A child nearly died from an over-eaten hot dog and a rare, undiagnosed disease. He went into cardiac arrest.
This could make you want a hot dog. It was in fact while eating this American sandwich that a young Turk presented a reaction which was unusual to say the least. At only 9 years old, he suffered a syncope followed by sudden cardiac arrest.
The day should have been auspicious for the boy whose story is the subject of this publication. A hot dog on the canteen menu has something to celebrate. But after a big bite of this festive meal, his condition suddenly deteriorated. Quickly taken care of by the emergency room, the child survived.
For 30 minutes, the doctors at the hospital where he was transported were determined to revive the child. On this occasion, they note ventricular fibrillation. His mishap will at least have allowed him to diagnose a rare heart disorder: Brugada syndrome.
A genetic mutation
It is only at the University Hospital of Istanbul (Turkey) that words are set on this unusual event. The doctors who took care of him summarize his career in Pediatrics. Sudden cardiac arrests are common in people with Brugada syndrome, they explain. Most often these incidents are related to vagus nerve stimulation – usually during sleep.
This time, it’s a big bite of hot dog that would be involved. The doctors therefore recommended to the child to avoid outbreaks of fever, alcohol and risky drugs … as well as to eat in small pieces. These factors increase the risk of cardiac arrest. An automatic defibrillator was also implanted in the patient’s chest. It will restart the heart in the event of a sudden stop.
A mutation located in the SCN5A gene is at the origin of the pathology. But in the absence of symptoms, spotting it is useless. Moreover, the examination of the boy’s relatives did not reveal any family history. Only his brother shows signs of such ailment. He received the same prevention advice.
Discovered in 1992
Last February, a young wife paid the price for this little-known disease. Her husband died of cardiac arrest after being thrown out of bed due to snoring. The error is understandable: this syndrome has only been known since 1992 and the number of patients is difficult to assess.
As the platform explains Orphanet, specializing in rare diseases, the age at diagnosis is extremely variable. He ranges from 2 to 84 years old. And for good reason: in the absence of sudden cardiac arrest, nothing suggests the disease. The EKGs themselves are quite normal.
Some signs may alert, such as restlessness, loss of urine or memory, or noisy breathing, intense and accompanied by snoring. But it is still necessary to know them.
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