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The primary manifestations in the lung are lymphangioleiomyomatosis (LAM) and multifocal micronodular pneumocyte hyperplasia (MMPH). LAM affects almost exclusively women, and causes cystic lung destruction, pneumothorax, and chylous pleural effusions. LAM can lead to dyspnea, oxygen dependence, and respiratory failure, with more rapid disease progression during the premenopausal years. In contrast, MMPH affects men and women equally, causing small nodular pulmonary deposits of type II pneumocytes that rarely progress to symptomatic disease. Here, we review the clinical features and pathogenesis of LAM and MMPH.<\/jats:p>","DOI":"10.1002\/ajmg.c.31638","type":"journal-article","created":{"date-parts":[[2018,7,29]],"date-time":"2018-07-29T07:26:55Z","timestamp":1532849215000},"page":"326-337","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":71,"title":["Pulmonary manifestations in tuberous sclerosis complex"],"prefix":"10.1002","volume":"178","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9112-1315","authenticated-orcid":false,"given":"Nishant","family":"Gupta","sequence":"first","affiliation":[{"name":"Division of Pulmonary, Critical Care and Sleep Medicine University of Cincinnati  Cincinnati Ohio"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elizabeth P.","family":"Henske","sequence":"additional","affiliation":[{"name":"Department of Medicine Brigham and Women's Hospital and Harvard Medical School  Boston 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