[Pediatric pneumological aspects in the care of children with neuromuscular diseases with focus on Duchenne muscular dystrophy].

Abstract

Duchenne muscular dystrophy is the most common inherited neuromuscular disease in children. In addition to the progressive loss of motor skills and cardiac involvement, respiratory muscle weakness leads to a restrictive lung disease and cough insufficiency. Specific respiratory interventions have significantly improved survival and quality of life of the affected boys. The pediatric pulmonologist's tasks include monitoring of the lung function, polysomnography, starting and adjusting of non-invasive or invasive nocturnal ventilation and optimizing of secretion management. Providing of vaccinations, enhancing of nutrition, treatment of intercurrent infections, and provision of advice and training are important tasks for the pediatric pulmonologist. This article emphasizes on the tasks for the pediatric pulmonologist in the treatment of children with neuromuscular diseases i. e. Duchenne muscular dystrophy in interaction with the other specialist disciplines.