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Objective: Our objective was to evaluate whether there is an enrichment of rare variants in familial hemophagocytic lymphohistiocytosis (HLH)‐associated genes among patients with systemic juvenile idiopathic arthritis (sJIA) with or without macrophage activation syndrome (MAS). Methods: Targeted sequencing of HLH genes (LYST, PRF1, RAB27A, STX11, STXBP2, UNC13D) was performed in patients with sJIA from an established cohort. Sequence data from control participants were obtained in silico (database of Genotypes and Phenotypes: phs000280.v8.p2). Rare variant association testing (RVT) was performed with sequence kernel association test package. Significance was defined as P < 0.05 after 100,000 permutations. Results: Sequencing data from 524 sJIA cases were jointly called and harmonized with exome‐derived target data from 3,000 controls. Quality control operations produced a set of 480 cases and 2,924 ancestrally matched control participants. RVT of cases and controls revealed a significant association with rare protein‐altering variants (minor allele frequency [MAF] < 0.01) of STXBP2 (P = 0.020) and ultrarare variants (MAF < 0.001) of STXBP2 (P = 0.006) and UNC13D (P = 0.046). A subanalysis of 32 cases with known MAS and 90 without revealed a significant difference in the distribution of rare UNC13D variants (P = 0.0047) between the groups. Additionally, patients with sJIA more often carried two or more HLH variants than did controls (P = 0.007), driven largely by digenic combinations involving LYST. Conclusion: We identified an enrichment of rare HLH variants in patients with sJIA compared with controls, driven by STXBP2 and UNC13D. Biallelic variation in HLH genes was associated with sJIA, driven by LYST. Only UNC13D displayed enrichment in patients with MAS. This suggests that HLH variants may contribute to the pathophysiology of sJIA, even without MAS. [ABSTRACT FROM AUTHOR]

We present the first evidence on the role of occupational choices and acquired skills for migrant selection. Combining novel data from a representative Mexican task survey with rich individual-level worker data, we find that Mexican migrants to the United States have higher manual skills and lower cognitive skills than nonmigrants. Results hold within narrowly defined region–industry–occupation cells and for all education levels. Consistent with a Roy/Borjas-type selection model, differential returns to occupational skills between the United States and Mexico explain the selection pattern. Occupational skills are more important to capture the economic motives for migration than previously used worker characteristics. [ABSTRACT FROM AUTHOR]

The Representative Concentration Pathway 2.6 (RCP2.6), which is broadly compatible with the Paris Agreement's temperature goal by 1.5–2°C, contains substantial reductions in agricultural non-CO2 emissions besides the deployment of Carbon Dioxide Removal (CDR). Failing to mitigate agricultural methane and nitrous oxide emissions could contribute to an overshoot of the RCP2.6 warming by about 0.4°C. We explore using additional CDR to offset alternative agricultural non-CO2 emission pathways in which emissions either remain constant or rise. We assess the effects on the climate of calculating CDR rates to offset agricultural emission under two different approaches: relying on the 100-year global warming potential conversion metric (GWP100) and maintaining effective radiative forcing levels at exactly those of RCP2.6. Using a reduced-complexity climate model, we find that the conversion metric leads to a systematic underestimation of needed CDR, reaching only around 50% of the temperature mitigation needed to remain on the RCP2.6 track. This is mostly because the metric underestimates, in the near term, forcing from short-lived climate pollutants such as methane. We test whether alternative conversion metrics, the GWP20 and GWP*, are more suitable for offsetting purposes, and found that they both lead to an overestimation of the CDR requirements. Under alternative agricultural emissions pathways, holding to RCP2.6 total radiative forcing requires up to twice the amount of CDR that is already included in the RCP2.6. We examine the costs of this additional CDR, and the effects of internalizing these in several agricultural commodities. Assuming an average CDR cost by $150/tCO2, we find increases in prices of up to 41% for beef, 14% for rice, and 40% for milk in the United States relative to current retail prices. These figures are significantly higher (for beef and rice) under a global scenario, potentially threatening food security and welfare. Although the policy delivers a mechanism to finance the early deployment of CDR, using CDR to offset remaining high emissions may well hit other non-financial constraints and can thus only support, and not substitute, emission reductions. [ABSTRACT FROM AUTHOR]

