Evolution of respiratory function in Duchenne muscular dystrophy from childhood to adulthood
نویسندگان
چکیده
منابع مشابه
Transition from childhood to adulthood in Duchenne muscular dystrophy (DMD)
Duchenne muscular dystrophy (DMD) is the most common childhood muscular dystrophy, affecting 1 in 3500 live male births. Mutations in the X chromosome result in an absence of dystrophin, causing progressive muscle degeneration and loss of ambulation by the early teens with respiratory, orthopaedic and cardiac complications. Without intervention these complications lead to death at a mean age of...
متن کاملRespiratory muscle training in Duchenne muscular dystrophy.
Eighteen boys with Duchenne muscular dystrophy were entered into trials to assess the effects of specific ventilatory strength and endurance training programmes. The findings showed an improvement in ventilatory muscle endurance but not in strength as a result of specific respiratory muscle training. The clinical significance of these findings is uncertain, however, and needs further evaluation.
متن کاملTransition to adulthood for young men with Duchenne Muscular Dystrophy
Introduction: Duchenne Muscular Dystrophy (DMD) is an inherited neuromuscular disorder which affects boys. Until recently the mean age of death was around 19 but there have been significant improvements in clinical management and they can now expect to live to around 25 years. Purpose: To investigate, from their own perspectives, how the well-being of young men living with DMD, and that of thei...
متن کاملP164: Adeno-Associated Viral Vectors in Duchenne Muscular Dystrophy
Duchenne muscular dystrophy (BMD) is an inherited X-link disease. The incidence of this muscle-wasting disease is 1:5000 male live births. Mutation in the gene coding for dystrophin is the main cause of BMD. Most cases of this disease succumb to respiratory and cardiac failure in 3rd to 4th decades. The slow progression of BMD and recent achievement of gene therapies make it as an appropriate c...
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...
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ژورنال
عنوان ژورنال: European Respiratory Journal
سال: 2018
ISSN: 0903-1936,1399-3003
DOI: 10.1183/13993003.01418-2017