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| __NOTOC__ | | __NOTOC__ |
| {{Andersen-Tawil syndrome}} | | {{Andersen-Tawil syndrome}} |
| {{CMG}}; {{AE}} {{CP}}; {{RT}} | | {{CMG}}; {{AE}} {{VKG}} |
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| <br />
| | == Overview == |
| ==Overview== | | There is no treatment for [[Andersen-Tawil syndrome|Andersen-Tawil Syndrome]]; the mainstay of therapy is to treat the [[symptoms]] and manage the patient. [[Potassium]] levels play an important role in the management of the [[symptoms]]. |
| There is no treatment for [[Andersen-Tawil syndrome|Andersen-Tawil Syndrome]]; the mainstay of therapy is to treat the [[symptoms]] and manage the patient. [[Potassium]] levels plays an important role in the management of the [[symptoms]]. | |
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| OR
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| Supportive therapy for [disease name] includes [therapy 1], [therapy 2], and [therapy 3].
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| OR
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| The majority of cases of [disease name] are self-limited and require only supportive care.
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| OR
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| [Disease name] is a medical emergency and requires prompt treatment.
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| OR
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| The mainstay of treatment for [disease name] is [therapy].
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| OR The optimal therapy for [malignancy name] depends on the stage at diagnosis.
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| OR
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| [Therapy] is recommended among all patients who develop [disease name].
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| OR
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| Pharmacologic medical therapy is recommended among patients with [disease subclass 1], [disease subclass 2], and [disease subclass 3].
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| OR
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| Pharmacologic medical therapies for [disease name] include (either) [therapy 1], [therapy 2], and/or [therapy 3].
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| OR
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| Empiric therapy for [disease name] depends on [disease factor 1] and [disease factor 2].
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| OR
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| Patients with [disease subclass 1] are treated with [therapy 1], whereas patients with [disease subclass 2] are treated with [therapy 2].
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| ==Medical Therapy== | | ==Medical Therapy== |
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| *Pharmacologic medical therapy is recommended among patients with [disease subclass 1], [disease subclass 2], and [disease subclass 3].
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| * Pharmacologic medical therapies for [disease name] include (either) [therapy 1], [therapy 2], and/or [therapy 3].
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| *Empiric therapy for [disease name] depends on [disease factor 1] and [disease factor 2].
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| *Patients with [disease subclass 1] are treated with [therapy 1], whereas patients with [disease subclass 2] are treated with [therapy 2].
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| ==== Serum potassium management ==== | | ==== Serum potassium management ==== |
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| *Flecainide also helps in reversing tachycardia-induced cardiomyopathy | | *Flecainide also helps in reversing tachycardia-induced cardiomyopathy |
| **Preferred regimen (1): Flecainide 50 mg PO BID, may increase by 50 mg but do not exceed 300 mg/day. | | **Preferred regimen (1): Flecainide 50 mg PO BID, may increase by 50 mg but do not exceed 300 mg/day. |
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| ===Disease Name===
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| *'''1 Stage 1 - Name of stage'''
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| **1.1 '''Specific Organ system involved 1'''
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| ***1.1.1 '''Adult'''
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| ****Preferred regimen (1): [[drug name]] 100 mg PO q12h for 10-21 days '''(Contraindications/specific instructions)'''
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| ****Preferred regimen (2): [[drug name]] 500 mg PO q8h for 14-21 days
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| ****Preferred regimen (3): [[drug name]] 500 mg q12h for 14-21 days
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| ****Alternative regimen (1): [[drug name]] 500 mg PO q6h for 7–10 days
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| ****Alternative regimen (2): [[drug name]] 500 mg PO q12h for 14–21 days
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| ****Alternative regimen (3): [[drug name]] 500 mg PO q6h for 14–21 days
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| ***1.1.2 '''Pediatric'''
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| ****1.1.2.1 (Specific population e.g. '''children < 8 years of age''')
