Low-dose sirolimus in retroperitoneal lymphangioleiomyomas

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 wi...

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Autores principales: Ussavarungsi, Kamonpun, Laroia, Archana T, Burger, Charles D
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Wolters Kluwer - Medknow 2019
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6625232/
https://www.ncbi.nlm.nih.gov/pubmed/31290423
http://dx.doi.org/10.4103/lungindia.lungindia_433_18
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author Ussavarungsi, Kamonpun
Laroia, Archana T
Burger, Charles D
author_facet Ussavarungsi, Kamonpun
Laroia, Archana T
Burger, Charles D
author_sort Ussavarungsi, Kamonpun
collection PubMed
description 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.
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spelling pubmed-66252322019-07-22 Low-dose sirolimus in retroperitoneal lymphangioleiomyomas Ussavarungsi, Kamonpun Laroia, Archana T Burger, Charles D Lung India Case Report 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. Wolters Kluwer - Medknow 2019 /pmc/articles/PMC6625232/ /pubmed/31290423 http://dx.doi.org/10.4103/lungindia.lungindia_433_18 Text en Copyright: © 2019 Indian Chest Society http://creativecommons.org/licenses/by-nc-sa/4.0 This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms.
spellingShingle Case Report
Ussavarungsi, Kamonpun
Laroia, Archana T
Burger, Charles D
Low-dose sirolimus in retroperitoneal lymphangioleiomyomas
title Low-dose sirolimus in retroperitoneal lymphangioleiomyomas
title_full Low-dose sirolimus in retroperitoneal lymphangioleiomyomas
title_fullStr Low-dose sirolimus in retroperitoneal lymphangioleiomyomas
title_full_unstemmed Low-dose sirolimus in retroperitoneal lymphangioleiomyomas
title_short Low-dose sirolimus in retroperitoneal lymphangioleiomyomas
title_sort low-dose sirolimus in retroperitoneal lymphangioleiomyomas
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6625232/
https://www.ncbi.nlm.nih.gov/pubmed/31290423
http://dx.doi.org/10.4103/lungindia.lungindia_433_18
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AT laroiaarchanat lowdosesirolimusinretroperitoneallymphangioleiomyomas
AT burgercharlesd lowdosesirolimusinretroperitoneallymphangioleiomyomas

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