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。

Tromsø Aorta 2024 からの報告 ~vEDSにおける薬物管理~

Tromsø Aorta 2024 Recap with Dr. Kim Eagle 13:03-15:02 One of the really interesting talks that we heard in Tromsø was a trial that is looking at the management of patients with vascular EDS. This was a small study, I think less than 60 pa…

ARBとβブロッカーの併用について

Applying AHA's 2024 Scientific Statement: Navigating Diagnosis & Management of Pediatric Aortopathy 52:50-54:51 Q: In the statement, you mentioned dual therapy if tolerated. What does "if tolerated" mean? What are indicators to change medi…

アテノロールとプロプラノロールについて

Applying AHA's 2024 Scientific Statement: Navigating Diagnosis & Management of Pediatric Aortopathy 51:52-52:44 Q: Is atenolol the preferred treatment for children four and under? 4歳以下の小児については、アテノロールのほうが好ましいですか…

ロサルタンの服用開始時期について

Applying AHA's 2024 Scientific Statement: Navigating Diagnosis & Management of Pediatric Aortopathy 43:21-43:53 Q: What age should Losartan be started or is it just based on z-score? ロサルタンは何歳から飲み始めればいいでしょうか?それとも…

小児における大動脈疾患の診断と管理に関するステートメント 〜薬物治療〜

Applying AHA's 2024 Scientific Statement: Navigating Diagnosis & Management of Pediatric Aortopathy 13:02-15:02 And so for all of these slides, what we do is we basically provide a framework for how to manage patients, and this framework n…

ステロイドについて

Navigating GI Management in LDS 49:38-51:39 Q: You mentioned that steroids could be harmful for bones. Do the benefits outweigh risks if using steroids to treat asthma or EoE? ステロイドを服用すると骨への悪影響の可能性があるというお話があ…

NSAIDのオススメはあるか

Navigating GI Management in LDS 41:49-42:46 Q: Do you have a recommended NSAID that we could keep on hand for general use and if there's a recommendation for pediatrics versus adult patients? 日常的にそばに置いておくNSAIDのお勧めはあります…

NSAIDの長期服用について

Navigating GI Management in LDS 39:40-41:46 Q: Doctors are quick to recommend NSAIDs for different problems related to inflammation that is seen in people with connective tissue conditions, people with LDS. However, we understand that chro…

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…