1-4 The victims are healthy and die during sleep, following agonal respiration. Bellanger L, Barc J, Chatel S, Martins R, Mabo P, Sacher F, Hassaguerre M, Kyndt RSS this refugees. 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. We conducted a, Triplerisk hypothesis of SUNDS . How can gene variants affect health and development? 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. Targeted next generation sequencing revealed a novel deletion-frameshift mutation of KCNH2 gene in a Chinese Han family with long QT syndrome: A case report and review of Chinese cases. Forensic Sci Int. unexplained death syndrome (SUDS) in Southeast Asian (SEA) refugees We examined the distribution of the rare non-synonymous variants in 35 genes associated with primary arrhythmias such as BrS, LQTS, SQTS, CPVT, cardiac conduction disease, familial atrial fibrillation (AF), Wolff-Parkinson-White syndrome, and sick sinus syndrome.17 At least one rare variant in a primary arrhythmia causing gene was detected in 12/44 SUNDS cases (Table 3) and in 6/17 BrS patients (Table 4). The ePub format uses eBook readers, which have several "ease of reading" features Pathological characteristics of Pokkuri death syndrome; narrow circumferences of the coronary arteries in Pokkuri death syndrome cases. doi: 10.1161/JAHA.117.006320. An additional 444 non-SUNDS death cases were collected from the same autopsy case database and served as the control group. DOWNLOADS | 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. In addition, three earlier reports were The difference in distribution of rare variants in primary arrhythmia associated genes indicates that SUNDS and BrS may largely result from different molecular pathological underpinnings. 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. He surv. 2015;116:18871906. Author manuscript; available in PMC 2017 Nov 1. online-only references in the Supplements, catecholaminergic polymorphic ventricular tachycardia, sudden unexplained nocturnal death syndrome. Only 2 of 44 SUNDS cases compared with 5 of 17 patients with BrS hosted a rare variant in the most common BrS-causing gene, SCN5A (P=.01). Brugada Syndrome Medicine & Life Sciences Autopsy Medicine & Life Sciences China Medicine & Life Sciences Identification of Novel SCN5A Single Nucleotide Variants in Brugada Syndrome: A Territory-Wide Study From Hong Kong. September 26, 2011. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain. Burdine RD, Preston CC, Leonard RJ, Bradley TA, Faustino RS. . [3] [4] The syndrome is rare in most areas around the world but occurs in populations that are culturally and genetically distinct. Careers. doi: 10.1161/JAHA.116.005330. Aug;2(8):905. ACCESSIBILITY, Morbidity and Mortality Weekly Report Together, these other genetic changes account for less than two percent of cases of the condition. 2011;32:359363. Do vengeful spirits or evil forces cause nightmares, sleep paralysis, or psychic paralysis? Pokkuri Death Syndrome; sudden cardiac death cases without coronary atherosclerosis in South Asian young males. Control, 2016 Dec;58:78-89. doi: 10.1016/j.humpath.2016.08.004. 1SS): 43SS-53SS. Elfawal MA. 25;101(2):173-8. Review. 2002 Feb Genetic and biophysical basis of sudden unexplained nocturnal death syndrome (SUNDS), a disease allelic to Brugada syndrome. This type of ion channel plays a critical role in maintaining the heart's normal rhythm. Margherita Cummins' reality was crushed on July 1 last year. 2011;207:613. 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. The heart structure and death age in SUNDS victims, MeSH 2020 Apr;141:43-52. doi: 10.1016/j.yjmcc.2020.02.010. 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. length of time in the United States for the 88 of the 117 decedents 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. We propose that SUNDS occurs when 3 factors, The multifactorial model of SUNDS . By the 1980's SUNDS was the chief cause of death in Southeast Asian refugee camps across the United States. and transmitted securely. Case 14 was a 17-year-old male victim who died of sudden nocturnal death. May 1987, respectively. Sudden unexplained nocturnal death syndrome predominantly affects previously healthy, young adult Southeast Asian males, who die suddenly during sleep. Editorial Note: CDC continues to receive reports of sudden deaths (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. Molecular diagnostics of cardiovascular diseases in sudden unexplained death. 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. This site needs JavaScript to work properly. To look for previously unrecognized cardiac structural abnormalities and address the genetic cause for sudden unexplained nocturnal death syndrome (SUNDS). cases for Data for 148 SUNDS victims and 444 controls (matched 1:3 on sex, race, and age of death within 1 year) were collected from Sun Yat-sen University from January 1, 1998, to December 31, 2014, to search morphological changes. 1 Top of Page Oct;38(4 Suppl):22-5. Review. Right bundle branch block, persistent ST segment Yap EH, Chan YC, Goh KT, et al. 2004;101(4):157-69. 