Andersen-Tawil syndrome medical therapy: Difference between revisions

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__NOTOC__
__NOTOC__
{{Andersen-Tawil syndrome}}
{{Andersen-Tawil syndrome}}
{{CMG}}; {{AE}} {{CP}}; {{RT}}
{{CMG}}; {{AE}} {{VKG}}


<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; 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.
 
OR
 
Supportive therapy for [disease name] includes [therapy 1], [therapy 2], and [therapy 3].
 
OR
 
The majority of cases of [disease name] are self-limited and require only supportive care.
 
OR
 
[Disease name] is a medical emergency and requires prompt treatment.
 
OR
 
The mainstay of treatment for [disease name] is [therapy].
 
OR The optimal therapy for [malignancy name] depends on the stage at diagnosis.
 
OR
 
[Therapy] is recommended among all patients who develop [disease name].
 
OR
 
Pharmacologic medical therapy is recommended among patients with [disease subclass 1], [disease subclass 2], and [disease subclass 3].
 
OR
 
Pharmacologic medical therapies for [disease name] include (either) [therapy 1], [therapy 2], and/or [therapy 3].
 
OR
 
Empiric therapy for [disease name] depends on [disease factor 1] and [disease factor 2].
 
OR
 
Patients with [disease subclass 1] are treated with [therapy 1], whereas patients with [disease subclass 2] are treated with [therapy 2].
==Medical Therapy==
==Medical Therapy==
*Pharmacologic medical therapy is recommended among patients with [disease subclass 1], [disease subclass 2], and [disease subclass 3].
* Pharmacologic medical therapies for [disease name] include (either) [therapy 1], [therapy 2], and/or [therapy 3].
*Empiric therapy for [disease name] depends on [disease factor 1] and [disease factor 2].
*Patients with [disease subclass 1] are treated with [therapy 1], whereas patients with [disease subclass 2] are treated with [therapy 2].


==== Serum potassium management ====
==== Serum potassium management ====


*Serum potassium plays an important role in managing the symptoms of the patients with Andersen-Tawil Syndrome.
*[[Serum]] [[potassium]] plays an important role in managing the [[symptoms]] of the patients with [[Andersen-Tawil syndrome|Andersen-Tawil Syndrome]].
*If serum potassium levels are '''<3.0''' mmol/L treat the patient with the following:
*If [[serum]] [[potassium]] levels are '''<3.0''' mmol/L treat the patient with the following:
**Preferred regimen (1): Oral potassium 20-30 mEq/L with the intervals of every 15-30 minutes until the patient reaches the normal levels.
**Preferred regimen (1): Oral [[potassium]] 20-30 mEq/L with the intervals of every 15-30 minutes until the patient reaches the normal levels.
**Physicians who are treating the patient have to keep in mind that anywhere not more than 200 mEq in a 12-hour period is considered to prevent the toxicity.
**'''Specific instructions:'''
***[[Physicians]] who are treating the patient have to keep in mind that anywhere not more than 200 mEq in a 12-hour period is considered to prevent the [[toxicity]].
**Preferred regimen (2): If [[intravenous]] [[potassium]] is considered then a 5% [[mannitol]] solution in the place of a [[saline]] or [[glucose]] solution is recommended
**'''Specific instructions:'''
***Giving [[Intravenous therapy|IV]] [[potassium]] with [[saline]] or [[glucose]] solution leads to worsen the [[Muscle weakness|weakness]].
*While giving the [[potassium]] to a patient it is very important to monitor very closely as to avoid secondary [[hyperkalemia]] which might leads to [[Diastolic|diastolic arrest]].
*If the patient's [[potassium]] levels are high and causes episodic [[paralysis]] it will resolve within an hour.
*High [[potassium]] levels can be managed by treating the patient with the following:
**Preferred regimen (3): [[Intravenous therapy|Intravenous]] [[calcium gluconate]]


===Disease Name===
'''Cardiac manifestations'''


