Lung Issues in Marfan Syndrome
6:07-8:18
So let's start with restrictive lung disease. About 50% or more of patients with Marfan syndrome have some degree of restriction and this ranges from relatively mild restriction, which is not something that's of clinical consequence, to a pretty significant restriction and this is usually attributable to musculoskeletal abnormalities of the chest.
では拘束性肺疾患についての説明を始めます。マルファン患者さんの半数以上にある程度の拘束性障害を認めます。比較的軽度で、臨床的な症状がみられないものから、かなり重度で、通常は胸部の筋骨格異常に起因するものまであります。
And effectively what happens is that a person is unable to expand the chest fully, so your lung capacity is limited. So what are the symptoms? The symptoms are typically shortness of breath with exertion or activity and this can actually progress to shortness of breath at rest and with further progression, this can lead to low oxygen levels in the blood or cyanosis and even heart strain.
実際には、十分に胸を膨らませることができていないということですが、これは肺容量が低下しているということを意味します。症状としては作業時の息切れが一般的です。症状が進行すれば、安静時でも息切れするようになり、さらには、血中酸素濃度の低下（チアノーゼ）から心臓への負荷が大きくなることもあります。
On this slide, I depict kind of the basic tests that pulmonologist will obtain for persons who have possible lung issues and that is spirometry and pulmonary function testing and this typically occurs in a pulmonary function lab where a person is asked to take a big breath in and breathe out as hard as they can and in this way, we're able to measure the volume of air that you're able to breathe in and out and also the rapidity with which you're able to breathe in and out.
このスライドでは、肺疾患が疑われる方に呼吸器科医が行う基本的な検査を説明します。この検査はスパイロメトリーと呼ばれている肺機能試験で、一般的に肺機能検査室で行います。力いっぱい息を吸って、吐き切ってもらい、呼吸可能な空気の量とそのときの呼吸速度を計測します。
Now in order to diagnose restriction, we look at a particular tracing that we generate from these studies and these are called flow volume loops and on the left we see the flow volume loop of a normal person and you can look on the left side of this and this is a point at which someone is asked to take a deep breath in and then blow it out as fast as they can. Now this measurement reflects your lung capacity or your vital capacity and what I want you to notice is that in a person with restrictive physiology which we see on the right that lung capacity is significantly reduced and that is how we make the diagnosis of restrictive lung disease.
肺機能の診断のため、これらの検査結果から得られた、フローボリューム曲線（FVL）を確認します。左側は正常な方のFVLです。この図の左側の点（0の点）で深呼吸して、思いっきり吐き出すのがこの点（4の点）です。この測定値（Volume の線）が肺活量を示します。注目していただきたいのは、右の図で示されている、拘束性障害が認められる方では肺活量がかなり低下しているということです。このようにして我々は、拘束性肺疾患の診断を行っています。
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。
