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| 2. |
- Mercuri, E., et al.
(author)
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Safety and effectiveness of ataluren: comparison of results from the STRIDE Registry and CINRG DMD Natural History Study
- 2020
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In: Journal of Comparative Effectiveness Research. - : Becaris Publishing Limited. - 2042-6305 .- 2042-6313. ; 9:5, s. 341-360
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Journal article (peer-reviewed)abstract
- Aim: Strategic Targeting of Registries and International Database of Excellence (STRIDE) is an ongoing, multicenter registry providing real-world evidence regarding ataluren use in patients with nonsense mutation Duchenne muscular dystrophy (nmDMD). We examined the effectiveness of ataluren + standard of care (SoC) in the registry versus SoC alone in the Cooperative International Neuromuscular Research Group (CINRG) Duchenne Natural History Study (DNHS), DMD genotype-phenotype/-ataluren benefit correlations and ataluren safety. Patients & methods: Propensity score matching was performed to identify STRIDE and CINRG DNHS patients who were comparable in established disease progression predictors (registry cut-off date, 9 July 2018). Results & conclusion: Kaplan-Meier analyses demonstrated that ataluren + SoC significantly delayed age at loss of ambulation and age at worsening performance in timed function tests versus SoC alone (p <= 0.05). There were no DMD genotype-phenotype/ataluren benefit correlations. Ataluren was well tolerated. These results indicate that ataluren + SoC delays functional milestones of DMD progression in patients with nmDMD in routine clinical practice. ClinicalTrials.gov identifier: NCT02369731. ClinicalTrials.gov identifier: NCT02369731.
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| 7. |
- Gryzlova, E. V., et al.
(author)
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Influence of an atomic resonance on the coherent control of the photoionization process
- 2022
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In: Physical Review Research. - 2643-1564. ; 4:3
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Journal article (peer-reviewed)abstract
- In coherent control schemes, pathways connecting an initial and a final state can be independently controlled by manipulating the complex amplitudes of their transition matrix elements. For paths characterized by the absorption of multiple photons, these quantities depend on the magnitude and phase between the intermediate steps, and are expected to be strongly affected by the presence of resonances. We investigate the coherent control of the photoemission process in neon using a phase-controlled two-color extreme ultraviolet pulse with frequency in proximity of an excited energy state. Using helium as a reference, we show that the presence of such a resonance in neon modifies the amplitude and phase of the asymmetric emission of photoelectrons. Theoretical simulations based on perturbation theory are in fair agreement with the experimental observations.
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| 8. |
- Jahnke, T., et al.
(author)
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Inner-Shell-Ionization-Induced Femtosecond Structural Dynamics of Water Molecules Imaged at an X-Ray Free-Electron Laser
- 2021
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In: Physical Review X. - : American Physical Society. - 2160-3308. ; 11:4
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Journal article (peer-reviewed)abstract
- The ultrafast structural dynamics of water following inner-shell ionization is a crucial issue in high-energy radiation chemistry. We have exposed isolated water molecules to a short x-ray pulse from a free-electron laser and detected momenta of all produced ions in coincidence. By combining experimental results and theoretical modeling, we can image dissociation dynamics of individual molecules in unprecedented detail. We reveal significant molecular structural dynamics in H2O2+, such as asymmetric deformation and bond-angle opening, leading to two-body or three-body fragmentation on a timescale of a few femtoseconds. We thus reconstruct several snapshots of structural dynamics at different time intervals, which highlight dynamical patterns that are relevant as initiating steps of subsequent radiation-damage processes.
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| 9. |
- Ayeni, O. R., et al.
(author)
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Clinical and Radiographic Criteria Define "Acceptable" Surgical Correction of Hip Femoroacetabular Impingement Syndrome as Well as Postoperative Complications: An International Modified Delphi Study
- 2023
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In: Arthroscopy-the Journal of Arthroscopic and Related Surgery. - : Elsevier BV. - 0749-8063. ; 39:5, s. 1198-1210
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Journal article (peer-reviewed)abstract
- Objectives: To develop recommendations for clinical and radiographic criteria to help define the "acceptable" surgical correction of femoroacetabular impingement syndrome (FAIS) and identify/define complications postoperatively. Methods: A 3-phase modified Delphi study was conducted involving a case-based survey; a Likert/multiple choice-based survey concerning radiographic and physical examination characteristics to help define FAIS correction, as well as the prevalence and definition of potential postoperative complications; and 2 consensus meetings. Results: Of the 75 experts invited, 54 completed the Phase I survey, 50 completed the Phase II survey (72% and 67% response rate), and 50 participated in the Phase III consensus meetings. For both typical and atypical (complex) cases, there was consensus that fluoroscopy with multiple views and dynamic hip assessment should be used intraoperatively (96% and 100%, respectively). For typical FAIS cases, the Expert Panel agreed that Dunn lateral and anteroposterior radiographs were the most important radiographs to evaluate the hip postoperatively (88%, consensus). When asked about evaluating the correction of cam impingement postoperatively, 87% voted that they use subjective evaluation of the "sphericity" of the femoral head. In the case of focal and global pincer-type FAIS, there was consensus that the reduction or elimination of the crossover sign (84%) and lateral center-edge angle (91%) were important to inform the extent of the FAIS correction. There was consensus for recommending further investigation at 6 months postoperatively if hip pain had increased/plateaued (92% agreed); that additional investigation and treatment should occur between 6 and 12 months (90% agreed); and that a reoperation may be recommended at 12 months or later following this investigation period (89% agreed). Conclusions: This consensus project identified the importance of using fluoroscopy and dynamic hip assessment intraoperatively; Dunn lateral and anteroposterior view radiographs postoperatively; evaluating the "sphericity" of the femoral head for cam-type correction and the use of dynamic hip assessment; reducing/eliminating the crossover sign for focal pincertype FAIS; evaluating the lateral center-edge angle for global pincer-type FAIS; and avoiding overcorrection of pincer-type FAIS. In cases in which postoperative hip pain increased/plateaued, further investigation and treatment is warranted between 6 and 12 months, and a reoperation may be recommended at a minimum of 12 months depending on the cause of the hip pain. Clinical Relevance: Hip arthroscopy surgeons have yet to reach a firm agreement on what constitutes an "acceptable" or "good" surgery radiographically and how they can achieve desired clinical outcomes. Although this was a comprehensive effort, more study is needed to determine therapeutic thresholds that can be universally applied.
