Erythrocytosis in children and adolescents-classification, characterization, and consensus recommendations for the diagnostic approach

During recent years, the increasing knowledge of genetic and physiological changes in polycythemia vera (PV) and of different types of congenital erythrocytosis has led to fundamental changes in recommendations for the diagnostic approach to patients with erythrocytosis. Although widely accepted for adult patients this approach may not be appropriate with regard to children and adolescents affected by erythrocytosis. The "congenital erythrocytosis" working group established within the framework of the MPN&MPNr-EuroNet (COST action BM0902) addressed this question in a consensus finding process and developed a specific algorithm for the diagnosis of erythrocytosis in childhood and adolescence which is presented here. Pediatr Blood Cancer 2013;9999:XX-XX. © 2013 Wiley Periodicals, Inc.

Revised response criteria for polycythemia vera and essential thrombocythemia:an ELN and IWG-MRT consensus project

Standardized response criteria to interpret and compare clinical trials are needed for approval of new therapeutic agents by regulatory agencies. The European LeukemiaNet (ELN) response criteria for essential thrombocythemia (ET) and polycythemia vera (PV) issued in 2009 have been widely adopted as end points in a number of recent clinical trials. However, evidence exists that they do not predict response or provide clinically relevant measures of benefit for the patients. This article presents revised recommendations for assessing response in ET and PV provided by a working group established by ELN and International Working Group-Myeloproliferative Neoplasms Research and Treatment. New definitions of complete and partial remission incorporate clinical, hematological, and histological response assessments that include a standardized symptom assessment form and consider absence of disease progression and vascular events. We anticipate that these criteria will be adopted widely to facilitate the development of new and more effective therapies for ET and PV.

A statistical process control approach for automatic anti-islanding detection using synchrophasors

Anti-islanding protection is becoming increasingly important due to the rapid installation of distributed generation from renewable resources like wind, tidal and wave, solar PV, bio-fuels, as well as from other resources like diesel. Unintentional islanding presents a potential risk for damaging utility plants and equipment connected from the demand side, as well as to public and personnel in utility plants. This paper investigates automatic islanding detection. This is achieved by deploying a statistical process control approach for fault detection with the real-time data acquired through a wide area measurement system, which is based on Phasor Measurement Unit (PMU) technology. In particular, the principal component analysis (PCA) is used to project the data into principal component subspace and residual space, and two statistics are used to detect the occurrence of fault. Then a fault reconstruction method is used to identify the fault and its development over time. The proposed scheme has been used in a real system and the results have confirmed that the proposed method can correctly identify the fault and islanding site.

Circulating YKL-40 in patients with essential thrombocythemia and polycythemia vera treated with the novel histone deacetylase inhibitor vorinostat

YKL-40 regulates vascular endothelial growth factors and induces tumor proliferation. We investigated YKL-40 before and after treatment with vorinostat in 31 polycythemia vera (PV) and 16 essential thrombocythemia (ET) patients. Baseline PV patient levels were 2 times higher than in healthy controls (P < 0.0001) and 1.7 times higher than in ET (P = 0.02). A significant correlation between YKL-40 at baseline and neutrophils, CRP, LDH, JAK2V617F and platelets in PV patients was observed, as well as a significantly greater reduction of YKL-40 levels in PV patients responding to therapy. YKL-40 might be a novel marker of disease burden and progression in myeloproliferative neoplasms.

How common are myeloproliferative neoplasms? A systematic review and meta-analysis

Myeloproliferative neoplasms (MPNs) are a heterogeneous group of diseases including polycythemia vera (PV), essential thrombocythemia (ET), and primary(idiopathic) myelofibrosis (PMF). In this systematic review, we provide a comprehensive report on the incidence and prevalence of MPNs across the globe. Electronic databases (PubMed, EMBASE, MEDLINE, and Web of Science) were searched from their inception to August 2012 for articles reporting MPN incidence or prevalence rates. A random effects meta-analysis was undertaken to produce combined incidence rates for PV, ET, and PMF. Both heterogeneity and small study bias were assessed. Thirty-four studies were included. Reported annual incidence rates ranged from 0.01 to 2.61, 0.21 to 2.27, and 0.22 to 0.99 per 100,000 for PV, ET, and PMF, respectively. The combined annual incidence rates for PV, ET, and PMF were 0.84, 1.03, and 0.47 per 100,000. There was high heterogeneity across disease entities (I(2) 97.1-99.8%) and evidence of publication bias for ET and PMF (Egger test, P = 50.007 and P ≤ 0.001, respectively).The pooled incidence reflects the rarity of MPNs. The calculated pooled incidence rates do not reflect MPN incidence across the globe due to the high unexplained heterogeneity. Improved, widespread registration of MPNs would provide better information for global comparison of the incidence and prevalence of MPNs.

