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53. Plasma cell deficiency in human subjects with heterozygous mutations in Sec61 translocon alpha 1 subunit (SEC61A1)

Author

Schubert, Desirée, Klein, Marie-Christine, Hassdenteufel, Sarah, Caballero-Oteyza, Andrés, Yang, Linlin, Proietti, Michele, Bulashevska, Alla, Kemming, Janine, Kühn, Johannes, Winzer, Sandra, Rusch, Stephan, Fliegauf, Manfred, Schäffer, Alejandro A, Pfeffer, Stefan, Geiger, Roger, Cavalié, Adolfo, Cao, Hongzhi, Yang, Fang, Li, Yong, Rizzi, Marta, Eibel, Hermann, Kobbe, Robin, Marks, Amy L, Peppers, Brian P, Hostoffer, Robert W, Puck, Jennifer M, Zimmermann, Richard, and Grimbacher, Bodo

Subjects
Biomedical and Clinical Sciences, Immunology, Rare Diseases, Genetics, Clinical Research, Aetiology, 2.1 Biological and endogenous factors, Agammaglobulinemia, B-Lymphocytes, Calcium, Cell Differentiation, Cell Line, Cell Line, Tumor, Exome, HEK293 Cells, HeLa Cells, Heterozygote, Humans, Immunologic Deficiency Syndromes, Mutation, Plasma Cells, Protein Transport, Respiratory Tract Infections, SEC Translocation Channels, T-Lymphocytes, Unfolded Protein Response, SEC61A1, translocon, protein translocation, antibody deficiency, plasma cell, multiple myeloma, calcium homeostasis, endoplasmic reticulum stress, Hela Cells, Allergy
Abstract

BackgroundPrimary antibody deficiencies (PADs) are the most frequent primary immunodeficiencies in human subjects. The genetic causes of PADs are largely unknown. Sec61 translocon alpha 1 subunit (SEC61A1) is the major subunit of the Sec61 complex, which is the main polypeptide-conducting channel in the endoplasmic reticulum membrane. SEC61A1 is a target gene of spliced X-box binding protein 1 and strongly induced during plasma cell (PC) differentiation.ObjectiveWe identified a novel genetic defect and studied its pathologic mechanism in 11 patients from 2 unrelated families with PADs.MethodsWhole-exome and targeted sequencing were conducted to identify novel genetic mutations. Functional studies were carried out ex vivo in primary cells of patients and in vitro in different cell lines to assess the effect of SEC61A1 mutations on B-cell differentiation and survival.ResultsWe investigated 2 families with patients with hypogammaglobulinemia, severe recurrent respiratory tract infections, and normal peripheral B- and T-cell subpopulations. On in vitro stimulation, B cells showed an intrinsic deficiency to develop into PCs. Genetic analysis and targeted sequencing identified novel heterozygous missense (c.254T>A, p.V85D) and nonsense (c.1325G>T, p.E381*) mutations in SEC61A1, segregating with the disease phenotype. SEC61A1-V85D was deficient in cotranslational protein translocation, and it disturbed the cellular calcium homeostasis in HeLa cells. Moreover, SEC61A1-V85D triggered the terminal unfolded protein response in multiple myeloma cell lines.ConclusionWe describe a monogenic defect leading to a specific PC deficiency in human subjects, expanding our knowledge about the pathogenesis of antibody deficiencies.

Published
2018

54. Hope for motherhood: pregnancy after allogeneic hematopoietic cell transplantation (a national multicenter study)

Author

Sockel, Katja, Neu, Annika, Goeckenjan, Maren, Ditschkowski, Markus, Hilgendorf, Inken, Kröger, Nicolaus, Ayuk, Francis A., Stoelzel, Friedrich, Middeke, Jan Moritz, Eder, Matthias, Bethge, Wolfgang, Finke, Jürgen, Bertz, Hartmut, Kobbe, Guido, Kaufmann, Martin, Platzbecker, Uwe, Beverungen, David, Schmid, Christoph, von Bonin, Malte, Egger-Heidrich, Katharina, Heberling, Lisa, Trautmann-Grill, Karolin, Teipel, Raphael, Bug, Gesine, Tischer, Johanna, Fraccaroli, Alessia, Fante, Matthias, Wolff, Daniel, Luft, Thomas, Winkler, Julia, Schäfer-Eckart, Kerstin, Scheid, Christof, Holtick, Udo, Klein, Stefan, Blau, Igor Wolfgang, Burchert, Andreas, Wulf, Gerald, Hasenkamp, Justin, Schwerdtfeger, Rainer, Kaun, Stephan, Junghanss, Christian, Wortmann, Friederike, Winter, Susann, Neidlinger, Helga, Theuser, Catrin, Beyersmann, Jan, Bornhaeuser, Martin, Schmeller, Sandra, Schetelig, Johannes, Jost, Edgar, Schmid, Christoph, Baurmann, Herrad, Blau, Igor, Holderried, Tobias, Hertenstein, Bernd, Hänel, Mathias, Bornhäuser, Martin, Neidlinger, Helga, Kobbe, Guido, Winkler, Julia, Schroeder, Thomas, Bug, Gesine, Hopfer, Olaf, Finke, Jürgen, Hasenkamp, Justin, Krüger, William, Müller, Lutz, Kröger, Nikolaus, Eder, Matthias, Luft, Thomas, Hilgendorf, Inken, Ringhoffer, Mark, Stölzel, Friedrich, Scheid, Christoph, Platzbecker, Uwe, Wortmann, Friederike, Heinicke, Thomas, Wagner, Eva, Klein, Stefan, Burchert, Andreas, Tischer, Johanna, Stelljes, Matthias, Schäfer-Eckart, Kerstin, Wolff, Daniel, Junghanss, Christian, Ritter, Markus, Kaufmann, Martin, Bethge, Wolfgang, Mytilineos, Joannis, and Einsel, Hermann

