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OP-077 [AJC » Inherited arrhythmia syndromes] Report of a Family with Wolff-Parkinson-White Syndrome and Craniofrontonasal Syndrome: Is it a new finding?

      Wolff-Parkinson-White (WPW) syndrome is a common cause of supraventricular tachycardia with prevalence in Western countries of 1.5 to 3.1 per 1000 persons, may cause sudden death. The familial occurrence of the WPW syndrome is well documented, is typically inherited in an autosomal dominant pattern, and is sometimes associated with familial cardiomyopathy. The molecular genetics of WPW have been investigated but to date only a single gene PRKAG2, which encodes the 5'-AMP-activated protein kinase subunit γ-2, has been identified and mutations in this gene explain only a small fraction (<5%) of WPW. We identified a family with craniofrontonasal syndrome (CFNS) in which the WPW syndrome was shown in members of the family. CFNS is a rare genetic entity with X-linked dominant inheritance, and is due to mutations in the Ephrin-B1 gene.
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