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- Title
Respiratory decline in Duchenne muscular dystrophy.
- Authors
LoMauro, Antonella; Marchi, E.; Diella, Eleonora; Aliverti, Andrea; D'Angelo, Maria Grazia
- Abstract
The improvement of care in patients with Duchenne Muscular Dystrophy (DMD), especially the introduction of home mechanical ventilation, corticosteroid therapy, and early cardiologic therapy, allowed an increase of life expectancy, till their 30s or 40s. However, respiratory complications remain one of the major causes of morbidity and mortality with respiratory muscle fatigue, mucus plugging, atelectasis, pneumonia, and respiratory failure. Monitoring of respiratory muscle function and the timely use of lung volume recruitment, assisted coughing, nocturnally assisted ventilation, and subsequent daytime ventilation are essential in the management. Respiratory parameters are important not only in the evaluation of the natural evolution of the disease and setting specific treatments but are becoming increasingly relevant as outcome measures in these areas of gene and gene-modulating therapies. Herein, we present the natural history of respiratory muscle decline in DMD, the role played on it by steroid therapy, and other treatments in curing and slowing the natural course of the disease. We also demonstrate how multidisciplinary and multi-instrumental approaches are essential for an optimal assessment.
- Subjects
DUCHENNE muscular dystrophy; ARTIFICIAL respiration; CORTICOSTEROIDS; LIFE expectancy; MUSCLE fatigue; PLETHYSMOGRAPHY
- Publication
European Journal of Translational & Clinical Medicine, 2023, Vol 6, p24
- ISSN
2657-3148
- Publication type
Article