Benefits of pulmonary rehabilitation in patients with advanced lymphangioleiomyomatosis (LAM) compared with COPD - a retrospective analysis.
Orphanet J Rare Dis
; 15(1): 255, 2020 09 22.
Article
em En
| MEDLINE
| ID: mdl-32962746
ABSTRACT
Lymphangioleiomyomatosis (LAM) is a rare and progressive cystic lung disease with limited therapeutic options. We retrospectively analyzed the effects of a comprehensive 4-week inpatient pulmonary rehabilitation (PR) program in 58 patients with advanced LAM (FEV1 45 ± 34%predicted, 6-min walk distance (6MWD) 338 ± 167 m). Exercise performance (6MWD + 49 ± 50 m; p < 0.001) and quality of life (SF-36 physical component + 2.4 ± 7.8 points; p = 0.049 and mental component + 5.2 ± 12.1 points; p < 0.001) increased significantly after PR comparable to an COPD cohort. There were no clinical parameters that predicted changes in outcomes following PR. PR seems to be an effective therapeutic option even in patients with advanced LAM. TRIAL REGISTRATION Clinical-Trials registration number NCT04184193 ; date of registration December 3, 2019.
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Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Linfangioleiomiomatose
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Doença Pulmonar Obstrutiva Crônica
Tipo de estudo:
Observational_studies
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Prognostic_studies
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Risk_factors_studies
Limite:
Female
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Humans
Idioma:
En
Ano de publicação:
2020
Tipo de documento:
Article