BACKGROUND: Waldenström\'s macroglobulinemia (WM) is defined as a lymphoplasmacytic lymphoma (LPL) involving the bone marrow (BM) with presence of IgM monoclonal protein, and comprises > 95% of all LPL cases. Rituximab-based regimens have been predominant in the management of WM. Infusion-related reactions (IRRs) are a primary concern with rituximab, although it is generally better tolerated with less toxicity than conventional anticancer agents. Here, we present an autopsy case of an elderly man who died suddenly after receiving the initial infusion of rituximab for WM/LPL.
METHODS: An 84-year-old man was found dead in his bedroom. He had undergone the initial intravenous rituximab infusion for progressive anemia related to Waldenström\'s macroglobulinemia/lymphoplasmacytic lymphoma (WM/LPL) approximately 15 h before death. Although the protocol for rituximab administration and additional medication was considered appropriate, he exhibited several symptoms consistent with infusion-related reactions (IRRs) during the infusion. Autopsy revealed monotonous proliferation of small-to-medium-sized lymphocytic cells in the bone marrow, consistent with the premortem diagnosis of WM/LPL. Additionally, immunoglobulin λ-light chain-derived amyloid (ALλ) deposition was identified in all organs other than the brain. Although ALλ deposition and LPL infiltration were found in the heart, they were not severe enough to cause severe functional impairment. Severe congestion and/or edema were observed in the lungs, liver, and brain. Although significant inflammatory cell infiltration was not found in any organs, laboratory tests revealed elevated serum levels of inflammatory cytokines, including interleukin-1β, interleukin-6, tumor necrosis factor-α and the presence of IgM-λ monoclonal protein.
CONCLUSIONS: Acute IRRs associated with the initial rituximab infusion were the major contributing factor to his sudden unexpected death. The autopsy findings of present case suggest the necessity for thorough monitoring of older patients with WM/LPL undergoing rituximab treatment, particularly when pronounced IRRs occur during the first administration, in addition to investigating complications of WM/LPL before infusion.

UNASSIGNED: Sudden death accounts for approximately 10% of deaths among working-age adults and is associated with poor air quality. Objectives: To identify high-risk groups and potential modifiers and mediators of risk, we explored previously established associations between fine particulate matter (PM2.5) and sudden death stratified by potential risk factors.
UNASSIGNED: Sudden death victims in Wake County, NC, from 1 March 2013 to 28 February 2015 were identified by screening Emergency Medical Systems reports and adjudicated (n = 399). Daily PM2.5 concentrations for Wake County from the Air Quality Data Mart were linked to event and control periods. Potential modifiers included greenspace metrics, clinical conditions, left ventricular hypertrophy (LVH), and neutrophil-to-lymphocyte ratio (NLR). Using a case-crossover design, conditional logistic regression estimated the OR (95%CI) for sudden death for a 5 μg/m3 increase in PM2.5 with a 1-day lag, adjusted for temperature and humidity, across risk factor strata.
UNASSIGNED: Individuals having LVH or an NLR above 2.5 had PM2.5 associations of greater magnitude than those without [with LVH OR: 1.90 (1.04, 3.50); NLR > 2.5: 1.25 (0.89, 1.76)]. PM2.5 was generally less impactful for individuals living in areas with higher levels of greenspace.
UNASSIGNED: LVH and inflammation may be the final step in the causal pathway whereby poor air quality and traditional risk factors trigger arrhythmia or myocardial ischemia and sudden death. The combination of statistical evidence with clinical knowledge can inform medical providers of underlying risks for their patients generally, while our findings here may help guide interventions to mitigate the incidence of sudden death.

Molecular autopsy has recently been gaining attention as a means of postmortem diagnosis; however, it is usually performed using the victim\'s blood sample at the time of death. Here, we report the first case of a deceased infant with Brugada syndrome whose diagnosis was made with banked cord blood. A seemingly healthy 1-year-old male infant collapsed while having a fever; this collapse was witnessed by his mother. Despite cardiopulmonary resuscitation, he died of ventricular fibrillation. No abnormalities of cardiac structure were identified on autopsy. Genomic samples were not stored at the time because of a lack of suspicion for familial arrhythmia. Five years later, his sister showed Brugada electrocardiogram pattern while febrile from Kawasaki disease. Their father showed a spontaneous type 1 Brugada electrocardiogram pattern. A heterozygous SCN5A p.R893C variant was found by genetic testing in the proband\'s father and sister. Furthermore, the proband\'s genetic testing was performed using his banked cord blood, which identified the same variant. Family history of Brugada syndrome with an SCN5A-R893C variant and clinical evidence led to a postmortem diagnosis of Brugada syndrome in the proband. Identification of this variant in this case later contributed to verifying SCN5A-R893C as a pathogenic variant through data accumulation. Banked cord blood may prove useful for conducting molecular autopsies in previously undiagnosed cases of sudden death in which genomic samples were not stored.

