-, Nakajima K, Takeichi S, Nakajima Y, Fujita MQ. Methodist Debakey Cardiovasc J. Sudden Unexplained Nocturnal Death Syndrome in Central China (Hubei linked the significant cardiac morphological changes (fibrosis, loss of gap junctions) with the BrS phenotype and life-threatening arrhythmia.31 Our findings initially highlight the possible important role of subtly increased heart size in the pathogenesis of SUNDS, and provide the direct morphological evidence for the hypothesis that ion channel diseases without obvious cardiac structural abnormality (such as BrS, LQTS, CPVT, and SUNDS) may be a subtype of caridomyopathies.3133. The cardiac pathology of Department of Health and Human Services, Social Security Five deaths occurred in 1987 and Right bundle branch block, persistent ST segment . The number of total number of SUDS in SEA refugees to 117 since CDC surveillance Diabetic Dead-in-Bed Syndrome: A Possible Link to a Cardiac Ion To look for previously unrecognized cardiac structural abnormalities and address the genetic cause for sudden unexplained nocturnal death syndrome (SUNDS). Gussak I, LeMarec H, Nademanee K, Perez Riera AR, Shimizu W, Schulze-Bahr E, Tan The genetic etiology of SUNDS has been focused on BrS which reportedly shares the type 1-BrS ECG pattern with about 60% of survived SUNDS patients.2,34 The SCN5A gene is the most common BrS-susceptibility gene accounting for 2030% of the disorder. Sudden Unexpected Nocturnal Death Syndrome Since 1977 more than a hundred Southeast Asian immigrants in the United States have died from the mysterious disorder known as sudden unexpected nocturnal death syndrome (SUNDS). SEA refugees in the United States, although the number of reported Cheng J, Kyle JW, Lang D, Wiedmeyer B, Guo J, Yin K, Huang L, Vaidyanathan R, Su T, Makielski JC. Cardiovasc Pathol. Mutations in SCN5A were originally linked to SUNDS (2002) in 3 of 10 (30%) probands with clinical evidence of SUNDS, and thus SUNDS and BrS were considered to be the same allelic disorder.16 However, we reported only a 6.5% prevalence of SCN5A putative pathogenic variants in a much larger cohort of 123 SUNDS victims (2014).13 In current analysis, only 4.5 % of SUNDS cases hosted SCN5A variants (the incidence of SCN5A mutation was comparable to our previous study13) compared to 29.4% of BrS patients (this incidence was also consistent with our previous studies).35,36 Moreover, according to the strict ACMG guideline definition, none of SUNDS cases had pathogenic or likely pathogenic SCN5A variant compared to 18% of our BrS cohort. Genetic Testing Registry: Brugada syndrome, Genetic Testing Registry: Brugada syndrome 1, National Organization for Rare Disorders (NORD). 2005 Mar 31 [updated 2022 Aug 25]. Genetic and biophysical basis of sudden unexplained nocturnal death Control, All but one were male and all died at night. Both SUNDS cases and controls showed no significant macroscopic pathological changes except for non-specific morphological changes related to acute death (such as visceral congestion). Although epidemiological studies on the impact of body mass index to SCD risk are conflicting,27 over 60% of chronic ischemia heart disease associated SCD were reported to have overweight hearts.28 Cardiomegaly was identified to be the sole arrhythmogenic substrate in approximately 40% of structural heart disease related SCD without specific cardiomyopathy.29 Indexed left ventricular mass by body surface area was reported as an independent predictor of SCD and may help improve the risk prediction of SCD beyond routine cardiovascular risk factors.30 For SUNDS with apparently normal heart, whether or not there are more specific and detailed characteristics to establish pathological markers based on slightly increased heart mass for diagnosing SUNDS or predicting SCD risk deserves further investigation. 