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

BESLİ G. E., Yildirim S., Akalin I., Ayhan Y. I., Kisioglu M., BERDELİ A.

TURKISH JOURNAL OF PEDIATRICS, cilt.60, sa.5, ss.571-575, 2018 (SCI-Expanded) identifier identifier identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 60 Sayı: 5
  • Basım Tarihi: 2018
  • Doi Numarası: 10.24953/turkjped.2018.05.016
  • Dergi Adı: TURKISH JOURNAL OF PEDIATRICS
  • Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus, TR DİZİN (ULAKBİM)
  • Sayfa Sayıları: ss.571-575
  • Anahtar Kelimeler: Brugada syndrome, cardiac sodium channel, chest pain, children, fever, SCN5A MUTATION, DILATED CARDIOMYOPATHY, SPLICE VARIANT, COMMON, ARRHYTHMIA, BLOCK
  • Karadeniz Teknik Üniversitesi Adresli: Hayır

Özet

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 alpha-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.