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430 results on '"Winiarski J"'

6. Hematopoietic stem cell transplantation for Wiskott-Aldrich syndrome: an EBMT Inborn Errors Working Party analysis

Author

Albert, M. H., Slatter, M. A., Gennery, A. R., Gungor, T., Bakunina, K., Markovitch, B., Hazelaar, S., Sirait, T., Courteille, V., Aiuti, A., Aleinikova, O. V., Balashov, D., Bernardo, M. E., Bodova, I., Bruno, B., Cavazzana, M., Chiesa, R., Fischer, A., Hauck, F., Ifversen, M., Kalwak, K., Klein, C., Kulagin, A., Kupesiz, A., Kuskonmaz, B., Lindemans, C. A., Locatelli, Franco, Lum, S. H., Maschan, A., Meisel, R., Moshous, D., Porta, F., Sauer, M. G., Sedlacek, P., Schulz, A., Suarez, F., Vallee, T. C., Winiarski, J. H., Zecca, M., Neven, B., Veys, P., Lankester, A. C., Locatelli F. (ORCID:0000-0002-7976-3654), Albert, M. H., Slatter, M. A., Gennery, A. R., Gungor, T., Bakunina, K., Markovitch, B., Hazelaar, S., Sirait, T., Courteille, V., Aiuti, A., Aleinikova, O. V., Balashov, D., Bernardo, M. E., Bodova, I., Bruno, B., Cavazzana, M., Chiesa, R., Fischer, A., Hauck, F., Ifversen, M., Kalwak, K., Klein, C., Kulagin, A., Kupesiz, A., Kuskonmaz, B., Lindemans, C. A., Locatelli, Franco, Lum, S. H., Maschan, A., Meisel, R., Moshous, D., Porta, F., Sauer, M. G., Sedlacek, P., Schulz, A., Suarez, F., Vallee, T. C., Winiarski, J. H., Zecca, M., Neven, B., Veys, P., Lankester, A. C., and Locatelli F. (ORCID:0000-0002-7976-3654)

Abstract

Allogeneic hematopoietic stem cell transplantation (HSCT) is a potentially curative treatment for patients affected by Wiskott-Aldrich syndrome (WAS). Reported HSCT outcomes have improved over time with respect to overall survival, but some studies have identified older age and HSCT from alternative donors as risk factors predicting poorer outcome. We analyzed 197 patients undergoing transplant at European Society for Blood and Marrow Transplantation centers between 2006 and 2017 who received conditioning as recommended by the Inborn Errors Working Party (IEWP): either busulfan (n = 103) or treosulfan (n = 94) combined with fludarabine ± thiotepa. After a median follow-up post-HSCT of 44.9 months, 176 patients were alive, resulting in a 3-year overall survival of 88.7% and chronic graft-versus-host disease (GVHD)-free survival (events include death, graft failure, and severe chronic GVHD) of 81.7%. Overall survival and chronic GVHD-free survival were not significantly affected by conditioning regimen (busulfan- vs treosulfan-based), donor type (matched sibling donor/matched family donor vs matched unrelated donor/mismatched unrelated donor vs mismatched family donor), or period of HSCT (2006-2013 vs 2014-2017). Patients aged <5 years at HSCT had a significantly better overall survival. The overall cumulative incidences of grade III to IV acute GVHD and extensive/moderate/severe chronic GVHD were 6.6% and 2.1%, respectively. Patients receiving treosulfan-based conditioning had a higher incidence of graft failure and mixed donor chimerism and more frequently underwent secondary procedures (second HSCT, unconditioned stem cell boost, donor lymphocyte infusion, or splenectomy). In summary, HSCT for WAS with conditioning regimens currently recommended by IEWP results in excellent survival and low rates of GVHD, regardless of donor or stem cell source, but age ≥5 years remains a risk factor for overall survival.

Published
2022

25. Improved survival after allogeneic haematopoietic stem cell transplantation in recent years: P646

Author

Ringden, O., Ackefors, M., Berglund, S., Blennow, O., Dahllof, G., Dlugosz, A., Garming-Legert, K., Gertow, J., Gustafsson, B., Hassan, M., Hassan, Z., Hauzenberger, D., Hagglund, H., Karlsson, H., Klingspor, L., Kumlien, G., Le Blanc, K., Ljungman, P., Machaczka, M., Marschall, H.-U., Mattsson, J., Olsson, R., Omazic, B., Sairafi, D., Schaffer, M., Svahn, B.-M., Svenberg, P., Swartling, L., Szakos, A., Uhlin, M., Uzunel, M., Watz, E., Wernerson, A., Wikman, A., Wikstrom, A.-C., Winiarski, J., and Remberger, M.

Published
2011

44. Oral Cryotherapy to Reduce the Incidence of Severe Oral Mucositis in Children Undergoing Hematopoietic Stem Cell Transplantation : Results of a Randomized Clinical Trial

Author

Kamsvåg-Magnusson, Tove, von Essen, Louise, Arvidson, Johan, Svanberg, Anncarin, Mellgren, K., Garming-Legert, K., Toporski, J., Winiarski, J., Ljungman, G., Kamsvåg-Magnusson, Tove, von Essen, Louise, Arvidson, Johan, Svanberg, Anncarin, Mellgren, K., Garming-Legert, K., Toporski, J., Winiarski, J., and Ljungman, G.

Published
2017

47. Effect of Silver Addition to LaFeO3 Perovskite on the Activity of Monolithic La1−xAgxFeO3 Perovskite Catalysts in Methane Hexane Oxidation.

Author

Kucharczyk, B., Adamska, K., Tylus, W., Miśta, W., Szczygieł, B., and Winiarski, J.

Subjects
HEXANE, SOLID solutions, SILVER catalysts, PEROVSKITE, CATALYSTS, SURFACE area measurement, OXIDATION of hydrocarbons
Abstract

La1−xAgxFeO3 (x = 0.05–0.25) perovskites deposited on a honeycomb-shaped monolithic metallic support were used as catalysts for the total oxidation of hydrocarbons. Monolithic catalysts were prepared by washcoating honeycomb substrates with lanthanum-stabilized γ-Al2O3, on which La1−xAgxFeO3 was deposited. Catalysts with La1−xAgxFeO3 perovskites show higher activity for methane oxidation at x ≥ 0.05 and hexane oxidation at x ≥ 0.1 than LaFeO3 catalyst. The perovskite catalysts were characterized by XRD, XPS, SEM, TEM, TPR-H2, O2-TPD and BET specific surface area measurement. Ag forms solid solutions with the La1−xAgxFeO3 perovskite lattice for x = 0.05–0.1. In La1−xAgxFeO3 perovskites (x ≥ 0.1), Ag0 particles with average crystallite sizes from 31 to 41 nm were also identified. When Ag is introduced into the LaFeO3 perovskite, both the changes occurring after some Ag is incorporated into its structure and the presence of large crystallites of metallic silver contribute to the catalysts' activity increase. [ABSTRACT FROM AUTHOR]

Published
2019
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