The Turkish Journal of Pediatrics 2018 , Vol 60 , Num 5
Fever-induced Brugada syndrome in a 9-year-old boy presenting with acute chest pain
Gülser Esen Besli 1 ,Sema Yıldırım 2 ,İbrahim Akalın 3 ,Yusuf İzzet Ayhan 4 ,Merve Kısıoğlu 2 ,Afig Berdeli 5
1 Departments of Pediatric Emergency, İstanbul Medeniyet University Goztepe Training and Research Hospital, İstanbul, Turkey
2 Departments of Pediatrics, İstanbul Medeniyet University Goztepe Training and Research Hospital, İstanbul, Turkey
3 Departments of Medical Genetics, İstanbul Medeniyet University Goztepe Training and Research Hospital, İstanbul,Turkey
4 Departments of Pediatric Cardiology, İstanbul Medeniyet University Goztepe Training and Research Hospital, İstanbul, Turkey
5 Department of Pediatrics, Molecular Medicine Laboratory, Ege University Faculty of Medicine, İzmir, Turkey
DOI : 10.24953/turkjped.2018.05.016 Besli GE, Yıldırım S, Akalın İ, Ayhan Yİ, Kısıoğlu M, Berdeli A. Fever-induced Brugada syndrome in a 9-year-old boy presenting with acute chest pain. Turk J Pediatr 2018; 60: 571-575.

Brugada syndrome, an arrhythmogenic disease, occurs due to mutations involving cardiac sodium channels. It is characterized by persistent or transient ST-segment elevation in the right precordial electrocardiogram leads that could be unmasked by several circumstances, with fever particularly. Molecular and cellular mechanisms leading to Brugada syndrome have not been completely elucidated. Mutations of the SCN5A gene encoding the pore-forming α-subunit of the cardiac sodium channel protein have been attributed in the molecular diagnosis. Although this syndrome is well-known in adults, it is less frequently reported in infants and children. We describe a 9-year-old Turkish boy with a family history of sudden cardiac death, who presented with chest pain and fever-induced expression of the Brugada syndrome phenotype that might be associated with a mutation in SCN5A gene. Keywords : Brugada syndrome, cardiac sodium channel, chest pain, children, fever

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