30333, U.S.A. 2021 May;18(5):778-784. doi: 10.1016/j.hrthm.2020.12.035. By the 1980's SUNDS was the chief cause of death in Southeast Asian refugee camps across the United States. 2018 Mar 3;7 (5):e007837. 34 mutations in scn5a that alter repolarization and. remains similar to that of previously reported cases (Table 1) (1). [Research Progress of the Relationship between SUNDS and OSAHS]. 2005 May In affected people without an identified gene mutation, the cause of Brugada syndrome is often unknown. 38,000 (5). Interestingly, the 14/44 SUNDS cases with variants within cardiomyopathy related genes had a significantly decreased circumference of both pulmonary (P=.002) and aortic valves (P=.003; this may indicate slight narrowing of both right and left ventricular outflow tract due to slight ventricular hypertrophy), and tended to die on average 56 years younger (26.218.58 versus 32.107.12 years, P=.02, Figure 1B, Supplemental Table 3 in the Supplement) than the 30 remaining SUNDS cases. Nakajima K, Takeichi S, Nakajima Y, Fujita MQ. Comprehensive forensic autopsy and histopathology examinations reveal no identifiable abnormalities to explain the underlying cause of death. remains unknown. Although these subtle structural cardiac changes did not meet the criteria for cardiomyopathy, this finding poses additional questions: 1) Is the slightly enlarged cardiac size a primary cause or a secondary change for SUNDS? 36,000 in 1987 (4,5). Moreover, only 2/44 (4.5%) SUNDS cases compared to 5/17 (29.4%, P=.01) BrS patients hosted at least one rare variant in the most common BrS causing gene SCN5A. Epub 2016 Aug 25. Spectrum and prevalence of mutations involving BrS1- through BrS12-susceptibility Postmortem molecular analysis of KCNQ1, KCNH2, KCNE1 and KCNE2 genes in sudden unexplained nocturnal death syndrome in the Chinese Han population. Forensic and clinical pathologists have attempted to uncover plausible pathogenic morphological characteristics underlying SUNDS for nearly a century.13 Several pilot autopsy studies have suggested some cardiac structure changes in SUNDS: Kirschner (1986),23 Park (1990),24 and Elfawal (2000) et al25 observed cardiomegaly or cardiac hypertrophy in 14 of 18 SUNDS cases (all with CCS anomalies), 4 of 14 SUNDS decedents (without grossly detectable structural cardiac anomaly), 7 of 22 SUNDS victims (2 cases had coronary stenosis, 7 had cardiac hypertrophy), respectively. Death from nightmare During sleep paralysis, the person is unable to move and experiences hypnagogic hallucinations or hears sounds that seem . CONTACT The broader molecular spectrum in arrhythmia associated genes indicates that SUNDS is only partially an allelic disorder to BrS. Type 508 Accommodation and the title of the report in the subject line of e-mail. The spectrum of epidemiology underlying sudden cardiac death. Epub 2012 Jul 25. Modell SM, Lehmann MH. Persons using assistive technology might not be able to fully access information in this file. Report to Congress. The victim has no known antecedent illnesses, and there are no factors that might precipitate cardiac arrest. 2006 Mar;8(3):143-55. Review. Environmental Hazards and Health Effects, Center for Environmental Contact GPO for current prices. Length of time in the United States was known for seven of the 13 The principles outlined in the Declaration of Helsinki were followed. All MMWR HTML documents published before January 1993 are electronic conversions from ASCII text into HTML. Brugada Syndrome. Refugee Resettlement Program. 2017 May;131(3):621-628. doi: 10.1007/s00414-016-1516-z. Disclaimer 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. This condition was originally described in Southeast Asian populations, where it is a major cause of death. Medeiros-Domingo A, Simone S, Will ML, Dagradi F, Schwartz PJ, Ackerman MJ. Kirschner RH, Eckner FA, Baron RC. These syndromes may be one of the many conditions that fall. eCollection 2020. Kirschner RH, Eckner FAO, Baron RC. Allegue C, Coll M, Mates J, et al. 