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Objective Tracheobronchomalacia (TBM) is common in neonates with bronchopulmonary dysplasia (BPD) and is associated with higher morbidity. This study evaluates the value of a CT protocol to assess the degree of TBM and gauge the adequacy of prescribed PEEP. Study Design Four infants with severe BPD on invasive mechanical ventilation underwent a chest CT protocol, including limited reduced-dose expiratory scans with varying PEEP levels. Results Baseline PEEP was adjusted in all subjects after performing the Dynamic PEEP CT. In two infants, the PEEP was increased due to significant TBM and in the other two without signs of TBM PEEP was decreased. The clinical course improved in all patients after adjusting PEEP. Conclusion A 'Dynamic PEEP' study may be reliable and non-invasive imaging modality for the evaluation of adequate ventilator settings in infants with severe BPD who are not optimal candidates for bronchoscopy., Author(s): Cassie Montoya [sup.1], Robin Steinhorn [sup.2], John Berger [sup.3], Harutyun Haroyan [sup.4], Mariam Said [sup.2], Geovanny F. Perez [sup.5] Author Affiliations: (1) grid.16753.36, 0000 0001 2299 3507, Division of [...]

Author(s): Cassie Montoya [sup.1], Robin Steinhorn [sup.2], John Berger [sup.3], Harutyun Haroyan [sup.4], Mariam Said [sup.2], Geovanny F. Perez [sup.5] Author Affiliations: (1) grid.413808.6, 0000 0004 0388 2248, Division of [...]

Purpose Patients with neuromuscular disease (NMD) experience weakened cough due to progressive respiratory muscle weakness. Peak cough flow (PCF) measurements derived from adult populations are used to recommend initiation of assisted cough therapies. The objective of this study was to characterize PCF values among pediatric patients with NMD. Methods Retrospective chart review was performed for patients seen in the multidisciplinary pediatric muscular dystrophy clinic from 2010 to 2016. Clinical and demographic variables included age, gender, ambulation status, and PCF measurements. Results 366 patients with an established diagnosis of NMD (median age 11.8 years) were included in this study. 102 (27.8%) out of the 366 patients were affected by Duchenne muscular dystrophy (DMD), 42 (11.5%) by congenital muscular dystrophy (CMD), 42 (11.5%) by Charcot Marie Tooth disease (CMT) and 24 (6.5%) by Becker's muscular dystrophy (BMD). The mean PCF values in DMD (255.8 L/min) and CMD (249.1 L/min) were lower than CMT (321.5 L/min) with p-values of 0.007 and 0.02, respectively. The mean PCF of BMD (333.3 L/min) was higher than that of DMD and CMD but the difference was not statistically significant. PCFs were not statistically different between ambulatory and non-ambulatory status (263.0 L/min versus 290.8 L/min, p = 0.12). Children under 10 years of age had lower PCF relative to older subjects (179.5 L/min versus 300.9 L/min, p < 0.0001). Conclusion Baseline PCF values in young children are below the adult-specific values suggested for starting assisted cough techniques. Further longitudinal trials are required to derive pediatric-specific reference values for PCF in patients with NMD., Author(s): Nidhi Kotwal [sup.1] [sup.2] , Prateek J. Shukla [sup.3] , Geovanny F. Perez [sup.2] [sup.4] Author Affiliations: (1) Division of Pulmonology and Allergy, Department of Pediatrics, University of Maryland, [...]