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。

ロイス・ディーツ症候群(LDS)

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 〜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…

ステロイドについて

Navigating GI Management in LDS 49:38-51:39 Q: You mentioned that steroids could be harmful for bones. Do the benefits outweigh risks if using steroids to treat asthma or EoE? ステロイドを服用すると骨への悪影響の可能性があるというお話があ…

好酸球性食道炎の発症タイミングについて

Navigating GI Management in LDS 46:23-47:56 Q: Can EoE be triggered hours after exposure to the allergen? Is that different than a long-term symptom onset? I think I'm not exactly sure how the question... maybe you understand what they're …

LDSと吸収不良との関連について

Navigating GI Management in LDS 42:52-45:03 Q: Is there a correlation between LDS and malabsorption? LDSと吸収不良との間に関連はありますか? A: Malabsorption is when your body cannot appropriately absorb all the nutrients you're eating. Th…

NSAIDの長期服用について

Navigating GI Management in LDS 39:40-41:46 Q: Doctors are quick to recommend NSAIDs for different problems related to inflammation that is seen in people with connective tissue conditions, people with LDS. However, we understand that chro…

消化器疾患に対する生物学的製剤について

Navigating GI Management in LDS 35:31-39:28 Q: I will say that there are a couple of themes coming up. One is about medication management and EoE, another's on like EoE. So I'm going to just try and sort through these and go through them i…

ロイス・ディーツ症候群における消化器管理 〜まとめ〜

Navigating GI Management in LDS 34:06-35:01 So, a summary and then I'm happy to take questions. では、まとめです。後ほど質問にお答えします。 BMI should be looked at at every visit. Proper nutrition can lead to a faster recovery from surger…

ロイス・ディーツ症候群における消化器管理 〜その他の消化器疾患〜

Navigating GI Management in LDS 32:58-34:05 Other anatomic issues which we've seen in Loeys-Dietz syndrome - these are rare events, so I can't conclusively say at this point that these are more likely to occur in Loeys-Dietz syndrome, only…

ロイス・ディーツ症候群における消化器管理 〜ヘルニア〜

Navigating GI Management in LDS 30:15-32:57 So last subject is just some anatomic considerations. Hernias tend to be very common in LDS. There are hernias like, for example, where you've had a surgery where there's been an incision in the …

ロイス・ディーツ症候群における消化器管理 〜機能性胃腸障害〜

Navigating GI Management in LDS 27:42-30:12 So the last thing in terms of... oh, there are a couple of other things. One of them is disorders of gut-brain interaction or functional GI disorders, and I want to talk about this because this i…

ロイス・ディーツ症候群における消化器管理 〜炎症性腸疾患(IBD)〜

Navigating GI Management in LDS 24:42-27:40 Another type of intestinal inflammation that is more common in people with LDS is something called inflammatory bowel disease (IBD). Inflammatory bowel diseases occur when your white blood cells …

ロイス・ディーツ症候群における消化器管理 〜好酸球性消化器疾患〜

Navigating GI Management in LDS 21:54-24:40 Let's talk about some inflammation. So I talked about the fast allergy; there's a slow motion food allergy that can occur in the lining of the intestines. This is eosinophilic GI disease. The mos…

ロイス・ディーツ症候群における消化器管理 〜食物アレルギー〜

Navigating GI Management in LDS 18:39-21:51 I'm gonna take some time to talk about food allergies here, so there's two kinds of food allergies. This first kind of food allergy I'm going to talk about is an anaphylactic or IgE-mediated food…

ロイス・ディーツ症候群における消化器管理 〜便秘〜

Navigating GI Management in LDS 12:25-18:38 We're going to talk about constipation a little bit. This is super common in people with connective tissue disorders. Well, it's super common in general, but in people with connective tissue diso…

ロイス・ディーツ症候群における消化器管理 〜骨〜

Navigating GI Management in LDS 8:24-12:23 So how does nutrition feed into bone health? People with Loeys-Dietz Syndrome have weak bones, we think. This is part of the Loeys-Dietz syndrome. About one-third of adults and about two-thirds of…

ロイス・ディーツ症候群における消化器管理 〜栄養補給と栄養療法〜

Navigating GI Management in LDS 5:22-8:23 So when should we think about supplementation? Again, if someone is dropping percentiles, that last graph I showed you, if someone is less than the 10th percentile for their body mass index or thei…

ロイス・ディーツ症候群における消化器管理 〜BMIグラフ〜

Navigating GI Management in LDS 1:53-5:18 In terms of nutrition, growth is frequently impaired in people with LDS. There's an increased baseline caloric expenditure if the person has significant food allergies, but those food allergies may…

結合組織疾患と加齢 〜56歳LDS女性の不安〜

Aging with a Connective Tissue Condition 2:09-3:25 I'm going to start with just talking about a patient I saw recently in my clinic. This lady is 56 and has Loeys-Dietz syndrome. She feels well. She had imaging of the chest done for anothe…

ロイス・ディーツ症候群の白内障手術におけるリスクについて

Insights on Ocular Management for Marfan Syndrome 38:38-40:04 Q: Are there any risk factors for cataract surgery for people with Loeys-Dietz syndrome? ロイス・ディーツ症候群の白内障手術では、どのようなリスク因子がありますか? A: Yeah, it’s…

ロイス・ディーツ症候群における婦人科疾患 〜避妊法〜

Women's Health: Beyond Family Planning 8:25-11:07 I just wanted to give a quick overview at the beginning about different contraceptive medications and whether or not they're safe for patients with certain medical conditions. We use guidel…

ロイス・ディーツ症候群における婦人科疾患 〜はじめに〜

Women's Health: Beyond Family Planning 5:23-8:15 So with this webinar we're going to be talking really about Loeys-Dietz syndrome, talking about a gynecological issues, we're going to overview some common problems and then discuss potentia…

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…

動脈瘤・解離の管理 ~vEDS/LDS/MFSでの動脈疾患分布~

動脈瘤・解離の管理Vascular Surgery 3:29-5:23 You've seen this picture. This is from Gustavo Oderich's paper where a few years ago he talked about the distribution of arteriopathy in vascular EDS and then on the right hand side is our paper…

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…

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…

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…

LDSガイドラインの改訂に向けて ~呼吸器疾患~

LDS Guidelines: Then vs. Now 58:37-59:43 From a lung, pulmonary perspective we know, do know that there is restrictive lung disease, that sleep apnea may be underdiagnosed in our population, so again in terms of like general fatigue and pe…

LDSガイドラインの改訂に向けて ~眼の疾患~

LDS Guidelines: Then vs. Now 57:36-58:37 In terms of eye disease we do see retinal detachment. I know some of your questions are, what's common, what's not, what's rare, what do I have to be worried about. 眼の疾患については、一般的な眼の…