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Real-time measurements of the differences in inhaled and exhaled, unlabeled and fully deuterated acetone concentration levels, at rest and during exercise, have been conducted using proton transfer reaction mass spectrometry. A novel approach to continuously differentiate between the inhaled and exhaled breath acetone concentration signals is used. This leads to unprecedented fine grained data of inhaled and exhaled concentrations. The experimental results obtained are compared with those predicted using a simple three compartment model that theoretically describes the influence of inhaled concentrations on exhaled breath concentrations for volatile organic compounds with high blood:air partition coefficients, and hence is appropriate for acetone. An agreement between the predicted and observed concentrations is obtained. Our results highlight that the influence of the upper airways cannot be neglected for volatiles with high blood:air partition coefficients, i.e. highly water soluble volatiles.
Optoelectronic system based on photonic integrated circuits to miniaturize spectral domain OCT
(2023)
We present a miniaturized optical coherence tomography (OCT) setup based on photonic integrated circuits (PIC) for the 850 nm range. We designed a 512-channel arrayed waveguide grating (AWG) on a PIC for spectral domain OCT (SD-OCT) that is co-integrated with PIN-photodiodes and analog-to-digital-converters on one single chip. This image sensor is combined with all the necessary electronics to act as a camera. It is integrated into a fiber-based OCT system, achieving a sensitivity of >80dB and various samples are imaged. This optoelectronic system will allow building small and cost-effective OCT systems to monitor retinal diseases.
X-ray micro tomography of three-dimensional embroidered current collectors for lithium-ion batteries
(2016)
Greater specific energy densities in lithium-ion batteries can be achieved by using three-dimensional (3D) porous current collectors, which allow for greater areal mass loadings of the electroactive material. In this paper, we present the use of embroidered current collectors for the preparation of thick, pouch-type Li-ion batteries. Experiments were performed on LiFePO 4 (LFP) water-based slurries using styrene-butadiene rubber (SBR) as binder and sodium carboxymethyl cellulose (CMC) as thickener, and formulations of different rheological characteristics were investigated. The electrochemical performance (cyclic voltammetry, rate capability) and morphological characteristics of the LFP half-pouch cells (X-ray micro computed tomography and scanning electron microscopy) were compared between the formulations. An optimum electrode formulation was identified, and a mechanism is proposed to explain differences between the formulations. With the optimum electrode formulation, 350 µm casted electrodes with high mechanical stability were achieved. Electrodes exhibited 4–6 times greater areal mass loadings (4–6 mAh cm −2 ) and 50% greater electroactive material weight than with foils. In tests of half- and full-pouch embroidered cells, a 50% capacity utilization at 1C-rate and 11% at 2C-rate were observed, with a full recovery at C/5-rate. The cycling stability was also maintained over 55 cycles.
Coupling is one of the most frequently mentioned metric in software systems. However, to measure logical coupling between microservices, runtime information is needed or the availability of service-log files to analyze the calls between services is required. This work presents our emerging results, in which we propose a metric to statically calculate logical coupling between microservices based on commits to versioning systems. We performed an initial validation of the proposed metric with a dataset containing 145 open-source microservices projects. The results illustrate how logical coupling affects every system and increases overtime. However, we did not find a correlation between the number of commits or the number of developers and the introduction of logical coupling. In future, we investigate why, how, and when logical coupling is introduced in a system.
Modern portable electronic devices have seen component heat load increasing, while the space available for heat dissipation has decreased. This requires the thermal management system to be optimized to attain the high performance heat sink. Heat sinks plays a major role for dissipating heat in electronic devices. Phase change material (PCM) is used to enhance the heat dissipation in heat sink. This paper reports the results of an experimental investigation of the performance of Pin fin heat sinks filled with phase change materials for thermal management of electronic devices. The experimental set ups are prepared with the graphical programming language with Lab VIEW (Laboratory Virtual Instruments for Engineering Workbench. Three different types of Pin fin Heat sink with and without PCM are investigated based on different operational timings and the temperature is acquired with the help of Data Acquisition Card (DAQ). The results indicated that the inclusion of the PCM could stabilize the temperature for a longer period and reduce the heating rates and peak temperatures of heat sink with increasing the number of fins can enhance the thermal performance of electronic devices.
