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。

Entries from 2022-03-01 to 1 month

上行大動脈と腹部大動脈の動脈瘤

Marfan Syndrome Management: Back to Basic 52:13-53:30 Dr. Malaisrie, are there protocols for aneurysms in the ascending aorta above the root. Is that different from aor, aneurysms at the root? Maisrie先生に質問です。大動脈基部の先にある上…

子どもの身長を予測する方法

Marfan Syndrome Management: Back to Basic 50:56-52:11 Dr. Morris, is there any way of knowing how tall a child will be by the time they finish growing? Morris先生に質問です。子どもの成長が止まるまでに身長がどのくらい伸びるか知る方法はあり…

大動脈基部手術から退院までの日数

Marfan Syndrome Management: Back to Basic 49:41-50:32 Over to Dr. Malaisrie, typically how long are you in the hospital for aortic surgery if it's prophylactic, you know, and then how long is the recovery after once you go home assuming th…

遺伝子検査の現在

Marfan Syndrome Management: Back to Basic 47:28-49:41 Dr. Morris, can you discuss the array of genetic testing available for Marfan-related conditions including... Morris先生、マルファン関連疾患の遺伝子検査について教えて下さい。 Sure and i…

平均寿命に到達するために必要なこと

Marfan Syndrome Management: Back to Basic 45:16-47:28 Can each of you talk about life, life expectancy, you know, whether somebody's diagnosed early, you know, what, what's the impact on life expectancy from aortic surgery? Question about …

経過観察中の運動と術後の運動について

Marfan Syndrome Management: Back to Basic 42:13-45:13 There are several questions about physical activity and Marfan syndrome and Loeys-Dietz, so Dr. Morris, can you just talk about that from, you know, from diagnosis and while you're moni…

マルファン症候群をはじめから ~大動脈基部手術のまとめ~

Marfan Syndrome Management: Back to Basic 39:47-40:28 So in conclusion this is what you need to know when you go see your cardiac surgeon. First I recommend to seek out an expert center for advice about timing of surgery. Not everyone need…

マルファン症候群をはじめから ~大動脈基部以外の心臓手術~

38:06-39:46 There's other considerations that your surgeon is going to talk to you about when thinking about any cardiac surgery for Marfan syndrome. The first is how much aorta to replace. If only the aortic roots is affected then the pat…

ロイス・ディーツ症候群とアレルギー

はじめに LDS患者に多いアレルギー症状 ・食物アレルギー ・皮膚炎 ・副鼻腔疾患 ・環境アレルギー ・喘息 ・中耳炎 アレルギーの治療法・管理法 ・食物アレルギー ・皮膚炎 ・副鼻腔疾患および季節性・環境アレルギー ・喘息 ・中耳炎 はじめに Loeys-Dietz…

マルファン症候群をはじめから ~ベントール手術とデイビット手術~

Marfan Syndrome Management: Back to Basic 34:43-38:04 Now we take all of these considerations into account in order to determine which operation is appropriate for you for the aortic root. では、大動脈基部手術の術式について説明します。 So …

マルファン症候群をはじめから ~画像検査~

Marfan Syndrome Management: Back to Basic 33:07-34:42 The most common tests that you'll be getting when you see us are three. 手術の前に受けていただく最も一般的な検査には3種類あります。 The first is the echocardiogram on the top and that i…

マルファン症候群をはじめから ~外科手術の目安となる動脈瘤の大きさ~

Marfan Syndrome Management: Back to Basic 30:21-32:32 Surgery is recommended when the aneurysm is measured at 5.0 centimeters and this is very well accepted guideline by both the Americans and the Europeans, the American College of Cardiol…

マルファン症候群をはじめから ~大動脈解離~

Marfan Syndrome Management: Back to Basic 28:09-30:19 The goal of surgery when you see us is to prevent aortic dissections. There's two times that you're going to meet a cardiac surgeon: one is electively in clinic and we can have small ta…

マルファン症候群をはじめから ~大動脈基部~

Marfan Syndrome Management: Back to Basic 26:12-28:05 The aorta is the largest vessel in the body. It accepts blood from the heart and distributes it to the rest of the body. On the left side is the normal aorta. It's spinning because this…

