Show simple item record

 
dc.contributorVall d'Hebron Barcelona Hospital Campus
dc.contributor.authorRevilla Lopez, Eva Maria
dc.contributor.authorBerastegui Garcia, Cristina
dc.contributor.authorMéndez Garrido, Maria Alejandra
dc.contributor.authorSaez Gimenez, Berta
dc.contributor.authorRuiz De Miguel, Victoria
dc.contributor.authorMonforte Torres, Victor
dc.contributor.authorBravo Masgoret, Carles
dc.contributor.authorRamon Belmonte, Maria Antonia
dc.contributor.authorGómez Olles, Susana
dc.contributor.authorRomán Broto, Antonio
dc.contributor.authorLópez Meseguer, Manuel
dc.date.accessioned2022-01-12T17:26:24Z
dc.date.available2022-01-12T17:26:24Z
dc.date.issued2021-05-13
dc.identifier.citationRevilla-López E, Berastegui C, Méndez A, Sáez-Giménez B, Ruiz de Miguel V, López-Meseguer M, et al. Long-term results of sirolimus treatment in lymphangioleiomyomatosis: a single referral centre experience. Sci Rep. 2021 May 13;11:10171.
dc.identifier.issn2045-2322
dc.identifier.urihttps://hdl.handle.net/11351/6769
dc.descriptionDiagnosis; Drug therapy; Respiratory tract diseases
dc.description.abstractThere 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.language.isoeng
dc.publisherNature Research
dc.relation.ispartofseriesScientific Reports;11
dc.rightsAttribution 4.0 International
dc.rights.urihttp://creativecommons.org/licenses/by/4.0/
dc.sourceScientia
dc.subjectAparell respiratori - Malalties - Tractament
dc.subjectAvaluació de resultats (Assistència sanitària)
dc.subject.meshLymphangioleiomyomatosis
dc.subject.mesh/drug therapy
dc.subject.meshTreatment Outcome
dc.titleLong-term results of sirolimus treatment in lymphangioleiomyomatosis: a single referral centre experience
dc.typeinfo:eu-repo/semantics/article
dc.identifier.doi10.1038/s41598-021-89562-0
dc.subject.decslinfangioleiomiomatosis
dc.subject.decs/farmacoterapia
dc.subject.decsresultado del tratamiento
dc.relation.publishversionhttps://doi.org/10.1038/s41598-021-89562-0
dc.type.versioninfo:eu-repo/semantics/publishedVersion
dc.audienceProfessionals
dc.contributor.organismesInstitut Català de la Salut
dc.contributor.authoraffiliation[Revilla-López E, Berastegui C, Méndez A, Sáez-Giménez B, Ruiz de Miguel V, López-Meseguer M, Ramon MA] Programa de Trasplantament de Pulmó, Servei de Pneumologia, Vall d’Hebron Institut de Recerca (VHIR), Barcelona, Spain. Vall d’Hebron Hospital Universitari, Barcelona, Spain. Departament de Medicina, Universitat Autònoma de Barcelona, Bellaterra, Spain. [Monforte V, Bravo C, Gómez-Ollés S, Roman A] Programa de Trasplantament de Pulmó, Servei de Pneumologia, Vall d’Hebron Institut de Recerca (VHIR), Barcelona, Spain. Vall d’Hebron Hospital Universitari, Barcelona, Spain. Departament de Medicina, Universitat Autònoma de Barcelona, Bellaterra, Spain. CIBER de Enfermedades Respiratorias (CIBERES), Instituto de Salud Carlos III, Madrid, Spain
dc.identifier.pmid33986388
dc.identifier.wos000656941100010
dc.rights.accessrightsinfo:eu-repo/semantics/openAccess


Files in this item

Thumbnail

This item appears in the following Collection(s)

Show simple item record