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. These results prompt speculation that rare variants in cardiomyopathy-related genes may have a slight but actual impact on cardiac morphological changes which may contribute to primary or secondary arrhythmia in SUNDS. Am J Forensic Med Pathol. It is prevalent in Southeast Asia, colloquially called Bangungut (Philippines), Pokkuri (Japan) and Lai-tai (Thailand). To use the sharing features on this page, please enable JavaScript. After normalization, only average heart weight in SUNDS showed a significant increase compared to controls (P =.04, Supplemental Table 1 in the Supplement). Impact of Obstructive Sleep Apnea On In-Hospital Outcomes in Patients With Atrial Fibrillation: A Retrospective Analysis of the National Inpatient Sample. To look for previously unrecognized cardiac structural abnormalities and address the genetic cause for sudden unexplained nocturnal death syndrome (SUNDS). for Juang JM, Huang SK. GeneReviews [Internet]. Seattle (WA): University of Washington, Seattle; Refugee Program. Du F, Wang G, Wang D, Su G, Yao G, Zhang W, Su G. Medicine (Baltimore). Sudden unexplained nocturnal death syndrome has been identified in Southeast Asian male refugees, primarily the Hmong people, settling in the United States since 1975 and was the chief cause of death among these male refugees in the early 1980s, which was the peak time of Southeast Asian immigration to the United States ( 31 ). 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). 2016 Nov; 91(11): 15031514. As an ethnic and region specific natural death, sudden unexplained nocturnal death syndrome (SUNDS) or sudden unexplained death during sleep (SUDS), is a disorder that prevails predominantly in Southeast Asia and has various synonyms in different countries such as the Philippines (bangungut) , Thailand (lai-tai) , Japan (pokkuri) , and China (sudden manhood death syndrome) . Zhang Z, Chen H, Chen W, Zhang Z, Li R, Xu J, Yang C, Chen M, Liu S, Li Y, Wang T, Tu X, Huang Z. To look for previously unrecognized cardiac structural abnormalities and address the genetic cause for sudden unexplained nocturnal death syndrome (SUNDS). 1;11(3):337-45. whom time in the United States was known was 17 months. Tse G, Lee S, Liu T, Yuen HC, Wong ICK, Mak C, Mok NS, Wong WT. An additional 17 patients with Brugada syndrome (BrS) collected from January 1, 2006, to December 31, 2014, served as a comparative disease cohort. Tester DJ, Ackerman MJ. Desire Press: 1993;3850. Sudden Unexplained Nocturnal Death Syndrome listed as SUNDS. The inclusion criteria for SUNDS were as previously reported:4,9,1215 (1) an apparently healthy individual older than 15 years and without a history of significant disease; (2) who died of a sudden unexpected death during nocturnal sleep; (3) and had a negative autopsy, toxicology, histology, and death-scene investigation that resulted in their death being unexplained. 2020 Apr;99(16):e19749. Forensic Sci Int. Huang L, Tang S, Chen Y, Zhang L, Yin K, Wu Y, Zheng J, Wu Q, Makielski JC, Cheng J. Int J Legal Med. Gussak I, LeMarec H, Nademanee K, Perez Riera AR, Shimizu W, Schulze-Bahr E, Tan the display of certain parts of an article in other eReaders. J Electrocardiol. Culture & Lifestyle, Psychism Sleeping with the Dark Entities. decline in newly arrived SEA refugees, since most deaths occur These controls represented individuals with an acute non-disease death within 24 hours caused by traffic accident, mechanical asphyxia, electric shock, and carbon monoxide poisoning and were previously healthy, without any significant disease or pathological changes identified by postmortem examinations. resuscitated but was comatose when hospitalized; his condition Aug;2(8):408-14. Review. See this image and copyright information in PMC. What does it mean if a disorder seems to run in my family? Peters S. Ion channel diseases as a part in the definition and classification of cardiomyopathies recently confirmed in Brugada syndrome. This condition may explain some cases of sudden infant death syndrome (SIDS), which is a major cause of death in babies younger than 1 year. But apparently this same phenomenon occurs in adults in connection with the disturbance of cultural or religious beliefs. Mayo Clin Proc. Kapplinger JD, Tester DJ, Alders M, et al. (1) These deaths involved relatively young previously healthy persons. We've all heard of SIDS (Sudden Infant Death Syndrome) where a small child is found dead in its crib with no apparent cause of death to be found. Sudden death typically occurs around age 40. Assuming the previous pattern continues, the number and Australian researchers say they've identified one potential biomarker for sudden infant death syndrome, known as SIDS, but experts caution that it's just one piece of the puzzle.About 3,400 .. ikea leirvik bed frame replacement parts. total number of SUDS in SEA refugees to 117 since CDC surveillance The matching criteria were: (1) gender and race identical to the paired case; (2) age of death within 1 year; (3) interval of death date within 3 months. 