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| bgcolor="LemonChiffon" |In patients with asymptomatic Brugada syndrome and a spontaneous type 1 Brugada electrocardiographic pattern, an electrophysiological study with programmed ventricular stimulation using single and double extrastimuli may be considered for further risk stratification.<ref name="pmid27491905" /><ref name="pmid26797467" /><ref name="pmid20978016">{{cite journal |vauthors=Delise P, Allocca G, Marras E, Giustetto C, Gaita F, Sciarra L, Calo L, Proclemer A, Marziali M, Rebellato L, Berton G, Coro L, Sitta N |title=Risk stratification in individuals with the Brugada type 1 ECG pattern without previous cardiac arrest: usefulness of a combined clinical and electrophysiologic approach |journal=Eur. Heart J. |volume=32 |issue=2 |pages=169–76 |date=January 2011 |pmid=20978016 |pmc=3021386 |doi=10.1093/eurheartj/ehq381 |url=}}</ref><ref name="KusumotoBailey2018">{{cite journal|last1=Kusumoto|first1=Fred M.|last2=Bailey|first2=Kent R.|last3=Chaouki|first3=Ahmad Sami|last4=Deshmukh|first4=Abhishek J.|last5=Gautam|first5=Sandeep|last6=Kim|first6=Robert J.|last7=Kramer|first7=Daniel B.|last8=Lambrakos|first8=Litsa K.|last9=Nasser|first9=Naseer H.|last10=Sorajja|first10=Dan|title=Systematic Review for the 2017 AHA/ACC/HRS Guideline for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death|journal=Circulation|volume=138|issue=13|year=2018|issn=0009-7322|doi=10.1161/CIR.0000000000000550}}</ref><ref name="pmid25904495">{{cite journal |vauthors=Sieira J, Conte G, Ciconte G, de Asmundis C, Chierchia GB, Baltogiannis G, Di Giovanni G, Saitoh Y, Irfan G, Casado-Arroyo R, Juliá J, La Meir M, Wellens F, Wauters K, Van Malderen S, Pappaert G, Brugada P |title=Prognostic value of programmed electrical stimulation in Brugada syndrome: 20 years experience |journal=Circ Arrhythm Electrophysiol |volume=8 |issue=4 |pages=777–84 |date=August 2015 |pmid=25904495 |doi=10.1161/CIRCEP.114.002647 |url=}}</ref>
| bgcolor="LemonChiffon" |In patients with asymptomatic Brugada syndrome and a spontaneous type 1 Brugada electrocardiographic pattern, an electrophysiological study with programmed ventricular stimulation using single and double extrastimuli may be considered for further risk stratification.<ref name="pmid27491905" /><ref name="pmid26797467" /><ref name="pmid20978016">{{cite journal |vauthors=Delise P, Allocca G, Marras E, Giustetto C, Gaita F, Sciarra L, Calo L, Proclemer A, Marziali M, Rebellato L, Berton G, Coro L, Sitta N |title=Risk stratification in individuals with the Brugada type 1 ECG pattern without previous cardiac arrest: usefulness of a combined clinical and electrophysiologic approach |journal=Eur. Heart J. |volume=32 |issue=2 |pages=169–76 |date=January 2011 |pmid=20978016 |pmc=3021386 |doi=10.1093/eurheartj/ehq381 |url=}}</ref><ref name="KusumotoBailey2018">{{cite journal|last1=Kusumoto|first1=Fred M.|last2=Bailey|first2=Kent R.|last3=Chaouki|first3=Ahmad Sami|last4=Deshmukh|first4=Abhishek J.|last5=Gautam|first5=Sandeep|last6=Kim|first6=Robert J.|last7=Kramer|first7=Daniel B.|last8=Lambrakos|first8=Litsa K.|last9=Nasser|first9=Naseer H.|last10=Sorajja|first10=Dan|title=Systematic Review for the 2017 AHA/ACC/HRS Guideline for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death|journal=Circulation|volume=138|issue=13|year=2018|issn=0009-7322|doi=10.1161/CIR.0000000000000550}}</ref><ref name="pmid25904495">{{cite journal |vauthors=Sieira J, Conte G, Ciconte G, de Asmundis C, Chierchia GB, Baltogiannis G, Di Giovanni G, Saitoh Y, Irfan G, Casado-Arroyo R, Juliá J, La Meir M, Wellens F, Wauters K, Van Malderen S, Pappaert G, Brugada P |title=Prognostic value of programmed electrical stimulation in Brugada syndrome: 20 years experience |journal=Circ Arrhythm Electrophysiol |volume=8 |issue=4 |pages=777–84 |date=August 2015 |pmid=25904495 |doi=10.1161/CIRCEP.114.002647 |url=}}</ref>
|-
|-
| bgcolor="LemonChiffon" |In patients with suspected or established Brugada syndrome, genetic counseling and genetic testing may be useful to facilitate cascade screening of relatives.<ref name="pmid22840528">{{cite journal |vauthors=Crotti L, Marcou CA, Tester DJ, Castelletti S, Giudicessi JR, Torchio M, Medeiros-Domingo A, Simone S, Will ML, Dagradi F, Schwartz PJ, Ackerman MJ |title=Spectrum and prevalence of mutations involving BrS1- through BrS12-susceptibility genes in a cohort of unrelated patients referred for Brugada syndrome genetic testing: implications for genetic testing |journal=J. Am. Coll. Cardiol. |volume=60 |issue=15 |pages=1410–8 |date=October 2012 |pmid=22840528 |pmc=3624764 |doi=10.1016/j.jacc.2012.04.037 |url=}}</ref>
| bgcolor="LemonChiffon" |In patients with suspected or established Brugada syndrome, genetic counseling and genetic testing may be useful to facilitate cascade screening of relatives.<ref name="pmid22840528">{{cite journal |vauthors=Crotti L, Marcou CA, Tester DJ, Castelletti S, Giudicessi JR, Torchio M, Medeiros-Domingo A, Simone S, Will ML, Dagradi F, Schwartz PJ, Ackerman MJ |title=Spectrum and prevalence of mutations involving BrS1- through BrS12-susceptibility genes in a cohort of unrelated patients referred for Brugada syndrome genetic testing: implications for genetic testing |journal=J. Am. Coll. Cardiol. |volume=60 |issue=15 |pages=1410–8 |date=October 2012 |pmid=22840528 |pmc=3624764 |doi=10.1016/j.jacc.2012.04.037 |url=}}</ref><ref name="pmid23414114">{{cite journal |vauthors=Risgaard B, Jabbari R, Refsgaard L, Holst AG, Haunsø S, Sadjadieh A, Winkel BG, Olesen MS, Tfelt-Hansen J |title=High prevalence of genetic variants previously associated with Brugada syndrome in new exome data |journal=Clin. Genet. |volume=84 |issue=5 |pages=489–95 |date=November 2013 |pmid=23414114 |doi=10.1111/cge.12126 |url=}}</ref>
|}
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Revision as of 19:54, 6 January 2020

