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

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…

マルファン症候群の薬物管理 〜循環器系の説明〜

Medical Management of Marfan Syndrome 10:18-15:56 So what, what issue am I trying to resolve now? Marfan syndrome obviously is a multisystem disorder. It affects the eyes, it affects the spine, it affects the muscular-skeletal system. In f…

兄がつなぐ命

2024年2月9日 カレン・ブルックス 謎の診断 未知との戦い 喪失と学び 調査と希望 意義ある目的 私は生まれた時にマルファン症候群と診断されました。突然変異による発症だった父から、4人きょうだいのうちの2人がマルファンを受け継ぎました。今年の夏で82歳…

大動脈基部および上行大動脈の散在性大動脈瘤手術

Surgery for Sporadic Aortic Aneurysms of the Aortic Root and Ascending Aorta On behalf of the GenTAC alliance I'll be speaking on surgery for sporadic aneurysms of the aortic root and ascending aorta. My name is Chris Malaisrie. I'm a card…

ロイス・ディーツ症候群における大動脈基部および上行大動脈置換術に対する手術閾値

Surgical Thresholds for Root and Ascending Aortic Replacement in Loeys- Dietz Syndrome Hi, I'm Alan Braverman. I'm the director of the Marfan syndrome and aortopathy center at Washington University in St Louis school of medicine and on beh…

ロイス・ディーツ症候群における大動脈基部および上行大動脈置換術に対する手術閾値

Surgical Thresholds for Root and Ascending Aortic Replacement in Loeys- Dietz Syndrome Hi, I'm Alan Braverman. I'm the director of the Marfan syndrome and aortopathy center at Washington University in St Louis school of medicine and on beh…

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…

分娩について(2022_AHA/ACC大動脈疾患ガイドライン)

AHA/ACC Aortic Disease Guidelines: Delivery 0:00-3:43 Hello, my name is Mary Shepard from the University of Kentucky and it is my pleasure to be here today to talk with you about delivery and people who have aortic disease. ケンタッキー大…

妊娠について(2022_ACC大動脈疾患ガイドライン) 〜心血管疾患カウンセリング〜

ACC Aortic Disease Guidelines: Pregnancy 4:02-5:52 Regarding cardiovascular counseling so as I mentioned this is important for, the for, the ladies, it, they should be informed that the risk for aortic dissection increases during pregnancy…

遺伝性胸部大動脈疾患小児向け運動ガイドライン 〜ガイドライン案〜

Pediatric Activity Guidelines - HTAAD 5:01-8:43 So in general when we are restricting individuals from activity that is to help them to avoid an aortic event or perhaps disease progression we know aortic dissection is a very rare event. In…

遺伝性胸部大動脈疾患小児向け運動ガイドライン 〜運動ガイドラインの紹介〜

Pediatric Activity Guidelines - HTAAD 1:30-5:00 Why should we be active? Originally the goal was survival so, you know, several decades ago really the main goal was trying to get people to be able to live long because the mortality rates w…

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…

小児循環器の観点で見る2022 ACC/AHA大動脈疾患ガイドライン 〜遺伝子検査の頻度〜

Pediatric Take on 2022 Adult Guidelines for Diagnosis and Treatment of Aortic Disease 7:22-8:20 Another point that was highlighted in the 2022 guidelines was that we should try to remember the family, so aortic disease is a progressive con…

小児循環器の観点で見る2022 ACC/AHA大動脈疾患ガイドライン 〜大動脈手術をどこで受けるか〜

Pediatric Take on 2022 Adult Guidelines for Diagnosis and Treatment of Aortic Disease 5:50-7:22 One of the other points that was highlighted in the 2022 guidelines were the recommendation that patients with stable aortic disease be referre…

小児循環器の観点で見る2022 ACC/AHA大動脈疾患ガイドライン 〜大動脈手術のしきい値に関する注意〜

Pediatric Take on 2022 Adult Guidelines for Diagnosis and Treatment of Aortic Disease 5:10-5:49 Because some degree of growth of the aorta is normal for children we must use caution when reviewing the 2022 AHA guidelines that point out tha…

小児循環器の観点で見る2022 ACC/AHA大動脈疾患ガイドライン 〜大動脈の拡張速度〜

Pediatric Take on 2022 Adult Guidelines for Diagnosis and Treatment of Aortic Disease 3:36-5:03 So as children are growing one of the questions that often comes up is "Is my aortic growth physiologic or pathologic?" and by physiologic grow…

小児循環器の観点で見る2022 ACC/AHA大動脈疾患ガイドライン 〜小児の成長曲線〜

Pediatric Take on 2022 Adult Guidelines for Diagnosis and Treatment of Aortic Disease 2:27-3:35 Children grow, is something we know well looking at the growth chart for infants. So on the pink growth chart, there you can see, there's very …

小児循環器の観点で見る2022 ACC/AHA大動脈疾患ガイドライン 〜小児科における大動脈疾患の診断〜

Pediatric Take on 2022 Adult Guidelines for Diagnosis and Treatment of Aortic Disease 0:00-2:25 Hi, I'm Dr. Glenn Iannucci. I'm a Pediatric Cardiologist at Children's Healthcare of Atlanta with Emory University and I run our aorta and Vasc…

LDSでの大腿動脈からのカテーテル挿入について

Vascular Surgery 19:52-21:09 Q: Does Loeys-Dietz change if you go through the femoral artery or open embolization due to more fragile tissue? LDSでは組織が脆弱ということなので、大腿動脈からコイル塞栓術を行う場合、処置に違いはあるのでしょう…

動脈瘤・解離の管理 ~総腸骨動脈瘤・外腸骨動脈解離~

Vascular Surgery 17:35-18:51 Common iliac artery aneurysms you heard I've said it's if we're going to see it in, in people with Marfan syndrome we will see frequently common iliac artery aneurysms and then again very frequently we'll see t…