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Papers of the Week


2018


Turk J Pediatr


60


5

Fever-induced Brugada syndrome in a 9-year-old boy presenting with acute chest pain.

Authors

Besli G E, Yıldırım S, Akalın İ, Ayhan Y İ, Kısıoğlu M, Berdeli A
Turk J Pediatr. 2018; 60(5):571-575.
PMID: 30968627.

Abstract

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.