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Neurofibromatosis (NF), including type 1 (NF1), type 2 (NF2), and schwannomatosis; tuberous sclerosis complex (TSC); and Sturge-Weber syndrome are 3 neurocutaneous disorders that typically present in childhood. Early recognition by the pediatrician can be critical to surveillance for treatable complications and genetic counseling. These conditions are diagnosed clinically, but genetic testing is available to clarify an uncertain diagnosis or help with genetic counseling. Although many of the complications can only be treated symptomatically, advances in understanding of the pathogenesis are opening new approaches to molecularly targeted therapeutics, which promise to alter the natural history of the conditions in the years to come.

L’hyperphagie boulimique (HB) représente le trouble alimentaire le plus fréquent avec une prévalence qui est plus élevée que celles combinées de l’anorexie nerveuse et de la boulimie (Hudson et al. , 2007). Bien que l’HB a fait officiellement son entrée dans le DSM-5, il demeure de grandes difficultés concernant l’accessibilité et la disponibilité des traitements s’appuyant sur des données probantes. Le but de cet article est de présenter une recension critique de la littérature qui fera ressortir l’utilisation de la cyberthérapie dans le traitement de l’HB ainsi que les implications au niveau de la pratique clinique et de la recherche. , Binge eating disorder (BED) is the most common eating disorder, with a prevalence higher than the prevalence of anorexia nervosa and bulimia combined (Hudson et a l., 2007). Although BED officially entered the DSM-5, there are still great difficulties regarding the accessibility and availability of evidence-based treatments. The purpose of this paper is to present a critical review of the literature highlighting the use of e-therapy for BED as well as the implications for clinical practice and research.

Adaptation to climate change is a challenge that is complex and involves increasing risk. Efforts to manage these risks involve many decision-makers, conflicting values, competing objectives and methodologies, multiple alternative options, uncertain outcomes, and debatable probabilities. Adaptation occurs at multiple levels in a complex decision environment and is generally evaluated as better–worse, not right–wrong, based on multiple criteria. Identifying the best adaptation response is difficult. Risk management techniques help to overcome these problems. Here, risk management is presented as a decision-making framework that assists in the selection of optimal strategies (according to various criteria) using a systems approach that has been well defined and generally accepted in public decision-making. In the context of adapting to climate change, the risk management process offers a framework for identifying, assessing, and prioritizing climate-related risks and developing appropriate adaptation responses. The theoretical discussion is illustrated with an example from Canada. It includes (a) the assessment of climate change-caused flood risk to the municipal infrastructure for the City of London, Ontario, Canada, and (b) analysis of adaptation options for management of the risk in one of the watersheds within the City of London – Dingman Creek.

Abstract Study Region: In Canada, dams which represent a high risk to human loss of life, along with important environmental and financial losses in case of failure, have to accommodate the Probable Maximum Flood (PMF). Five Canadian basins with different physiographic characteristics and geographic locations, and where the PMF is a relevant metric have been selected: Nelson, Mattagami, Kenogami, Saguenay and Manic-5. Study Focus: One of the main drivers of the PMF is the Probable Maximum Precipitation (PMP). Traditionally, the computation of the PMP relies on moisture maximization of high efficiency observed storms without consideration for climate change. The current study attempts to develop a novel approach based on traditional methods to take into account the non-stationarity of the climate using an ensemble of 14 regional climate model (RCM) simulations. PMPs, the 100-year snowpack and resulting PMF changes were computed between the 1971-2000 and 2041-2070 periods. New Hydrological Insights for the Region: The study reveals an overall increase in future spring PMP with the exception of the most northern basin Nelson. It showed a projected increase of the 100-year snowpack for the two northernmost basins, Nelson (8%) and Manic-5 (3%), and a decrease for the three more southern basins, Mattagami (-1%), Saguenay (-5%) and Kenogami (-9%). The future spring PMF is projected to increase with median values between -1.5% and 20%.

Variability and nonstationarity in flood regimes of cold regions are examined using data from hydrometric reference streamflow gauging stations from 27 natural watersheds in Canada and adjacent areas of the United States. Choosing stations from reference networks with nearly 100 years of data allows for the investigation of changes that span several phases of some of the atmospheric drivers that may influence flood behavior. The reference hydrologic networks include only stations considered to have good quality data and were screened to avoid the influences of regulation, diversions, or land use change. Changes and variations in flood regimes are complex and require a multifaceted approach to properly characterize the types of changes that have occurred and are likely to occur in the future. Peaks over threshold (POT) data are extracted from daily flow data for each watershed, and changes to the magnitude, timing, frequency, volume, and duration of threshold exceedences are investigated. Seasonal statistics are used to explore changes in the nature of the flood regime based on changes in the timing of flood threshold exceedences. A variety of measures are developed to infer flood regime shifts including from a nival regime to a mixed regime and a mixed regime to a more pluvial-dominated regime. The flood regime at many of the watersheds demonstrates increased prominence of rainfall floods and decreased prevalence of snowmelt contributions to flood responses. While some individual stations show a relationship between flood variables and climate indices, these relationships are generally weak.

