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Scottish Researcher Warns That vCJD Remains a Threat
 

James Ironside, Professor of Clinical Neuropathology at the University of Edinburgh, warns that variant Creutzfeldt-Jakob disease (vCJD) could be more widespread than has been commonly supposed. vCJD is the human form of mad cow disease, a fatal brain disorder. Ironside made this statement during a presentation he gave at the Prion2007 conference at the Edinburgh International Conference Centre in Scotland on September 25, 2007. 

The vast majority of vCJD cases, which are predominantly linked to eating meat contaminated with bovine spongiform encephalopathy (BSE), have occurred in the United Kingdom (UK). Of the 166 confirmed cases of vCJD in the UK, approximately 161 people have died from definite or probable CJD infection.

Ironside, who specializes in research on brain tumors and the pathology of human prion diseases such as vCJD, is also a head of the University’s Neuropathology Laboratories in the National CJD Surveillance Unit. He asserts that vCJD could potentially be lying dormant in thousands of people who could be asymptomatic carriers. Since no vCJD rapid test currently exists, Ironside believes the disease has the potential to become endemic.

Ironside pointed to possible infection routes such as BSE-tainted blood donations from unwitting carriers and use of contaminated surgical instruments. There have been four reported cases of vCJD transmission via blood transfusions in the UK. To date, there have been no known cases of vCJD transmission through the use of plasma-derived factor concentrates used to treat hemophilia.

Although the Scottish National Blood Transfusion Service has implemented targeted donor deferrals and blood filtering to reduce possible risk--already considered low by some--Ironside and others stress that a rapid test is needed to prevent more vCJD infections.

“We are closer than ever before to actually implementing new screening technologies to prevent further secondary infection of these diseases. The latest published research that I am aware of clearly demonstrates this,” said Graham Steel, co-founder of the CJD Alliance and the information resource manager of the CJD International Support Alliance. “The time has almost come for screening to become a reality.”  The CJD Alliance is a UK-based organization dedicated to researching new diagnostic tools and treatments for CJD, publishing its results, and offering support to families and individuals with CJD.    

In the U.S. there has been no reported case of vCJD transmission through the blood supply. However, tests are being developed to detect vCJD and vCJD infections in blood and plasma donors. A risk assessment performed by the U.S. Food and Drug Administration (FDA) stated that the threat of vCJD in plasma-derived factor products is exceedingly low but possibly not zero. Since 1999, the (FDA) has enforced donor deferral policies among all blood donations from people who lived in or visited the UK for three months or longer between 1980 and 1996.

Source: The Scotsman and BBC News (online), September 24, 2007 

 

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