A 24-year-Old woman with recurrent pneumothoraces

Autor: Daniel J. Greenberg, Anuraag Sah, Amit Chopra, Nagendra Madisi
Jazyk: angličtina
Rok vydání: 2024
Předmět:
Zdroj: Respiratory Medicine Case Reports, Vol 49, Iss , Pp 102015- (2024)
Druh dokumentu: article
ISSN: 2213-0071
DOI: 10.1016/j.rmcr.2024.102015
Popis: Lymphangioleiomyomatosis (LAM) is an abnormal proliferation of smooth muscle-like cells and may occur sporadically or in association with tuberous sclerosis complex. Patients are typically female, nonsmoking and may have cystic lung disease with pneumothorax. Diagnosis can be made by compatible imaging findings with a history of tuberous sclerosis complex, or in conjunction with vascular endothelial growth factor-D 800 pg/ml or greater, a highly specific finding. Sirolimus is first line treatment for LAM.
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