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。

Entries from 2025-01-01 to 1 month

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…

Tromsø Aorta 2024 からの報告 〜医療体制〜

Tromsø Aorta 2024 Recap with Dr. Kim Eagle 11:18-12:58 One of the things that's very clear from presentations that we heard at Tromsø is that regions that have organized themselves to try to make diagnosis and rapid management of aortic di…

Tromsø Aorta 2024 からの報告 〜大動脈解離〜

Tromsø Aorta 2024 Recap with Dr. Kim Eagle 8:14-11:13 There was a lot of discussion about where we're at with diagnosis of this dangerous condition called aortic dissection when the aorta tears. The hallmark feature of an aortic dissection…

Q&A集(TMFの機関誌より)

Q1 マルファン症候群の特徴があります。どうしたらいいですか? Q2 検査のためには何科を受診すればいいですか? Q3 検査を受けなければなりませんか? Q4 今から診断を受けるのは遅すぎませんか? Q4 遺伝子検査は必要ですか? Q5 ロイス・ディーツ症候群で…

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…

小児におけるアテノロールとプロプラノロールの投与について

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…

マルファンやロイス・ディーツ患者による管楽器の演奏について

Applying AHA's 2024 Scientific Statement: Navigating Diagnosis & Management of Pediatric Aortopathy 39:20-41:15 Q: And then what about musical instruments? My middle school LDS1 child was just told no wind instruments. 楽器についてはどうで…

フェンシングについて

Applying AHA's 2024 Scientific Statement: Navigating Diagnosis & Management of Pediatric Aortopathy 37:25-38:56 Q: Fencing - I don't know that fencing is on your list there. How would that fit into the categories here? There's obviously pr…

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…

ハイリスクな運動とは?

Applying AHA's 2024 Scientific Statement: Navigating Diagnosis & Management of Pediatric Aortopathy 28:21-30:41 Q: Can you clarify what determines low versus high risk on the exercise table? ステートメントの表にあるハイリスク(ダークブルー…

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…