Atypical presentation of Takayasu aortitis and myocarditis contributing to sudden death in a young Canadian first nations adult.

This is a rare presentation of Takayasu arteritis in a 30-year-old Canadian First Nations woman with cardiac and aortic root-predominant disease, which manifested in complete heart block. She had a past medical history significant for substance misuse. At presentation, cardiac magnetic resonance imaging identified diffuse thickening of the left atrium and ventricular outflow tract with left ventricular cavity dilation and preserved systolic function. A pacemaker was inserted at this time. Nine months later, the patient died following an out-of-hospital cardiac arrest in the context of cocaine intoxication. At autopsy, the cardiac thickening was also found to involve the proximal aortic root, which on microscopy demonstrated non-infectious aortitis and myocarditis with a granulomatous inflammatory pattern and dense fibrosis indicative of Takayasu arteritis. Important clinical clues to the diagnosis include age, sex, and Pacific Islands, American indigenous and Asian ethnicity. The case also underscores the need to rule out secondary causes of complete heart block, including systemic vasculitides, for all patients regardless of substance use history.

Sudden death in heart failure with preserved ejection fraction and beyond: an elusive target.

Heart failure (HF) with preserved ejection fraction (HFpEF) represents half of HF patients, who are more likely older, women, and hypertensive. Mortality rates in HFpEF are higher compared with age- and comorbidity-matched non-HF controls and lower than in HF with reduced ejection fraction (HFrEF); the majority (50-70%) are cardiovascular (CV) deaths. Among CV deaths, sudden death (SD) (~ 35%) and HF-death (~ 20%) are the leading cardiac modes of death; however, proportionally, CV deaths, SD, and HF-deaths are lower in HFpEF, while non-CV deaths constitute a higher proportion of deaths in HFpEF (30-40%) than in HFrEF (~ 15%). Importantly, the underlying mechanism of SD has not been clearly elucidated and non-arrhythmic SD may be more prominent in HFpEF than in HFrEF. Furthermore, there is no specific strategy for identifying high-risk patients, probably due to wide heterogeneity in presentation and pathophysiology of HFpEF and a plethora of comorbidities in this population. Thus, the management of HFpEF remains problematic due to paucity of data on the clinical benefits of current therapies, which focus on symptom relief and reduction of HF-hospitalization by controlling fluid retention and managing risk-factors and comorbidities. Matching a specific pathophysiology or mode of death with available and novel therapies may improve outcomes in HFpEF. However, this still remains an elusive target, as we need more information on determinants of SD. Implantable cardioverter-defibrillators (ICDs) have changed the landscape of SD prevention in HFrEF; if ICDs are to be applied to HFpEF, there must be a coordinated effort to identify and select high-risk patients.

Forensic Pathology Analysis of 363 Sudden Death Cases in Yunnan Province.

OBJECTIVES: To study the epidemiological and pathological features of sudden death （SD） in Yunnan Province and to provide scientific evidence for prevention and forensic identification of sudden death. METHODS: Totally 363 SD cases were collected from the autopsies between 2009 and 2017 in the Forensic Centre of Kunming Medical University. The related factors such as etiology, age, inducing factor, time interval between the onset of disease and death, morbidity season and pathological change were retrospectively analysed. RESULTS: The incidence of SD in males was significantly higher than that of females. The peak age was ≥35-55 years. The mortality rate was relatively high within 6 h after the onset of disease. The season order with descending number of deaths was spring, summer, winter and autumn. The top ten causes of SD were coronary heart disease, sudden unexplained death （SUD）, cerebral hemorrhage, acute hemorrhagic necrotic pancreatitis, aortic dissection rupture, cardiomyopathy, pneumonia, pulmonary thromboembolism, amniotic fluid embolism and allergy. Exercise, infusion, surgery, medication and minor injury were the most common predisposing factors of sudden coronary death. Consciousness disorder or coma, chest pain or chest tightness, and abdominal pain were the most common premortem symptoms of sudden coronary death. CONCLUSIONS: The SD is more common in middle-aged males, which is the key population for the prevention of SD. For the forensic identification and prevention of SD, the attention on SUD should be paid.

