Effect of pulmonary function on right heart function in Duchenne muscular dystrophy patients
نویسندگان
چکیده
منابع مشابه
Effect of pulmonary function on right heart function in Duchenne muscular dystrophy patients
Background Duchenne muscular dystrophy (DMD) is an x-linked neuromuscular disorder in which cardiopulmonary disease leads to death in the third to fourth decade of life. Diaphragm and myocardium suffer progressive damage. While abnormalities in pulmonary and left ventricular function have been independently described, the relationship between pulmonary function and the right ventricle has not b...
متن کاملCharacterization of pulmonary function in Duchenne Muscular Dystrophy
Decline in pulmonary function in Duchenne Muscular Dystrophy (DMD) contributes to significant morbidity and reduced longevity. Spirometry is a widely used and fairly easily performed technique to assess lung function, and in particular lung volume; however, the acceptability criteria from the American Thoracic Society (ATS) may be overly restrictive and inappropriate for patients with neuromusc...
متن کاملBrain function in Duchenne muscular dystrophy.
Duchenne muscular dystrophy (DMD) is the second most commonly occurring genetically inherited disease in humans. It is an X-linked condition that affects approximately one in 3300 live male births. It is caused by the absence or disruption of the protein dystrophin, which is found in a variety of tissues, most notably skeletal muscle and neurones in particular regions of the CNS. Clinically DMD...
متن کاملDuchenne Muscular Dystrophy and Brain Function
Muscular dystrophies have historically been characterised according to clinical criteria, however in the genomic age the muscular dystrophies are now subdivided into groups according to the primary gene defect. Currently identified are 29 different loci and encoded proteins, giving rise to 34 distinct forms of muscular dystrophy (Dalkilic & Kunkel 2003; Hsu 2004). The majority of these types of...
متن کاملTreatment effect of idebenone on inspiratory function in patients with Duchenne muscular dystrophy
Assessment of dynamic inspiratory function may provide valuable information about the degree and progression of pulmonary involvement in patients with Duchenne muscular dystrophy (DMD). The aims of this study were to characterize inspiratory function and to assess the efficacy of idebenone on this pulmonary function outcome in a large and well-characterized cohort of 10-18 year-old DMD patients...
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ژورنال
عنوان ژورنال: Journal of Cardiovascular Magnetic Resonance
سال: 2015
ISSN: 1532-429X
DOI: 10.1186/1532-429x-17-s1-p279