Market Monitor: Development of the Wholesale Electricity Market in 2006

Competition in the Dutch electricity market is stagnating. The market remains concentrated, with relatively high prices. The north-west European market must be further integrated by expanding the available interconnection capacity in order to achieve a structural improvement. That could cut consumers’ annual energy bills by several dozen euros per household. Consumers would also benefit indirectly since electricity prices for business would also fall, putting downward pressure on prices of other products. In order to achieve these benefits for consumers, the TSO’s must make headway with the expansion of the available interconnection capacity.Monitoring, electricity, competition, infrastructure

The influence of first and second language on the acquisition of pragmatic markers in Spanish : Evidence from an experimental study

This paper reports on an experimental study of the use of two Spanish markers of epistemic modality and evidentiality, creo que 'I believe that' and pienso que 'I think that', by native speakers, and by Dutch and German learners of Spanish. We found a clear preference for creo que among the native speakers of Spanish, but with differences between the main varieties of the language. For Dutch and German learners the preference for creo que was significantly weaker, and for beginning learners of Spanish it was significantly weaker than for advanced learners. While for the Dutch learners of Spanish this pattern reflects the preference for I think over I believe in their L1, the preference that the German-speaking learners have for creo que can be seen as evidence for a general tendency noticed in the literature, namely the interference of an L2 (in this case English) instead of the L1 (in this case German) in L3 performance (in this case Spanish)

Long-Term Performance of Epicardial versus Transvenous Left Ventricular Leads for Cardiac Resynchronization Therapy

Aims: to study the technical performance of epicardial left ventricular (LV) leads placed via video assisted thoracic surgery (VATS), compared to transvenously placed leads for cardiac resynchronization therapy (CRT).Methods: From 2001 until 2013, a total of 644 lead placement procedures were performed for CRT. In the case of unsuccessful transvenous LV lead placement, the patient received an epicardial LV lead. Study groups consist of 578 patients with a transvenous LV lead and 66 with an epicardial LV lead. The primary endpoint was LV-lead failure necessitating a replacement or deactivation. The secondary endpoint was energy consumption.Results: The mean follow up was 5.9 years (epicardial: 5.5 ± 3.1, transvenous: 5.9 ± 3.5). Transvenous leads failed significantly more frequently than epicardial leads with a total of 66 (11%) in the transvenous leads group vs. 2 (3%) in the epicardial lead group ( p = 0.037). Lead energy consumption was not significantly different between groups.Conclusions: Epicardial lead placement is feasible, safe and shows excellent long-term performance compared to transvenous leads. Epicardial lead placement should be considered when primary transvenous lead placement fails or as a primary lead placement strategy in challenging cases. </p

Obesity is associated with impaired long-term success of pulmonary vein isolation:A plea for risk factor management before ablation

Aims: Obesity is an increasing health problem and is an important risk factor for the development of atrial fibrillation (AF). We investigated the association of body mass index (BMI) on the safety and long-term efficacy of pulmonary vein isolation (PVI) for drug-refractory AF. Methods: 414 consecutive patients who underwent transcatheter PVI for AF between 2003 and 2013 were included. Successful PVI was defined as absence of atrial arrhythmia on Holter monitoring or ECG, without and with antiarrhythmic drugs during follow-up. Obesity was defined as BMI≥30 kg/m². Results: Mean age was 56±10 years, 316 (76%) were male, 311 (75%) had paroxysmal AF and 111 (27%) were obese. After a mean follow-up of 46±32 months (1590 patient-years), freedom from atrial arrhythmia and antiarrhythmic drugs was significantly lower in patients with obesity compared with non-obese patients (30% vs 46%, respectively, P=0.005, log-rank 0.016). With antiarrhythmic drugs, freedom from atrial arrhythmia was 56% vs 68% (P=0.036). No differences in minor and major adverse events were observed between patients with obesity and non-obese patients (major 6% vs 3%, P=0.105, and minor 5% vs 5%, P=0.512). Sensitivity analyses demonstrated that BMI (as continuous variable) was associated with PVI outcome (HR 1.08, 95% CI 1.02 to 1.14, P=0.012). Conclusion: Obesity is associated with reduced efficacy of PVI for drug-refractory AF. No relation between obesity and adverse events was found

