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Conclusion We present a clinical odyssey showing the natural progression and history of FCPs in TSC and comment on the mechanistic pathways allowing potential interventions in this disfiguring condition. TSC cases can be successfully managed and complications - particularly in the brain and kidney, can be avoided over an entire lifetime. This is encouraging for long term prospects for patients with TSC. Copyright © 2020 Ulster Medical Society.We provide a probabilistic approach using renewal theory to derive some novel identities involving Eulerian numbers and uniform B-splines. The renewal perspective leads to a unified treatment for the normalized binomial coefficients and the normalized Eulerian numbers when studying their limits of sums, as well as their associated distributions - the binomial distributions (Bernstein polynomials) and the Irwin-Hall distributions (uniform B-splines). We further extend the probabilistic perspective to h-Bernstein polynomials (Pólya-Eggenberger distributions) through conditional renewal processes, and derive new limits of various ways of summing for the two special numbers and associated distributions. The proposed probabilistic unification dramatically simplifies the proofs of some identities, which are far from obvious (such as for h-Bernstein polynomials) or do not otherwise even appear promising (such as for Eulerian numbers).Biodiversity is suffering dramatic declines across the globe, threatening the ability of ecosystems to provide the services on which humanity depends. Mainstreaming biodiversity into the plans, strategies and policies of different economic sectors is key to reversing these declines. The importance of this mainstreaming is recognized by the Convention on Biological Diversity (CBD) and its Aichi targets. Individual countries can implement the goals of the CBD through their National Biodiversity Strategies and Action Plans (NBSAPs), which aim to, inter alia, support the mainstreaming of biodiversity into the policies of key economic sectors, such as agriculture, forestry and fisheries. This paper investigates the performance of countries at incorporating biodiversity mainstreaming into their post-2010 NBSAPs. We conduct a large-scale review of 144 NBSAPs against five criteria and calculate a national-level indicator for comparing levels of mainstreaming among countries. Our results show that developing countries, particularly those in Africa, have higher scores, indicating that they have a higher awareness of the importance of biodiversity mainstreaming. Developing nations were also more likely to involve a greater range of stakeholders in the NBSAP development process, whilst developed nations were less likely to give specific details about the monetary contributions of biodiversity to their economies. Overall, our findings suggest that biodiversity mainstreaming remains a challenge across much of the world, but that progress in some areas can provide direction and momentum in the future. © 2019 The Authors.Oil pipelines are vulnerable at river crossings since floods can expose and rupture pipes, releasing oil that floats and coats floodplain vegetation. This study investigated the consequences of oil coatings on leaves of cottonwoods (riparian poplars), the predominant trees in floodplain woodlands around the Northern Hemisphere. The study compared conventional crude oil (CO) versus diluted bitumen (dilbit, DB), heavy oil originating from the Alberta oil sands; with petroleum jelly (PJ) as a reference. The treatments increased leaf surface temperatures (Tleaf) in narrowleaf and plains cottonwoods (Populus angustifolia, P. https://www.selleckchem.com/products/vx-661.html deltoides) and balsam poplars (P. balsamifera) (Control = 21.8°C, PJ = 23.7°C; CO = 26.2°C; DB = 28.1°C; Tair = 25°C). The leaf warming followed stomatal occlusion from the foliar coating, which would reduce transpiration and evaporative cooling, combined with increased solar warming with the darker oils. Tleaf varied across the three cottonwood species, with cooler, narrow, narrowleaf cottonwood leaves; intermediate plains cottonwood leaves; and warmer, darker, balsam poplar leaves (average Tleaf narrowleaf = 23.8°C, plains = 24.3°C, and balsam = 26.7°C), with similar warming in each species following the different treatments. Across species and treatments, Tleaf was tightly correlated with foliar condition, which assessed turgor versus wilting of leaf blades and petioles, along with leaf necrosis and senescence (r2 = 0.980, narrowleaf; 0.998, plains; 0.852, balsam). This tight association indicates validity of both Tleaf and foliar condition as diagnostic measures. Crude oil and dilbit had similar foliar impacts, and for both, leaf abscission occurred within 2 to 3 weeks. Consequently, following an oil spill, remediation should commence quickly but extending vegetation removal beyond a few weeks would have limited benefit since the contaminated leaves would have abscised.Despite the diversity in fish auditory structures, it remains elusive how otolith morphology and swim bladder-inner ear (= otophysic) connections affect otolith motion and inner ear stimulation. A recent study visualized sound-induced otolith motion; but tank acoustics revealed a complex mixture of sound pressure and particle motion. To separate sound pressure and sound-induced particle motion, we constructed a transparent standing wave tube-like tank equipped with an inertial shaker at each end while using X-ray phase contrast imaging. Driving the shakers in phase resulted in maximised sound pressure at the tank centre, whereas particle motion was maximised when shakers were driven out of phase (180°). We studied the effects of two types of otophysic connections-i.e. the Weberian apparatus (Carassius auratus) and anterior swim bladder extensions contacting the inner ears (Etroplus canarensis)-on otolith motion when fish were subjected to a 200 Hz stimulus. Saccular otolith motion was more pronounced when the swim bladder walls oscillated under the maximised sound pressure condition. The otolith motion patterns mainly matched the orientation patterns of ciliary bundles on the sensory epithelia. Our setup enabled the characterization of the interplay between the auditory structures and provided first experimental evidence of how different types of otophysic connections affect otolith motion.
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