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BackgroundAnaphylaxis is a potentially fatal allergic reaction. However, many patients at risk of anaphylaxis who should permanently carry a life-saving epinephrine auto injector (EAI) do not carry one at the moment of allergen exposure. The proximity-based emergency response communities (ERC) strategy suggests speeding EAI delivery by alerting patient-peers carrying EAI to respond and give their EAI to a nearby patient in need. ObjectivesThis study had two objectives: (1) to analyze 10,000 anaphylactic events from the European Anaphylaxis Registry (EAR) by elicitor and location in order to determine typical anaphylactic scenarios and (2) to identify patients’ behavioral and spatial factors influencing their response to ERC emergency requests through a scenario-based survey. MethodsData were collected and analyzed in two phases: (1) clustering 10,000 EAR records by elicitor and incident location and (2) conducting a two-center scenario-based survey of adults and parents of minors with severe allergy who were prescribed EAI, in Israel and Germany. Each group received a four-part survey that examined the effect of two behavioral constructs—shared identity and diffusion of responsibility—and two spatial factors—emergency time and emergency location—in addition to sociodemographic data. We performed descriptive, linear correlation, analysis of variance, and t tests to identify patients’ decision factors in responding to ERC alerts. ResultsA total of 53.1% of EAR cases were triggered by food at patients’ home, and 46.9% of them were triggered by venom at parks. Further, 126 Israeli and 121 German participants completed the survey and met the inclusion criteria. Of the Israeli participants, 80% were parents of minor patients with a risk of anaphylaxis due to food allergy; their mean age was 32 years, and 67% were women. In addition, 20% were adult patients with a mean age of 21 years, and 48% were female. Among the German patients, 121 were adults, with an average age of 47 years, and 63% were women. In addition, 21% were allergic to food, 75% were allergic to venom, and 2% had drug allergies. The overall willingness to respond to ERC events was high. Shared identity and the willingness to respond were positively correlated (r=0.51, P

BackgroundMedical emergencies such as anaphylaxis may require immediate use of emergency medication. Because of the low adherence of chronic patients (ie, carrying anti-anaphylactic medication) and the potentially long response time of emergency medical services (EMSs), alternative approaches to provide immediate first aid are required. A smartphone-based emergency response community (ERC) was established for patients with allergies to enable members to share their automatic adrenaline injector (AAI) with other patients who do not have their AAI at the onset of anaphylactic symptoms. The community is operated by a national EMS. In the first stage of the trial, children with food allergies and their parents were invited to join. ObjectiveThis study aimed to identify the factors that influence the willingness to join an ERC for a group of patients at risk of anaphylaxis. MethodsThe willingness to join an ERC was studied from different perspectives: the willingness of children with severe allergies to join an ERC, the willingness of their parents to join an ERC, the willingness of parents to enroll their children in an ERC, and the opinions of parents and children about the minimum age to join an ERC. Several types of independent variables were used: demographics, medical data, adherence, parenting style, and children's autonomy. A convenience sample of children and their parents who attended an annual meeting of a nonprofit organization for patients with food allergies was used. ResultsA total of 96 questionnaires, 73 by parents and 23 by children, were collected. Response rates were approximately 95%. Adherence was high: 22 out of 23 children (96%) and 22 out of 52 parents (42%) had their AAI when asked. Willingness to join the community was high among parents (95%) and among children (78%). Willingness of parents to enroll their children was 49% (36/73). The minimum age to join an ERC was 12.27 years (SD 3.02) in the parents’ opinion and 13.15 years (SD 3.44) in the children’s opinion. ConclusionsParents’ willingness to join an ERC was negatively correlated with parents’ age, child’s age, and parents’ adherence. This can be explained by the free-rider effect: parents who carried an AAI for their young child, but had low adherence, wanted to join the ERC to get an additional layer of emergency response. Children’s willingness to join the community was positively correlated with age and negatively correlated with the child’s emotional autonomy. Parents’ willingness to enroll their children in an ERC was positively correlated with child’s age and negatively correlated with parents’ adherence: again, this can be explained by the aforementioned free-rider effect. Parents’ and children’s opinions about the minimum age to join an ERC were negatively correlated with protective parenting style and positively correlated with monitoring parenting style.

Despite progress in elucidation of disease mechanisms, identification of risk factors, biomarker discovery, and the approval of two medications to slow lung function decline in idiopathic pulmonary fibrosis and one medication to slow lung function decline in progressive pulmonary fibrosis, pulmonary fibrosis remains a disease with a high morbidity and mortality. In recognition of the need to catalyze ongoing advances and collaboration in the field of pulmonary fibrosis, the NHLBI, the Three Lakes Foundation, and the Pulmonary Fibrosis Foundation hosted the Pulmonary Fibrosis Stakeholder Summit on November 8-9, 2022. This workshop was held virtually and was organized into three topic areas: 1) novel models and research tools to better study pulmonary fibrosis and uncover new therapies, 2) early disease risk factors and methods to improve diagnosis, and 3) innovative approaches toward clinical trial design for pulmonary fibrosis. In this workshop report, we summarize the content of the presentations and discussions, enumerating research opportunities for advancing our understanding of the pathogenesis, treatment, and outcomes of pulmonary fibrosis.

Genome-wide association studies (GWAS) have become well-powered to detect loci associated with telomere length. However, no prior work has validated genes nominated by GWAS to examine their role in telomere length regulation. We conducted a multi-ancestry meta-analysis of 211,369 individuals and identified five novel association signals. Enrichment analyses of chromatin state and cell-type heritability suggested that blood/immune cells are the most relevant cell type to examine telomere length association signals. We validated specific GWAS associations by overexpressing KBTBD6 or POP5 and demonstrated that both lengthened telomeres. CRISPR/Cas9 deletion of the predicted causal regions in K562 blood cells reduced expression of these genes, demonstrating that these loci are related to transcriptional regulation of KBTBD6 and POP5. Our results demonstrate the utility of telomere length GWAS in the identification of telomere length regulation mechanisms and validate KBTBD6 and POP5 as genes affecting telomere length regulation.