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http://hdl.handle.net/2445/178631
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DC Field | Value | Language |
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dc.contributor.author | Revilla López, Eva | - |
dc.contributor.author | Berastegui, Cristina | - |
dc.contributor.author | Méndez, Alejandra | - |
dc.contributor.author | Sáez Giménez, Berta | - |
dc.contributor.author | Ruiz de Miguel, Victoria | - |
dc.contributor.author | López Meseguer, Manuel | - |
dc.contributor.author | Monforte, Victor | - |
dc.contributor.author | Bravo, Carlos | - |
dc.contributor.author | Pujana Genestar, M. Ángel | - |
dc.contributor.author | Ramon, Maria Antonia | - |
dc.contributor.author | Gómez Ollés, Susana | - |
dc.contributor.author | Roman, Antonio | - |
dc.contributor.author | Vall d’Hebron Multidisciplinary Cystic Lung Disease Group | - |
dc.date.accessioned | 2021-06-25T10:43:25Z | - |
dc.date.available | 2021-06-25T10:43:25Z | - |
dc.date.issued | 2021-05-13 | - |
dc.identifier.uri | http://hdl.handle.net/2445/178631 | - |
dc.description.abstract | There are few published data on long-term treatment with sirolimus in lymphangioleiomyomatosis (LAM). The objective of this study was to describe the long-term effect of sirolimus in a series of LAM patients followed up in a referral centre, focusing on pulmonary function. We retrospectively reviewed a series of 48 patients with LAM diagnosed, followed up and treated with sirolimus in a single centre. Response to sirolimus was evaluated at 1 and 5 years. A negative sirolimus response was defined as an FEV1 decline greater than - 75 ml/year. A mixed-effects model was used to estimate the longitudinal changes in FEV1 (average slope), both as absolute (ml/year) and as predicted values (%predicted/year). From a total of 48 patients, 9 patients underwent lung transplantation and 4 died during the study. Mean (95% CI) FEV1 slope over 5 years was - 0.14 (- 26.13 to 25.85) ml/year in the whole LAM group, 42.55 (14.87 to 70.22) ml/year in the responder group, - 54.00 (- 71.60 to - 36.39) ml/year in the partial responder group and - 84.19 (- 113.5 to - 54.0) ml/year in the non-responder group. After 5 years of sirolimus treatment 59% had a positive response, 30% had a partial response and 11% had a negative response. Our study found that sirolimus treatment had a positive long-term effect on most LAM patients. | - |
dc.format.extent | 9 p. | - |
dc.format.mimetype | application/pdf | - |
dc.language.iso | eng | - |
dc.publisher | Springer Nature | - |
dc.relation.isformatof | Reproducció del document publicat a: https://doi.org/10.1038/s41598-021-89562-0 | - |
dc.relation.ispartof | Scientific Reports, 2021, vol. 11 | - |
dc.relation.uri | https://doi.org/10.1038/s41598-021-89562-0 | - |
dc.rights | cc by (c) Revilla López et al., 2021 | - |
dc.rights.uri | http://creativecommons.org/licenses/by/3.0/es/ | * |
dc.source | Articles publicats en revistes (Institut d'lnvestigació Biomèdica de Bellvitge (IDIBELL)) | - |
dc.subject.classification | Malalties del pulmó | - |
dc.subject.other | Pulmonary diseases | - |
dc.title | Long-term results of sirolimus treatment in lymphangioleiomyomatosis: a single referral centre experience | - |
dc.type | info:eu-repo/semantics/article | - |
dc.type | info:eu-repo/semantics/publishedVersion | - |
dc.date.updated | 2021-06-25T09:36:52Z | - |
dc.rights.accessRights | info:eu-repo/semantics/openAccess | - |
dc.identifier.pmid | 33986388 | - |
Appears in Collections: | Articles publicats en revistes (Institut d'lnvestigació Biomèdica de Bellvitge (IDIBELL)) |
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File | Description | Size | Format | |
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s41598-021-89562-0.pdf | 1.22 MB | Adobe PDF | View/Open |
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