マルファン症候群をはじめから ~外科手術の準備~

Marfan Syndrome Management: Back to Basic 23:29-26:08 I've been asked to talk about what you need to know about cardiac surgery. My name is Chris Malaisrie. I'm a cardiac surgeon at Northwestern Memorial hospital, a professor of surgery at…

マルファン症候群をはじめから ~内科的治療のまとめ~

Marfan Syndrome Management: Back to Basic 20:24-21:00 So basically in summary, Marfan syndrome is caused by an FBN1 gene variant and causes primarily heart, eye, and skeletal problems. ではまとめです。Marfan症候群はFBN1遺伝子の変異によって…

大動脈手術を受けた子ども・ティーンからのアドバイス

はじめに - 手術を受けることになった時はどう思いましたか? - 家族やお医者さんにどのような質問をしましたか? - 友達に手術のことを伝えた時どのような反応でしたか? - 手術を受けたのは何歳の時ですか? - 手術について友達にどう説明しましたか? ☆ …

マルファン症候群をはじめから ~心臓以外の所見のフォロー・治療~

Marfan Syndrome Management: Back to Basic 19:14-20:23 And what about non-cardiac surveillance and treatment? 心臓以外の所見に関する検査と治療について説明します。 We recommend serial ophthalmology evaluations in children. It's every year. I…

マルファン症候群をはじめから ~画像検査・薬物治療・予防的大動脈置換~

Marfan Syndrome Management: Back to Basic 16:31-19:13 So what can we do to prevent bad outcomes in Marfan syndrome? How can we manage this, how can, how have we changed the life expectancy from 30 to near normal? では、Marfan症候群で最悪の…

マルファン症候群をはじめから ~大動脈瘤vs大動脈解離vs大動脈破裂~

Marfan Syndrome Management: Back to Basic 13:18-16:30 So I'm going to go a little more into the difference between an aneurysm, a rupture, and a dissection, so just looking at this. This is a diagram of the body so as I mentioned here's th…

マルファン症候群をはじめから ~心臓の所見~

Marfan Syndrome Management: Back to Basic 11:39-13:17 And then we'll get to the heart. This is my main area of expertise and interest and the two most common problems in Marfan syndrome are dilation of parts of the aorta and something call…

マルファン症候群をはじめから ~眼の所見~

Marfan Syndrome Management: Back to Basic 10:23-11:39 So what about the eyes in Marfan syndrome? So this is the second most affected besides skeleton and so the eye is the most common finding or the most, the most pathognomonic finding is …

マルファン症候群をはじめから ~骨格・皮膚の所見~

Marfan Syndrome Management: Back to Basic 9:35-10:22 So what are the systems that are affected? So a major component is skeleton and skin so people can have long arms, legs and fingers, tall and thin body type, curved spine, a chest that s…

マルファン症候群をはじめから ~細胞外マトリックスとTGF-β~

Marfan Syndrome Management: Back to Basic 8:16-9:35 So what, a little bit more about the gene defect. So I mentioned that the gene this changes is called FBN1 and that codes a protein called fibrillin 1 and this is a major component of som…

マルファン症候群をはじめから ~遺伝子検査~

Marfan Syndrome Management: Back to Basic 7:23-8:15 So what about genetic testing? So, you know, historically we, not everyone got genetic testing. You could just look at someone and say, "Well, I think they probably have Marfan syndrome,"…

マルファン症候群をはじめから ~遺伝子~

Marfan Syndrome Management: Back to Basic 5:41-7:22 So I'm just showing genetics over here just showing a gene or a, a chromosome. If you guys remember from school we all we have 46 of these chromosomes that sit here. There's 23 pairs of c…

マルファン症候群をはじめから ~有病率・男女比・寿命~

Marfan Syndrome Management: Back to Basic 4:23-5:40 Now I'm, I am a pediatric cardiologist. I'm mainly going to focus on general findings in Marfan syndrome but when we have Q&A I'm happy to answer anything and if there's specific pediatri…

大動脈手術後のリスク

Living with Risk 56:30-57:36 Several people are asking about risks after aortic surgery so Juan, do you want to talk about that from the aortic point of view? 大動脈手術後のリスクについてもいくつか質問が寄せられています。Juan先生、大動脈の…