{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,13]],"date-time":"2026-01-13T02:58:19Z","timestamp":1768273099515,"version":"3.49.0"},"reference-count":64,"publisher":"F1000 Research Ltd","license":[{"start":{"date-parts":[[2018,6,18]],"date-time":"2018-06-18T00:00:00Z","timestamp":1529280000000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Chinese Academy of Medical Sciences (CAMS) Initiative for Innovative Medicine","award":["2017-12M-2-001"],"award-info":[{"award-number":["2017-12M-2-001"]}]},{"name":"Beijing Municipal Science and Technology Project","award":["Z151100003915126"],"award-info":[{"award-number":["Z151100003915126"]}]},{"DOI":"10.13039\/501100001809","name":"National Nature Science Foundation of China","doi-asserted-by":"crossref","award":["81570061"],"award-info":[{"award-number":["81570061"]}],"id":[{"id":"10.13039\/501100001809","id-type":"DOI","asserted-by":"crossref"}]},{"DOI":"10.13039\/501100012166","name":"National Key Research and Development Program of China","doi-asserted-by":"crossref","award":["2016YFC0901502"],"award-info":[{"award-number":["2016YFC0901502"]}],"id":[{"id":"10.13039\/501100012166","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":["f1000research.com"],"crossmark-restriction":false},"short-container-title":["F1000Res"],"abstract":"<ns4:p>Lymphangioleiomyomatosis is a rare disorder that predominantly affects women and is characterized by progressive cystic changes in the lung, leading to gradually worsening shortness of breath and lung function impairment. Pleural complications such as pneumothorax and chylothorax commonly occur in these patients. Lymphangioleiomyomatosis can occur as a form of lung involvement in tuberous sclerosis complex or as a sporadic form (without tuberous sclerosis complex). Etiology in both forms of this disease centers on mutations in the tuberous sclerosis genes. Advances in our understanding of the regulatory role of tuberous sclerosis gene products (hamartin\/tuberin) in the mechanistic target of rapamycin (mTOR) signaling pathway have led to the identification of effective therapy (mTOR inhibitors) for a rare disorder, once considered uniformly fatal. Here, we summarize the evolution of current concepts regarding lymphangioleiomyomatosis with an emphasis on recent advances and unresolved issues.<\/ns4:p>","DOI":"10.12688\/f1000research.14564.1","type":"journal-article","created":{"date-parts":[[2018,6,18]],"date-time":"2018-06-18T15:00:47Z","timestamp":1529334047000},"page":"758","update-policy":"https:\/\/doi.org\/10.12688\/f1000research.crossmark-policy","source":"Crossref","is-referenced-by-count":11,"title":["Recent advances in the management of lymphangioleiomyomatosis"],"prefix":"10.12688","volume":"7","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-7662-531X","authenticated-orcid":false,"given":"Kai-Feng","family":"Xu","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Xinlun","family":"Tian","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9576-2272","authenticated-orcid":false,"given":"Jay H","family":"Ryu","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"2560","published-online":{"date-parts":[[2018,6,18]]},"reference":[{"key":"ref-1","doi-asserted-by":"publisher","first-page":"1210-2","DOI":"10.1164\/rccm.201205-0848OE","article-title":"Lymphangioleiomyomatosis: calling it what it is: a low-grade, destructive, metastasizing neoplasm.","volume":"186","author":"F McCormack","year":"2012","journal-title":"Am J Respir Crit Care Med."},{"key":"ref-2","doi-asserted-by":"publisher","first-page":"316-27","DOI":"10.1007\/s11684-013-0269-z","article-title":"Diffuse cystic lung diseases.","volume":"7","author":"J Ryu","year":"2013","journal-title":"Front Med."