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。

Q & A

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…

運動に関するアドバイス

LDS Guidelines: Then vs. Now 1:21:13-1:24:12 Q:Last question for you, Dr Dietz. In terms of exercising what are your suggestions in terms of kind of like heart rate recommendations or kind of what guidelines say and how you think or feel a…

骨と関節について

LDS Guidelines: Then vs. Now 1:17:49-1:20:45 Q:If anyone has any thoughts on just osteoporosis or joint degradation we might see in Loeys-Dietz syndrome and any type of supplement in terms of calcium or things like that? LDSで見られる骨粗…

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、…

LDSでは自己免疫疾患を起こしやすいか?

LDS Guidelines: Then vs. Now 1:13:55-1:16:15 Q:Any evidence of prevalence of autoimmune conditions with Loeys-Dietz syndrome like rheumatoid arthritis or other immunity, I guess, autoimmune conditions? LDSでは、関節リウマチのような自己免疫…

POTS(体位性頻脈症候群)について

LDS Guidelines: Then vs. Now 1:11:03-1:13:52 Q:Any correlation or conversation about POTS in Loeys-Dietz syndrome? POTSとLDSは関連がありますか? A:Yeah, so POTS stands for Postural Orthostatic Tachycardia Syndrome. Basically what it's desc…

LDSにおける神経障害について

LDS Guidelines: Then vs. Now 1:09:38-1:11:01 Q:Any thoughts on neuropathy in Loeys-Dietz syndrome and/or, you know, how they impact pain management? LDSでの神経障害について教えてください。痛みの管理法に影響はあるのでしょうか? A:Yeah, so I…

血管内アプローチのリスクについて

LDS Guidelines: Then vs. Now 1:08:02-1:09:32 Q:Is there any precautions with arterial line insertion for anesthesia and the risk of arterial dissection in Loeys-Dietz patients, so maybe also like catheter procedures. LDSでは、血管に管を入…

大動脈が正常かつLDS遺伝子変異が認められる患者の薬物治療

LDS Guidelines: Then vs. Now 1:04:25-1:07:52 Q:Kind of along the lines of medication in that first question or that first slide we were talking about non-penetrance. Can you talk a little bit just about your thought process when you're cou…

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…

PEARS(Personalised External Aortic Root Support)について

New ACC/AHA Aortic Disease Management Guidelines: What do they mean for you? 1:05:49-1:07:41 Q:There's a lot of questions about PEARS surgery. Abe, are you comfortable talking about the PEARS? PEARSについての質問を多くいただいています。DeA…

マルファン症候群における大動脈解離の発症率について

New ACC/AHA Aortic Disease Management Guidelines: What do they mean for you? 1:05:09-1:05:47 Q:What percentage of Marfan patients will experience in aortic dissection or require surgery? マルファン患者で大動脈解離を発症する人の割合、外科手…

子どもが遺伝子の突然変異により発症した場合、両親の画像検査の必要はあるか?

New ACC/AHA Aortic Disease Management Guidelines: What do they mean for you? 1:04:10-1:05:06 Q:Here's a question my daughter was diagnosed with Loeys-Dietz but dad and I were tested and don't have it. Should we get an echo to check the aor…

内蔵動脈瘤の管理について

New ACC/AHA Aortic Disease Management Guidelines: What do they mean for you? 1:02:52-1:04:10 Q:Abe, maybe you can answer this if you have, if you know what's typically done with celiac and splenic artery dilations at what size is typically…

親族の大動脈検査について

New ACC/AHA Aortic Disease Management Guidelines: What do they mean for you? 1:00:51-1:01:29 Q:When to start aortic, how old to start aortic screening relatives? 家系に結合組織疾患患者がいるのですが、その者の親族に対して大動脈の検査を行う…

動脈瘤がある場合に遺伝子検査が不要となる年齢上限はあるか?

