Explore the vast range of titles available.

The DNA-repair enzyme Artemis is essential for rearrangement of T- and B-cell receptors. Mutations in , which encodes Artemis, cause Artemis-deficient severe combined immunodeficiency (ART-SCID), which is poorly responsive to allogeneic hematopoietic-cell transplantation. We carried out a phase 1-2 clinical study of the transfusion of autologous CD34+ cells, transfected with a lentiviral vector containing , in 10 infants with newly diagnosed ART-SCID. We followed them for a median of 31.2 months. Marrow harvest, busulfan conditioning, and lentiviral-transduced CD34+ cell infusion produced the expected grade 3 or 4 adverse events. All the procedures met prespecified criteria for feasibility at 42 days after infusion. Gene-marked T cells were detected at 6 to 16 weeks after infusion in all the patients. Five of 6 patients who were followed for at least 24 months had T-cell immune reconstitution at a median of 12 months. The diversity of T-cell receptor β chains normalized by 6 to 12 months. Four patients who were followed for at least 24 months had sufficient B-cell numbers, IgM concentration, or IgM isohemagglutinin titers to permit discontinuation of IgG infusions. Three of these 4 patients had normal immunization responses, and the fourth has started immunizations. Vector insertion sites showed no evidence of clonal expansion. One patient who presented with cytomegalovirus infection received a second infusion of gene-corrected cells to achieve T-cell immunity sufficient for viral clearance. Autoimmune hemolytic anemia developed in 4 patients 4 to 11 months after infusion; this condition resolved after reconstitution of T-cell immunity. All 10 patients were healthy at the time of this report. Infusion of lentiviral gene-corrected autologous CD34+ cells, preceded by pharmacologically targeted low-exposure busulfan, in infants with newly diagnosed ART-SCID resulted in genetically corrected and functional T and B cells. (Funded by the California Institute for Regenerative Medicine and the National Institute of Allergy and Infectious Diseases; ClinicalTrials.gov number, NCT03538899.).

How information is communicated influences the public's environmental perceptions and behaviors. Information channels and sources both play an important role in the dissemination of information. Trust in a source is often used as a proxy for whether a particular piece of information is credible. To determine preferences for information channels and trust in various sources for information on nature-related topics, a mail-out survey was sent to randomly selected U.S. addresses (n = 1,030). Diverse groups of people may have differing communication preferences. Therefore, we explored differences in channel preferences and trust by demographics using regression models. Overall, the most preferred channels were personal experience, reading online content, and watching visual media online. The most trusted sources were science organizations, universities, and friends/family. Channel preferences varied the most by education level and age, while source trust was most influenced by education, race, age, and size of current residence (rural-urban). The influence of demographics varied depending on the individual channel and source, with some groups preferring certain channels or sources but not others. Results are useful to consider when disseminating information on nature-related topics to a general public audience. More broadly, results also suggest spreading information using different channels and sources depending on the specific audience being targeted.

Understanding public perceptions can enhance the success of landscape-level conservation by helping recognize how to gather public support. However, perceptions of wetlands’ importance may differ based on proximity to a wetland. This study used a mail-out survey across the entire U.S. ( n  = 1030) to investigate the public’s knowledge of local wetlands, visitation to wetlands, concern over losing ecosystem services provided by wetlands, and involvement in wetlands conservation. Regression models were run to explore the impact of proximity to the nearest wetland on wetlands visitation, concern, and conservation involvement. Additionally, sociodemographics and outdoor recreation participation were tested as predictors. While proximity to wetlands did impact knowledge of wetlands in the local area and wetlands visitation, it was not directly a significant predictor of concern for wetlands ecosystem services or conservation involvement. However, wetlands visitation did increase concern for ecosystem services and conservation involvement. Furthermore, participation in birdwatching, wildlife viewing, and fishing were correlated with higher concern for ecosystems services provided by wetlands and involvement in wetlands/waterfowl conservation. Results suggest that fostering awareness of wetlands, encouraging visitation, and promoting non-consumptive outdoor recreation opportunities may increase support for wetlands regardless of individuals’ proximity to wetlands.

Of the more than two million persons incarcerated in U.S. prisons, the majority are also parents to children under the age of 18. A growing body of research has explored the impact of parental incarceration on these children and has consistently found a link between this experience and negative life outcomes. Fewer studies, however, examined the longitudinal impact of parental incarceration on offspring. This analysis attempts to address this shortcoming by exploring the relationship between parental incarceration during childhood and adult outcomes later in life. More specifically, we examine the associations between paternal incarceration during childhood and health, educational, and economic outcomes in young adulthood. Using data from the Add Health, we utilize a series of regression analyses to examine these relationships. Results suggest that parental incarceration is significantly related to a number of outcomes in early adulthood, including educational attainment, physical and mental health, and receipt of public assistance.

Abstract Rationale “Noninfectious” pulmonary complications are significant causes of morbidity and mortality after allogeneic hematopoietic cell transplant. Early-onset viral reactivations or infections are common after transplant. Whether the first-onset viral infection causes noninfectious pulmonary complications is unknown. Objectives To determine whether the first-onset viral infection within 100 days after transplant predisposes to development of noninfectious pulmonary complications. Methods We performed a retrospective review of 738 allogeneic hematopoietic cell transplant patients enrolled from 2005 to 2011. We also established a novel bone marrow transplantation mouse model to test whether herpesviral reactivation after transplant causes organ injury. Measurements and Main Results First-onset viral infections with human herpesvirus 6 or Epstein-Barr virus within 100 days after transplant increase the risk of developing idiopathic pneumonia syndrome (adjusted hazard ratio [aHR], 5.52; 95% confidence interval [CI], 1.61–18.96; P = 0.007; and aHR, 9.21; 95% CI, 2.63–32.18; P = 0.001, respectively). First infection with human cytomegalovirus increases risk of bronchiolitis obliterans syndrome (aHR, 2.88; 95% CI, 1.50–5.55; P = 0.002) and grade II–IV acute graft-versus-host disease (aHR, 1.59; 95% CI, 1.06–2.39; P = 0.02). Murine roseolovirus, a homolog of human herpesvirus 6, can also be reactivated in the lung and other organs after bone marrow transplantation. Reactivation of murine roseolovirus induced an idiopathic pneumonia syndrome–like phenotype and aggravated acute graft-versus-host disease. Conclusions First-onset herpesviral infection within 100 days after allogeneic hematopoietic cell transplant increases risk of pulmonary complications. Experimentally reactivating murine roseolovirus causes organ injury similar to phenotypes seen in human transplant recipients.