The genetic basis of apparently idiopathic ventricular fibrillation:A retrospective overview

Aims: During the diagnostic work-up of patients with idiopathic ventricular fibrillation (VF), next-generation sequencing panels can be considered to identify genotypes associated with arrhythmias. However, consensus for gene panel testing is still lacking, and variants of uncertain significance (VUS) are often identified. The aim of this study was to evaluate genetic testing and its results in idiopathic VF patients. Methods and results: We investigated 419 patients with available medical records from the Dutch Idiopathic VF Registry. Genetic testing was performed in 379 (91%) patients [median age at event 39 years (27-51), 60% male]. Single-gene testing was performed in 87 patients (23%) and was initiated more often in patients with idiopathic VF before 2010. Panel testing was performed in 292 patients (77%). The majority of causal (likely) pathogenic variants (LP/P, n = 56, 15%) entailed the DPP6 risk haplotype (n = 39, 70%). Moreover, 10 LP/P variants were found in cardiomyopathy genes (FLNC, MYL2, MYH7, PLN (two), TTN (four), RBM20), and 7 LP/P variants were identified in genes associated with cardiac arrhythmias (KCNQ1, SCN5A (2), RYR2 (four)). For eight patients (2%), identification of an LP/P variant resulted in a change of diagnosis. In 113 patients (30%), a VUS was identified. Broad panel testing resulted in a higher incidence of VUS in comparison to single-gene testing (38% vs. 3%, P &lt; 0.001). Conclusion: Almost all patients from the registry underwent, albeit not broad, genetic testing. The genetic yield of causal LP/P variants in idiopathic VF patients is 5%, increasing to 15% when including DPP6. In specific cases, the LP/P variant is the underlying diagnosis. A gene panel specifically for idiopathic VF patients is proposed.</p

Early mobilisation versus plaster immobilisation of simple elbow dislocations: Results of the FuncSiE multicentre randomised clinical trial

Background/aim To compare outcome of early mobilisation and plaster immobilisation in patients with a simple elbow dislocation. We hypothesised that early mobilisation would result in earlier functional recovery. Methods From August 2009 to September 2012, 100 adult patients with a simple elbow dislocation were enrolled in this multicentre randomised controlled trial. Patients were randomised to early mobilisation (n=48) or 3 weeks plaster immobilisation (n=52). Primary outcome measure was the Quick Disabilities of the Arm, Shoulder, and Hand (Quick-DASH) score. Secondary outcomes were the Oxford Elbow Score, Mayo Elbow Performance Index, pain, range of motion, complications and activity resumption. Patients were followed for 1 year. Results Quick-DASH scores at 1 year were 4.0 (95% CI 0.9 to 7.1) points in the early mobilisation group versus 4.2 (95% CI 1.2 to 7.2) in the plaster immobilisation group. At 6 weeks, early mobilised patients reported less disability (Quick-DASH 12 (95% CI 9 to 15) points vs 19 (95% CI 16 to 22); p<0.05) and had a larger arc of flexion and extension (121° (95% CI 115° to 127°) vs 102° (95% CI 96° to 108°); p<0.05). Patients returned to work sooner after early mobilisation (10 vs 18 days; p=0.020). Complications occurred in 12 patients; this was unrelated to treatment. No recurrent dislocations occurred. Conclusions Early active mobilisation is a safe and effective treatment for simple elbow dislocations. Patients recovered faster and returned to work earlier without increasing the complication rate. No evidence was found supporting treatment benefit at 1 year

Functional treatment versus plaster for simple elbow dislocations (FuncSiE): a randomized trial

