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Neuropediatrics. 1999 Jun;30(3):146-8.

Reduced heart rate variability in patients affected with Rett syndrome. A possible explanation for sudden death.

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1
Institute of Clinical Medicine, School of Medicine, Siena, Italy.

Abstract

Incidence of sudden death in Rett syndrome is greater than that of the general population and cardiac electrical instability is a prime suspect cause. Our study shows that girls with Rett syndrome had significantly lower heart rate variability (marker of autonomic disarray) and longer corrected QT intervals compared with an age-matched group of healthy girls. These abnormalities increased with advancing stages of the syndrome. These findings suggest a possible role of cardiac dysfunction in the sudden death associated with Rett syndrome.

PMID:
10480210
DOI:
10.1055/s-2007-973480
[Indexed for MEDLINE]
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