Impact dynamics of mud flows against rigid walls

Mud flows represent one of the major causes of natural hazards in mountain regions. Similarly to debris flows, they consist of a hyper-concentrated mixture of water and sediments flowing down a slope and may cause serious damages to people and structures. The present paper investigates the force produced by a dam-break wave of mud impacting against a rigid wall. A power-law shearthinning model is used to describe the rheology of the hyper-concentrated mixture. A onedimensional shallow water model is adopted and a second-order Finite Volume scheme is employed to numerically solve the governing equations. The results indicate that depending on the fluid rheological parameters and on the bottom slope, there exists a minimum value of the wall distance above which the peak force does not exceed the asymptotic value of the hydrostatic final condition. For two different values of the channel slope, the dimensionless value of this lower bound is individuated for several values of the power-law exponent and of a dimensionless Basal Drag coefficient. An estimation of the maximum peak force for wall distance smaller than the minimum value is also provided

Energy transfer from the freshwater to the wastewater network using a PAT-equipped turbopump

A new strategy to increase the energy efficiency in a water network exists using turbo pumps, which are systems consisting of a pump and a turbine directly coupled on a same shaft. In a turbo pump, the pump is fed by a turbine that exploits a surplus head in a freshwater network in order to produce energy for one system (wastewater) and reduce the excess pressure in another (drinking water). A pump as turbine (PAT) may be preferred over a classic turbine here due to its lower cost. The result of such a coupling is a PAT-pump turbocharger (P&P). In this research, the theoretical performance of a P&P plant is employed using data from a real water distribution network to exploit the excess pressure of a freshwater stream and to feed a pump conveying wastewater toward a treatment plant. Therefore, the P&P plant is a mixed PAT-pump turbocharger, operating with both fresh and wastewater. A new method to perform a preliminary geometric selection of the machines constituting the P&P plant has been developed. Furthermore, the plant operation has been described by means of a new mathematical model under different boundary conditions. Moreover, the economic viability of the plant has been assessed by comparison with a conventional wastewater pumping system working in ON/OFF mode. Therefore, the net present value (NPV) of the investment has been evaluated in both situations for different time periods. According to the economical comparison, the PAT-pump turbocharger represents the most economically advantageous configuration, at least until the useful life of the plant. Such convenience amounts to 175% up to a time period equal to 20 years

A performance prediction model for pumps as turbines (PATs)

In recent years, the interest towards the use of pumps operating as turbines (PATs) for the generation of electrical energy has increased, due to the low cost of implementation and maintenance. The main issue that inhibits a wider use of PATs is the lack of corresponding characteristic curves, because manufacturers usually provide only the pump-mode performance characteristics. In the PAT selection phase, the lack of turbine-mode characteristic curves forces users to expend expensive and time-consuming efforts in laboratory testing. In the technical literature, numerous methods are available for the prediction of PAT turbine-mode performance based on the pump-mode characteristics, but these models are usually calibrated making use of few devices. To overcome this limit, a performance database called Redawn is presented and the data collected are used to calibrate novel PAT performance models

Potentialities of Complex Network Theory Tools for Urban Drainage Networks Analysis

Urban drainage networks (UDNs) represent important infrastructures to protect and maintain community health and safety. For these reasons, technicians and researcher are focusing more and more on topics related to vulnerability, resilience and monitoring for controlling illicit intrusions, contaminant and pathogenic spread. In the last years the complex network theory (CNT) is attracting attention as a new, useful and structured approach to analyze urban systems. The aim of this work is to evaluate potentialities of CNT approaches for UDNs vulnerability assessment and monitoring system planning. Limits and potentialities of applicability of CNT tools to UDNs are first provided evaluating the performances of standard centrality metrics. Then, it is proposed the use of tailored metrics embedding prior information, as intrinsic relevance of each node and pipe flow direction, which derive from the Horton's hierarchy and geometric data (pipe slope), respectively, without performing hydraulic simulations. The analysis is applied on two schematic literature networks of different complexity and to a real case-study. The results suggest that vulnerability/resilience, monitoring design, contaminant and pathogenic spreads can be effectively analyzed using tailored metrics. Therefore, the proposed approach represents a complementary tool respect the more complex and computationally expensive methodologies and it is particular useful for large complex networks

Lipid Antigen Presentation by CD1b and CD1d in Lysosomal Storage Disease Patients