Provision of sudden unexpected death in epilepsy (SUDEP) information among Malaysian parents of children with epilepsy.

Sudden unexpected death in epilepsy (SUDEP) is an important cause of mortality in epilepsy. To date, there is only one published UK study evaluating information provision of SUDEP among parents of children with epilepsy (CWE), and there are no studies published from Asia. Although SUDEP information provision is recommended among parents of CWE, it is uncertain if these recommendations are applicable to Asian countries due to the different cultural attitude towards epilepsy. Our prospective cohort study consisted of multiethnic parents of children with epilepsy (CWE) seen in a tertiary hospital in Malaysia. Information on SUDEP was delivered to parents using an epilepsy educational software program. Participants completed a set of standardized questionnaire and Depression Anxiety Stress Scales-Short Form (DASS-21) immediately after and retested 3-6months after the SUDEP information provision. A total of 127 parents (84 mothers) participated in the study. The CWE consisted of 3 ethnic groups (38% Malay, 30% Chinese, 32% Indian) with a mean age of 9.6years. Majority (70.9%) felt positive after SUDEP information provision, 90.6% wanted SUDEP discussion for themselves with 70.1% wanted SUDEP discussion with their child, and a lower proportion (58.3%) would discuss SUDEP with their child. None of the participants reported increased symptoms of depression, stress or anxiety attributed to SUDEP information provision. Most parents took steps to reduce SUDEP risk, and most parents did not report an impact on their own functioning. However, there was an increase in parental report over time of impact on their child's functioning following SUDEP information (P<0.05). In conclusion, most Malaysian parents of CWE wanted SUDEP information. Following SUDEP information disclosure, majority did not report negative emotions; however, an increase in parents over time reported an impact on their child. Our findings reiterate that provision of SUDEP information should form part of care of CWE and parents should receive ongoing support as they undergo a period of parenting adjustment when dealing with the information provided.

Relation of Household Income to Incidence of Sudden Unexpected Death in Wake County, North Carolina.

The incidence of out-of-hospital sudden unexpected death (OHSUD) in a racially and socioeconomically diverse population has been inadequately studied. We collated all OHSUDs over a 24-month period among 18- to 64-year olds in Wake County, North Carolina, to investigate geographic and socioeconomic disparity in incidence of OHSUD. An electronic query of Wake County Emergency Medical Services (EMS) identified all EMS attended out-of-hospital deaths. After excluding trauma, expected deaths, and deaths occurring in non-free-living subjects, medical records and medical examiner's reports were reviewed by a committee of cardiologists to make the determination of OHSUD. Victims were geocoded to census tracts, and demographic and socioeconomic data were obtained from the 2014 American Community Survey and 2010 US Census. Incidence was examined by sociodemographic group with univariate analysis and multivariable regression. There were 397 OHSUDs, and 53% of census tracts had >1 event. The incidence of OHSUD was 64 of 100,000; 107 of 100,000 among blacks; and 60 of 100,000 among whites. Census tracts with >1 OHSUD had a higher population of blacks, a greater proportion unmarried, a lower median household income, and a greater proportion residing in a rural area. Only median household income remained a significant predictor of OHSUD after adjustment in multivariable analysis. Low median household income of a community portends a higher incidence of sudden death. In conclusion, interventions to reduce the incidence of sudden death need to be developed with these specific communities in mind.

Genetic variants in KCNE1, KCNQ1, and NOS1AP in sudden unexplained death during daily activities in Chinese Han population.

Fifty-six sudden unexplained death (SUD) cases were collected from Chinese Han population, which occurred during daily activities and were autopsy negative in comprehensive postmortem autopsy. The coding exons of potassium channel genes KCNE1, KCNQ1, and nitric oxide synthase gene NOS1AP were sequenced. A synonymous mutation, KCNE1 F54F T>C was identified in 2 SUD cases, which was absent in the control subjects. Neither genotype nor allele frequencies of KCNE1 and KCNQ1 exhibited a significant difference between the SUD and control group. In contrast, the allele frequency (p = 2.7 × 10(-10)) and genotype frequency (p = 5.9 × 10(-7)) of rs3751284, and the genotype frequency (p = 2.9 × 10(-2)) of rs348624 in NOS1AP of SUD were significantly different from that of controls (p < 0.05). Our study suggested that rs3751284 and rs348624 might be susceptibility loci for SUD during daily activities. Larger sample sizes and further molecular studies are needed to confirm or exclude an effect of the NOS1AP SNPs on SUD risk.

