Lymphangioleiomyomatosis: a clinical update.

  title={Lymphangioleiomyomatosis: a clinical update.},
  author={Francis X McCormack},
  volume={133 2},
Lymphangioleiomyomatosis (LAM) is a rare, cystic lung disease that is associated with mutations in tuberous sclerosis genes, renal angiomyolipomas, lymphatic spread, and remarkable female gender restriction. The clinical course of LAM is characterized by progressive dyspnea on exertion, recurrent pneumothorax, and chylous fluid collections. Lung function declines at approximately twofold to threefold times the rate of healthy subjects, based on an annual drop in FEV1 of 75 to 120 mL in reported… CONTINUE READING


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