Chapter

Lymphangioleiomyomatosis

S.J. Bourke and D.J. Hendrick

in Oxford Textbook of Medicine

Fifth edition

Published on behalf of Oxford University Press

ISBN: 9780199204854
Published online May 2012 | e-ISBN: 9780199570973 | DOI: http://dx.doi.org/10.1093/med/9780199204854.003.181406_update_002

Series: Oxford Textbooks

Lymphangioleiomyomatosis

More Like This

Show all results sharing this subject:

  • Respiratory Medicine and Pulmonology

GO

Show Summary Details

Preview

Lymphangioleiomyomatosis is caused by mutations (usually sporadic, sometimes in tuberous sclerosis) of the TSC1 or TSC2 genes and results in cystic destruction of the lungs, with CT features being sufficiently characteristic to establish the diagnosis in many cases. Two-thirds of patients suffer pneumothoraces. Hormonal therapy with progesterone or tamoxifen is usually given for progressive disease; medical or surgical pleurodesis is advisable; lung transplantation is the main option for advanced disease.

Chapter.  833 words.  Illustrated.

Subjects: Respiratory Medicine and Pulmonology

Full text: subscription required

How to subscribe Recommend to my Librarian

Buy this work at Oxford University Press »

Users without a subscription are not able to see the full content. Please, subscribe or login to access all content.