sudden unexplained nocturnal death syndrome

Juang JM, Huang SK. JAMA. Association of common variants in NOS1AP gene with sudden unexplained nocturnal death syndrome in the southern Chinese Han population. 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. Environmental Hazards and Health Effects, Center for Environmental doi: 10.7759/cureus.20770. Brugada syndrome--an under-recognized electrical disease All The categorical variables were evaluated using the Pearson Chi-square test or Fishers exact test, with OR (95% CI) given afterwards if appropriate. A framework for variation discovery and genotyping using next-generation DNA sequencing data. Researchers suspect that testosterone, a sex hormone present at much higher levels in men, may account for this difference. While phenotypically similar, SUNDS and BrS may not be genetically and functionally the same allelic disorder. We present the first comprehensive autopsy evidence that SUNDS victims had concealed cardiac morphological changes, and that cardiomyopathy-related rare genetic variants may contribute conceivably to the cardiac abnormal structure and lethal arrhythmia underlying SUNDS. Approximately The long QT syndrome family of cardiac ion In: CDC surveillance summaries, Feb. A multicenter report. Forensic Sci Int. Although Brugada syndrome affects both men and women, the condition appears to be 8 to 10 times more common in men. An East Asian Common Variant Vinculin P.Asp841His Was Associated With Sudden Unexplained Nocturnal Death Syndrome in the Chinese Han Population. Although studies have We propose that SUNDS occurs when 3 factors, The multifactorial model of SUNDS . only Brgdar A, Yi J, Awan A, Taha M, Ogunti R, Gharbin J, Prafulla M, Opoku I. Cureus. Nakajima K, Takeichi S, Nakajima Y, Fujita MQ. DISCLAIMER | Structural heart disease were gradually excluded from this disorder and the vast majority of reported SUNDS cases have remained an autopsy-negative enigma.1,35,79,1215, In this study we provide from a large SUNDS cohort, the first morphological evidence of a slight but significantly larger heart size. decedents and ranged from 1 to 11 years (median: 4 years). 2012 Oct Figure 1. The principles outlined in the Declaration of Helsinki were followed. Introduction. If untreated, the irregular heartbeats can cause fainting (syncope), seizures, difficulty breathing, or sudden death. This site needs JavaScript to work properly. The heart structure and death age in SUNDS victims, MeSH 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. Sudden unexplained death during sleep occurred commonly in the general population in the Philippines: a sub study of the National Nutrition and Health Survey. and 2016 Dec;58:78-89. doi: 10.1016/j.humpath.2016.08.004. Liu C, Tester DJ, Hou Y, Wang W, Lv G, Ackerman MJ, Makielski JC, Cheng J. Forensic Sci Int. The . These abnormal rhythms are called ventricular arrhythmia, and they prevent the heart from pumping regularly. 2006 Mar;8(3):143-55. Review. 2020 Apr;99(16):e19749. He had an episode of syncope few months prior to his death but he did not seek medical attention. What is the prognosis of a genetic condition? Brugada syndrome can be caused by mutations in one of several genes. Huang L, Wu KH, Zhang L, Wang Q, Tang S, Wu Q, Jiang PH, Lin JJ, Guo J, Wang L, Loh SH, Cheng J. J Am Heart Assoc. 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 . CDC. A case-control study of sudden unexplained nocturnal death syndrome in the southern Chinese Han population. 1600 Clifton Rd, MailStop E-90, Atlanta, GA 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. Genet Med. HHS Vulnerability Disclosure, Help Type 508 Accommodation and the title of the report in the subject line of e-mail. Cheng J, Kyle JW, Lang D, Wiedmeyer B, Guo J, Yin K, Huang L, Vaidyanathan R, Su T, Makielski JC. FOIA DC: in Sudden unexplained death syndrome (SUDS) occurs when a relatively young healthy person, usually male and Asian, dies unexpectedly while sleeping. Epub 2016 Jun 7. government site. 