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X-linked hypophosphatemia (XLH) is the most common monogenetic cause of chronic hypophosphatemia, characterized by rickets and osteomalacia. Disease manifestations and treatment of XLH patients in the Netherlands are currently unknown. Characteristics of XLH patients participating in the Dutch observational registry for genetic hypophosphatemia and acquired renal phosphate wasting were analyzed. Eighty XLH patients, including 29 children, were included. Genetic testing, performed in 78.8% of patients, showed a PHEX mutation in 96.8%. Median (range) Z-score for height was − 2.5 (− 5.5; 1.0) in adults and − 1.4 (− 3.7; 1.0) in children. Many patients were overweight or obese: 64.3% of adults and 37.0% of children. All children received XLH-related medication e.g., active vitamin D, phosphate supplementation or burosumab, while 8 adults used no medication. Lower age at start of XLH-related treatment was associated with higher height at inclusion. Hearing loss was reported in 6.9% of children and 31.4% of adults. Knee deformities were observed in 75.0% of all patients and osteoarthritis in 51.0% of adult patients. Nephrocalcinosis was observed in 62.1% of children and 33.3% of adults. Earlier start of XLH-related treatment was associated with higher risk of nephrocalcinosis and detection at younger age. Hyperparathyroidism longer than six months was reported in 37.9% of children and 35.3% of adults. This nationwide study confirms the high prevalence of adiposity, hearing loss, bone deformities, osteoarthritis, nephrocalcinosis and hyperparathyroidism in Dutch XLH patients. Early start of XLH-related treatment appears to be beneficial for longitudinal growth but may increase development of nephrocalcinosis.

Magnetic imaging using nitrogen-vacancy (NV) spins in diamonds is a powerful technique for acquiring quantitative information about sub-micron scale magnetic order. A major challenge for its application in the research on two-dimensional (2D) magnets is the positioning of the NV centers at a well-defined, nanoscale distance to the target material required for detecting the small magnetic fields generated by magnetic monolayers. Here, we develop a diamond “dry-transfer” technique akin to the state-of-the-art 2D-materials assembly methods and use it to place a diamond micro-membrane in direct contact with the 2D interlayer antiferromagnet CrSBr. We harness the resulting NV-sample proximity to spatially resolve the magnetic stray fields generated by the CrSBr, present only where the CrSBr thickness changes by an odd number of layers. From the magnetic stray field of a single uncompensated ferromagnetic layer in the CrSBr, we extract a monolayer magnetization of M CSB = 0.46(2) T, without the need for exfoliation of monolayer crystals or applying large external magnetic fields. The ability to deterministically place NV-ensemble sensors into contact with target materials and detect ferromagnetic monolayer magnetizations paves the way for quantitative analysis of a wide range of 2D magnets assembled on arbitrary target substrates., QN/van der Zant Lab, QN/vanderSarlab, QN/Kavli Nanolab Delft

Superconductors are materials with zero electrical resistivity and the ability to expel magnetic fields, which is known as the Meissner effect. Their dissipationless diamagnetic response is central to magnetic levitation and circuits such as quantum interference devices. In this work, we used superconducting diamagnetism to shape the magnetic environment governing the transport of spin waves-collective spin excitations in magnets that are promising on-chip signal carriers-in a thin-film magnet. Using diamond-based magnetic imaging, we observed hybridized spin-wave-Meissner-current transport modes with strongly altered, temperature-tunable wavelengths and then demonstrated local control of spin-wave refraction using a focused laser. Our results demonstrate the versatility of superconductor-manipulated spin-wave transport and have potential applications in spin-wave gratings, filters, crystals, and cavities., Green Open Access added to TU Delft Institutional Repository 'You share, we take care!' - Taverne project https://www.openaccess.nl/en/you-share-we-take-care Otherwise as indicated in the copyright section: the publisher is the copyright holder of this work and the author uses the Dutch legislation to make this work public., QN/vanderSarlab, QN/Kavli Nanolab Delft, QN/Blanter Group

Nitrogen-vacancy (NV) magnetometry is a new technique for imaging spin waves in magnetic materials. It detects spin waves by their microwave magnetic stray fields, which decay evanescently on the scale of the spin-wavelength. Here, we use nanoscale control of a single-NV sensor as a wavelength filter to characterize frequency-degenerate spin waves excited by a microstrip in a thin-film magnetic insulator. With the NV probe in contact with the magnet, we observe an incoherent mixture of thermal and microwave-driven spin waves. By retracting the tip, we progressively suppress the small-wavelength modes until a single coherent mode emerges from the mixture. In-contact scans at low drive power surprisingly show occupation of the entire isofrequency contour of the two-dimensional spin-wave dispersion despite our one-dimensional microstrip geometry. Our distance-tunable filter sheds light on the spin-wave band occupation under microwave excitation and opens opportunities for imaging magnon condensates and other coherent spin-wave modes., QN/vanderSarlab, QN/Quantum Nanoscience, QN/Afdelingsbureau

