How are the lungs affected in Marfan syndrome?
You know, the way I like to kind of discuss them are from the, the features that are the most common to the features, that are a little less common and a little more exotic.
最もよく見られる所見、あまり一般的でない所見、珍しい所見についてお話したいと思います。
And so the most common kind of respiratory manifestation of Marfan syndrome involves a chest wall and most patients with Marfan syndrome are very familiar with kind of the chest wall abnormalities, which include like pectus abnormalities; pectus excavatum where you have the sunken in chest, or carinatum where the chest actually is very prominent, and scoliosis which is curvature of the spine.
マルファン症候群で最もよく見られる呼吸器の所見は胸壁に現れます。マルファン患者のほとんどが胸壁の異常を認めます。例えば、胸が陥没する漏斗胸、胸が突出する鳩胸、そして背骨が湾曲する側弯症などです。
And lastly you can have a respiratory muscle, kind of this function and respiratory muscle weakness and that's because the skeletal muscles in patients with Marfan syndrome are decidedly weaker than the skeletal muscles and patients who don't have the syndrome.
さらに、呼吸筋に関する所見があります。マルファン患者の骨格筋は健常者と比べると確実に脆弱であるため、呼吸筋の機能も低下しています。
And so the respiratory muscle weakness and some of these chest wall abnormalities serve to reduce the respiratory capacity of the lungs and so what happens is, what we call, a restrictive defect and so the chest is largely restricted and that you can't take as deep a breath as you need in order to bring in the oxygen but also to get rid of carbon dioxide which the body normally makes.
呼吸筋の脆弱性や胸壁の異常により、肺の呼吸機能が低下し、いわゆる拘束性障害が起きます。胸部が大きな制限を受け、必要な酸素の取り込みや不要な二酸化炭素の排出ができるだけの深い呼吸ができなくなるのです。
Now well, one of the things that I notice and, you know, I focus on with parents especially, is an awareness of when kind of an abnormality develops in the context of kind of a child's normal developmental life. Now those of us who are lung specialists, we
think about the longest forming from before birth up until about the age of five or six years of age and so any type of disturbance in the thoracic cage or, what we call, the chest cage will actually translate into abnormal formation of the lung and so any child
with Marfan syndrome who has scoliosis or a pectus abnormality before that time is actually definitely going to have some sequelae in terms of lung function.
私がご両親に特にお伝えしたいのが、子供の成長過程のどの時期に症状が進行するかということですが、我々肺の専門医は、最長形成期である出生前から5、6歳までだと考えています。（この時期に発症する）胸郭のいかなる異常も、実際肺の異常形成に繋がります。ですから、この時期以前に、側弯症や胸部の異常の認められるマルファン症候群の子供は、肺機能に関して後に症状が出てくることは間違いありません。
The Marfan Foundation did not participate in the translation of these materials and does not in any way endorse them. If you are interested in this topic, please refer to our website, Marfan.org, for materials approved by our Professional Advisory Board.
The Marfan Foundationは、当翻訳には関与しておらず、翻訳内容に関してはいかなる承認も行っておりません。このトピックに興味をお持ちの方は、Marfan.orgにアクセスし、当協会の専門家から成る諮問委員会が承認した内容をご参照ください。