The socio-cultural perception of death in Turkish society recorded in lament epics (1955-1975).

This study explores the socio-cultural perception of death among Turkish people. For this reason, 210 published lament epics written by Turkish folk singers across all of Turkey concerning deaths between 1955 and 1975 were selected for analysis. These epics were published on single pages and were sold. The statistical analysis based on detailed content analysis was done at the univariate, bivariate, and multivariate levels. The results of the study provide a full picture of perception of cases of death in Turkish society. These results show Turkish society is especially sensitive to cases of death at young age and to the murdered. Further, a clear perception of the working of fate is encountered in deaths resulting from disaster and accidents; but the desire for vengeance is recorded in those laments concerning martyrs and the murdered. The statistical data show that most commonly cited reasons for death after road accidents, were a consequence of relationships with the opposite sex and from a sense of honor.

[KCNQ1, KCNH2, KCNE1 and KCNE2 potassium channels gene variants in sudden manhood death syndrome].

OBJECTIVE: To investigate KCNQ1, KCNH2, KCNE1 and KCNE2 gene variants in the cases of sudden manhood death syndrome (SMDS). METHODS: One hundred and sixteen sporadic cases of SMDS and one hundred and twenty-five healthy controlled samples were enrolled. Genomic DNA was extracted from blood samples. Gene variants of KCNQ1, KCNH2, KCNE1 and KCNE2 were screened by direct sequencing. RESULTS: A total of 14 mutations and 14 SNP were detected. Two non-synonymous mutations of them were newfound. There was no non-synonymous mutation found in the control group. CONCLUSION: There are KCNQ1, KCNH2, KCNE1 and KCNE2 gene variants found in Chinese SMDS cases. KCNQ1, KCNH2, KCNE1 and KCNE2 gene mutation may correlate partly with the occurrence of some cases of the SMDS in China.

Maternal and infant characteristics associated with accidental suffocation and strangulation in bed in US infants.

To identify maternal and infant characteristics associated with accidental suffocation and strangulation in bed (ASSB) in US infants. Using 2000-2002 US linked infant birth and death certificate cohort files, we compared ASSB deaths to survivors. Adjusted odds ratios (aOR) from logistic regression were used to analyze associations between selected maternal and infant characteristics and ASSB mortality. During 2000-2002, 1,064 infants died from ASSB, resulting in an ASSB mortality rate of 9.2 per 100,000 live births. Most ASSB deaths (71%) occurred before an infant reached 4 months old. Maternal factors associated with an increased risk of ASSB were younger age (using maternal age of 25-29 years as reference aOR 2.6 for mothers <20 years old and 1.6 for mothers 20-24 years old), lower educational attainment (aOR 4.3 for <12 years and 3.3 for 12 years compared to ≥16 years), multiparity (aOR 1.7, 2.2, and 3.5 for parity 2, 3, and 4 or higher, respectively) and smoking during pregnancy (aOR 2.8). Compared to non-Hispanic whites, non-Hispanic blacks (aOR 1.8) and American Indians (aOR 1.8) were more likely to have an ASSB death. Being male and born preterm were also associated with a higher ASSB mortality risk. Younger, less educated, mulitparous, non-Hispanic black or American Indian women and their families who smoke during their pregnancy and deliver male or preterm infants, may need more intense safe sleeping education during the infant's first year of life, especially during the first 4 months of age.

KCNQ1, KCNH2, KCNE1 and KCNE2 potassium channels gene variants in sudden manhood death syndrome / 法医学杂志

OBJECTIVE@#To investigate KCNQ1, KCNH2, KCNE1 and KCNE2 gene variants in the cases of sudden manhood death syndrome (SMDS).@*METHODS@#One hundred and sixteen sporadic cases of SMDS and one hundred and twenty-five healthy controlled samples were enrolled. Genomic DNA was extracted from blood samples. Gene variants of KCNQ1, KCNH2, KCNE1 and KCNE2 were screened by direct sequencing.@*RESULTS@#A total of 14 mutations and 14 SNP were detected. Two non-synonymous mutations of them were newfound. There was no non-synonymous mutation found in the control group.@*CONCLUSION@#There are KCNQ1, KCNH2, KCNE1 and KCNE2 gene variants found in Chinese SMDS cases. KCNQ1, KCNH2, KCNE1 and KCNE2 gene mutation may correlate partly with the occurrence of some cases of the SMDS in China.