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| *****Preferred regimen (1): [[drug name]] 50 mg/kg PO per day q8h (maximum, 500 mg per dose)
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| *****Preferred regimen (2): [[drug name]] 30 mg/kg PO per day in 2 divided doses (maximum, 500 mg per dose)
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| *****Alternative regimen (1): [[drug name]]10 mg/kg PO q6h (maximum, 500 mg per day)
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| *****Alternative regimen (2): [[drug name]] 7.5 mg/kg PO q12h (maximum, 500 mg per dose)
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| *****Alternative regimen (3): [[drug name]] 12.5 mg/kg PO q6h (maximum, 500 mg per dose)
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| ****1.1.2.2 (Specific population e.g. '<nowiki/>'''''children < 8 years of age'''''')
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| *****Preferred regimen (1): [[drug name]] 4 mg/kg/day PO q12h（maximum, 100 mg per dose）
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| *****Alternative regimen (1): [[drug name]] 10 mg/kg PO q6h (maximum, 500 mg per day)
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| *****Alternative regimen (2): [[drug name]] 7.5 mg/kg PO q12h (maximum, 500 mg per dose)
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| *****Alternative regimen (3): [[drug name]] 12.5 mg/kg PO q6h (maximum, 500 mg per dose)
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| **1.2 '''Specific Organ system involved 2'''
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| ***1.2.1 '''Adult'''
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| ****Preferred regimen (1): [[drug name]] 500 mg PO q8h
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| ***1.2.2 '''Pediatric'''
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| ****Preferred regimen (1): [[drug name]] 50 mg/kg/day PO q8h (maximum, 500 mg per dose)
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| *2 '''Stage 2 - Name of stage'''
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| **2.1 '''Specific Organ system involved 1 '''
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| **:'''Note (1):'''
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| **:'''Note (2)''':
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| **:'''Note (3):'''
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| ***2.1.1 '''Adult'''
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| ****Parenteral regimen
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| *****Preferred regimen (1): [[drug name]] 2 g IV q24h for 14 (14–21) days
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| *****Alternative regimen (1): [[drug name]] 2 g IV q8h for 14 (14–21) days
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| *****Alternative regimen (2): [[drug name]] 18–24 MU/day IV q4h for 14 (14–21) days
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| ****Oral regimen
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| *****Preferred regimen (1): [[drug name]] 500 mg PO q8h for 14 (14–21) days
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| *****Preferred regimen (2): [[drug name]] 100 mg PO q12h for 14 (14–21) days
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| *****Preferred regimen (3): [[drug name]] 500 mg PO q12h for 14 (14–21) days
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| *****Alternative regimen (1): [[drug name]] 500 mg PO q6h for 7–10 days
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| *****Alternative regimen (2): [[drug name]] 500 mg PO q12h for 14–21 days
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| *****Alternative regimen (3):[[drug name]] 500 mg PO q6h for 14–21 days
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| ***2.1.2 '''Pediatric'''
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| ****Parenteral regimen
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| *****Preferred regimen (1): [[drug name]] 50–75 mg/kg IV q24h for 14 (14–21) days (maximum, 2 g)
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| *****Alternative regimen (1): [[drug name]] 150–200 mg/kg/day IV q6–8h for 14 (14–21) days (maximum, 6 g per day)
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| *****Alternative regimen (2): [[drug name]] 200,000–400,000 U/kg/day IV q4h for 14 (14–21) days (maximum, 18–24 million U per day) '<nowiki/>'''''(Contraindications/specific instructions)''''''
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| ****Oral regimen
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| *****Preferred regimen (1): [[drug name]] 50 mg/kg/day PO q8h for 14 (14–21) days (maximum, 500 mg per dose)
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| *****Preferred regimen (2): [[drug name]] '''(for children aged ≥ 8 years)''' 4 mg/kg/day PO q12h for 14 (14–21) days (maximum, 100 mg per dose)
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| *****Preferred regimen (3): [[drug name]] 30 mg/kg/day PO q12h for 14 (14–21) days (maximum, 500 mg per dose)
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| *****Alternative regimen (1): [[drug name]] 10 mg/kg PO q6h 7–10 days (maximum, 500 mg per day)
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| *****Alternative regimen (2): [[drug name]] 7.5 mg/kg PO q12h for 14–21 days (maximum, 500 mg per dose)
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| *****Alternative regimen (3): [[drug name]] 12.5 mg/kg PO q6h for 14–21 days (maximum,500 mg per dose)
