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From: TSS (216-119-139-80.ipset19.wt.net)
Subject: Creutzfeldt-Jakob disease in Slovenia from 1985 to 2003 with pronounced increase between January 2001 and December 2003
Date: October 11, 2004 at 1:45 pm PST

-------- Original Message --------
Subject: Creutzfeldt-Jakob disease in Slovenia from 1985 to 2003 with pronounced increase between January 2001 and December 2003
Date: Mon, 11 Oct 2004 15:36:16 -0500
From: "Terry S. Singeltary Sr."
Reply-To: Bovine Spongiform Encephalopathy
To: BSE-L@UNI-KARLSRUHE.DE


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

1: Wien Klin Wochenschr. 2004 Aug 31;116(15-16):524-9.


Creutzfeldt-Jakob disease in Slovenia from 1985 to 2003.

Popovic M, Glavac D, Smerkolj S, Svigelj V, Kalan G, Galvani V, Cernilec
M, Bresjanac M.

Institute of Pathology, Medical Faculty, University of Ljubljana,
Slovenia. mara.popovic@mf.uni-lj.si

AIM: The epidemic of bovine spongiform encephalopathy and subsequent
emergence of a new variant of Creutzfeldt-Jakob disease have raised
great public concern, initiating improved and prospective surveillance
of human prion diseases in Europe and all over the world. This report
briefly presents the epidemiology, clinical data, neuropathology,
immunohistochemistry, biochemistry, and prion-protein gene analysis of
Slovenian cases of Creutzfeldt-Jakob disease from January 1985 to the
end of 2003. MATERIALS, METHODS AND RESULTS: During the 19-year period,
39 suspected cases of Creutzfeldt-Jakob disease were referred and 22
were confirmed. The prion-protein gene was analyzed in 12 of the
confirmed cases and the protein glycosylation pattern in 11. There was a
low average incidence of Creutzfeldt-Jakob disease (0.5/million)
throughout the surveillance period, but a pronounced increase between
January 2001 and December 2003 (to 1.9/million/year). A high female to
male ratio (2.5/1) was noted. All of the confirmed cases were defined as
sporadic Creutzfeldt-Jakob disease based on the clinical data,
neuropathological findings, glycosylation pattern, and gene analysis.
All tested cases had a type-2 glycosylation pattern; eleven of the
twelve tested patients were homozygous at codon 129 of the prion-protein
gene (1 VV and 10 MM) and one was heterozygous. CONCLUSION: The small
number of Slovenian cases of sporadic Creutzfeldt-Jakob disease during
the last 19 years has shown a pronounced increase in incidence,
reflecting improved surveillance, and a high female to male ratio, where
female cases are more than twice as numerous as male cases.

http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=15471179TSS

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