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From: TSS ()
Subject: Re: Kuru in the 21st century—an acquired human prion disease
Date: June 25, 2006 at 7:19 am PST

In Reply to: Re: Kuru in the 21st century—an acquired human prion disease posted by TSS on June 23, 2006 at 6:53 am:

Human mad cow infection could hide for 50 years

Jennifer Cooke
June 24, 2006


INFECTION with the lethal prions that cause the human form of "mad cow" disease could last more than 50 years before symptoms appear, research based on an old cannibal disease from Papua New Guinea shows.

During that time, victims with silent infection could pass on the prions via blood transfusions, organ or tissue donations or insufficiently sterilised metal surgical instruments.

In a unique piece of research which continues the meticulous records that have tracked the epidemic of kuru - the "shivering" disease - since 1957, Australian, British and PNG researchers tracked a group of 11 former cannibals dying from the always fatal prion disease between 1996 and 2004.

Forming the tail-end of the epidemic, they were all born before 1950 and took part in endocannibalism, a ritual in which the whole body of deceased relatives was consumed as a sign of love and respect. The ritual was eradicated by Australian authorities by 1960.

The data gathered on these victims and published today in The Lancet have provided a model using actual cases instead of the usual mathematical modelling for future predictions for those infected after eating beef products contaminated with bovine spongiform encephalopathy (BSE). The human equivalent of BSE is variant Creutzfeldt-Jakob disease (vCJD).

Apart from the emerging epidemic of vCJD, in which 192 people have died since 1995 - mainly in Britain, but also in France, Ireland, Canada, the US, Saudi Arabia, Japan, Italy, Hong Kong and the Netherlands - kuru is the only other major human epidemic of prion disease with an oral transmission route.

British prion experts, including Professor John Collinge from University College London, and Professor Michael Alpers from Curtin University - the Australian kuru expert who has followed the disease since arriving in PNG in 1962 - calculated the minimum incubation period for kuru starting at 1960 to the birth year of the last recorded patient.

That minimum incubation time ranged from 34 to 41 years but in men it was calculated to be between 39 and 56 years - and possibly up to seven years longer.

For vCJD it might be even longer, because the infection was transmitted between species, from cows to humans, which usually takes longer than infection within the same species.

Australian prion experts have closely monitored the decade-old vCJD epidemic, and have long recognised from continuing kuru cases that incubation periods could top 50 years.

A professor of pathology and a CJD expert at the University of Melbourne, Colin Masters, commented yesterday: "These new data reinforce our need to maintain vigilance over potential risks to the safety of Australia's blood supply, since it has now been demonstrated that vCJD can be transmitted by blood transfusions."

In an accompanying editorial, The Lancet stated: "Any belief that vCJD incidence has peaked and that we are through the worst of this sinister disease must now be treated with extreme scepticism."

http://www.smh.com.au/news/world/human-mad-cow-infection-could-hide-for-50-years/2006/06/23/1150845378945.html


TSS



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