BACKGROUND: Sudden Unexplained Death in Childhood (SUDC) needs to be fully assessed considering its impact on the family, parents and siblings. Inborn Errors of Metabolism (IEM) such as Medium-Chain Acyl-CoA Dehydrogenase Deficiency (MCADD) should be taken into consideration when SUDC occurres. Our aim is to present a family with two successive SUDC and to discuss the post-mortem genetics investigations revealing an IEM implication.
METHODS: A complete autopsy with genetic testing was performed when the proband, a 4-year-old girl, died. A few years previously, her older brother had died at the same age and off the same condition. Years later, his exhumation was necessary in order to perform a post-mortem diagnosis.The two siblings were revealed to have had the same pathogenic genotype of the ACADM gene, heterozygous substitutions in ACADM (NM_000016.5): c.985 A>G p.(Lys329Glu) and c.347 G>A p.(Cys116Tyr). In addition, they also both carried a VUS in TECRL, a gene implicated in Catecholaminergic Polymorphic Tachycardia Ventricular (CPVT) and SUDC.
CONCLUSIONS: We illustrate the importance of exome analyses for investigating unexplained sudden death, especially in children, with the possible impact for genetic counselling in the family. The finding of the implication of ACADM gene in this case, raises likely responsibility of the public health system in countries such as France, who delayed implementation of new born screening for these conditions. Exome analyses in this case detected unexpected complexity in interpretation linked to the identification of a second candidate gene for SUDC.

马某，女，70岁，某年12月5日11：00许因“胸痛3 h”到医院就诊，门诊查心电图显示“急性前壁心肌梗死”，以“冠心病-急性心肌梗死”收住院。入院后冠状动脉造影显示：冠状动脉左前降支近段闭塞。入院后对症治疗，完善各项检查提示无手术禁忌证，于12月13日11：46行“冠状动脉造影和支架植入术”，手术过程顺利。术后患者于当日19：30左右出现头晕、恶心、呕吐症状，20：30左右开始嗜睡，于12月14日08：08左右出现叹息样呼吸，呼之不应，意识不清，经抢救无效，于当日08：48宣布死亡。.

暂无摘要。

UNASSIGNED: In view of anecdotal reports of sudden unexplained deaths in India\'s apparently healthy young adults, linking to coronavirus disease 2019 (COVID-19) infection or vaccination, we determined the factors associated with such deaths in individuals aged 18-45 years through a multicentric matched case-control study.
METHODS: This study was conducted through participation of 47 tertiary care hospitals across India. Cases were apparently healthy individuals aged 18-45 years without any known co-morbidity, who suddenly (<24 h of hospitalization or seen apparently healthy 24 h before death) died of unexplained causes during 1 st October 2021-31 st March 2023. Four controls were included per case matched for age, gender and neighborhood. We interviewed/perused records to collect data on COVID-19 vaccination/infection and post-COVID-19 conditions, family history of sudden death, smoking, recreational drug use, alcohol frequency and binge drinking and vigorous-intensity physical activity two days before death/interviews. We developed regression models considering COVID-19 vaccination ≤42 days before outcome, any vaccine received anytime and vaccine doses to compute an adjusted matched odds ratio (aOR) with 95 per cent confidence interval (CI).
RESULTS: Seven hundred twenty nine cases and 2916 controls were included in the analysis. Receipt of at least one dose of COVID-19 vaccine lowered the odds [aOR (95% CI)] for unexplained sudden death [0.58 (0.37, 0.92)], whereas past COVID-19 hospitalization [3.8 (1.36, 10.61)], family history of sudden death [2.53 (1.52, 4.21)], binge drinking 48 h before death/interview [5.29 (2.57, 10.89)], use of recreational drug/substance [2.92 (1.1, 7.71)] and performing vigorous-intensity physical activity 48 h before death/interview [3.7 (1.36, 10.05)] were positively associated. Two doses lowered the odds of unexplained sudden death [0.51 (0.28, 0.91)], whereas single dose did not.
UNASSIGNED: COVID-19 vaccination did not increase the risk of unexplained sudden death among young adults in India. Past COVID-19 hospitalization, family history of sudden death and certain lifestyle behaviors increased the likelihood of unexplained sudden death.