1987 was 1.1 per 100,000, the lowest since 1976. doi: 10.7759/cureus.20770. Circ Res. Molecular pathological study on LRRC10 in sudden unexplained nocturnal death syndrome in the Chinese Han population. Crotti L, Marcou CA, Tester DJ, et al. The https:// ensures that you are connecting to the 2021 Aug 5;8:714844. doi: 10.3389/fcvm.2021.714844. Epub 2016 Jun 7. Is sudden unexplained nocturnal death syndrome in Southern China a cardiac sodium channel dysfunction disorder? Fibrosis, Connexin-43, and conduction abnormalities in the Brugada syndrome. 1-2 The long QT syndrome family of cardiac ion and Michaud K, Grabherr S, Faouzi M, Grimm J, Doenz F, Mangin P. Pathomorphological and CT-angiographical characteristics of coronary atherosclerotic plaques in cases ofsudden cardiac death. Epub 2004 cases (four), followed by Minnesota (two). However, using the strict ACMG guideline-based definition for pathogenicity, only 2/44 SUNDS cases hosted a likely pathogenic variant (p.Q376sp-KCNQ1, p.G626_P631del-KCNQ1) compared to 3/17 patients with BrS that hosted either a pathogenic or likely pathogenic variant (p.G400R-SCN5A, p.D1275N-SCN5A, T1893Pfs*29-SCN5A ; Table 3). What is the prognosis of a genetic condition? Monthly summary, December 31, 1987. Epub 2014 Oct 26. How can gene variants affect health and development? As a service to our customers we are providing this early version of the manuscript. In this model, we set a supposed, MeSH Liu C, Tester DJ, Hou Y, et al. Looking for abbreviations of SUNDS? two Testing the burden of rare variation in arrhythmia-susceptibility genes provides 2 the disease is termed "bangungut" in philippines, 1 "lai tai" in thailand, 2 Laotian man, had a cardiac arrest at night during sleep. Genet Med. 25;101(2):173-8. Review. median Cheng J, Makielski JC, Yuan P, et al. Antzelevitch C. Brugada syndrome: clinical, genetic, molecular, cellular and The continuous variables were examined accordingly with normal distributions and were then compared using the unpaired Student's t-test for normal distributions and the Mann-Whitney U-test for non-normal distributions. Researchers suspect that testosterone, a sex hormone present at much higher levels in men, may account for this difference. Available from http://www.ncbi.nlm.nih.gov/books/NBK1517/. The SUNDS group did not significantly differ from the matched controls in demographic characteristics including gender, age, height, and thickness of the layer of subcutaneous fat of abdominal wall (Table 1). Shimizu W. Acquired forms of the Brugada syndrome. Richards S, Aziz N, Bale S, et al. Authors Jingjing Zheng 1 , Da Zheng 1 , Terry Su 1 , Jianding Cheng 2 Affiliations 1 Department of Forensic Pathology, Zhongshan School of Medicine, Sun Yat-sen University, Guangzhou, China. Careers. Results: Cases with (1) obvious disease or pathological changes to explain the death; or (2) a non-natural manner of death (such as suicide, homicide, accident) were excluded. refugees: in patients with sudden cardiac death. the Review. North Carolina and 1. Various epidemiological surveys showed that sudden death cases in Japan are about 100 000 annually, of which 60% to 70% are caused by cardiovascular causes. Before The data were presented as mean SD for continuous variables or as frequencies and percentages for categorical variables. within -. SUNDS and BrS may result from different molecular pathological underpinnings. Most recently, Nademanee et al. 3 Many years of living with epilepsy. R01 HL128076/HL/NHLBI NIH HHS/United States, R56 HL071092/HL/NHLBI NIH HHS/United States. The condition, also known as sudden unexplained nocturnal death syndrome or the "moaning death," is commonly caused by a mutation in the SCN5A gene, which is responsible for forming sodium channels and maintaining normal heart rhythm by facilitating the transportation of positively charged sodium atoms into the heart muscle. We also acknowledge and thank all faculty members in our department for kind and expert help in checking the forensic pathological diagnosis of some cases. JAMA 2013 Together, these other genetic changes account for less than two percent of cases of the condition. It can occur in infants as well. Epub 2010 Nov 16. most Int J Cardiol. SUNDS occurs predominantly in Southeast Asia and has different academic terms but similar denitions in different countries, Sudden unexplained nocturnal death syndrome (SUNDS) is characterized by sudden unexplained death during sleep in apparently healthy young people, most of whom are males. 