New ACC/AHA Aortic Disease Management Guidelines: What do they mean for you? 59:51-1:00:45 Q:One person asked the important question. Is there a recommended age cutoff for peripheral aneurysms when considering genetic testing? 末梢の動脈瘤…

疾患別画像検査の頻度と種類について

New ACC/AHA Aortic Disease Management Guidelines: What do they mean for you? 58:58-59:50 Q:Here's a question when my husband and children go to their cardiology appointments do they need an MR, CT as well as an echo? 夫と子供が循環器内科で…

栄養チューブについて

Loeys-Dietz Gastroenterology and Allergy Q&A 42:34-45:52 Q:At what point do you decide a child needs a feeding tube? In this example as a seven-year-old that's really struggling to put on weight. 栄養チューブが必要となるのはどの時点でしょ…

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…

慢性的な嘔吐について

Loeys-Dietz Gastroenterology and Allergy Q&A 35:55-37:00 Q:Would you say the same for chronic or even like periodic vomiting? 慢性的な嘔吐や周期的な嘔吐の対処法を教えてください。 A:Absolutely, so, if, if, if you have chronic vomiting that …

慢性的な下痢について

Loeys-Dietz Gastroenterology and Allergy Q&A 34:39-35:54 Q:How about treatments or medications to identify or alleviate with chronic diarrhea? 慢性的な下痢の原因を調べる方法、対処法を教えてください。 A:I think there again I think a GI work…

栄養チューブは不要になるか?

Loeys-Dietz Gastroenterology and Allergy Q&A 32:43-34:24 Q:When, when it gets to the point that a child would need a feeding tube like ages two or three because of the they're not able to gain weight should they expect to have that tube fo…

体重を増やすにはどうしたらいいか?

Loeys-Dietz Gastroenterology and Allergy Q&A 28:59-32:41 Q:Several questions about sort of like general weight gain I know you touched on that, so if we could expand a little bit on, you know, aside from Miralax, exercise, fiber for kids o…

ロイス・ディーツ症候群に多い腸疾患と内視鏡検査時の注意事項

Loeys-Dietz Gastroenterology and Allergy Q&A 19:00-24:14 Q:Inflammation of the colon, is this common a common part of LDS? What is a solution and then this person is saying, "I've had two scopes, both just showed inflammation", so wonderin…

年齢によるアレルギー症状の変化と抗ヒスタミン薬について

Loeys-Dietz Gastroenterology and Allergy Q&A 17:05-18:53 Q:Can allergies, food allergies specifically, change with time in adults and/or children? We've had a couple questions about that. One specifically citing like had, had an allergy to…

小児科で食物アレルギーの検査をしてくれないのはなぜか?

Loeys-Dietz Gastroenterology and Allergy Q&A 15:05-16:54 Q:This one is about an eczema flare. It says the pediatrician will not run a food panel for allergies, only environmental. Any suggestions? じんましんが出ているのに、小児科では食物ア…

食物不耐症と食物アレルギーの違いについて

Loeys-Dietz Gastroenterology and Allergy Q&A 13:24-14:48 Q:At what point does an intolerance become an allergy? 食物不耐症が食物アレルギーに変わるのはどの時点ですか? A:So they're actually very different things. One doesn't necessarily tur…

懸念される大動脈のサイズ変化は?

Aortic Imaging: Your Questions Answered 55:55-56:22 Q:How much change in dimension is too much? 大動脈について、どの程度のサイズ変化であれば大きいと考えられますか? A:So, that my understanding is about three millimeters every year or less.…

心エコーレポートの「圧較差(pressure gradient)」とは何か?

Aortic Imaging: Your Questions Answered 52:04-53:35 Q:Somebody says every time I get an echo and they're post-surgery they have a quote-unquote increased gradient pressure. Can you explain what that is, why, how it affects, what's the effe…

画像検査で大動脈の厚さはわかるか?

Aortic Imaging: Your Questions Answered 50:01-51:59 Q:You know, I had a, we got a couple of questions in advance about looking at the thickness or thinness of the aortic wall, so can you talk about that a little bit like, are you looking a…