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. Twenty-one male patients with DM1 and 16 healthy volunteers were registered in the study. Body composition was measured using dual-energy X-ray absorptiometry (DEXA). Fat mass (FM) index (kg/m2), fat-FM index (kg/m2), and skeletal mass index (kg/m2) were calculated. The measurements were taken breath by breath with a portable indirect calorimeter. The REE was calculated using the oxygen intake (VO2) and carbon dioxide output (VCO2) in the Weir equation. Basal energy expenditure (BEE) was calculated by substituting height, weight, and age into the Harris-Benedict equation. The study enrolled male patients with DM1 (n = 12) and healthy male volunteers (n = 16). Patients with DM1 (n = 7) and healthy volunteers (n = 14) could be followed in 1 year. The body composition of patients with DM1 was significantly higher in the FM index and significantly lower in the fat-FM index and skeletal mass index. The REE of patients with DM1 was significantly lower and was not associated with body composition. Patients with DM1 had poor metabolism that was not related to body composition. FM was high and lean body mass was low.

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). METHODS: The study included 34 patients with NMDs. The patients were categorized into air stacking group (n=15), no air stacking group (n=9), and tracheostomy/tracheostomy positive-pressure ventilation (TPPV) group (n=10). RESULTS: In each group, the cough peak flow (CPF) at 75% of the forced vital capacity (V̇75), V̇50, V̇25, and V̇10 were investigated during the approaches. In the air stacking group, the CPF was higher with MAC-UT, MAC-LT, and MAC-UT/A than with MI-E (p < 0.05). Additionally, V̇75 was higher with MAC-LT and MAC-UT/A than with MI-E (p < 0.05 and p < 0.01, respectively). In the no air stacking group, V̇75 was higher with MAC-UT/A than with MI-E (p < 0.05). In the tracheotomy/TPPV group, there were no significant differences. CONCLUSIONS: MAC approaches, especially MAC-LT and MAC-UT/A, are preferred in air stacking patients. However, in tracheostomy/TPPV patients, the CPF might not increase with MAC.

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. This study aimed to compare CPF with different cough-assistance methods and to examine the reliability of CPF data. METHODS: The study included 12 subjects with DMD (mean age, 34 ± 8 y). CPF, CPF + manually-assisted coughing (assisted CPF), maximum insufflation capacity (MIC) + CPF (MIC-CPF), MIC + manually-assisted coughing (MIC+assisted CPF), MI-E (MI-E-CPF), and MI-E + assisted CPF were measured. A spirometer was used to compare CPF measurements obtained with each of the cough-assist techniques. The reliability of the measured CPF values was analyzed using Bland-Altman analysis. RESULTS: CPF was 59 ± 34 L/min, assisted CPF was 113 ± 32 L/min, MIC-CPF was 170 ± 30 L/min, MIC+assisted CPF was 224 ± 62 L/min, MI-E-CPF was 199 ± 40 L/min, and MI-E + assisted CPF was 240 ± 38 L/min. A fixed and proportional bias was found in the CPF measurements made with the peak flow meter and the spirometer. The average 95% CI in the difference between peak flow meter, MI-E, and CPF obtained using the spirometer were -7.45 to -1.95 and -1.45 to 4.95, respectively. Test for correlation was r = 0.54 (P < .001) for CPF (peak flow meter) and CPF (spirometer) and r = 0.17 (P = .17) in CPF (MI-E) and CPF (spirometer), respectively. CONCLUSION: MI-E + assisted CPF was the highest. The CPF measured with the peak flow meter suggested underestimation.