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| 10. |
- Makos, I., et al.
(author)
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Attosecond photoelectron spectroscopy using high-harmonic generation and seeded free-electron lasers
- 2023
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In: 2023 Photonics North, PN 2023. - 9798350326734
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Conference paper (peer-reviewed)abstract
- In this work, we use attosecond time-resolved techniques to investigate photoionization dynamics on its natural timescale, employing both high harmonic generation and seeded free-electron lasers to generate extreme ultraviolet attosecond pulse trains for our studies. With the former approach, we examine the role of nuclear motion in molecular photoionization dynamics, while with the latter we introduce a novel attosecond timing tool for single-shot characterization of the relative phase between the XUV and the infrared field.
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| 14. |
- Iablonskyi, D., et al.
(author)
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Interatomic Coulombic Decay Processes after Multiple Valence Excitations in Ne Clusters
- 2015
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In: Journal of Physics: Conference Series. - : IOP Publishing. - 1742-6596 .- 1742-6588. ; 635, s. 112067-112067
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Conference paper (peer-reviewed)abstract
- We present a comprehensive analysis of autoionization processes in Ne clusters (similar to 5000 atoms) after multiple valence excitations by free electron laser radiation. The evolution from 2-body interatomic Coulombic decay (ICD) to 3-body ICD is demonstrated when changing from surface to bulk Frenkel exciton excitation. Super Coster-Kronig type 2-body ICD is observed at Wannier exciton which quenches the main ICD channel.
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| 15. |
- Maroju, Praveen Kumar, et al.
(author)
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Attosecond coherent control of electronic wave packets in two-colour photoionization using a novel timing tool for seeded free-electron laser
- 2023
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In: Nature Photonics. - : Springer Science and Business Media LLC. - 1749-4885 .- 1749-4893. ; 17, s. 200-207
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Journal article (peer-reviewed)abstract
- In ultrafast spectroscopy, the temporal resolution of time-resolved experiments depends on the duration of the pump and probe pulses, and on the control and characterization of their relative synchronization. Free-electron lasers operating in the extreme ultraviolet and X-ray spectral regions deliver pulses with femtosecond and attosecond duration in a broad array of pump-probe configurations to study a wide range of physical processes. However, this flexibility, together with the large dimensions and high complexity of the experimental set-ups, limits control of the temporal delay to the femtosecond domain, thus precluding a time resolution below the optical cycle. Here we demonstrate a novel single-shot technique able to determine the relative synchronization between an attosecond pulse train-generated by a seeded free-electron laser-and the optical oscillations of a near-infrared field, with a resolution of one atomic unit (24 as). Using this attosecond timing tool, we report the first example of attosecond coherent control of photoionization in a two-colour field by manipulating the phase of high-order near-infrared transitions.
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| 18. |
- Iablonskyi, D., et al.
(author)
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Slow Interatomic Coulombic Decay of Multiply Excited Neon Clusters
- 2016
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In: Physical Review Letters. - 0031-9007. ; 117:27
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Journal article (peer-reviewed)abstract
- Ne clusters (∼5000 atoms) were resonantly excited (2p→3s) by intense free electron laser (FEL) radiation at FERMI. Such multiply excited clusters can decay nonradiatively via energy exchange between at least two neighboring excited atoms. Benefiting from the precise tunability and narrow bandwidth of seeded FEL radiation, specific sites of the Ne clusters were probed. We found that the relaxation of cluster surface atoms proceeds via a sequence of interatomic or intermolecular Coulombic decay (ICD) processes while ICD of bulk atoms is additionally affected by the surrounding excited medium via inelastic electron scattering. For both cases, cluster excitations relax to atomic states prior to ICD, showing that this kind of ICD is rather slow (picosecond range). Controlling the average number of excitations per cluster via the FEL intensity allows a coarse tuning of the ICD rate.