},{"key":"ref-3","doi-asserted-by":"publisher","first-page":"14-26","DOI":"10.1183\/09031936.00076209","article-title":"European Respiratory Society guidelines for the diagnosis and management of lymphangioleiomyomatosis.","volume":"35","author":"S Johnson","year":"2010","journal-title":"Eur Respir J."},{"key":"ref-4","doi-asserted-by":"publisher","first-page":"105-11","DOI":"10.1164\/rccm.200409-1298OC","article-title":"The NHLBI lymphangioleiomyomatosis registry: characteristics of 230 patients at enrollment.","volume":"173","author":"J Ryu","year":"2006","journal-title":"Am J Respir Crit Care Med."},{"key":"ref-5","doi-asserted-by":"publisher","first-page":"1595-606","DOI":"10.1056\/NEJMoa1100391","article-title":"Efficacy and safety of sirolimus in lymphangioleiomyomatosis.","volume":"364","author":"F McCormack","year":"2011","journal-title":"N Engl J Med."},{"key":"ref-6","doi-asserted-by":"publisher","first-page":"140-51","DOI":"10.1056\/NEJMoa063564","article-title":"Sirolimus for angiomyolipoma in tuberous sclerosis complex or lymphangioleiomyomatosis.","volume":"358","author":"J Bissler","year":"2008","journal-title":"N Engl J Med."},{"key":"ref-7","doi-asserted-by":"publisher","first-page":"243-54","DOI":"10.1016\/j.pediatrneurol.2013.08.001","article-title":"Tuberous Sclerosis Complex Diagnostic Criteria Update: Recommendations of the 2012 International Tuberous Sclerosis Complex Consensus Conference.","volume":"49","author":"H Northrup","year":"2013","journal-title":"Pediatr Neurol."},{"key":"ref-8","doi-asserted-by":"publisher","first-page":"255-65","DOI":"10.1016\/j.pediatrneurol.2013.08.002","article-title":"Tuberous sclerosis complex surveillance and management: recommendations of the 2012 International Tuberous Sclerosis Complex Consensus Conference.","volume":"49","author":"D Krueger","year":"2013","journal-title":"Pediatr Neurol."},{"key":"ref-9","doi-asserted-by":"publisher","first-page":"1243-60","DOI":"10.1097\/JTO.0000000000000630","article-title":"The 2015 World Health Organization Classification of Lung Tumors: Impact of Genetic, Clinical and Radiologic Advances Since the 2004 Classification.","volume":"10","author":"W Travis","year":"2015","journal-title":"J Thorac Oncol."},{"key":"ref-10","doi-asserted-by":"publisher","first-page":"748-61","DOI":"10.1164\/rccm.201607-1384ST","article-title":"Official American Thoracic Society\/Japanese Respiratory Society Clinical Practice Guidelines: Lymphangioleiomyomatosis Diagnosis and Management.","volume":"194","author":"F McCormack","year":"2016","journal-title":"Am J Respir Crit Care Med."},{"key":"ref-11","doi-asserted-by":"publisher","first-page":"1337-48","DOI":"10.1164\/rccm.201709-1965ST","article-title":"Lymphangioleiomyomatosis Diagnosis and Management: High-Resolution Chest Computed Tomography, Transbronchial Lung Biopsy, and Pleural Disease Management. An Official American Thoracic Society\/Japanese Respiratory Society Clinical Practice Guideline.","volume":"196","author":"N Gupta","year":"2017","journal-title":"Am J Respir Crit Care Med."},{"key":"ref-12","doi-asserted-by":"publisher","first-page":"6085-90","DOI":"10.1073\/pnas.97.11.6085","article-title":"Mutations in the tuberous sclerosis complex gene TSC2 are a cause of sporadic pulmonary lymphangioleiomyomatosis.","volume":"97","author":"T Carsillo","year":"2000","journal-title":"Proc Natl Acad Sci U S A."},{"key":"ref-13","doi-asserted-by":"publisher","first-page":"69-83","DOI":"10.1146\/annurev-med-050715-104245","article-title":"Lymphangioleiomyomatosis: A Monogenic Model of Malignancy.","volume":"68","author":"V Krymskaya","year":"2017","journal-title":"Annu Rev Med."},{"key":"ref-14","doi-asserted-by":"publisher","first-page":"1348-9","DOI":"10.1016\/S0140-6736(03)13044-9","article-title":"Mutation in TSC2 and activation of mammalian target of rapamycin signalling pathway in renal angiomyolipoma.","volume":"361","author":"N El-Hashemite","year":"2003","journal-title":"Lancet."