Brugada syndrome Microchapters

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Examples of Type I Brugada Syndrome

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Implantation of a cardiac defibrillator is the only proven method of treatment in Brugada syndrome.Patients with aborted sudden cardiac death are at high risk for recurrence and should undergo AICD implantation, and do not require an electrophysiologic study to assess inducibility. Patients with symptoms (either syncope, seizures or nocturnal agonal respirations) should undergo implantation of a defibrillator if no other cause of their symptoms can be identified. Asymptomatic patients should undergo electrophysiologic testing, and if VT / VF can be induced, they should undergo implantation of an ICD. Asymptomatic patients who cannot be induced should followed-up closely. Patients who are asymptomatic with no family history of Brugada syndrome can be followed-up closely.

The Two Patient Groups

The 2005 consensus statement divides patients into two groups:

  • Higher risk patients with spontaneous Type I Brugada pattern
  • A less high risk cohort of patients who require infusion of a sodium channel blocker to induce a Type I Brugada pattern.

The management of these two groups of patients will be discussed separately.

Management of Patients with a Spontaneous Type I Brugada Pattern

Implantation of a cardiac defibrillator should be considered in the following patients:

Symptomatic Patients

The flowchart below summarizes the recommendations of the 2005 consensus panel:

Asymptomatic Patients

  • Patients with a family history of sudden cardiac death that is suspected to be due to Brugada syndrome in whom VT VF can be induced on electrophysiologic testing.
  • Patients with no family history of sudden cardiac death in whom VT VF can be induced on electrophysiologic testing.

In essence, if VT VF can be induced on electrophysiologic testing in these patients, a cardiac defibrillator should be implanted. It is unclear if the same recommendations apply to those patients who require that the electrodes be placed one to two intercostal spaces higher to demonstrate a Brugada type I electrocardiographic pattern.

The flowchart below summarizes the recommendations of the 2005 consensus panel:

Management of Patients with a Sodium Channel Induced Type I Brugada Pattern

Implantation of a cardiac defibrillator should be considered in the following patients:

Symptomatic Patients

The flowchart below summarizes the recommendations of the 2005 consensus panel:

Asymptomatic Patients

  • Patients with a family history of sudden cardiac death that is suspected to be due to Brugada syndrome in whom VT VF can be induced on electrophysiologic testing.

The flowchart below summarizes the recommendations of the 2005 consensus panel.

Pharmacotherapy

  • Pharmacotherapy alone may not be sufficient to treat Brugada syndrome, but it may be required in regions of the world where ICD implantation is cost prohibitive or in infants.[1]
  • Quinidine reduces the number of VF episodes and corrects spontaneous ECG changes, possibly via inhibiting I(to) channels. No drug has demonstrated long term efficacy in the prevention of sudden cardiac death.[2][3][4][5][6][7]
  • Pharmacological tests using a sodium-channel blocker drug should be used to evaluate suspected Brugada syndrome. Intravenous ajmaline and flecainide are the most widely used agents. The test is considered positive if a type 1 ECG pattern is identified during drug infusion.


Drugs with Potential Antiarrhythmic Effect

(Alphabetical order generic name)

Generic name Brand name® Class / Clinical use References Recommendation
Cilostazol e.g.
Pletal® 		Phosphodiesterase inhibitor Tsuchiya 2002
Abud 2006
Matsui 1999 		Class IIb
Isoproterenol
Isoprenaline 		e.g.
Isuprel® 		Beta-adrenergic receptor stimulation Miyazaki 1996
Suzuki 2000
Watanabe 2006
Ohgo 2007
Ganesan 2006 		Class I
Orciprenaline e.g.
Alotec®
Metaprel®
Novasmasol® 		Beta-adrenergic receptor stimulation Kyriazis 2009 Class IIa
Quinidine e.g.
Quinalan®
Chinidin® 		Antiarrhythmic Agent Suzuki 2000
Alings 2001
Belhassen 2004
Mizusawa 2006
Probst 2007
Ohgo 2007
Yan 1999 		Class I
  • Recommendation: Class I: convincing evidence/opinion; Class IIa: evidence/opinion less clear; Class IIb: conflicting evidence/opinion; Class III: very little evidence.

Treatment of VT Storm

  • VT storm has been successfully treated with isoproterenol. The mechanism is thought to be augmenting the cardiac L type channel.

Treatment of Coronary Ischemia

Treatment of Factors that may Precipitate Brugada Type EKG Changes and Clinical Symptoms

  • Fever in a Brugada syndrome patient should be treated with an antipyretic.
  • Brugada syndrome patients should avoid hot tubs, very hot baths or extremely hot climates.
  • Hypokalemia, hyperkalemia, and hypercalcemia should be treated aggressively.
  • Carbohydrate loading should be avoided.

