Reviewed By:
Phillip Aguila, MD, MBA (Pulmonology, Critical Care)
Dr. Aguila graduated from West Virginia University School of Medicine. He has trained in Pulmonary and Critical Care Medicine at The University of North Carolina in Chapel Hill and Internal Medicine at Medical College of Pennsylvania/Hahnemann University at Allegheny General Hospital in Pittsburgh Pennsylvania. He has served as Assistant Professor since 2010.
Eisaku Kamakura, MD (Pulmonology)
Dr. Kamakura graduated from the Tokyo Medical and Dental University, School of Dentistry, and the Niigata University School of Medicine. He trained at Yokosuka Kyosai Hospital and held positions in the Respiratory Medicine departments at Yokosuka Kyosai Hospital, Tokyo Medical and Dental University, Ome City General Hospital, and Musashino Red Cross Hospital. In 2021, he became the specially appointed assistant professor at the Department of General Medicine, Niigata University School of Medicine.
Content updated on Sep 20, 2022
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Lymphangioleiomyomatosis (LAM) results from abnormal growth of muscle cells, particularly in the lungs and lymph system. This can lead to the formation of holes or cysts in the lung and blockage or rupture of lymph vessels, causing lymph and fatty acid accumulation in the chest cavity. It is caused by gene mutations that produce proteins which cannot regulate cell growth and movement in the body.
Your doctor may ask these questions to check for this disease:
There is no cure for lymphangioleiomyomatosis, but treatment can help alleviate symptoms, prevent complications, and slow disease progression.
Xu KF, Xu W, Liu S, Yu J, Tian X, Yang Y, Wang ST, Zhang W, Feng R, Zhang T. Lymphangioleiomyomatosis. Semin Respir Crit Care Med. 2020 Apr;41(2):256-268. doi: 10.1055/s-0040-1702195. Epub 2020 Apr 12. PMID: 32279296.
https://www.thieme-connect.de/products/ejournals/abstract/10.1055/s-0040-1702195Sclafani A, VanderLaan P. Lymphangioleiomyomatosis. N Engl J Med. 2018 Jun 7;378(23):2224. doi: 10.1056/NEJMicm1712581. PMID: 29874537.
https://www.nejm.org/doi/10.1056/NEJMicm1712581McCarthy C, Gupta N, Johnson SR, Yu JJ, McCormack FX. Lymphangioleiomyomatosis: pathogenesis, clinical features, diagnosis, and management. Lancet Respir Med. 2021 Nov;9(11):1313-1327. doi: 10.1016/S2213-2600(21)00228-9. Epub 2021 Aug 27. PMID: 34461049.
https://www.thelancet.com/journals/lanres/article/PIIS2213-2600(21)00228-9/fulltextDoubková M, Štefániková M, Čan V, Merta Z, Svoboda M. Lymphangioleiomyomatosis. Klin Onkol. 2019 Fall;32(5):367-374. English. doi: 10.14735/amko2019367. PMID: 31610670.
https://www.linkos.cz/english-summary/klinicka-onkologie-journal/2019-10-15-5-en/lymfangioleiomyomatoza-1/Male, 30s
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Reviewed By:
Phillip Aguila, MD, MBA (Pulmonology, Critical Care)
Dr. Aguila graduated from West Virginia University School of Medicine. He has trained in Pulmonary and Critical Care Medicine at The University of North Carolina in Chapel Hill and Internal Medicine at Medical College of Pennsylvania/Hahnemann University at Allegheny General Hospital in Pittsburgh Pennsylvania. He has served as Assistant Professor since 2010.
Eisaku Kamakura, MD (Pulmonology)
Dr. Kamakura graduated from the Tokyo Medical and Dental University, School of Dentistry, and the Niigata University School of Medicine. He trained at Yokosuka Kyosai Hospital and held positions in the Respiratory Medicine departments at Yokosuka Kyosai Hospital, Tokyo Medical and Dental University, Ome City General Hospital, and Musashino Red Cross Hospital. In 2021, he became the specially appointed assistant professor at the Department of General Medicine, Niigata University School of Medicine.
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