Andersen syndrome: the newest variant of the hereditary-familial long QT syndrome.

Andrés Ricardo Pérez Riera, Celso Ferreira, Sérgio J Dubner, Edgardo Schapachnik

Journal: Annals of noninvasive electrocardiology : the official journal of the International Society for Holter and Noninvasive Electrocardiology, Inc 2004;9(2):175-9

PMID: 15084216

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Abstract

Andersen's Syndrome is a rare disease, hereditary with autosomal dominant transmission, of the ion channels of the sarcolemmal membranes of the cardiac and skeletal muscles (channelopathy), which affects chromosome 17 of the KCNJ2 gene, responsible for encoding the outward potassium delayed rectifier current KIR2.1, resulting in a loss or suppression of the function of this channel.

Address: ABC University Fundação Santo André, São Paulo, Brazil. [email protected]

DOI: 10.1111/j.1542-474X.2004.92552.x

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MeSH Terms: Action Potentials, Carbonic Anhydrase Inhibitors, Electrocardiography, Genetic Predisposition to Disease, Glycogen Storage Disease Type IV, Heart Conduction System, Humans, Long QT Syndrome, Paralyses, Familial Periodic, Potassium Channels