A 24-year-Old woman with recurrent pneumothoraces

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 imag...

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Bibliographic Details
Published inRespiratory medicine case reports Vol. 49; p. 102015
Main Authors Greenberg, Daniel J, Sah, Anuraag, Chopra, Amit, Madisi, Nagendra
Format Report
LanguageEnglish
Published 01.01.2024
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Summary: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.
Bibliography:ObjectType-Case Study-2
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SourceType-Reports-1
ObjectType-Report-1
ISSN:2213-0071
2213-0071
DOI:10.1016/j.rmcr.2024.102015