Three-port DC-DC Converter for Stand-Alone Photovoltaic Systems

System efficiency and cost effectiveness are of critical importance for photovoltaic (PV) systems. This paper addresses the two issues by developing a novel three-port DC-DC converter for stand-alone PV systems, based on an improved Flyback-Forward topology. It provides a compact single-unit solution with a combined feature of optimized maximum power point tracking (MPPT), high step-up ratio, galvanic isolation and multiple operating modes for domestic and aerospace applications. A theoretical analysis is conducted to analyze the operating modes followed by simulation and experimental work. The paper is focused on a comprehensive modulation strategy utilizing both PWM and phase-shifted control that satisfies the requirement of PV power systems to achieve MPPT and output voltage regulation. A 250 W converter was designed and prototyped to provide experimental verification in term of system integration and high conversion efficiency.

New modular structure DC-DC converter without electrolytic capacitors for renewable energy applications

New multi-stage DC-DC converters for grid-connected photovoltaic systems

Renewable energy is high on international and national agendas. Currently, grid-connected photovoltaic (PV) systems are a popular technology to convert solar energy into electricity. Existing PV panels have a relatively low and varying output voltage so that the converter installed between the PVs and the grid should be equipped with high step-up and versatile control capabilities. In addition, the output current of PV systems is rich in harmonics which affect the power quality of the grid. In this paper, a new multi-stage hysteresis control of a step-up DC-DC converter is proposed for integrating PVs into a single-phase power grid. The proposed circuitry and control method is experimentally validated by testing on a 600W prototype converter. The developed technology has significant economic implications and could be applied to many distributed generation (DG) systems, especially for the developing countries which have a large number of small PVs connected to their single-phase distribution network. 

Spitzer observations of the asteroid-comet transition object and potential spacecraft target 107P (4015) Wilson-Harrington

Context: Near-Earth asteroid-comet transition object 107P/ (4015) Wilson-Harrington is a possible target of the joint European Space Agency (ESA) and Japanese Aerospace Exploration Agency (JAXA) Marco Polo sample return mission. Physical studies of this object are relevant to this mission, and also to understanding its asteroidal or cometary nature. Aims: Our aim is to obtain significant new constraints on the surface thermal properties of this object. Methods: We present mid-infrared photometry in two filters (16 and 22 μm) obtained with NASA's Spitzer Space Telescope on February 12, 2007, and results from the application of the Near Earth Asteroid Thermal Model (NEATM). We obtained high S/N in two mid-IR bands allowing accurate measurements of its thermal emission. Results: We obtain a well constrained beaming parameter (η = 1.39±0.26) and obtain a diameter and geometric albedo of D = 3.46±0.32 km, and pV = 0.059±0.011. We also obtain similar results when we apply this best-fitting thermal model to single-band mid-IR photometry reported by Campins et al. (1995, P&SS, 43, 733), Kraemer et al. (2005, AJ, 130, 2363) and Reach et al. (2007, Icarus, 191, 298). Conclusions: The albedo of 4015 Wilson-Harrington is low, consistent with those of comet nuclei and primitive C-, P-, D-type asteorids. We establish a rough lower limit for the thermal inertia of W-H of 60 Jm-2s-0.5 K-1 when it is at r = 1 AU, which is slightly over the limit of 30 Jm-2 s-0.5 K-1 derived by Groussin et al. (2009, Icarus, 199, 568) for the thermal inertia of the nucleus of comet 22P/Kopff.

Net energy analysis of a solar combi system with Seasonal Thermal Energy Store

EU targets require nearly zero energy buildings (NZEB) by 2020. However few monitored examples exist of how NZEB has been achieved in practise in individual residential buildings. This paper provides an example of how a low-energy building (built in 2006), has achieved nearly zero energy heating through the addition of a solar domestic hot water and space heating system (“combi system”) with a Seasonal Thermal Energy Store (STES). The paper also presents a cumulative life cycle energy and cumulative life cycle carbon analysis for the installation based on the recorded DHW and space heating demand in addition to energy payback periods and net energy ratios. In addition, the carbon and energy analysis is carried out for four other heating system scenarios including hybrid solar thermal/PV systems in order to obtain the optimal system from a carbon efficiency perspective.

Managing Future offshore wind power variability in a European Supergrid

The future European power system will have a hierarchical structure created by layers of system control from a Supergrid via regional high-voltage transmission through to medium and low-voltage distribution. Each level will have generation sources such as large-scale offshore wind, wave, solar thermal, nuclear directly connected to this Supergrid and high levels of embedded generation, connected to the medium-voltage distribution system. It is expected that the fuel portfolio will be dominated by offshore wind in Northern Europe and PV in Southern Europe. The strategies required to manage the coordination of supply-side variability with demand-side variability will include large scale interconnection, demand side management, load aggregation and storage in the context of the Supergrid combined with the Smart Grid. The design challenge associated with this will not only include control topology, data acquisition, analysis and communications technologies, but also the selection of fuel portfolio at a macro level. This paper quantifies the amount of demand side management, storage and so-called 'back-up generation' needed to support an 80% renewable energy portfolio in Europe by 2050. © 2013 IEEE.

