Duchenne Muscular Dystrophy is an X-linked inherited disorder and the
most common fatal degenerative muscle disease in pediatric patients. Its
first symptoms usually manifest at an early age, with weakness,
psychomotor developmental delay, evolving to cardiac and ventilatory
muscle impairment. The involvement of the pulmonary musculature is
demonstrated by the frequent episodes of obstruction and aspirations,
sleep-disordered breathing, and by the development of a chronic
restrictive breathing pattern. Failure of respiratory mechanics is a
major cause of mortality in Duchenne Muscular Dystrophy, and the
beginning of lung function deterioration is not always accompanied by
symptoms, which reinforces the need for serial evaluations for early
initiation of a ventilatory support. The present article is thought to
provide a synthesized overview of the assessment and management of
Duchenne Muscular Dystrophy ventilatory disturbances.