Gas hydrates are usually synthesized by bringing together a pressurized gas and liquid or solid water. In both cases, the transport of gas or water to the hydrate growth site is hindered once an initial film of hydrate has grown at the water–gas interface. A seemingly forgotten gas-phase technique overcomes this problem by slowly depositing water vapor on a cold surface in the presence of the pressurized guest gas. Despite being used for the synthesis of low-formation-pressure hydrates, it has not yet been tested for hydrates of CO 2 and CH 4 . Moreover, the potential of the technique for the study of hydrate decomposition has not been recognized yet. We employ two advanced implementations of the condensation technique to form hydrates of CO 2 and CH 4 and demonstrate the applicability of the process for the study of hydrate decomposition and the phenomenon of self-preservation. Our results show that CO 2 and CH 4 hydrate samples deposited on graphite at 261–265 K are almost pure hydrates with an ice fraction of less than 8%. Rapid depressurization experiments with thin deposits (approx. 330 mm thickness) of CO 2 hydrate on an aluminum surface at 265 K yield identical dissociation curves when the deposition is done at identical pressure. However, hydrates deposited at 1 MPa almost completely withstand decomposition after rapid depressurization to 0.1 MPa, while samples deposited at 2 MPa decompose 7 times faster. Therefore, this synthesis technique is not only applicable for the study of hydrate decomposition but can also be used for the controlled deposition of a super-preserved hydrate.
Signatures of the optical stark effect on entangled photon pairs from resonantly-pumped quantum dots
(2023)
Two-photon resonant excitation of the biexciton-exciton cascade in a quantum dot generates highly polarization-entangled photon pairs in a near-deterministic way. However, the ultimate level of achievable entanglement is still debated. Here, we observe the impact of the laser-induced ac-Stark effect on the quantum dot emission spectra and on entanglement. For increasing pulse-duration-to-lifetime ratios and pump powers, decreasing values of concurrence are recorded. Nonetheless, additional contributions are still required to fully account for the observed below-unity concurrence.
Hot water heat pumps are well suited for demand side management, as the heat pump market faced a rapid growth in the past years with the trend to decentralized domestic hot water use. Sales were accelerated through wants and needs of energy conservation, energy efficiency, and less restrictive rules regarding Legionella. While in literature the model predictive control potential for heat pumps is commonly shown in simulations, the share of experimental studies is relatively low. To this day, experimental studies considering solely domestic hot water use are not available. In this paper, the realistic achievable model predictive control potential of a hot water heat pump is compared to the standard hysteresis control, to provide an experimental proof. We show for the first time, how state-of-the-art approaches (model predictive control, system identification, live state estimation, and demand prediction) can be transferred from electric hot water heaters to hot water heat pumps, combined, and implemented into a real-world hot water heat pump setup. The optimization approach, embedded in a realistic experimental setting, leads to a decrease in electric energy demand and cost per unit electricity by approximately 12% and 14%, respectively. Further, an increase in efficiency by approximately 13% has been achieved.
Activation of heat pump flexibilities is a viable solution to support balancing the grid via Demand Side Management measures and fulfill the need for flexibility options. Aggregators as interface between prosumers, distribution system operators and balance responsible parties face the challenge due to data privacy and technical restrictions to transform prosumer information into aggregated available flexibility to enable trading thereof. Thereby, literature lacks a generic, applicable and widely accepted flexibility estimation method for heat pumps,which incorporates reduced sensor and system information, system- and demand-dependent behaviour. In this paper, we adapt and extend a method from literature, by incorporating domain knowledge to overcome reduced sensor and system information. We apply data of five real-world heat pump systems, distinguish operation modes, estimate power and energy flexibility of each single heat pump system, proof transferability of the method, and aggregate the flexibilities available to showcase a small HP pool as a proof of concept.
Statistische Methodenkompetenz wird im Controlling zunehmend bedeutender, so ist die evidenzbasierte Prognose durch algorithmische Analyse von Datenbeständen ein Schwerpunkt von Controlling Analytics. Die Fallstudie durchleuchtet daher anhand von Datenmaterial des Schweizer Bundesamts für Statistik das Vorgehen bei einer Datenanalyse, insbesondere beim Einsatz der Zeitregression für Prognosezwecke, und geht dabei auf methodische Besonderheiten, Caveats und Einsatzmöglichkeiten in Microsoft Excel ein. Die Fallbeschreibung und Aufgaben sind im WiSt-Heft Nr. 4/23 zu finden.
