Child tonsil tests set to predict vCJD fatalities
http://www.guardian.co.uk/bse/article/0,2763,1221710,00.html

James Meikle, health correspondent
Friday May 21, 2004
The Guardian

Tonsils removed from 100,000 patients, mostly children or teenagers, over the next three years will be tested in an attempt to gather better estimates of how many Britons may eventually succumb to the human form of BSE.

The latest best guess published today suggests the figure may be as high as 3,800, based on infected tissue samples stored in the late 1990s and the age group of those most at risk of developing the disease, 10 to 30-year-olds

This is far higher than might be suggested by the present British toll over nine years: 141 dead and five living patients with variant CJD, nearly all thought to have been infected with BSE-contaminated food from cattle, although one possibly developed the disease after a blood transfusion.

All the victims so far have had a genetic signature present in 40% of the population.

The figure is also far lower than some of the more grisly possibilities of up to 500,000 victims aired shortly after the link between the human and animal diseases was first made.

Since December, patients or parents have been asked permission to be included in a project run by the government's Health Protection Agency that may take three years and involve tissues from as many as four in five of all tonsil operations over that period.

It may be the last chance to gauge the size of the vCJD epidemic, since risk of exposure to BSE-infected meat should have ended with the reinforcement of controls in 1996, and, even allowing for long incubation periods, the number of young people exposed to risk should be falling rapidly. Measures have already been implemented to cut the risk of further infection through transfusions and contaminated instruments.

The tissues will be deliberately kept anonymous so any later finding of the abnormal prion protein linked to variant CJD cannot be reported back. The disease is still incurable, despite some apparently promising treatment options.

A study in the Journal of Pathology today, based on appendix and tonsil tissues removed during the late 1990s, floats the 3,800 figure, but its authors advise severe caution in interpreting, as the results found evidence of the abnormal prion in three appendix samples.

It has long been known that abnormal prions collect in the appendix well before clinical signs of vCJD occur. It is also assumed prions accumulate in tonsils at a similar early stage, but tonsils are far more rarely kept for subsequent research than appendixes, and far fewer were tested in the study reported today.

"It is important we clarify the significance of these findings by prospective screening of fresh tonsillectomy tissue," said David Hilton, from the department of histopathology at Derriford Hospital, Plymouth, one of the author's of the report.

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