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Advisor(s)
Abstract(s)
Introdução: A Esclerose Lateral Amiotrófica (ELA) é considerada uma das doenças
neuromusculares (DNM) com características mais limitantes e fatais, sendo
caracterizada por fraqueza muscular progressiva.
Objectivo: Analisar a evolução da Capacidade Vital e da Máxima Capacidade
Inspiratória em doentes com ELA e a sua relação causal com a função bulbar.
Procedimentos: A partir de uma população alvo de 203 pacientes com ELA, foram
incluídos no estudo aqueles que tinham entre 2 a 4 testes de função respiratória
considerados válidos (CV <2000ml) perfazendo um total de 22 indivíduos. As medidas
CV e MCI foram analisadas.
Resultados: A CV diminuiu ao longo do tempo (media±desvio padrão no 1ºmomento
de avaliação =1779,5±692,3; media±desvio padrão no 4ºmomento de avaliação
=1108,6±475,7). O comportamento da MCI foi mais estável ao longo dos 4 momentos
de avaliação. Avaliou-se a correlação entre as duas variáveis, destacando-se a relação
existente entre as duas nos doentes bulbares (coeficiente = 1). Quando avaliada a
diferença entre a CV e a MCI, verificamos que o nível de significância no grupo (n=22)
aumentou ao longo do tempo. Ao comparamos esta diferença por subgrupos, registou-se
uma diferença significativa apenas nos doentes bulbares (1ºmomento – p=0,008 e
último momento de avaliação – p 0).
Conclusão: Nos doentes com disfunção bulbar a CV diminui ao longo do tempo. A
relação entre MCI e CV é um bom factor preditivo da evolução e prognóstico da doença
e de diagnóstico do envolvimento da musculatura bulbar.
Introduction: Amyotrophic Lateral Sclerosis (ALS) is considered one of the most severe neuromuscular diseases (NMD) and life threatening and is characterized by progressive muscle weakness. Objective: To analyze Vital Capacity (VC) and Maximal Insufflation Capacity (MIC) evolution and their relationship to bulbar function. Design: From a target population of 203 ALS patients 22 were included in the study, those who had between 2 to 4 respiratory function tests were considered valid (CV <2000ml). CV and MIC measures were analyzed. Results: The CV decreased over time (mean ± standard deviation in the 1st time of evaluation = 1779.5 ± 692.3, mean ± standard deviation on the 4th time of evaluation = 1108.6 ± 475.7). MCI was more stable over the four time points. When evaluated the correlation between two variables the relationship between both was relevant in bulbar patients (coefficient = 1). When evaluating the difference of VC and MCI it was found that the level of significance in the group (n = 22) increased over time. By comparing this difference in subgroups there was a significant difference in bulbar patients (time 1 - p = 0.008 and the last minute evaluation - p 0). Conclusions: In patients with bulbar dysfunction CV decreases over the time. The relationship between MIC and CV is a good predictor of the evolution, prognosis and diagnosis of bulbar muscles involvement.
Introduction: Amyotrophic Lateral Sclerosis (ALS) is considered one of the most severe neuromuscular diseases (NMD) and life threatening and is characterized by progressive muscle weakness. Objective: To analyze Vital Capacity (VC) and Maximal Insufflation Capacity (MIC) evolution and their relationship to bulbar function. Design: From a target population of 203 ALS patients 22 were included in the study, those who had between 2 to 4 respiratory function tests were considered valid (CV <2000ml). CV and MIC measures were analyzed. Results: The CV decreased over time (mean ± standard deviation in the 1st time of evaluation = 1779.5 ± 692.3, mean ± standard deviation on the 4th time of evaluation = 1108.6 ± 475.7). MCI was more stable over the four time points. When evaluated the correlation between two variables the relationship between both was relevant in bulbar patients (coefficient = 1). When evaluating the difference of VC and MCI it was found that the level of significance in the group (n = 22) increased over time. By comparing this difference in subgroups there was a significant difference in bulbar patients (time 1 - p = 0.008 and the last minute evaluation - p 0). Conclusions: In patients with bulbar dysfunction CV decreases over the time. The relationship between MIC and CV is a good predictor of the evolution, prognosis and diagnosis of bulbar muscles involvement.
Description
Keywords
Doença neuromuscular Capacidade de insuflação pulmonar Capacidade vital Esclerose lateral amiotrófica Disfunção bulbar Air stacking Neuromuscular disease Lung insufflation capacity Vital capacity Amyotrophic lateral sclerosis Bulbar dysfunction
Citation
Publisher
Instituto Politécnico do Porto. Escola Superior de Tecnologia da Saúde do Porto