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55 Cards in this Set
- Front
- Back
What are the MCC of rhabdomyolysis? |
1. Nueroletpic malignant syndrome 2. Malignant hyperthermia 3. Crush or blast injuries |
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What is involved in Duchenne's muscular dystrophy? |
1. Cardiac muscle 2. Deltoid/infraspinatus 3. Calves |
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What is the inheritance of Duchenne's? |
1. Sex-linked recessive |
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How do you dx Duchenne's? |
1. 3-5 years-- waddling gait and lordosis 2. Gower's sign 3. Death by 20 |
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What labs can you run for Duchenne's? |
1. CPK extremely elevated (highest of all) 2. EKG--- abnormal in 80% 3. Genetic testing for dystrophin |
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What sex does Becker's affect? |
1. Male--- x-linked recessive |
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What is the onset of Becker's MD? |
1. 5-15 y/o |
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What are the ssx of Becker's? |
1. Same as Duchenne's, but milder |
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What are the lab results for Becker's? |
1. Serum CPK elevated 20-80x 2. ECG abnormal in 30-40% 3. EMG is myopathic |
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What is involved in facioscapulohumeral dystrophy? |
1. Vere severe early weakness and wasting of spinates, trapezius, and pectoralis |
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What is the inheritance of facioscapulohumeral dystrophy? |
1. Autosomal dominant |
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1. Facioscapulohumeral dystrophy |
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What are the lab results in facioscapulohumeral dystrophy? |
1. Serum CK elevated 2. EMG shows it is myopathic 3. Muscle bx shows no pathognomonic findings |
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What is the gene deletion in facioscapulohumeral dystrophy? |
1. 4q35 |
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What is involved in limb-girdle dystrophy? |
1. Hip flexors and glutei weak early in disease 2. Early wasting of medial quadriceps and tibialis anterior 3. Symmetrical proximal weakness, but spares facial muscles |
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What is the inheritance of limb-girdle dystrophy? |
1. AD and AR |
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What are the lab results for limb-girdle dystrophy? |
1. CPK 10x normal 2. Myoglobinuria |
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What is the MC adult MD? |
1. Myotonic dystrophy |
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What is involved in myotonic dystrophy? |
1. Early frontal baldness 2. Cardiac muscle 3. Multisystem |
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What is the inheritance of myotonic MD? On what chromosome is the abnormality found? |
1. AD 2. Chromosome 19 |
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What is anticipation in myotonic MD? |
1. Increasing length of trinucleotide repeat sequence for coding of myotonic protein kinase 2. Shows up earlier and is more severe in later generations |
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What are the clinical features of myotonic MD? |
1. Hatchet faced 2. Distal>proximal weakness 3. Frontal balding 4. Myotonia on examination |
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What are the ssx of myotonic MD in babies? |
1. Baby won't open eyes after getting face washed 2. Have characteristic V shaped mouth |
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What is the first test you should run on a patient with myotonic MD? |
1. Baseline EKG--- look for conduction abnormalities |
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What is deficient in McArdle's disease? |
1. Myophosphorylase |
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What are the ssx of McArdle's disease? |
1. Exercise intolerance 2. Myalgia 3. Cramps 4. **Myoglobinuria, rhabdo, RF |
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What are the lab results of McArdle's disease? |
1. CPK increased 2. K increased 3. Uric acid increased |
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How do you dx McArdle's disease? |
1. Ischemic forearm exercise test 2. Draw samples at regular intervals during exercise 3. No rise in lactic acid |
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What should you avoid in McArdle's disease? |
1. General anesthesia |
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How do you tx McArdle's disease? |
1. Avoid intense exercise 2. Have a candy bar on hand |
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What is deficient in hypokalemic periodic paralysis? |
1. CaV or ligand-gated Na channel |
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What are the ssx of hypokalemic periodic paralysis? |
1. Attacks of paralysis upon awakening or after resting following strenuous exercise 2. Or after a heavy carbohydrate meal |
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What are the lab results in hypokalemic periodic paralysis? |
1. Low serum K 2. Abnormal EMG |
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How do you tx hypokalemic periodic paralysis? |
1. KCL 2. Avoid high carb meals |
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What is deficient in hyperkalemic periodic paralysis? |
1. NaV channels |
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What are the ssx of hyperkalemic periodic paralysis? |
1. Proximal muscle weakness <2 hours after exercise and during fasting |
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What are the lab results in hyperkalemic periodic paralysis? |
1. High serum K |
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How do you prevent hyperkalemic periodic paralysis? |
1. Low K diet 2. Avoid dieting 3. Ingestion of carbs and slight exercise at onset of weakness 4. Thiazide or acetazolamide |
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What is deficient in myotonia congenita? |
1. Chloride channel |
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What are the ssx of myotonia congenita? |
1. Painless muscle stiffness 2. Myotonia at birth--- peculiar cry, difficulty feeding, inability to re-open eyes while having face washed 3. Myotonia worse in cold |
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How do you tx myotonia congenita? |
1. Procainamide 2. Quinidine |
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What is deficient in malignant hypothermia? |
1. Ligand-gated Ca channel |
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What triggers malignant hyperthermia? |
1. Exposure to certain drugs during general anesthesia---> 2. Volateile anesthetic agents 3. Succinylcholine |
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What are the ssx of malignant hyperthermia? |
1. Marked hyperthermia 2. Marked rigidity 3. Tachycardia 4. Tachypnea 5. Hyperkalemia, acidosis, myoglobinuria |
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How do you tx malignant hyperthermia? |
1. Dantrolene (muscle relaxant) 2. Stop anesthesia 3. Start cooling procedures |
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What are the ssx of polymyositis? |
1. No muscle pain +/- minimal muscle tenderness 2. Pain without muscle weakness=polymyalgia rheumatica
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What are the lab results in polymyositis? |
1. Elevated: CPK, aldolase, ESR, and CRP |
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How do you tx polymyositis? |
1. Prednisone 2. Azathioprine 3. Methotrexate |
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What are the ssx of dermatomyositis? |
1. Helitrope rash 2. Symmetric proximal muscle weakness 3. Gorton's papules on knuckles
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What should you check for in dermatomyositis? |
1. CANCER |
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How do you dx dermatomyositis? |
1. Muscle bx |
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How do you tx dermatomyositis? |
1. Prednisone 2. Methotrexate |
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What are the ssx of inclusion body myositis? |
1. Proximal and distal muscle weakness 2. Chronic onset |
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How do you dx inclusion body myositis? |
1. Muscle bx 2. Usually patient thought to have polymyositis but does not respond to steroids |
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What labs should you run for muscle disease? |
1. CPK 2. TFTs 3. ESR 4. CRP 5. EKG |