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PHYSIOTHERAPY IN LYMPHANGIOLEIOMYOMATOSIS: A SYSTEMATIC REVIEW
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Instituto Nacional de Cardiologia. Departamento de Ensino e Pesquisa. Rio de Janeiro, RJ, Brasil.
Instituto Nacional de Cardiologia. Departamento de Ensino e Pesquisa. Rio de Janeiro, RJ, Brasil.
Instituto Nacional de Cardiologia. Serviço de Fisioterapia. Rio de Janeiro, RJ, Brasil.
Instituto Nacional de Cardiologia. Serviço de Fisioterapia. Rio de Janeiro, RJ, Brasil.
Fundação Oswaldo Cruz. Instituto Nacional de Infectologia Evandro Chagas. Laboratório de Pesquisa Clínica em Doença de Chagas. Rio de Janeiro, RJ, Brasil / Instituto Nacional de Cardiologia. Departamento de Ensino e Pesquisa. Rio de Janeiro, RJ, Brasil.
Instituto Nacional de Cardiologia. Departamento de Ensino e Pesquisa. Rio de Janeiro, RJ, Brasil / Universidade Federal do Estado do Rio de Janeiro. Instituto Biomédico. Departamento de Ciências Fisiológicas. Rio de Janeiro, RJ, Brasil.
Instituto Nacional de Cardiologia. Departamento de Ensino e Pesquisa. Rio de Janeiro, RJ, Brasil.
Instituto Nacional de Cardiologia. Serviço de Fisioterapia. Rio de Janeiro, RJ, Brasil.
Instituto Nacional de Cardiologia. Serviço de Fisioterapia. Rio de Janeiro, RJ, Brasil.
Fundação Oswaldo Cruz. Instituto Nacional de Infectologia Evandro Chagas. Laboratório de Pesquisa Clínica em Doença de Chagas. Rio de Janeiro, RJ, Brasil / Instituto Nacional de Cardiologia. Departamento de Ensino e Pesquisa. Rio de Janeiro, RJ, Brasil.
Instituto Nacional de Cardiologia. Departamento de Ensino e Pesquisa. Rio de Janeiro, RJ, Brasil / Universidade Federal do Estado do Rio de Janeiro. Instituto Biomédico. Departamento de Ciências Fisiológicas. Rio de Janeiro, RJ, Brasil.
Abstract
Background: Lymphangioleiomyomatosis (LAM) is associated with progressive dyspnoea and exercise intolerance, but despite the central role of physiotherapy on pulmonary rehabilitation, there is a huge lack of physiotherapy approaches used specifically for LAM patients.
Objective: to identify the physiotherapeutic strategies used in the treatment of patients with LAM.
Materials and methods: This is a systematic review of literature. Searches were performed (in PubMed, Lilacs, Embase and PEDro databases) with the keywords "Lymphangioleiomyomatosis" and "Physiotherapy," and its variations. Articles describing physiotherapy interventions were included in the study. Data extracted from the studies were authors, year, country of publication, sample size, physiotherapy intervention, time/frequency/duration of intervention protocols, instruments used to measure results and main findings. Methodological quality of studies was evaluated by PEDro Scale (clinical trials), Newcastle-Ottawa Scale (NOS; observational studies) and CARE checklist (case reports), respectively.
Results: A total of 82 articles identified, three duplicates were removed, 71 studies were excluded after title and abstract reading and four after full-text reading, all due to absence of association with the study topic. Four studies were included in the present review. Cardiorespiratory physiotherapy with endurance and resistance training were identified as physiotherapeutic strategies to improve lung function, functional capacity, depression symptoms and quality of life in LAM.
Conclusions: Endurance and resistance training is the keystone for physiotherapy in patients with LAM, but despite the reported benefits, there is a huge lack of studies related to the modalities, safety and dosage of physiotherapy prescription for patients with LAM.KEY MESSAGESLymphangioleiomyomatosis (LAM), a rare disease, leads to progressive dyspnoea and exercise intolerance;Physiotherapy can improve dyspnoea and exercise intolerance in LAM through endurance and resistance exercises.
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