Background. Elbow dislocations can be classified as simple or complex. Simple dislocations are characterized by the absence of fractures, while complex dislocations are associated with fractures. After reduction of a simple dislocation, treatment options include immobilization in a static plaster for different periods of time or so-called functional treatment. Functional treatment is characterized by early active motion within the limits of pain with or without the use of a sling or hinged brace. Theoretically, functional treatment should prevent stiffness without introducing increased joint instability. The primary aim of this randomized controlled trial is to compare early functional treatment versus plaster immobilization following simple dislocations of the elbow. Methods/Design. The design of the study will be a multicenter randomized controlled trial of 100 patients who have sustained a simple elbow dislocation. After reduction of the dislocation, patients are randomized between a pressure bandage for 5-7 days and early functional treatment or a plaster in 90 degrees flexion, neutral position for pro-supination for a period of three weeks. In the functional group, treatment is started with early active motion within the limits of pain. Function, pain, and radiographic recovery will be evaluated at regular intervals over the subsequent 12 months. The primary outcome measure is the Quick Disabilities of the Arm, Shoulder, and Hand score. The secondary outcome measures are the Mayo Elbow Performance Index, Oxford elbow score, pain level at both sides, range of motion of the elbow joint at both sides, rate of secondary interventions and complication rates in both groups (secondary dislocation, instability, relaxation), health-related quality of life (Short-Form 36 and EuroQol-5D), radiographic appearance of the elbow joint (degenerative changes and heterotopic ossifications), costs, and cost-effectiveness. Discussion. The successful completion of this trial will provide evidence on the effectiveness of a functional treatment for the management of simple elbow dislocations. Trial Registration. The trial is registered at the Netherlands Trial Register (NTR2025)

Biallelic loss-of-function variants in PLD1 cause congenital right-sided cardiac valve defects and neonatal cardiomyopathy

Congenital heart disease is the most common type of birth defect, accounting for one-third of all congenital anomalies. Using whole-exome sequencing of 2718 patients with congenital heart disease and a search in GeneMatcher, we identified 30 patients from 21 unrelated families of different ancestries with biallelic phospholipase D1 (PLD1) variants who presented predominantly with congenital cardiac valve defects. We also associated recessive PLD1 variants with isolated neonatal cardiomyopathy. Furthermore, we established that p.I668F is a founder variant among Ashkenazi Jews (allele frequency of ~2%) and describe the phenotypic spectrum of PLD1-associated congenital heart defects. PLD1 missense variants were overrepresented in regions of the protein critical for catalytic activity, and, correspondingly, we observed a strong reduction in enzymatic activity for most of the mutant proteins in an enzymatic assay. Finally, we demonstrate that PLD1 inhibition decreased endothelial-mesenchymal transition, an established pivotal early step in valvulogenesis. In conclusion, our study provides a more detailed understanding of disease mechanisms and phenotypic expression associated with PLD1 loss of function

Characterization, Optimization and Design of the Sorbent System for a Continuous Direct Air Capture System

