以前、このブログでショパンの死因について検討したことがありました。

そこで、ショパンが「Cystic fibrosis」に罹患していたと紹介しました。それについて、最近興味深いニュースを見つけました。

Row over plan to DNA test Chopin’s heart
Scientists refused permission to analyse organ to prove composer died of cystic fibrosis, not TB

Robin McKie, science editor
The Observer, Sunday July 27 2008
Article history
Scientists are locked in a battle with the Polish government over their request to test the heart of Frédéric Chopin for evidence he suffered from cystic fibrosis. They believe the Polish composer was not a victim of tuberculosis, as commonly supposed, but died because he suffered from one of Europe’s most widespread hereditary disorders.

The researchers want to test his preserved heart to prove this point but have just been rebuffed by Polish authorities. However last week they pledged to continue to press for access to the composer’s remains to prove their theory.

‘This is a very important request,’ said Dr Michael Witt, of Warsaw’s Institute of Molecular and Cell Biology. ‘If we can prove Chopin suffered from cystic fibrosis, then we will have shown that a serious medical disability is still no barrier to achieving fame and success.’

Chopin died in France in 1849 and was buried in Paris though his heart was removed and placed in a jar of cognac, since kept in Warsaw’s Church of the Holy Cross. Now scientists want to use some of the tissue to test their theory.

The musician, born in 1810, is considered Poland’s greatest composer. However, his life was plagued by bad health. According to a Journal of Applied Genetics paper by Witt and fellow researchers Lucyna Majka and Joanna Gozdzik, Chopin was frail, slim, with sunken cheeks, suffered continual infections of the lung, and displayed symptoms of delayed puberty.

‘Chopin was a disabled person who depended on his friends’ care during the last four years of his life,’ they state. ‘He had to be carried up stairs and teach the piano in a lying position.’

After his 10-year relationship with the writer George Sand came to end in 1847, Chopin’s health deteriorated even further and he spent the last year of his life confined to bed, suffering from intense pains in his joints, depression and shortness of breath. He died in 1849, of ‘tuberculosis of the lungs and larynx’, according to his post mortem report.

But Witt’s team believe his symptoms were really those of cystic fibrosis, a genetic disorder that affects the lungs, pancreas, liver and intestines. The disease is inherited from symptomless parents. Each can pass on an affected gene to their children. Those who inherit a double dose develop the disease which manifests from early childhood. About one in 2,500 new-born children in Europe has cystic fibrosis. Current treatments can keep sufferers alive until they are over 40.

Witt points out that two of Chopin’s three sisters had similar complaints, including multiple organ failure, and died prematurely. These symptoms strongly suggest that the Chopin family was afflicted by cystic fibrosis, says the scientist. The theory would be impossible to prove if it were not for the fact that Chopin’s heart is pickled in cognac, offering the prospect of providing a few cells that could determine, from DNA tests, if he suffered from the condition. ‘Once we have that in our hands it is a small matter to do a DNA test,’ Tadeusz Dobosz, a geneticist at Wroclaw Medical University, told the journal Science

However, the Polish government said last week it would not give permission for such tests. Witt and his team insist that they will continue to press for access to Chopin’s heart.

They state in their paper: ‘Is it not right to make an attempt to prove to many suffering people that many things count in life much more than a weak physical body, and they are not predestined to vanish without leaving something that will influence, inspire and enrich generations to come?’

研究者達が、保存された心臓から、ショパンの遺伝子を調べたいと考えたものの、ポーランド政府から拒否されていたというのです（その他、診断根拠についての記載がありますが、私が以前とりあげたChopinというエントリーに書かれたものとほぼ同様です）。Cystic fibrosisは遺伝性疾患なので、遺伝子検査が極めて有用なツールとなります。

遺伝子検査をすれば診断がつくのではないかと思う一方で、診断が確定してしまえば、診断について議論を戦わせる病跡学のロマンの一つが失われそうな気もしてなかなか複雑な心境です。

個人的には、ショパンの診断「cystic fibrosis」はほぼ正しいのではないかとは思っています。ヨーロッパにはこの疾患は多く、マクロライド系抗生剤が何故か効くのではないかと考えられているのですが、実際にはあまり使用されてなさそうだということを methyl先生から聞いたことがあります。

ジェラゾワ・ヴォラのショパンの生家を訪れたことを思い出して、ちょっと感傷にひたりました。

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