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 2020-09-01 to 1 month

遺伝カウンセラーの役割 ~症例① 5歳男児 マルファン症候群の疑い(2)~

The Role of the Genetic Counselor - Empowerment Series - 3/14/19 10:37-11:56 All right so now that I've finished out drawing out the family tree for this family, what do I usually do next? The very first thing I usually do is review the fa…

遺伝カウンセラーの役割 ~症例① 5歳男児 マルファン症候群の疑い(1)~

The Role of the Genetic Counselor - Empowerment Series - 3/14/19 8:00-10:35 All right. Now into a real case example. So the patient we have for case one is a five year-old male, who was referred to our clinic by a pediatrician. His doctor …

遺伝カウンセラーの役割 ~家系図~

The Role of the Genetic Counselor - Empowerment Series - 3/14/19 6:06-7:59 So now we'll work our way down that list of genetic counseling roles by running through some specific case examples that help illustrate exactly how genetic counsel…

遺伝カウンセラーの役割 ~遺伝カウンセラーの仕事~

The Role of the Genetic Counselor - Empowerment Series - 3/14/19 3:20-6:05 So first to give you a little background on how genetic counselors fit into the medical team, so we're, we're healthcare professionals with a specialized education …

遺伝カウンセラーの役割 ~はじめに~

The Role of the Genetic Counselor - Empowerment Series - 3/14/19 2:00-3:18 Hi, everyone and thank you for joining us this evening. So as Susan mentioned, I work with the cardiovascular genetics team at Texas Children's Hospital, which incl…

低侵襲心臓手術について

Children's Heart Issues in Marfan, Loeys Dietz, and vEDS - Virtual Symposium Series (4/9/19) 53:44-55:08 Q:It's a question about minimally invasive heart surgery to repair valves and she's wondering if it might be also possible to use mini…

MRI検査はどのくらいの頻度で受ければいいか?

Children's Heart Issues in Marfan, Loeys Dietz, and vEDS - Virtual Symposium Series (4/9/19) 51:56-53:42 Q:So what about the scheduling of MRI monitoring from head to pelvis, is different from Marfan, Loeys-Dietz, vEDS? MRI検査はどのくらい…

マルファン症候群に対する大動脈手術の進歩

2020年8月20日 Lars G. Svensson, MD, PhD はじめに コンポジットグラフト術による転帰改善 自己弁温存大動脈基部置換手術はremodeling法からre-implantation法へ 健康で長生きは可能 はじめに 私がマルファン症候群に興味を持ったきっかけは、かなり昔にな…

ロサルタンやβブロッカーを服用中の子どもでは、運動時の心拍数はどのくらいがいいか?

Children's Heart Issues in Marfan, Loeys Dietz, and vEDS - Virtual Symposium Series (4/9/19) 47:09-51:09 Q:Can you talk a little bit about a safe heart rate when the child is on losartan or I guess, even on a beta-blocker, you know, what's…

子どもが大動脈解離を発症する可能性はどのくらいか?

Children's Heart Issues in Marfan, Loeys Dietz, and vEDS - Virtual Symposium Series (4/9/19) 46:11-47:06 Q:Let me just ask you. You know, people are afraid of aortic dissection obviously. How common is that in children, that something peop…

マルファン症候群での肺疾患の割合と拘束性肺疾患の診断方法について

Children's Heart Issues in Marfan, Loeys Dietz, and vEDS - Virtual Symposium Series (4/9/19) 44:26-46:07 Q:This is not about the heart but close to the heart and cardiovascular system. Can you just talk briefly about lung problems in Marfa…

ロサルタンとドキシサイクリンの併用について

Children's Heart Issues in Marfan, Loeys Dietz, and vEDS - Virtual Symposium Series (4/9/19) 43:38-44:24 Q:What about combining losartan and doxycycline? Um, somebody says they've read that that can reduce the aortic aneurysm size. ロサル…

ARBのリコールについて

Children's Heart Issues in Marfan, Loeys Dietz, and vEDS - Virtual Symposium Series (4/9/19) 42:28-43:34 I mean, I forgot to mention you, you asked me to talk a little bit about the recall on losartan. So as many of you have heard or you k…

ロサルタンの服用を始めるZスコアはどのくらいがいいか?

