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 2019-01-01 to 1 year

ロイス・ディーツ症候群について ~身体的特徴④(頚椎)~

Loeys Dietz Syndrome: Virtual Medical Symposium Series (11/8/18) 19:36-21:07 Something that's unique to Loyes-Dietz syndrome that we pay a lot of attention to is the possibility of a problem in the spine in the neck, something we called ce…

ロイス・ディーツ症候群について ~身体的特徴③(骨格系)~

Loeys Dietz Syndrome: Virtual Medical Symposium Series (11/8/18) 17:48-19:35 There are many skeletal features that are seen in Loeys-Dietz syndrome, including the long fingers that we often observe in people with Marfan syndrome. ロイス・…

ロイス・ディーツ症候群について ~身体的特徴②(眼)~

15:58-There are features that we notice in young children with Marfan syndrome. ロイス・ディーツ症候群(訳者注:動画ではマルファン症候群とおっしゃっていますが、ロイス・ディーツ症候群の誤りではないかと思います)の子供にみられる特徴がありま…

ロイス・ディーツ症候群について ~身体的特徴①(口蓋垂)~

Loeys Dietz Syndrome: Virtual Medical Symposium Series (11/8/18) 14:21-15:57 Now I'm going to go through some of the physical features and I'll stress when a physical feature only helps us in making the diagnosis and also when a physical f…

ロイス・ディーツ症候群について ~ロイス・ディーツ症候群が認知されるまで~

Loeys Dietz Syndrome: Virtual Medical Symposium Series (11/8/18) 11:25-14:20 Now Loeys-Dietz syndrome is a condition that we first recognized and described about 13 years ago, back in 2005. ロイス・ディーツ症候群は、およそ13年前の2005年に…

ロイス・ディーツ症候群について ~βブロッカーとARBの非臨床試験~

Loeys Dietz Syndrome: Virtual Medical Symposium Series (11/8/18) 6:58-11:24 So that led us to the concept that a special class of blood-pressure medications called angiotensin receptor blockers, with one prominent example being losartan, m…

ロイス・ディーツ症候群について ~TGF-βとは~

Loeys Dietz Syndrome: Virtual Medical Symposium Series (11/8/18) 5:43-6:59 Now many different experiments taught us that these microfibrils that are composed of fibrillin-1 serve a second important function in that they normally control th…

ロイス・ディーツ症候群について ~マルファン症候群の概要~

As many of you know, Marfan syndrome is a connective tissue disorder that's related to Loeys-Dietz syndrome. It shows features in the eye, in the skeleton and also in the cardiovascular system. 皆さんの多くはご存知だと思いますが、マルファ…

ロイス・ディーツ症候群について ~はじめに~

Loeys Dietz Syndrome: Virtual Medical Symposium Series (11/8/18) 2:01-2:48 Good evening, everyone! It's really a pleasure to be you. What I'd like to try to do today is to give you a comprehensive overview of Loeys-Dietz syndrome, but also…

大動脈を守るために ~まとめ~

Taking Care of Your Aorta 10/15/18 (Virtual Medical Symposium Series) 41:34-42:15 So take care of your aorta by doing the following. Get a routine evaluation by imaging, know and remember your aortic size and have that followed, take medic…

大動脈を守るために ~大動脈解離発症後の管理~

Taking Care of Your Aorta 10/15/18 (Virtual Medical Symposium Series) 40:49-41:33And then finally it's important to recognize after a dissection, this is a Type B dissection, the aorta will grow over time. That's a natural history and it h…

大動脈を守るために ~大動脈解離の手術~

Taking Care of Your Aorta 10/15/18 (Virtual Medical Symposium Series) 39:41-40:49 The surgery of an aortic dissection involves replacing the aorta with a graft. 大動脈解離の手術では、大動脈を人工血管で置き換えます。 But not with an endogra…

大動脈を守るために ~大動脈解離の症状~

Taking Care of Your Aorta 10/15/18 (Virtual Medical Symposium Series) 38:54-39:41The symptom of a dissection is usually abrupt severe chest pain or back pain, something that's never had before. Sharp and severe are the most typical adjecti…

大動脈を守るために ~大動脈解離の基礎知識~

Taking Care of Your Aorta 10/15/18 (Virtual Medical Symposium Series) 37:30-38:53 Finally, what is an aortic dissection? What does it feel like? This is a intraoperative example of an aortic dissection provided by Joe Bavaria from Pennsylv…

大動脈を守るために ~大動脈手術の目安(ロイス・ディーツ症候群など)~

Taking Care of Your Aorta 10/15/18 (Virtual Medical Symposium Series) 36:08-37:29 In Loeys-Dietzs syndrome, we recommend the aorta be operated between4 and 4.5 cm depending on the specific mutation, the family history, the gender, craniofa…