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| 19. |
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| 20. |
- Takanashi, T, et al.
(author)
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Time-Resolved Measurement of Interatomic Coulombic Decay Induced by Two-Photon Double Excitation of Ne2
- 2017
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In: Physical Review Letters. - 0031-9007. ; 118:3
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Journal article (peer-reviewed)abstract
- The hitherto unexplored two-photon doubly excited states [Ne∗(2p-13s)]2 were experimentally identified using the seeded, fully coherent, intense extreme ultraviolet free-electron laser FERMI. These states undergo ultrafast interatomic Coulombic decay (ICD), which predominantly produces singly ionized dimers. In order to obtain the rate of ICD, the resulting yield of Ne2+ ions was recorded as a function of delay between the extreme ultraviolet pump and UV probe laser pulses. The extracted lifetimes of the long-lived doubly excited states, 390(-130/+450) fs, and of the short-lived ones, less than 150 fs, are in good agreement with ab initio quantum mechanical calculations.
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| 21. |
- Ashizawa, T., et al.
(author)
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Consensus-based care recommendations for adults with myotonic dystrophy type 1
- 2018
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In: Neurology-Clinical Practice. - : Ovid Technologies (Wolters Kluwer Health). - 2163-0402 .- 2163-0933. ; 8:6, s. 507-520
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Research review (peer-reviewed)abstract
- Purpose of review Myotonic dystrophy type 1 (DM1) is a severe, progressive genetic disease that affects between 1 in 3,000 and 8,000 individuals globally. No evidence-based guideline exists to inform the care of these patients, and most do not have access to multidisciplinary care centers staffed by experienced professionals, creating a clinical care deficit. Recent findings The Myotonic Dystrophy Foundation (MDF) recruited 66 international clinicians experienced in DM1 patient care to develop consensus-based care recommendations. MDF created a 2-step methodology for the project using elements of the Single Text Procedure and the Nominal Group Technique. The process generated a 4-page Quick Reference Guide and a comprehensive, 55-page document that provides clinical care recommendations for 19 discrete body systems and/or care considerations. The resulting recommendations are intended to help standardize and elevate care for this patient population and reduce variability in clinical trial and study environments.
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| 22. |
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| 23. |
- Guillemin, R., et al.
(author)
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Isotope effects in dynamics of water isotopologues induced by core ionization at an x-ray free-electron laser
- 2023
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In: STRUCTURAL DYNAMICS-US. - 2329-7778. ; 10:5
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Journal article (peer-reviewed)abstract
- Dynamical response of water exposed to x-rays is of utmost importance in a wealth of science areas. We exposed isolated water isotopologues to short x-ray pulses from a free-electron laser and detected momenta of all produced ions in coincidence. By combining experimental results and theoretical modeling, we identify significant structural dynamics with characteristic isotope effects in H2O2+, D2O2+, and HDO2+, such as asymmetric bond elongation and bond-angle opening, leading to two-body or three-body fragmentation on a timescale of a few femtoseconds. A method to disentangle the sequences of events taking place upon the consecutive absorption of two x-ray photons is described. The obtained deep look into structural properties and dynamics of dissociating water isotopologues provides essential insights into the underlying mechanisms.
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| 24. |
- Maroju, P. K., et al.
(author)
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Analysis of two-color photoelectron spectroscopy for attosecond metrology at seeded free-electron lasers
- 2021
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In: New Journal of Physics. - : IOP Publishing. - 1367-2630. ; 23:4
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Journal article (peer-reviewed)abstract
- The generation of attosecond pulse trains at free-electron lasers opens new opportunities in ultrafast science, as it gives access, for the first time, to reproducible, programmable, extreme ultraviolet (XUV) waveforms with high intensity. In this work, we present a detailed analysis of the theoretical model underlying the temporal characterization of the attosecond pulse trains recently generated at the free-electron laser FERMI. In particular, the validity of the approximations used for the correlated analysis of the photoelectron spectra generated in the two-color photoionization experiments are thoroughly discussed. The ranges of validity of the assumptions, in connection with the main experimental parameters, are derived.
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| 25. |
- Gonzalez-Beltran, AN, et al.
(author)
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Community standards for open cell migration data
- 2020
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In: GigaScience. - : Oxford University Press (OUP). - 2047-217X. ; 9:5
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Journal article (peer-reviewed)abstract
- Cell migration research has become a high-content field. However, the quantitative information encapsulated in these complex and high-dimensional datasets is not fully exploited owing to the diversity of experimental protocols and non-standardized output formats. In addition, typically the datasets are not open for reuse. Making the data open and Findable, Accessible, Interoperable, and Reusable (FAIR) will enable meta-analysis, data integration, and data mining. Standardized data formats and controlled vocabularies are essential for building a suitable infrastructure for that purpose but are not available in the cell migration domain. We here present standardization efforts by the Cell Migration Standardisation Organisation (CMSO), an open community-driven organization to facilitate the development of standards for cell migration data. This work will foster the development of improved algorithms and tools and enable secondary analysis of public datasets, ultimately unlocking new knowledge of the complex biological process of cell migration.
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