Contraindicated medications

Brugada syndrome is considered an absolute contraindication to the use of the following medications:

ACC/AHA/ESC 2006 Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death (DO NOT EDIT) [8]

Recommendations for Brugada Syndrome

Class I
Suggested phrases for writing recommendations:

1. In asymptomatic patients with only inducible type 1 Brugada electrocardiographic pattern, observation without therapy is recommended.[9][10]

2. In patients with Brugada syndrome with spontaneous type 1 Brugada electrocardiographic pattern and cardiac arrest, sustained VA or a recent history of syncope presumed due to VA, an ICD is recommended if meaningful survival of greater than 1 year is expected.[11][12]

3. In patients with Brugada syndrome experiencing recurrent ICD shocks for polymorphic VT, intensification of therapy with quinidine or catheter ablation is recommended.[13][14]

4. In patients with spontaneous type 1 Brugada electrocardiographic pattern and symptomatic VA who either are not candidates for or decline an ICD, quinidine or catheter ablation is recommended.[13][15][14]

Class IIa
In patients with suspected long QT syndrome, ambulatory electrocardiographic monitoring, recording the ECG lying and immediately on standing, and/or exercise treadmill testing can be useful for establishing a diagnosis and monitoring the response to therapy.[16][17]
Class IIb
In patients with asymptomatic Brugada syndrome and a spontaneous type 1 Brugada electrocardiographic pattern, an electrophysiological study with programmed ventricular stimulation using single and double extrastimuli may be considered for further risk stratification.[9][12][18][19][20]
In patients with suspected or established Brugada syndrome, genetic counseling and genetic testing may be useful to facilitate cascade screening of relatives.[21][22]


