Literature DB >> 18662176

Congenital long-QT syndrome concealed by hypercalcemia in Williams Syndrome.

Richard J Czosek1, Charles I Berul.   

Abstract

We report a case of gene-positive long-QT syndrome (KCNH2) in a patient with concomitant Williams Syndrome. The hypercalcemia that developed in association with Williams Syndrome pseudo-normalized the QTc interval on surface ECG, concealing the clinical and electrocardiographic manifestations of the disease. Initiation of medical therapy for hypercalcemia unmasked the prolonged QT interval, allowing for the diagnosis of long-QT syndrome to be made.

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Year:  2008        PMID: 18662176     DOI: 10.1111/j.1540-8167.2008.01263.x

Source DB:  PubMed          Journal:  J Cardiovasc Electrophysiol        ISSN: 1045-3873


  2 in total

Review 1.  Congenital long-QT syndromes: a clinical and genetic update from infancy through adulthood.

Authors:  Gregory Webster; Charles I Berul
Journal:  Trends Cardiovasc Med       Date:  2008-08       Impact factor: 6.677

2.  Williams syndrome with a "twist".

Authors:  Despoina Maritsi; Lydia Kossiva; George Vartzelis
Journal:  Case Rep Med       Date:  2010-06-16
  2 in total

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