Lung Issues in Marfan Syndrome
8:46-10:09
And we will start with this. So another term for respiratory muscle weakness is the neuromuscular consequences of Marfan syndrome. We know that persons with Marfan syndrome have skeletal muscle weakness and skeletal muscles involve your limbs, involve your arms, but they also involve your chest muscles that are involved in every breath that you take and these weaken respiratory muscles can lead to reduced lung capacity and restriction as we saw before.
このスライドでは呼吸筋の筋力低下を神経筋疾患として見ていきます。マルファン患者さんでは、骨格筋の筋力低下が認められます。骨格筋とは四肢や腕などですが、胸の筋肉も含まれます。この筋肉は呼吸に関係しており、この筋力が低下すると肺の機能が低下し、先程見たような拘束肺疾患へとつながります。
So how do we make the diagnosis well? We make it using those same pulmonary function tests that I talked about, but I also add these two extra tests and they're called maximal inspiratory and maximal expertory pressures and these are diagnostic of respiratory muscle dysfunction and in all patients that I evaluate with Marfan syndrome and shortness of breath, I obtain these studies.
では診断はどうすればいいのかというと、先程と同じ肺機能試験を行うのですが、さらに2つの検査を追加します。これらは最大呼気・吸気圧測定と呼ばれており、呼吸筋の機能不全の診断に用います。マルファン症候群や息切れのある患者さん全てに、私はこれらの検査を行います。
So you may ask well, "What are the treatments if you have respiratory muscle weakness?" Well, the critical intervention is pulmonary rehabilitation, which we'll talk about a little later, but if the respiratory muscle weakness is very severe, we may even have to consider ventilation support, which we'll also talk about a little later.
呼吸筋の機能が低下している場合の治療ですが、極めて重要なのは、肺リハビリテーションです。また、かなり重篤な場合には、人工呼吸管理が必要になります。これらについては後でお話します。
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。