PURPOSE: While the immediate care and access disruptions associated with the COVID-19 pandemic have received growing attention in certain areas, the full range of gaps in cancer screenings and treatment is not yet well understood or well documented throughout the country comprehensively. METHODS: This study used a large medical claims clearinghouse database representing 5%-7% of the Medicare fee-for-service population to characterize changes in the utilization of cancer care services and gain insight into the impact of COVID-19 on the US cancer population, including identification of new patients, gaps in access to care, and disruption of treatment journeys. RESULTS: In March-July 2020, in comparison with the baseline period of March-July 2019, there is a substantial decrease in cancer screenings, visits, therapy, and surgeries, with variation by cancer type and site of service. At the peak of the pandemic in April, screenings for breast, colon, prostate, and lung cancers were lower by 85%, 75%, 74%, and 56%, respectively. Significant utilization reductions were observed in April for hospital outpatient evaluation and management (E&M) visits (−74%), new patient E&M visits (−70%), and established patient E&M visits (−60%). A decrease in billing frequency was observed for the top physician-administered oncology products, dropping in both April (−26%) and July (−31%). Mastectomies were reduced consistently in April through July, with colectomies similarly reduced in April and May and prostatectomies dipping in April and July. CONCLUSION: The current impact of the COVID-19 pandemic on cancer care in the United States has resulted in decreases and delays in identifying new cancers and delivery of treatment. These problems, if unmitigated, will increase cancer morbidity and mortality for years to come. [ABSTRACT FROM AUTHOR]

Background: Evaluation of surgical skill competency is necessary as graduate medical education moves toward a competency-based curriculum. This study by the American Board of Orthopaedic Surgery (ABOS) and the Council of Orthopaedic Residency Directors (CORD) compares 2 web-based evaluation tools that assess the level of autonomy that is demonstrated by residents during surgical procedures in the operating room as measured by faculty.Methods: Two hundred and ninety-four residents from 16 orthopaedic surgery residency programs were evaluated by 370 faculty using 2 web-based evaluation tools in a crossover design in which residents requested faculty review of their surgical skills before starting a case. One thousand, one hundred and fifty Ottawa Surgical Competency Operating Room Evaluation (O-Score) assessments, which included a 9-question evaluation of 8 steps of the surgical procedure, were compared with 1,186 P-score evaluations, which included a single-question summative evaluation. Twenty-five different surgical procedures were evaluated.Results: There were no significant differences in rates of resident requests or faculty completion of the 2 scores. The most common surgical procedures that were assessed were total knee arthroplasty (n = 254, 11%), carpal tunnel release (n = 191, 8%), open reduction and internal fixation (ORIF) of stable hip fractures (n = 170, 7%), ORIF of simple ankle fractures (n = 169, 7%), and total hip arthroplasty (n = 166, 7%). Both instruments disclosed significant differences in competency among entry, intermediate, and advanced-level residents. The findings support the construct validity of the evaluation method. The survey results indicated that >70% of the faculty were confident that use of either the P-score or the O-score allowed them to distinguish a resident who can perform the surgery independently from one who needs additional training.Conclusions: This research has led to the modification of the O-score and the P-score into a combined OP-score instrument. The ABOS envisions that the OP-score instrument can be used with an expanded number of surgical procedures as a required element of residency training in the near future.Clinical Relevance: This study allows the profession of orthopaedic surgery education to take a leadership role in the measurement of competence for surgical skills for orthopaedic surgeons in residency training, an important clinically relevant topic to the practice of orthopaedic surgery. [ABSTRACT FROM AUTHOR]

Background: Excessive radiation to health-care providers has been linked to risks of cancer and cataracts, but its negative effects can be substantially reduced by lead aprons, thyroid shields, and leaded glasses. Hospitals are required to provide education and proper personal protective equipment, yet discrepancies exist between recommendations and compliance. This article presents the results of a survey of U.S. orthopaedic surgery residents concerning attitudes toward radiation exposure and personal protective equipment behavior.Methods: An invitation to participate in a web-based, anonymous survey was distributed to 46 U.S. allopathic orthopaedic surgery residency programs (1,207 potential resident respondents). The survey was conceptually divided into the following areas: demographic characteristics, training and attitudes concerning occupational hazards, personal protective equipment provision and use, and general safety knowledge. Prevalence ratios (PRs) and 95% confidence intervals (CIs) were calculated for the association between these characteristics and compliance with thyroid shield or lead gown wear.Results: In this study, 518 surveys were received, with 1 survey excluded because of insufficient response, leaving 517 surveys for analysis (42.8% response rate). Ninety-eight percent of residents believed that personal protective equipment should be provided by the hospital or residency program. However, provision of personal protective equipment was not universal, with 33.8% reporting none and 54.2% reporting provision of a gown and thyroid shield. The prevalence of leaded glasses usage was 21%. Poor lead gown compliance and thyroid shield wear were associated with difficulty finding the corresponding equipment: PR, 2.51 (95% CI, 1.75 to 3.62; p < 0.001) for poor lead gown compliance and PR, 2.14 (95% CI, 1.46 to 3.16; p < 0.0001) for poor thyroid shield wear. Not being provided with personal protective equipment was also significantly associated with low compliance with both lead gowns (PR, 1.47 [95% CI, 1.04 to 2.08]; p = 0.03) and thyroid shields (PR, 1.69 [95% CI, 1.18 to 2.41]; p = 0.004). Respondents from the Southeast, West, or Midwest had lower compliance with lead gown usage. Forgetting was the number 1 reason to not wear a lead apron (42%).Conclusions: Radiation exposure is associated with increased risk of serious health problems. Our findings identified that the availability of lead personal protective equipment leads to increased compliance among residents surveyed. In addition to yearly occupational hazard training specific to orthopaedic surgery, greater efforts by residency programs and hospitals are needed to improve access to lead personal protective equipment and compliance for orthopaedic residents. [ABSTRACT FROM AUTHOR]