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| **2.2 '<nowiki/>'''''Other Organ system involved 2''''''
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| **:'''Note (1):'''
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| **:'''Note (2)''':
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| **:'''Note (3):'''
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| ***2.2.1 '''Adult'''
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| ****Parenteral regimen
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| *****Preferred regimen (1): [[drug name]] 2 g IV q24h for 14 (14–21) days
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| *****Alternative regimen (1): [[drug name]] 2 g IV q8h for 14 (14–21) days
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| *****Alternative regimen (2): [[drug name]] 18–24 MU/day IV q4h for 14 (14–21) days
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| ****Oral regimen
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| *****Preferred regimen (1): [[drug name]] 500 mg PO q8h for 14 (14–21) days
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| *****Preferred regimen (2): [[drug name]] 100 mg PO q12h for 14 (14–21) days
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| *****Preferred regimen (3): [[drug name]] 500 mg PO q12h for 14 (14–21) days
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| *****Alternative regimen (1): [[drug name]] 500 mg PO q6h for 7–10 days
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| *****Alternative regimen (2): [[drug name]] 500 mg PO q12h for 14–21 days
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| *****Alternative regimen (3):[[drug name]] 500 mg PO q6h for 14–21 days
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| ***2.2.2 '''Pediatric'''
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| ****Parenteral regimen
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| *****Preferred regimen (1): [[drug name]] 50–75 mg/kg IV q24h for 14 (14–21) days (maximum, 2 g)
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| *****Alternative regimen (1): [[drug name]] 150–200 mg/kg/day IV q6–8h for 14 (14–21) days (maximum, 6 g per day)
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| *****Alternative regimen (2): [[drug name]] 200,000–400,000 U/kg/day IV q4h for 14 (14–21) days (maximum, 18–24 million U per day)
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| ****Oral regimen
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| *****Preferred regimen (1): [[drug name]] 50 mg/kg/day PO q8h for 14 (14–21) days (maximum, 500 mg per dose)
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| *****Preferred regimen (2): [[drug name]] 4 mg/kg/day PO q12h for 14 (14–21) days (maximum, 100 mg per dose)
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| *****Preferred regimen (3): [[drug name]] 30 mg/kg/day PO q12h for 14 (14–21) days (maximum, 500 mg per dose)
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| *****Alternative regimen (1): [[drug name]] 10 mg/kg PO q6h 7–10 days (maximum, 500 mg per day)
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| *****Alternative regimen (2): [[drug name]] 7.5 mg/kg PO q12h for 14–21 days (maximum, 500 mg per dose)
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| *****Alternative regimen (3): [[drug name]] 12.5 mg/kg PO q6h for 14–21 days (maximum,500 mg per dose)
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| ==Medical Therapy==
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| Management of individuals with ATS requires the coordinated input of a neurologist familiar with the treatment of periodic paralysis and a cardiologist familiar with the treatment of cardiac arrhythmias. To date, no randomized clinical therapeutic trials have been conducted on ATS.
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| Management of attacks of episodic weakness depends on the associated serum potassium concentration:
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| * If the serum potassium concentration is low (<3.0 mmol/L), administration of oral potassium (20-30 mEq/L) every 15-30 minutes until the serum concentration normalizes often shortens the attack. Monitoring of serum potassium concentrations and ECG may be useful during potassium replacement therapy in an emergency setting to avoid secondary hyperkalemia.
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| * Attacks of weakness when serum potassium concentration is high usually resolve within 60 minutes. Episodes may be shortened by ingesting carbohydrates or continuing mild exercise. Intravenous calcium gluconate is rarely necessary for management in an individual seen in an emergency setting.
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| Vasovagal syncope in individuals with ATS mandates a careful cardiology assessment.
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| ==References== | | ==References== |
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| {{WikiDoc Help Menu}} | | {{WikiDoc Help Menu}} |
| {{WikiDoc Sources}} | | {{WikiDoc Sources}} |
| [[CME Category::Cardiology]]
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| [[Category:Electrophysiology]] | | [[Category:Rare Disease]] |
| [[Category:Disease]]
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| [[Category:Genetic disorders]] | | [[Category:Genetic disorders]] |
| [[Category:Cardiology]] | | [[Category:Cardiology]] |