Changes in extreme precipitation should be one of the primary impacts of climate change (CC) in urban areas. To assess these impacts, rainfall data from climate models are commonly used. The main goal of this paper is to report on the state of knowledge and recent works on the study of CC impacts with a focus on urban areas, in order to produce an integrated review of various approaches to which future studies can then be compared or constructed. Model output statistics (MOS) methods are increasingly used in the literature to study the impacts of CC in urban settings. A review of previous works highlights the non-stationarity nature of future climate data, underscoring the need to revise urban drainage system design criteria. A comparison of these studies is made difficult, however, by the numerous sources of uncertainty arising from a plethora of assumptions, scenarios, and modeling options. All the methods used do, however, predict increased extreme precipitation in the future, suggesting potential risks of combined sewer overflow frequencies, flooding, and back-up in existing sewer systems in urban areas. Future studies must quantify more accurately the different sources of uncertainty by improving downscaling and correction methods. New research is necessary to improve the data validation process, an aspect that is seldom reported in the literature. Finally, the potential application of non-stationarity conditions into generalized extreme value (GEV) distribution should be assessed more closely, which will require close collaboration between engineers, hydrologists, statisticians, and climatologists, thus contributing to the ongoing reflection on this issue of social concern.

Abstract Large wood (LW) is a ubiquitous feature in rivers of forested watersheds worldwide, and its importance for river diversity has been recognized for several decades. Although the role of LW in fluvial dynamics has been extensively documented, there is a need to better quantify the most significant components of LW budgets at the river scale. The purpose of our study was to quantify each component (input, accumulation, and output) of a LW budget at the reach and watershed scales for different time periods (i.e. a 50‐year period, decadal cycle, and interannual cycle). The LW budget was quantified by measuring the volumes of LW inputs, accumulations, and outputs within river sections that were finally evacuated from the watershed. The study site included three unusually large but natural wood rafts in the delta of the Saint‐Jean River (SJR; Québec, Canada) that have accumulated all LW exported from the watershed for the last 50 years. We observed an increase in fluvial dynamics since 2004, which led to larger LW recruitment and a greater LW volume trapped in the river corridor, suggesting that the system is not in equilibrium in terms of the wood budget but is rather recovering from previous human pressures as well as adjusting to hydroclimatic changes. The results reveal the large variability in the LW budget dynamics during the 50‐year period and allow us to examine the eco‐hydromorphological trajectory that highlights key variables (discharge, erosion rates, bar surface area, sinuosity, wood mobility, and wood retention). Knowledge on the dynamics of these variables improves our understanding of the historical and future trajectories of LW dynamics and fluvial dynamics in gravel‐bed rivers. Extreme events (flood and ice‐melt) significantly contribute to LW dynamics in the SJR river system. Copyright © 2017 John Wiley & Sons, Ltd.

Abstract Spatial and temporal trends in historical temperature and precipitation extreme events were evaluated for southern Ontario, Canada. A number of climate indices were computed using observed and regional and global climate datasets for the area of study over the 1951–2013 period. A decrease in the frequency of cold temperature extremes and an increase in the frequency of warm temperature extremes was observed in the region. Overall, the numbers of extremely cold days decreased and hot nights increased. Nighttime warming was greater than daytime warming. The annual total precipitation and the frequency of extreme precipitation also increased. Spatially, for the precipitation indices, no significant trends were observed for annual total precipitation and extremely wet days in the southwest and the central part of Ontario. For temperature indices, cool days and warm night have significant trends in more than 90% of the study area. In general, the spatial variability of precipitation indices is much higher than that of temperature indices. In terms of comparisons between observed and simulated data, results showed large differences for both temperature and precipitation indices. For this region, the regional climate model was able to reproduce historical observed trends in climate indices very well as compared with global climate models. The statistical bias-correction method generally improved the ability of the global climate models to accurately simulate observed trends in climate indices.

Climate change is likely to affect windthrow risks at northern latitudes by potentially changing high wind probabilities and soil frost duration. Here, we evaluated the effect of climate change on windthrow risk in eastern Canada’s balsam fir (Abies balsamea [L.] Mill.) forests using a methodology that accounted for changes in both wind speed and soil frost duration. We used wind speed and soil temperature projections at the regional scale from the CRCM5 regional climate model (RCM) driven by the CanESM2 global climate model (GCM) under two representative concentration pathways (RCP4.5, RCP8.5), for a baseline (1976–2005) and two future periods (2041–2070, 2071–2100). A hybrid mechanistic model (ForestGALES) that considers species resistance to uprooting and wind speed distribution was used to calculate windthrow risk. An increased risk of windthrow (3 to 30%) was predicted for the future mainly due to an increased duration of unfrozen soil conditions (by up to 2 to 3 months by the end of the twenty-first century under RCP8.5). In contrast, wind speed did not vary markedly with a changing climate. Strong regional variations in wind speeds translated into regional differences in windthrow risk, with the easternmost region (Atlantic provinces) having the strongest winds and the highest windthrow risk. Because of the inherent uncertainties associated with climate change projections, especially regarding wind climate, further research is required to assess windthrow risk from the optimum combination of RCM/GCM ensemble simulations.