Controlling Analytics: Einsatz für Prognosen im Controlling - Teil 1: Fallbeschreibung und Aufgaben
(2023)
Statistische Methodenkompetenz wird im Controlling zunehmend bedeutender, so ist die evidenzbasierte Prognose durch algorithmische Analyse von Datenbeständen ein Schwerpunkt von Controlling Analytics. Die Fallstudie durchleuchtet daher anhand von Datenmaterial des Schweizer Bundesamts für Statistik das Vorgehen bei einer Datenanalyse, insbesondere beim Einsatz der Zeitregression für Prognosezwecke, und geht dabei auf methodische Besonderheiten, Caveats und Einsatzmöglichkeiten in Microsoft Excel ein.
Rückblicke zur 5. ogsaTAGUNG
(2021)
The dynamics of self-adaptive multi-recombinant evolution strategies on the general ellipsoid model
(2014)
Quasilineare Tauchankerspule
(2020)
Combining parallel pattern generation of electrohydrodynamic lithography with serial addressing
(2018)
For a given set of banks, how big can losses in bad economic or financial scenarios possibly get, and what are these bad scenarios? These are the two central questions of stress tests for banks and the banking system. Current stress tests select stress scenarios in a way which might leave aside many dangerous scenarios and thus create an illusion of safety; and which might consider highly implausible scenarios and thus trigger a false alarm. We show how to select scenarios systematically for a banking system in a context of multiple credit exposures. We demonstrate the application of our method in an example on the Spanish and Italian residential real estate exposures of European banks. Compared to the EBA 2016 stress test our method produces scenarios which are equally plausible as the EBA stress scenario but yield considerably worse system wide losses.
Recently the use of microRNAs (miRNAs) as biomarkers for a multitude of diseases has gained substantial significance for clinical as well as point-of-care diagnostics. Amongst other challenges, however, it holds the central requirement that the concentration of a given miRNA must be evaluated within the context of other factors in order to unambiguously diagnose one specific disease. In terms of the development of diagnostic methods and devices, this implies an inevitable demand for multiplexing in order to be able to gauge the abundance of several components of interest in a patient’s sample in parallel. In this study, we design and implement different multiplexed versions of our electrochemical microfluidic biosensor by dividing its channel into subsections, creating four novel chip designs for the amplification-free and simultaneous quantification of up to eight miRNAs on the CRISPR-Biosensor X (‘X’ highlighting the multiplexing aspect of the device). We then use a one-step model assay followed by amperometric readout in combination with a 2-minute-stop-flow-protocol to explore the fluidic and mechanical characteristics and limitations of the different versions of the device. The sensor showing the best performance, is subsequently used for the Cas13a-powered proof-of-concept measurement of two miRNAs (miRNA-19b and miRNA-20a) from the miRNA-17∼92 cluster, which is dysregulated in the blood of pediatric medulloblastoma patients. Quantification of the latter, alongside simultaneous negative control measurements are accomplished on the same device. We thereby confirm the applicability of our platform to the challenge of amplification-free, parallel detection of multiple nucleic acids.
The spatial redistribution of Japanese direct investment in the United Kingdom between 1991 and 2010
(2013)
The goal of this paper is to design a low-loss 1 x 32 Y-branch optical splitter for optical transmission systems, using two different design tools employing Beam Propagation Method. As a first step, a conventional 1 x 32 Y-branch splitter was designed and simulated in two-dimensional environment of OptiBPM photonic tool. The simulated optical properties feature high loss, high asymmetric splitting ratio and a large size of the designed structure, too. In the second step of this work we propose an optimization of the conventional splitter design leading to suppression of the asymmetric splitting ratio to one-third of its initial value and to the improvement of the losses by nearly 2 dB. In addition, 50% size reduction of the designed structure was also achieved. This length-optimized low-loss splitter was then modelled in a three-dimensional environment of RSoft photonic tool and the simulated results confirm the strong improvement of the optical properties.
