Syndrome of Brugada

The syndrome of Brugada is a Genetic disease characterized by anomalies of the Segment ST on the level of the Dérivation S V1, V2 and V3 with the electrocardiogram associated at the high risk of ventricular Arythmie and brutal death. The demonstrations of this pathology generally appear during the adulthood but it can start between two days of life until more than 80 years. Sudden death occurs on average towards forty.

The clinical picture of this syndrome includes/understands the Sudden death of the newborn and the Syndrome of brutal death during the sleep particularly in the Asian populations. It is about a frequent cause of death in the young people Laotian inhabitants of Thailand and

Etiology

The syndrome of Brugada is a sodic Canalopathie by change of the Gène SCN5A located on the Chromosome 3

Incidence & prevalence

Of recent discovery, the data let think of a frequency of 1 over 2000 in the Japanese populations where the syndrome of brutal death during the sleep is frequent

Description

Diagnosis

  • Previous family of Syndrome of Brugada, sudden death.
  • Previous personnel of ressucitée sudden death.
  • With the electrocardiogram: aspect of block of branch right associated with a know-shift of the segment ST and anomalies with the wave T in right derivations précordiales (V1 with V3) >>> One describes 3 types of anomalies ECG.
Type 1: to supplement - it is about the traditional aspect most frequently associated with a sudden death Type 2: to supplement Type 3: to supplement
  • Positivity of the test to the Ajmaline or Flécaïne
  • Identification of the change of gene coding SCN5A

Assumption of responsibility

  • Variable, it depends on the type of anomaly ECG, the result of the pharmacological test, the family antécédants of sudden death, the symptoms and disorders of the rate/rhythm presented by the patient.
  • If an assumption of responsibility is decided, it rests especially on the installation of Défibrillateur Automatique Plantable which will ensure an Electric shock (=defibrillation) in the event of occurred of disorders of the ventricular rate/rhythm.

The genetic Council

Mode of transmission

dominant autosomic Transmission

Sources

  • Online Mendelian Inheritance in Man, OMIM (TM). Johns Hopkins University, Baltimore, MANDELEVIUM. MIM Number: 601144 * GeneTests: Medical Genetics Information Resource (database online). Copyright, University off Washington, Seattle. 1993-2005 the authors of this article are Doctor Brugada.

External bonds

New Scientist Sept. 20, 2002-->
  • Algado et al.: http://www.medspain.com/ant/n13_jun00/Brugada.htm
  • Behr: http://www.c-r-y.org.uk/long_qt_syndrome.htm
  • List of the dangerous drugs or possibly dangerous

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