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Low-dose sirolimus in retroperitoneal lymphangioleiomyomas
Journal article   Open access   Peer reviewed

Low-dose sirolimus in retroperitoneal lymphangioleiomyomas

Kamonpun Ussavarungsi, Archana T. Laroia and Charles D. Burger
Lung India, Vol.36(4), pp.349-352
07/01/2019
DOI: 10.4103/lungindia.lungindia_433_18
PMCID: PMC6625232
PMID: 31290423
url
https://doi.org/10.4103/lungindia.lungindia_433_18View
Published (Version of record) Open Access

Abstract

Lymphangioleiomyomatosis (LAM) is a rare disease associated with cystic lung destruction and abdominal tumors, including lymphangioleiomyomas, which frequently occur in the retroperitoneal region. Sirolimus therapy is currently recommended for LAM patients with abnormal or declining lung function with an adjusted dose to maintain a serum trough level of 5-15 ng/mL. We describe a significant reduction of retroperitoneal lymphangioleiomyomas after treatment with low-dose sirolimus therapy (serum trough level <5 ng/mL) in a patient with sporadic LAM.
Life Sciences & Biomedicine Respiratory System Science & Technology

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