Electronic gaming has recently been reported as a precipitant of life-threatening cardiac arrhythmia in susceptible individuals.
The purpose of this study was to describe the population at risk, the nature of cardiac events, and the type of game linked to cardiac arrhythmia associated with electronic gaming.
A multisite international case series of suspected or proven cardiac arrhythmia during electronic gaming in children and a systematic review of the literature were performed.
Twenty-two patients (18 in the case series and 4 via systematic review; aged 7-16 years; 19 males [86%]) were identified as having experienced suspected or proven ventricular arrhythmia during electronic gaming; 6 (27%) had experienced cardiac arrest, and 4 (18%) died suddenly. A proarrhythmic cardiac diagnosis was known in 7 (31%) patients before their gaming event and was established afterward in 12 (54%). Ten patients (45%) had catecholaminergic polymorphic ventricular tachycardia, 4 (18%) had long QT syndrome, 2 (9%) were post-congenital cardiac surgery, 2 (9%) had \"idiopathic\" ventricular fibrillation, and 1 (after Kawasaki disease) had coronary ischemia. In 3 patients (14%), including 2 who died, the diagnosis remains unknown. In 13 (59%) patients for whom the electronic game details were known, 8 (62%) were war games.
Electronic gaming can precipitate lethal cardiac arrhythmias in susceptible children. The incidence appears to be low, but syncope in this setting should be investigated thoroughly. In children with proarrhythmic cardiac conditions, electronic war games in particular are a potent arrhythmic trigger.

Previous studies have reported a substantial between-country variation in the case fatality rates (CFRs) of aneurysmal subarachnoid hemorrhage (SAH). However, contrary to comparisons among countries, nationwide comparisons within countries that focus on populations with equal access to health care and include out-of-hospital deaths in analyses are lacking. Thus, we aimed to investigate whether the SAH CFRs vary between geographic regions within Finland.
We identified all hospitalized and nonhospitalized (sudden-death) cases with aneurysmal SAH in Finland during 1998-2017 through 2 externally validated nationwide registers. According to the municipality of residence, we divided the cases with SAH into 5 geographic regions: Southern, Central, Western, Northern, and Eastern Finland, each served by a University Central Hospital with a neurosurgical service. In addition to overall 30-day CFRs, we computed sudden death rates and 30-day CFRs after hospitalization for each region. Using logistic and Poisson regression models, we calculated regional age-adjusted, sex-adjusted, and year-adjusted odds ratios and annual percent changes with 95% CIs for CFRs.
During 1998-2017, we identified a total of 9,443 cases with SAH, of which 3,484 (36.9%) occurred in Southern Finland. In comparison with the overall 30-day CFR of Southern Finland (35.1%), the age-adjusted, sex-adjusted, and study year-adjusted odds of SAH death were 32% (16%-50%) higher in Central Finland (42.7%), 39% (23%-58%) higher in Eastern Finland (43.4%), and 52% (33%-74%) higher in Western Finland (47.1%). The regional differences were present among both sexes, in all age groups, in sudden death rates, and in 30-day CFRs after hospitalization. Between 1998 and 2017, the overall 30-day CFRs decreased in Central (2.4% [1.0%-3.8%] per year) and Southern (1.2% [0.2%-2.2%] per year) Finland, whereas CFRs remained stable in the other regions. In the last 4 years of the study period (2014-2017), Southern Finland had the lowest 30-day CFR (16.5%) among hospitalized patients.
SAH CFRs seem to vary significantly even within a country with relatively equal access to health care. Future studies with detailed individual-level data are needed to explore whether health inequities explain the reported findings.

Primary mediastinal large B-cell lymphoma (PMLBCL) is an aggressive tumor originating from thymic B-cells. Clinically, it presents with general signs such as cough, chest pain and dyspnea. Although these symptoms are not specific, they are severe enough to reveal the disease. We report an autopsy case of a 25-year-old man, with a recent past history of cough and dyspnea, for which he consulted twice the emergency department and no diagnosis was made. He presented to the Emergency Unit, with a sudden onset of a dyspnea followed by a loss of consciousness. He was shortly declared dead after, a medico-legal autopsy was requested. On external examination, no traumatic lesions on the body were found, an important cyanosis of the face and ears, was, however, found. On autopsy, a mediastinal mass was found, measuring 19 cm × 25 cm and weighing 600 g, extending to the infra-hyoid region and to the thoracic cage and infiltrating the pericardium. Trachea had a necrotic mucosa with a partially obstructive lymph node mass. The diagnosis of a primary mediastinal large B-cell lymphoma was suspected based on pathological and immunohistochemical findings. The cause of death was finally attributed to respiratory failure due to this tumor.