LDS Guidelines: Then vs. Now 1:11:03-1:13:52 Q:Any correlation or conversation about POTS in Loeys-Dietz syndrome? POTSとLDSは関連がありますか？ A:Yeah, so POTS stands for Postural Orthostatic Tachycardia Syndrome. Basically what it's desc…

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…

2023年9月23日 The Marfan Foundation 「人生が一変したあの瞬間…」 2020年7月31日。僕の人生が一瞬で変わってしまった日。早めの夕食を済ませて帰宅すると、左腕が突然感覚を失った。家はひんやりとしていたのに、汗が吹き出してくる。左腕の感覚はない。妹…

LDS Guidelines: Then vs. Now 1:08:02-1:09:32 Q:Is there any precautions with arterial line insertion for anesthesia and the risk of arterial dissection in Loeys-Dietz patients, so maybe also like catheter procedures. LDSでは、血管に管を入…

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 Guidelines: Then vs. Now 1:01:25-1:04:24 Q:One of the questions, I'll just kind of start picking some, is about you talked about the meta-analysis which was awesome and then you touched upon adding a beta blocker. Can you just let us k…

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 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…

LDS Guidelines: Then vs. Now 54:34-57:35 In 2014 we did talk about kind of neurological issues. In the first reports of Loeys-Dietz syndrome we had some cases of individuals with intellectual disability, however, we think this is really re…

LDS Guidelines: Then vs. Now 52:45-54:31 Great and then we just want to end with a few slides of kind of like all the other stuff and so we know that in patients with Loeys-Dietz syndrome there can be dental anomalies that can include clea…

LDS Guidelines: Then vs. Now 50:21-52:43 Do you want to say anything about this or this is a kind of an outline, you know, when we look at Loeys-Dietz syndrome compared to Marfan syndrome, compared to Shprintzen-Goldberg syndrome, which Dr…

LDS Guidelines: Then vs. Now 46:57-50:15 Same thing for gastroenterology and nutrition for Loeys-Dietz syndrome. We do follow the growth of our patients, especially our young kiddos very closely, especially if someone is looking at a surge…

LDS Guidelines: Then vs. Now 44:57-46:54 So I know Tony is taking notes for us, Dr. Guerrerio and if he has anything to jump in but we do know or we have appreciated, I'm going back to 2014 that there is an increased evidence of allergic d…