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Peds MD Lecture

Quiz yourself by thinking what should be in each of the black spaces below before clicking on it to display the answer.
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Question
Answer
Definition of MD   show
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show Destruction of Myofibrils  
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show Dystrophin  
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Duchenne   show
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show 5-10 yo; Slow progression; Gait to early teens; Lifespan into 30s; X-linked  
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show More pronounced in adulthood; Proximal wkness  
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Conegnital   show
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Facioscapulohumeral   show
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Myotonic   show
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Emery-Dreifus   show
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show Onset 40-50; Wkness of eyelids & throat, facial & limb mm later; Swallowing & keeping eyes open are difficult  
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Distal   show
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show 4 or 5 but parent concern prior to that age  
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Dx of Duchenne   show
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show Generalized proximal wkness; Enlarge calves (pseudohypertrophy); Delayed motor skills (ambulation); Frequent falls; Difficulty getting off floor; (+) Gowers sign  
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Complication- Contractures   show
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show Increased side to side sway  
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Complication- Decreased mobility&   show
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Other complications   show
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Dx Progression   show
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Typical Progression   show
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show Hx/MD notes; Parent assessment; ROM/strength; Posture; Gait; Fxnal mobility; Balance; Motor milestone attainment  
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PT Intervention- Educate family & pt   show
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Treatment areas for concern   show
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show Splint/night splints; Tight gastroc/soleus increases ankle sprain risk leading to gait loss; Increased lumbar lordosis to compensate poor balance; Scoliosis common (affects respiration)  
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Treatment   show
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show Posture; Safety; Allow person being transferred to help as much as possible; Slide boards/discs; Gait belts; Hoyer/track lifts  
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Sensory Integration   show
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Sensory Integration   show
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show Diaphragm breathing; Intercostals; Upper rib cage; Manual assistance; Mechanical ex's latera (cough assist, active breathing cycle, autogenic drainage)  
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show Daily  
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show Resting, Night; AFO's for gait; Serial casting; No intervention; Orthotics; Ankle supports  
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AD's/Equipment   show
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show Steroids (prednisone, deflazicort); Respiratory meds; Pain mgmt; Vitamins (coQ10)  
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SMA- Type I (Acute Childhood)   show
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SMA- Type II (Chronic Childhood)   show
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show First decade onset; Calf pseudohypertophy; Do gait but if sx noted by age 2, most likely use scooter/WC for primary mobility during school age; Vocational planning; Live into adulthood  
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show Brain development- seizures, progressive decreasing cognitions (not decreasing, just not developing); Herat problems; Pulmonary fxn  
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Helping prepare parents for _____ is important but so is what?   show
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