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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.
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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.
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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.
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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.
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Abstract Urban political ecology (UPE) has mainly evolved within the discipline of geography to examine the power relations that produce uneven urban spaces (infrastructures and natures) and unequal access to resources in cities. Its increasingly poststructuralist orientation demands the questioning of received categories and concepts, including those of (neoliberal) governance, government, and of the state. This paper attempts to open this black box by referring to the mostly anthropological literature on everyday governance and the everyday state. We argue that UPE could benefit from ethnographic governance studies to unveil multiple state and non‐state actors that influence the local environment, their diverse rationalities, normative registers, and interactions across scales. This would also to enrich and nuance geographical UPE accounts of neoliberal environmental governance and potentially render the framework more policy relevant.