Cooperative International Neuromuscular Research Group Duchenne Natural History Study demonstrates insufficient diagnosis and treatment of cardiomyopathy in Duchenne muscular dystrophy.
Muscle Nerve
; 50(2): 250-6, 2014 Aug.
Article
em En
| MEDLINE
| ID: mdl-24395289
ABSTRACT
INTRODUCTION:
Cardiomyopathy is a common cause of morbidity and death in patients with Duchenne muscular dystrophy (DMD).METHODS:
This investigation was a cross-sectional cross-sectional analysis of clinical data from the multi-institutional Cooperative International Neuromuscular Research Group (CINRG) DMD Natural History Study of 340 DMD patients aged 2-28 years. Cardiomyopathy was defined as shortening fraction (SF) <28% or ejection fraction (EF) <55%.RESULTS:
Two hundred thirty-one participants reported a prior clinical echocardiogram study, and 174 had data for SF or EF. The prevalence of cardiomyopathy was 27% (47 of 174), and it was associated significantly with age and clinical stage. The association of cardiomyopathy with age and clinical stage was not changed by glucocorticoid use as a covariate (P > 0.68). In patients with cardiomyopathy, 57% (27 of 47) reported not taking any cardiac medications. Cardiac medications were used in 12% (15 of 127) of patients without cardiomyopathy.CONCLUSIONS:
We found that echocardiograms were underutilized, and cardiomyopathy was undertreated in this DMD natural history cohort.Palavras-chave
Texto completo:
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Base de dados:
MEDLINE
Assunto principal:
Distrofia Muscular de Duchenne
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Pesquisa Biomédica
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Cooperação Internacional
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Cardiomiopatias
Tipo de estudo:
Diagnostic_studies
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Etiology_studies
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Risk_factors_studies
Limite:
Adolescent
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Adult
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Child
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Child, preschool
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Female
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Humans
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Male
Idioma:
En
Ano de publicação:
2014
Tipo de documento:
Article