No abstract

Background: There is an increasing number of adults with phenylketonuria (PKU) on a low phenylalanine diet. In the general population, an increasing body mass index (BMI) in the UK is a major problem with associated co-morbidities. The present study aimed to identify whether patients with diet-treated PKU have obesity rates comparable to those in the general population. Methods: Two hundred and thirty-six PKU subjects (49% male, 51% female), aged >16 years, who were diagnosed by newborn screening and were receiving a low phenylalanine diet, were identified from seven metabolic centres in the UK. Retrospective data were collated on age, sex, BMI and mean phenylalanine concentration over the previous 12 months. Results: Mean (SD) phenylalanine concentration for all 236 subjects was 789 (311) lM; mean (SD) BMI was 26 (5.4) kg m À2 [males 25 (4.3) kg m À2 ,

Dietary management of 418 adult patients with galactosaemia (from 39 centres/12 countries) was compared. All centres advised lactose restriction, 6 restricted galactose from galactosides ± fruits and vegetables and 12 offal. 38% (n=15) relaxed diet by: 1) allowing traces of lactose in manufactured foods (n=13) or 2) giving fruits, vegetables and galactosides (n=2). Only 15% (n=6) calculated dietary galactose. 32% of patients were lost to dietetic follow-up. In adult galactosaemia, there is limited diet relaxation.

Background: Populations are simultaneously exposed to outdoor concentrations of oxidant gases (i.e., O 3 and NO 2 ) and fine particulate air pollution (PM 2.5 ). Since oxidative stress is thought to be an important mechanism explaining air pollution health effects, the adverse health impacts of oxidant gases may be greater in locations where PM 2.5 is more capable of causing oxidative stress. Methods: We conducted a cohort study of 2 million adults in Canada between 2001 and 2016 living within 10 km of ground-level monitoring sites for outdoor PM 2.5 components and oxidative potential. O x exposures (i.e., the redox-weighted average of O 3 and NO 2 ) were estimated using a combination of chemical transport models, land use regression models, and ground-level data. Cox proportional hazards models were used to estimate associations between 3-year moving average O x and mortality outcomes across strata of transition metals and sulfur in PM 2.5 and three measures of PM 2.5 oxidative potential adjusting for possible confounding factors.Results: Associations between O x and mortality were consistently stronger in regions with elevated PM 2.5 transition metal/sulfur content and oxidative potential. For example, each interquartile increase (6.27 ppb) in O x was associated with a 14.9% (95% CI = 13.0, 16.9) increased risk of nonaccidental mortality in locations with glutathione-related oxidative potential (OP GSH ) above the median whereas a 2.50% (95% CI = 0.600, 4.40) increase was observed in regions with OP GSH levels below the median (interaction P value <0.001). Conclusion: Spatial variations in PM 2.5 composition and oxidative potential may contribute to heterogeneity in the observed health impacts of long-term exposures to oxidant gases.

There is growing interest to move beyond fine particle mass concentrations (PM 2.5 ) when evaluating the population health impacts of outdoor air pollution. However, few exposure models are currently available to support such analyses. In this study, we conducted large-scale monitoring campaigns across Montreal and Toronto, Canada during summer 2018 and winter 2019 and developed models to predict spatial variations in (1) the ability of PM 2.5 to generate reactive oxygen species in the lung fluid (ROS), (2) PM 2.5 oxidative potential based on the depletion of ascorbate (OP AA ) and glutathione (OP GSH ) in a cell-free assay, and (3) anhysteretic magnetic remanence (X ARM ) as an indicator of magnetite nanoparticles. We also examined how exposure to PM oxidative capacity metrics (ROS/OP) varied by socioeconomic status within each city. In Montreal, areas with higher material deprivation, indicating lower area-level average household income and employment, were exposed to PM 2.5 characterized by higher ROS and OP. This relationship was not observed in Toronto. The developed models will be used in epidemiologic studies to assess the health effects of exposure to PM 2.5 and iron-rich magnetic nanoparticles in Toronto and Montreal.