海外マルファン情報

米国マルファン症候群患者団体The Marfan Foundationからの情報を中心に、マルファン症候群や関連疾患についての海外情報を翻訳して発信します。

マルファン症候群の薬物管理 〜トピックの紹介〜

 
5:35-10:18
 
So I've been asked to talk about the, thankfully, the area that I know most about, which is what, what I think of is the medical treatment of Marfan syndrome. What, what do I mean by medical treatment, well that really is the, the medications as opposed to the other aspects of treatment, which I, I think are extremely important, which I think of as the lifestyle measures and also surgical treatment. We could talk a little bit about that later if you want to but they are important, so I'm going to come on to that in a second.
 
本日は、私の専門であるマルファン症候群における内科的治療についてお話しさせていただきます。内科的治療とは、薬物治療を指します。薬物治療以外にも、生活習慣の改善や外科手術など、極めて重要な選択肢があります。これらについては後ほどお話しします。では始めたいと思います。
 
So I'm going to tell you at the beginning what I want you to take away as the main messages and then I'm going to, you know, go on to talk in more detail about it and at the end I'm going to tell you what the main messages are again, so just I want to, I'm going to tell you three time basically.
 
まず、覚えておいていただきたい項目を紹介します。これらについては後ほど詳しく説明しますが、このウェビナーでは3回ほど繰り返すことになると思います。
 
So the first message is that the lifestyle measures for patients with Maran syndrome are important and if you're a patient with Marfan syndrome you can control these to a very large extent, so I don't want anything that I say today to detract from that very important message looking after yourself, looking after your body, looking after your aorta is a really important part of Marfan syndrome and you are the main person who's in control of those and you can get lots of good advice from your care providers and, and if you're not getting good advice then you, you need to ask them for it and get help with, with it.
 
最初のメッセージは、マルファン症候群の患者さんでは、生活習慣の改善が重要であるということです。生活習慣については、大部分が患者さん自身でコントロールできます。自身の体調や大動脈に気を配るということが大切になりますが、これらは患者さん自身が主体となって取り組む必要があります。生活習慣については、医療者から多くの有益なアドバイスを受けることができますので、必要な場合は質問してみてください。
 
The second thing is that, you know, I think this is a really optimistic message to come out of the meta analysis and the, and the, the studies that everyone has worked so hard on is that there are now two proven to my mind and I'm going to show you the, the reason why I think it's the case and explain the reason that I, I used that word proven, classes of medications that have been shown to reduce the rate of aortic enlargement in Marfan syndrome.
 
二つ目は、メタアナリシスから得られた希望の持てる内容です。研究者らが懸命に取り組んだことで、二種類の薬がマルファン症候群において大動脈拡張を遅らせることが示されました。後ほど理由を説明します。
 
And that these medications or classes of medication are effective when used alone and you can choose either one or you can choose both and I think they are probably most effective when they're used in combination and if you use both medications you probably get an additive effect and the, the two drugs work together to, to give you double the benefits and so I think that's a pretty positive message and if I had, you know, Maran syndrome I would want to be well, I would want to give serious consideration to taking both of these classes of medication together. That's what I'd want to do now. If there was some reason why I couldn't take one or the other then that's okay. I would just take the one that I could take but, but I would want to be thinking about taking both.
 
これらの薬はどちらか一方のみでも効果がありますが、両方服用することもできます。おそらく、二種類飲んでいただいたほうが、最も効果が発揮されるのではないかと考えています。付加的効果が得られ、2倍の恩恵が得られると思いますので、非常に前向きな内容です。もし私自身がマルファン症候群だとしたら、両方の薬を飲むことを真剣に考えます。理由があって片方しか飲めないということなら仕方ありませんが、そうであったとしても両方飲むことを考えたいと思います。
 
They're also generally very well tolerated medications. Most people can take one or other of the medications and many patients can take both.
 
これらの薬は一般に忍容性が非常に高い薬です。ほとんどの患者さんがどちらか一方は服用できますし、どちらも服用できる患者さんも大勢おられます。
 
There are also some medications that should be avoided. Now most of you are not going to ever need these medications but I think it would be nice for you to know what they are in case the circumstances arose where a physician might want to put you on these medications and you could say, "I think I'll pass on that one," or "do you have an alternative?"
 
避けるべき薬もいくつか知られています。こうした薬が必要とならない方が大半ですが、こうした薬を処方される場面も考えられますので、どういった薬なのかを知っておいたほうがいいと思います。処方された場合には、その薬は飲めませんと言って断ったり、代わりの薬がないかどうかを確認してください。
 
And of course there is the option of surgery to the aorta, the main blood vessel if that ever grows too big and that's a really good treatment option and surgery on the aorta, especially the part of the aorta that is most vulnerable in Marfan syndrome. We'll talk about that, is now an effective and generally low risk option but it's not risk-free and that's why we want to look at things like lifestyle measures and medications to try and delay the need for surgery or in some patients perhaps even remove the need for surgery.
 
当然ですが、手術による治療もあります。大動脈が太くなりすぎた場合には、妥当な選択肢となります。特に、マルファン症候群で最も影響を受けやすい大動脈の部位については手術が有効で、一般にリスクも低くなっています。影響を受けやすい大動脈の部位については後ほど説明しますが、リスクが低いと言ってもゼロというわけではありませんので、生活習慣の改善や薬で手術を遅らせたり、患者さんによってはこうした手術が不要となることもあります。
 
So we want to use all the tool, tools in our toolbox and if we, if, you know, it's my belief that if we use all three treatment options: lifestyle, medications and surgery, you know, we can get the best outcomes for patients. That's the goal, so those, those are the main options. I'm going to repeat all this at the as we go through the talk and, and elaborate on these things and at the end, I'm going to come back and summarize it all again.
 
まとめると、このスライドの内容は全て使うということになります。生活習慣の改善、薬、手術を全て用いることで、最善の結果が得られると信じています。それぞれの内容は、ウェビナーを進めながら詳しく説明し、最後にもう一度まとめたいと思います。
 

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。