Department of Pediatrics and Adolescent Medicine, Division of General Pediatrics and Neonatology, Medical University of Vienna, Austria.
Dev Med Child Neurol. 2010 Oct;52(10):962-5. doi: 10.1111/j.1469-8749.2010.03716.x. Epub 2010 Jun 22.
rett syndrome is a severe neurodevelopmental disorder that typically affects females. Little is known about the natural history and survival time of these females.
we compared the survival of all Austrian female participants from Rett's historical cohort (1966) with that of affected females registered in the Australian Rett Syndrome Database. The analysis included both Kaplan-Meier analysis and a log-rank test for equality of survivor functions.
of females in the original Austrian group, three are still alive. The median age at death was 13 years 4.8 months. The probability of survival up to the age of 25 years was 21%, compared with 71% in the Australian cohort (p<0.001). We found no practical or statistically significant differences in survival between the various birth year groups within the Australian cohort.
our data indicate that survival of females with Rett syndrome has improved since the late 1960s but that there has been shown no change in survival over the last 30 years, possibly because the follow-up time has been too short.
雷特综合征是一种严重的神经发育障碍，通常影响女性。对于这些女性的自然病史和生存时间知之甚少。
我们比较了雷特氏历史队列（1966 年）中所有奥地利女性参与者的生存情况，以及澳大利亚雷特综合征数据库中登记的受影响女性的生存情况。分析包括 Kaplan-Meier 分析和对数秩检验生存函数的均等性。
原始奥地利组中有 3 名女性仍然存活。死亡时的中位年龄为 13 岁 4.8 个月。25 岁时的生存率为 21%，而澳大利亚队列为 71%（p<0.001）。我们在澳大利亚队列的各个出生年份组中没有发现生存方面的实际或统计学显著差异。
我们的数据表明，自 20 世纪 60 年代末以来，雷特综合征女性的生存率有所提高，但在过去 30 年中，生存率没有变化，这可能是因为随访时间太短。
