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GALE ENCYCLOPEDIA OF GENETIC DISORDERS 777 Myasthenia Gravis Familial Familial inheritance of Myasthenia gravis cheap ciplox 500 mg free shipping. Heat 500 mg ciplox overnight delivery, including heat from the sun discount ciplox 500mg without prescription, hot botulism, and inherited muscular dystrophies. MG causes characteristic changes in the electrical Infection and stress may worsen symptoms. Symptoms responses of muscles that may be observed with an elec- may vary from day to day and month to month, with tromyogram, which measures muscular response to elec- intervals of no weakness interspersed with a progressive trical stimulation. Symptoms include weak and shallow breathing, body to the acetylcholine receptor, though up to a quarter shortness of breath, pale or bluish skin color, and a rac- of MG patients will not have detectable levels. Myasthenic crisis is an emergency condition ray or chest computed tomography scan (CT scan) may requiring immediate treatment. Treatment and management While there is no cure for myasthenia gravis, there Pregnancy worsens MG in about one third of are a number of treatments that effectively control symp- women, has no effect in one third, and improves symp- toms in most people. About 12% of infants born to women with MG have neonatal myasthenia, a temporary Edrophonium (Tensilon) blocks the action of acetyl- but potentially life-threatening condition. It is caused by cholinesterase, prolonging the effect of acetylcholine and the transfer of maternal antibodies into the fetal circula- increasing strength. Symptoms include weakness, poor leads to a marked improvement in most people with MG. Loss of responsiveness and disease Diagnosis progression combine to eventually make pyridostigmine ineffective in tolerable doses in many patients. Myasthenia gravis is often diagnosed accurately by a careful medical history and a neuromuscular exam, but Thymectomy, or removal of the thymus gland, has several tests are used to confirm the diagnosis. Up to ditions causing worsening of bulbar and skeletal muscles 85% of people with MG improve after thymectomy, with must be considered, including drug-induced myasthenia, complete remission eventually seen in about 30%. The 778 GALE ENCYCLOPEDIA OF GENETIC DISORDERS Myasthenia Gravis Sporadic Sporatic occurance of Myathenia gravis in a family. Drugs include corticosteroids such as prednisone, A Medic-Alert card or bracelet provides an impor- and the non-steroids azathioprine (Imuran) and tant source of information to emergency providers about cyclosporine (Sandimmune). Plasma exchange may be performed to treat myas- thenic crisis or to improve very weak patients before thymectomy. In this procedure, blood plasma is Prognosis removed and replaced with purified plasma free of autoantibodies. It can produce a temporary improve- Most people with MG can be treated successfully ment in symptoms, but is too expensive for long-term enough to prevent their condition from becoming debili- treatment. In some cases, however, symptoms may worsen immunoglobulin therapy, is also used for myasthenic even with vigorous treatment, leading to generalized crisis. There is no known way to unknown reasons, this leads to symptomatic improve- prevent myasthenia gravis. It is also too expensive toms significantly in many patients, and relieves them for long-term treatment. Avoiding heat can help minimize symp- People with weakness of the bulbar muscles may toms. In more severe cases, it may be necessary to obtain Resources nutrition through a feeding tube placed into the stomach BOOKS (gastrostomy tube). Neuromuscular Some drugs should be avoided by people with MG Diseases: A Practical Approach to Diagnosis and Man- because they interfere with normal neuromuscular func- agement. Drugs to be avoided or used with caution include: PERIODICALS • Many types of antibiotics, including erythromycin, Drachman, D. GALE ENCYCLOPEDIA OF GENETIC DISORDERS 779 ORGANIZATIONS There are many types of myopia. By far the most common form, physiologic myopia WEBSITES Immune Deficiency Foundation. This condition begins as physiologic myopia, but rather than stabilizing, the eye continues to enlarge at an abnor- Catherine L. This more advanced type of myopia may lead to degenerative changes in the eye (degenerative myopia).