Effects of hole-transport layer homogeneity in organic solar cells – A multi-length scale study
(2017)
A Telecom optical fibers are still being the best transmission medium of digital data and analogue signals for long distance applications. Progress in integrated photonics enables development of photonic chips with new unique properties, circuits of the future, and overcomes current limits in information and communication technologies. The packaging of photonic chips is necessary for taking them out of research laboratories into real implementation in the information and communication technology applications. One important step of packaging is effective coupling of optical radiation between telecom optical fiber with ten microns core dimension and photonic chip optical waveguide with submicron dimensions. For complex photonic chips, it is necessary to couple not one optical fiber but several optical fibers, which are arranged in fiber arrays. In this case, it is necessary to use a 6D positioning system, which allows to optimally adjust the relative position of the photonic chip and the fiber arrays. After setting the optimal relative position of the photonic chip and the fiber array, the process of their fixation follows. One possibility of fixation is gluing with an adhesive in the optical path between the photonic chip and an array of optical fibers with a refractive index close to the refractive index of the optical fiber core. This paper is focused on the experimental test set-up for the temperature characterization of fiber array to photonics chip butt coupling at 1310 nm and 1550 nm wavelengths fixed themselves by UV adhesive in the optical path. The main aims of this works are selection of better adhesive from two types for gluing of photonic chip and fiber array in packaging process of photonics chips and validation of gluing process developing. The coupling and alignment of fiber arrays to photonics chip were done by automated active alignments system and they were fixed themselves by curable epoxy adhesive. Temperature changes of coupling insertion losses are measured and investigated for two different UV adhesives during three temperature cycles from -40 °C to 80 °C in climatic chamber according to Telcordia. Spectral dependence of insertion losses were measured and compared before and after three temperature cycles for 1530 nm to 1570 nm spectral range at room temperature.
This work was supported by the Slovak Research and Development Agency under the contracts APVV-17-0662 and SK-AT-20-0017 and by the COST Action “European Network for High Performance Integrated Microwave Photonics” (EUIMWP) CA16220.
The paper deals with designing and numerical modelling a 2 x 2 optical switch for photonic integrated circuits based on 2 x 2 MMI elements and phase modulators. The 2 x 2 optical switch was modelled in the RsoftCAD with the simulation tool BeamPROP. The 2 x 2 optical switch is a common element for creating more complex 1 x N or N x N optical switches in all-optical signal processing.
Compression of ultrashort laser pulses via gated multiphoton intrapulse interference phase scans
(2014)
In this work, parametric excitation is introduced in a fully balanced flexible rotor mounted on two identical active gas foil bearings. The active gas foil bearings change the top foil shape harmonically with a specific amplitude and frequency. The deformable foil shape is approximated by an analytical function, while the gas pressure distribution is evaluated by the numerical solution of the Reynolds equation for compressible flow. The harmonic variation of the foil shape generates a respective variation in the bearings’ stiffness and damping properties and the system experiences parametric resonances and antiresonances in specific excitation frequencies. The nonlinear gas bearing forces generate bifurcations in the solutions of the system at certain rotating speeds and excitation frequencies; period doubling and Neimark-Sacker bifurcations are noticed in the examined system, and their progress is evaluated as the two bifurcation parameters (rotating speed and parametric excitation frequency) are changed, though a codimension-2 numerical continuation of limit cycles. It is found that at specific range of excitation frequency there are parametric anti-resonances and the bifurcations collide and vanish. Therefore, a bifurcation-free operating range is established and the system can operate stable at a wide speed range.
Transparent laser-structured glasses with superhydrophilic properties for anti-fogging applications
(2019)
Ultrashort pulse laser structuring enables direct modification of glass surfaces to generate superhydrophilic properties for anti-fogging applications. This approach makes coatings dispensable and the generated surfaces remain thermally, mechanically, and chemically resistant. However, the laser-generated structures usually cause scattering, which decreases transmission and may disturb the vision through the modified glass in the dry state. The aim of this study was to find a laser-processing strategy to achieve superhydrophilic, anti-fogging properties on glass surfaces with maximum transmission and minimal visual perception of the generated structure. For this purpose, we used an ultrashort-pulsed laser to generate periodic patterns of rippled circles or rough holes with varying pitch. The water contact angle and transmission of the structured glasses were measured as a function of the structured area. It was found that a periodic pattern of holes, which covers less than 1% of the surface, is already sufficient to reach the superhydrophilic state (contact angle < 5°) and provides nearly the same transmission as pristine glass. Pictures of objects imaged through dry, structured glasses, which were placed close to the lens or object, showed in both cases only a minimal decrease of contrast. If this minor drawback can be accepted, this direct laser structuring approach could be an interesting alternative to coating-based techniques and leaves even room to apply additional coatings for the fabrication of multi-functional special glasses.