Transition metal dichalcogenide (TMD) monolayers are two-dimensional semiconductors with two valleys in their band structure that can be selectively addressed using circularly polarized light. Their photoluminescence spectrum is characterized by neutral and charged excitons (trions) that form a chemical equilibrium governed by the net charge density. Here, we use chemical doping to drive the conversion of excitons into trions in WS 2 monolayers at room temperature, and study the resulting valley polarization via photoluminescence measurements under valley-selective optical excitation. We show that the doping causes the emission to become dominated by trions with a strong valley polarization associated with rapid non-radiative recombination. Simultaneously, the doping results in strongly quenched but highly valley-polarized exciton emission due to the enhanced conversion into trions. A rate equation model explains the observed valley polarization in terms of the doping-controlled exciton-trion equilibrium. Our results shed light on the important role of exciton-trion conversion on valley polarization in monolayer TMDs., QN/vanderSarlab

Transition metal dichalcogenide (TMD) monolayers are two-dimensional semiconductors with two valleys in their band structure that can be selectively addressed using circularly polarized light. Their photoluminescence spectrum is characterized by neutral and charged excitons (trions) that form a chemical equilibrium governed by the net charge density. Here, we use chemical doping to drive the conversion of excitons into trions in WS 2 monolayers at room temperature, and study the resulting valley polarization via photoluminescence measurements under valley-selective optical excitation. We show that the doping causes the emission to become dominated by trions with a strong valley polarization associated with rapid non-radiative recombination. Simultaneously, the doping results in strongly quenched but highly valley-polarized exciton emission due to the enhanced conversion into trions. A rate equation model explains the observed valley polarization in terms of the doping-controlled exciton-trion equilibrium. Our results shed light on the important role of exciton-trion conversion on valley polarization in monolayer TMDs., QN/vanderSarlab

Contains fulltext : 215593.pdf (publisher's version ) (Closed access), Although blood pressure control is a major goal in chronic kidney disease, no worldwide overview of either its achievement or antihypertensive prescriptions is currently available. To evaluate this we compared crude prevalence of uncontrolled blood pressure among 17 cohort studies, including 34 602 individuals with estimated glomerular filtration rate under 60 ml/min/1.73 m(2) and treated hypertension across four continents, and estimated observed to expected prevalence ratios, adjusted for potential confounders. Crude prevalence of blood pressure of 140/90 mm Hg or more varied from 28% to 61% and of blood pressure of 130/80 or more from 54% to 84%. Adjusted prevalence ratios indicated poorer hypertension control than expected in cohorts from European countries, India, and Uruguay, and better control in patients from North American and high-income Asian countries. Four antihypertensive drug classes or more were prescribed to more than 30% of participants in North American and some European cohorts, but this practice was less common elsewhere. Renin angiotensin-aldosterone system inhibitors were the most common antihypertensive drugs, prescribed for 54% to 91% of cohort participants. Differences for other drug classes were much stronger, ranging from 11% to 79% for diuretics, 22% to 70% for beta-blockers, and 27% to 75% for calcium-channel blockers. The confounders studied explain only a part of the international variation in blood pressure control among individuals with chronic kidney disease. Thus, considerable heterogeneity in prescription patterns worldwide calls for further investigation into the impact of different approaches on patient outcomes.

Contains fulltext : 215593.pdf (publisher's version ) (Closed access), Although blood pressure control is a major goal in chronic kidney disease, no worldwide overview of either its achievement or antihypertensive prescriptions is currently available. To evaluate this we compared crude prevalence of uncontrolled blood pressure among 17 cohort studies, including 34 602 individuals with estimated glomerular filtration rate under 60 ml/min/1.73 m(2) and treated hypertension across four continents, and estimated observed to expected prevalence ratios, adjusted for potential confounders. Crude prevalence of blood pressure of 140/90 mm Hg or more varied from 28% to 61% and of blood pressure of 130/80 or more from 54% to 84%. Adjusted prevalence ratios indicated poorer hypertension control than expected in cohorts from European countries, India, and Uruguay, and better control in patients from North American and high-income Asian countries. Four antihypertensive drug classes or more were prescribed to more than 30% of participants in North American and some European cohorts, but this practice was less common elsewhere. Renin angiotensin-aldosterone system inhibitors were the most common antihypertensive drugs, prescribed for 54% to 91% of cohort participants. Differences for other drug classes were much stronger, ranging from 11% to 79% for diuretics, 22% to 70% for beta-blockers, and 27% to 75% for calcium-channel blockers. The confounders studied explain only a part of the international variation in blood pressure control among individuals with chronic kidney disease. Thus, considerable heterogeneity in prescription patterns worldwide calls for further investigation into the impact of different approaches on patient outcomes.