},{"key":"ref-15","doi-asserted-by":"publisher","first-page":"308-10","DOI":"10.1136\/thoraxjnl-2017-211135","article-title":"Rapamycin for lymphangioleiomyomatosis: optimal timing and optimal dosage.","volume":"73","author":"K Xu","year":"2018","journal-title":"Thorax."},{"key":"ref-16","doi-asserted-by":"publisher","first-page":"34","DOI":"10.1186\/s13023-018-0775-9","article-title":"Functional improvements in patients with lymphangioleiomyomatosis after sirolimus: an observational study.","volume":"13","author":"Y Zhan","year":"2018","journal-title":"Orphanet J Rare Dis."},{"key":"ref-17","doi-asserted-by":"publisher","first-page":"4071-81","DOI":"10.1158\/1078-0432.CCR-11-0445","article-title":"Sirolimus therapy for angiomyolipoma in tuberous sclerosis and sporadic lymphangioleiomyomatosis: a phase 2 trial.","volume":"17","author":"D Davies","year":"2011","journal-title":"Clin Cancer Res."},{"key":"ref-18","doi-asserted-by":"publisher","first-page":"66","DOI":"10.1186\/1465-9921-12-66","article-title":"Is sirolimus a therapeutic option for patients with progressive pulmonary lymphangioleiomyomatosis?","volume":"12","author":"C Neurohr","year":"2011","journal-title":"Respir Res."},{"key":"ref-19","doi-asserted-by":"publisher","first-page":"797-805","DOI":"10.7326\/0003-4819-154-12-201106210-00007","article-title":"Changes in lung function and chylous effusions in patients with lymphangioleiomyomatosis treated with sirolimus.","volume":"154","author":"A Taveira-DaSilva","year":"2011","journal-title":"Ann Intern Med."},{"key":"ref-20","doi-asserted-by":"publisher","first-page":"1273-82","DOI":"10.1164\/rccm.201405-0918OC","article-title":"Sustained effects of sirolimus on lung function and cystic lung lesions in lymphangioleiomyomatosis.","volume":"190","author":"J Yao","year":"2014","journal-title":"Am J Respir Crit Care Med."},{"key":"ref-21","doi-asserted-by":"publisher","first-page":"1912-22","DOI":"10.1513\/AnnalsATS.201605-335OC","article-title":"Efficacy and Safety of Long-Term Sirolimus Therapy for Asian Patients with Lymphangioleiomyomatosis.","volume":"13","author":"T Takada","year":"2016","journal-title":"Ann Am Thorac Soc."},{"key":"ref-22","doi-asserted-by":"publisher","first-page":"124-32","DOI":"10.1016\/j.chest.2017.05.012","article-title":"Long-Term Effect of Sirolimus on Serum Vascular Endothelial Growth Factor D Levels in Patients With Lymphangioleiomyomatosis.","volume":"153","author":"A Taveira-DaSilva","year":"2018","journal-title":"Chest."},{"key":"ref-23","doi-asserted-by":"publisher","first-page":"369-75","DOI":"10.1136\/thoraxjnl-2017-210872","article-title":"Lung function response and side effects to rapamycin for lymphangioleiomyomatosis: a prospective national cohort study.","volume":"73","author":"J Bee","year":"2018","journal-title":"Thorax."},{"key":"ref-24","doi-asserted-by":"publisher","first-page":"457-67","DOI":"10.1055\/s-0036-1580690","article-title":"Diffuse Cystic Lung Diseases: Diagnostic Considerations.","volume":"37","author":"K Xu","year":"2016","journal-title":"Semin Respir Crit Care Med."},{"key":"ref-25","doi-asserted-by":"publisher","first-page":"175-83","DOI":"10.1016\/j.resinv.2013.03.002","article-title":"Efficacy and safety of low-dose sirolimus for treatment of lymphangioleiomyomatosis.","volume":"51","author":"K Ando","year":"2013","journal-title":"Respir Investig."},{"key":"ref-26","doi-asserted-by":"publisher","first-page":"691-700","DOI":"10.2147\/TCRM.S50784","article-title":"Lymphangioleiomyomatosis: differential diagnosis and optimal management.","volume":"10","author":"K Xu","year":"2014","journal-title":"Ther Clin Risk Manag."