*'''1 Stage 1 - Name of stage'''
*[[Cardiac]] manifestations like [[ventricular arrhythmias]] occurs in patients with [[Andersen-Tawil syndrome|Andersen-Tawil Syndrome]] treat the patient with the following:<ref name="BökenkampWilde2007">{{cite journal|last1=Bökenkamp|first1=Regina|last2=Wilde|first2=Arthur A.|last3=Schalij|first3=Martin J.|last4=Blom|first4=Nico A.|title=Flecainide for recurrent malignant ventricular arrhythmias in two siblings with Andersen-Tawil syndrome|journal=Heart Rhythm|volume=4|issue=4|year=2007|pages=508–511|issn=15475271|doi=10.1016/j.hrthm.2006.12.031}}</ref><ref name="pmid18621769">{{cite journal| author=Fox DJ, Klein GJ, Hahn A, Skanes AC, Gula LJ, Yee RK | display-authors=etal| title=Reduction of complex ventricular ectopy and improvement in exercise capacity with flecainide therapy in Andersen-Tawil syndrome. | journal=Europace | year= 2008 | volume= 10 | issue= 8 | pages= 1006-8 | pmid=18621769 | doi=10.1093/europace/eun180 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18621769 }}</ref>
**1.1 '''Specific Organ system involved 1'''
***1.1.1 '''Adult'''
****Preferred regimen (1): [[drug name]] 100 mg PO q12h for 10-21 days '''(Contraindications/specific instructions)'''
****Preferred regimen (2): [[drug name]] 500 mg PO q8h for 14-21 days
****Preferred regimen (3): [[drug name]] 500 mg q12h for 14-21 days
****Alternative regimen (1): [[drug name]] 500 mg PO q6h for 7–10 days
****Alternative regimen (2): [[drug name]] 500 mg PO q12h for 14–21 days
****Alternative regimen (3): [[drug name]] 500 mg PO q6h for 14–21 days
***1.1.2 '''Pediatric'''
****1.1.2.1 (Specific population e.g. '''children < 8 years of age''')
*****Preferred regimen (1): [[drug name]] 50 mg/kg PO per day q8h (maximum, 500 mg per dose)
*****Preferred regimen (2): [[drug name]] 30 mg/kg PO per day in 2 divided doses (maximum, 500 mg per dose)
*****Alternative regimen (1): [[drug name]]10 mg/kg PO q6h (maximum, 500 mg per day)
*****Alternative regimen (2): [[drug name]] 7.5 mg/kg PO q12h (maximum, 500 mg per dose)
*****Alternative regimen (3): [[drug name]] 12.5 mg/kg PO q6h (maximum, 500 mg per dose)
****1.1.2.2 (Specific population e.g. '<nowiki/>'''''children < 8 years of age'''''')
*****Preferred regimen (1): [[drug name]] 4 mg/kg/day PO q12h（maximum, 100 mg per dose）
*****Alternative regimen (1): [[drug name]] 10 mg/kg PO q6h (maximum, 500 mg per day)
*****Alternative regimen (2): [[drug name]] 7.5 mg/kg PO q12h (maximum, 500 mg per dose)
*****Alternative regimen (3): [[drug name]] 12.5 mg/kg PO q6h (maximum, 500 mg per dose)
**1.2 '''Specific Organ system involved 2'''
***1.2.1 '''Adult'''
****Preferred regimen (1): [[drug name]] 500 mg PO q8h
***1.2.2 '''Pediatric'''
****Preferred regimen (1): [[drug name]] 50 mg/kg/day PO q8h (maximum, 500 mg per dose)


*2 '''Stage 2 - Name of stage'''
==== Flecainide ====
**2.1 '''Specific Organ system involved 1 '''
**:'''Note (1):'''
**:'''Note (2)''':
**:'''Note (3):'''
***2.1.1 '''Adult'''
****Parenteral regimen
*****Preferred regimen (1): [[drug name]] 2 g IV q24h for 14 (14–21) days
*****Alternative regimen (1): [[drug name]] 2 g IV q8h for 14 (14–21) days
*****Alternative regimen (2): [[drug name]] 18–24 MU/day IV q4h for 14 (14–21) days
****Oral regimen
*****Preferred regimen (1): [[drug name]] 500 mg PO q8h for 14 (14–21) days
*****Preferred regimen (2): [[drug name]] 100 mg PO q12h for 14 (14–21) days
*****Preferred regimen (3): [[drug name]] 500 mg PO q12h for 14 (14–21) days
*****Alternative regimen (1): [[drug name]] 500 mg PO q6h for 7–10 days
*****Alternative regimen (2): [[drug name]] 500 mg PO q12h for 14–21 days
*****Alternative regimen (3):[[drug name]] 500 mg PO q6h for 14–21 days
***2.1.2 '''Pediatric'''
****Parenteral regimen
*****Preferred regimen (1): [[drug name]] 50–75 mg/kg IV q24h for 14 (14–21) days (maximum, 2 g)
*****Alternative regimen (1): [[drug name]] 150–200 mg/kg/day IV q6–8h for 14 (14–21) days (maximum, 6 g per day)
*****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)''''''
****Oral regimen
*****Preferred regimen (1):  [[drug name]] 50 mg/kg/day PO q8h for 14 (14–21) days  (maximum, 500 mg per dose)
*****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)
*****Preferred regimen (3): [[drug name]] 30 mg/kg/day PO q12h for 14 (14–21) days  (maximum, 500 mg per dose)
*****Alternative regimen (1):  [[drug name]] 10 mg/kg PO q6h 7–10 days  (maximum, 500 mg per day)
*****Alternative regimen (2): [[drug name]] 7.5 mg/kg PO q12h for 14–21 days  (maximum, 500 mg per dose)
*****Alternative regimen (3): [[drug name]] 12.5 mg/kg PO q6h for 14–21 days  (maximum,500 mg per dose)
**2.2  '<nowiki/>'''''Other Organ system involved 2''''''
**:'''Note (1):'''
**:'''Note (2)''':
**:'''Note (3):'''
***2.2.1 '''Adult'''
****Parenteral regimen
*****Preferred regimen (1): [[drug name]] 2 g IV q24h for 14 (14–21) days
*****Alternative regimen (1): [[drug name]] 2 g IV q8h for 14 (14–21) days
*****Alternative regimen (2): [[drug name]] 18–24 MU/day IV q4h for 14 (14–21) days
****Oral regimen
*****Preferred regimen (1): [[drug name]] 500 mg PO q8h for 14 (14–21) days
*****Preferred regimen (2): [[drug name]] 100 mg PO q12h for 14 (14–21) days
*****Preferred regimen (3): [[drug name]] 500 mg PO q12h for 14 (14–21) days
*****Alternative regimen (1): [[drug name]] 500 mg PO q6h for 7–10 days
*****Alternative regimen (2): [[drug name]] 500 mg PO q12h for 14–21 days
*****Alternative regimen (3):[[drug name]] 500 mg PO q6h for 14–21 days
***2.2.2 '''Pediatric'''
****Parenteral regimen
*****Preferred regimen (1): [[drug name]] 50–75 mg/kg IV q24h for 14 (14–21) days (maximum, 2 g)
*****Alternative regimen (1): [[drug name]] 150–200 mg/kg/day IV q6–8h for 14 (14–21) days (maximum, 6 g per day)
*****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)
****Oral regimen
*****Preferred regimen (1):  [[drug name]] 50 mg/kg/day PO q8h for 14 (14–21) days  (maximum, 500 mg per dose)
*****Preferred regimen (2): [[drug name]] 4 mg/kg/day PO q12h for 14 (14–21) days (maximum, 100 mg per dose)
*****Preferred regimen (3): [[drug name]] 30 mg/kg/day PO q12h for 14 (14–21) days  (maximum, 500 mg per dose)
*****Alternative regimen (1):  [[drug name]] 10 mg/kg PO q6h 7–10 days  (maximum, 500 mg per day)
*****Alternative regimen (2): [[drug name]] 7.5 mg/kg PO q12h for 14–21 days  (maximum, 500 mg per dose)
*****Alternative regimen (3): [[drug name]] 12.5 mg/kg PO q6h for 14–21 days  (maximum,500 mg per dose)


