What pulmonary tests do you recommend for Marfan patients?
One of the things I actively encouraged and it almost becomes like a, like a broken record with me is I think anyone with Marfan syndrome should get a good
set of pulmonary function tests and this is for a number of reasons.
私が積極的かつ何度も繰り返し推奨しているのは、マルファン患者は適切な肺機能検査を受けるべきということです。理由はいくつかあります。
First of all, many of the organ systems that are affected in Marfan syndrome have a feature of progressive deterioration and that's one of the kind of unfortunate aspects of Marfan syndrome.
まず、残念ではありますが、マルファン症候群では多くの器官が影響を受け、次第に悪化していくという特徴があります。
Since we're unable to kind of rebuild a tissue at this point, in time, hopefully, at some point, we will, we need to kind of deal with the fact that there is going to be this accelerated course where the tissues actually become more and more vulnerable.
こうなると組織の修復はできないので、組織がますます弱まっていくという事実をどこかの時点で受けて止められるようにしておく必要があります。
In the lung, it becomes very important because many interventions are based on the determination of abnormal lung function and using kind of preventive strategies to try to avoid or delay the development of very, very severe kind of respiratory dysfunction or shortness of breath, or disability.
肺の場合、検査は非常に重要になります。なぜなら、多くの介入は異常な肺機能を特定することで行われ、非常に重篤な肺の機能不全、息切れ、肺障害の進行を避けたり、遅らせるための予防戦略を用いるためです。
And so what I suggest is that we get pulmonary function tests and these are typically tests that tell us what the capacity of someone is. Someone has to have to take a deep breath in and to blow that breath out efficiently and those studies are usually able to be conducted in children over the age of about five.
ですから、私は肺機能検査を受けることを推奨してます。この検査では、通常、肺容量を調べます。患者さんは、深呼吸した後、息をすべて吐き切ります。5歳以上であれば、たいていこの検査を受けられます。
Now, there are certain ancillary studies that go along with pulmonary function tests that aren't necessarily conducted all the time and I encourage them to be conducted to Marfan patients and these are studies of respiratory muscle strength and they include what we call maximal expiratory pressure and maximal inspiratory pressure and what they'll do is they'll tell us how of the respiratory or the breathing limitations are caused by just generalized muscle weakness
肺機能検査に付随して行われる検査もありますが、必ずしも行われるという訳ではありません。マルファン患者さんには受けることをおすすめしています。付随検査は、呼吸筋の筋力を測定する検査で、最大呼気圧と最大吸気圧を計測します。全体的な筋肉の弱体化により、どれほど呼吸機能が障害されているかを調べます。
And lastly I asked that they have, we call, a sniff fluoroscopy that sounds like a weird term, but it's basically a way of looking at the diaphragm which is a respiratory muscle and determining whether the diaphragm is actually moving appropriately and one way that we can do that is by having people sniff because the diaphragm presumably should move down when you sniff and then when the sniff is over, it'll come back up.
最後になりますが、sniff fluoroscopyと呼ばれている検査があります。呼吸筋の一種である横隔膜を調べ、動きに問題が無いことを確認します。患者さんに鼻から空気を吸い込んでもらいます。その際横隔膜は下がります。吸い込みをやめると横隔膜は元に戻ります。
And so these battery of studies can give us kind of a narrative of how the lungs are performing when they have very various tests of their function and so that's why I think they're very useful.
これらの検査を組み合わせて行うことで、肺機能を調べることができます。ですから、肺の検査は非常に重要になるのです。
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。
