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Brugada syndrome

From SNPedia

Brugada syndrome (BrS) is a genetic disease characterised by abnormal electrocardiogram (ECG) findings and an increased risk of sudden cardiac death. It is named for the Spanish cardiologists Pedro Brugada and Josep Brugada. It is a major cause of sudden unexplained death syndrome (SUDS), also known as sudden adult death syndrome (SADS), and is the most common cause of sudden death in young men without known underlying cardiac disease in Thailand and Laos.Wikipedia

Since it's definition as a clinical syndrome in 1992, Brugada syndrome has evolved from being seen as a rare disease to one second only to automobile accidents as a cause of death among relatively young adults, at least in some countries. The syndrome typically manifests during adulthood, with a mean age of sudden death of 41 +/- 15 years, but also occurs in infants and children.[PMID 15655131OA-icon.png]

Brugada syndrome is rare, affecting perhaps 5 in 10,000 people worldwide, although occuring more frequently in people of Asian ancestry, particularly in Japanese and Southeast Asian populations. Men are about 10 times more likely to be affected than women.

Mutations in at least 16 different genes (listed below) may cause Brugada syndrome, all of which are inherited in an autosomal dominant manner. The most commonly mutated gene leading to BrS is SCN5A, accounting for ~30% of affected individuals.Genetics Home Reference

Genes causing BrS include: