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PHENOTYPES OF MUTATIONS RELATED TO DEPENDENT SODIUM VOLTAGE CHANNELS ON CHILDREN AND ADOLESCENTS
  • +4
  • Andressa Ferreira,
  • Carlos Dias Filho,
  • Nivaldo Junior,
  • Carlos Dias,
  • Sally Cristina,
  • Rafael Andrade,
  • Cristiano Mostarda
Andressa Ferreira
UFMA
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Carlos Dias Filho
UFMA
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Nivaldo Junior
UFMA
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Carlos Dias
UFMA
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Sally Cristina
UFMA
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Rafael Andrade
UFMA
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Cristiano Mostarda
UFMA
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Abstract

Cardiac channelopathies are a heterogeneous group of inherited cardiac diseases that are associated with mutations in the genes that encode the expression of cardiac ion channels. In view of this, it can be mentioned that the main hereditary arrhythmias in children and adolescents, caused by dysfunction of the ion channels are Brugada Syndrome (BrS) and Long QT Syndrome (LQTS). However, few studies address the physiological effects of these conditions on children and adolescents. Thus, the aim of this study is to describe the mutation phenotype related to voltage-gated sodium channels in children and adolescents. A search was performed in the literature of Pubmed, Scielo and Google scholar. The search was limited to articles written in English in the last 5 years, so articles published between January 2014 and January 2019 were included. Among 2196 studies identified through a systematic literature review, thirty studies related to the theme were identified for complete review and after applying exclusion criteria 4 articles were included in the results of this research. As the most frequently observed channelopathy, BrS was also more identified in children and adolescents, characterized by episodes of syncope or sudden cardiac death. LQTS shows clinical manifestations with a mild phenotype and good prognosis, although it is necessary to monitor and correct serum electrolyte disturbances to prevent ventricular arrhythmias and, consequently, sudden death in patients with the pathology.