| Literature DB >> 27514586 |
Simon R Johnson1, Angelo M Taveira-DaSilva2, Joel Moss3.
Abstract
Lymphangioleiomyomatosis is a rare multisystem disease predominantly affecting women that can occur sporadically or in association with tuberous sclerosis. Lung cysts progressively replace the lung parenchyma, which leads to dyspnea, recurrent pneumothorax, and in some cases respiratory failure. Patients may also have lymphatic disease in the thorax, abdomen, and pelvis, and renal angiomyolipomas. Treatment includes supportive care, bronchodilators, and for those with progressive disease, mammalian target of rapamycin (mTOR) inhibitors.Entities:
Keywords: Angiomyolipoma; Cystic lung disease; Lymphatic disease; Pneumothorax; mTOR inhibitor
Mesh:
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Year: 2016 PMID: 27514586 DOI: 10.1016/j.ccm.2016.04.002
Source DB: PubMed Journal: Clin Chest Med ISSN: 0272-5231 Impact factor: 2.878