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。

小児

小児における大動脈弓部の問題について

Applying AHA's 2024 Scientific Statement: Navigating Diagnosis & Management of Pediatric Aortopathy 58:51-1:01:05 Q: How often do you see complications in the aortic arch? 大動脈弓部の合併症はどのくらいの頻度でみられますか? A: I can start…

子どもへの病気の告知について

Applying AHA's 2024 Scientific Statement: Navigating Diagnosis & Management of Pediatric Aortopathy 54:55-58:37 Q: Here's a question that touches on the ethics of disclosing diagnosis. If an adult has genetically confirmed heritable thorac…

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…

ステートメント付録の画像検査の表について

Applying AHA's 2024 Scientific Statement: Navigating Diagnosis & Management of Pediatric Aortopathy 48:09-51:48 Q: Does the statement include specifics on baseline imaging when newly diagnosed? ステートメントには、診断が付いてから最初に行…

小児の大動脈解離発症リスクについて

Applying AHA's 2024 Scientific Statement: Navigating Diagnosis & Management of Pediatric Aortopathy 44:00-48:06 Q: For the shared decision making, it would be helpful to know, uh, to better, to know, excuse me, to know better estimates of …

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…

遊園地のアトラクションについて

Applying AHA's 2024 Scientific Statement: Navigating Diagnosis & Management of Pediatric Aortopathy 35:49-37:23 Let's talk about amusement parks. What is doable there? 遊園地で乗ってもいいアトラクションは何ですか? A: You want to start, Ro…

スキー(ダウンヒル)について

Applying AHA's 2024 Scientific Statement: Navigating Diagnosis & Management of Pediatric Aortopathy 33:50-35:47 Q: There are parts of the world where skiing is a way of life. What are your thoughts around counseling families with aortopath…

就学前および小学校における運動についてのアドバイス

Applying AHA's 2024 Scientific Statement: Navigating Diagnosis & Management of Pediatric Aortopathy 30:46-33:47 Q: Any tips for discussing the exercise at the preschool age and also with a child but also with the school? What recommendatio…

早期発症型マルファン症候群(Early-onset Marfan syndrome)とは?

Applying AHA's 2024 Scientific Statement: Navigating Diagnosis & Management of Pediatric Aortopathy 25:30-28:18 Q: What would be considered “Early-onset Marfan syndrome”? 早期発症型マルファン症候群(eoMFS:Early-onset Marfan syndrome)と考…

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 〜まとめ〜

Applying AHA's 2024 Scientific Statement: Navigating Diagnosis & Management of Pediatric Aortopathy 20:59-24:18 So in summary, this statement is the first attempt at really putting everything about management of pediatric aortopathy in one…

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 〜付録〜

Applying AHA's 2024 Scientific Statement: Navigating Diagnosis & Management of Pediatric Aortopathy 18:30-20:57 And these are the different sections that are included in the paper. So you might imagine that the sections on Marfan syndrome,…

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 〜予防的外科手術〜

Applying AHA's 2024 Scientific Statement: Navigating Diagnosis & Management of Pediatric Aortopathy 17:38-18:26 And then there's a table on surgery, when to do surgery. Again, the lighter blue is standard risk. If there are high-risk featu…

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 〜運動〜

Applying AHA's 2024 Scientific Statement: Navigating Diagnosis & Management of Pediatric Aortopathy 15:05-17:34 Now this is the table, as Dr. Morris mentioned earlier, we wanted to approach exercise in a little bit different way than it ha…

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…

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 〜遺伝子検査〜

Applying AHA's 2024 Scientific Statement: Navigating Diagnosis & Management of Pediatric Aortopathy 11:34-12:59 Good evening everyone. So there is a section in the statement outlining how to do a genetic evaluation and when to do genetic t…

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 〜画像検査の表の見方〜

Applying AHA's 2024 Scientific Statement: Navigating Diagnosis & Management of Pediatric Aortopathy 9:30-11:30 So just to give some highlights, this is big picture taking a step back of the Imaging table here. We'll do a zoom up, but the I…

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 〜トピックについて〜

Applying AHA's 2024 Scientific Statement: Navigating Diagnosis & Management of Pediatric Aortopathy 8:20-9:29 So this is the main outline of the statement. The first part of the statement is evaluation of the child with suspected aortopath…

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 〜ステートメントについて〜

Applying AHA's 2024 Scientific Statement: Navigating Diagnosis & Management of Pediatric Aortopathy 5:03-8:18 So first of all, why did we put this together? Well, we know that aortopathy, which is a word that encompasses various conditions…

小児の睡眠時無呼吸を確認する方法について              

Investigation of Sleep Disorders and Lung Injury in Marfan Syndrome 41:44-43:05 Q: For parents and guardians how can they tell if their children have sleep apnea? 子どもが睡眠時無呼吸かどうかわかる方法はありますか? A: Typically for kids t…

学校の保健の先生に知ってもらいたいこと

1. はじめに マルファン症候群とは マルファン症候群の特徴 マルファン症候群の原因 マルファン症候群の発症率 マルファン症候群の診断 マルファン症候群患者の人生 マルファン症候群において急を要する疾患 マルファン症候群の類縁疾患 2. 検査が必要な生徒…

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…

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 …