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 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 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 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…

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 …

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 …

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…

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…

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…

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…

Lung Issues in Marfan Syndrome Now we move to scoliosis, which again is very common in Marfan syndrome, and I'm certain that many of the people who are listening now have encountered this particular complication. On this slide I've listed …

Lung Issues in Marfan Syndrome 12:54-14:16 So you can ask the question of whether correction of a pectus deformities will improve lung function. If you have abnormal lung function, to begin with, most of the data suggests that it probably …

Lung Issues in Marfan Syndrome Now what are the approaches to repair? What has become kind of the most common procedure is what's called a NUSS procedure, and many of you are familiar with that. では胸骨異常に対しては、どのような治療法があ…

Lung Issues in Marfan Syndrome 10:47-11:49 Now what is the natural history of pectus abnormalities？ Unfortunately, they've been very, very, few studies in Marfan syndrome but what we do know from studies in the non Marfan population is th…

Lung Issues in Marfan Syndrome Now let's move to pectus abnormalities, which are quite common in Marfan syndrome and these are typically either pectus excavatum where you have the depression in the middle of your chest or the depression ca…

Lung Issues in Marfan Syndrome 8:46-10:09 And we will start with this. So another term for respiratory muscle weakness is the neuromuscular consequences of Marfan syndrome. We know that persons with Marfan syndrome have skeletal muscle wea…

Lung Issues in Marfan Syndrome 8:19-8:45 Now what are the causes of restrictive lung disease in persons with Marfan syndrome? These are the three big causes: scoliosis, pectus deformities, particularly pectus excavatum where you get the de…

Lung Issues in Marfan Syndrome 6:07-8:18 So let's start with restrictive lung disease. About 50% or more of patients with Marfan syndrome have some degree of restriction and this ranges from relatively mild restriction, which is not someth…

Lung Issues in Marfan Syndrome 5:33-6:06 And you may ask, well, "Why are there lung problems in Marfansyndrome?" and that's because the mutant protein in Marfan syndrome, specifically fibrillin-1, is expressed in the lung and it's expresse…