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。

呼吸器

マルファン症候群をはじめから ~心臓以外の所見のフォロー・治療~

Marfan Syndrome Management: Back to Basic 19:14-20:23 And what about non-cardiac surveillance and treatment? 心臓以外の所見に関する検査と治療について説明します。 We recommend serial ophthalmology evaluations in children. It's every year. I…

リスクと生きる ~大動脈疾患に関わる遺伝子および気胸に関連する疾患~

Living with Risk 28:32-30:17 Here's a patient who has vEDS and Loeys-Dietz, VUS. So this is a not exactly a disease-causing mutation has had a pneumothorax and so what imaging does this person need. vEDSとLoeys-Dietz症候群両方の遺伝子検査…

反応性気道疾患があるとCOVIDの重症化リスクは高まるか?

Covid and Aortic Conditions: The Latest Research 57:17-58:18 Q:Somebody's asking if they have R-A-D, RAD would that put them at risk, so what is RAD?RADでは高リスクとなるか、という質問です。RADとは何ですか? A:I, I think they're talking ab…

再発性気胸でCOVIDの重症化リスクは高まるか?

Covid and Aortic Conditions: The Latest Research 54:00-55:01 Q:Would you say that those with recurrent pneumothoracies, or spontaneous, spontaneous lung collapse, so people with recurrent pneumothoracies, are they at high risk regardless o…

新型コロナウイルス感染症と大動脈疾患についての最新研究 ~高リスク肺疾患について~

Covid and Aortic Conditions: The Latest Research 8:13-12:31 So to get started, let's, you know, tackle the question about who is at high risk of bad outcomes if they get COVID-19. So having Marfan, LDS or vEDS or most of the other genetic …

マルファン症候群での肺疾患の割合と拘束性肺疾患の診断方法について

Children's Heart Issues in Marfan, Loeys Dietz, and vEDS - Virtual Symposium Series (4/9/19) 44:26-46:07 Q:This is not about the heart but close to the heart and cardiovascular system. Can you just talk briefly about lung problems in Marfa…

肺とマルファン症候群

はじめに マルファン症候群に多い肺疾患 拘束性肺疾患 突然の肺虚脱(自然気胸) 肺気腫 喘息 睡眠時無呼吸 マルファン症候群での肺疾患の治療法 拘束性肺疾患 肺虚脱(気胸) 肺気腫 喘息 睡眠時無呼吸 日常的にできること はじめに マルファン症候群および…

肺を強くする方法はあるか?

COVID-19 and Marfan/VEDS/LDS: Your Questions Answered 1:15:08-1:16:30 "Is there anything that we can do now to strengthen our lungs to prevent a worse outcome with this virus and in general just for us and our children?" 「COVID-19の重症化…

人工呼吸器を使用することで、気胸になったり肺が弱くなるリスクが上昇するか?

COVID-19 and Marfan/VEDS/LDS: Your Questions Answered 1:11:34-1:12:27 The third question is "Will the use of a ventilator increased the risks of lung collapse and fragile lungs?" 「人工呼吸器を使用することで、気胸になったり肺が弱くなるリス…

COPDなどの拘束性肺疾患がある場合、COVID-19に感染するとどのような影響があるか?

COVID-19 and Marfan/VEDS/LDS: Your Questions Answered 1:11:02-1:11:33 So the second question is "How will the lungs be affected if you already had COPD or another restrictive lung disease?" 「COPDなどの拘束性肺疾患がある場合、COVID-19に感…

肺の基礎疾患があると、COVID-19により悪化しやすいか?

COVID-19 and Marfan/VEDS/LDS: Your Questions Answered 1:07:51-1:11:07 So these are questions that deal with lung issues and this virus, so Enid? このスライドの質問は肺に関するものですので、Enid先生お願いします。 Yeah, these we covered some…

COVID-19の症状があっても、ステロイド吸入器を使用できるか?

COVID-19 and Marfan/VEDS/LDS: Your Questions Answered 58:54-1:00:40 And the last issue, "if you use an inhaler that contains a steroid can you still use it if you have COVID-19 symptoms?" では、このスライドの最後の「COVID-19の症状があって…

Marfan患者がCOVID-19に感染した場合、CPAPの利用は可能か?

