Andersen-Tawil syndrome medical therapy: Difference between revisions

Jump to navigation Jump to search
 
(5 intermediate revisions by the same user not shown)
Line 1: Line 1:
__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|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 ====
Line 68: Line 24:
'''Cardiac manifestations'''
'''Cardiac manifestations'''


*[[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>
*[[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>
*Flecainide: Flecainide should be considered especially in patients who are prone to more frequent ventricular arrhythmias with reduced left ventricular function
**Preferred regimen (1): Flecainide 50 mg PO BID, may increase by 50 mg but do not exceed 300 mg/day.
*


===Disease Name===
==== Flecainide ====


*'''1 Stage 1 - Name of stage'''
* Flecainide should be considered especially in patients who are prone to more frequent ventricular arrhythmias with reduced left ventricular function
**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 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>
**2.1 '''Specific Organ system involved 1 '''
*Flecainide also helps in reversing tachycardia-induced cardiomyopathy
**:'''Note (1):'''
**Preferred regimen (1): Flecainide 50 mg PO BID, may increase by 50 mg but do not exceed 300 mg/day.
**:'''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==
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:
* 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.
* 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.


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


==References==
==References==
Line 174: Line 40:
{{WikiDoc Help Menu}}
{{WikiDoc Help Menu}}
{{WikiDoc Sources}}
{{WikiDoc Sources}}
[[CME Category::Cardiology]]


[[Category:Electrophysiology]]
[[Category:Rare Disease]]
[[Category:Disease]]
[[Category:Genetic disorders]]
[[Category:Genetic disorders]]
[[Category:Cardiology]]
[[Category:Cardiology]]

Latest revision as of 14:48, 17 February 2020

Andersen-Tawil syndrome Microchapters

Home

Patient Information

Overview

Historical Perspective

Classification

Pathophysiology

Differentiating Andersen-Tawil syndrome from other Diseases

Epidemiology and Demographics

Screening

Natural History, Complications and Prognosis

Diagnosis

Diagnostic Criteria

History and Symptoms

Physical Examination

Laboratory Findings

Electrocardiogram

Chest X Ray

CT

MRI

Echocardiography or Ultrasound

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Primary Prevention

Secondary Prevention

Tertiary Prevention

Cost-Effectiveness of Therapy

Future or Investigational Therapies

Case Studies

Case #1

Andersen-Tawil syndrome medical therapy On the Web

Most recent articles

Most cited articles

Review articles

CME Programs

slides

Images

American Roentgen Ray Society Images of Andersen-Tawil syndrome medical therapy

All Images
X-rays
Echo & Ultrasound
CT Images
MRI

Ongoing Trials at Clinical Trials.gov

US National Guidelines Clearinghouse

NICE Guidance

FDA on Andersen-Tawil syndrome medical therapy

CDC on Andersen-Tawil syndrome medical therapy

Andersen-Tawil syndrome medical therapy in the news

Blogs on Andersen-Tawil syndrome medical therapy

Directions to Hospitals Treating Andersen-Tawil syndrome

Risk calculators and risk factors for Andersen-Tawil syndrome medical therapy

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.


Template:WikiDoc Sources