Hepatocellular Carcinoma ( HCC) incidence is increasing in the USA. Gemcitabine (G) and oxaliplatin (O) are active in HCC and biliary duct cancer ( BDC). Erlotinib (E) is an EGFR tyrosine kinase inhibitor ( TKI) with known activity against both. We sought to evaluate the efficacy of the combination G+O+E. Patients with either of the two diagnosis were treated in a phase II trial. Simons 2 stage design was used. A disease-control rate ( DCR), complete response ( CR) + partial response ( PR)+ stable disease ( SD) at 24 weeks of ≤20% and >40% (P0 and P1 of 0.2 and 0.4, respectively) were set as undesirable (null) and desirable results. 26 HCC and 7 BDC patients were accrued. In HCC, 1 PR, 10 SD, and 9 PDs were seen. DCR in HCC was 42%. Among seven (7) patients with BDC, one patient was not evaluable; one achieved a long lasting PR, and five patients had SD and DCR was 86%. Median overall survival ( OS) times and progression-free survivals ( PFS) were 196 and 149 days in HCC and 238 days and not reached in BDC. PFS at 26 weeks in HCC was 41% and at 21 weeks in BDC was 60%. Grade 3 toxicities in >5% of patients were fatigue (12.9%), neutropenia (9.6%), thrombocytopenia (9.6%), and diarrhea (6.4%). G+O+E exceeded both preset P0a and P1 of the primary objective with a PFS of 41% at 26 weeks for HCC and preliminary BDC data may warrant further investigations. [ABSTRACT FROM AUTHOR]

Background: Preoperative assessment of geriatric patients with a hip fracture may include transthoracic echocardiography (TTE), which increases resource utilization and cost and may delay surgery. The purpose of this study was to evaluate preoperative TTE utilization at a single institution in order to determine (1) how often TTE is ordered in accordance with clinical practice guidelines (CPGs), (2) how frequently TTE reveals cardiac disease that may alter medical or anesthesia management, and (3) whether following CPGs reduces unnecessary TTE utilization without potentially missing important disease.Methods: A retrospective review of data on 100 geriatric patients with a hip fracture who had undergone preoperative TTE was performed. Charts were reviewed to evaluate if TTE had been obtained in accordance with the published CPGs from the American College of Cardiology/American Heart Association (ACC/AHA). TTE reports were reviewed for the presence of disease that was important enough to cause modifications in anesthesia or perioperative management, including new left ventricular systolic or diastolic dysfunction, moderate or severe valvular disease, and pulmonary hypertension. Finally, the sensitivity and specificity of accordance with the ACC/AHA CPGs for predicting which patients would have TTE that identified important disease were calculated.Results: The TTE was ordered in accordance with the published ACC/AHA CPGs for 66% of the patients. TTE revealed disease with the potential to modify anesthesia or medical management in 14% of the patients-for all of whom the TTE had been indicated according to ACC/AHA guidelines (i.e., the guidelines were 100% sensitive). In this study population, following the ACC/AHA guidelines could have prevented the performance of TTE in 34% of the patients without missing any disease (40% specificity).Conclusions: Preoperative TTE for patients with a hip fracture is frequently obtained outside the recommendations of established CPGs. Utilization of CPGs such as the ACC/AHA guidelines should be considered, as it may decrease variability in care and reduce unnecessary resource utilization without adversely affecting patient outcomes. [ABSTRACT FROM AUTHOR]

After 9/11, new security duties were instituted at many U.S. air carriers and existing safety and security duties received increased emphasis. Concurrently, in-flight services were changed and in many cases, cabin crews were reduced. This article examines the post-9/11 conflict between passenger service and the timely performance of safety and security duties at 1 major U.S. air carrier. In-flight data were obtained on both international and domestic flights. The data suggest that the prompt performance of the safety and security duties is adversely affected by the number of service duties occurring in the later part of both international and domestic flights. [ABSTRACT FROM AUTHOR]