Contains fulltext : 215593.pdf (publisher's version ) (Closed access), Although blood pressure control is a major goal in chronic kidney disease, no worldwide overview of either its achievement or antihypertensive prescriptions is currently available. To evaluate this we compared crude prevalence of uncontrolled blood pressure among 17 cohort studies, including 34 602 individuals with estimated glomerular filtration rate under 60 ml/min/1.73 m(2) and treated hypertension across four continents, and estimated observed to expected prevalence ratios, adjusted for potential confounders. Crude prevalence of blood pressure of 140/90 mm Hg or more varied from 28% to 61% and of blood pressure of 130/80 or more from 54% to 84%. Adjusted prevalence ratios indicated poorer hypertension control than expected in cohorts from European countries, India, and Uruguay, and better control in patients from North American and high-income Asian countries. Four antihypertensive drug classes or more were prescribed to more than 30% of participants in North American and some European cohorts, but this practice was less common elsewhere. Renin angiotensin-aldosterone system inhibitors were the most common antihypertensive drugs, prescribed for 54% to 91% of cohort participants. Differences for other drug classes were much stronger, ranging from 11% to 79% for diuretics, 22% to 70% for beta-blockers, and 27% to 75% for calcium-channel blockers. The confounders studied explain only a part of the international variation in blood pressure control among individuals with chronic kidney disease. Thus, considerable heterogeneity in prescription patterns worldwide calls for further investigation into the impact of different approaches on patient outcomes.

This document replaces the DGP recommendations published in 1998 and 2013. Based on recent studies and a consensus conference, the indications, choice and performance of the adequate exercise testing method and its necessary technical and staffing setting are discussed. Detailed recommendations are provided: for blood gas analysis and right heart catheterization during exercise, walk tests, spiroergometry, and stress echocardiography. The correct use of different exercise tests is discussed for specific situations in respiratory medicine: exercise induced asthma, obesity, monitoring of rehabilitation or therapeutical interventions, preoperative risk stratification, and evaluation in occupational medicine.

This document replaces the DGP recommendations published in 1998 and 2013. Based on recent studies and a consensus conference, the indications, choice and performance of the adequate exercise testing method and its necessary technical and staffing setting are discussed. Detailed recommendations are provided: for blood gas analysis and right heart catheterization during exercise, walk tests, spiroergometry, and stress echocardiography. The correct use of different exercise tests is discussed for specific situations in respiratory medicine: exercise induced asthma, obesity, monitoring of rehabilitation or therapeutical interventions, preoperative risk stratification, and evaluation in occupational medicine.

The development of large commercial aircraft such as the Airbus A350 and the Boeing 787, together with military aircraft such as the Lockheed Martin F35, with a large share of composite components of up to 50%, make it necessary for attention to be focussed on the development of new and effective maintenance strategies. In this paper, we address the development of ultrasonic measurement techniques, with a focus on structural health monitoring (SHM) techniques that are able to quickly inspect large structural parts of the aircraft. The combination of real-time SHM techniques, together with the parallel development of automated repair techniques for bonded structures, will make it possible to operate the aircraft at lower costs and for longer periods of time, thereby increasing the economic life of these aircraft structures., Aerospace Structures and Materials, Aerospace Engineering

The development of large commercial aircraft such as the Airbus A350 and the Boeing 787, together with military aircraft such as the Lockheed Martin F35, with a large share of composite components of up to 50%, make it necessary for attention to be focussed on the development of new and effective maintenance strategies. In this paper, we address the development of ultrasonic measurement techniques, with a focus on structural health monitoring (SHM) techniques that are able to quickly inspect large structural parts of the aircraft. The combination of real-time SHM techniques, together with the parallel development of automated repair techniques for bonded structures, will make it possible to operate the aircraft at lower costs and for longer periods of time, thereby increasing the economic life of these aircraft structures., Aerospace Structures and Materials, Aerospace Engineering

