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The Baron's complaint

DOI: 10.1186/2041-2223-2-18

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Abstract:

There's fondness for these elements of 1980s life, cheesy though they may have been. The politicians of the time tended to be much more polarising. Two such figures, one deceased and one retired, made the news again recently. On US Independence Day, a new statue was unveiled outside the American Embassy in London. Ten feet tall, and cast in a chocolaty bronze, Ronald Reagan now smiles benignly down on passersby. One week previously, an iconic handbag belonging to Margaret Thatcher was sold for £25,000 at a charity auction.Apart from being a mutual admiration society, these old transatlantic allies were united by a physical condition: They both suffered from something called Dupuytren's contracture. 'Whose what?', you are probably asking yourself. The overbearing and authoritarian Baron Guillaume Dupuytren was a French physician, and Napoleon Bonaparte's doctor. He described this thickening in the palm of the hand, and the progressive and permanent contraction of the fingers [1], in a lecture in 1831, and gave his name to the condition despite not being the first to report it. The contracture severely limits hand function; it can be corrected by surgery, but may recur, and can eventually require amputations.Dupuytren's disease is more prevalent among men than women, and appears rather late in life: its average age of onset is 60 years. For a geneticist, the interest comes from its reported heritability: it possesses an Online Mendelian Inheritance in Man database number [OMIM:126900] and is described as a dominant trait with incomplete penetrance as well as the commonest heritable disorder of connective tissue. One linkage study in a five-generation Swedish pedigree [2] fits this pattern and provides evidence for a locus on chromosome 16q. For the historian, however, the condition is intriguing because of its alleged population distribution and origins: It is widely regarded as a 'Viking disease'.The condition was always known to be relatively common in northern Euro

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