Lymphangioleiomyomatosis (LAM)
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- Опубликовано: 27 июл 2024
- In this video, I cover the cystic lung disease, lymphangioleiomyomatosis (LAM).
There are a few important clinical practice guideline articles that are very useful to both clinicians and radiologists which can be viewed here:
www.thoracic.org/statements/g...
Textbooks I like for chest radiology-
Med students and all residents: Felson’s Principles of Chest Roentgenology
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Radiology residents: Thoracic Imaging: Pulmonary and Cardiovascular Radiology
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Thoracic radiology fellows: Muller’s Imaging of the Chest: Expert Radiology Series
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This is the best thoracic radiology channel. Thank you!!!
Thank you!! Thank you so much😊
I greatly appreciate ur excellence in explaining ur knowledge on the Lam Lung disease and all the extras. so much valuable information. Sad to say very hard to find. Most professionals lack it. I have 2 years diagnosed with Lam no treatment yet and still have problems understanding it. So again Thank you for ur time and services God Bless. ❤
GREAT LECTURE! I really love these.
Amazing presentation and explanation of LAM. Thank you sir.
Thank you so much
Great lecture
May you do more cases like this please
Thank you for a great lecture
Thank you Dr. Rishi , we are greately benifiting from you Throracic Videos and recently your apperence at Ref Cafe was very impormative on Chronic lung Consolodation not responding to antibiotics . GOD BLESS YOU !
Thank you! It was a fun lecture!
Thank you sir!
Thank you so much for directing me to this video. I love your presentations. I'm having an MRI at your institution on November 17. Perhaps you will be interpreting it.
Keep up the good work!
Well presented!!!!
thank u so much for interesting and informative lecture :3
it can also appear on the utarus as someone who has both TSC,LAm and endometriosis when my uterus was removed from endo they found LAM cysts on it and another lammie sister had it in her stomach
I didn't know that. Thank you
Good Video
Could you please post a video of pneumoconiosis please
❤️❤️
I have LAM and was just diagnosed with pneumonia. What are the warning signs (pain signals) of a pneumothorax? Thank you very much.
Where are you located? Can I make an appointment for you to view my imaging? You are so thorough and I feel something is being overlooked with me.
Very informative video, Sir may I ask what software do you use for editing the videos?
I use Adobe Premiere
Hi Rishi, excellent video. I have a couple of questions.
1. How to differentiate with emphysema in a middle aged female with heavy smoking history? Some of the advanced images you showed could be mistaken for that?
2. In early disease when you may only have a few cysts, how do you differentiate a pneumotocele from early LAM? Recently saw a young female patient post COVID with 4-5 cysts primarily in the lower lobes which I think were pneumotoceles? CT A/P negative, VEGF normal and asymptomatic.
Thank you.
Great questions. For emphysema, the distribution is upper lung predominant. Also the holes in emphysema should not have perceptible walls and there are frequently small vessels running through them. But you're right, in cases of advanced LAM in smokers, it can be difficult to tell and in fact both can coexist.
The European Respiratory Society addressed the number of cysts question. They said it was characteristic of LAM if there are more than 10 cysts and no other lung findings that could explain them. And if it is 2-10 cysts, it could be compatible with LAM but only if you have other evidence (VEG-F, AMLs, lymphangioleiomyomas, positive path, etc.). Yeah i think you're right, most likely your case was from pneumatoceles from COVID, particularly if it was a more severe case.
Thank you for an informative video. If I understood you correctly, pneumothorax in fertile females should always be followed up by an HRCT to exclude LAM? So, I can infer that spontaneous pneumothorax is very rare in young females?
Hi, it depends on how sensitive and specific you want to be. I would definitely do a CT in a female with recurrent pneumothorax or bilateral pneumothorax without known cause. And also would probably do it in hydropneumothorax. I think doing CT on the first pneumothorax is still reasonable but the practitioner should realize that the number of cases that will be positive for LAM is low. Yes spontaneous pneumothorax is uncommon, rare might be too strong of a word.