The common African American polymorphism SCN5A-S1103Y interacts with mutation SCN5A-R680H to increase late Na current.

The common polymorphism SCN5A-S1103Y (∼13% allelic frequency in African Americans) is a risk factor for arrhythmia, sudden unexplained death (SUD), and sudden infant death syndrome. Prompted by a case of autopsy-negative SUD in a 23-year-old African American man who collapsed while playing football, we hypothesized that S1103Y interacted with other SCN5A variants to pathologically modify sodium current (I(Na)). Mutational analysis of arrhythmia-associated genes in the victim revealed the variants SCN5A-R680H and SCN5A-S1103Y. These variants were made both separately and in the same cDNA construct of the alternative splice variant backgrounds (SCN5A-Q1077del and Q1077) and expressed in HEK293 cells. In the most abundant SCN5A-Q1077del, late I(Na) for S1103Y alone was not significantly different from wild type (WT). However, late I(Na) for R680H, R680H+S1103Y (coexpressed), and R680H/S1103Y (on the same cDNA) was increased 2.1-, 3.4-, and 3.6-fold, respectively, compared with WT. Intracellular acidosis (pH 6.7) increased late I(Na) for S1103Y, R680H, R680H+S1103Y, and R680H/S1103Y by 2.2-, 2.4-, 5.0-, and 5.5-fold, respectively, compared with WT at pH 6.7. Expression in the less abundant SCN5A-Q1077 showed no increased late I(Na). This is the initial report of a functional interaction for the common polymorphism S1103Y with another mutation in the major transcript Q1077del of SCN5A. The "double hit" and environmental factor of acidosis may have converged to cause arrhythmic sudden death in this case.

Voodoo death.

Scholarly writing on voodoo death is reviewed. Criticisms that voodoo deaths in indigenous societies have never been well documented are refuted with cases medically documented in developed nations. The work of Cannon and Richter on sudden death in animals is reviewed and dismissed as irrelevant for understanding voodoo death. The role of starvation and dehydration is discussed, and it is suggested that the given-up/giving-up hypothesis best fits the phenomenon of voodoo death. Hypotheses for future research are suggested.

The lived experience of losing a loved one to a sudden death in KwaZulu-Natal, South Africa.

AIM: The aim of this study was to describe the lived experiences of families in KwaZulu-Natal, South Africa, who had lost a loved one to a sudden death. BACKGROUND: Sudden death implies a natural or unnatural death which is unexpected; occurs without warning; and in some cases, could have been prevented. It usually occurs out of hospital, in the emergency department, or shortly after handing the client over to either critical care or operating theatre personnel. A sudden death has the capacity to leave the bereaved emotionally damaged and may exaggerate the responses to grief. METHOD: An interpretive hermeneutic phenomenological research approach was chosen for this study, as this allows the researcher to describe a phenomenon as experienced and to attempt to provide an understanding of the internal meanings of a person's experiences in the lived world. SAMPLE: The sample included five bereaved family members who had lost a loved one to a sudden death and who were all members of a bereavement support group held in Durban, South Africa. RESULTS: Bereaved families interviewed described five themes namely: 'cold reception' of the hospital staff, lack of closure, acknowledgement of loss, 'loneliness of grief' and helping others. RELEVANCE FOR CLINICAL PRACTICE: This qualitative study provides rich data regarding what bereaved family members view as important in their care during this time of crisis. The value of simple, small gestures by the health professional is enlightening. This information can be used to ensure that health professionals purposely direct their interventions and interactions with the bereaved family to ensure that they are managed in the most therapeutic way possible.

Alleged lethal sorcery in East Timor.

