| 1. |
- Abolhassani, H, et al.
(författare)
-
Care of patients with inborn errors of immunity in thirty J Project countries between 2004 and 2021
- 2022
-
Ingår i: Frontiers in immunology. - : Frontiers Media SA. - 1664-3224. ; 13, s. 1032358-
-
Tidskriftsartikel (refereegranskat)abstract
- The J Project (JP) physician education and clinical research collaboration program was started in 2004 and includes by now 32 countries mostly in Eastern and Central Europe (ECE). Until the end of 2021, 344 inborn errors of immunity (IEI)-focused meetings were organized by the JP to raise awareness and facilitate the diagnosis and treatment of patients with IEI.ResultsIn this study, meeting profiles and major diagnostic and treatment parameters were studied. JP center leaders reported patients’ data from 30 countries representing a total population of 506 567 565. Two countries reported patients from JP centers (Konya, Turkey and Cairo University, Egypt). Diagnostic criteria were based on the 2020 update of classification by the IUIS Expert Committee on IEI. The number of JP meetings increased from 6 per year in 2004 and 2005 to 44 and 63 in 2020 and 2021, respectively. The cumulative number of meetings per country varied from 1 to 59 in various countries reflecting partly but not entirely the population of the respective countries. Altogether, 24,879 patients were reported giving an average prevalence of 4.9. Most of the patients had predominantly antibody deficiency (46,32%) followed by patients with combined immunodeficiencies (14.3%). The percentages of patients with bone marrow failure and phenocopies of IEI were less than 1 each. The number of patients was remarkably higher that those reported to the ESID Registry in 13 countries. Immunoglobulin (IgG) substitution was provided to 7,572 patients (5,693 intravenously) and 1,480 patients received hematopoietic stem cell therapy (HSCT). Searching for basic diagnostic parameters revealed the availability of immunochemistry and flow cytometry in 27 and 28 countries, respectively, and targeted gene sequencing and new generation sequencing was available in 21 and 18 countries. The number of IEI centers and experts in the field were 260 and 690, respectively. We found high correlation between the number of IEI centers and patients treated with intravenous IgG (IVIG) (correlation coefficient, cc, 0,916) and with those who were treated with HSCT (cc, 0,905). Similar correlation was found when the number of experts was compared with those treated with HSCT. However, the number of patients treated with subcutaneous Ig (SCIG) only slightly correlated with the number of experts (cc, 0,489) and no correlation was found between the number of centers and patients on SCIG (cc, 0,174).Conclusions1) this is the first study describing major diagnostic and treatment parameters of IEI care in countries of the JP; 2) the data suggest that the JP had tremendous impact on the development of IEI care in ECE; 3) our data help to define major future targets of JP activity in various countries; 4) we suggest that the number of IEI centers and IEI experts closely correlate to the most important treatment parameters; 5) we propose that specialist education among medical professionals plays pivotal role in increasing levels of diagnostics and adequate care of this vulnerable and still highly neglected patient population; 6) this study also provides the basis for further analysis of more specific aspects of IEI care including genetic diagnostics, disease specific prevalence, newborn screening and professional collaboration in JP countries.
|
|
| 2. |
- Faatz, B., et al.
(författare)
-
Simultaneous operation of two soft x-ray free-electron lasers driven by one linear accelerator
- 2016
-
Ingår i: New Journal of Physics. - : IOP Publishing. - 1367-2630. ; 18
-
Tidskriftsartikel (refereegranskat)abstract
- Extreme-ultraviolet to x-ray free-electron lasers (FELs) in operation for scientific applications are up to now single-user facilities. While most FELs generate around 100 photon pulses per second, FLASH at DESY can deliver almost two orders of magnitude more pulses in this time span due to its superconducting accelerator technology. This makes the facility a prime candidate to realize the next step in FELs-dividing the electron pulse trains into several FEL lines and delivering photon pulses to several users at the same time. Hence, FLASH has been extended with a second undulator line and self-amplified spontaneous emission (SASE) is demonstrated in both FELs simultaneously. FLASH can now deliver MHz pulse trains to two user experiments in parallel with individually selected photon beam characteristics. First results of the capabilities of this extension are shown with emphasis on independent variation of wavelength, repetition rate, and photon pulse length.
|
|
| 3. |
|
|
| 4. |
|
|
| 5. |
|
|
| 6. |
|
|
| 7. |
|
|
| 8. |
|
|
| 9. |
|
|
| 10. |
|
|
| 11. |
- Correa, J., et al.