The retroperitoneal lymphanioleiomyomas look a lot like lymph node conglomerates, is that essentially what they are? Or is the underlying path not really affecting the LN? The name seems like it would. Basically in isolation, it kind of looks like lymphoma? Thank you for the great lecture and channel!
Good question. I think if you came across a patient with the lymphangiomas, perhaps lymphoma would be in your differential on first glance. But they do behave differently from enlarged nodes or nodal conglomerates. One of the biggest differences in my mind is that they can change throughout the day or with positioning. I have a case where the patient is supine and they are large and on prone they get smaller.
i have sporadic lymphangioleiomyomatosis
How can we prevent this desease
l am suffering from this desease lam from india
11:20 DIFFERENTIAL DIAGNOSIS OF LAM
A question... How to differentiate between retroperitoneal lymphangioleiomyoma n matted necrotic lymph nodes, or necrotic paraganglioma from CT alone?
Lymphangioleiomyoma will have a diurnal variation as opposed to the other processes.
Hey Rishi... Is it true that a cystic lesion accompanied by an eccentric vessel is almost always bronchiesctesis. Like, this is what we have been taught in classes. Of late, i find many cases of cyst like diseases, not clinically bronchiesctesis, also have such eccentric vessel
not always, sometimes a cyst with an eccentric vessel is just a cyst. This is a classic description of the cysts in LIP. If it looks like it is connecting with the rest of the bronchi, then it is bronchiectasis.
@@ThoracicRadiology thank you 😀
1:33 a frequent cause for (25% one sample) pneumothorax in premenopausal women is diaphragmatic endometriosis, and since LAM affects women almost exclusively, LAM seems a bit sus, especially given how rare LAM is.
(LAM cells are 'similar to cancer cells'. Since both cancer and endometriosis are linked to local iron excess, the lam cells might just be a coincidental finding to endometriosis causing pneumothorax)
Clinical presentation and treatment of catameinal pneumothorax and endometriosis-related pneumothorax, 2018
3:54 making my point: how can angiomyolympoma (most common benign kidney tumor) be a sufficient diagnosis for LAM?
Interesting theory but endometriosis in the chest is super rare. It is nowhere near 25%.
AML in the kidney alone is not sufficient. The criteria requires more than that.
@@ThoracicRadiology from the cited paper:
"it is still considered relatively rare although accounting for 20-35% of spontaneous pneumothoraces occurring in premenopausal women"
At issue is ageism. It is a very frequent cause for pneumothorax in child bearing age women. Which is only a small portion of the pneumothorax population since most are geriatrics. But it is clearly incorrect to apply geriatric diagnostics when a patient doesn't fall in that category and the statistics are so different for old vs young such as in spontaneous pneumothorax.
Finally, endometriosis is extremely common in women, while LAM is a super rare genetic disease (which inconveniently is impossible to genetically confirm because it is not possible to gauge if any given mutation in this huge gene would render it disfunctional, apart from some known mutations). So seems clear to me which is going to be more indicative in pneumothorax.
I got told I had it just over 2 years ago and if I didn't stop smoking I'd not have much more than 2 yr left . It's a horrible disease some days I think this is it it's all over then others I feel fine . What's all that about ?
Sorry to hear that. I'm not sure what's causing the wavering of symptoms. If you are on treatment, usually it is either stable or slowly progressive. Maybe the symptoms have more to do with smoking? Not sure. Probably best to talk to your lung doc about it.
I have Lam..what is the treatment of this disease? Is it curable?
If you have cysts in the lung, they won't go away but there are medications you can take to slow the disease. Best to see a lung doc who has some experience in this area. Thx.
@@ThoracicRadiology i had done vats operation in 2020 and after that i am suffering from breathless problem,, and its related to alaric problem.. I use oxygen. Plz can you tell sir what is the medication?
Hi from you? I also suffering this lam desease pls share your experience what should we do?
I can't afford expensive medical care
Pls tell me how many years l can survive this desease pls reply