A wide range of cultural and social perspectives exists on the concept of sudden and unexpected death. In countries, without a formal system of death investigation, sudden death is shrouded in mysticism often based on traditional belief systems. This cultural perspective on sudden death is often at variance with medical and forensic concepts and may include explanations such as sorcery, magic, and voodoo. In this case report, the postmortem findings in an alleged victim of lethal 'black magic', known as ema halo by the indigenous people of East Timor, is described. The alleged victim died suddenly in front of witnesses. At autopsy, marked dilation of a bicuspid aortic valve with annuloaortic ectasia and a sinus of Valsalva aneurysm was found after exhumation of the body. The findings mitigated the local belief in witchcraft and established a natural manner of death.

Sudden maternal deaths in Malaysia: a case report.

We report on a retrospective study of maternal deaths in Malaysia that occurred within 24 hours of delivery, abortion or operative termination of the pregnancy (defined as sudden deaths) in the years 1995-1996. There were 131 sudden maternal deaths (20.6% of all maternal deaths); postpartum hemorrhage, obstetric embolisms, trauma and hypertensive disorders of pregnancy were the main causes. There was a disproportionately increased risk of sudden maternal deaths in the Chinese and the 'other bumiputra' racial groups. The proportion of mothers who had no obstetric risk factors in the pregnancy that led to death was 16.8%. Fourteen mothers died in transit Twenty mothers died after a cesarean section. The findings of this review emphasize the fact that caregivers in obstetrics need to be forever vigilant. All maternity staff need to be well trained in emergency care and there needs to be quick referral to centers that can provide expertise in handling these emergencies.

Helpfulness of nursing actions to suddenly bereaved family members in an accident and emergency setting in Hong Kong.

BACKGROUND: The sudden death of a family member is always traumatic to the family. Nursing actions such as providing comfort measures and providing an opportunity to view the deceased were consistently perceived as helpful by the suddenly bereaved. Little is known about the perception of bereaved family members in Hong Kong concerning the care they received in accident and emergency (A & E) departments. This study serves to identify actions, which were perceived as helpful in A & E setting in Hong Kong. AIM: To gain knowledge about what nursing actions bereaved family members in Hong Kong perceived as helpful. METHOD: This study was exploratory and descriptive in nature, and used a quantitative approach. Data were collected through structured telephone interviews using a modified Tye's questionnaire. RESULTS: Seventy-six bereaved subjects were recruited in an A & E department in Hong Kong. The subjects perceived written information, opportunity to view the deceased, and respecting individual customs and religious procedures as most helpful and conversely, offering sedation, discouraging viewing of the body, and providing comfort measures as most unhelpful. No statistically significant differences among the overall mean of helpful nursing actions according to the subjects' age, gender, education level, family income and religions were found while using Mann-Whitney's U and Kruskal-Wallis' tests. Significant correlations between some actions and the respondents' age, family income and educational level were found. CONCLUSION: Accident and emergency nurses have unique role in offering helpful actions to support suddenly bereaved family members.

Pattern of inheritance in three sudden unexplained death syndrome ("Lai-tai") families.

Sudden Unexplained Death Syndrome (SUDS) (or Lai-tai) is sudden death in previously healthy young adults without any structural cause of death from autopsy findings. Our previous data showed that familial SUDS is not X-linked recessive. The objective of this study was to determine the pattern of inheritance in familial SUDS using the ECG markers of Brugada syndrome (RBBB and ST-segment elevation in V1 to V3), SUDS and presumptive SUDS as phenotypes. We employed the standard 12-lead ECG and higher intercostal space (ICS) V1 to V3 (-V1 to -V3 and -2V1 to -2V3) leads ECG in SUDS relatives after procainamide and drew the pedigree. We studied 62 relatives of 9 SUDS victims who died in Singapore and selected 3 families (n = 34) for the procainamide test and ECG. The mean age was 36.4 +/- 23.6 years (4-78 years). Three SUDS families showed the same pattern of inheritance of autosomal dominant.

Critical thinking, health policy, and the Hmong culture group, Part I.

The author discusses the historical connection between the Hmong and the U.S. military operations in South Eastern Asia; gives an overview of Hmong culture health traditions, such as efficacious, neutral and harmful; socioeconomic data; immigration and the local community; and health problems specific to the Hmong.