The lysosome has a key role in the presentation of lipid antigens by CD1 molecules. While defects in lipid antigen presentation and in invariant Natural Killer T (iNKT) cell response were detected in several mouse models of lysosomal storage diseases (LSD), the impact of lysosomal engorgement in human lipid antigen presentation is poorly characterized. Here, we analyzed the capacity of monocyte-derived dendritic cells (Mo-DCs) from Fabry, Gaucher, Niemann Pick type C and Mucopolysaccharidosis type VI disease patients to present exogenous antigens to lipid-specific T cells. The CD1b- and CD1d-restricted presentation of lipid antigens by Mo-DCs revealed an ability of LSD patients to induce CD1-restricted T cell responses within the control range. Similarly, freshly isolated monocytes from Fabry and Gaucher disease patients had a normal ability to present α-Galactosylceramide (α-GalCer) antigen by CD1d. Gaucher disease patients' monocytes had an increased capacity to present α-Gal-(1-2)-αGalCer, an antigen that needs internalization and processing to become antigenic. In summary, our results show that Fabry, Gaucher, Niemann Pick type C, and Mucopolysaccharidosis type VI disease patients do not present a decreased capacity to present CD1d-restricted lipid antigens. These observations are in contrast to what was observed in mouse models of LSD. The percentage of total iNKT cells in the peripheral blood of these patients is also similar to control individuals. In addition, we show that the presentation of exogenous lipids that directly bind CD1b, the human CD1 isoform with an intracellular trafficking to the lysosome, is normal in these patients.This work was financed by Gaucher Generation Program, supported by Sanofi-Genzyme and by Norte-01-0145-FEDER-000012—Structured program on bioengineered therapies for infectious diseases and tissue regeneration, supported by the Norte Portugal Regional Operational Programme (NORTE 2020), under the PORTUGAL 2020 Partnership Agreement, through the European Regional Development Fund (FEDER). BP-C was temporally supported by a grant from the American Portuguese Biomedical Research Fund (APBRF).info:eu-repo/semantics/publishedVersio

Stable or improved neurological manifestations during miglustat therapy in patients from the international disease registry for Niemann-Pick disease type C: an observational cohort study

Background: Niemann-Pick disease type C (NP-C) is a rare neurovisceral disease characterised by progressive neurological degeneration, where the rate of neurological disease progression varies depending on age at neurological onset. We report longitudinal data on functional disease progression and safety observations in patients in the international NPC Registry who received continuous treatment with miglustat. Methods: The NPC Registry is a prospective observational cohort of NP-C patients. Enrolled patients who received ≥1 year of continuous miglustat therapy (for ≥90 % of the observation period, with no single treatment interruption >28 days) were included in this analysis. Disability was measured using a scale rating the four domains, ambulation, manipulation, language and swallowing from 0 (normal) to 1 (worst). Neurological disease progression was analysed in all patients based on: 1) annual progression rates between enrolment and last follow up, and; 2) categorical analysis with patients categorised as 'improved/stable' if ≥3/4 domain scores were lower/unchanged, and as 'progressed' if <3 scores were lower/unchanged between enrolment and last follow-up visit. Results: In total, 283 patients were enrolled from 28 centers in 13 European countries, Canada and Australia between September 2009 and October 2013; 92 patients received continuous miglustat therapy. The mean (SD) miglustat exposure during the observation period (enrolment to last follow-up) was 2.0 (0.7) years. Among 84 evaluable patients, 9 (11 %) had early-infantile (<2 years), 27 (32 %) had late-infantile (2 to <6 years), 30 (36 %) had juvenile (6 to <15 years) and 18 (21 %) had adolescent/adult (≥15 years) onset of neurological manifestations. The mean (95%CI) composite disability score among all patients was 0.37 (0.32,0.42) at enrolment and 0.44 (0.38,0.50) at last follow-up visit, and the mean annual progression rate was 0.038 (0.018,0.059). Progression of composite disability scores appeared highest among patients with neurological onset during infancy or childhood and lowest in those with adolescent/adult-onset. Overall, 59/86 evaluable patients (69 %) were categorized as improved/stable and the proportion of improved/stable patients increased with age at neurological onset. Safety findings were consistent with previous data. Conclusions: Disability status was improved/stable in the majority of patients who received continuous miglustat therapy for an average period of 2 years

A new low-cost installation scheme of PATs for pico-hydropower to recover energy in residential areas

A limiting factor in the use of hydropower plants in water supply networks consists in the cost of the electromechanical production device and the equipment necessary for the plant regulation. Pumps as Turbines (PATs) are electromechanical devices that have already been used in many mini and micro hydropower plants. Due to their low cost and reliability, PATs are an optimal solution for pico-hydropower plants, whose regulation has been widely analysed in literature, for power installations of only a few kW. A new power plant for small residential areas is presented herein. The new low-cost plant is provided with two PATs, that can work as single, series or parallel (SSP) turbines, and three on/off valves, whose synchronous operations regulate the flows and the head loss. The proposed design method is based on the maximization of the effectiveness of the system. Experimental data on a PAT are used to simulate the plant in several hydraulic conditions, with the application of the turbomachinery affinity law. The simple plant operations and the resulting interactions with the network confirm the viability of the proposed scheme. An economic assessment shows the feasibility of the new plant in different hydraulic situations