| 1. |
- Allaf Navirian, Hengameh, et al.
(författare)
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Repetitive ultrafast melting of InSb as an x-ray timing diagnostic
- 2008
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Ingår i: Applied Physics Reviews. - : AIP Publishing. - 1931-9401. ; 103:10, s. 6-103510
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Tidskriftsartikel (refereegranskat)abstract
- We have demonstrated the possibility of using repetitive ultrafast melting of InSb as a timing diagnostic in connection with visible-light pump/x-ray probe measurements at high-repetition-rate x-ray facilities. Although the sample was molten and regrown approximately 1x10(6) times, a distinct reduction in time-resolved x-ray reflectivity could be observed using a streak camera with a time resolution of 2.5 ps. The time-resolved x-ray reflectivity displayed this distinct decrease despite the fact that the average reflectivity of the sample had fallen to approximately 50% of its original value due to accumulated damage from the prolonged laser exposure. The topography of the laser-exposed sample was mapped using an optical microscope, a stylus profilometer, photoelectron microscopy, and a scanning tunneling microscope. Although the surface of the sample is not flat following prolonged exposure at laser fluences above 15 mJ/cm(2), the atomic scale structure regrows, and thus, regenerates the sample on a nanosecond timescale. In the fluence range between 15 and 25 mJ/cm(2), the laser power is sufficient to melt the sample, while regrowth occurs with a sufficiently good structure to allow the extraction of timing information via ultrafast time-resolved x-ray measurements. This can be applied for timing purposes at synchrotron radiation and x-ray free-electron laser facilities. It is also noteworthy that we were able to reproduce the fluence dependencies of melting depth and disordering time previously obtained in single-shot, nonthermal melting experiments with higher temporal resolution.
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| 2. |
- Berntorp, Erik, et al.
(författare)
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Sixth Åland Island Conference on von Willebrand disease
- 2022
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Ingår i: Haemophilia. - : Wiley. - 1351-8216 .- 1365-2516. ; 28:S5, s. 3-15
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Tidskriftsartikel (refereegranskat)abstract
- Introduction: The sixth Åland Islands Conference on von Willebrand disease (VWD) on the Åland Islands, Finland, was held from 20 to 22 September 2018. Aim: The meeting brought together experts in the field of VWD from around the world to share the latest advances and knowledge in VWD. Results and discussion: The topics covered both clinical aspects of disease management, and biochemical and laboratory insights into the disease. The clinical topics discussed included epidemiology, diagnosis and treatment of VWD in different countries, management of children with VWD, bleeding control during surgery, specific considerations for the management of type 3 VWD and bleeding control in women with VWD. Current approaches to the management of acquired von Willebrand syndrome were also discussed. Despite significant advances in the understanding and therapeutic options for VWD, there remain many challenges to be overcome in order to optimise patient care. In comparison with haemophilia A, there are very few registries of VWD patients, which would be a valuable source of data on the condition and its management. VWD is still underdiagnosed, and many patients suffer recurrent or severe bleeding that could be prevented. Awareness of VWD among healthcare practitioners, including non-haematologists, should be improved to allow timely diagnosis and intervention. Diagnosis remains challenging, and the development of fast, simple assays may help to facilitate accurate and rapid diagnosis of VWD.
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| 3. |
- Corrales-Medina, Fernando F., et al.
(författare)
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A need to increase von Willebrand disease awareness : vwdtest.com – A global initiative to help address this gap
- 2023
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Ingår i: Blood Reviews. - : Elsevier BV. - 0268-960X. ; 58
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Forskningsöversikt (refereegranskat)abstract
- Von Willebrand disease (VWD) is an inherited bleeding disorder caused by quantitative or qualitative deficiencies in von Willebrand factor (VWF). People with VWD may experience excessive, recurrent or prolonged bleeding, particularly during menstruation, childbirth, surgery or following trauma. However, many VWD patients are undiagnosed, and therefore inadequately treated. Reasons for the underdiagnosis of VWD include its relatively mild symptoms, complex diagnosis, lack of awareness among non-specialist healthcare providers and the general population, and a lack of prioritisation of disorders disproportionately affecting females. The vwdtest.com platform was launched as part of a global initiative to raise awareness and improve diagnosis of VWD. Besides providing VWD-specific educational resources, the website includes an online bleeding self-assessment tool and offers diagnostic support for individuals, and their providers, who have a score suggestive of a bleeding disorder. vwdtest.com helps to address these unmet needs, especially in regions with limited access to educational and diagnostic resources.
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| 4. |
- Gadalla, Shahinaz M, et al.
(författare)
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Outcomes of allogeneic hematopoietic cell transplant in patients with dyskeratosis congenita.
