الفهرس | Only 14 pages are availabe for public view |
Abstract Paediatric neuromuscular disease compromises both the gas exchange and pump functions of the respiratory system. This can have profound implications for both growth and development of the respiratory system, as well as morbidity and mortality. Aspiration lung disease is common, and leads to increasingly restrictive pulmonary physiology over time. Abnormal lung and chest wall mechanics, and weak respiratory muscles, can combine to cause respiratory failure. Neuromuscular disease (NMD) is a very broad term that encompasses many diseases that either directly, via intrinsic muscle pathology, or indirectly, via nerve pathology, impair the function of the muscles. These neuromuscular diseases may be genetically determined, congenital, or acquired, acute or chronic, and progressive or static (Polkey et al, 1999). Neuromuscular diseases are characterized by progressive atrophy and weakness of skeletal muscle, skeletal-spinal deformities, limb contractures, and restrictive lung disease leading to poor respiratory function. Increased morbidities frequently result in shortened life expectancy (Wang & Finkel, 2007). |