Managing the variability of supply in the European power system with high wind energy penetration

The power system of the future will have a hierarchical structure created by layers of system control from via regional high-voltage transmission through to medium and low-voltage distribution. Each level will have generation sources such as large-scale offshore wind, wave, solar thermal, nuclear directly connected to this Supergrid and high levels of embedded generation, connected to the medium-voltage distribution system. It is expected that the fuel portfolio will be dominated by offshore wind in Northern Europe and PV in Southern Europe. The strategies required to manage the coordination of supply-side variability with demand-side variability will include large scale interconnection, demand side management, load aggregation and storage in the concept of the Supergrid combined with the Smart Grid. The design challenge associated with this will not only include control topology, data acquisition, analysis and communications technologies, but also the selection of fuel portfolio at a macro level. This paper quantifies the amount of demand side management, storage and so-called ‘back-up generation’ needed to support an 80% renewable energy portfolio in Europe by 2050.

Myeloproliferative neoplasm patient symptom burden and quality of life: evidence of significant impairment compared to controls

The myeloproliferative neoplasms (MPN) including polycythaemia vera (PV), essential thrombocythaemia and primary myelofibrosis (PMF) are rare diseases contributing to significant morbidity. Symptom management is a prime treatment objective but current symptom assessment tools have not been validated compared to the general population. The MPN-symptom assessment form (MPN-SAF), a reliable and validated clinical tool to assess MPN symptom burden, was administered to MPN patients (n = 106) and, for the first time, population controls (n = 124) as part of a UK case–control study. Mean symptom scores were compared between patients and controls adjusting for potential confounders. Mean patient scores were compared to data collected by the Mayo Clinic, USA on 1,446 international MPN patients to determine patient group representativeness. MPN patients had significantly higher mean scores than controls for 25 of the 26 symptoms measured (P < 0.05); fatigue was the most common symptom (92.4% and 78.1%, respectively). Female MPN patients suffered worse symptom burden than male patients (P < 0.001) and substantially worse burden than female controls (P < 0.001). Compared to the Mayo clinic patients, MPN-UK patients reported similar symptom burden but lower satiety (P = 0.046). Patients with PMF reported the worst symptom burden (88.3%); significantly higher than PV patients (P < 0.001). For the first time we report quality of life was worse in MPN-UK patients compared with controls (P < 0.001).

Topographical optimisation of single-storey non-domestic steel framed buildings using photovoltaic panels for net-zero carbon impact

A methodology is presented that combines a multi-objective evolutionary algorithm and artificial neural networks to optimise single-storey steel commercial buildings for net-zero carbon impact. Both symmetric and asymmetric geometries are considered in conjunction with regulated, unregulated and embodied carbon. Offsetting is achieved through photovoltaic (PV) panels integrated into the roof. Asymmetric geometries can increase the south facing surface area and consequently allow for improved PV energy production. An exemplar carbon and energy breakdown of a retail unit located in Belfast UK with a south facing PV roof is considered. It was found in most cases that regulated energy offsetting can be achieved with symmetric geometries. However, asymmetric geometries were necessary to account for the unregulated and embodied carbon. For buildings where the volume is large due to high eaves, carbon offsetting became increasingly more difficult, and not possible in certain cases. The use of asymmetric geometries was found to allow for lower embodied energy structures with similar carbon performance to symmetrical structures.

Investigating Cyber-Physical Attacks against IEC 61850 Photovoltaic Inverter Installations

Cyber-attacks against Smart Grids have been found in the real world. Malware such as Havex and BlackEnergy have been found targeting industrial control systems (ICS) and researchers have shown that cyber-attacks can exploit vulnerabilities in widely used Smart Grid communication standards. This paper addresses a deep investigation of attacks against the manufacturing message specification of IEC 61850, which is expected to become one of the most widely used communication services in Smart Grids. We investigate how an attacker can build a custom tool to execute man-in-the-middle attacks, manipulate data, and affect the physical system. Attack capabilities are demonstrated based on NESCOR scenarios to make it possible to thoroughly test these scenarios in a real system. The goal is to help understand the potential for such attacks, and to aid the development and testing of cyber security solutions. An attack use-case is presented that focuses on the standard for power utility automation, IEC 61850 in the context of inverter-based distributed energy resource devices; especially photovoltaic (PV) generators.