Happiness – a composite of life satisfaction, coping resources, and positive emotions – predicts desirable life outcomes in many domains. The broaden-and-build theory suggests that this is because positive emotions help people build lasting resources. To test this hypothesis we measured emotions daily for one month in a sample of students (N=86) and assessed life satisfaction and trait resilience at the beginning and end of the month. Positive emotions predicted increases in both resilience and life satisfaction. Negative emotions had weak or null effects, and did not interfere with the benefits of positive emotions. Positive emotions also mediated the relation between baseline and final resilience, but life satisfaction did not. This suggests that it is in-the-moment positive emotions, and not more general positive evaluations of one’s life, that form the link between happiness and desirable life outcomes. Change in resilience mediated the relation between positive emotions and increased life satisfaction, suggesting that happy people become more satisfied not simply because they feel better, but because they develop resources for living well.

Purpose: To evaluate the safety and efficacy of recombinant human iduronate-2-sulfatase (idursulfase) in the treatment of mucopolysaccharidosis II. Methods: Ninety-six mucopolysaccharidosis II patients between 5 and 31 years of age were enrolled in a double-blind, placebo-controlled trial. Patients were randomized to placebo infusions, weekly idursulfase (0.5 mg/kg) infusions or every-other-week infusions of idursulfase (0.5 mg/kg).Efficacy was evaluated using a composite endpoint consisting of distance walked in 6 minutes and the percentage of predicted forced vital capacity based on the sum of the ranks of change from baseline. Results: Patients in the weekly and every-other-week idursulfase groups exhibited significant improvement in the composite endpoint compared to placebo (P ϭ 0.0049 for weekly and P ϭ 0.0416 for every-other-week) after one year. The weekly dosing group experienced a 37-m increase in the 6-minute-walk distance (P ϭ 0.013), a 2.7% increase in percentage of predicted forced vital capacity (P ϭ 0.065), and a 160 mL increase in absolute forced vital capacity (P ϭ 0.001) compared to placebo group at 53 weeks. Idursulfase was generally well tolerated, but infusion reactions did occur.

Purpose: This study evaluated the safety and effectiveness of long-term enzyme replacement therapy with idursulfase (recombinant human iduronate-2-sulfatase) in patients with Hunter syndrome. Methods: All 94 patients who completed a 53-week double-blinded study of idursulfase enrolled in this open-labeled extension study and received intravenous idursulfase at a dose of 0.5 mg/kg weekly for 2 years, and clinical outcomes and safety were assessed. Results: No change in percent predicted forced vital capacity was seen, but absolute forced vital capacity demonstrated sustained improvement and was increased 25.1% at the end of the study. Statistically significant increases in 6-minute walking test distance were observed at most time points. Mean liver and spleen volumes remained reduced throughout the 2-year extension study. Mean joint range of motion improved for the shoulder and remained stable in other joints. Both the parent-and child-assessed Child Health Assessment Questionnaire Disability Index Score demonstrated significant improvement. Infusion-related adverse events occurred in 53% of patients and peaked at Month 3 of treatment and declined thereafter. Neutralizing IgG antibodies were detected in 23% of patients and seemed to attenuate the improvement in pulmonary function. Conclusions: Weekly infusions of idursulfase result in sustained clinical improvement during 3 years of treatment. Genet Med 2011:13(2):95-101.

Prior studies on the association between weather and psychological changes have produced mixed results. In part, this inconsistency may be because weather's psychological effects are moderated by two important factors: the season and time spent outside. In two correlational studies and an experiment manipulating participants' time outdoors (total N = 605), pleasant weather (higher temperature or barometric pressure) was related to higher mood, better memory, and "broadened" cognitive style during the spring as time spent outside increased. The same relationships between mood and weather were not observed during other times of year, and indeed hotter weather was associated with lower mood in the summer. These results are consistent with findings on seasonal affective disorder, and suggest that pleasant weather improves mood and broadens cognition in the spring because people have been deprived of such weather during the winter.

Despite an extensive history underscoring the role of social processes and child contributions to the development of executive functions (Lewis & Carpendale, 2009; Vygotsky, 1987), research on these relations is sparse. To address this gap, sixty-eight mother-child dyads were examined to determine whether maternal attention-directing behaviors (attention-maintaining, attention-redirection) and toddlers’ temperament predicted executive processes during preschool (mean age = 4.5 years, SD = 0.46) - delay and conflict inhibition. Maternal attention-maintaining was associated with high levels of conflict inhibition for inhibited and exuberant children, whereas attention- redirection was associated with low levels of delay and conflict inhibition for inhibited children. Therefore, maternal attention-directing behaviors may enhance the development of executive functions but only for children with inhibited and exuberant temperaments.