brain.oupjournals.org/cgi/content/abstract/127/10/2348?etoc
>
> TSS
>
> ################# BSE-L-subscribe-request@uni-karlsruhe.de
> #################
>

################# BSE-L-subscribe-request@uni-karlsruhe.de #################





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