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Smart Energy Management and Conversion

Cross‐sectional associations between violent video and computer game playing and weapon carrying in a national cohort of children

Data were collected from 9 to 18 year olds surveyed nationally in a three‐wave longitudinal survey. The population‐average (generalized estimating equation, GEE) odds of carrying a weapon to school in the last month were estimated as a function of past‐year exposure to violent content in video, computer, and Internet games, as well as peer aggression and biological sex. The sample included youth who were at risk for both the exposure (i.e., game play) and the outcome (i.e., who attended public or private school). 3,397 observations from 1,489 youth were included in analyses. 1.4% of youth reported carrying a weapon to school in the last month and 69% reported that at least some of the games they played depicted violence. After adjusting for other potentially influential characteristics (e.g., aggressive behavior), playing at least some violent games in the past year was associated with a fourfold increase in odds of also reporting carrying a weapon to school in the last month. Although youth who reported frequent and intense peer victimization in the past year were more likely to report carrying a weapon to school in the last month, this relation was explained by other influential characteristics. Consistent with the predictions of social‐cognitive, observational learning theory, this study supports the hypothesis that carrying weapons to school is associated with violent game play. As one of the first studies of its kind, findings should be interpreted cautiously and need to be replicated. Aggr. Behav. 40:345–358, 2014. © 2014 Wiley Periodicals, Inc.

Phenylbutyrate increases pyruvate dehydrogenase complex activity in cells harboring a variety of defects

Objective Deficiency of pyruvate dehydrogenase complex ( PDHC ) is the most common genetic disorder leading to lactic acidosis. PDHC deficiency is genetically heterogenous and most patients have defects in the X‐linked E1‐ α gene but defects in the other components of the complex encoded by PDHB , PDHX , DLAT , DLD genes or in the regulatory enzyme encoded by PDP 1 have also been found. Phenylbutyrate enhances PDHC enzymatic activity in vitro and in vivo by increasing the proportion of unphosphorylated enzyme through inhibition of pyruvate dehydrogenase kinases and thus, has potential for therapy of patients with PDHC deficiency. In the present study, we investigated response to phenylbutyrate of multiple cell lines harboring all known gene defects resulting in PDHC deficiency. Methods Fibroblasts of patients with PDHC deficiency were studied for their enzyme activity at baseline and following phenylbutyrate incubation. Drug responses were correlated with genotypes and protein levels by Western blotting. Results Large deletions affecting PDHA 1 that result in lack of detectable protein were unresponsive to phenylbutyrate, whereas increased PDHC activity was detected in most fibroblasts harboring PDHA 1 missense mutations. Mutations affecting the R349‐ α residue were directed to proteasome degradation and were consistently unresponsive to short‐time drug incubation but longer incubation resulted in increased levels of enzyme activity and protein that may be due to an additional effect of phenylbutyrate as a molecular chaperone. Interpretation PDHC enzyme activity was enhanced by phenylbutyrate in cells harboring missense mutations in PDHB , PDHX , DLAT , DLD , and PDP 1 genes. In the prospect of a clinical trial, the results of this study may allow prediction of in vivo response in patients with PDHC deficiency harboring a wide spectrum of molecular defects.

Early Systemic Sclerosis: Analysis of the Disease Course in Patients With Marker Autoantibody and/or Capillaroscopic Positivity

Objective To investigate whether patients affected by 1 of the 3 subsets of early systemic sclerosis (SSc; scleroderma), i.e., subset I, Raynaud's phenomenon with SSc marker autoantibodies and typical capillaroscopic findings; subset II, autoantibody positive only; and subset III, capillaroscopy positive only and not satisfying the 2013 American College of Rheumatology (ACR)/European League Against Rheumatism (EULAR) classification criteria for SSc at admission, differ from each other in the time to satisfy the criteria. Methods Early SSc patients subdivided into the 3 subsets indicated above consecutively admitted to a rheumatology/angiology center were monitored for 12–102 months (median 36 months). Patients were reevaluated twice yearly to assess whether and when each patient satisfied the new ACR/EULAR classification criteria for SSc. Patients with undifferentiated connective tissue disease (UCTD) served as the comparator group. Results During followup, 11 (52.3%) of 21 subset I, 10 (66.6%) of 15 subset II, 0 of 24 subset III, and 0 of 44 UCTD patients satisfied the criteria ( P = 0.0001). The difference was significant between early SSc and UCTD patients ( P = 0.0001) and, within the group of early SSc patients, between each of the 2 autoantibody‐positive subsets (subsets I and II) and the capillaroscopic‐positive/autoantibody‐negative subset (subset I versus III: P = 0.0001; subset II versus III: P = 0.0009). There was no difference between the 2 autoantibody‐positive subsets ( P = 0.454). In addition to marker autoantibody positivity, preclinical lung or heart involvement was associated with an increased risk to satisfy the criteria during followup. Conclusion Our data demonstrated faster progression of SSc in autoantibody‐positive patients, particularly in those with preclinical internal organ involvement at baseline, than in autoantibody‐negative patients.