INTRODUCTION The relationship between skeletal muscle strength and respiratory dysfunction in Pompe disease has not been examined by quantitative methods. We investigated correlations among lower extremity proximal muscle strength, respiratory function, and motor performance. METHODS Concentric strength of the knee extensor and flexor muscles was measured with a dynamometer, and pulmonary fun...

The natural course of progressive neuromuscular diseases can be complicated by respiratory muscle involvement. In muscular dystrophies such as Duchenne muscular dystrophy and myotonic dystrophy, respiratory muscle involvement is common. In others such as Becker, limb-girdle, and facioscapulo-humeral dystrophies, respiratory muscle involvement is infrequent and generally occurs in the more sever...

Background: XLMTM is a rare, currently untreated, life-threatening congenital myopathy caused by mutations in the MTM1 gene, with profound muscle weakness and impairment of motor development, respiratory failure, chronic ventilator dependency.

Background and Objectives: Idiopathic pulmonary fibrosis (IPF) is characterized by progressively worsening lung function, ventilation capacity, dyspnea, and finally reduced exercise intolerance. All of these have a significant negative impact on functional capacity and quality of life. In this study, we aim to evaluate the effects of pulmonary rehabilitation (PR) in IPF and assess the predictor...

With age, physical functions decline, which influences respiratory performance. One of the physical changes associated with aging is sarcopenia, a reduction in muscle strength and power. Sarcopenia has been extensively studied in the elderly with regard to limb function but less with regard to respiratory function. Elderly individuals experience reduced muscle mass and strength in respiratory m...

[Purpose] The purpose of this study was to present aerobic exercise that can be performed together with respiratory muscle strength training and examine whether the vital capacity of individuals can be enhanced when respiratory muscle strength training is conducted together with aerobic exercise. [Subjects and Methods] The subjects were 10 male students and 8 female students. The sling exercise...

Primary culture models of single adult skeletal muscle fibers dissociated from locomotor muscles adhered to glass coverslips are routine and allow monitoring of functional processes in living cultured fibers. To date, such isolated fiber cultures have not been established for respiratory muscles, despite the fact that dysfunction of core respiratory muscles leading to respiratory arrest is the ...

It is known that respiratory muscles undergo adaptation in response to overload stimuli during exercise training in stable COPD patients, thus resulting in significant increase of respiratory muscle function as well as the individual's improvements. The present article reviews the most updated evidence with regard to the use of respiratory muscle training (RMT) methods in COPD patients. Basical...

Respiratory impairment, due to respiratory muscle weakness, is a major cause of morbidity and mortality in patients with amyotrophic lateral sclerosis/motor neuron disease (ALS/MND). Threshold loading may strengthen the inspiratory muscles and thereby improve patient prognosis. A phase II, double-blind, randomized-controlled trial was undertaken to determine whether a 12-week inspiratory muscle...