Key result
Jervell and Lange-Nielsen syndrome has an estimated prevalence of at least 1:200,000 in Norway, driven by genetic homogeneity, mutational heterogeneity, and a founder mutation.
Population
Patients with Jervell and Lange-Nielsen syndrome (JLNS) and Long QT syndrome (LQTS), particularly in Norway
Design
Review
Authors
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May support QTc screening in deaf children with syncope; leaves open global prevalence and full mutation spectrum.
This review highlights the unusually high prevalence of Jervell and Lange-Nielsen syndrome in Norway and outlines the genetic basis and clinical implications for screening and monitoring.
Tranebj�rg et al. (1999) conducted a review in Jervell and Lange-Nielsen syndrome. Jervell and Lange-Nielsen syndrome has an estimated prevalence of at least 1:200,000 in Norway, driven by genetic homogeneity, mutational heterogeneity, and a founder mutation.