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。

循環器

動脈瘤・解離の管理 ~動脈全体の画像検査~

Vascular Surgery 8:50-9:42 I do follow people with vascular surveillance. You heard about that from Dr. Young who talked about the surveillance for the aortic root. Similarly for the rest of the vasculature I like to get a baseline CT scan…

動脈瘤・解離の管理 ~腹部大動脈瘤・内臓動脈瘤の修復サイズ~

Vascular Surgery 7:06-8:49 So aortic aneurysms, this is specifically for abdominal aortic aneurysms, not, not ascending you've heard a lot about the ascending and root aneurysm thresholds but this is for abdominal aortic aneurysms and you …

動脈瘤・解離の管理 ~治療法~

Vascular Surgery 5:24-7:04 So in terms of repair principles we do know that most aneurysms and dissections can be managed non-operatively and I think this is a very important concept to make because when people hear that they have a dissec…

動脈瘤・解離の管理 ~vEDS/LDS/MFSでの動脈疾患分布~

動脈瘤・解離の管理Vascular Surgery 3:29-5:23 You've seen this picture. This is from Gustavo Oderich's paper where a few years ago he talked about the distribution of arteriopathy in vascular EDS and then on the right hand side is our paper…

動脈瘤・解離の管理 ~解離とは~

Vascular Surgery 1:55-3:27 Dissection on the other hand is different. Dissection means that there's a tear in the wall of the artery or the aorta and blood then flows between those layers and what that does is then as you can see in the pi…

動脈瘤・解離の管理 ~動脈瘤とは~

Vascular Surgery 0:18-1:52 Basically my goal for the audience here is to learn the difference between aneurysms and dissections because frequently people can confuse the two. I will show you the distribution of the arterial pathology in va…

POTS(体位性頻脈症候群)について

LDS Guidelines: Then vs. Now 1:11:03-1:13:52 Q:Any correlation or conversation about POTS in Loeys-Dietz syndrome? POTSとLDSは関連がありますか? A:Yeah, so POTS stands for Postural Orthostatic Tachycardia Syndrome. Basically what it's desc…

太陽が昇るまで ― A型解離からの生還

2023年9月23日 The Marfan Foundation 「人生が一変したあの瞬間…」 2020年7月31日。僕の人生が一瞬で変わってしまった日。早めの夕食を済ませて帰宅すると、左腕が突然感覚を失った。家はひんやりとしていたのに、汗が吹き出してくる。左腕の感覚はない。妹…

血管内アプローチのリスクについて

LDS Guidelines: Then vs. Now 1:08:02-1:09:32 Q:Is there any precautions with arterial line insertion for anesthesia and the risk of arterial dissection in Loeys-Dietz patients, so maybe also like catheter procedures. LDSでは、血管に管を入…

大動脈が正常かつLDS遺伝子変異が認められる患者の薬物治療

LDS Guidelines: Then vs. Now 1:04:25-1:07:52 Q:Kind of along the lines of medication in that first question or that first slide we were talking about non-penetrance. Can you talk a little bit just about your thought process when you're cou…

LDSガイドラインの改訂に向けて ~LDSマウスに対するカルシウムチャネル遮断薬の影響~

LDS Guidelines: Then vs. Now 31:08-33:34 So we have now directly tested this in mouse models of Loeys-Dietz syndrome. I'm here using a calcium channel blocker, a common one called amlodipine. Importantly, in these studies it didn't matter …

LDSガイドラインの改訂に向けて ~心血管系の管理~

LDS Guidelines: Then vs. Now 27:01-28:55 In terms of cardiovascular management, what we would say and continue to say is that people should be getting at least yearly echocardiograms. Clearly we're doing that a little bit more frequently i…

LDSガイドラインの改訂に向けて ~画像検査~

LDS Guidelines: Then vs. Now 21:49-24:13 In terms of vascular imaging guidelines these have generally remained the same. In the very beginning we are recommending head to pelvis imaging at baseline as well as imaging about every two years.…

LDSガイドラインの改訂に向けて ~大動脈手術~

LDS Guidelines: Then vs. Now 7:54-10:15 So one of the big things that differentiated Loeys-Dietz syndrome from Marfan syndrome in the very beginning was this recognition, right? that aortic diameters could show dissections or tears at smal…

マルファン症候群における大動脈解離の発症率について

New ACC/AHA Aortic Disease Management Guidelines: What do they mean for you? 1:05:09-1:05:47 Q:What percentage of Marfan patients will experience in aortic dissection or require surgery? マルファン患者で大動脈解離を発症する人の割合、外科手…

大動脈疾患の診断と管理に関するACC/AHAガイドライン2022 ~大動脈弓部の動脈瘤に関する推奨事項~

New ACC/AHA Aortic Disease Management Guidelines: What do they mean for you? 53:22-54:33 And and then finally some patients will have an arch, aortic arch aneurysm and they may have symptoms that are attributed to the aneurysm. If, as long…

