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October 16, 2013

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One in 2,000 in UK has mad cow disease protein

Around one person in 2,000 in Britain carries the protein linked to the human version of mad cow disease, according to a study published yesterday.

But how many people will actually develop the crippling and ultimately fatal disease is unknown, its authors say.

They also warn Britain to fight the risk of wider contamination through blood transfusions and surgical instruments.

The study, published online by the British Medical Journal, is the most exhaustive attempt yet to quantify the risk to Britons from the variant form of Creutzfeldt-Jakob disease.

This is a brain-destroying disorder whose suspected cause lies with eating beef contaminated by mad cow disease, a bovine illness that erupted in Britain in the late 1980s and spread to other countries through cattle exports. Culls to curb mad cow disease went into higher gear in the 1990s.

But experts have struggled to calculate the risk for people exposed to the rogue prion protein, called abnormal PrP.

The problem is that little is known about the incubation time for Creutzfeldt-Jakob, although it is likely to be long, and not everyone who carries the prion may develop the disease itself.

Researchers led by Sebastian Brandner, a professor of neuropathology at University College London, studied 32,441 appendixes that were removed at 41 British hospitals, and tested them for PrP. Sixteen samples were positive, a figure that, when extrapolated across the United Kingdom’s population, equates to around one in 2,000.

“Our study detected the presence of abnormal prions in the population,” Brandner said.

“However, it is not possible to predict how many will ever develop the disease. Should anyone develop a disease, it may present differently from Creutzfeldt-Jakob.”

An official count by British watchdogs gives a relatively tiny number of Creutzfeldt-Jakob cases, just 177 compared to the millions likely to have been exposed to abnormal PrP.

The positive samples came from people of all ages and geographical background.

The study suggests a broad range of Britons may carry the prion, on the basis of a genetic profile of the appendix samples, and warns the authorities to maintain their guard.




 

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