The Asian citrus psyllid, Diaphorina citri (Hemiptera: Psyllidae), is an invasive pest of citrus in the United States. The psyllid feeds and reproduces primarily on new flush growth of citrus and other rutaceous plants. Because it vectors the bacterial causal agents of the deadly citrus greening disease, D. citri is potentially a pest of economic importance in all citrus growing areas where it occurs together with the disease. We investigated the diurnal patterns of its flight activity in the field and the effects of light on its host selection and egg laying behaviors. The numbers of adult psyllids caught on yellow sticky traps were 3 to 4-fold higher during daytime than nighttime. Daytime flight activity of D. citri adults also varied with time of the day with peak catches occurring at midday from 1200 to 1500 h. Illumination of the traps at night increased their attractiveness to adult psyllids by 5-fold. Similarly, light significantly increased plant colonization by adults and female egg deposition on potted plants in the laboratory. These results showed that the flight activity and host selection behavior of adult psyllids are regulated by light and circadian rhythms. Thus, adult psyllids utilize light as visual cues in their host-plant selection process. [ABSTRACT FROM AUTHOR]

Empirical evidence suggests that anger plays a significant role in posttraumatic stress disorder (PTSD) and may impede recovery from traumatic events. The purpose of this study was to assess the role of anger and its relationship to distress and social/occupational functioning in disaster relief workers (DRWs) who had PTSD symptoms who were deployed to the World Trade Center after September 11, 2001. Six hundred twenty-six utility workers (96% male) completed measures of anger, distress, PTSD severity, and social/occupational functioning. Results indicated that anger was significantly higher in DRWs who had PTSD symptoms than in those who did not, and statistically significant associations were found among anger, distress, PTSD severity, and social/occupational functioning in workers who had PTSD symptoms. Careful assessment of anger in DRWs exposed to traumatic events is warranted as well as longitudinal studies to further understand the relationship between anger and PTSD. [ABSTRACT FROM AUTHOR]

Studies the role of aflatoxin B1 (AFB1) in the etiology of liver carcinoma in the United States. Risk factors from previous research; Search for AFB1 adducts and -53 alterations by immunoassays and codon 249 p53 mutation by restriction enzyme analysis; Recommended further investigations.

The article attempts to explain the change in U.S. states' Supreme Court jurisprudence on public school finance schemes. Public school systems are supported by a mix of federal, state and local sources. Federal funds account for a small fraction of total education expenditure and are focused on specific programs such as school lunches and special education. Most of the money for education comes from local and state funds. With one exception, all states fund their public schools systems with some amount of local property taxes assessed upon the value of property within each local school district. This article examines decisions by the supreme courts of Arizona and Ohio, the two courts which have switched positions on school finance, and argues that the reversals may be the product of political developments in those states and evolving attitudes toward education, not a change in the focus of litigation. The article concludes by observing that if success in the courts is really a function of attitudes toward education then reformers should cultivate public opinion as they seek to influence judicial opinion.

The article discusses the authors's insights regarding the problems faced by the textile industry in the U.S. He asserts that such problems, including spots, stains and poor colorfastness, are caused by the changes in markets and technology. He looks into the steps to problem solving, which include data gathering, formulation of hypothesis, experimentation and interpretation of results. Moreover, he suggests that one should be aware of the unexpected events that might happen. He also emphasizes the importance of intuition in problem solving.

The article offers the author's insights on the importance of informatics to improve the cancer care and treatment. Topics mentioned include the decline in cancer mortality due to the development in medical care, the need to improve the medical care quality, and the support to improve cancer research. Also mentioned are the development of precision oncology and clinical informatics journal and the medical care management in the U.S.

In this article, the authors discuss the use of medical imaging drug in clinical trials as a measure of efficacy for managing individual patients in the U.S. They indicate that the diagnostic-derived blinded read process used across therapeutic sites is derived from the U.S. Food & Drug Administration's (FDA) guidance documents. They suggest the need for time and expense investments to improve the quality of onsite and offsite image reads before efficacy of a drug can be based on the site read.

The article presents comments on old issues related to the textile industry in the U.S. It states the foul taste of chlorinated drinking water at Danville, Virginia in 1940s due to the use of soap in scouring wool. One reader shared an installed pressurized room that was big enough to accommodate a couple of 8 to 10-foot becks and a laboratory pot-unit for polyester dyeing. Another reader recalled that the National Cotton Council of America had undertaken a cotton care research and sessions after Du Pont Co. Ltd.'s introduction of Dacron polyester as easy care apparel in the early 1950s.