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What is the prevalence of Jervell And Lange-Nielsen Syndrome?

How many people does Jervell And Lange-Nielsen Syndrome affect? Does it have the same prevalence in men and women? And in the different countries?

Prevalence of Jervell And Lange-Nielsen Syndrome

Jervell and Lange-Nielsen Syndrome is a rare genetic disorder characterized by a prolonged QT interval in the heart's electrical cycle, leading to potentially life-threatening arrhythmias. It is estimated to affect approximately 1 in every 200,000 to 250,000 individuals worldwide. The syndrome is typically inherited in an autosomal recessive manner, meaning both parents must carry the gene mutation for their child to be affected. Early diagnosis and appropriate management, such as medication or implantation of a cardioverter-defibrillator, can significantly improve the prognosis for individuals with this syndrome.



Jervell and Lange-Nielsen Syndrome is a rare genetic disorder characterized by a prolonged QT interval in the heart's electrical cycle, leading to potentially life-threatening arrhythmias. It is an autosomal recessive condition, meaning both parents must carry the gene mutation for their child to be affected.


The prevalence of Jervell and Lange-Nielsen Syndrome is estimated to be around 1 in 200,000 to 250,000 individuals worldwide. Although it is considered a rare disorder, its occurrence may vary among different populations. The syndrome has been reported in various ethnic groups, including individuals of Norwegian, Swedish, and Danish descent.


Due to its rarity, Jervell and Lange-Nielsen Syndrome often goes undiagnosed or misdiagnosed, leading to potential complications. Early detection and appropriate management are crucial to prevent sudden cardiac events and improve patient outcomes. Genetic testing and evaluation of family history are essential for accurate diagnosis and identification of individuals at risk.


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