This report describes the second experiment in a program of research designed to identify characteristics of computer software which are related to its psychological complexity. Thirty-six experienced programmers were given unlimited time to make specified modifications to a preliminary program and three experimental programs. The correctness of the modification and the time required to make each modification served as dependent variables. Results indicated that the difficulty of the modification was significantly related to the time to solution. This relationship was described by a hyperbolic function relating time to the number of statements to be inserted in the code. Modest effects on the score and time were observed for order presentation, suggesting a learning effect. On two of the three programs studied, better modifications were made when the control flow of the original programs was well-structured. No performance effects were related to the absence of comments or the type of comments (in-line versus global) used. Moderate relationships with the criteria were observed for several complexity metrics, and these were strongest where metrics were obtained from the modified code rather than the original programs.

This report describes the first experiment in a program of research designed to identify characteristics of computer software which are related to its psychological complexity. This experiment evaluated the effect of three independent variables (mnemonic variable names, complexity of control flow, and general type of program) on a programmer's understanding of a computer program. The contributions of several variables, including Halstead's software science metric and McCabe's complexity metric, to the prediction of program understanding were also evaluated. In a pilot study significant results were achieved with materials and procedures employed here. Thirty-six experienced programmers were instructed to study a computer program for 20 minutes, and were then given 25 minutes to reconstruct a functionally equivalent one. Performance was measured by the percentage of functionally correct statements recalled. Results indicated that complexity of control flow affected program understanding, while no relationship was found for mnemonic variable names and general program type. The metrics of both Halstead and McCabe were related to program understanding when differences between subjects and specific programs were taken into consideration.

RIVM rapport:Bij het onderzoek in de psychofarmacologie kan kennis van de effecten van stoffen op de omzettingssnelheid van neurotransmitters een belangrijk aspect zijn. Met de huidige psychofarmaca lijken vooral de klassieke neurotransmitters zoals de monoaminen, noradrenaline, dopamine en serotonine van belang. In dit rapport worden enkele methoden beschreven voor de analyse van de monoaminen in hersenweefsel van de rat. De bepalingen van noradrenaline, dopamine en de metaboliet hiervan, DOPAC zijn vernieuwde methoden terwijl voor serotonine en de metaboliet hiervan 5-HIAA, geheel nieuwe methoden binnen de afdeling opgezet zijn. HVA (homovanilline zuur), een andere metaboliet van dopamine bleek niet voldoende nauwkeurig bepaald te kunnen worden. De overige methoden zijn geschikt om voor het lopend onderzoek op de afdeling ingezet te kunnen worden.

RIVM rapport:Bij het onderzoek in de psychofarmacologie kan kennis van de effecten van stoffen op de omzettingssnelheid van neurotransmitters een belangrijk aspect zijn. Met de huidige psychofarmaca lijken vooral de klassieke neurotransmitters zoals de monoaminen, noradrenaline, dopamine en serotonine van belang. In dit rapport worden enkele methoden beschreven voor de analyse van de monoaminen in hersenweefsel van de rat. De bepalingen van noradrenaline, dopamine en de metaboliet hiervan, DOPAC zijn vernieuwde methoden terwijl voor serotonine en de metaboliet hiervan 5-HIAA, geheel nieuwe methoden binnen de afdeling opgezet zijn. HVA (homovanilline zuur), een andere metaboliet van dopamine bleek niet voldoende nauwkeurig bepaald te kunnen worden. De overige methoden zijn geschikt om voor het lopend onderzoek op de afdeling ingezet te kunnen worden.

RIVM rapport:Bij het onderzoek in de psychofarmacologie kan kennis van de effecten van stoffen op de omzettingssnelheid van neurotransmitters een belangrijk aspect zijn. Met de huidige psychofarmaca lijken vooral de klassieke neurotransmitters zoals de monoaminen, noradrenaline, dopamine en serotonine van belang. In dit rapport worden enkele methoden beschreven voor de analyse van de monoaminen in hersenweefsel van de rat. De bepalingen van noradrenaline, dopamine en de metaboliet hiervan, DOPAC zijn vernieuwde methoden terwijl voor serotonine en de metaboliet hiervan 5-HIAA, geheel nieuwe methoden binnen de afdeling opgezet zijn. HVA (homovanilline zuur), een andere metaboliet van dopamine bleek niet voldoende nauwkeurig bepaald te kunnen worden. De overige methoden zijn geschikt om voor het lopend onderzoek op de afdeling ingezet te kunnen worden.