2020 Apr;141:43-52. doi: 10.1016/j.yjmcc.2020.02.010. eCollection 2020. Night terrors and sudden unexplained nocturnal death Nonischemic left ventricular scar and cardiac sudden death in the young. An original paper copy of this issue can be obtained from the Superintendent of Documents, U.S. Government Printing Office (GPO), Washington, DC 20402-9371; telephone: (202) 512-1800. Baron RC, Thacker SB, Gorelkin L, Vernon AA, Taylor WR, Choi K. We conducted a, Triplerisk hypothesis of SUNDS . Unable to load your collection due to an error, Unable to load your delegates due to an error, Predicted protein topology of ion channels and the location of variations responsible for, Proteintoprotein interaction of the 33 genes responsible for. 148 consecutive SUNDS cases were collected from January 1, 1998 to December 31, 2014 at the National Center for Medicolegal Expertise at Sun Yat-sen University. Report to Congress. decline in newly arrived SEA refugees, since most deaths occur By the 1980's SUNDS was the chief cause of death in Southeast Asian refugee camps across the United States. Sudden Unexplained Nocturnal Death Syndrome in Central : Medicine Together, these other genetic changes account for less than two percent of cases of the condition. Laukkanen JA, Khan H, Kurl S, Willeit P, Karppi J, Ronkainen K, Di Angelantonio E. Left ventricular mass and the risk of sudden cardiac death: a population-based study. HHS Vulnerability Disclosure, Help ionic aspects. Length of time in the United States was known for seven of the 13 The definition of SUNDS described a perplexing entity with special clinic phenotype:19 (1) predominantly occurs in Southeast Asia or immigrants from Southeast Asia without a significant disease history; (2) prevails preponderantly in apparently healthy males (>90%); (3) >80% of victims are at the ages between 2040; (4) occurs during nocturnal sleep with typical symptoms such as moaning and tachypnea which last for just a few minutes prior to death; (5) there is no pathological changes to identify the cause of death; (6) most victims were sporadic; (7) death most frequently occurred out of hospital without any clinical record, giving first access to forensic pathologists rather than the clinicians. Sudden arrhythmic death syndrome ( SADS) is a sudden unexpected death of adolescents and adults, mainly during sleep. Available from http://www.ncbi.nlm.nih.gov/books/NBK1517/. new insights into molecular diagnosis for Brugada syndrome. Mayo Clin Proc. Gervacio-Domingo G, Punzalan FE, Amarillo ML, Dans A. The .gov means its official. Only 2/44 SUNDS cases compared to 5/17 BrS patients hosted a rare variant in the most common BrS causing gene, SCN5A (P=.01). Sudden Unexpected Nocturnal Death Syndrome | Encyclopedia.com Mutations in these genes disrupt the flow of ions, impairing the heart's normal rhythm. Post-mortem Whole exome sequencing with gene-specific analysis for autopsy-negative sudden unexplained death in the young: a case series. Pokkuri Death Syndrome; sudden cardiac death cases without coronary atherosclerosis in South Asian young males. 1. 34 mutations in scn5a that alter repolarization and. Vatta M, Dumaine R, Varghese G, et al. It is prevalent in Southeast Asia, colloquially called Bangungut (Philippines), Pokkuri (Japan) and Lai-tai (Thailand). Epub 2016 Dec 28. A medical mystery - what causes Sudden unexplained nocturnal death Zhang L, Zhou F, Huang L, Wu Q, Zheng J, Wu Y, Yin K, Cheng J. Int J Legal Med. As with sodium channels, proper flow of ions through calcium and potassium channels in the heart muscle helps maintain a regular heartbeat. An additional 17 patients with Brugada syndrome (BrS) collected from January 1, 2006, to December 31, 2014, served as a comparative disease cohort. The pathological diagnosis for each case was confirmed by at least two forensic pathologists independently. Assuming the previous pattern continues, the number and This may limit a more precise assessment of normalized organ weight. 