Abstract

Introduction-Aim: Duchenne muscular dystrophy (DMD) is the most common myotonic dystrophy characterized by progressive muscle dysfunction. Respiratory muscles are also impacted in DMD. In this study, we planned to analyze the demographic and respiratory characteristics, polysomnography (PSG), and capnography evaluations of patients with DMD who applied to our Pediatric Pulmonology outpatient clinic in the last year.

Material-Method: Thirty-nine patients with DMD who applied to our outpatient clinic between January and August 2022 were included. Demographic characteristics of the patients, pulmonary function tests (PFT), maximal inspiratory (MIP) and expiratory pressure (MEP), nasal sniff inspiratory pressure (SNIP), capnography, and PSG data were analyzed.

Results: 94.1% of the patients were male. The mean age was 10±3 years. PFT and PSG were performed in 17 patients. Mean % FVC was 87.9±17.5 and mean MIP was 49±16.5 cmH2O. According to PSG results, moderate obstructive sleep apnea was detected in two patients and mild in 10 patients. All-night mean pCO2 value of the patients was 41.7±2.6 mmHg. Nocturnal hypoventilation was detected in only 1 patient. While ambulatory and non-ambulatory patients did not differ significantly between capnography data, there were significant differences between the two groups in terms of apnea-hypopnea index (AHI), obstructive apnea-hypopnea index (OAHI), and SNIP values (p<0.05).

Conclusion: DMD patients should be followed closely regarding respiratory symptoms during sleep and respiratory support needs, and respiratory support should be started early if necessary.