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. Shimizu W, Aiba T, Kamakura S. Mechanisms of disease: current understanding SUNDS and BrS may result from different molecular pathological underpinnings. The information on this site should not be used as a substitute for professional medical care or advice. Bellanger L, Barc J, Chatel S, Martins R, Mabo P, Sacher F, Hassaguerre M, Kyndt Informed consent was obtained from the patients or legal representatives of the victims. Publisher's Disclaimer: This is a PDF file of an unedited manuscript that has been accepted for publication. Brugada Syndrome Medicine & Life Sciences Autopsy Medicine & Life Sciences China Medicine & Life Sciences 2005 Mar 31 [updated 2022 Aug 25]. Le Scouarnec S, Karakachoff M, Gourraud JB, Lindenbaum P, Bonnaud S, Portero most Takeichi S, Nakajima K, Nakajima Y, Fujita MQ. remains unknown. These findings revealed that SUNDS cases were not associated with obvious structural cardiac diseases (such as viral myocarditis and typical cardiomyopathy). 2017 Apr 3;6(4):e005330. the first 2 years after arrival in the United States. Washington, 8600 Rockville Pike 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). He was Refugee Program. Bethesda, MD 20894, Web Policies Contact a health care provider if you have questions about your health. Mayo Clin Proc. Epub 2004 Epub 2015 Feb 3. Twelve of Mutations in the SCN5A gene alter the structure or function of the channel, which reduces the flow of sodium ions into cells. at death ranged from 19 to 57 years (median: 33 years). HHS Vulnerability Disclosure, Help Postmortem molecular analysis of KCNQ1, KCNH2, KCNE1 and KCNE2 genes in sudden unexplained nocturnal death syndrome in the Chinese Han . Comprehensive forensic autopsy and histopathology examinations reveal no identifiable abnormalities to explain the underlying cause of death. This site needs JavaScript to work properly. This condition was originally described in Southeast Asian populations, where it is a major cause of death. Tse G, Lee S, Liu T, Yuen HC, Wong ICK, Mak C, Mok NS, Wong WT. The consecutive 17 BrS patients during January 1, 2006 to December 31, 2014 from the Department of Cardiology at the First Affiliated Hospital of Sun Yat-Sen University were collected and designed as a comparative disease cohort for the genetic screening with the SUNDS victims. and future challenges in Brugada syndrome. 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. 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. 1983;250:29472951. [2] One relatively common type is known as Brugada syndrome. and Health Effects, Center for Environmental Health and Injury Health and Injury Control, CDC, telephone: (404) 488-4780. Front Cardiovasc Med. 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. Nine were Laotian (five Hmong, two lowland Lao, and two Maron BJ, Towbin JA, Thiene G, et al. In. (A) 148 SUNDS cases showed statistically significant increased circumferences of cardiac valves versus 444 controls. Assuming the previous pattern continues, the number and See our, URL of this page: https://medlineplus.gov/genetics/condition/brugada-syndrome/. This conversion may have resulted in character translation or format errors in the HTML version. These 13 reports bring the The cardiac pathology of sudden, unexplained nocturnal death in Southeast Asian refugees. SUNDS had slight but statistically significantly increased heart weight and valve circumference compared to controls. Impact of Obstructive Sleep Apnea On In-Hospital Outcomes in Patients With Atrial Fibrillation: A Retrospective Analysis of the National Inpatient Sample. At least one rare variant in a cardiomyopathy-associated gene was identified in 14/44 SUNDS cases (Table 3) and 7/17 patients with BrS (Table 4). 2021 Dec 28;13(12):e20770. First reported in 1917 in the Philippines (1,2), sudden unexplained nocturnal death syndrome (SUNDS) remains a puzzling disorder with unknown etiology. for Li A, Behr ER. Sudden arrhythmic death syndrome ( SADS) is a sudden unexpected death of adolescents and adults, mainly during sleep. Young E, Xiong S, Finn L, Young T. Unique sleep disorders profile of a population-based367 sample of 747 Hmong immigrants in Wisconsin. Blackwell JN, Walker M, Stafford P, Estrada S, Adabag S, Kwon Y. Circ Rep. 2019;1(12):568-574. doi: 10.1253/circrep.cr-19-0085. Laotian man, had a cardiac arrest at night during sleep. Sudden unexplained nocturnal death among Southeast Asians Since July 1977 at least 96 cases of SUNDS occurring in Southeast Asian refugees have been reported to the Center for Disease Control (CDC). Cardiovasc Pathol. He surv. F, Schmitt S, Bzieau S, Le Marec H, Dina C, Schott JJ, Probst V, Redon R. The 14/44 SUNDS cases with cardiomyopathy-related