2012 Oct 2020 Apr;99(16):e19749. DePristo MA, Banks E, Poplin R, et al. within What is the prognosis of a genetic condition? Sudden unexplained nocturnal death syndrome (SUNDS) is a condition characterized by unexpected cardiac arrest in young adults, usually at night during sleep. Sudden Unexpected Nocturnal Death Syndrome, Sudden Unexpected Nocturnal Death Syndrome (SUNDS), or Sudden Unknown Nocturnal Death Syndrome are all terms for a syndrome first noted in 1915 in the Philippines and again in Japan in 1959, where it was named pokkuri ("sudden death"). F, Schmitt S, Bzieau S, Le Marec H, Dina C, Schott JJ, Probst V, Redon R. Federal government websites often end in .gov or .mil. The victim has no known antecedent illnesses, and there are no factors that might precipitate cardiac arrest. SUNDS had slight but statistically significantly increased heart weight and valve circumference compared to controls. This type of ion channel plays a critical role in maintaining the heart's normal rhythm. Is sudden unexplained nocturnal death syndrome in Southern China a cardiac sodium channel dysfunction disorder? What Is Sudden Death Syndrome? - Health Cardiomegaly is a common arrhythmogenic substrate in adult sudden cardiac deaths, and is associated with obesity. However, due to the small sample sizes, the uncertain definition of cardiomegaly (heart weight >350g, but not normalized to body size), the selection of cases (a large portion had structural heart disease), or the lack of controls, these findings and their significance need further confirmation. He surv. ABOUT MMWR | In: CDC surveillance summaries, Feb. The SUNDS victims had slight but statistically significant increased heart weight and valve circumference compared with controls. Shimizu W, Aiba T, Kamakura S. Mechanisms of disease: current understanding Sudden unexplained death syndrome (SUDS) occurs when a relatively young healthy person, usually male and Asian, dies unexpectedly while sleeping. 1 1 lai-tai in thailand, 2 pokkuri in japan, 3 4 the incidence of sunds (per 100,000 people-years) has been These 13 reports bring the What Is Sudden Death Syndrome, and Is Prevention Possible? - Healthline Tavora F, Zhang Y, Zhang M, et al. the DOI: 10.1161/JAHA.117.007837 Corpus ID: 30273935; Sudden Unexplained Nocturnal Death Syndrome: The Hundred Years' Enigma @article{Zheng2018SuddenUN, title={Sudden Unexplained Nocturnal Death Syndrome: The Hundred Years' Enigma}, author={Jingjing Zheng and Da Zheng and Terry Su and Jianding Cheng}, journal={Journal of the American Heart Association: Cardiovascular and Cerebrovascular Disease . Refugee Resettlement Program. Hertz CL, Ferrero-Miliani L, Frank-Hansen R, Morling N, Bundgaard H. Europace. 2005 Apr;2(4):429-40. Review. Otherwise, the geographic distribution of Since the initial report in 1917, sudden unexplained nocturnal death syndrome (SUNDS) has been considered an autopsy negative disorder with unknown etiology and describes a distinct subgroup of individuals with idiopathic sudden death.1 SUNDS prevails preponderantly in Southeast Asia and has multiple academic terms in different nations such as in the Philippines (bangungut),1 Thailand (lai-tai),2 Japan (pokkuri),3 and China (sudden manhood death syndrome).4 The incidence of SUNDS (per 100,000 people years) has been reported to be as high as 43 in the Philippines5 and 38 in Thailand.6 Kampuchea, Laos, and Hmong refugees in the United States were also reported to have a high incidence (59, 82, and 92 per 100,000 people years, respectively) of SUNDS.7,8 The annual incidence of SUNDS is approximately 13 per 100,000 people in Southern China.4,9. Author manuscript; available in PMC 2017 Nov 1. online-only references in the Supplements, catecholaminergic polymorphic ventricular tachycardia, sudden unexplained nocturnal death syndrome. Spectrum and prevalence of mutations involving BrS1- through BrS12-susceptibility SUNDS prevails mainly in Southeast Asian ethnic groups especially among Thai, Laotian, Cambodian, Vietnamese, Japanese and Chinese. Vatta M, Dumaine R, Varghese G, Richard TA, Shimizu W, Aihara N, Nademanee K, The long QT syndrome family of cardiac ion 5 Drinking alcohol. 2005 May 12/44 SUNDS victims (SCN5A, SCN1B, CACNB2, CACNA1C, AKAP9, KCNQ1, KCNH2, KCNJ5, GATA4, NUP155, ABCC9) and 6/17 BrS patients (SCN5A, CACNA1C, P>.05) carried rare variants in primary arrhythmia-susceptibility genes. Sudden unexplained nocturnal death syndrome (SUNDS) is a genetic disorder that can cause sudden death in young and healthy adults during sleep at night. 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