In-person school attendance and adolescent exposure to injury-related risk behaviors during the COVID-19 pandemic in the United States.

School closures during the COVID-19 pandemic have been reported to influence adolescents' behavioral health and may have altered their exposure to injury risk. We aimed to determine how in-person school attendance of individual adolescents in the United States during the pandemic was correlated with a range of risky health behaviors. We used self-reported data from adolescents 14-18 years old enrolled in grades 9-12 who participated in the 2020 Adolescent Behaviors and Experiences Survey. The exposure of interest was in-person vs remote school attendance in the previous 30 days. Risk behavior outcomes included not wearing a seatbelt when riding in a car; riding with someone who was drinking and driving; suffering intimate partner violence (IPV); forced sexual encounters; suicidal ideation; suicidal planning; electronic bullying; gun carrying; and physical fighting. Based on a multivariable analysis of 5202 students (65% attending school in-person) adjusted for age, sex, race, ethnicity, sexual orientation, parental unemployment, food insecurity, and homelessness, we found that in-person school attendance was associated with increased odds of every risk behavior except suicidal ideation and electronic bullying, with adjusted odds ratios ranging from 1.40 (95% confidence interval [CI]: 1.04, 1.88) for not wearing a seatbelt to 3.43 for IPV (95% CI: 1.97, 5.97). Our analyses demonstrate that in-person school attendance during the COVID-19 pandemic was associated with higher rates of risk behavior among adolescents. Further research is needed explore if this relationship is causal, and how these risks could be mitigated, as most adolescents have now returned to in-person schooling.

Implementation and Evaluation of a Novel High-Value Care Curriculum in a Single Academic Surgery Department.

BACKGROUND: High value care (HVC), maximizing quality while minimizing cost, has become a major focus of surgical practice. Effective education in healthcare value concepts is critical during residency to ensure graduates are able to deliver high value surgical care and participate in interprofessional teams to improve the system. STUDY DESIGN: An HVC curriculum was implemented at a single academic medical center. Sixty-six residents from general surgery, plastic surgery, otolaryngology, and urology completed the curriculum over 3 academic years (2016 to 2019). The 1-year curriculum taught residents the concepts of HVC before participating in a value improvement project the following year. Residents' knowledge of value was assessed pre- and post-participation using a validated assessment tool, the Quality Improvement Knowledge Application Tool Revised (QIKAT-R), and a curriculum-specific assessment tool. The overall success of the program was evaluated by assessing residents' skills in completing value improvement projects using a novel scoring rubric. RESULTS: After completing the program, residents expressed improved confidence in their ability to complete a value improvement project. Residents also demonstrated improved knowledge on the curriculum-specific assessment (4.7/13 to 10.9/13) and the scenario assessment using the QIKAT-R tool (8.5/27 to 16.4/27). As the program underwent iterative improvements each year, the quality of the residents' projects also improved, as assessed by the novel scoring rubric. CONCLUSIONS: Multimodal assessment demonstrated improvement in residents' objective knowledge of HVC principles, residents' ability to design and lead clinical value improvement projects, and residents' confidence they could use HVC principles in their current and future practice.

Expanded Phenotypic Definition Identifies Hundreds of Potential Causative Genes for Leukodystrophies and Leukoencephalopathies.

BACKGROUND: The genes responsible for genetic white matter disorders (GWMD; leukodystrophies and leukoencephalopathies) are incompletely known. Our goal was to revise the list of genes considered to cause GWMD. We considered a GWMD to consist of any genetic disease causing T2 signal white matter changes in magnetic resonance images. METHODS AND RESULTS: Using a systematic review of PubMed, Google, published literature reviews, and commercial gene panels, we identified 399 unique genes meeting the GWMD definition. Of this, 87 (22%) genes were hypomyelinating. Only 3 genes had contrast enhancement on magnetic resonance imaging (MRI): ABCD1, GFAP, and UNC13D. CONCLUSIONS: A significantly greater number of genes than previously recognized, 399, are associated with white matter signal changes on T2 MRI. This expansion of GWMD genes can be useful in analysis and interpretation of next-generation sequencing results for GWMD diagnosis, and for understanding shared pathophysiological mechanisms of GWMDs.

Association of Diagnosis of Leukodystrophy With Race and Ethnicity Among Pediatric and Adolescent Patients.

Importance: Inherited leukodystrophies are a group of neurological diseases affecting myelin that cause significant morbidities and death. Timely and correct diagnosis is important for initiating treatment, designing disease screening, and offering care and guidance to patients and families. Objective: To determine whether there are disparities in leukodystrophy diagnosis in different racial backgrounds. Design, Setting, and Participants: This case-control study involved a retrospective review of patients aged 18 years or younger who were diagnosed with 1 of 4 leukodystrophies (metachromatic leukodystrophy, X-linked adrenoleukodystrophy, Krabbe disease, and Hurler disease) in the US Children's Hospital Association's Pediatric Health Information System database from October 1, 2015, through September 30, 2017. Main Outcomes and Measures: Leukodystrophy diagnosis and racial background of the patients were analyzed. Adjusted prevalence estimates of leukodystrophies were obtained by controlling for sex, insurance type, urban or rural status, 2010 median household income for patient zip code, number of inpatient days, and age at first visit. Pathogenic leukodystrophy gene allele frequencies in different racial backgrounds for ABCD1, ARSA, GALC, and IDUA were determined using the gnomAD database. Results: Of the 557 patients identified with a leukodystrophy (221 [40%] female; 321 [58%] white non-Hispanic, 54 [10%] black non-Hispanic, and 51 [9%] white Hispanic; median [range] age, 7 [0-18] years), nonwhite race, including black non-Hispanic, black Hispanic, and white Hispanic, was associated with not having a leukodystrophy diagnosis. The adjusted prevalence for a leukodystrophy diagnosis in white non-Hispanic patients was 13.8 (95% CI, 10.6-17.9) per 100 000 patients, compared with 5.8 (95% CI, 3.8-8.9), 2.4 (95% CI, 1.1-5.2), and 7.4 (95% CI, 5.2-10.4) per 100 000 in black non-Hispanic, black Hispanic, and white Hispanic patients, respectively. This reduced rate of diagnosis was out of proportion to the frequency of the different races in the Pediatric Health Information System database. Similar or higher frequencies of missense or loss-of-function alleles were measured in populations of Latino and African descent for the pathogenic leukodystrophy gene alleles. For example, for ABCD1, allele frequencies in those of Latino or African descent were 2.1 × 10-5 and 2.2 × 10-5, as compared with 1.4 × 10-5 for those of European non-Finnish descent. Conclusions and Relevance: Patients of racial/ethnic minorities, including those from black, black Hispanic, and white Hispanic backgrounds, were significantly less likely to be diagnosed with a leukodystrophy. Leukodystrophy disease-associated allele frequencies were the same or higher in populations of Latino or African descent, arguing against a genetic founder effect being responsible for the lower diagnosis rates. This underdiagnosis has implications for newborn screening programs and treatment access and may reflect a more widespread problem in pediatric neurological and orphan diseases.