variants had a subtle but significantly decreased circumference of cardiac valves, and tended to die on average 56 years younger compared to the remaining 30 cases (P=.02). September 26, 2011. and 1987 was 1.1 per 100,000, the lowest since 1976. Each variant was then scrutinized for additional supporting evidence for pathogenicity as outlined in the ACMG guidelines22. Spectrum and prevalence of mutations involving BrS1-through BrS12-susceptibility genes in a cohort of unrelated patients referred for Brugada syndrome genetic testing: implications for genetic testing. In addition to causing a nongenetic form of this disorder, these factors may trigger symptoms in people with an underlying mutation in SCN5A or another gene. Mayo Clin Proc. Quick Summary: Brugada Syndrome is a disorder that affects the lower chambers of the heart leading to irregular heartbeats and disruption of the heart's normal rhythm. Genetic Characteristics and Transcriptional Regulation of Sodium Channel Related Genes in Chinese Patients With Brugada Syndrome. were men. SUNDS prevails mainly in Southeast Asian ethnic groups especially among Thai, Laotian, Cambodian, Vietnamese, Japanese and Chinese. Allegue C, Coll M, Mates J, et al. Eranti A, Aro AL, Kerola T, et al. The collected autopsy findings included gender, age, height, time of death, the investigation record of death scene, and the macroscopic and microscopic examinations of vital organs. 1SS): 43SS-53SS. cases (four), followed by Minnesota (two). Brugada syndrome: an update. Federal government websites often end in .gov or .mil. Epub 2016 Dec 28. Standards and guidelines for the interpretation of sequence variants: a joint consensus recommendation of the American College of Medical Genetics and Genomics and the Association for Molecular Pathology. J Mol Cell Cardiol. . cases and the crude death rate continue to decline. 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. Epub 2016 Aug 25. Leonard RJ, Preston CC, Gucwa ME, Afeworki Y, Selya AS, Faustino RS. U.S. Department of Health and Human Services, Idiopathic ventricular fibrillation, Brugada type, Sudden unexpected nocturnal death syndrome. Brugada R, Campuzano O, Sarquella-Brugada G, Brugada P, Brugada J, Hong K. Epub 2012 Jul 25. Sudden Unexpected Nocturnal Death Syndrome. In this model, we set a supposed, MeSH The main risk factors for SUDEP are: Uncontrolled or frequent seizures 1 Generalized convulsive (also called tonic-clonic or grand mal) seizures 1 Other possible risk factors may include Seizures that begin at a young age. deteriorated, and he was pronounced brain dead 4 days later. 1992 Nov 15;20(6):1391-6. Fourteen of 44 SUNDS cases with cardiomyopathy-related variants had a subtle but significantly decreased circumference of cardiac valves, and tended to die on average 5 to 6 years younger compared with the remaining 30 cases (P=.02). Researchers have determined that SUNDS and Brugada syndrome are the same disorder. JAMA 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. 2018 Jan 6;7(1):e006320. Mutations in these genes disrupt the flow of ions, impairing the heart's normal rhythm. SEA At least one ultra-rare variant (absent in all publically available control databases) was observed in 7/44 SUNDS cases (1 case with a CACNA1C variant, 1 DSP, 1 EYA4, 1 GATA4, 1 MYBPC3, 1 MYH7, and 1 case with both a KCNQ1 and KCNH2 variant) and 6/17 patients with BrS (3 with a SCN5A variant, 2 MYH6, and 1 CACNA1C). refugees directly to the Surveillance and Programs Branch, Division CDC. the In people of this age, the unexplained death is known as sudden adult death syndrome (SADS). Statistical analyses were conducted using IBM SPSS version 20.0 (IBM, Chicago IL, USA) and a P value <.05 was considered to be significant. ACCESSIBILITY, Morbidity and Mortality Weekly Report 1;11(3):337-45. We conducted a, Triplerisk hypothesis of SUNDS . After normalization, only average heart weight in SUNDS showed a significant increase compared to controls (P =.04, Supplemental Table 1 in the Supplement). Li H, Durbin R. Fast and accurate short read alignment with Burrows-Wheeler transform. which previously had not reported cases, had SUDS cases in January Epub 2017 Feb 25. Review. GeneReviews [Internet]. 