Sudden unexplained death syndrome: Difference between revisions

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#Redirect [[Brugada syndrome]]
 
 
'''Sudden unexplained death syndrome''' was first noted in 1977 among Hmong refugees in the US.<ref>{{cite journal|MMWR Morb Mortal Wkly Rep|year=1981|volume=30|issue=47|pages=581&ndash;4,589|title=Sudden, unexpected, nocturnal deaths among Southeast Asian refugees|author=Centers for Disease Control (CDC)|
|pmid=6796814}}</ref><ref>{{cite journal|journal=MMWR CDC Surveill Summ|year=1987|volume=36|issue=1|pages=43SS-53SS|title=Sudden unexplained death syndrome in Southeast Asian refugees: a review of CDC surveillance|author=Parrish RG, Tucker M, Ing R, Encarnacion C, Eberhardt M|pmid=3110586}}</ref> The disease was again noted in Singapore, when a retrospective survey of records showed that 230 otherwise healthy Thai men died suddenly of unexplained causes between 1982 and 1990:<ref>{{cite journal|author=Goh KT, Chao TC, Chew CH|title=Sudden nocturnal deaths among Thai construction workers in Singapore|journal=Lancet|year=1990|volume=335|pages=1154|pmid=1971883}}</ref> publication of this data provoked a diplomatic incident.
 
==Features==
The condition appears to affect primarily young Hmong men from Laos (median age 33)<ref>{{cite journal|journal=Am J Public Health|year=1987|volume=77|issue=9|pages=1187&ndash;90|title=Sudden death in sleep of Laotian-Hmong refugees in Thailand: a case-control study|author=Munger RG}}</ref> and northeastern Thailand (where the population are mainly of Laotian descent).<ref name="Tatsanavivat">{{cite journal|journal=Int J Epidemiol|year=1992|volume=21|issue=5|pages=904&ndash;10|title=Sudden and unexplained deaths in sleep (Laitai) of young men in rural northeastern Thailand|author=Tatsanavivat P, Chiravatkul A, Klungboonkrong V, Chaisiri S, Jarerntanyaruk L, Munger RG, Saowakontha S|pmid=1468851}}</ref><ref>{{cite journal|journal=Int J Epidemiol|year=1993|volume=22|issue=1|pages=81&ndash;7|title=Sudden unexplained death syndrome in north-east Thailand|author=Tungsanga K, Sriboonlue P|pmid=8449651}}</ref>  There is a strong hereditary component and the victims tend to die in their sleep.
 
Survivors describe a feeling of intense fear and paralysis.  There is a sensation of pressure in the chest, the presence of an alien being in the room and altered sensation.
 
==Pathology==
The cause of this syndrome is currently believed to be a form of [[Brugada Syndrome]].<ref>{{cite journal|author=Nademanee K, Veerakul G, Nimmannit S, ''et al.''|title=Arrhythmogenic marker for the sudden unexplained death syndrome in Thai men|journal=Circulation|year=1997|volume=96|pages=2595&ndash;2600}}</ref>
 
It was noted very early on that the disease had the characteristics of a familial cardiac conduction defect (i.e., a problem with the electrical pathways of the heart).<ref>{{cite journal|journal=JAMA|year=1986|volume=256|issue=19|pages=2700&ndash;5|title=The cardiac pathology of sudden, unexplained nocturnal death in Southeast Asian refugees|author=Kirschner RH, Eckner FA, Baron RC|pmid=3773176}}</ref> and one study has shown evidence for a [[long-QT syndrome]] in populations at risk.<ref>{{cite journal|journal=Lancet|year=1991|volume=338|issue=8762|pages=280&ndash;1|title=Prolonged QT interval and risk of sudden death in South-East Asian men|author=Munger RG, Prineas RJ, Crow RS, Changbumrung S, Keane V, Wangsuphachart V, Jones MP|pmid=1677112}}</ref>  [[Thiamine deficiency]] is common in the risk population because of diet, and is also a cause of a prolonged QT-interval;<ref>{{cite journal|journal=Lancet|year=1990|volume=335|issue=8698|pages=1154&ndash;5
|title=Thiamine and sudden death in sleep of South-East Asian refugees|author=Munger RG, Booton EA|pmid=1971884}}</ref> but proof that inducible ventricular arrhythmia is the cause of this disease came only with the publication of the DEBUT trial in 2003.<ref name="DEBUT">{{cite journal|journal=Circulation|year=2003|volume=107|issue=17|pages=2221&ndash;6|title=Defibrillator Versus beta-Blockers for Unexplained Death in Thailand (DEBUT): a randomized clinical trial|author=Nademanee K, Veerakul G, Mower M, ''et al.''|pmid=}}</ref>
 
==Treatment==
The only proven way to prevent death is by implantation of a cardiovertor defibrillator.  Oral antiarrhythmics such as [[propranolol]] are ineffective.<ref name="DEBUT"/>
 
==Folk beliefs==
This phenomenon is well known among the Hmong people of Laos,<ref name="Alder1995">{{cite journal|journal=Soc Sci Med|year=1995|volume=40|issue=12|pages=1623&ndash;9|title=Refugee stress and folk belief: Hmong sudden deaths|author=Adler SR|pmid=7660175}}</ref> who ascribe these deaths to a malign spirit, ''dab tsog'' (pronounced "da cho"), said to take the form of a jealous woman.  Hmong men may even go to sleep dressed as women so as to avoid the attentions of this spirit.
 
==Synonyms==
* Sudden Unexpected Nocturnal Death Syndrome (SUNDS)
* Bangungut (Phillipines)<ref>{{cite journal|journal=Int J Epidemiol|year=1998|volume=27|issue=4|pages=677&ndash;84|title=Bangungut in Manila: sudden and unexplained death in sleep of adult Filipinos|author=Munger RG, Booton EA|pmid=9758125}}</ref>
* Dab tsog (Laos)<ref name="Alder1995"/>
* Laitai (Thailand)<ref name="Tatsanavivat"/>
* Pokkuri disease (Japan)<ref>{{cite journal|journal=Jpn Circ J|year=1976|volume=40|issue=7|pages=753&ndash;68|title=A histopathological study on the conduction system of the so-called "Pokkuri disease" (sudden unexpected cardiac death of unknown origin in Japan|author=Gotoh K|pmid=966364}}</ref>
 
==References==
<references/>
 
 
 
[[Category:Pathology|Pathology]]
 
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Latest revision as of 16:00, 14 May 2014

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