Characteristics of Sudden Unexpected Infant Deaths on Shared and Nonshared Sleep Surfaces.

OBJECTIVES: Describe characteristics of sudden unexpected infant deaths (SUID) occurring on shared or nonshared sleep surfaces. METHODS: We examined SUID among residents of 23 US jurisdictions who died during 2011 to 2020. We calculated frequencies and percentages of demographic, sleep environment, and other characteristics by sleep surface sharing status and reported differences of at least 5% between surface sharing and nonsharing infants. RESULTS: Of 7595 SUID cases, 59.5% were sleep surface sharing when they died. Compared with nonsharing infants, sharing infants were more often aged 0 to 3 months, non-Hispanic Black, publicly insured, found supine, found in an adult bed or chair/couch, had a higher number of unsafe sleep factors present, were exposed to maternal cigarette smoking prenatally, were supervised by a parent at the time of death, or had a supervisor who was impaired by drugs or alcohol at the time of death. At least 76% of all SUID had multiple unsafe sleep factors present. Among surface-sharing SUID, most were sharing with adults only (68.2%), in an adult bed (75.9%), and with 1 other person (51.6%). Surface sharing was more common among multiples than singletons. CONCLUSIONS: Among SUID, surface sharing and nonsharing infants varied by age at death, race and ethnicity, insurance type, presence of unsafe sleep factors, prenatal smoke exposure, and supervisor impairment. Most SUID, regardless of sleep location, had multiple unsafe sleep factors present, demonstrating the need for comprehensive safe sleep counseling for every family at every encounter.

The impact of damaging epilepsy and cardiac genetic variant burden in sudden death in the young.

BACKGROUND: Sudden unexpected death in children is a tragic event. Understanding the genetics of sudden death in the young (SDY) enables family counseling and cascade screening. The objective of this study was to characterize genetic variation in an SDY cohort using whole genome sequencing. METHODS: The SDY Case Registry is a National Institutes of Health/Centers for Disease Control and Prevention surveillance effort to discern the prevalence, causes, and risk factors for SDY. The SDY Case Registry prospectively collected clinical data and DNA biospecimens from SDY cases < 20 years of age. SDY cases were collected from medical examiner and coroner offices spanning 13 US jurisdictions from 2015 to 2019. The cohort included 211 children (median age 0.33 year; range 0-20 years), determined to have died suddenly and unexpectedly and from whom DNA biospecimens for DNA extractions and next-of-kin consent were ascertained. A control cohort consisted of 211 randomly sampled, sex- and ancestry-matched individuals from the 1000 Genomes Project. Genetic variation was evaluated in epilepsy, cardiomyopathy, and arrhythmia genes in the SDY and control cohorts. American College of Medical Genetics/Genomics guidelines were used to classify variants as pathogenic or likely pathogenic. Additionally, pathogenic and likely pathogenic genetic variation was identified using a Bayesian-based artificial intelligence (AI) tool. RESULTS: The SDY cohort was 43% European, 29% African, 3% Asian, 16% Hispanic, and 9% with mixed ancestries and 39% female. Six percent of the cohort was found to harbor a pathogenic or likely pathogenic genetic variant in an epilepsy, cardiomyopathy, or arrhythmia gene. The genomes of SDY cases, but not controls, were enriched for rare, potentially damaging variants in epilepsy, cardiomyopathy, and arrhythmia-related genes. A greater number of rare epilepsy genetic variants correlated with younger age at death. CONCLUSIONS: While damaging cardiomyopathy and arrhythmia genes are recognized contributors to SDY, we also observed an enrichment in epilepsy-related genes in the SDY cohort and a correlation between rare epilepsy variation and younger age at death. These findings emphasize the importance of considering epilepsy genes when evaluating SDY.

Explaining Sudden Unexpected Infant Deaths, 2011-2017.

BACKGROUND: Sudden unexpected infant death (SUID) represents a broad group of explained and unexplained infant deaths (<1 year old). Explaining why SUID occurs is critical to understanding etiology and prevention. Death certificate data cannot differentiate explained from unexplained SUID cases nor describe the surrounding circumstances. We report SUID rates by explained and unexplained categories and describe demographics and history of recent injury or illness using the Centers for Disease Control and Prevention SUID Case Registry. METHODS: The registry is a population-based surveillance system built on Child Death Review programs. Data are derived from multiple sources, including death certificates, scene investigations, and autopsy reports. Cases included SUIDs reported by states or jurisdictions participating in the registry during 2011-2017. Cases were classified into explained and unexplained categories by using the registry's classification system. Frequencies, percentages, and mortality rates per 100 000 live births were calculated. RESULTS: Of the 4929 SUID cases, 82% were categorized as unexplained. Among all cases, 73% had complete case information. Most SUIDs (72%) occurred in an unsafe sleep environment. The SUID mortality rate was 97.3 per 100 000 live births. Among explained and possible suffocation deaths, ∼75% resulted from airway obstruction attributed to soft bedding. CONCLUSIONS: Unsafe sleep factors were common in explained and unexplained SUID cases, but deaths could only be classified as explained suffocation for ∼20% of cases. Further analysis of unexplained deaths, including continued improvements to death scene investigation and documentation, may generate hypotheses for physiologic and genetic research, advance our understanding of gaps in SUID investigation, and enhance our understanding of infants at highest risk.

Epidemiology of Sudden Death in a Population-Based Study of Infants and Children.

