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Spontaneous Pneumothorax in a Young Female With Lymphangioleiomyomatosis

Lymphangioleiomyomatosis (LAM) is a rare disorder of abnormal proliferation of smooth muscle-like cells which results in the formation of thin-walled cysts and progressive lung destruction. It commonly presents with progressive dyspnea that is often associated with a history of pneumothorax or chylo...

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Detalles Bibliográficos
Autores principales: Ukponmwan, Osato, Gorantla, Asher, Patel, Krunal H, Gabutan, Elmer, Zhonghua, Li, McFarlane, Samy I
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Cureus 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10581502/
https://www.ncbi.nlm.nih.gov/pubmed/37854755
http://dx.doi.org/10.7759/cureus.45413
Descripción
Sumario:Lymphangioleiomyomatosis (LAM) is a rare disorder of abnormal proliferation of smooth muscle-like cells which results in the formation of thin-walled cysts and progressive lung destruction. It commonly presents with progressive dyspnea that is often associated with a history of pneumothorax or chylothorax particularly among females of reproductive age. In this report, we present a case of hydropneumothorax as the initial presentation of LAM in a 33-year-old woman, a rather rare presentation. We also discuss the pathogenetic mechanisms, the diagnosis, and treatment strategies using mTOR inhibitors like sirolimus.