Angioedema as a Rare Presentation of Systemic Lupus Erythematosus.

Angioedema (AE) is an immune-mediated tissue swelling that can be life-threatening if it compromises the airway. This makes prompt diagnosis and management of the condition excruciatingly important. It can be hereditary or associated with infections, malignancies, and autoimmune diseases. There have been reported cases in the literature where Systemic lupus erythematosus (SLE) patients developed acquired angioedema raising suspicion of a possible association between the two conditions. We describe a case of a patient with no known medical issues, presenting with acute onset of her first episode of angioedema with airway compromise. Because of the rarity of awareness of the possible association of our conditions of interest, there was an inevitable delay in diagnosis and the patient was eventually diagnosed to have SLE and associated acquired angioedema as its first presentation.  This case report highlights the importance of maintaining high suspicion for SLE in patients with an isolated first episode of AE and discusses mechanisms involved in the disease process to shed light on available treatment modalities.

Homicide Incidents Involving Children with Autism Spectrum Disorder as Victims Reported in the US News Media, 2000-2019.

Recent research indicates that children with autism are at increased risk of maltreatment. We examined news media reports on homicide incidents involving children with autism as victims in the United States between 2000 and 2019. Of the 52 victims studied, 47 (90.4%) were male. Age of victims ranged from 2 to 20 years (mean = 10.4 ± 5.3 years). Parents and other caregivers accounted for 63.5% and 13.5% of the perpetrators, respectively. The leading injury mechanism was gunshot wounds (23.1%), followed by drowning (19.2%), and suffocation, strangulation, or asphyxiation (19.2%). The most commonly cited contributing factor (47.1%) was overwhelming stress from caring for the autistic child. These results underscore the importance of supporting services for caregivers of children with autism.

The Effects of Dual Task Cognitive Interference and Fast-Paced Walking on Gait, Turns, and Falls in Men and Women with FXTAS.

Fragile X-associated tremor/ataxia syndrome (FXTAS) is a genetic neurodegenerative disorder characterized by cerebellar ataxia, tremor, and cognitive dysfunction. We examined the impact of dual-task (DT) cognitive-motor interference and fast-paced (FP) gait on gait and turning in FXTAS. Thirty participants with FXTAS and 35 age-matched controls underwent gait analysis using an inertial sensor-based 2-min walk test under three conditions: (1) self-selected pace (ST), (2) FP, and (3) DT with a concurrent verbal fluency task. Linear regression analyses were performed to assess the association between FXTAS diagnosis and gait and turn outcomes. Correlations between gait variables and fall frequency were also calculated. FXTAS participants had reduced stride length and velocity, swing time, and peak turn velocity and greater double limb support time and number of steps to turn compared to controls under all three conditions. There was greater dual task cost of the verbal fluency task on peak turn velocity in men with FXTAS compared to controls. Additionally, stride length variability was increased and cadence was reduced in FXTAS participants in the FP condition. Stride velocity variability under FP gait was significantly associated with the number of self-reported falls in the last year. Greater motor control requirements for turning likely made men with FXTAS more susceptible to the negative effects of DT cognitive interference. FP gait exacerbated gait deficits in the domains of rhythm and variability, and increased gait variability with FP was associated with increased falls. These data may inform the design of rehabilitation strategies in FXTAS.

Research utility of the National Violent Death Reporting System: a scoping review.