COVID-19 and Marfan/VEDS/LDS: Your Questions Answered 57:25-58:54 I'll step in with the CPAP question. The general consensus among the pulmonologist and intensivist about CPAP with COVID is that it's probably not an ideal choice and that i…

COVID-19 ハイリスク患者と人工呼吸器

肺疾患および心疾患の基礎疾患のある患者はハイリスクか? ハイリスク肺疾患 ハイリスク循環器疾患 その他のハイリスク疾患 人工呼吸器の使用 肺疾患および心疾患の基礎疾患のある患者はハイリスクか? マルファン症候群、血管型エーラス・ダンロス症候群(V…

COVID-19とマルファン関連疾患 ~ハイリスクな肺疾患~

COVID-19 and Marfan/VEDS/LDS: Your Questions Answered 21:49-26:31 So you know, many patients even though we have all of these subgroups of whom is at higher risk are asking about certain features that they have, so maybe we can just go thr…

COVID-19とマルファン関連疾患 ~肺に関する注意事項~

COVID-19 and Marfan/VEDS/LDS: Your Questions Answered 19:21-20:42 I share one other point before we move on. 私の方から一点補足したいと思います。 So as Enid was mentioning, people with many of these diagnoses can be at risk for a pneumotho…

COVID-19とマルファン関連疾患 ~肺疾患~

COVID-19 and Marfan/VEDS/LDS: Your Questions Answered 7:12-9:20 So let's go over some of those characteristics that the high-risk features that the PAB has put together and the first part that we'regoing to look at is those that affect the…

加齢とマルファン関連疾患 ~呼吸器~

Aging with Marfan and Related Conditions (VIrtual Medical Symposium Series, 5/6/19) 20:44-23:26 Now the respiratory system ages in all of us. Even though the Marfan patient will have a worse lung function because of the impairment of lung …

マルファン症候群における呼吸器疾患 ~睡眠時無呼吸のまとめ~

Lung Issues in Marfan Syndrome 37:46-38:23 So the take-home message here is that sleep apnea is underdiagnosed in Marfan syndrome that all adult patients with Marfan syndrome should be considered for screening and hopefully with our study …

マルファン症候群における呼吸器疾患 ~睡眠時無呼吸の診断・治療について~

Lung Issues in Marfan Syndrome 35:30-37:44 Now how do you make the diagnosis? You typically make the diagnosis with a sleep study and a sleep study in a clinic or in a hospital that will give us information about the brain activity, the ey…

マルファン症候群における呼吸器疾患 ~睡眠時無呼吸とマルファン症候群の関係について~

Lung Issues in Marfan Syndrome 33:17-35:29 Now studies from our group as well as others have shown a very high prevalence of sleep apnea in persons with Marfan syndrome, particularly obstructive sleep apnea, and we think that's because of …

マルファン症候群における呼吸器疾患 ~睡眠時無呼吸について~

Lung Issues in Marfan Syndrome 31:31-33:16 We'll end on sleep apnea and the definition of sleep apnea is that this is an intermittent cessation of airflow at the nose and the mouth during sleep and it affects a significant number of non-Ma…

マルファン症候群における呼吸器疾患 ~肺気腫のまとめ~

Lung Issues in Marfan Syndrome 31:00-31:30 And so the take-home message is that emphysema is probably a sequela of disturbances in lung development in Marfan patients. We see this especially in persons with neonatal Marfan syndrome and in …

マルファン症候群における呼吸器疾患 ~肺気腫の症状・診断・治療法~

Lung Issues in Marfan Syndrome 29:43-30:59 And so the symptoms as we see here are the shortness of breath with activity. You can also get bouts of bronchitis because your airway clearance is also compromised with emphysema and sometimes if…

マルファン症候群における呼吸器疾患 ~肺気腫について~

Lung Issues in Marfan Syndrome 28:09-29:42 Now let's talk a little about emphysema. So emphysema can be seen with Marfan syndrome and what it is, is the loss of alveolar walls and generally this can be developmental or genetic or it can be…

マルファン症候群における呼吸器疾患 ~気胸のまとめ~

Lung Issues in Marfan Syndrome So the take-home message is that first, as we know, pneumothorax is a common manifestation of Marfan syndrome and if these are recurrent, a pulmonologist should be consulted so there can be some assessment of…

マルファン症候群における呼吸器疾患 ~気胸の手術適応~

nLung Issues in Marfan Syndrome 26:10-27:27 So, what are the indications for surgery in pneumothorax? And surgery often revolves a removal of the blebs as well as a mechanical pleurodesis. では気胸において手術適応となるのはどのような場合で…

マルファン症候群における呼吸器疾患 ~気胸の治療~

Lung Issues in Marfan Syndrome 23:19-26:09 So how do we treat pneumothorax? If it's small, hospitalization and supplemental oxygen will be sufficient, however if the pneumothorax is either moderate or large, a chest tube placement for evac…

マルファン症候群における呼吸器疾患 ~気胸~

Lung Issues in Marfan Syndrome 21:22-23:18 Let's move on to pneumothorax. So the first question is, "What is a pneumothorax?" and it's often called lung collapse and it reflects when the lung detaches from the chest wall. では気胸に移りま…

マルファン症候群における呼吸器疾患 ~拘束性肺疾患について知っておくべきこと~

Lung Issues in Marfan Syndrome 20:45-21:21 So the take-home message is that with restrictive lung disease in persons with Marfan syndrome, serial pulmonary function tests to follow the progression of restriction are needed and there should…