Management of respiratory disease in children with muscular weakness

Children with chronic muscular weakness from any cause are prone to develop varying degrees and patterns of respiratory muscle weakness. This leads to ineffective cough, atelectasis, pneumonia, restrictive pulmonary disease and eventually respiratory failure. Over the past 20 years there have been improvements in clinical care of children with muscular weakness, including improved monitoring of lung function and hypoventilation during sleep, coordinated multidisciplinary respiratory care, physiotherapy and introduction of non-invasive ventilation. This article reviews the current knowledge base and the evidence for management of children and provides practical advice for paediatricians.