Shaolin Wu Fo Dao – die Synthese japanischer und chinesischer Kampfkunst-Lehre auf der Grundlage zen-buddhistischer Theorie und Praxis ist eine neu geschaffene Disziplin. In dieser Fusion verbinden sich wesentliche Elemente aus Budo und Shaolin (-Wudao), deren traditionellen Körper-Geist-Übungen von Kampftechnik (Bu/Wu) und Meditation (Zen/Chan) als originäre Weg- und Heilslehre (Do/Dao) seit jeher der Persönlichkeits- und spirituellen Entwicklung des Ausübenden dienten.
Hier nun, in der Überwindung alter Grenzen zwischen japanischer und chinesischer Kampfkunstsysteme („hart“ und „weich“), wurde hier nun erstmaligen ein zusammengewachsenes Kampfkunstsystem kreiert, um die beidseits positiven Wirkungen auf den Menschen zu bündeln und – und das ist das Besondere – sie in den Dienst buddhistischer (Butsu, jap.: Buddha / chin. Fo) Psychologie und Psychotherapie zu stellen.
Die Verbindung von Budo mit Shaolin als eigenständiges praktisches Lehrkonzept, allein schon eine „revolutionäre Innovation“, erfährt durch ihren Einsatz als spezielle Methode einer Heilkunst zusätzlich Pioniercharakter in der Behandlung von Leid. Gemeint sind sowohl Ansätze aus der Budopädagogik und speziellen Budotherapie zur Heilung, Linderung oder Prävention von physischen, psychosomatischen und psychischen Krisen, Störungen, Krankheiten, Behinderungen und Leiden, als auch im Sinne des philosophischen und wissenschaftlichen Buddhismus, „das Leid in der Welt zu mindern“, zum Wohle des Menschen, für sein Glück und Frieden zu arbeiten.
Vast amounts of oily wastewater are byproducts of the petrochemical and the shipping industry and to this day frequently discharged into water bodies either without or after insufficient treatment. To alleviate the resulting pollution, water treatment processes are in great demand. Bubble column humidifiers (BCHs) as part of humidification–dehumidification systems are predestined for such a task, since they are insensitive to different feed liquids, simple in design and have low maintenance requirements. While humidification in a bubble column has been investigated plentiful for desalination, a systematic investigation of oily wastewater treatment is missing in literature. We filled this gap by analyzing the treatment of an oil–water emulsion experimentally to derive recommendations for future design and operation of BCHs. Our humidity measurements indicate that the air stream is always saturated after humidification for a liquid height of only 10 cm. A residual water mass fraction of 3.5 wt% is measured after a batch run of six hours. Furthermore, continuous measurements show that an increase in oil mass fraction leads to a decrease in system productivity especially for high oil mass fractions. This decrease is caused by the heterogeneity of the liquid temperature profile. A lower liquid height mitigates this heterogeneity, therefore decreasing the heat demand and improving the overall efficiency. The oil content of the produced condensate is below 15 ppm, allowing discharge into various water bodies. The results of our systematic investigation prove suitability and indicate a strong future potential for the use of BCHs in oily wastewater treatment.
Bubble columns are recently used for the humidification of air in water treatment systems and fuel cells. They are well applicable due to their excellent heat and mass transfer and their low technical complexity. To design and operate such devices with high efficiency, the humidification process and the impact of the operating parameters need to be understood to a sufficient degree. To extend this knowledge, we use a refined and novel method to determine the volumetric air–liquid heat and mass transfer coefficients and the humidifier efficiency for various parametric settings. The volumetric transfer coefficients increase with both of the superficial air velocity and the liquid temperature. It is further shown that the decrease of vapor pressure with an increase of the salinity results in a corresponding decrease in the outlet humidity ratio. In contrast to previous studies, liquid heights smaller than 0.1 m are investigated and significant changes in the humidifier efficiency are seen in this range. We present the expected humidifier efficiency with respect to the superficial air velocity and the liquid height in an efficiency chart, such that optimal operating conditions can be determined. Based on this efficiency chart, recommendations for industrial applications as well as future scientific challenges are drawn.
