Propafenone is not effective for severe ventricular arrhythmias in Andersen-Tawil syndrome
نویسندگان
چکیده
Corresponding author: Piotr Bienias Department of Internal Medicine and Cardiology Medical University of Warsaw 4 Lindleya St 02-005 Warsaw, Poland Phone: +48 604 405 365 E-mail: [email protected] 1 Department of Internal Medicine and Cardiology, Medical University of Warsaw, Warsaw, Poland 2 Department of Neurology, Medical University of Warsaw, Warsaw, Poland 3 Department of Pediatric Cardiology and General Pediatrics, Medical University of Warsaw, Warsaw, Poland 4 Department of Cardiology, Children’s Memorial Health Institute, Warsaw, Poland
منابع مشابه
Child neurology: Andersen-Tawil syndrome.
Andersen-Tawil syndrome (ATS) is one of the periodic paralyses. This autosomal dominant disorder was initially named after Andersen, who in 1971 reported the case of a young boy presenting with intermittent muscle weakness, ventricular arrhythmias, and other developmental abnormalities. It was subsequently renamed Andersen-Tawil syndrome following the additional work of Dr. Rabi Tawil. Periodic...
متن کاملAndersen-Tawil Syndrome
Andersen-Tawil syndrome (ATS) is a rare condition consisting of ventricular arrhythmias, periodic paralysis, and dysmorphic features. In 2001, mutations in KCNJ2, which encodes the a subunit of the potassium channel Kir2.1, were identified in patients with ATS. To date, KCNJ2 is the only gene implicated in ATS, accounting for approximately 60% of cases. ATS is a unique channelopathy, and repres...
متن کاملAtrial pacing for the management of ventricular arrhythmias in Andersen-Tawil syndrome
Introduction Andersen-Tawil syndrome (ATS) is a rare autosomal dominant or sporadic disorder defined by a triad of periodic muscle paralysis, ventricular arrhythmias (long QT-7), and dysmorphic features. The estimated prevalence is 1 in 1,000,000. The underlying mutation is in the KCNJ2 gene encoding the inward rectifier potassium channels (Kir2.1) present in both skeletal and cardiac muscles. ...
متن کاملReduction of complex ventricular ectopy and improvement in exercise capacity with flecainide therapy in Andersen-Tawil syndrome.
Andersen-Tawil syndrome (ATS) is a rare inherited autosomal disorder characterized by the clinical triad of ventricular arrhythmias, hypokalaemic periodic paralyses, and skeletal developmental abnormalities, resulting in dysmorphic features. Although ATS patients have a high incidence of ventricular arrhythmias, the occurrence of sudden cardiac death is rare. In this report, we describe the suc...
متن کاملHypokalemic periodic paralysis, facial dysmorphism and ventricular arrhythmia (clinical triad of Andersen-Tawil syndrome).
Andersen-Tawil Syndrome (ATS) is a rare potassium channel disorder, characterized by episodic weakness, ventricular arrhythmias and dysmorphic features (short stature, scoliosis, clinodactyly, hypertelorism, small or prominent low set ears, micrognathia and broad forehead). We report a case of hypokalemic periodic paralysis with dysmorphic facial features and ventricular arrhythmia resembling A...
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عنوان ژورنال:
دوره 14 شماره
صفحات -
تاریخ انتشار 2018