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。

マルファン症候群における肺気腫・睡眠時無呼吸の研究 〜PPARγ経路とロサルタン〜

Investigation of Sleep Disorders and Lung Injury in Marfan Syndrome 9:36-13:34 Now several years ago when I was working in the Dietz’s lab we showed that the enlarged airspaces in the Marfan lung was caused by developmental perturbations o…

マルファン症候群の薬物管理 〜最後のまとめ〜

Medical Management of Marfan Syndrome 54:54-57:10 So let's go go back to the take-home messages. What I would like to to convey is that there is now a very substantial evidence base for good medical treatment for patients with Marfan syndr…

マルファン症候群の薬物管理 〜トリプタン系製剤〜

Medical Management of Marfan Syndrome 53:29-54:53 This is a bit more controversial perhaps. There are a group of medications called triptans. These are used for migraine and usually used as, as an acute treatment. They're not used for prev…

マルファン症候群の薬物管理 〜カルシウム拮抗薬〜

Medical Management of Marfan Syndrome 52:14-53:28 The, the next thing I want to bring to your attention is that there are medications called calcium channel blockers. These are medications like verapamil, diltiazem, things like that. They'…

マルファン症候群の薬物管理 〜フルオロキノロン系抗菌薬〜

Medical Management of Marfan Syndrome 48:55-52:09 I'm going to just talk about a couple of other issues now. There is and the, in particular I want to talk about the medications that we should not be giving and I'm sorry I'm running a bit …

マルファン症候群の薬物管理 〜ここまでのまとめ〜

Medical Management of Marfan Syndrome 48:17-48:54 So I'm now going to just skip forward and just draw some conclusions from the trials. So angiotensin receptor blockers reduce the rate of aortic growth in Marfan syndrome. The effects of AR…

マルファン症候群の薬物管理 〜ARBとβ遮断薬の大動脈イベントに対する効果について〜

Medical Management of Marfan Syndrome 44:21-48:07 I'm just going to go back and say that we were able to explore, you know, one of the questions was, you know, was there evidence that these medications reduced the rate of tearing of the ao…

マルファン症候群の薬物管理 〜ARBとβ遮断薬の比較〜

Medical Management of Marfan Syndrome 42:43-44:20 And then, we, then went on to look at trials where they had compared angiotensin receptor blockers with beta blockers. This is a different kind of trial. ARB versus beta blockers head-to-he…

マルファン症候群の薬物管理 〜ARBのメタアナリシス:β遮断薬とFBN1変異〜

Medical Management of Marfan Syndrome 40:34-42:42 I think one, there a couple of things that I think, are important that is I need to emphasize that when we looked specifically at beta blockers we found that patients with beta blockers had…

マルファン症候群の薬物管理 〜ARBのメタアナリシス〜

Medical Management of Marfan Syndrome 28:25-40:30 So a number of trials now were, were, were then undertaken and we then organized with the, with the investigators who, who did these trials. We put these trials all together to try to give …

マルファン症候群の薬物管理 〜アンギオテンシン受容体阻害薬(ARB)の説明〜

Medical Management of Marfan Syndrome 23:42-28:24 So I want to talk now about the two different classes of medications and we're going to dig into this in a lot of detail and people are asking really sensible questions in the Q&A and I'm g…

マルファン症候群における薬物治療(2022 ACC/AHAガイドライン)

Medical Therapy in Marfan Syndrome: Update from ACC/AHA Guidelines Hi, I'm Alan Braverman from Washington University in St Louis School of Medicine, Barnes Jewish Hospital. Today it's my pleasure to provide information on medical therapy i…

妊娠について(2022_ACC大動脈疾患ガイドライン) 〜薬物治療〜

ACC Aortic Disease Guidelines: Pregnancy 5:53-6:34 To end some words about medication during pregnancy, so it is recommended that women take beta blockers throughout pregnancy and as contra-indicated this should be based on a shared decisi…

大動脈疾患小児における薬物治療 〜大動脈拡張速度に関するシミュレーション〜

Medical Therapy in the Pediatric Patient 4:53-7:40 So in many cases families will say, "Well, if my aorta is not enlarged now or not substantially enlarge now then why do I need to take medication?" So when approaching this discussion with…