大動脈を守るために ~大動脈の大きさが5cm未満でも手術を行うケース~

Taking Care of Your Aorta 10/15/18 (Virtual Medical Symposium Series) 34:34-36:07 In those caveats are as follows. So it's not strictly a line in the sand, and we often choose an aortic dimension a little less than 5 cm and we start thinki…

大動脈を守るために ~大動脈手術のタイミング~

Taking Care of Your Aorta 10/15/18 (Virtual Medical Symposium Series) 33:26-34:33When should one have aortic surgery? In general, in Marfan and related disorders, once the aorta achieves 5 cm, it's recommended to have prophylactic surgery.…

大動脈を守るために ~妊娠・出産について~

Taking Care of Your Aorta 10/15/18 (Virtual Medical Symposium Series) 30:50-33:25Pregnancy is a risk factor for aortic dissection in women who have Marfan and other genetic aortic conditions. Most of the information we have about that risk…

大動脈を守るために ~薬と運動~

Taking Care of Your Aorta 10/15/18 (Virtual Medical Symposium Series) 29:40-30:48 It's important to recognize that being on a medication for the aorta such as a beta blocker, which slows the heart rate and lessens, lessens force of contrac…

大動脈を守るために ~運動について~

Taking Care of Your Aorta 10/15/18 (Virtual Medical Symposium Series) 26:31-29:40When you think about exercise and physical activity, of course, they're different whether you're doing leisurely walking, which would be a dynamic or isotonic…

きっとあなたを忘れない―グリーフとの新たな向き合い方

2018年4月25日 Dominga Noe 私はすごくポジティブな人間―特にマルファン症候群に関してはそうありたいと思っています。この考えにたどり着くまでには長い時間がかかりました。毎日が幸せというわけではありません。でもトータルで見れば、マルファン症候群は…

マイ マルファン ストーリー:ケーシー・ランフィア

2018年2月27日Kacey Lamphier 2月は心臓月間ということで、私にとっては特別重要な月です。私は30年間マルファン症候群と共に生きてきましたが、診断を受けたのは22年前です。私は日々マルファン症候群についての自らの体験や知識を広げる活動をしています。…

思春期の怒りと自覚:自分と身体を理解するために

2019年2月11日 Sarah E. McMillan はじめに 思春期と慢性疾患 自覚 アイデンティティとマルファン 受容とマルファン 医療とQOL はじめに 自分の病気のことを話題にしたくなくて、新しい友達の輪に入って、少し(orメチャクチャ)喋り過ぎたことはありません…

大動脈を守るために ~病気の理解と運動~

Taking Care of Your Aorta 10/15/18 (Virtual Medical Symposium Series) 25:05-26:30 Taking care of your aorta; knowledge is power, so know about your condition. Everybody with Marfan or a related disorder should know they have the condition,…

大動脈を守るために ~推奨される薬物療法~

Taking Care of Your Aorta 10/15/18 (Virtual Medical Symposium Series) 23:41-25:04 And unfortunately, it's so fortunately, it is helpful, but it's, it has not been yet proven in the largest trials to be better than beta blocker in single tr…

大動脈を守るために ~マウスモデルでのロサルタンの効果~

Taking Care of Your Aorta 10/15/18 (Virtual Medical Symposium Series) 22:30-23:40 So in a Marfan mouse, losartan, by blocking TGF-β, improved the aorta. So this wild-type means a normal mouse and between these arrowheads is a normal aortic…

大動脈を守るために ~TGF-βについて~

Taking Care of Your Aorta 10/15/18 (Virtual Medical Symposium Series) 20:29-22:30 And research from many investigators throughout the world, particularly the group at Johns Hopkins, have demonstrated that there's more to it than just blood…

ロイス・ディーツ症候群について

はじめに ロイス・ディーツ症候群の有病率 ロイス・ディーツ症候群の特徴 ロイス・ディーツ症候群の原因 遺伝について ロイス・ディーツ症候群の診断 正確な診断が重要である理由 ロイス・ディーツ症候群の管理方法 心臓・血管 骨・関節 アレルギー・消化器 …

大動脈を守るために ~βブロッカーについて~

Taking Care of Your Aorta 10/15/18 (Virtual Medical Symposium Series) 18:20-20:28 One of the basic concepts in medical management, in other words, non-surgical therapy of aortic aneurysm disease, we'll talk about medications, learning abou…

大動脈を守るために ~大動脈検査の頻度~

Taking Care of Your Aorta 10/15/18 (Virtual Medical Symposium Series) 15:39-18:19 Now, how often should I have a or take imaging and it really depends on the condition, the size of the aorta, and how would, how rapidly it's growing. So for…