==Medical Therapy==
* Flecainide should be considered especially in patients who are prone to more frequent ventricular arrhythmias with reduced left ventricular function
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.


Management of attacks of episodic weakness depends on the associated serum potassium concentration:
*Flecainide is very potent anti arrhythmic which helps with suppressing bidirectional ventricular tachycardia (BVT)<ref name="pmid17655675">{{cite journal| author=Pellizzón OA, Kalaizich L, Ptácek LJ, Tristani-Firouzi M, Gonzalez MD| title=Flecainide suppresses bidirectional ventricular tachycardia and reverses tachycardia-induced cardiomyopathy in Andersen-Tawil syndrome. | journal=J Cardiovasc Electrophysiol | year= 2008 | volume= 19 | issue= 1 | pages= 95-7 | pmid=17655675 | doi=10.1111/j.1540-8167.2007.00910.x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17655675  }}</ref>
* 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.
*Flecainide also helps in reversing tachycardia-induced cardiomyopathy
* 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.
**Preferred regimen (1): Flecainide 50 mg PO BID, may increase by 50 mg but do not exceed 300 mg/day.


Vasovagal syncope in individuals with ATS mandates a careful cardiology assessment.


==References==
==References==
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Latest revision as of 14:48, 17 February 2020

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Vamsikrishna Gunnam M.B.B.S [2]

Overview

There is no treatment for 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.

Medical Therapy

Serum potassium management

Cardiac manifestations

Flecainide

  • Flecainide should be considered especially in patients who are prone to more frequent ventricular arrhythmias with reduced left ventricular function
  • Flecainide is very potent anti arrhythmic which helps with suppressing bidirectional ventricular tachycardia (BVT)[3]
  • 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.


References

  1. ↑ Bökenkamp, Regina; Wilde, Arthur A.; Schalij, Martin J.; Blom, Nico A. (2007). "Flecainide for recurrent malignant ventricular arrhythmias in two siblings with Andersen-Tawil syndrome". Heart Rhythm. 4 (4): 508–511. doi:10.1016/j.hrthm.2006.12.031. ISSN 1547-5271.
  2. ↑ Fox DJ, Klein GJ, Hahn A, Skanes AC, Gula LJ, Yee RK; et al. (2008). "Reduction of complex ventricular ectopy and improvement in exercise capacity with flecainide therapy in Andersen-Tawil syndrome". Europace. 10 (8): 1006–8. doi:10.1093/europace/eun180. PMID 18621769.
  3. ↑ Pellizzón OA, Kalaizich L, Ptácek LJ, Tristani-Firouzi M, Gonzalez MD (2008). "Flecainide suppresses bidirectional ventricular tachycardia and reverses tachycardia-induced cardiomyopathy in Andersen-Tawil syndrome". J Cardiovasc Electrophysiol. 19 (1): 95–7. doi:10.1111/j.1540-8167.2007.00910.x. PMID 17655675.


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