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Typical inf dose: 2–20 µg/kg/min Dopamine (Intropin) INDICATIONS: Second line for symptomatic bradycardia quality ciplox 500 mg. Hypotension (BP <70–100 mm Hg) with signs of symptoms of shock SUPPLIED: 40 mg/mL or 160 mg/mL buy 500mg ciplox mastercard. Initial purchase 500 mg ciplox mastercard, 5–10 µg/kg/min; typical: 2–20 µg/kg/min Note: If >20 µg/kg/min is required, consider use of alternative adrenergic agent (eg, epinephrine) Epinephrine INDICATIONS: Cardiac arrest: VF, pulseless VT, asystole, PEA. Anaphylaxis, severe allergic reactions: Combine with large fluid volumes, corticosteroids, antihistamines. Inf: 30 mg epinephrine (30 mL of 1:1000 solution) to 250 mL NS or D5W, run at 100 mL/h, titrate. Profound bradycardia/hy- potension: 2–10 µg/min (1 mg of 1:1000 in 500 mL NS, infuse 1–5 mL/min). Do NOT use with history of active bleeding or surgery within 30 d or if platelets <150,000/mm3. Continue 12 IU/kg/h (max 1000 IU/h for patients >70 kg) round to the nearest 50 IU. Heparin (Low Molecular Weight) (Fragmin, Lovenox) INDICATIONS: ACS with non-Q wave or unstable angina SUPPLIED: Dalteparin (Fragmin), Enoxaparin (Lovenox) DOSAGE: 1 mg/kg bid SQ for 2–8 d with aspirin Ibutilide INDICATIONS: Supraventricular arrhythmias (AFiB, A flutter); short-acting SUPPLIED: 1 mg/10 mL DOSAGE: 1 mg IV over 10 min (if <60 kg 0. Stable VT, wide-complex tachycardias of uncertain type, wide-complex PSVT SUPPLIED: 20 mg/mL in preloaded 5-mL syringe, 10 mg/mL in 5-mL vial. Perfusing arrhythmia: For stable VT, wide-complex tachycardia or uncertain type, signifi- cant ectopy, use as follows: 1. Maintenance inf: 1–4 mg/min (30–50 µg/min) Magnesium Sulfate INDICATIONS: Cardiac arrest associated with torsades de pointes or suspected hypomagnesemic state, refractory VF, life-threatening ventricular arrhythmias due to digitalis toxicity, tricyclic over- dose. Torsades de pointes: Loading dose of 1–2 g mixed in 50–100 mL of D5W, over 5–60 min IV. Mannitol INDICATIONS: Increased intracranial pressure in management of neurologic emergencies SUPPLIED: 150-, 250-, and 1000-mL IV containers (strengths: 5%, 10%, 15%, 20%, and 25%). Morphine Sulfate INDICATIONS: Chest pain and anxiety associated with AMI or cardiac ischemia, acute cardiogenic pulmonary edema (if blood pressure is adequate) SUPPLIED: 2–10 mg/mL in a 1-mL syringe DOSAGE: Adults. Bolus IV dose: For total reversal of narcotic effects (smaller doses may be used if total reversal not required), as fol- lows: Birth–5 y (≤ 10 kg): 0. Use infusion pump; hemodynamic monitor- ing for optimal safety Norepinephrine INDICATIONS: Severe cardiogenic shock and significant hypotension. Procainamide (Pronestyl) INDICATIONS: Recurrent VT not controlled by lidocaine, refractory PSVT, refractory VF/pulseless VT, stable wide-complex tachycardia of unknown origin, AF with rapid rate in WPW SUPPLIED: 100 mg/mL in 10-mL vial, 500 mg/mL in 2-mL vial DOSAGE: Adults. Other indications: 20 mg/min IV until one of the following occurs: arrhythmia suppression, hypotension, QRS widens by more than 50%, total dose of 17 mg/kg is given. Maintenance: 1–4 mg/min Sodium Bicarbonate INDICATIONS: Specific indications for bicarbonate use are as follows: Class I (usually indicated) if known preexisting hyperkalemia. Class IIa (accepted, possibly controversial) if known preexisting bicarbonate-responsive acidosis (eg, DKA); tricyclic antidepressant overdose; alkalinize urine in aspirin overdose. Class IIb (accepted, but may not help, probably not harmful) if prolonged resusci- tation with effective ventilation; on return of spontaneous circulation after long arrest interval. Class III (harmful) in hypoxic lactic acidosis (eg, cardiac arrest and CPR without intubation) SUPPLIED: 50-mL syringe (8. Sodium Bicarbonate (Pediatric) INDICATIONS: Severe metabolic acidosis (documented or following prolonged arrest) unresponsive to oxygenation and hyperventilation, hyperkalemia, tricyclic antidepressant toxicity DOSAGE: Adults. Thrombolytic Agents (Fibrinolytic Agents) INDICATIONS: AMI in adults: ST-segment elevation of 1 mm or more in at least two contiguous leads in the setting of AMI. NS flush before and after each bolus Verapamil (Colan, Isoptin) INDICATIONS: Second line for PSVT with narrow QRS complex and adequate BP SUPPLIED: 2. Alternative: 5 mg bolus every 15 min to total dose of 30 mg Electrical Defibrillation and Cardioversion Although the defibrillator is the basic piece of equipment for both defibrillation and car- dioversion, they are two distinctly different procedures. New devices include shock advisory defibrillators (automated external defibrillators). Shout “Charging defibrillator-stand clear,” synchronization switch off (if on, the de- fibrillator may not fire).

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