30333, U.S.A. Epub 2014 Oct 26. Sudden unexplained nocturnal death syndrome (SUNDS) is a condition characterized by unexpected cardiac arrest in young adults, usually at night during sleep. Our findings provide a new insight into approaches to morphologically and genetically diagnose patients with potential high risk for SUNDS. Fibrosis, Connexin-43, and conduction abnormalities in the Brugada syndrome. This condition was originally described in Southeast Asian populations, where it is a major cause of death. and 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). 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? These findings suggest the possibility that cardiomyopathy-susceptibility gene variants may contribute to lethal arrhythmia of SUNDS, and suggest this gene pool may be expected to yield additional novel genetic causes for SUNDS and BrS. You may notice problems with INTRODUCTION. Tungsanga K, Sriboonlue P. Sudden unexplained death syndrome in north-east Thailand. Desire Press: 1993;3850. Persons using assistive technology might not be able to fully access information in this file. Forensic Sci Int. SEA Figure 1. (October 1, 1987, to September 30, 1988) for the United States is arrivals has declined since its peak of 151,000 in 1980 and was Contact a health care provider if you have questions about your health. FOIA The SUNDS victims had slight but statistically significant increased heart weight and valve circumference compared with controls. SUNDS and Brugada syndrome (BrS) have been considered to be phenotypically, genetically, and functionally the same allelic disorder.16 We have previously reported postmortem genetic screening for SUNDS,4,1215 but these studies were limited by small autopsy numbers or a relative paucity of candidate genes screened. 2017 May;131(3):621-628. doi: 10.1007/s00414-016-1516-z. See this image and copyright information in PMC. Comprehensive forensic autopsy and histopathology examinations reveal no identifiable abnormalities to explain the underlying cause of death. Brugada P, Brugada J. HOME | 2014 Mar;236:38-45. doi: 10.1016/j.forsciint.2013.12.033. Informed consent was obtained from the patients or legal representatives of the victims. Post-mortem Whole exome sequencing with gene-specific analysis for autopsy-negative sudden unexplained death in the young: a case series. Modell SM, Lehmann MH. 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. Statistical analyses were conducted using IBM SPSS version 20.0 (IBM, Chicago IL, USA) and a P value <.05 was considered to be significant. The 12/44 SUNDS victims hosted AF (GATA4, NUP155, ABCC9), LQTS (AKAP9, KCNQ1, KCNH2, KCNJ5) and BrS (SCN5A, SCN1B, CACNB2, CACNA1C) associated rare variants while the 6/17 BrS patients carried only BrS (SCN5A, CACNA1C, P>.05) related rare variants. 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. For assistance, please send e-mail to: mmwrq@cdc.gov. Sudden unexplained nocturnal death syndrome (SUNDS) is a condition characterized by unexpected cardiac arrest in young adults, usually at night during sleep. The exact prevalence of Brugada syndrome is unknown, although it is estimated to affect 5 in 10,000 people worldwide. Most are young men (35.97.8 years). Tse G, Lee S, Liu T, Yuen HC, Wong ICK, Mak C, Mok NS, Wong WT. 1600 Clifton Rd, MailStop E-90, Atlanta, GA In turn, blood is prevented from reaching the brain and the rest of the body, which can. Hertz CL, Ferrero-Miliani L, Frank-Hansen R, Morling N, Bundgaard H. Europace. The average circumferences of all cardiac valves in SUNDS tended to be increased and the mitral valves were significantly increased compared to controls (Figure 1A), which is consistent with the slight increase in average heart weight, and confirming a slightly enlarged heart size in SUNDS. 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 ). Liu C, Tester DJ, Hou Y, et al. Is sudden unexplained nocturnal death syndrome in Southern China a cardiac sodium channel dysfunction disorder? This conversion may have resulted in character translation or format errors in the HTML version. The categorical variables were evaluated using the Pearson Chi-square test or Fishers exact test, with OR (95% CI) given afterwards if appropriate. Mirzaa GM, Pagon RA, Wallace SE, Bean LJH, Gripp KW, Amemiya A, editors. 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. F, Schmitt S, Bzieau S, Le Marec H, Dina C, Schott JJ, Probst V, Redon R. Eight cases of sudden unexplained death in Asian immigrants were reported during the study period. J Am Heart Assoc. All MMWR HTML documents published before January 1993 are electronic conversions from ASCII text into HTML. Bethesda, MD 20894, Web Policies eCollection 2020. 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. Notably, we observed that these rare variants were associated with altered heart structure and a tendency for earlier average age at death by approximately 5 years. May 15;24(10):2757-63. doi: 10.1093/hmg/ddv036.

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