What You and Your Healthcare Professionals Should Know About Marfan Syndrome - Webinar
45:18-47:40
Now we're going to move on to lung concerns. So what we're most concerned about is spontaneous pneumothorax. Pneumothorax is the medical term for lung collapse. Now, anybody can have a pneumothorax, usually associated with trauma. People with Marfan Syndrome have a risk for spontaneous pneumothorax. In other words, the lung collapses just out of the blue for no good reason. And this is a schematic of what it looks like.
では、肺の問題に移ります。最も懸念すべきは、自然気胸です。気胸とは、肺が潰れた状態を示す医療用語です。誰でも気胸を起こす可能性があり、通常は外傷によって起こります。マルファン症候群の患者さんは、自然気胸の発症リスクがあります。つまり、特に理由もなく突然発症する可能性があるということです。
So this is the normal lung on the left, and here on the right, you can see that there's been collapse. The symptoms are sudden onset of chest pain. So this is another thing to consider in addition to the dissection pain. Pain is frequently worse with taking a deep breath in, and frequently there will be shortness of breath. This is another emergency. You need to get to the emergency room, and there the diagnosis can be made actually by somebody listening over the chest and not hearing any breath sounds there, or by a chest X-ray. And you can see here's all of this lung tissue that's collapsed, and it's right next to the heart. This usually needs to be treated by insertion of a chest tube, which basically sucks out the air and re-expands the lung, which you can see over here. Sometimes the pneumothorax will recur, and it's necessary to actually do a procedure to make it so that the lung is attached to the lining. This is a procedure known as a pleurodesis.
こちらが肺の図です。左側の肺（向かって右）は正常ですが、右側の肺（向かって左）はしぼんでいるのがわかります。気胸の症状は、突然発症する胸の痛みです。胸痛の原因としては、大動脈解離以外にも気胸の可能性もありますので覚えておいてください。気胸による痛みは多くの場合、深呼吸によって悪化し息切れが多く見られます。気胸は緊急事態ですので救急外来へ急いでください。胸の音を聴いても呼吸音がしない、あるいは胸部レントゲンによって気胸の診断が付けられます。こちらのレントゲン画像では、気胸を起こした肺の組織が心臓の真横に見えます。通常、この状況では、胸腔ドレーンを挿入して空気を吸い出し、肺を膨らませる治療が行われます。気胸は再発することもありますので、（再発を防ぐために）肺を胸壁の裏打ち部分にくっつくける処置を行う必要があります。この処置は「胸膜癒着術」として知られています。
Another lung concern is what we call restrictive lung disease. What that means is that the lung expansion is restricted, and a person can't fully inflate the lungs. So this is again that Pectus Excavatum that I showed you. This is a CAT scan showing the Pectus Excavatum, and you can see that the heart has been displaced into the left side of the chest, and also some of the lung tissue is being compressed. So this is what's known as restrictive lung disease. That's another concern in people with Marfan syndrome.
肺に関するもう一つの問題は、拘束性肺疾患と呼ばれるものです。肺の拡張が制限され、十分に膨まない状態を指します。ここでもう一度、漏斗胸の画像をお見せします。右側がCT画像ですが、心臓が胸の左側に押しやられ、肺組織も一部が圧迫されているのがわかります。これが拘束性肺疾患として知られる状態であり、マルファン症候群で懸念されるもう一つの問題です。
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。