BACKGROUND: To better understand and prevent suicide and homicide, the National Center for Injury Prevention and Control of the US Centers for Disease Control and Prevention launched the National Violent Death Reporting System (NVDRS) in six states in 2002. As of 2018, the NVDRS has been expanded to include all 50 states, the District of Columbia and Puerto Rico. The purpose of this review was to assess the research utility of the NVDRS based on studies indexed in major bibliographical databases. METHODS: We performed a scoping review of published studies that were based on data from the NVDRS, identified by searching six electronic databases: PubMed, EMBASE, Google Scholar, OVID, Scopus, and Web of Science. We examined the time trend of annual NVDRS-based research output, generated a word cloud using the keywords listed in the publications, and mapped the knowledge domains covered by NVDRS-based studies. RESULTS: Our review included a total of 150 studies published between 2005 and 2018. There was a marked increase in the annual number of NVDRS-based publications, with 120 (80.0%) of the 150 studies published between 2011 and 2018. Overall, 104 (69.3%) studies focused on suicide and 39 (26.0%) on homicide. Of the included studies, 100 (66.7%) were descriptive epidemiology, 31 (20.7%) were risk factor analyses, 9 (6.0%) were evaluations, 7 (4.7%) were trend analyses, and 4 (2.7%) were data quality assessments. Knowledge domain mapping identified two major clusters of studies, one on suicide and the other on homicide. The cluster on suicide was commonly linked to "circumstance," "alcohol" and "substance abuse" and the cluster on homicide was commonly linked to "firearm," "injury," and "gang." The two clusters were interlinked to overlapping networks of keywords, such as "firearm" and "mental health problem." CONCLUSIONS: Research utility of the NVDRS has increased considerably in recent years. Studies based on data from the NVDRS are clustered in two knowledge domains - suicide and homicide. The vast potential of the NVDRS for violence research and prevention remains to be fully exploited.

Characteristics of unintentional drowning deaths in children with autism spectrum disorder.

BACKGROUND: The reported prevalence of autism spectrum disorder (ASD) has increased markedly in the past two decades. Recent research indicates that children with ASD are at a substantially increased risk of injury mortality, particularly from unintentional drowning. The purpose of this study was to explore the circumstances of fatal unintentional drowning incidents involving children with ASD under 15 years of age. FINDINGS: During January 2000 through May 2017, US newspapers reported a total of 23 fatal drowning incidents involving 18 boys and 5 girls with ASD. Age of victims ranged from 3 to 14 years (mean = 7.7 ± 2.9 years). These drowning incidents most commonly occurred in ponds (52.2%), followed by rivers (13.0%), and lakes (13.0%). For 11 incidents with location data available, the distance between victim residence and the water body where drowning occurred averaged 290.7 m (± 231.5 m). About three-quarters (73.3%) of the drowning incidents occurred in the afternoon hours from 12:00 to 18:59. Wandering was the most commonly reported activity that led to drowning, accounting for 73.9% of the incidents. CONCLUSIONS: Fatal drowning in children with ASD typically occur in water bodies near the victims' homes in the afternoon hours precipitated by wandering. Multifaceted intervention programs are urgently needed to reduce the excess risk of drowning in children with ASD.

Injury Mortality in Individuals With Autism.

OBJECTIVES: To examine epidemiological patterns of injury fatalities in individuals with a diagnosis of autism. METHODS: We identified individuals with a diagnosis of autism who died between 1999 and 2014 by screening causes of death in the multiple cause-of-death data files in the National Vital Statistics System based on the International Classification of Diseases, 10th Revision, code F84.0. We used the general US population as the reference to calculate proportionate mortality ratios (PMRs) and 95% confidence intervals (CIs). RESULTS: During the study period, 1367 deaths (1043 males and 324 females) in individuals with autism were recorded in the United States. The mean age at death for individuals with autism was 36.2 years (SD = 20.9 years), compared with 72.0 years (SD = 19.2 years) for the general population. Of the deaths in individuals with autism, 381 (27.9%) were attributed to injury (PMR = 2.93; 95% CI = 2.64, 3.24), with suffocation (n = 90; PMR = 31.93; 95% CI = 25.69, 39.24) being the leading cause of injury mortality, followed by asphyxiation (n = 78; PMR = 13.50; 95% CI = 10.68, 16.85) and drowning (n = 74; PMR = 39.89; 95% CI = 31.34, 50.06). CONCLUSIONS: Individuals with autism appear to be at substantially heightened risk for death from injury.