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社区来源的天使综合征死亡率报告(1979 - 2022年)

Community-Sourced Reporting of Mortalities in Angelman Syndrome (1979-2022).

作者信息

Gomes Adriana T, Moore Amanda, Cross Meagan, Hardesty Cristy, David Kelly, Sampedro Maria Galan, Dube Sophie, Weil-Chalker Sharon, Montepagano Adela Gentil, Christel Ursula, Martin Rachel, Wheeler Anne, Tan Wen-Hann, Bird Lynne M, Bichell Terry Jo

机构信息

UCSD, San Diego, California, USA.

Angelman Syndrome Foundation, Aurora, Illinois, USA.

出版信息

Am J Med Genet A. 2025 Apr;197(4):e63961. doi: 10.1002/ajmg.a.63961. Epub 2024 Dec 16.

Abstract

Angelman syndrome (AS) is a severe genetic neurodevelopmental disorder with an estimated prevalence of 1:20,000. Life expectancy appears to be normal, however, data regarding lifespan in AS are scarce. Until 2018, there was no unique diagnosis code for AS, thus true incidence, prevalence, mortality and morbidity rates are unknown. A social media effort was launched by caregivers of people with AS to gather community-sourced data to understand AS mortality risks. Information on 220 deaths was verified with obituaries and public postings. Respiratory illness was the primary cause of death among people with AS overall, followed by accidents and seizures. Surprisingly, sudden unexpected death in sleep (SUDS) was the fourth most common cause, which has not been reported previously. Approximately 91% of people with AS have epilepsy, thus some SUDS cases may represent sudden unexpected deaths in epilepsy (SUDEP). Causes of death vary by age, and differ from the general population. Though there are obvious limitations to data collected through social media, grass roots science is a starting point to amass preliminary data and inform future epidemiological research on AS.

摘要

天使综合征(AS)是一种严重的遗传性神经发育障碍,估计患病率为1:20000。预期寿命似乎正常,然而,关于天使综合征患者寿命的数据很少。直到2018年,天使综合征都没有唯一的诊断代码,因此其真实发病率、患病率、死亡率和发病率尚不清楚。天使综合征患者的护理人员发起了一项社交媒体活动,以收集社区提供的数据,了解天使综合征的死亡风险。通过讣告和公开帖子核实了220例死亡信息。呼吸系统疾病是天使综合征患者总体上的主要死因,其次是事故和癫痫发作。令人惊讶的是,睡眠中意外猝死(SUDS)是第四大常见死因,此前尚未有过相关报道。约91%的天使综合征患者患有癫痫,因此一些睡眠中意外猝死病例可能代表癫痫猝死(SUDEP)。死因因年龄而异,与普通人群不同。尽管通过社交媒体收集的数据存在明显局限性,但草根科学是积累初步数据并为未来天使综合征流行病学研究提供信息的起点。

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本文引用的文献

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Provisional Mortality Data - United States, 2021.临时死亡率数据 - 美国,2021 年。
MMWR Morb Mortal Wkly Rep. 2022 Apr 29;71(17):597-600. doi: 10.15585/mmwr.mm7117e1.

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