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Scientific Articles

Breathe Duchenne: what natural history studies tell us about the progression of pulmonary morbidity in DMD

key information

source: Neuromuscular Disorders

year: 2018

authors: Mayer OH, Aliverti A, Meier T

summary/abstract:

“When you can’t breathe, nothing else matters”. This is not just a tagline used by the American Lung Association to raise awareness and save lives by improving lung health and preventing lung disease through education, advocacy and research. It resembles the clear and present danger faced by patients with progressive neuromuscular diseases.

Duchenne muscular dystrophy (DMD) is the most common and devastating type of muscular dystrophy. Its clinical course is characterised by loss of muscle strength in upper and lower extremities, a progressive loss of inspiratory and expiratory muscle strength causing pulmonary function decline, and weakness of the muscles of the axillary skeleton causing scoliosis. After loss of lower and upper limb muscle strength and motor function, and particularly after patients become non-ambulant, pulmonary function relentlessly declines, leading to increased risk of bronchopulmonary illnesses, hypoventilation, and dependence on ventilatory support. Pulmonary morbidity is a main cause of death in DMD.

organization: Perelman School of Medicine at the University of Pennsylvania, USA; Politecnico di Milano, Italy; Santhera Pharmaceuticals, Switzerland

DOI: 10.1016/j.nmd.2018.08.005

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