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…

Lung Issues in Marfan Syndrome 3:58-5:32 And so, I start with this triangle because this slide focuses on the most common symptom that patients with Marfan syndrome develop and that can possibly involve the lung and that is shortness of br…

Lung Issues in Marfan Syndrome セミナー資料： https://info.marfan.org/hubfs/Marfan%20Lung%20Disease%20Webinar%20Final.pdf 3:16-3:58 I first I want to thank everyone who's joining me with this webinar and what I want to do is I want to talk…

Can "complementary therapies" such as yoga help the lung problems for people with Marfan syndrome? Many patients with Marfan syndrome go into the general world where they have a sense of being different and having a sense that the differen…

What is pulmonary rehabilitation? What I advocate for many patients is going through what we call pulmonary rehabilitation and that is kind of a multidisciplinary practice where you learn ways of breathing that actually can reduce the sens…

How are wheezing and asthma connected to Marfan syndrome? Interestingly, we see a great deal of wheezing in both children and adults with Marfan syndrome and it's somewhat of a complex kind of symptom in Marfan syndrome because the cause o…

What is a "6 minute walk test" and why is it helpful for people with Marfan syndrome? One study that and I think more and more of the Mafan patients that I consult on are having is what I call an exercise study, which is a quantitative mea…

What is the appropriate treatment for restrictive lung disease? One of the aspects of restrictive lung disease that I encourage patients to really think about and discuss with their physicians is whether the surgical intervention that are …

What pulmonary tests do you recommend for Marfan patients? One of the things I actively encouraged and it almost becomes like a, like a broken record with me is I think anyone with Marfan syndrome should get a goodset of pulmonary function…

How are the lungs affected in Marfan syndrome? You know, the way I like to kind of discuss them are from the, the features that are the most common to the features, that are a little less common and a little more exotic. 最もよく見られる所…

Why does Marfan syndrome affect the lungs? Well, the striking aspect of lung function in Marfan syndrome is that it's so varied and it involves multiple different organ systems and the way that, I typically think about it, is that the the …