Cause of Ronald Reagan's and Margaret Thatcher's hand disease

Main Category: Genetics
Article Date: 19 Nov 2005 - 0:00 PDT

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A crippling condition that can result in sufferers losing their fingers is to be investigated by scientists in one of the most detailed studies into the genetic causes of the disease ever carried out.

Dupuytren's disease or contracture, a condition that affects the hands and sometimes the feet and penis, occurs gradually, beginning with a small, sometimes tender lump in the palm.

Over time, tough bands of tissue or cords can form that force the fingers, most commonly the small and ring fingers, to curl towards the palm.

The only treatment currently available to sufferers, who include former British Prime Minister Lady Thatcher and the late US President Ronald Reagan, is surgical removal of the excess tissue growth, which provides some respite from the onset of the disease.

But scientists at The University of Manchester want to look at the genetic influences behind the disease in the hope that a cure can be developed.

"Some of the characteristics of the condition are very peculiar," said Dr Ardeshir Bayat, the academic surgeon within the University's Centre for Integrated Genomic Medical Research who is leading the research.

"It mainly affects people of north European or Scandinavian descent and runs in families, so we know there is a genetic link involved.

"We have already identified a couple of genes that contribute to the disease but we want to extend that research to look at the entire human gene map or genome."

Named after Baron Dupuytren, the 19th Century French surgeon who first described it, the disease can affect both sexes but is most prevalent amongst men over the age of 40.

In the UK and the US, epidemiological studies have suggested that Dupuytren's disease can affect between five and 15% of men over the age of 50.

"We're interested in finding the cause of the disease by understanding the way it develops, its pathogenesis; only then will we be able to look for a potential cure," said Dr Bayat.

"At present, cutting out the diseased tissue is the only option but even then you are not eradicating the disease, it still comes back.

"It's not a lethal condition but its effects can still be devastating, leading to severe loss of hand function and, in extreme cases, amputation of the affected digits becomes necessary.

"The problem, like with other disabling diseases that are not life-threatening, is a lack of research funding; only through public and corporate donations, often by people who have been affected by the disease in some way, can we continue our work."

Aeron Haworth
aeron.haworth@manchester.ac.uk
University of Manchester
http://www.manchester.ac.uk

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