Recent Scientific Articles

JUNE 2015 - Current Publications
Atochina-Vasserman, Elena N., Chang-Jiang Guo, Elena Abramova, Thea N. Golden, Michael Sims, Melane L. James, Michael F. Beers, Andrew J. Gow, and Vera P. Krymskaya. 2015. “Surfactant Dysfunction and Lung Inflammation in the Female Mouse Model of Lymphangioleiomyomatosis.” American Journal of Respiratory Cell and Molecular Biology 53 (1). doi:10.1165/rcmb.2014-0224OC.

Bee, Janet, Rupesh Bhatt, Ian McCafferty, and Simon R. Johnson. 2015. “A 4-Year Prospective Evaluation of Protocols to Improve Clinical Outcomes for Patients with Lymphangioleiomyomatosis in a National Clinical Centre.” Thorax, June. doi:10.1136/thoraxjnl-2015-207171.

Ellender, Claire M., Trevor J. Williams, Julian Gooi, Gregory I. Snell, and Helen M. Whitford. 2015. “Management of Refractory Chylothorax in Pulmonary Lymphangioleiomyomatosis.” Respirology Case Reports 3 (2): 72–74. doi:10.1002/rcr2.105.

Goldberg, Hilary J., Sergio Harari, Vincent Cottin, Ivan O. Rosas, Elizabeth Peters, Shibadas Biswal, Yi Cheng, et al. 2015. “Everolimus for the Treatment of Lymphangioleiomyomatosis: A Phase II Study.” The European Respiratory Journal, June. doi:10.1183/09031936.00210714.

Grzegorek, Irmina, Dido Lenze, Mariusz Chabowski, Dariusz Janczak, Malgorzata Szolkowska, Renata Langfort, Andrzej Szuba, and Piotr Dziegiel. 2015. “Immunohistochemical Evaluation of Pulmonary Lymphangioleiomyomatosis.” Anticancer Research 35 (6): 3353–60.

Grzegorek, Irmina, Ewa Zuba-Surma, Mariusz Chabowski, Dariusz Janczak, Andrzej Szuba, and Piotr Dziegiel. 2015. “Characterization of Cells Cultured from Chylous Effusion from a Patient with Sporadic Lymphangioleiomyomatosis.” Anticancer Research 35 (6): 3341–51.

Gupta, Nishant, Robert Vassallo, Kathryn A. Wikenheiser-Brokamp, and Francis X. McCormack. 2015a. “Diffuse Cystic Lung Disease. Part I.” American Journal of Respiratory and Critical Care Medicine 191 (12): 1354–66. doi:10.1164/rccm.201411-2094CI.
———. 2015b. “Diffuse Cystic Lung Disease. Part II.” American Journal of Respiratory and Critical Care Medicine 192 (1). doi:10.1164/rccm.201411-2096CI.

Priolo, Carmen, Stephane J. H. Ricoult, Damir Khabibullin, Harilaos Filippakis, Jane Yu, Brendan D. Manning, Clary Clish, and Elizabeth P. Henske. 2015. “Tuberous Sclerosis Complex 2 Loss Increases Lysophosphatidylcholine Synthesis in  Lymphangioleiomyomatosis.” American Journal of Respiratory Cell and Molecular Biology 53 (1). doi:10.1165/rcmb.2014-0379RC.

Richards, J. Caleb, David A. Lynch, and Jonathan H. Chung. 2015. “Cystic and Nodular Lung Disease.” Clinics in Chest Medicine 36 (2). doi:10.1016/j.ccm.2015.02.011.

Spalgais, S., D. Gothi, and A. K. Verma. 2015. “Pulmonary Choriostoma in a Case of Tuberous Sclerosis Complex.” Journal of Postgraduate Medicine 61 (3): 193–96. doi:10.4103/0022-3859.159426.

Taveira-DaSilva, Angelo M., Amanda M. Jones, Patricia Julien-Williams, Thomas Shawker, Connie G. Glasgow, Mario Stylianou, and Joel Moss. 2015. “Effect of Fasting on the Size of Lymphangioleiomyomas in Patients with Lymphangioleiomyomatosis.” Chest, June. doi:10.1378/chest.15-0456.

Von Ranke, Felipe Mussi, Glaucia Zanetti, Jorge Luiz Pereira E Silva, Cesar Augusto Araujo Neto, Myrna C. B. Godoy, Carolina A. Souza, Alexandre Dias Mancano, et al. 2015. “Tuberous Sclerosis Complex: State-of-the-Art Review with a Focus on Pulmonary Involvement.” Lung, June. doi:10.1007/s00408-015-9750-6.

Wu, Kai, and Hui-Yu Lu. 2015. “Sporadic Lymphangioleiomyomatosis with Bloody Sputum as an Initial Symptom: A Case Report and Review of the Literature.” Experimental and Therapeutic Medicine 9 (6). doi:10.3892/etm.2015.2397.


MAY 2015 - Current Publications


Cai, Xiong, Gustavo Pacheco-Rodriguez, Mary Haughey, Leigh Samsel, Suowen Xu, Hai-Ping Wu, J. Philip McCoy, Mario Stylianou, Thomas N. Darling, and Joel Moss. 2014a. “Sirolimus Decreases Circulating Lymphangioleiomyomatosis Cells in Patients with Lymphangioleiomyomatosis.” Chest 145 (1): 108–12. doi:10.1378/chest.13-1071.

