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Duchenne muscular dystrophy

Key sources
The following summarized guidelines for the evaluation and management of duchenne muscular dystrophy are prepared by our editorial team based on guidelines from the American College of Chest Physicians (ACCP 2023), the Heart Rhythm Society (HRS 2022), the European Society of Cardiology (ESC 2022), the American Heart Association (AHA/HRS/ACC 2018), the American Academy of Neurology (AAN 2016), and the British Thoracic Society (BTS 2012).
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Guidelines

1.Diagnostic investigations

Respiratory evaluation: as per ACCP 2023 guidelines, obtain pulmonary function testing to assist with management decisions in patients with neuromuscular disease at risk for respiratory complications.
E
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  • Sleep evaluation

  • Cardiac evaluation

  • Assessment of dysphagia

  • Assessment of pain

2.Respiratory support

Airway clearance, manually assisted cough, ACCP: consider using manually assisted cough techniques, independently or added to other modalities (such as lung volume recruitment), in patients with reduced cough effectiveness.
C

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  • Airway clearance (lung volume recruitment)

  • Airway clearance (mechanical insufflation-exsufflation)

  • Airway clearance (high-frequency chest wall oscillation)

  • Airway clearance (sputum mobilization)

  • Airway clearance (nebulized saline)

  • Airway clearance (noninvasive ventilation)

  • Airway clearance (acute infection)

  • Noninvasive ventilation

  • Invasive ventilation

3.Medical management

Corticosteroids
Offer prednisone at a preferred dose of 0.75 mg/kg/day to improve strength and pulmonary function in pediatric patients with DMD.
B
Consider offering prednisone to improve timed motor function, reduce the need for scoliosis surgery, and delay cardiomyopathy onset by 18 years of age. Recognize that prednisone 0.75 mg/kg/day is associated with a significant risk of weight gain, hirsutism, and cushingoid appearance.
B
Consider offering deflazacort to improve strength and timed motor function, delay age at loss of ambulation by 1.4-2.5 years, improve pulmonary function, reduce the need for scoliosis surgery, delay cardiomyopathy onset, and increase survival at 5-15 years of follow-up in pediatric patients with DMD. Recognize that deflazacort may be associated with a greater risk of cataracts than prednisone.
C

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  • Opioids

  • Anticoagulant therapy

4.Nonpharmacologic interventions

Nutritional support
Provide feeding advice from a specialist therapist in pediatric patients with swallowing difficulties.
E
Provide a problem-orientated approach to nutrition aiming to minimize the risk of aspiration, optimize nutritional status, promote comfort, and balance the positive social consequences of continued oral feeding.
E

5.Therapeutic procedures

Permanent pacemaker implantation
Perform permanent pacemaker implantation in patients with DMD with documented symptomatic bradycardia due to any degree of SND or AV block, if concordant with the patient's goals of care and clinical status.
B
Perform permanent pacemaker implantation in patients with DMD and third-degree or advanced second-degree AV block at any anatomical level, with or without symptoms, if concordant with the patient's goals of care and clinical status.
B

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  • CRT

  • ICD

6.Perioperative care

Preoperative planning: assess pediatric patients with neuromuscular disorders requiring surgery (including scoliosis surgery) by a multidisciplinary team before any intervention.
E
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7.Patient education

General counseling: engage patients, parents, and carers in a process of mutual participation in decision-making with full disclosure of all information in a sensitive and timely fashion in order to make informed choices consistent with their own values and preferences requires physicians.
B
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8.Follow-up and surveillance

End-of-life care: provide periodic education on function including deactivation in patients (as well as their family members and/or healthcare decision makers) with neuromuscular disorders eligible for or having a pacemaker or ICD.
B
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