(författare)
-
The PERCIVAL soft X-ray Detector
- 2018
-
Ingår i: 2018 IEEE Nuclear Science Symposium and Medical Imaging Conference, NSS/MIC 2018 - Proceedings. - : Institute of Electrical and Electronics Engineers (IEEE). - 9781538684948
-
Konferensbidrag (refereegranskat)abstract
- The PERCIVAL collaboration to develop a soft X-ray imager able to address the challenges of high brilliance light sources, such as new-generation synchrotrons and Free Electron Lasers, has reached one of its major milestones: a full 2-MegaPixel (P2M) system (uninterrupted 4 × 4 cm2 active area) has already seen its first light.Smaller prototypes of the device, a monolithic active pixel sensor based on CMOS technology, have already been fully characterised, and have demonstrated high frame rate, large dynamic range, and relatively high quantum efficiency.The PERCIVAL modular layout allows for clover-leaf like arrangement of up to four P2M systems. Moreover, it will be post-processed in order to achieve a high quantum efficiency in its primary energy range (250 eV to 1 keV).We will present the P2M system, its status and newest results, bring these in context with achieved prototype performance, and outline future steps.
|
|
| 12. |
|
|
| 13. |
- Marras, A., et al.
(författare)
-
Percival P2M-FSI detector : First test at a Synchrotron Ring beamline with tender x-ray photons
- 2019
-
Ingår i: 2019 IEEE Nuclear Science Symposium and Medical Imaging Conference, NSS/MIC 2019. - : IEEE. - 9781728141640
-
Konferensbidrag (refereegranskat)abstract
- In this paper, we are presenting the results of the first test of the Percival P2M-FSI detector with tender x-rays photons at a synchrotron beamline. Percival is a monolithic CMOS Imager for detection of x-rays in Synchrotron Rings and Free Electron Lasers: the Front-Side-Illuminated (FSI) version of the detector has been proven able to successfully distinguish tender (2keV) x-ray single photons.
|
|
| 14. |
- Sedgwick, I., et al.
(författare)
-
P2M : First Optical Characterisation Results of a 2MPixel CMOS Image Sensor for Soft X-Ray Detection
- 2019
-
Ingår i: 2019 IEEE NUCLEAR SCIENCE SYMPOSIUM AND MEDICAL IMAGING CONFERENCE (NSS/MIC). - : IEEE. - 9781728141640
-
Konferensbidrag (refereegranskat)abstract
- High brilliance synchrotrons and FELs require high performing detector systems to realise their full potential. High dynamic range, low noise and high frame rate are all of great importance. In this paper we present first optical characterization results of the P2M CMOS sensor, designed for soft X-ray detection at such facilities. Previous work is summarised and an overview of the sensor is presented. Test results for the sensor's column-parallel ADC and readout chain are presented, and first test results for the pixel acquired using the Photon Transfer Curve (PTC) method are shown. Finally, an outline of future work is provided.
|
|
| 15. |
|
|
| 16. |
|
|
| 17. |
- Willasch, AM, et al.
(författare)
-
Myeloablative conditioning for allo-HSCT in pediatric ALL: FTBI or chemotherapy?-A multicenter EBMT-PDWP study
- 2020
-
Ingår i: Bone marrow transplantation. - : Springer Science and Business Media LLC. - 1476-5365 .- 0268-3369. ; 55:98, s. 1540-1551
-
Tidskriftsartikel (refereegranskat)abstract
- Although most children with acute lymphoblastic leukemia (ALL) receive fractionated total body irradiation (FTBI) as myeloablative conditioning (MAC) for allogeneic hematopoietic stem cell transplantation (allo-HSCT), it is an important matter of debate if chemotherapy can effectively replace FTBI. To compare outcomes after FTBI versus chemotherapy-based conditioning (CC), we performed a retrospective EBMT registry study. Children aged 2–18 years after MAC for first allo-HSCT of bone marrow (BM) or peripheral blood stem cells (PBSC) from matched-related (MRD) or unrelated donors (UD) in first (CR1) or second remission (CR2) between 2000 and 2012 were included. Propensity score weighting was used to control pretreatment imbalances of the observed variables. 3.054 patients were analyzed. CR1 (1.498): median follow-up (FU) after FTBI (1.285) and CC (213) was 6.8 and 6.1 years. Survivals were not significantly different. CR2 (1.556): median FU after FTBI (1.345) and CC (211) was 6.2 years. Outcomes after FTBI were superior as compared with CC with regard to overall survival (OS), leukemia-free survival (LFS), relapse incidence (RI), and nonrelapse mortality (NRM). However, we must emphasize the preliminary character of the results of this retrospective “real-world-practice” study. These findings will be prospectively assessed in the ALL SCTped 2012 FORUM trial.
