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。

加齢とマルファン関連疾患 ~症例紹介~


Aging with Marfan and Related Conditions (VIrtual Medical Symposium Series, 5/6/19)

 

資料:https://info.marfan.org/hubfs/Marfan%20Syndrome%20and%20Aging.pdf

 

4:53-6:20

 

We're going to talk about aging a little bit in general and then, then in aging with regard to Marfan syndrome and related conditions.

一般的なお話を少ししてから、マルファン症候群に関連する疾患と加齢との関係についてのお話に移ります。

 

(Can everybody hear me? Okay? You can hear me all right? Okay? So you're good, okay, good. Now let me just see if I can advance my slides, okay.)

(司会者とのやり取り)

 

I'm going to start off with a case. So this is one of our patients who's 59 and she's been known to have Marfan syndrome for a long time.

まず59歳マルファン症候群女性の症例を紹介します。我々が診察している患者さんですが、かなり前にマルファン症候群であるということが分かりました。

 

When she was 53, she did have an operation and had the valve sparing root repair done so that took care of an immediate problem of the enlarging aorta and this is an image of her aorta. So you can, you know, hardly tell, but it's been repaired and so that's out of the way.

53歳で自己弁温存手術を行い、急を要する大動脈拡張を治療しました。これが彼女の大動脈の画像です。ほとんどわからないと思いますが、修復されており問題は見当たりません。

 

However, now at age 59 there's been a new problem where she's developed some unsteadiness in her gait and she came and had a pretty comprehensive evaluation with imaging and neurology consultation and a EMG test of muscles and nerves, and basically several conditions were found that; she has lumbar dural ectasia, which I'll show you an image, as many people with Marfan syndrome already have; she has a degenerative arthritis in her hips; and she also had a peripheral neuropathy. It wasn't completely clear which of these were responsible for her problem but probably all three were.

ですが59歳の時に歩行時のふらつきという新たな問題が出てきました。全身の画像検査を行い、神経内科を受診して筋肉と神経の筋電図検査を受けてもらった結果、幾つかの問題が見つかりました。まず腰部の硬膜拡張です。これについては後で画像をお見せしますが、多くのマルファン患者さんでみられる所見です。それから臀部の変形性関節炎、そして末梢神経障害です。このうちのどれがふらつきの原因かははっきりとは分かりませんが、おそらくすべてが原因であると考えられます。

 

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。