大動脈疾患小児における薬物治療 〜臨床試験の歴史〜

Medical Therapy in the Pediatric Patient 0:00-4:53 Hi, I'm Dr. Glenn Iannucci. I'm a pediatric cardiologist at Children's Healthcare of Atlanta and director of the aorta and vascular program and on behalf of the GenTAC alliance I'll be pre…

動脈瘤・解離の管理 ~抗血小板療法と抗凝固療法のランダム化比較試験(頚動脈解離・椎骨動脈解離)~

Vascular Surgery 14:42-15:07 This is important in terms of data. The question always comes up if we want to use aspirin or anti-platelet therapy versus anticoagulation that's coumadin in carotid artery dissections or vertebral artery disse…

吸入ステロイドについて

LDS Guidelines: Then vs. Now 1:16:18-1:17:26 Q:There are few questions about the use of inhalers and flovent and albuterol, which is kind of in your realm, too. Can you address that? フルタイドやサルブタモールなどの吸入ステロイドについて、…

ARBとβ遮断薬の両方を服用することで効果は高まるか?

LDS Guidelines: Then vs. Now 1:01:25-1:04:24 Q:One of the questions, I'll just kind of start picking some, is about you talked about the meta-analysis which was awesome and then you touched upon adding a beta blocker. Can you just let us k…

LDSガイドラインの改訂に向けて ~避けるべき薬~

LDS Guidelines: Then vs. Now 33:36-38:54 So there are other medications that we suggested avoiding back in 2014. 2014年のガイドラインでは、避けることが推奨されていた薬があります。 One was a class of medications commonly used to treat migra…

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 ~カルシウムチャネル遮断薬~

LDS Guidelines: Then vs. Now 29:04-31:03 So we, as we've talked about we were, we and other groups are trying to understand what medications might be good for someone with a vascular connective tissue disorder. A different question that I …

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 ~LDSマウスでのロサルタンによる動脈瘤予防メカニズム~

LDS Guidelines: Then vs. Now 19:26-21:34 We're now understanding exactly why medications like losartan work in these conditions. Again by work that was done by Elena McFarlane we've learned that cells within the aortic root, so-called seco…

LDSガイドラインの改訂に向けて ~マルファン症候群におけるロサルタンの効果~

LDS Guidelines: Then vs. Now 10:33-16:15 So back 10 years ago we knew that Marfan syndrome related to alterations in the activity of a molecule called TGF beta. That's a mechanism by which cells talk to themselves or talk to each other and…

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…

エピペン使用の判断基準・食物経口負荷試験について

Loeys-Dietz Gastroenterology and Allergy Q&A 38:57-40:39 Q:We've had a couple of questions. I see Julie, I know Lincoln has tree nut allergies, so asking about a young child a type 2, you know, when would you use an EpiPen, would you not u…

大動脈疾患の診断と管理に関する新ガイドライン(ACC/AHA)

2022年11月3日 THE MARFAN FOUNDATION 大動脈疾患の診断と管理のための新しいガイドラインが、昨日、Journal of the American College of Cardiology と Circulation に掲載された。この ガイドライン は、ACC(米国心臓病学会)とAHA(米国心臓協会)を代表…

機械弁でも出産は可能か?

Family Planning and Pregnancy: US and Europe Perspectives 1:05:26-1:07:51 Q: Do you have any women with Marfan who have also received a mechanical heart valve and are on blood thinners carrying a baby to term? You want to take that? Julie …

妊娠・出産について(アメリカ・ヨーロッパの専門医の視点から) ~ロイス・ディーツ症候群患者の場合~

49:01-50:02 So to conclude I would also make some, some discuss some aspects of pregnancy in patients with Loeys-Dietz syndrome although the data is much scarcer in this group and of course we know that the risk of aortic dissection during…

妊娠・出産について(アメリカ・ヨーロッパの専門医の視点から) ~妊娠中の服薬~

41:21-42:23 So one of the previous speaker already discussed the use of the prophetic use of beta blocks in pregnancy. Well, some studies actually observed a reduced rate of progression of aortic dilatation, so according to the European gu…