|
|
| 18. |
- Wunderer, C. B., et al.
(författare)
-
The Percival 2-Megapixel monolithic active pixel imager
- 2019
-
Ingår i: Journal of Instrumentation. - 1748-0221. ; 14:1
-
Tidskriftsartikel (refereegranskat)abstract
- The peak brilliance reached by today's Free-Electron Laser and Synchrotron light sources requires photon detectors matching their output intensity and other characteristics in order to fully realize the sources' potential. The Pixellated Energy Resolving CMOS Imager, Versatile And Large (Percival) is a dedicated soft X-ray imager (0.25-1 keV) developed for this purpose by a collaboration of DESY, Rutherford Appleton Laboratory/STFC, Elettra Sincrotrone Trieste, Diamond Light Source, and Pohang Accelerator Laboratory. Following several generations of prototypes, the Percival "P2M" 2-Megapixel imager - a 4.5x5 cm monolithic, stitched sensor with an uninterrupted imaging area of 4x4 cm(2) (1408x1484 pixels of 27x27 mu m - was produced and has demonstrated basic functionality with a first-light image using visible light. It is currently being brought to full operation in a front-illuminated configuration. The readout system being commissioned in parallel has been developed specifically for this imager which will produce - at full 300 Hz frame rate - data at 20 Gbit/s. A first wafer with eight Percival P2M chips has undergone backthinning to enable soft X-ray detection. It has been diced and chips are currently being wirebonded. We summarize here the P2M system, the project status, and show the P2M sensor's first response to visible light.
|
|
| 19. |
- Bencsik, G., et al.
(författare)
-
Conducting polymer based multifunctional composite electrodes
- 2009
-
Ingår i: Reaction Kinetics and Catalysis Letters. - : Springer. - 0133-1736 .- 1588-2837. ; 96, s. 421-428
-
Tidskriftsartikel (refereegranskat)abstract
- In this paper, we report a novel pattern of composite electrocatalysts. PPy/iron-oxalate films exhibit photo-electrochemical activity. The PPy/B12 composite electrode on stainless steal (SS) support shows high catalytic activity in the electrochemical reduction of methylviologen. Thin polymer layers filled with magnetite particles can be applicable in magneto-selective electrochemical reactions.In this paper, we report a novel pattern of composite electrocatalysts. PPy/iron-oxalate films exhibit photo-electrochemical activity. The PPy/B12 composite electrode on stainless steal (SS) support shows high catalytic activity in the electrochemical reduction of methylviologen. Thin polymer layers filled with magnetite particles can be applicable in magneto-selective electrochemical reactions.
|
|
| 20. |
- Correa, J., et al.
(författare)
-
The PERCIVAL detector : first user experiments
- 2023
-
Ingår i: Journal of Synchrotron Radiation. - 0909-0495 .- 1600-5775. ; 30, s. 242-250
-
Tidskriftsartikel (refereegranskat)abstract
- The PERCIVAL detector is a CMOS imager designed for the soft X-ray regime at photon sources. Although still in its final development phase, it has recently seen its first user experiments: ptychography at a free-electron laser, holographic imaging at a storage ring and preliminary tests on X-ray photon correlation spectroscopy. The detector performed remarkably well in terms of spatial resolution achievable in the sample plane, owing to its small pixel size, large active area and very large dynamic range; but also in terms of its frame rate, which is significantly faster than traditional CCDs. In particular, it is the combination of these features which makes PERCIVAL an attractive option for soft X-ray science.
|
|
| 21. |
|
|