El-Chemaly, Souheil, and Elizabeth P. Henske. 2015. “The next Breakthrough in LAM Clinical Trials May Be Their Design: Challenges in Design and Execution of Future LAM Clinical Trials.” Expert Review of Respiratory Medicine 9 (2).  doi:10.1586/17476348.2015.1024663.

El-Chemaly, Souheil, Hilary J. Goldberg, and Allan R. Glanville. 2014. “Should Mammalian Target of Rapamycin Inhibitors Be Stopped in Women with Lymphangioleiomyomatosis Awaiting Lung Transplantation?” Expert Review of Respiratory Medicine 8 (6): 657–60. doi:10.1586/17476348.2014.956728.

Fan, Daming, Li Ding, Hui Liu, Jigang Wang, Wenwen Ran, Yujun Li, and Dongliang Lin. 2014. “Effusion Cytology: An Effective Method for the Diagnosis of Pulmonary Lymphangioleiomyomatosis.” Journal of Thoracic Disease 6 (5): E54–57. doi:10.3978/j.issn.2072-1439.2014.03.12.

Gao, Ling, Michael M. Yue, Jennifer Davis, Elisabeth Hyjek, and Lucia Schuger. 2014. “In Pulmonary Lymphangioleiomyomatosis Expression of Progesterone Receptor Is Frequently Higher than that of Estrogen Receptor.” Virchows Archiv : An International Journal of Pathology 464 (4). doi:10.1007/s00428-014-1559-9.

Gupta, R., M. Kitaichi, Y. Inoue, R. Kotloff, and F. X. McCormack. 2014a&b. “Lymphatic Manifestations of Lymphangioleiomyomatosis.” Lymphology 47 (3): 106–17.

Li, Jing, Alfredo Csibi, Sun Yang, Gregory R. Hoffman, Chenggang Li, Erik Zhang, Jane J. Yu, and John Blenis. 2015. “Synthetic Lethality of Combined Glutaminase and Hsp90 Inhibition in mTORC1-Driven Tumor Cells.” Proceedings of the National Academy of Sciences of the United States of America 112 (1): E21–29. doi:10.1073/pnas.1417015112.

McCormack, Francis X., William D. Travis, Thomas V. Colby, Elizabeth P. Henske, and Joel Moss. 2012. “Lymphangioleiomyomatosis: Calling It What It Is: A Low-Grade, Destructive, Metastasizing Neoplasm.” American Journal of Respiratory and Critical Care Medicine 186 (12): 1210–12. doi:10.1164/rccm.201205-0848OE.

Henske, Elizabeth P., and Francis X. McCormack. 2012. “Lymphangioleiomyomatosis - a Wolf in Sheep’s Clothing.” The Journal of Clinical Investigation 122 (11): 3807–16. doi:10.1172/JCI58709.

Priolo, Carmen, Stephane J. H. Ricoult, Damir Khabibullin, Harilaos Filippakis, Jane Yu, Brendan D. Manning, Clary Clish, and Elizabeth P. Henske. 2015. “TSC2 Loss Increases Lysophosphatidylcholine Synthesis in Lymphangioleiomyomatosis.” American Journal of Respiratory Cell and Molecular Biology, March. doi:10.1165/rcmb.2014-0379RC.

Yao, Jianhua, Angelo M. Taveira-DaSilva, Amanda M. Jones, Patricia Julien-Williams, Mario Stylianou, and Joel Moss. 2014. “Sustained Effects of Sirolimus on Lung Function and Cystic Lung Lesions in Lymphangioleiomyomatosis.” American Journal of Respiratory and Critical Care Medicine 190 (11): 1273–82. doi:10.1164/rccm.201405-0918OC.

Yeoh, Zhao W., Vidya Navaratnam, Rupesh Bhatt, Ian McCafferty, Richard B. Hubbard, and Simon R. Johnson. 2014a. “Natural History of Angiomyolipoma in Lymphangioleiomyomatosis: Implications for Screening and Surveillance.” Orphanet Journal of Rare Diseases 9. doi:10.1186/s13023-014-0151-3.

Young, Lisa, Hye-Seung Lee, Yoshikazu Inoue, Joel Moss, Lianne G. Singer, Charlie Strange, Koh Nakata, et al. 2013. “Serum VEGF-D a Concentration as a Biomarker of Lymphangioleiomyomatosis Severity and Treatment Response: A Prospective Analysis of the Multicenter International Lymphangioleiomyomatosis Efficacy of Sirolimus (MILES) Trial.” The Lancet. Respiratory Medicine 1 (6): 445–52. doi:10.1016/S2213-2600(13)70090-0.

Diagnostic Blood Test Can Identify LAM in Some Patients

Click here to read about this important study conducted by Dr. Lisa Young at the University of Cincinnati and the Cincinnati Children's Hospital Medical Center.

Clinical Availability of Serum VEGF-D Test

The VEGF-D test is now available in a clinical lab at Cincinnati Children's. It can be ordered as a send out test from your clinic or hospital, and it is now done in a CAP/CLIA certified lab such that the results can be used for clinical care. Patients and referring physicians should note that although a serum VEGF-D level of 800 pg/ml or greater in the setting of a typical HRCT scan is diagnostic for VEGF-D, a normal VEGF-D level does not exclude the diagnosis of LAM.

Click here to visit the website for the lab at Cincinnati Children's that does the testing. The VEGF-D requisition form is then in an orange box on the right-hand side.

Or, click here to take you directly to the requisition form.


Continuing Education Opportunities

The European Respiratory Society (ERS) is offering several courses as continuing medical education. Please visit the ERS website at click here for a list of courses and a description of each.