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2018 29 GIU

The new guidelines in the management of Duchenne Muscular Dystrophy

In April 2018 new guidelines were published for optimal management of primary manifestations and secondary complications of Duchenne  Muscular Dystrophy. 

Respiratory complications are a major cause of morbidity and mortality in people with Duchenne Muscular Dystrophy. Complications include respiratory muscle fatigue, mucus plugging, atelectasis, pneumonia, and respiratory failure. An anticipatory approach to management includes monitoring of respiratory muscle function and the timely use of lung volume recruitment, assisted coughing, nocturnally assisted ventilation, and subsequent daytime ventilation. These core therapies can decrease respiratory complications, improve quality of life, and prolong survival. Implementation of respiratory care considerations and guidelines requires a multidisciplinary team — including physicians, respiratory therapists, and home caregivers — to perform pulmonary function testing and sleep studies and to initiate and manage lung volume recruitment, manual and mechanically assisted coughing, non-invasive ventilation, and invasive ventilation via tracheostomy. In this update, we endorse higher pulmonary function thresholds for initiation of assisted coughing and assisted ventilation than were recommended in the 2010 care considerations. The new criteria are intended to result in more anticipatory use of these interventions, with the possibility that therapy will be initiated in slightly younger patients.

Fonte: Diagnosis and management of Duchenne muscular dystrophy, part 2: respiratory, cardiac, bone health, and orthopaedic management. Lancet Neurol. 2018 April ; 17(4): 347–361. doi:10.1016/S1474-4422(18)30025-5.

Article by Claudio Cherchi