References

  1. ↑ Al-Khatib, Sana M.; Stevenson, William G.; Ackerman, Michael J.; Bryant, William J.; Callans, David J.; Curtis, Anne B.; Deal, Barbara J.; Dickfeld, Timm; Field, Michael E.; Fonarow, Gregg C.; Gillis, Anne M.; Granger, Christopher B.; Hammill, Stephen C.; Hlatky, Mark A.; Joglar, José A.; Kay, G. Neal; Matlock, Daniel D.; Myerburg, Robert J.; Page, Richard L. (2018). "2017 AHA/ACC/HRS Guideline for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death". Circulation. 138 (13). doi:10.1161/CIR.0000000000000549. ISSN 0009-7322.
  2. ↑ Belhassen B, Glick A, Viskin S (2004). "Efficacy of quinidine in high-risk patients with Brugada syndrome". Circulation. 110 (13): 1731–7. doi:10.1161/01.CIR.0000143159.30585.90. PMID 15381640.
  3. ↑ Tsuchiya, Takeshi; Ashikaga, Keiichi; Honda, Toshihiro; Arita, Makoto (2002). "Prevention of Ventricular Fibrillation by Cilostazol, an Oral Phosphodiesterase Inhibitor, in a Patient with Brugada Syndrome". Journal of Cardiovascular Electrophysiology. 13 (7): 698–701. doi:10.1046/j.1540-8167.2002.00698.x. ISSN 1045-3873.
  4. ↑ Abud, Atilio; Bagattin, Daniel; Goyeneche, Raul; Becker, Carlos (2006). "Failure of Cilostazol in the Prevention of Ventricular Fibrillation in a Patient with Brugada Syndrome". Journal of Cardiovascular Electrophysiology. 17 (2): 210–212. doi:10.1111/j.1540-8167.2005.00290.x. ISSN 1045-3873.
  5. ↑ Matsui, Kazunori; Kiyosue, Tatsuto; Wang, Jin-Cheng; Dohi, Kazuhiro; Arita, Makoto (1999). Cardiovascular Drugs and Therapy. 13 (2): 105–113. doi:10.1023/A:1007779908346. ISSN 0920-3206. Missing or empty |title= (help)
  6. ↑ Miyazaki, Toshihisa; Mitamura, Hideo; Miyoshi, Shunichiro; Soejima, Kyoko; Aizawa, Yoshifusa; Ogawa, Satoshi (1996). "Autonomic and antiarrhythmic drug modulation of ST segment elevation in patients with Brugada syndrome". Journal of the American College of Cardiology. 27 (5): 1061–1070. doi:10.1016/0735-1097(95)00613-3. ISSN 0735-1097.
  7. ↑ Suzuki, Hiroshi; Torigoe, Katsumi; Numata, Osamu; Yazaki, Satoshi (2000). "Infant Case with a Malignant Form of Brugada Syndrome". Journal of Cardiovascular Electrophysiology. 11 (11): 1277–1280. doi:10.1046/j.1540-8167.2000.01277.x. ISSN 1045-3873.
  8. ↑ Zipes DP, Camm AJ, Borggrefe M, Buxton AE, Chaitman B, Fromer M; et al. (2006). "ACC/AHA/ESC 2006 Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death: a report of the American College of Cardiology/American Heart Association Task Force and the European Society of Cardiology Committee for Practice Guidelines (writing committee to develop Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death): developed in collaboration with the European Heart Rhythm Association and the Heart Rhythm Society". Circulation. 114 (10): e385–484. doi:10.1161/CIRCULATIONAHA.106.178233. PMID 16935995.
