To adverse, subscribe a colleague or to unsubscribe please contact : Secretariat, Asian Pacific Society of Respirology, 2F, UK's Bldg. 2-29-3 Hongo, Bunkyo-ku, Tokyo 113-0033 Japan. Email: [email protected]Inside this issue: Lymphangioleiomyomatosis Efficacy and safety of long-term sirolimus therapy in Asian paents with lymphangioleiomyomatosis. 2 Official American Thoracic Society and Japanese Respiratory Society Clinical Pracce Guideline: Lym- phangioleiomyomatosis Diagnosis and Management. 2 A total pleural covering for lymphangioleiomyomatosis prevents pneumothorax recurrence. 3 Incidence of Pneumothorax in Paents With Lymphangioleiomyomatosis Undergoing Pulmonary Funcon and Exercise Tesng. 3 Isolaon of individual cellular components from lung ssues of paents with lymphangioleiomy- omatosis. 4 Bronchial involvement in advanced stage lymphangioleiomyomatosis: histopathologic and molecu- lar analyses. 4 Pulmonary rehabilitaon in lymphangioleiomyomatosis: a controlled clinical trial. 4 Wild type mesenchymal cells contribute to the lung pathology of lymphangioleiomyomatosis. 5 Genec heterogeneity of circulang cells from paents with lymphangioleiomyomatosis with and without lung transplantaon. 5 Sustained effects of sirolimus on lung funcon and cysc lung lesions in lymphangioleiomyomatosis. 6 Sirolimus decreases circulang lymphangioleiomyomatosis cells in paents with lymphangioleiomy- omatosis. 6 Exonic mutaons of TSC2/TSC1 are common but not seen in all sporadic pulmonary lymphangioleio- myomatosis. 6 APSR EDUCATION PUBLICATION Newsletter Date: September 2016 Volume 8, Issue 9 APSR RESPIRATORY UPDATES Arcles selected and commented on by: Kuniaki Seyama, M.D., Ph.D. Associate Professor. Division of Respiratory Medicine, Juntendo University Faculty of Medicine and Graduate School of Medicine. To- kyo, Japan. Email: [email protected]Keep informed about the latest news and published articles in Respirology and Respirology Case Reports with direct links to the articles! Sign up to follow us on Twitter today!
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To advertise, subscribe a colleague or to unsubscribe please contact : Secretariat, Asian Pacific Society of Respirology, 2F, UK's Bldg. 2-29-3 Hongo, Bunkyo-ku, Tokyo 113-0033 Japan. Email: [email protected]
Inside this issue: Lymphangioleiomyomatosis
Efficacy and safety of long-term sirolimus therapy in Asian patients with lymphangioleiomyomatosis. 2
Official American Thoracic Society and Japanese Respiratory Society Clinical Practice Guideline: Lym-phangioleiomyomatosis Diagnosis and Management.
2
A total pleural covering for lymphangioleiomyomatosis prevents pneumothorax recurrence. 3
Incidence of Pneumothorax in Patients With Lymphangioleiomyomatosis Undergoing Pulmonary Function and Exercise Testing.
3
Isolation of individual cellular components from lung tissues of patients with lymphangioleiomy-omatosis.
4
Bronchial involvement in advanced stage lymphangioleiomyomatosis: histopathologic and molecu-lar analyses. 4
Pulmonary rehabilitation in lymphangioleiomyomatosis: a controlled clinical trial. 4
Wild type mesenchymal cells contribute to the lung pathology of lymphangioleiomyomatosis. 5
Genetic heterogeneity of circulating cells from patients with lymphangioleiomyomatosis with and without lung transplantation.
5
Sustained effects of sirolimus on lung function and cystic lung lesions in lymphangioleiomyomatosis. 6
Sirolimus decreases circulating lymphangioleiomyomatosis cells in patients with lymphangioleiomy-omatosis.
6
Exonic mutations of TSC2/TSC1 are common but not seen in all sporadic pulmonary lymphangioleio-myomatosis.
6
APSR EDUCATION PUBLICATION
Newsletter Date: September 2016 Volume 8, Issue 9
APSR RESPIRATORY UPDATES
Articles selected and commented on by: Kuniaki Seyama, M.D., Ph.D. Associate Professor. Division of Respiratory Medicine, Juntendo University Faculty of Medicine and Graduate School of Medicine. To-kyo, Japan. Email: [email protected]
Keep informed about the latest news and
published articles in Respirology and
Respirology Case Reports with direct links to the articles! Sign up to follow us on
To advertise, subscribe a colleague or to unsubscribe please contact : Secretariat, Asian Pacific Society of Respirology, 2F, UK's Bldg. 2-29-3 Hongo, Bunkyo-ku, Tokyo 113-0033 Japan. Email: [email protected]
Page 2 APSR RESPIRATORY UPDATES
Takada T et al. Annals ATS. DOI: 10.1513/AnnalsATS.201605-335OC
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Page 3 APSR RESPIRATORY UPDATES
Kurihara M et al. PLoS One. 2016 Sep 22;11(9):e0163637. doi: 10.1371/journal.pone.0163637. eCollection 2016.
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Page 4 APSR RESPIRATORY UPDATES
Ando K et al. Isolation Am J Physiol Lung Cell Mol Physiol. 2016;310:L899-908.
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Page 6 APSR RESPIRATORY UPDATES
Yao J et al. Am J Respir Crit Care Med. 2014;190:1273-82.
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Disclaimer: This publication is not intended as a replacement for regular medical education. The comments are an interpretation of the published study and reflect the opinion of the writer rather than those of the research group or scientific journal. It is suggested readers review the full trial data before forming a final conclusion on its merits. Privacy Policy: The APSR Secretariat will record your email details on a secure database and will not release it to anyone without your prior approval. The APSR and you have the right to inspect, update or delete your details at any time.
Asian Pacific Society of Respirology Page 7
APSR Respiratory Updates is an initiative of the APSR Education Committee
Articles selected and commented on by Kuniaki Seyama, M.D., Ph.D. Associate Professor. Divi-sion of Respiratory Medicine, Juntendo University Faculty of Medicine and Graduate School of Medicine. Tokyo, Japan
Editor in chief: Dr David CL Lam, Department of Medicine, University of Hong Kong, Hong Kong, China
Compiled by Dr Christel Norman, Respirology Editorial Office, Perth, Australia
samples showed TSC2 two-hit inactivation. Interestingly, two patients with sporadic LAM showed no TSC1/
TSC2 mutation. The important considerations as to the pathogenesis of LAM raised here are; 1) TSC2 mutations
may not be the primary driver of LAM development but occur in a subset of LAM cells after other unknown
initiating events: 2) TSC2-mutant cells may recruit stromal cells to adopt a LAM cell phenotype: and 3) the ex-
istence of the two individuals without TSC1 or TSC2 mutations suggests that alternative genetic mechanisms