Florian Stehling1, Christian Dohna-Schwake2, Uwe Mellies3, Jörg Große-Onnebrink2. 1. Department of Pediatric Respiratory Medicine florian.stehling@uk-essen.de. 2. Department of Pediatric Respiratory Medicine Department of Pediatric Intensive Care Medicine, University Children's Hospital Essen, Essen, Germany. 3. Department of Pediatric Respiratory Medicine.
Abstract
BACKGROUND: Advanced stages of Duchenne muscular dystrophy (DMD) result in severe lung volume decline and are associated with high respiratory morbidity and mortality. The aim of this study was to investigate whether lung volume decline in subjects with DMD is associated with ventilation inhomogeneity measured with the multiple-breath washout technique. METHODS: This cross-sectional study of lung function included 45 subjects with DMD and 16 healthy controls using multiple-breath washout, spirometry, and cough peak flow. RESULTS: Subjects with DMD exhibited an elevated lung clearance index (> 7.0) defined as the cumulative exhaled volume divided by the functional residual capacity to lower the sulfur hexafluoride concentration below 2.5% compared with controls (8.16 ± 2.55 vs 6.23 ± 0.46, P < .001). Lung clearance index elevation was negatively correlated with vital capacity (% predicted: r = -0.79, P < .001) and cough peak flow (L/min: r = -0.41, P = .005). Furthermore, dead-space ventilation (dead-space-to-tidal-volume ratio) and functional residual capacity showed a positive correlation with lung clearance index elevation (r = 0.81 and 0.48, P < .001). An FVC of < 24% predicted lung clearance index elevation with a sensitivity of 96% and a specificity of 80%. CONCLUSIONS: Moderate-to-severe lung volume decline in subjects with DMD is associated with ventilation inhomogeneity. Lung clearance index elevation may be the result of altered ventilation geometry or retention of airway secretions in the infection-free DMD subject.
BACKGROUND: Advanced stages of Duchenne muscular dystrophy (DMD) result in severe lung volume decline and are associated with high respiratory morbidity and mortality. The aim of this study was to investigate whether lung volume decline in subjects with DMD is associated with ventilation inhomogeneity measured with the multiple-breath washout technique. METHODS: This cross-sectional study of lung function included 45 subjects with DMD and 16 healthy controls using multiple-breath washout, spirometry, and cough peak flow. RESULTS: Subjects with DMD exhibited an elevated lung clearance index (> 7.0) defined as the cumulative exhaled volume divided by the functional residual capacity to lower the sulfur hexafluoride concentration below 2.5% compared with controls (8.16 ± 2.55 vs 6.23 ± 0.46, P < .001). Lung clearance index elevation was negatively correlated with vital capacity (% predicted: r = -0.79, P < .001) and cough peak flow (L/min: r = -0.41, P = .005). Furthermore, dead-space ventilation (dead-space-to-tidal-volume ratio) and functional residual capacity showed a positive correlation with lung clearance index elevation (r = 0.81 and 0.48, P < .001). An FVC of < 24% predicted lung clearance index elevation with a sensitivity of 96% and a specificity of 80%. CONCLUSIONS: Moderate-to-severe lung volume decline in subjects with DMD is associated with ventilation inhomogeneity. Lung clearance index elevation may be the result of altered ventilation geometry or retention of airway secretions in the infection-free DMD subject.
Authors: Mathis Steindor; Anna Pichler; Laura Heitschmidt; Eva Pitsikoulis; Alexandra Kavvalou; Eser Orhan; Margerete Olivier; Florian Stehling Journal: BMC Pulm Med Date: 2022-06-04 Impact factor: 3.320
Authors: Ami Mankodi; William Kovacs; Gina Norato; Nathan Hsieh; W Patricia Bandettini; Courtney A Bishop; Hirity Shimellis; Rexford D Newbould; Eunhee Kim; Kenneth H Fischbeck; Andrew E Arai; Jianhua Yao Journal: Ann Clin Transl Neurol Date: 2017-07-28 Impact factor: 4.511