- 2013
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Ingår i: Biology of blood and marrow transplantation : journal of the American Society for Blood and Marrow Transplantation. - : Elsevier BV. - 1523-6536. ; 19:8, s. 1238-1243
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Tidskriftsartikel (refereegranskat)abstract
- We describe outcomes after allogeneic transplantation in 34 patients with dyskeratosis congenita transplanted between 1981 and 2009. The median age at transplantation was 13 years (range 2 - 35). Approximately 50% of transplants were from related donors. Bone marrow was the predominant source of stem cells (n=24/34). The day-28 probability of neutrophil recovery was 73% and the day-100 platelet recovery was 72%. The day-100 probability of grade II-IV acute GVHD and the 3-year probability of chronic GVHD were 24% and 37%, respectively. The 10-year probability of survival was 30%; 14 patients were alive at last follow-up. Ten deaths occurred within 4 months from transplantation due to graft failure (n=6) or other transplant-related complications; 9 of these patients had been transplanted from mismatched related or from unrelated donors. Another 10 deaths occurred after 4 months; 6 of them occurred more than 5 years from transplantation, 4 of these were attributed to pulmonary failure. Transplant-regimen intensity and transplants from mismatched related or unrelated donors were associated with early mortality. Transplantation of grafts from HLA-matched siblings with cyclophosphamide-containing non-radiation regimens was associated with early low toxicity. Late mortality was attributed mainly to pulmonary complications and likely related to the underlying disease.
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| 5. |
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| 6. |
- Rodeghiero, Francesco, et al.
(författare)
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Fundamentals for a Systematic Approach to Mild and Moderate Inherited Bleeding Disorders : An EHA Consensus Report
- 2019
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Ingår i: HemaSphere. - 2572-9241. ; 3:5
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Tidskriftsartikel (refereegranskat)abstract
- Healthy subjects frequently report minor bleedings that are frequently 'background noise' of normality rather than a true disorder. Nevertheless, unexpected or unusual bleeding may be alarming. Thus, the distinction between normal and pathologic bleeding is critical. Understanding the underlying pathologic mechanism in patients with an excessive bleeding is essential for their counseling and treatment. Most of these patients with significant bleeding will result affected by non-severe inherited bleeding disorders (BD), collectively denominated mild or moderate BD for their relatively benign course. Unfortunately, practical recommendations for the management of these disorders are still lacking due to the current state of fragmented knowledge of pathophysiology and lack of a systematic diagnostic approach. To address this gap, an International Working Group (IWG) was established by the European Hematology Association (EHA) to develop consensus-based guidelines on these disorders. The IWG agreed that grouping these disorders by their clinical phenotype under the single category of mild-to-moderate bleeding disorders (MBD) reflects current clinical practice and will facilitate a systematic diagnostic approach. Based on standardized and harmonized definitions a conceptual unified framework is proposed to distinguish normal subjects from affected patients. The IWG proposes a provisional comprehensive patient-centered initial diagnostic approach that will result in classification of MBD into distinct clinical-pathological entities under the overarching principle of clinical utility for the individual patient. While we will present here a general overview of the global management of patients with MBD, this conceptual framework will be adopted and validated in the evidence-based, disease-specific guidelines under development by the IWG.
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| 7. |
- Sondhauss, Peter, et al.
(författare)
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Metal-like heat conduction in laser-excited InSb probed by picosecond time-resolved x-ray diffraction
- 2008
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Ingår i: Physical Review B (Condensed Matter and Materials Physics). - : American Physical Society. - 1098-0121. ; 78:11
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Tidskriftsartikel (refereegranskat)abstract
- A semiconductor (InSb) showed transient metal- like heat conduction after excitation of a dense electron- hole plasma via short and intense light pulses. A related ultrafast strain relaxation was detected using picosecond time-resolved x-ray diffraction. The deduced heat conduction was, by a factor of 30, larger than the lattice contribution. The anomalously high heat conduction can be explained once the contribution from the degenerate photocarrier plasma is taken into account. The magnitude of the effect could provide the means for guiding heat in semiconductor nanostructures. In the course of this work, a quantitative model for the carrier dynamics in laser-irradiated semiconductors has been developed, which does not rely on any adjustable parameters or ad hoc assumptions. The model includes various light absorption processes (interband, free carrier, two photon, and dynamical Burstein- Moss shifts), ambipolar diffusion, energy transport (heat and chemical potential), electrothermal effects, Auger recombination, collisional excitation, and scattering (elastic and inelastic). The model accounts for arbitrary degrees of degeneracy.
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| 8. |
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| 9. |
- Synnergren, Ola, et al.
(författare)
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Coherent Phonon Control
- 2007
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Ingår i: Applied Physics Letters. - : AIP Publishing. - 0003-6951 .- 1077-3118. ; 90
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Tidskriftsartikel (refereegranskat)abstract
- Trains of ultrashort laser pulses have been used to generate and to coherently control acoustic phonons in bulk InSb. The coherent acoustic phonons have been probed via time-resolved x-ray diffraction. The authors show that phonons of a particular frequency can either be enhanced or canceled. They have carried out simulations to understand the size of the effects and the levels of cancellation. (c) 2007 American Institute of Physics.
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