Pediatrics: Interventions: Duchenne's Muscular Dystrophy

Question 1

Duchenne’s Muscular Dystrophy

Answer 1

• X linked
• Recessive
• Inherited by boys

Question 2

Duchenne’s Muscular Dystrophy: Dystrophin

Answer 2

• Missing

- Causes increased permeability of sarcolemma and destruction of muscle cells

Question 3

Duchenne’s Muscular Dystrophy: Collagen and Adipose

Answer 3

• Laid down in muscles

- Causes psuedohypertrophic muscles

Question 4

Impairments: Diagnosis

Answer 4

• Diagnosis made in 3-6 years of age

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Question 5

Impairments: Early Signs

Answer 5

• Progressive weakness
• Delay in walking
• Difficulty rising from supine or sitting
• Frequent falls
• Proximal weakness before distal weakness

Question 6

Impairments: Gower’s sign

Answer 6

• Child must use arms to “walk up” legs to rise to standing

Question 7

Impairments: Contractures and Deformities

Answer 7

• Heel cords
• TFL
• Lumbar Lordosis
• Kyphoscoliosis

Question 8

Functional Limitation

Answer 8

• Milestones delayed
• Ambulation ability will eventually be lost, necessitating eventual use of a wheel chair
• Cardiopulmonary limitations

Question 9

Interventions Goals and Prognosis: Red Flag

Answer 9

• Do no over exercise children with DMD at a maximal level.

- This can lead to over work injury due to musculoskeletal impairments

Question 10

Medical and Surgical Management

Answer 10

• Steroids for decreasing pulmonary dysfunction
• Antibiotics for pulmonary infections
• Orthopedic surgery for scoliosis
• Muscle lengthening of the gastrocnemius

Question 11

Becker’s Muscular Dystrophy

Answer 11

• Slower variant to DMD