Sudden Unexplained Nocturnal Death Syndrome: The Hundred Years' Enigma.

Sudden Unexplained Nocturnal Death Syndrome: The Hundred Years' Enigma.
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不明原因的夜间死亡综合症:百年之谜。

DOI:
10.1161/jaha.117.007837
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发表时间:
2018-03-03
影响因子:
5.4
通讯作者:
Cheng J
Cheng J
中科院分区:
医学2区
文献类型:
--
作者:
Zheng J;Zheng D;Su T;Cheng J

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Sudden cardiac death (SCD) is a leading cause of mortality worldwide with an estimated 15% to 20% of all deaths. SCD mostly occurs in older adults with acquired structural heart disease. 1 Among these structural heart diseases, coronary heart disease, presumed to be the most common pathology underlying SCD, accounts for 75% of SCD in Western countries and 50% to 60% in Japan, followed by cardiomyopathies of 10% to 15% in Western countries and 30% to 35% in Japan. Only 1% to 2% of SCD in Western countries compared with 10% in Japan occurs because of rare inherited cardiac arrhythmias without obvious structural change in the heart. 1, 2 For these cases, a routine postmortem autopsy cannot explain their death, and these can be classified as sudden unexplained death (SUD). Here, we focus on a specific SUD called sudden unexplained nocturnal death syndrome (SUNDS) and attempt to provide a comprehensive understanding of SUNDS. SUNDS occurs predominantly in Southeast Asia and has different academic terms but similar definitions in different countries, such as bangungut in Philippines, Lai Tai in Thailand, Pokkuri Death Syndrome (PDS) in Japan, and SUNDS in the United States and China. 3–7 Despite these multiple terms, the common characteristics (modalities) of these victims are sudden death of young healthy individuals (the vast majority are males) during nocturnal sleep and postmortem routine autopsy that cannot explain their deaths. It has been 100 years since the first description of SUNDS in 1917 in the Philippines. 7 Since then, 2 major active periods of studies on SUNDS have accumulated vast information for further understanding the pathogenesis of SUNDS. As a disorder mainly prevalent in Southeast Asia, SUNDS did not attract worldwide attention until 1981, when its first report was submitted to the Centers for Disease Control and Prevention. 5 Since then, numerous scholars have sought to understand the enigma of SUNDS. Over the next 20 years, studies mainly focused on collecting epidemiological characteristics to reveal probable pathogenesis associated with external environment factors. Since the first literature revealed the correlation between SUNDS and pathogenic mutation on SCN5A in 2002, the pathogenetic study on SUNDS has entered a new stage of molecular genetics aimed at uncovering the intrinsic gene susceptibility in different individuals (Figure 1). To better understand SUNDS, we conduct this comprehensive review and outline an academic history of SUNDS over the past 100 years, including epidemiology, pathology, and pathogenetic hypotheses, particularly the molecular genetics of SUNDS.