},{"key":"ref-27","doi-asserted-by":"publisher","first-page":"1302-10","DOI":"10.1016\/j.chest.2017.01.033","article-title":"Sirolimus and Autophagy Inhibition in Lymphangioleiomyomatosis: Results of a Phase I Clinical Trial.","volume":"151","author":"S El-Chemaly","year":"2017","journal-title":"Chest."},{"key":"ref-28","doi-asserted-by":"publisher","first-page":"919-28","DOI":"10.1513\/AnnalsATS.201610-824OC","article-title":"A Phase II Clinical Trial of an Aromatase Inhibitor for Postmenopausal Women with Lymphangioleiomyomatosis.","volume":"14","author":"C Lu","year":"2017","journal-title":"Ann Am Thorac Soc."},{"key":"ref-29","doi-asserted-by":"publisher","first-page":"e13-64","DOI":"10.1164\/rccm.201309-1634ST","article-title":"An official American Thoracic Society\/European Respiratory Society statement: key concepts and advances in pulmonary rehabilitation.","volume":"188","author":"M Spruit","year":"2013","journal-title":"Am J Respir Crit Care Med."},{"key":"ref-30","doi-asserted-by":"publisher","first-page":"1452-60","DOI":"10.1183\/13993003.01683-2015","article-title":"Pulmonary rehabilitation in lymphangioleiomyomatosis: a controlled clinical trial.","volume":"47","author":"M Araujo","year":"2016","journal-title":"Eur Respir J."},{"key":"ref-31","doi-asserted-by":"publisher","first-page":"699-705","DOI":"10.1007\/s00408-017-0045-y","article-title":"Experience of Lung Transplantation in Patients with Lymphangioleiomyomatosis at a Brazilian Reference Centre.","volume":"195","author":"B Baldi","year":"2017","journal-title":"Lung."},{"key":"ref-32","doi-asserted-by":"publisher","first-page":"e0146749","DOI":"10.1371\/journal.pone.0146749","article-title":"Lung Transplantation for Lymphangioleiomyomatosis in Japan.","volume":"11","author":"K Ando","year":"2016","journal-title":"PLoS One."},{"key":"ref-33","doi-asserted-by":"publisher","first-page":"1354-9","DOI":"10.1016\/j.rmed.2015.08.014","article-title":"Mayo clinic experience of lung transplantation in pulmonary lymphangioleiomyomatosis.","volume":"109","author":"K Ussavarungsi","year":"2015","journal-title":"Respir Med."},{"key":"ref-34","doi-asserted-by":"publisher","first-page":"1977-82","DOI":"10.1016\/j.transproceed.2015.04.103","article-title":"Lung Transplantation for Lymphangioleiomyomatosis in a Single Japanese Institute, With a Focus on Late-onset Complications.","volume":"47","author":"T Nakagiri","year":"2015","journal-title":"Transplant Proc."},{"key":"ref-35","doi-asserted-by":"publisher","first-page":"1-7","DOI":"10.1016\/j.healun.2008.09.014","article-title":"Lung transplantation for lymphangioleiomyomatosis: the European experience.","volume":"28","author":"C Benden","year":"2009","journal-title":"J Heart Lung Transplant."},{"key":"ref-36","doi-asserted-by":"publisher","first-page":"1247-53","DOI":"10.1016\/j.healun.2004.09.013","article-title":"The US experience with lung transplantation for pulmonary lymphangioleiomyomatosis.","volume":"24","author":"J Kpodonu","year":"2005","journal-title":"J Heart Lung Transplant."},{"key":"ref-37","doi-asserted-by":"publisher","first-page":"1-15","DOI":"10.1016\/j.healun.2014.06.014","article-title":"A consensus document for the selection of lung transplant candidates: 2014--an update from the Pulmonary Transplantation Council of the International Society for Heart and Lung Transplantation.","volume":"34","author":"D Weill","year":"2015","journal-title":"J Heart Lung Transplant."},{"key":"ref-38","doi-asserted-by":"publisher","first-page":"126-33","DOI":"10.1016\/j.trre.2014.03.002","article-title":"Strategies for the management of adverse events associated with mTOR inhibitors.","volume":"28","author":"B Kaplan","year":"2014","journal-title":"Transplant Rev (Orlando)."