Assessment of body composition, metabolism, and pulmonary function in patients with myotonic dystrophy type 1.

Abnormal body composition in myotonic dystrophy type 1 (DM1) are affected by energy intake above resting energy expenditure (REE). We aim to investigate the characteristics and relationship between body composition, REE, and pulmonary function in patients with DM1, and to examine their changes in 1 year. The study design was a single-center, cross-sectional, and longitudinal study of body composition, REE characteristics, and pulmonary function.

Cough peak flow with different mechanically assisted coughing approaches under different conditions in patients with neuromuscular disorders.

Purpose: Mechanically assisted coughing (MAC) is an airway clearance method in which the thorax/abdomen is compressed in synchronization with mechanical insufflation-exsufflation (MI-E). MAC can be performed with manual assistance at the upper thorax (MAC-UT), lower thorax (MAC-LT), and upper thorax + abdomen (MAC-UT/A). This study aimed to determine the most effective approach under different conditions (air stacking or tracheostomy) in patients with neuromuscular disorders (NMDs).

Approaches to Cough Peak Flow Measurement With Duchenne Muscular Dystrophy.

Background: Manually-assisted coughing and mechanical insufflation-exsufflation (MI-E) are commonly used in patients with Duchenne muscular dystrophy (DMD). Few studies have compared cough peak flow (CPF) with manually-assisted coughing to other methods, such as MI-E + manually-assisted coughing. In addition, few studies have reported the reliability of the measured CPF values.