Guideri F, Acampa M, Hayek G, Zappella M, Di Perri T
Institute of Clinical Medicine, School of Medicine, Siena, Italy.
Neuropediatrics. 1999 Jun;30(3):146-8. doi: 10.1055/s-2007-973480.
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.
雷特综合征患者的猝死发生率高于普通人群，心脏电不稳定是主要的可疑病因。我们的研究表明，与年龄匹配的健康女孩组相比，雷特综合征女孩的心率变异性（自主神经紊乱的标志物）显著降低，校正QT间期更长。这些异常随着综合征阶段的进展而增加。这些发现提示心脏功能障碍可能在雷特综合征相关的猝死中起作用。