},{"key":"ref-39","doi-asserted-by":"publisher","first-page":"657-60","DOI":"10.1586\/17476348.2014.956728","article-title":"Should mammalian target of rapamycin inhibitors be stopped in women with lymphangioleiomyomatosis awaiting lung transplantation?","volume":"8","author":"S El-Chemaly","year":"2014","journal-title":"Expert Rev Respir Med."},{"key":"ref-40","doi-asserted-by":"publisher","first-page":"547-61","DOI":"10.1097\/TP.0b013e3182551021","article-title":"Wound healing complications and the use of mammalian target of rapamycin inhibitors in kidney transplantation: a critical review of the literature.","volume":"94","author":"B Nashan","year":"2012","journal-title":"Transplantation."},{"key":"ref-41","doi-asserted-by":"publisher","first-page":"1274-81","DOI":"10.1378\/chest.129.5.1274","article-title":"Management of pneumothorax in lymphangioleiomyomatosis: effects on recurrence and lung transplantation complications.","volume":"129","author":"K Almoosa","year":"2006","journal-title":"Chest."},{"key":"ref-42","doi-asserted-by":"publisher","first-page":"e0163637","DOI":"10.1371\/journal.pone.0163637","article-title":"A Total Pleural Covering for Lymphangioleiomyomatosis Prevents Pneumothorax Recurrence.","volume":"11","author":"M Kurihara","year":"2016","journal-title":"PLoS One."},{"key":"ref-43","doi-asserted-by":"publisher","first-page":"623-7","DOI":"10.1378\/chest.123.2.623","article-title":"Chylothorax in lymphangioleiomyomatosis.","volume":"123","author":"J Ryu","year":"2003","journal-title":"Chest."},{"key":"ref-44","doi-asserted-by":"publisher","first-page":"1521-6","DOI":"10.1016\/j.rmed.2010.05.003","article-title":"Clinical analysis of patients with pulmonary lymphangioleiomyomatosis (PLAM) in mainland China.","volume":"104","author":"L Ye","year":"2010","journal-title":"Respir Med."},{"key":"ref-45","doi-asserted-by":"publisher","first-page":"9-19","DOI":"10.1089\/lrb.2009.0024","article-title":"The natural history of lymphangioleiomyomatosis: markers of severity, rate of progression and prognosis.","volume":"8","author":"A Taveira-DaSilva","year":"2010","journal-title":"Lymphat Res Biol."},{"key":"ref-46","doi-asserted-by":"publisher","first-page":"1342-8","DOI":"10.1177\/0284185116688381","article-title":"Computed tomography lymphangiography findings in 27 cases of lymphangioleiomyomatosis.","volume":"58","author":"C Zhang","year":"2017","journal-title":"Acta Radiol."},{"key":"ref-47","doi-asserted-by":"publisher","first-page":"151-4","DOI":"10.1080\/140174301750164934","article-title":"Bilateral chylothorax caused by pleuropulmonary lymphangiomyomatosis: a challenging problem in thoracic surgery.","volume":"35","author":"M Kaptanoglu","year":"2001","journal-title":"Scand Cardiovasc J."},{"key":"ref-48","doi-asserted-by":"publisher","first-page":"E11-4","DOI":"10.3978\/j.issn.2072-1439.2014.01.15","article-title":"Surgical treatment of chylothorax caused by lymphangioleiomyomatosis.","volume":"6","author":"M Liu","year":"2014","journal-title":"J Thorac Dis."},{"key":"ref-49","doi-asserted-by":"publisher","first-page":"921-4","DOI":"10.1016\/j.healun.2008.05.012","article-title":"Sirolimus amelioration of clinical symptoms of recurrent lymphangioleiomyomatosis after living-donor lobar lung transplantation.","volume":"27","author":"R Sugimoto","year":"2008","journal-title":"J Heart Lung Transplant."},{"key":"ref-50","doi-asserted-by":"publisher","first-page":"e7-8","DOI":"10.1016\/j.athoracsur.2008.07.062","article-title":"Sirolimus ameliorated post lung transplant chylothorax in lymphangioleiomyomatosis.","volume":"86","author":"T Ohara","year":"2008","journal-title":"Ann Thorac Surg."