With the influx of the industrial revolution of the past centuries, the global energy demand has grown proportional to the global economical growth. Consequently, an enormous rise in greenhouse gas emissions has been observed, where the major contribution to global warming comes from the rising concentration of CO2. Therefore, the concept of capturing CO2 directly from the air (DAC) has gained world wide attention as it can reduce the carbon footprint of men kind.Zero Emission Fuels (ZEF), an inspiring start-up based in Delft, aims to develop a micro plant that utilizes the DAC concept to produce methanol as a fuel, using only the sun as its energy source. CO2 and H2O is captured directly from the atmosphere by the DAC unit which operates continuously with the help of an amine sorbent that flows through an absorption and stripping column. Previous research shows pure tetraethylenepentamine (TEPA) having great potential as a CO2 capturing sorbent. However, it has some limitations regarding slow absorption and desorption kinetics due to a high sorbent viscosity, that prevent ZEF from reaching their goal of capturing 825 grams of CO2 per DAC unit ineight hours of operation. The focus of this research is on the sorbent selection process of ZEF’s DAC system. Research has shown that adding a diluent to TEPA could potentially improve the performance of the ZEF DAC unit. Therefore, a total of nine diluents, DEG, PEG-200, PEG-400, PEG-600, selexol-250, selexol-500, glycerol, 1,4-butanediol, sulfolane, mixed in different ratio’s with TEPA, have been put to the test through the developed framework of sorbent selection.In the framework of sorbent selection all the sorbents are tested through four different experiments, keeping efficiency and costs in consideration. The Airfarm experiments are used to evaluate absorption capacity and viscosity of the sorbent at specific ambient conditions. The Vapor-Liquid-Equilibrium (VLE) experiments are utilized to evaluate the desorption characteristics of the sorbent from where the regeneration energy demand can be calculated with the help of a mathematical model. The re-pumping experiments are used to evaluate the absorption kinetics of the sorbent and lastly, the degradation experimentsare used to evaluate the sorbent life time performance. After the experimental procedure the sorbents can be judged on the key performance indicators regarding the ZEF DAC unit operated in a specific climate. It was concluded through the literature research and proved by an extensive experimental researchthat selexol, glycerol and sulfolane did not improve the characteristics of the sorbent. However, adding DEG, PEG or 1,4-butanediol to TEPA did have a profound positive effect on the overall performance of the DAC unit. Since the diluents reduce the sorbent viscosity, and therefore, increase the absorptionand desorption kinetics. Based on the experimental results and the design specifications set by ZEF, PEG-200 proved the most promising out of all diluents in the mixing ratio TEPA:PEG-200 2:5. This sorbent has been tested for two different climates; the dry Sahara climate and the more humid Mediterranean climate to see how the sorbent would have to be changed for different climates. It was concluded, for a more humid climate, it requires less diluent for the sorbent to stay within the 2 푃푎 ⋅ 푠 viscosity limit. The experimental results were included in a full DAC model to design the DAC unit utilizing the optimized sorbent for the two different climates considering the design specifications set by ZEF. Followinga sensitivity analysis an optimized conceptual DAC design was obtained for both climates. Where it was concluded that the optimized sorbent resulted in reduction in energy requirement for regeneration of the sorbent, where 1554 푘푊ℎ/푡표푛 of CO2 is needed for the Sahara climate and 1636 푘푊ℎ/푡표푛 of CO2 for the Mediterranean climate. Finally, a cost analysis has been performed regarding the operational and capital costs of the newly designed conceptual DAC units for both climates

Biochar Effect on Maize Yield and Soil Characteristics in Five Conservation Farming Sites in Zambia

-Biochar addition to agricultural soils can improve soil fertility, with the added bonus of climate change mitigation through carbon sequestration. Conservation farming (CF) is precision farming, often combining minimum tillage, crop rotation and residue retention. In the present farmer-led field trials carried out in Zambia, the use of a low dosage biochar combined with CF minimum tillage was tested as a way to increase crop yields. Using CF minimum tillage allows the biochar to be applied to the area where most of the plant roots are present and mirrors the fertilizer application in CF practices. The CF practice used comprised manually hoe-dug planting 10-L sized basins, where 10%–12% of the land was tilled. Pilot trials were performed with maize cob biochar and wood biochar on five soils with variable physical/chemical characteristics. At a dosage as low as 4 tons/ha, both biochars had a strong positive effect on maize yields in the coarse white aeolian sand of Kaoma, West-Zambia, with yields of 444% ± 114% (p = 0.06) and 352% ± 139% (p = 0.1) of the fertilized reference plots for maize and wood biochar, respectively. Thus for sandy acidic soils, CF and biochar amendment can be a promising combination for increasing harvest yield. Moderate but non-significant effects on yields were observed for maize and wood biochar in a red sandy clay loam ultisol east of Lusaka, central Zambia (University of Zambia, UNZA, site) with growth of 142% ± 42% (p > 0.2) and 131% ± 62% (p > 0.2) of fertilized reference plots, respectively. For three other soils (acidic and neutral clay loams and silty clay with variable cation exchange capacity, CEC), no significant effects on maize yields were observed (p > 0.2). In laboratory trials, 5% of the two biochars were added to the soil samples in order to study the effect of the biochar on physical and chemical soil characteristics. The large increase in crop yield in Kaoma soil was tentatively explained by a combination of an increased base saturation (from <50% to 60%–100%) and cation exchange capacity (CEC; from 2–3 to 5–9 cmol/kg) and increased plant-available water (from 17% to 21%) as well as water vapor uptake (70 mg/g on maize cob biochar at 50% relative humidity)