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Lymphangioleiomyomatosis 

Lymphangioleiomyomatosis

Chapter:
Lymphangioleiomyomatosis
Author(s):

S.J. Bourke

and D.J. Hendrick

DOI:
10.1093/med/9780199204854.003.181406_update_003

July 30, 2015: This chapter has been re-evaluated and remains up-to-date. No changes have been necessary.

Update:

Notes on treatment with sirolimus (rapamycin).

Updated on 29 May 2014. The previous version of this content can be found here.
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date: 23 June 2017

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....

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