The humidification-dehumidification process (HDH) for desalination is a promising technology to address water scarcity issues in rural regions. However, a low humidifier efficiency is a weakness of the process. Bubble column humidifiers (BCH) are promising for HDH, as they provide enhanced heat and mass transfer and have low maintenance requirements. Previous studies of HDH-systems with BCHs draw different conclusions regarding the impact of superficial air velocity and liquid height on the humidification. Furthermore, the impact of flow characteristics has never been investigated systematically at all. In this study, an optimized BCH test setup that allows for optical analysis of the humidifier is used and evaluated. Our test setup is validated, since the influence of water temperature on the humidification, which is exponential, is reproduced. Measurements with seawater show that the normalised system productivity is increased by about 56 % with an increase in superficial air velocity from 0.5 to 5 cm/s. Furthermore, the system productivity is increased by around 29 % with an increase in liquid height from 60 to 378 mm. While the impact of superficial air velocity can be traced back to temperature changes at the humidifier and dehumidifier outlets, the impact of liquid height is shown to be caused by a smaller heat loss surface in the humidifier with an increase in liquid height. For the impact of sieve plate orifice diameter, a clear influence on the humidification is not apparent, this parameter needs to be investigated further. Finally, our new test setup allows for analysing the humidification of air (1) in a systematic way, (2) in relevant measurement ranges and (3) in comparison with optical analyses of the flow characteristics.
A step change is needed in the deployment of renewable energy if the triple challenge of ensuring climate change mitigation, energy security, and energy affordability is to be met. Yet, social acceptance of infrastructure projects and policies remains a key concern. While there has been decades of fruitful research on the social acceptance of wind energy and other renewables, much of the extant research is cross-sectional in nature, failing to capture the important dynamic processes that can make or break renewable energy projects. This paper introduces a Special Issue of Energy Policy which focuses on the neglected topic of the dynamics of social acceptance of renewable energy, drawing on contributions made at an international research conference held in St. Gallen (Switzerland) in June 2022. In addition to introducing these papers and drawing out common themes, we also seek to offer some conceptual clarity on the issue of dynamics in social acceptance, taking into account the influence of time, power, and scale in shaping decision-making processes. We conclude by highlighting a number of avenues of potential future research.
In engineering design, optimization methods are frequently used to improve the initial design of a product. However, the selection of an appropriate method is challenging since many
methods exist, especially for the case of simulation-based optimization. This paper proposes a systematic procedure to support this selection process. Building upon quality function deployment, end-user and design use case requirements can be systematically taken into account via a decision
matrix. The design and construction of the decision matrix are explained in detail. The proposed
procedure is validated by two engineering optimization problems arising within the design of box-type boom cranes. For each problem, the problem statement and the respectively applied optimization methods are explained in detail. The results obtained by optimization validate the use
of optimization approaches within the design process. The application of the decision matrix shows the successful incorporation of customer requirements to the algorithm selection.
Objectives: The MetabQoL 1.0 is the first disease-specific health related quality of life (HrQoL) questionnaire for patients with intoxication-type inherited metabolic disorders. Our aim was to assess the validity and reliability of the MetabQoL 1.0, and to investigate neuropsychiatric burden in our patient population. Methods: Data from 29 patients followed at a single center, aged between 8 and 18 years with the diagnosis of methylmalonic acidemia (MMA), propionic acidemia (PA) or isovaleric acidemia (IVA), and their parents were included. The Pediatric Quality of Life Inventory (PedsQoL) was used to evaluate the validity and reliability of MetabQoL 1.0.
Results: The MetabQoL 1.0 was shown to be valid and reliable (Cronbach's alpha: 0.64–0.9). Fourteen out of the 22 patients (63.6%) formally evaluated had neurological findings. Of note, 17 out of 20 patients (85%) had a psychiatric disorder when evaluated formally by a child and adolescent psychiatrist. The median mental scores of the MetabQoL 1.0 proxy report were significantly higher than those of the self report (p = 0.023). Patients with neonatal-onset disease had higher MetabQoL 1.0 proxy physical (p = 0.008), mental (p = 0.042), total scores (p = 0.022); and self report social (p = 0.007) and total scores (p = 0.043) than those with later onset disease.
Conclusions: This study continues to prove that the MetabQoL 1.0 is an effective tool to measure what matters in intoxication-type inherited metabolic disorders. Our results highlight the importance of clinical assessment complemented by patient reported outcomes which further expands the evaluation toolbox of inherited metabolic diseases.