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11 Cards in this Set
- Front
- Back
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Characterize Duchennes Muscular Dystrophy.
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Deterioration of muscle secondary to absence of structural dystrophin gene. Characterized by pseuodhypertrophy, and progressive muscle weakness. Without treatment patients die in 20's from cardiac or respiratory problems.
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Desicribe the typical DMD patient.
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Starts at birth. Dx made by 5-6 years of age.
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What is Beckers dysrophy?
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A milder form of DMD in which patients do not experience as rapid a rate of decline. Abnormal Biopsy.
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What laboratory abnormalities are observed if any at all in Duchennes Muscular Dystrophy?
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Elevations of Creatine Kinase (20-30,000)
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How is DMD treated?
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Prednisone. Allows muscle fibers to last longer due to supression of inflammatory macrophage response following myofiber damage.
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Characterize the pathology of myotonic dystrophy.
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Auo-dom dz characterized by inability to relax muscle typically presenting distally (hands and feet)
Patients characteristically show a steppage gait. |
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How is Myasthenia Gravis treated?
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Acetylchonlinesterase blockers.
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What is the pathology underlying Myasthenia Gravis?
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Immune mediated response to a viral trigger producing antibodies that bind acetylcholine receptor sites. Thus MG is characterized by a progressive decline of ACH Receptors.
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Discuss symptoms typically associated with Myasthenia Gravis.
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fatigable weakness in which patient utilization of muscle facilitates weakness.
Patients may hold their mouths closed or have difficulty closing their eyes. |
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What is the mode of DMD inheritance?
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X-linked recessive
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Differentiate Polymyositis vs. Dermatomyositis.
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Rash. Both presesent with proximal weakness and elevated CK and are immune mediated. However, dermatomyositis manifests with maccular rash.
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