Self-control and psychopathy are general theories of antisociality that have considerable empirical support, are conceptually similar, and have occasionally been studied together. A recent head-to-head test of the theories and found that self-control generally outperformed psychopathy among assorted criminal outcomes among institutionalized delinquents. Using data from university students (N = 1611) and different measures of self-control (Grasmick et al. scale) and psychopathy (Levenson Self-Report Psychopathy Scale), the current study revisited this work and found that self-control had robust associations with sexual aggression, general aggression, and substance abuse problems, and extreme scores on these outcomes variables. However, the effects of self-control were negated once psychopathy was specified, suggesting that psychopathy is more important for understanding assorted forms of deviance than self-control in the undergraduate population. Given the empirical heft of both theories, we encourage further study to determine which has greater predictive validity for understanding various forms of crime among different populations spanning student, community, forensic, and correctional samples. We also encourage the specification of both self-control and psychopathy as standard control variables.

American pet owners spend billions of dollars on food and treats so it is important to understand what products they want and what they think their dog would enjoy. This study analyzed video recordings of dogs engaging in dental chews in their home environment and compared the observed appetitive behaviors to owner preference and owner-reported dog preference. Overall, appetitive behavior differed significantly between some dental chews. Owner preference for the chews correlated significantly with dog appetitive behavior, but the effect was small (r (702) = 0.22, p = 0.001), whereas owner-reported dog preference correlated significantly with dog appetitive behavior and showed a moderate effect size (r (702) = 0.43, p = 0.001)—similar in magnitude to findings when parents are asked to report on their children’s behavior. By merging objective behavioral observation of owner-recorded videos with their survey responses, we were able to preliminarily parse out what factors owners may use to assess preference and encourage the future use of in-home video recordings to better understand dog and owner engagement and interaction with pet products.

Little is known about the early history of the chicken (Gallus gallus domesticus), including the timing and circumstances of its introduction into new cultural environments. To evaluate its spatio-temporal spread across Eurasia and north-west Africa, the authors radiocarbon dated 23 chicken bones from presumed early contexts. Three-quarters returned dates later than those suggested by stratigraphy, indicating the importance of direct dating. The results indicate that chickens did not arrive in Europe until the first millennium BC. Moreover, a consistent time-lag between the introduction of chickens and their consumption by humans suggests that these animals were initially regarded as exotica and only several centuries later recognised as a source of ‘food’.

Hydrologic processes that control river flow in Bhutan's Chamkhar Chhu basin are important for understanding water supply vulnerability to downstream populations in a changing climate. Seasonal source waters and flow paths of streamflow of the basin were determined using isotopic and geochemical tracers for water year 2016. Samples including surface water, groundwater, glacier meltwater, and precipitation were collected in premonsoon, monsoon, and postmonsoon seasons along an elevation transect from 2,538 to 5,158 m. Solute trends in surface waters demonstrate the major influence that tributaries can have on main stem hydrochemistry and the increasingly important role of groundwater below 3,500 m. Groundwater's role in flow is supported by a two-component hydrograph separation using SO 4 2− as a tracer and shows that groundwater is especially important to river flow in the premonsoon. Source waters to river flow were calculated using δ 18 O as a tracer, indicating that rain and meltwater are more evenly important across the elevation transect in the early monsoon period than in the postmonsoon period when ice melt contributions rapidly wane with distance from the glacier.

Abstract PurposeThe incidence of severe combined immunodeficiency (SCID) in the USA was reported as 1 in 58,000 live births. In Arizona, it was anticipated that newborn screening would identify two to four cases of SCID per year. This estimate did not consider ethnic nuances in Arizona, with higher percentages of Native American and Hispanic populations compared to national percentages. The true incidence of SCID and non-SCID T cell lymphopenia has not previously been reported in Arizona.MethodsA retrospective chart review was performed on all abnormal SCID newborn screening (NBS) tests in Arizona from January 1, 2018, to December 31, 2019, using data from the Arizona Department of Health Services and the Phoenix Children’s Hospital’s electronic medical record [IRB# 20–025].ResultsSeven infants were diagnosed with SCID, yielding an incidence of 1 in 22,819 live births. Four of these infants had Artemis-type SCID. Thirteen infants were identified with an abnormal initial NBS which ultimately did not lead to a diagnosis of SCID. Four of these infants were diagnosed with congenital syndromes associated with T cell lymphopenia. Infants of Hispanic ethnicity were over-represented in this cohort.ConclusionOver 2 years, NBS in Arizona confirmed an incidence more than 2.5 times that reported nationally. This increased incidence is likely reflective of Arizona’s unique population profile, with a higher percentage of Native American population. The findings in our non-SCID cohort are in alignment with previously published data, except for an increased percentage of infants of Hispanic/Latino ethnicity, possibly reflecting Arizona’s increased percentage of Hispanic/Latino population compared to the general US population.