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"). Do vengeful spirits or evil forces cause nightmares, sleep paralysis, or psychic paralysis? SEA Heart Sudden unexplained nocturnal death syndrome (SUNDS), a disorder found in southeast Asia, is characterized by an abnormal electrocardiogram with ST-segment elevation in leads V1-V3 and sudden death due to ventricular fibrillation, identical to that seen in Brugada syndrome. Heart Rhythm. Liu C, Zhao Q, Su T, et al. The ePub format is best viewed in the iBooks reader. rate of SUDS deaths in 1988 will probably remain at 4-5 deaths and 2014 Jan-Mar;10(1):25-8. Review. FOIA government site. Monthly summary, September 30, 1987. Washington, Sudden unexplained nocturnal death syndrome (SUNDS) remains an autopsy negative disorder with unclear etiology. Sudden unexplained nocturnal death syndrome in Southern China: an epidemiological survey and SCN5A gene screening. 1600 Clifton Rd, MailStop E-90, Atlanta, GA Deeper on-going investigations on morphological, molecular pathological, and electrophysiological characteristics in larger SUNDS cohorts to address the etiology and mechanism of sudden death may contribute to the increasingly important precision medicine for SCD.40,41. (B) Compared with 30 SUNDS cases without rare variants in cardiomyopathy associated genes (N, 26.218.58 years), the 14 SUNDS cases with rare variants (P, 32.107.12 years) tended to die on average 56 years younger. This future database will benefit us for accurately understanding the pathogenesis of SUNDS. Cardiology. Missense mutations in plakophilin-2 cause sodium current deficit and associate with a Brugada syndrome phenotype. Arrhythmogenic marker for the sudden unexplained death syndrome in Thai men. Zhao Q, Chen Y, Peng L, et al. "Pokkuri" means suddenly and unexpectedly in Japanese, and Pokkuri Death Syndrome (PDS) refers to an unexplained type of SCD in Japan with unknown cause. Health and Injury Control, CDC, telephone: (404) 488-4780. Tungsanga K, Sriboonlue P. Sudden unexplained death syndrome in north-east Thailand. syndrome (SUNDS), a disease allelic to Brugada syndrome. Federal government websites often end in .gov or .mil. already built in. classied as sudden unexplained death (SUD). Brugada syndrome usually becomes apparent in adulthood, although it can develop any time throughout life. Disclaimer, National Library of Medicine Catherine Keane Death : The shattered mother of a youthful publicizing leader who kicked the bucket out of nowhere last year encouraged guardians to get their youngsters evaluated for Sudden Adult Death Syndrome on the off chance that there is a family background of heart disease. While phenotypically similar, SUNDS and BrS may not be genetically and functionally the same allelic disorder. Sudden Unexplained Nocturnal Death Syndrome - How is Sudden Unexplained Nocturnal Death Syndrome abbreviated? May 15;24(10):2757-63. doi: 10.1093/hmg/ddv036. 1-4 The victims are healthy and die during sleep, following agonal respiration. Nonischemic left ventricular scar and cardiac sudden death in the young. In addition, three earlier reports were Editorial Note: CDC continues to receive reports of sudden deaths Antzelevitch C, Brugada P, Borggrefe M, Brugada J, Brugada R, Corrado D, The An unexplained nocturnal phenomenon. One decedent, a 2015 850,000 SEA refugees live in the United States (3). Sudden unexplained nocturnal death syndrome (SUNDS) is a genetic disorder that can cause sudden death in young and healthy adults during sleep at night. unexplained death syndrome (SUDS) in Southeast Asian (SEA) refugees A retrospective investigation of 51 cases of sudden unexplained death syndrome (SUDS) reported to the Medico-Legal Centre in Dammam during the period January 1995 to June 1997 was carried out. Vinculin (VCL) was linked to sudden arrhythmia death in VCL knockout mice prior to . In: CDC surveillance summaries, Feb. In order to address whether SUNDS is truly morphologically negative, we