@entity1789 ( @entity1789 ) is a @entity299 linked to loss of function mutation in the @entity1788 gene which affects the @entity1372 current. The diagnosis is made on the ECG showing characteristic cove-shaped ST elevation in leads V(1) to V(3) in the absence of structural @entity299 , electrolyte disturbance or ischaemia. This condition is genetically transmitted as an @entity74 with incomplete penetrance. It is responsible for 20% of all sudden deaths in those without structural @entity299 . Diagnosis of @entity1789 can be difficult as the ECG changes are dynamic and variable. Genetic mutation in @entity1788 gene is found in 25-30% of @entity1 with @entity1789 . @entity1 may present with @entity1163 due to polymorphic VT or resuscitated @entity944 in the third or fourth decade of life. Symptoms frequently occur at night or at rest and @entity221 is a common trigger in @entity1 . @entity1 presenting with @entity1163 or resuscitated @entity1164 should have an implantable defibrillator. Management of asymptomatic @entity1 is controversial and risk stratification is required. www.brugadadrugs.org gives a list of drugs that should be avoided by @entity1 suffering from @entity1789 .

Diagnosis and management of XXXX .

@entity1 :: ('9606', 'Species') :: ['children', 'Patients', 'patients'] @entity1789 :: ('MESH:D053840', 'Disease') :: ['BS', 'Brugada Syndrome'] @entity1164 :: ('MESH:D016757', 'Disease') :: ['sudden cardiac death'] @entity1788 :: ('6331', 'Gene') :: ['SCN5A'] @entity944 :: ('MESH:D003645', 'Disease') :: ['sudden death'] @entity1372 :: ('MESH:D012964', 'Chemical') :: ['sodium'] @entity1163 :: ('MESH:D013575', 'Disease') :: ['syncope'] @entity74 :: ('MESH:D030342', 'Disease') :: ['autosomal dominant syndrome'] @entity299 :: ('MESH:D006331', 'Disease') :: ['heart disease', 'cardiac ion channel disorder'] @entity221 :: ('MESH:D005334', 'Disease') :: ['fever']
@entity1789 :: (MESH:D053840,Disease) :: ['Brugada Syndrome']