Abstract Retrospective estimation of daily streamflow for all rivers within a territory is of practical interest for sustainable and optimal water management. This implies, however, the availability of methods for providing accurate estimations of flow for ungauged rivers. This study compares the potential of statistical interpolation (SI)—a simple data assimilation technique that combines observations and simulations from hydrological modelling—with four other approaches: nearest neighbour, direct use of outputs from hydrological modelling, ordinary and topological kriging. Through subsampling cross-validation analyses based on the modified Kling-Gupta efficiency indicator, we show that SI compares favourably with these other approaches. While the performance of other methods depends on the configuration of the ungauged site in regards to the neighbouring reference sites, SI is less affected by these configurations. SI outperforms the other approaches particularly where the ungauged site is relatively distant from observation sites. In these cases, SI performance depends on the performance of the background model that relies on simulations of hydrological processes forced by precipitation and temperature observations. Our findings offer the potential for heightened performance estimates through an improvement of hydrological modelling and the use of more complex assimilation techniques for exploiting the model.

Several businesses and industries rely on rainfall forecasts to support their day-to-day operations. To deal with the uncertainty associated with rainfall forecast, some meteorological organisations have developed products, such as ensemble forecasts. However, due to the intensive computational requirements of ensemble forecasts, the spatial resolution remains coarse. For example, Environment and Climate Change Canada’s (ECCC) Global Ensemble Prediction System (GEPS) data is freely available on a 1-degree grid (about 100 km), while those of the so-called High Resolution Deterministic Prediction System (HRDPS) are available on a 2.5-km grid (about 40 times finer). Potential users are then left with the option of using either a high-resolution rainfall forecast without uncertainty estimation and/or an ensemble with a spectrum of plausible rainfall values, but at a coarser spatial scale. The objective of this study was to evaluate the added value of coupling the Gibbs Sampling Disaggregation Model (GSDM) with ECCC products to provide accurate, precise and consistent rainfall estimates at a fine spatial resolution (10-km) within a forecast framework (6-h). For 30, 6-h, rainfall events occurring within a 40,000-km2 area (Quebec, Canada), results show that, using 100-km aggregated reference rainfall depths as input, statistics of the rainfall fields generated by GSDM were close to those of the 10-km reference field. However, in forecast mode, GSDM outcomes inherit of the ECCC forecast biases, resulting in a poor performance when GEPS data were used as input, mainly due to the inherent rainfall depth distribution of the latter product. Better performance was achieved when the Regional Deterministic Prediction System (RDPS), available on a 10-km grid and aggregated at 100-km, was used as input to GSDM. Nevertheless, most of the analyzed ensemble forecasts were weakly consistent. Some areas of improvement are identified herein.

En marge de la Cinquième Plateforme régionale pour la Réduction des risques de catastrophes des Amériques (PRA), le gouvernement du Canada a approché l’Institut des sciences de l’environnement(ISE) de l’Université du Québec à Montréal(UQAM) afin d’organiser un forum public. Les échanges de ce dernier devaient servir à alimenter les discussions de la PRA. Au total, 21 experts ont discuté avec une centaine de participants lors de panels organisés à l’UQAM sous les thèmes de la santé, de la sécurité civile et de l’aménagement du territoire. Plusieurs thèmes transversaux ont aussi émergé tout au long du forum. Il importe de pérenniser le rôle de la recherche et d’améliorer les capacités de formation technique et universitaire afin de former des spécialistes en mesure d’appréhender la complexité de la gestion du risque dans un contexte de changements environnementaux et climatiques. Ceci est également essentiel pour l’identification des facteurs de risque (multisources ou multidimensionnels), pour tirer des leçons apprises des événements majeurs passés et récents, et pour développer ou mettre à jour la connaissance sur les tendances en cours et à venir des aléas météorologiques, ainsi que des facteurs de vulnérabilité et d’exposition. Tous les panels ont discuté de l’importance de favoriser le décloisonnement intra/interorganisationnel pour promouvoir la transsectorialité et les retours d’expériences systématiques. Pour ce faire, il faut s’inspirer des modèles internationaux, notamment du système d’alertes hydrométéorologiques présenté par Météo-France. Celui-ci inclut une vigilance météorologique qui cible des populations et des autorités publiques, et les informe des comportements et des règles à suivre lors d’alertes plus problématiques (vigilance aux stades orange et rouge). Finalement, l’amélioration de la communication et le libre accès à l’information sont des éléments essentiels pour protéger les individus et développer une société plus résiliente.