Dose-dependent effect of individualized respiratory muscle training in children with Duchenne muscular dystrophy

Inspiratory muscle training in obstructive lung disease

“…Accordingly, there may be some value in implementing IMT in patients with conditions such as sarcoidosis [53], obesity [54], type 1 diabetes mellitus [55], idiopathic pulmonary hypertension [56], amyotrophic lateral sclerosis [57], hypothyroidism [58], arthritis [59,60] or cancer [61,62]. Furthermore, there are a small number of randomised, controlled trials in which IMT has elicited a positive effect upon dyspnoea in cystic fibrosis [63], heart failure [64,65], neuromuscular disease [66,67], spinal cord injury [68], as well as less rigorously designed and executed studies in conditions such as kyphoscoliosis [69] and post-polio [70]. Finally, in a randomised controlled trial, IMT has been shown to offset the myopathic influence of high-dose oral steroids upon inspiratory muscle strength [71].…”

Regenerative capacity of the dystrophic (mdx) diaphragm after induced injury

“…In support of this hypothesis, Barton et al (4) have shown that overexpression of insulin-like growth factor I in mdx mice augments muscle mass and reduces fibrosis within the diaphragm. Second, there is evidence that short-term strength or endurance training is useful for reducing the loss of diaphragmatic force-generating capacity and increased muscle fatigability associated with dystrophin deficiency (8,10,50). However, there has also been a fear that training could increase the level of contraction-induced injury and hence satellite cell turnover, thereby leading to an accelerated decline of regenerative potential (39).…”

Surgical management of severe scoliosis with high-risk pulmonary dysfunction in Duchenne muscular dystrophy

“…As well as in patients with obstructive lung disease, there is a theoretical rationale for strengthening the inspiratory muscles of any patient who experiences inappropriate breathlessness, abnormal respiratory mechanics and/or inspiratory muscle weakness/fatigue. Furthermore, there are a small number of randomised, controlled trials in which IMT has elicited a positive effect upon neuromuscular diseases [23,24] as well as less rigorously designed and executed studies in conditions such as kyphoscoliosis [10]. Although the number of our patients is small, the efficacy of preoperative respiratory training using Threshold IMT is shown clinically in this study.…”