  9. ↑ 9.0 9.1 Casado-Arroyo R, Berne P, Rao JY, Rodriguez-Mañero M, Levinstein M, Conte G, Sieira J, Namdar M, Ricciardi D, Chierchia GB, de Asmundis C, Pappaert G, La Meir M, Wellens F, Brugada J, Brugada P (August 2016). "Long-Term Trends in Newly Diagnosed Brugada Syndrome: Implications for Risk Stratification". J. Am. Coll. Cardiol. 68 (6): 614–623. doi:10.1016/j.jacc.2016.05.073. PMID 27491905.
  10. ↑ Probst V, Veltmann C, Eckardt L, Meregalli PG, Gaita F, Tan HL, Babuty D, Sacher F, Giustetto C, Schulze-Bahr E, Borggrefe M, Haissaguerre M, Mabo P, Le Marec H, Wolpert C, Wilde AA (February 2010). "Long-term prognosis of patients diagnosed with Brugada syndrome: Results from the FINGER Brugada Syndrome Registry". Circulation. 121 (5): 635–43. doi:10.1161/CIRCULATIONAHA.109.887026. PMID 20100972.
  11. ↑ Priori SG, Gasparini M, Napolitano C, Della Bella P, Ottonelli AG, Sassone B, Giordano U, Pappone C, Mascioli G, Rossetti G, De Nardis R, Colombo M (January 2012). "Risk stratification in Brugada syndrome: results of the PRELUDE (PRogrammed ELectrical stimUlation preDictive valuE) registry". J. Am. Coll. Cardiol. 59 (1): 37–45. doi:10.1016/j.jacc.2011.08.064. PMID 22192666.
  12. ↑ 12.0 12.1 Sroubek J, Probst V, Mazzanti A, Delise P, Hevia JC, Ohkubo K, Zorzi A, Champagne J, Kostopoulou A, Yin X, Napolitano C, Milan DJ, Wilde A, Sacher F, Borggrefe M, Ellinor PT, Theodorakis G, Nault I, Corrado D, Watanabe I, Antzelevitch C, Allocca G, Priori SG, Lubitz SA (February 2016). "Programmed Ventricular Stimulation for Risk Stratification in the Brugada Syndrome: A Pooled Analysis". Circulation. 133 (7): 622–30. doi:10.1161/CIRCULATIONAHA.115.017885. PMC 4758872. PMID 26797467.
  13. ↑ 13.0 13.1 Belhassen B, Rahkovich M, Michowitz Y, Glick A, Viskin S (December 2015). "Management of Brugada Syndrome: Thirty-Three-Year Experience Using Electrophysiologically Guided Therapy With Class 1A Antiarrhythmic Drugs". Circ Arrhythm Electrophysiol. 8 (6): 1393–402. doi:10.1161/CIRCEP.115.003109. PMID 26354972.
  14. ↑ 14.0 14.1 Zhang P, Tung R, Zhang Z, Sheng X, Liu Q, Jiang R, Sun Y, Chen S, Yu L, Ye Y, Fu G, Shivkumar K, Jiang C (November 2016). "Characterization of the epicardial substrate for catheter ablation of Brugada syndrome". Heart Rhythm. 13 (11): 2151–2158. doi:10.1016/j.hrthm.2016.07.025. PMID 27453126.
  15. ↑ Nademanee K, Veerakul G, Chandanamattha P, Chaothawee L, Ariyachaipanich A, Jirasirirojanakorn K, Likittanasombat K, Bhuripanyo K, Ngarmukos T (March 2011). "Prevention of ventricular fibrillation episodes in Brugada syndrome by catheter ablation over the anterior right ventricular outflow tract epicardium". Circulation. 123 (12): 1270–9. doi:10.1161/CIRCULATIONAHA.110.972612. PMID 21403098.