大動脈疾患の診断と管理に関するACC/AHAガイドライン2022 ~急性A型解離に対する推奨クラス2Aの推奨事項~

New ACC/AHA Aortic Disease Management Guidelines: What do they mean for you? 50:55-51:51 And finally, the, there's class 2A recommendation when it comes to type A aortic dissections and this, this did come from a lot of discussion and that…

大動脈疾患の診断と管理に関するACC/AHAガイドライン2022 ~A型解離と腸間膜潅流障害に関する論文紹介~

New ACC/AHA Aortic Disease Management Guidelines: What do they mean for you? 49:22-50:51 Some of the reasons why this became a class one recommendation in, in terms of malperfusion, this is paper that came out of the University of Michigan…

大動脈疾患の診断と管理に関するACC/AHAガイドライン2022 ~A型解離に関する推奨事項~

New ACC/AHA Aortic Disease Management Guidelines: What do they mean for you? 46:22-49:18 For the class, I'm going to go through this fairly quickly. These are the class one recommendations for type A aortic dissections and I've included th…

大動脈疾患の診断と管理に関するACC/AHAガイドライン2022 ~妊娠・出産について~

New ACC/AHA Aortic Disease Management Guidelines: What do they mean for you? 40:54-46:17 Okay moving on to pregnancy. As Maya commented earlier that the emphasis on shared decision making and the importance of counseling, genetic counselin…

大動脈疾患の診断と管理に関するACC/AHAガイドライン2022 ~血管型エーラス・ダンロス症候群に関する推奨事項~

New ACC/AHA Aortic Disease Management Guidelines: What do they mean for you? 38:08-40:48 Moving on to vascular Ehlers-Danlos syndrome in, and in vEDS. In imaging the aorta and the branches is very important because it may identify arterial…

大動脈疾患の診断と管理に関するACC/AHAガイドライン2022 ~ロイス・ディーツ症候群に関する推奨事項~

30:17-36:57 This, this slide just emphasizes a little bit of difference in management and Loeys-Dietz syndrome, so in Loeys-Dietz syndrome imaging because aneurysm disease is more widespread than in Marfan syndrome that baseline MR or CT a…

大動脈疾患の診断と管理に関するACC/AHAガイドライン2022 ~大動脈基部手術の基準値(マルファン症候群)~

New ACC/AHA Aortic Disease Management Guidelines: What do they mean for you? 27:36-30:06 Dr. DeAnda is going to walk us through surgical thresholds in Marfan syndrome aortic root enlargement. では、DeAnda先生からマルファン症候群の大動脈基…

大動脈疾患の診断と管理に関するACC/AHAガイドライン2022 ~薬物治療~

New ACC/AHA Aortic Disease Management Guidelines: What do they mean for you? 25:57-27:33 Okay, what's new in Marfan syndrome? Well, moving on to medical therapy. Those on the, on the webinar tonight with Marfan syndrome they're probably we…

大動脈疾患の診断と管理に関するACC/AHAガイドライン2022 ~予防的外科手術の目安~

New ACC/AHA Aortic Disease Management Guidelines: What do they mean for you? 20:53-25:55 Dr. DeAnda is going to talk about some of the surgical thresholds. では、DeAnda先生から外科手術のしきい値についてお話いただきます。 Some of this has c…

大動脈疾患の診断と管理に関するACC/AHAガイドライン2022 ~遺伝学的推奨事項~

New ACC/AHA Aortic Disease Management Guidelines: What do they mean for you? 17:08-20:51 The recommendations are written like this, so I won't go over every single one but it emphasizes again in somebody, in a patient who has thoracic aort…

大動脈疾患の診断と管理に関するACC/AHAガイドライン2022 ~遺伝子検査~

New ACC/AHA Aortic Disease Management Guidelines: What do they mean for you? 14:07-17:03 So what we're going to do over the next half hour or so is we're going to review some of the topics that are encompassed in the new guidelines based o…

大動脈疾患の診断と管理に関するACC/AHAガイドライン2022 ~共同での意思決定に関する医師の見解~

New ACC/AHA Aortic Disease Management Guidelines: What do they mean for you? 10:57-14:08 So this is not a new concept but I think what's so important about the document is how all these various numbers and sizes and things that we talk abo…

大動脈疾患の診断と管理に関するACC/AHAガイドライン2022 ~患者と医師による共同の意思決定~

New ACC/AHA Aortic Disease Management Guidelines: What do they mean for you? 9:03-10:48 Maya Brown Zimmerman is going to talk to us about one of the most important aspects of the guidelines, the concept of shared decision making. では、ガ…

大動脈疾患の診断と管理に関するACC/AHAガイドライン2022 ~大動脈径の計測方法~

New ACC/AHA Aortic Disease Management Guidelines: What do they mean for you? 7:10-9:00 One of the most important parts of the guidelines is really a whole section on emphasizing how to measure the aorta. Everyone who sees individuals and, …