Children's Heart Issues in Marfan, Loeys Dietz, and vEDS - Virtual Symposium Series (4/9/19) 40:53-42:27 Q:At what z-score would you advise starting losartan? And this particular person says that her son is three years old and the z-score …

マルファン症候群、ロイス・ディーツ症候群、血管型エーラス・ダンロス症候群小児の心疾患について ~フルオロキノロン系抗菌薬についての注意喚起~

Children's Heart Issues in Marfan, Loeys Dietz, and vEDS - Virtual Symposium Series (4/9/19) 37:28-39:00 And then my last thing will be about this FDA warning about fluoroquinolones that you may have heard about. 最後は、ご存じの方もいらっ…

マルファン症候群、ロイス・ディーツ症候群、血管型エーラス・ダンロス症候群小児の心疾患について ~運動についてのQ&A~

Children's Heart Issues in Marfan, Loeys Dietz, and vEDS - Virtual Symposium Series (4/9/19) 35:35-37:27 Some basic questions again, I can't I don't have time to give all of the evidence but I can basically answer some of these questions a…

マルファン症候群、ロイス・ディーツ症候群、血管型エーラス・ダンロス症候群小児の心疾患について ~運動について~

Children's Heart Issues in Marfan, Loeys Dietz, and vEDS - Virtual Symposium Series (4/9/19) 32:43-35:34 In terms of physical activity and exercise, there are many benefits to exercise. We all realize that and I think that exercise can be …

マルファン症候群、ロイス・ディーツ症候群、血管型エーラス・ダンロス症候群小児の心疾患について ~血管型エーラス・ダンロス症候群に対する薬物療法~

Children's Heart Issues in Marfan, Loeys Dietz, and vEDS - Virtual Symposium Series (4/9/19) 31:17-32:19j There's not very much information about medical therapy for patients with vascular EDS. This is a result of a study that was publishe…

マルファン症候群、ロイス・ディーツ症候群、血管型エーラス・ダンロス症候群小児の心疾患について ~βブロッカーとARBの比較試験~

Children's Heart Issues in Marfan, Loeys Dietz, and vEDS - Virtual Symposium Series (4/9/19) 27:24-31:16 And there was some evidence and again I'm coming to skip some of these slides but there's there was some evidence treating some kids w…

マルファン症候群、ロイス・ディーツ症候群、血管型エーラス・ダンロス症候群小児の心疾患について ~マウスモデルでのロサルタンの効果~

Children's Heart Issues in Marfan, Loeys Dietz, and vEDS - Virtual Symposium Series (4/9/19) 26:49-27:23 More recently, a mouse model was introduced showing that Losartan, the other kind of medicine, can rescue the aortic phenotype. 近年マ…

マルファン症候群、ロイス・ディーツ症候群、血管型エーラス・ダンロス症候群小児の心疾患について ~マルファン患者に対するβブロッカーの効果~

Children's Heart Issues in Marfan, Loeys Dietz, and vEDS - Virtual Symposium Series (4/9/19) 26:17-26:48 The big, probably, the biggest study looking at the efficacy of these medicines for people with connective tissue conditions was a tri…

マルファン症候群、ロイス・ディーツ症候群、血管型エーラス・ダンロス症候群小児の心疾患について ~βブロッカーとARB~

Children's Heart Issues in Marfan, Loeys Dietz, and vEDS - Virtual Symposium Series (4/9/19) 25:07-26:16 All right, what about medical therapy? So if there are two basic classes of medications that we use. 薬物療法で用いられる薬は基本的に2…

マルファン症候群、ロイス・ディーツ症候群、血管型エーラス・ダンロス症候群小児の心疾患について ~Zスコアについて~

Children's Heart Issues in Marfan, Loeys Dietz, and vEDS - Virtual Symposium Series (4/9/19) 20:28-25:06 Now, what about those aorta z-scores (ARz) scores? In adults with Marfan syndrome, Loeys-Dietz or Ehlers-Danlos syndrome, most of the …

マルファン症候群、ロイス・ディーツ症候群、血管型エーラス・ダンロス症候群小児の心疾患について ~心エコー・CT・MRI~

Children's Heart Issues in Marfan, Loeys Dietz, and vEDS - Virtual Symposium Series (4/9/19) 16:28-20:28 Let's move on now to imaging techniques. How do we measure the aorta or evaluates a function of valves and the function of the muscle …

マルファン症候群、ロイス・ディーツ症候群、血管型エーラス・ダンロス症候群小児の心疾患について ~共通の心疾患(2)~

Children's Heart Issues in Marfan, Loeys Dietz, and vEDS - Virtual Symposium Series (4/9/19) 14:38-16:27 We already mentioned arterial tortuosity and here again are the pictures of the vertebral arteries in patients with Loeys-Dietz. We di…