OBJECTIVE: To describe epidemiologic data from the Sudden Death in the Young (SDY) Case Registry. Understanding the scope of SDY may optimize prevention efforts. STUDY DESIGN: We analyzed sudden, unexpected deaths of infants (<365 days) and children (1-17 years) from a population-based registry of 8 states/jurisdictions in 2015 and 9 in 2016. Natural deaths and injury deaths from drowning, motor vehicle accident drivers, and infant suffocation were included; other injury deaths, homicide, suicide, intentional overdose, and terminal illness were excluded. Cases were categorized using a standardized algorithm. Descriptive statistics were used to characterize deaths, and mortality rates were calculated. RESULTS: Of 1319 cases identified, 92% had an autopsy. We removed incomplete cases, leaving 1132 analyzable deaths (889 infants, 243 children). The SDY rate for infants was 120/100 000 live births and for children was 1.9/100 000 children. Explained Cardiac rates were greater for infants (2.7/100 000 live births) than children (0.3/100 000 children). The pediatric Sudden Unexpected Death in Epilepsy (SUDEP) mortality rate was 0.2/100 000 live births and children. Blacks comprised 42% of infant and 43% of child deaths but only 23% of the population. In all ages, myocarditis/endocarditis was the most common Explained Cardiac cause; respiratory illness was the most common Explained Other cause. SDY occurred during activity in 13% of childhood cases. CONCLUSIONS: Prevention strategies include optimizing identification and treatment of respiratory and cardiac diseases.

Sleep-Related Infant Suffocation Deaths Attributable to Soft Bedding, Overlay, and Wedging.

BACKGROUND: Unintentional suffocation is the leading cause of injury death among infants <1 year old in the United States, with 82% being attributable to accidental suffocation and strangulation in bed. Understanding the circumstances surrounding these deaths may inform prevention strategies. METHODS: We analyzed data from the population-based Sudden Unexpected Infant Death Case Registry from 2011 to 2014. Cases categorized as explained suffocation with unsafe sleep factors (suffocation), per the Centers for Disease Control and Prevention's Sudden Unexpected Infant Death Case Registry classification system, were included and assigned a mechanism of obstruction, including soft bedding, overlay, or wedging. We calculated frequencies and percentages of suffocation deaths by mechanism and selected demographic and sleep-environment characteristics. RESULTS: Fourteen percent of sudden unexpected infant death cases were classified as suffocation; these cases were most frequently attributed to soft bedding (69%), followed by overlay (19%) and wedging (12%). Median age at death in months varied by mechanism: 3 for soft bedding, 2 for overlay, and 6 for wedging. Soft-bedding deaths occurred most often in an adult bed (49%), in a prone position (82%), and with a blanket (or blankets) obstructing the airway (34%). Overlay deaths occurred most often in an adult bed (71%), and infants were overlaid by the mother (47%). Wedging deaths occurred most often when the infant became entrapped between a mattress and a wall (48%). CONCLUSIONS: Safe sleep environments can reduce infant suffocation deaths. Increased knowledge about the characteristics of suffocation deaths can help inform prevention strategies by targeting highest-risk groups.

The Sudden Death in the Young Case Registry: Collaborating to Understand and Reduce Mortality.

Knowledge gaps persist about the incidence of and risk factors for sudden death in the young (SDY). The SDY Case Registry is a collaborative effort between the National Institutes of Health, the Centers for Disease Control and Prevention, and the Michigan Public Health Institute. Its goals are to: (1) describe the incidence of SDY in the United States by using population-based surveillance; (2) compile data from SDY cases to create a resource of information and DNA samples for research; (3) encourage standardized approaches to investigation, autopsy, and categorization of SDY cases; (4) develop partnerships between local, state, and federal stakeholders toward a common goal of understanding and preventing SDY; and (5) support families who have lost loved ones to SDY by providing resources on bereavement and medical evaluation of surviving family members. Built on existing Child Death Review programs and as an expansion of the Sudden Unexpected Infant Death Case Registry, the SDY Case Registry achieves its goals by identifying SDY cases, providing guidance to medical examiners/coroners in conducting comprehensive autopsies, evaluating cases through child death review and an advanced review by clinical specialists, and classifying cases according to a standardized algorithm. The SDY Case Registry also includes a process to obtain informed consent from next-of-kin to save DNA for research, banking, and, in some cases, diagnostic genetic testing. The SDY Case Registry will provide valuable incidence data and will enhance understanding of the characteristics of SDY cases to inform the development of targeted prevention efforts.

Novel Use of an Ultrafiltration Device as an Alternative Method for Fluid Removal in Critically Ill Pediatric Patients with Cardiac Disease: A Case Series.

Fluid overload (FO) is a common complication for pediatric patients in the intensive care unit. When conventional therapy fails, hemodialysis or peritoneal dialysis is classically used for fluid removal. Unfortunately, these therapies are often associated with cardiovascular or respiratory instability. Ultrafiltration, using devices such as the Aquadex™ system (Baxter Healthcare, Deerfield, IL, USA), is an effective tool for fluid removal in adult patients with congestive heart failure. As compared to hemodialysis, ultrafiltration can be performed using smaller catheters, and the extracorporeal volume and minimal blood flow rates are lower. In addition, there is no associated abdominal distension as is seen in peritoneal dialysis. Consequently, ultrafiltration may be better tolerated in critically ill pediatric patients. We present three cases of challenging pediatric patients with FO in the setting of congenital heart disease in whom ultrafiltration using the Aquadex™ system was successfully utilized for fluid removal while cardiorespiratory stability was maintained.