},{"key":"ref-51","doi-asserted-by":"publisher","first-page":"408-9","DOI":"10.1513\/AnnalsATS.201212-125OC","article-title":"Efficacy of sirolimus therapy for chylous effusions in lymphangioleiomyomatosis.","volume":"10","author":"P Barrera","year":"2013","journal-title":"Ann Am Thorac Soc."},{"key":"ref-52","doi-asserted-by":"publisher","first-page":"256-64","DOI":"10.1159\/000444264","article-title":"Characteristics of Patients with Lymphangioleiomyomatosis and Pleural Effusion: A Systematic Review.","volume":"91","author":"A Lama","year":"2016","journal-title":"Respiration."},{"key":"ref-53","doi-asserted-by":"publisher","first-page":"817-24","DOI":"10.1016\/S0140-6736(12)61767-X","article-title":"Everolimus for angiomyolipoma associated with tuberous sclerosis complex or sporadic lymphangioleiomyomatosis (EXIST-2): a multicentre, randomised, double-blind, placebo-controlled trial.","volume":"381","author":"J Bissler","year":"2013","journal-title":"Lancet."},{"key":"ref-54","doi-asserted-by":"publisher","first-page":"e0180939","DOI":"10.1371\/journal.pone.0180939","article-title":"Everolimus long-term use in patients with tuberous sclerosis complex: Four-year update of the EXIST-2 study.","volume":"12","author":"J Bissler","year":"2017","journal-title":"PLoS One."},{"key":"ref-55","doi-asserted-by":"publisher","first-page":"1573-8","DOI":"10.1378\/chest.07-1205","article-title":"Pulmonary artery pressure in lymphangioleiomyomatosis: an echocardiographic study.","volume":"132","author":"A Taveira-DaSilva","year":"2007","journal-title":"Chest."},{"key":"ref-56","doi-asserted-by":"publisher","first-page":"630-40","DOI":"10.1183\/09031936.00093111","article-title":"Pulmonary hypertension in lymphangioleiomyomatosis: characteristics in 20 patients.","volume":"40","author":"V Cottin","year":"2012","journal-title":"Eur Respir J."},{"key":"ref-57","doi-asserted-by":"publisher","first-page":"74","DOI":"10.1186\/s13023-017-0626-0","article-title":"Pulmonary hypertension in lymphangioleiomyomatosis: prevalence, severity and the role of carbon monoxide diffusion capacity as a screening method.","volume":"12","author":"C Freitas","year":"2017","journal-title":"Orphanet J Rare Dis."},{"key":"ref-58","doi-asserted-by":"publisher","DOI":"10.1007\/s11684-018-0634-z","article-title":"Clinical characteristics in lymphangioleiomyomatosis-related pulmonary hypertension: an observation on 50 patients.","author":"X Wu","year":"2018","journal-title":"Front Med."},{"key":"ref-59","doi-asserted-by":"publisher","first-page":"176-80","DOI":"10.1136\/thx.2006.058537","article-title":"Air travel in women with lymphangioleiomyomatosis.","volume":"62","author":"S Pollock-BarZiv","year":"2007","journal-title":"Thorax."},{"key":"ref-60","doi-asserted-by":"publisher","first-page":"665-70","DOI":"10.1378\/chest.08-3034","article-title":"Pneumothorax after air travel in lymphangioleiomyomatosis, idiopathic pulmonary fibrosis, and sarcoidosis.","volume":"136","author":"A Taveira-DaSilva","year":"2009","journal-title":"Chest."},{"key":"ref-61","doi-asserted-by":"publisher","first-page":"688-94","DOI":"10.1378\/chest.13-2363","article-title":"Air travel and pneumothorax.","volume":"145","author":"X Hu","year":"2014","journal-title":"Chest."},{"key":"ref-62","doi-asserted-by":"publisher","first-page":"1052-7","DOI":"10.1136\/thorax.55.12.1052","article-title":"Clinical experience of lymphangioleiomyomatosis in the UK.","volume":"55","author":"S Johnson","year":"2000","journal-title":"Thorax."},{"key":"ref-63","doi-asserted-by":"publisher","first-page":"766-72","DOI":"10.1016\/j.rmed.2008.11.007","article-title":"Pregnancy experiences among women with lymphangioleiomyomatosis.","volume":"103","author":"M Cohen","year":"2009","journal-title":"Respir Med."},{"key":"ref-64","doi-asserted-by":"publisher","first-page":"3807-16","DOI":"10.1172\/JCI58709","article-title":"Lymphangioleiomyomatosis - a wolf in sheep's clothing.","volume":"122","author":"E Henske","year":"2012","journal-title":"J Clin Invest."