Dmd – clinical care

The decline of respiratory function in patients with Duchenne muscular dystrophy (DMD) leads to alteration of nocturnal gas exchange, with nocturnal hypoventilation (NH) representing the first manifestation. Current expert guidelines warrant the implementation of nocturnal non-invasive ventilation (NIV) when forced vital capacity (FVC)% falls below 50. However, there is limited evidence on the pulmonary function threshold correlating with the onset of NH. With this retrospective, longitudinal study on pediatric patients who underwent a sleep study (SS) at Great Ormond Street Hospital, London, between 2010-2020, we aim to identify the distinct deterioration of pulmonary function and prevalence/severity of sleep-disordered breathing (SDB) in DMD and to explore the relationship between the two.
Source: Neuromuscular Disorders - Category: Neurology Authors: Source Type: research