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long QT syndrome type 6
Pathology:
- KCNE2 mutants form channels that open slowly & close rapidly, thus diminishing K+ currents
Genetics:
- associated with defects in KCNE2
Clinical manifestations:
- see long QT syndrome
- syncope & sudden death in response to exercise or emotional stress
Special laboratory:
- electrocardiogram
a) prolonged QT interval
b) ventricular ectopy
Interactions
disease interactions
General
long QT syndrome
Database Correlations
OMIM 603796
MORBIDMAP 603796