  16. ↑ Antzelevitch C, Yan GX, Ackerman MJ, Borggrefe M, Corrado D, Guo J, Gussak I, Hasdemir C, Horie M, Huikuri H, Ma C, Morita H, Nam GB, Sacher F, Shimizu W, Viskin S, Wilde A (April 2017). "J-Wave syndromes expert consensus conference report: Emerging concepts and gaps in knowledge". Europace. 19 (4): 665–694. doi:10.1093/europace/euw235. PMC 5834028. PMID 28431071. Vancouver style error: initials (help)
  17. ↑ Somani R, Krahn AD, Healey JS, Chauhan VS, Birnie DH, Champagne J, Sanatani S, Angaran P, Gow RM, Chakrabarti S, Gerull B, Yee R, Skanes AC, Gula LJ, Leong-Sit P, Klein GJ, Gollob MH, Talajic M, Gardner M, Simpson CS (June 2014). "Procainamide infusion in the evaluation of unexplained cardiac arrest: from the Cardiac Arrest Survivors with Preserved Ejection Fraction Registry (CASPER)". Heart Rhythm. 11 (6): 1047–54. doi:10.1016/j.hrthm.2014.03.022. PMID 24657429.
  18. ↑ Delise P, Allocca G, Marras E, Giustetto C, Gaita F, Sciarra L, Calo L, Proclemer A, Marziali M, Rebellato L, Berton G, Coro L, Sitta N (January 2011). "Risk stratification in individuals with the Brugada type 1 ECG pattern without previous cardiac arrest: usefulness of a combined clinical and electrophysiologic approach". Eur. Heart J. 32 (2): 169–76. doi:10.1093/eurheartj/ehq381. PMC 3021386. PMID 20978016.
  19. ↑ Kusumoto, Fred M.; Bailey, Kent R.; Chaouki, Ahmad Sami; Deshmukh, Abhishek J.; Gautam, Sandeep; Kim, Robert J.; Kramer, Daniel B.; Lambrakos, Litsa K.; Nasser, Naseer H.; Sorajja, Dan (2018). "Systematic Review for the 2017 AHA/ACC/HRS Guideline for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death". Circulation. 138 (13). doi:10.1161/CIR.0000000000000550. ISSN 0009-7322.
  20. ↑ Sieira J, Conte G, Ciconte G, de Asmundis C, Chierchia GB, Baltogiannis G, Di Giovanni G, Saitoh Y, Irfan G, Casado-Arroyo R, Juliá J, La Meir M, Wellens F, Wauters K, Van Malderen S, Pappaert G, Brugada P (August 2015). "Prognostic value of programmed electrical stimulation in Brugada syndrome: 20 years experience". Circ Arrhythm Electrophysiol. 8 (4): 777–84. doi:10.1161/CIRCEP.114.002647. PMID 25904495.
  21. ↑ Crotti L, Marcou CA, Tester DJ, Castelletti S, Giudicessi JR, Torchio M, Medeiros-Domingo A, Simone S, Will ML, Dagradi F, Schwartz PJ, Ackerman MJ (October 2012). "Spectrum and prevalence of mutations involving BrS1- through BrS12-susceptibility genes in a cohort of unrelated patients referred for Brugada syndrome genetic testing: implications for genetic testing". J. Am. Coll. Cardiol. 60 (15): 1410–8. doi:10.1016/j.jacc.2012.04.037. PMC 3624764. PMID 22840528.
  22. ↑ Risgaard B, Jabbari R, Refsgaard L, Holst AG, Haunsø S, Sadjadieh A, Winkel BG, Olesen MS, Tfelt-Hansen J (November 2013). "High prevalence of genetic variants previously associated with Brugada syndrome in new exome data". Clin. Genet. 84 (5): 489–95. doi:10.1111/cge.12126. PMID 23414114.

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