はじめに マルファン症候群で一般的にみられる心血管疾患 大動脈拡張・大動脈瘤 大動脈解離 僧帽弁逸脱 大動脈弁逆流 心不全 マルファン症候群で一般的な心血管疾患の管理法 薬 検査 日常生活で注意すべきこと 緊急事態（例：大動脈解離）にすべきこと はじ…

はじめに マルファン症候群における一般的な心臓外科手術の種類 大動脈基部に対する一般的な心臓外科手術の術式 コンポジットグラフト手術（ベントール術） メリット デメリット 自己弁温存術 メリット デメリット 生体弁置換術 メリット デメリット 動脈内…

Taking Care of Your Aorta 10/15/18 (Virtual Medical Symposium Series) 13:22-15:38 This is a CT scan with contrast lighting up the ascending aorta and descending aorta in a person with a very large thoracic aneurysm, this large circle here.…

Taking Care of Your Aorta 10/15/18 (Virtual Medical Symposium Series) 11:48-13:21 Now a chest X-ray can show features on it, aneurysm in the chest, but it's notoriously inaccurate. では、動脈瘤は胸部レントゲンでどう見えるかについて説明して…

Taking Care of Your Aorta 10/15/18 (Virtual Medical Symposium Series) 10:23-11:47 Now in Marfan and Loyes Dietz and familial aneurism syndrome, as I mentioned, this is an echocardiogram showing the aorta. This is the muscle part, this is t…

Taking Care of Your Aorta 10/15/18 (Virtual Medical Symposium Series) 8:59-10:22 An aneurysm is an enlarged aortic segment, so it's pathologic meaning, it's abnormal and it's larger than expected by at least 50%, so that's one criteria, so…

Q: 治療・手術においては、Zスコアと大動脈基部の大きさ、どちらが信頼できる指標となりますか？より重要なのはどちらですか？ A: 医師は大動脈の太さを表すのに「Zスコア」という言葉を使います。「Zスコア」とは、患者さんの大動脈のサイズが標準サイズか…

Taking Care of Your Aorta 10/15/18 (Virtual Medical Symposium Series) 7:18-8:58 Now the aorta size again depends on a person's size. So when we see somebody in the clinic for "Is the aorta enlarged?", how does this fit for somebody? 繰り返…

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 …

Q: マルファン症候群では背が高くなりますか？ A: マルファン症候群では、必ず身長が高くなるというのはよくある誤解です。高身長、つまり家族の中で背が高いということは、最も一般的な特徴の1つですが、必ずしもそうなるわけではありません。目につきやす…

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…

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 …

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…

Taking Care of Your Aorta 10/15/18 (Virtual Medical Symposium Series) 6:03-7:17 It's that condition of the aorta, cystic medial degeneration, which is also called in the old days, cystic medial necrosis or currently, we sometimes call it m…

Taking Care of Your Aorta 10/15/18 (Virtual Medical Symposium Series) 4:54-6:01 And the aorta is not just an inert structure. It's alive and it has vital parts to it. It has three layers; one layer, the inner lining so the blood goes throu…

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 …

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…

Taking Care of Your Aorta 10/15/18 (Virtual Medical Symposium Series) 3:07-4:52 The aorta is the largest artery of the body so it arises from the heart, this red tube, and that's the ascending aorta and it arises and it carries all the blo…

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…

Taking Care of Your Aorta 10/15/18 (Virtual Medical Symposium Series) 1:55-3:05 Thank you Eileen for the introduction and for the invitation to speak about a topic that I'm very interested in and really is one of my passions is helping eve…

2019年7月に開催された、第35回年次総会のプレゼン動画です。 内容 38年間にわたる研究結果 大動脈手術について FDAからの警告およびリコール情報 オーダーメード医療 血管型エーラス・ダンロス症候群について 大動脈基部に対する運動の効果 Backpack Health…

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…

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…

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…

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…

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…

Lung Issues in Marfan Syndrome 19:02-20:45 And here I've summarized kind of the causes of restrictive lung disease in persons with Marfan syndrome. このスライドでは、マルファン症候群における拘束肺疾患の原因をまとめました。 And the points a…

Lung Issues in Marfan Syndrome Many of you are familiar with the interventions that are used for scoliosis and they range from observation to casting embracing, if you have more severe curvatures, and finally, the surgical options that I'v…

Lung Issues in Marfan Syndrome 16:26-18:06And so it's very important that if there is going to be an intervention for scoliosis that a pulmonary evaluation is performed and this should include a full set of pulmonary function tests with a …

Lung Issues in Marfan Syndrome 15:15-16:25 On this slide, we see kind of how we actually measure the cobb angle and we also see an equation that suggests that we can predict the lung capacity based on the severity of the curvature. このス…