Abstract

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.