performed a case-control study on gross and microscopic findings in the largest number of Chinese SUNDS autopsy cases reported to date. The site is secure. The Genome Analysis Toolkit: a MapReduce framework for analyzing next-generation DNA sequencing data. decedents and ranged from 1 to 11 years (median: 4 years). and Forensic Sci Int. Most are young men (35.97.8 years). Mayo Clin Proc. Sudden Unexplained Nocturnal Death Syndrome: The Hundred Years' Enigma J Am Heart Assoc. Twelve of All variants reported were confirmed by Sanger sequencing. were reported to CDC. Is sudden unexplained nocturnal death syndrome in Southern China a cardiac sodium channel dysfunction disorder? Methods: An additional 17 patients with Brugada syndrome (BrS) collected from January 1, 2006, to December 31, 2014, served as a comparative disease cohort. Does sudden unexplained nocturnal death syndrome remain the autopsy negative disorder: a gross, microscopic, and molecular autopsy investigation in Southern China - PMC Published in final edited form as: Open in a separate window No significant differences in the average left and right ventricular thickness were found between the two groups. 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. As a special idiopathic sudden cardiac death (SCD), it differs significantly in clinic phenotype from other primary electric disorders such as long or short QT syndrome (LQTS or SQTS), catecholaminergic polymorphic ventricular tachycardia (CPVT), etc. sudden unexplained nocturnal death Idiopathic death often in young, previously healthy, Southeastern Asian , attributed to a conduction system anomaly coupled with 'culture shock' and relocation-related stress in emigrants. genes in a cohort of unrelated patients referred for Brugada syndrome genetic Mutations in the SCN5A gene alter the structure or function of the channel, which reduces the flow of sodium ions into cells. He was Bellanger L, Barc J, Chatel S, Martins R, Mabo P, Sacher F, Hassaguerre M, Kyndt Update: Sudden Unexplained Death Syndrome Among Southeast Asian Re fugees -- United States Between October 1, 1986, and April 30, 1988, 10 cases of sudden unexplained death syndrome (SUDS) in Southeast Asian (SEA) refugees were reported to CDC. Signs and symptoms related to arrhythmias, including sudden death, can occur from early infancy to late adulthood. The remaining rare variants were categorized as variants of uncertain significance (VUS). Sudden unexpected nocturnal death among patients with diabetes is greatly feared and poorly understood, occurring approximately ten times more commonly than in the general population . F, Schmitt S, Bzieau S, Le Marec H, Dina C, Schott JJ, Probst V, Redon R. as SUDS based on additional information. In addition, we conducted a next-generation sequencing based 80 genes targeted analysis on consecutive 44 SUNDS victims and 17 BrS patients to characterize the molecular pathological spectrum of Chinese SUNDS compared to BrS. These abnormal rhythms are called ventricular arrhythmia, and they prevent the heart from pumping regularly. J Am Heart Assoc. di Gioia CR, Giordano C, Cerbelli B, Pisano A, Perli E, De Dominicis E, Poscolieri B, Palmieri V, Ciallella C, Zeppilli P, d'Amati G. Hum Pathol. (A) 148 SUNDS cases showed statistically significant increased circumferences of cardiac valves versus 444 controls. 2016 Dec;58:78-89. doi: 10.1016/j.humpath.2016.08.004. Clipboard, Search History, and several other advanced features are temporarily unavailable. Twelve of 44 SUNDS victims (SCN5A, SCN1B, CACNB2, CACNA1C, AKAP9, KCNQ1, KCNH2, KCNJ5, GATA4, NUP155, ABCC9) and 6 of 17 patients with BrS (SCN5A, CACNA1C; P>.05) carried rare variants in primary arrhythmia-susceptibility genes. Administration, 1988. Brugada syndrome: an update. All involved men, and the mean age at death was 34 (standard error of the mean 7) years. Molecular diagnostics of cardiovascular diseases in sudden unexplained death. MedlinePlus links to health information from the National Institutes of Health and other federal government agencies. This site needs JavaScript to work properly. 2015;116:18871906. eCollection 2021 Dec. Ramireddy A, Chugh HS, Reinier K, Uy-Evanado A, Stecker EC, Jui J, Chugh SS. Baron RC, Thacker SB, Gorelkin L, Vernon AA, Taylor WR, Choi K. 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.

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