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. Nademanee K, Veerakul G, Nimmannit S, et al. The https:// ensures that you are connecting to the 9;60(15):1410-8. doi: 10.1016/j.jacc.2012.04.037. Heart Rhythm. The spectrum of epidemiology underlying sudden cardiac death. SUNDS - Sudden Unexplained Nocturnal Death Syndrome. Bookshelf 2021 May;18(5):778-784. doi: 10.1016/j.hrthm.2020.12.035. Right bundle branch block, persistent ST segment (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. CONTACT Liu M, Yang KC, Dudley SC., Jr Cardiac sodium channel mutations: why so many phenotypes? The ePub format uses eBook readers, which have several "ease of reading" features Kapplinger JD, Tester DJ, Alders M, et al. Brugada Syndrome. Available from http://www.ncbi.nlm.nih.gov/books/NBK1517/. 2005 Apr;2(4):429-40. Review. The most commonly mutated gene in this condition is SCN5A, which is altered in approximately 30 percent of affected individuals. Brugada R, Campuzano O, Sarquella-Brugada G, Brugada J, Brugada P. Brugada JAMA 1983;250:2947-51. Although the present study was the largest Chinese SUNDS autopsy case series to characterize the morphological changes and the primary genetic spectrum, the relatively small sample size for genetic testing as well as the lack of clinic records including ECG and genetic screening of family members limited a deeper analysis of association between clinical phenotype, morphological changes and genetic findings. Nakajima K, Takeichi S, Nakajima Y, Fujita MQ. Pokkuri Death Syndrome; sudden cardiac death cases without coronary atherosclerosis in South Asian young males. 2015 A comparison of genetic findings in sudden cardiac death victims and cardiac patients: the importance of phenotypic classification. Do vengeful spirits or evil forces cause nightmares, sleep paralysis, or psychic paralysis? official website and that any information you provide is encrypted Vatta M, Dumaine R, Varghese G, et al. This gene provides instructions for making a sodium channel, which normally transports positively charged sodium atoms (ions) into heart muscle cells. This condition occurs much more frequently in people of Asian ancestry, particularly in Japanese and Southeast Asian populations. The exact prevalence of Brugada syndrome is unknown, although it is estimated to affect 5 in 10,000 people worldwide. Washington, How are genetic conditions treated or managed? decline in newly arrived SEA refugees, since most deaths occur FOIA sudden unexplained nocturnal death syndrome (sunds) has gained attention all over the world since it was first reported in 1917. the phenomenon of sudden unexplained nocturnal death in patients without known cardiac disease has been examined for more than a century. 1. Hertz CL, Ferrero-Miliani L, Frank-Hansen R, Morling N, Bundgaard H. Europace. Epub 2010 Nov 16. An additional 17 patients with Brugada syndrome (BrS) collected from January 1, 2006, to December 31, 2014, served as a comparative disease cohort. Thus further studies for the morphological and molecular pathological characterizations of SUNDS are justified. Brugada P, Brugada J. 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. Cheng J, Makielski JC, Yuan P, et al. 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. 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. Sangwatanaroj S, Prechawat S, Sunsaneewitayakul B, Sitthisook S, Tosukhowong P, Tungsanga K. New electrocardiographic leads and the procainamide test for the detection of the Brugada sign in sudden unexplained death syndrome survivors and their relatives. 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. CDC. 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. Epub 2013 Oct 22. Disclaimer 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. Looking for abbreviations of SUNDS? new 3 Missed doses of medicine. arrhythmia; sudden cardiac death; sudden death; sudden unexplained nocturnal death syndrome. length of time in the United States for the 88 of the 117 decedents Sudden Unexplained Nocturnal Death Syndrome - How is Sudden Unexplained Nocturnal Death Syndrome abbreviated? Since 1982, the number of SUDS cases has Sudden unexplained nocturnal death syndrome (SUNDS) is a condition characterized by unexpected cardiac arrest in young adults, usually at night during sleep. 1993-2022. All involved men, and the mean age at death was 34 (standard error of the mean 7) years. SUNDS and BrS may result from different molecular pathological underpinnings. 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