RT Journal Article
SR Electronic
T1 Challenge already established: Identification of risk factors for early need for ventilatory support in Duchenne muscular dystrophy
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP p4014
VO 38
IS Suppl 55
A1 Leonardo Augusto Tavares
A1 Maria Teresa Fonseca
A1 Laura Maria Lasmar
A1 Alessandra Lúcia Machado
A1 Vera Lúcia Anastácio
YR 2011
UL http://erj.ersjournals.com/content/38/Suppl_55/p4014.abstract
AB Introduction: Duchenne muscular dystrophy (DMD) is a genetic, progressive and disabling disease. Functionally, it puts a restrictive respiratory disorder. Faced with signs and symptoms of chronic respiratory failure should be started as ventilatory support (VS), usually in the second half of adolescence, which was initially due to nocturnal hypoventilation. Ventilar Program (VP) assist DMD patients VS-users and VS-non users in John Paul II Child Hospital/Hospitalar Foundation of Minas Gerais State (JPIICH/FHEMIG).Objective: To identify among patients with DMD assisted by the VP/JPIICH/FHEMIG, possible risk factors for the need early VS.Patients and methods: Cohort study between 2002-2010. Clinical score 20 points (1 point for each sign of hypoventilation). Risk factors associated with VS were evaluated in multivariate analysis by proportional hazards model of Cox. Multivariate model of Cox: all variables p<0,20 in univarate anaysis. Final level of significance p<0.05. Group A: 16 (25.8) VS-users; group B: 46 (74.2) VS-non-users. Group C: loss of ambulation before 10 years and group B after 10 years.Results: Statitically significant difference in univariate analysis for risk factors: body mass index: p=0.15, difficulty swallowing (p=0.05), moderate to severe scoliosis (p=0.01), age at loss of ambulation (p<0.001) and clinical score 0-17 and >17 points (p<0.001). Cox final model: clinical score: relative risk 1.89 (p= 0.001) and loss of ambulation before 10 years: relative risk 2.04 (p=0.01).Conclusion: Age at loss of ambulation and clinical score were independent risk factors for the installation of VS before 20 years of age of DMD patients.