}],"container-title":["F1000Research"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/f1000research.com\/articles\/7-758\/v1\/xml","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/f1000research.com\/articles\/7-758\/v1\/pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/f1000research.com\/articles\/7-758\/v1\/iparadigms","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2020,5,19]],"date-time":"2020-05-19T17:47:35Z","timestamp":1589910455000},"score":1,"resource":{"primary":{"URL":"https:\/\/f1000research.com\/articles\/7-758\/v1"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2018,6,18]]},"references-count":64,"URL":"https:\/\/doi.org\/10.12688\/f1000research.14564.1","relation":{"has-review":[{"id-type":"doi","id":"10.5256\/f1000research.15851.r34947","asserted-by":"subject"},{"id-type":"doi","id":"10.5256\/f1000research.15851.r34949","asserted-by":"subject"},{"id-type":"doi","id":"10.5256\/f1000research.15851.r34948","asserted-by":"subject"},{"id-type":"doi","id":"10.5256\/f1000research.15851.r34950","asserted-by":"subject"}]},"ISSN":["2046-1402"],"issn-type":[{"value":"2046-1402","type":"electronic"}],"subject":[],"published":{"date-parts":[[2018,6,18]]},"assertion":[{"value":"Indexed","URL":"https:\/\/f1000research.com\/articles\/7-758\/v1#article-reports","order":0,"name":"referee-status","label":"Referee status","group":{"name":"current-referee-status","label":"Current Referee Status"}},{"value":"10.5256\/f1000research.15851.r34947, Srihari Veeraraghavan, Division of Pulmonary, Allergy and Critical Care, Emory University School of Medicine, Atlanta, Georgia, USA, 18 Jun 2018, version 1, indexed","URL":"https:\/\/f1000research.com\/articles\/7-758\/v1#referee-response-34947","order":0,"name":"referee-response-34947","label":"Referee Report","group":{"name":"article-reports","label":"Article Reports"}},{"value":"10.5256\/f1000research.15851.r34950, Adrian Shifren, Department of Internal Medicine, Washington University School of Medicine, St. Louis, Missouri, USA, 18 Jun 2018, version 1, indexed","URL":"https:\/\/f1000research.com\/articles\/7-758\/v1#referee-response-34950","order":1,"name":"referee-response-34950","label":"Referee Report","group":{"name":"article-reports","label":"Article Reports"}},{"value":"10.5256\/f1000research.15851.r34948, David Neal Franz, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA, 18 Jun 2018, version 1, indexed","URL":"https:\/\/f1000research.com\/articles\/7-758\/v1#referee-response-34948","order":2,"name":"referee-response-34948","label":"Referee Report","group":{"name":"article-reports","label":"Article Reports"}},{"value":"10.5256\/f1000research.15851.r34949, Nabeel Hamzeh, Department of Internal Medicine, University of Iowa, Iowa City, USA, 18 Jun 2018, version 1, indexed","URL":"https:\/\/f1000research.com\/articles\/7-758\/v1#referee-response-34949","order":3,"name":"referee-response-34949","label":"Referee Report","group":{"name":"article-reports","label":"Article Reports"}},{"value":"The work of K-FX and XT was supported by the National Nature Science Foundation of China (81570061), the National Key Research and Development Program of China (2016YFC0901502), the Beijing Municipal Science and Technology Project (Z151100003915126), and the Chinese Academy of Medical Sciences (CAMS) Initiative for Innovative Medicine (2017-12M-2-001).","order":4,"name":"grant-information","label":"Grant Information"},{"value":"This is an open access article distributed under the terms of the Creative Commons Attribution Licence, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.","order":0,"name":"copyright-info","label":"Copyright"}]}}