Background: Spinal muscular atrophy (SMA) is a neuromuscular progressive disease,
characterized by decreased amounts of survival motor neuron (SMN) protein, due to an …

Neuromuscular disorders (NMDs) may be associated with a gradual loss of muscle function
over time, and children with these disorders are at risk of developing clinically significant …

Background Despite recommendations for regular lung volume recruitment (LVR) use in
clinical practice guidelines for children with neuromuscular disease, adherence to LVR is …

Dysferlin-deficient limb girdle muscular dystrophy (LGMD R2), also referred to as
dysferlinopathy, can be associated with respiratory muscle weakness as the disease …

This chapter pertains to respiratory care of the individual with muscular dystrophy. It
discusses clinical presentation, disease progression, and available treatments for these …

Children who require technology for chronic respiratory insufficiency or failure constitute a
small but varied group. The US Congress's Office of Technology Assessment (OTA) defines …