Abstract

•Successful pregnancies are possible among female alloHCT recipients, although birth rate is >6 times lower than in the general population.•Key factors increasing the chance of pregnancy were younger age at HCT, nonmalignant disease, no TBI or dose <8 Gy and RIC/NMA conditioning

Published
2024
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63. Virological outcomes and genotypic resistance on dolutegravir-based antiretroviral therapy versus standard of care in children and adolescents: a secondary analysis of the ODYSSEY trial

Author

White, Ellen, Kityo, Cissy, Spyer, Moira J, Mujuru, Hilda A, Nankya, Immaculate, Kekitiinwa, Adeodata R, Lugemwa, Abbas, Kaudha, Elizabeth, Liberty, Afaaf, Cassim, Haseena, Archary, Moherndran, Cotton, Mark F, Ahimbisibwe, Grace Miriam, Cressey, Tim R, Ngampiyaskul, Chaiwat, Srirompotong, Ussanee, Behuhuma, Osee, Saidi, Yacine, Bamford, Alasdair, Kobbe, Robin, Nastouli, Eleni, Rojo, Pablo, Giaquinto, Carlo, Gibb, Diana M, Ford, Deborah, and Turkova, Anna

Abstract

ODYSSEY showed superior efficacy for dolutegravir-based antiretroviral therapy (ART) versus standard of care (SOC) in children living with HIV starting first-line or second-line ART aged 4 weeks or older. Here, we aim to compare virological outcomes and resistance in the dolutegravir group versus SOC for first-line and second-line ART up to 96 weeks.

Published
2025
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65. Genetic Analysis of a Cohort of 275 Patients with Hyper-IgE Syndromes and/or Chronic Mucocutaneous Candidiasis

Author

Frede, Natalie, Rojas-Restrepo, Jessica, Caballero Garcia de Oteyza, Andrés, Buchta, Mary, Hübscher, Katrin, Gámez-Díaz, Laura, Proietti, Michele, Saghafi, Shiva, Chavoshzadeh, Zahra, Soler-Palacin, Pere, Galal, Nermeen, Adeli, Mehdi, Aldave-Becerra, Juan Carlos, Al-Ddafari, Moudjahed Saleh, Ardenyz, Ömür, Atkinson, T. Prescott, Kut, Fulya Bektas, Çelmeli, Fatih, Rees, Helen, Kilic, Sara S., Kirovski, Ilija, Klein, Christoph, Kobbe, Robin, Korganow, Anne-Sophie, Lilic, Desa, Lunt, Peter, Makwana, Niten, Metin, Ayse, Özgür, Tuba Turul, Karakas, Ayse Akman, Seneviratne, Suranjith, Sherkat, Roya, Sousa, Ana Berta, Unal, Ekrem, Patiroglu, Turkan, Wahn, Volker, von Bernuth, Horst, Whiteford, Margo, Doffinger, Rainer, Jouhadi, Zineb, and Grimbacher, Bodo

Published
2021
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69. Allogeneic hematopoietic stem cell transplantation for advanced mycosis fungoides and Sézary syndrome. An updated experience of the Lymphoma Working Party of the European Society for Blood and Marrow Transplantation

Author

Domingo-Domenech, E., Duarte, R. F., Boumedil, A., Onida, F., Gabriel, I., Finel, H., Arcese, W., Browne, P., Beelen, D., Kobbe, G., Veelken, H., Arranz, R., Greinix, H., Lenhoff, S., Poiré, X., Ribera, J. M., Thompson, J., Zuckerman, T., Mufti, G. J., Cortelezzi, A., Olavarria, E., Dreger, P., Sureda, A., and Montoto, S.

Published
2021
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75. Upfront stem cell transplantation for newly diagnosed multiple myeloma with del(17p) and t(4;14): a study from the CMWP-EBMT

Author

Gagelmann, Nico, Eikema, Diderik-Jan, de Wreede, Liesbeth C., Rambaldi, Alessandro, Iacobelli, Simona, Koster, Linda, Caillot, Denis, Blaise, Didier, Remémyi, Péter, Bulabois, Claude-Eric, Passweg, Jakob, Leleu, Xavier, Zver, Samo, Kobbe, Guido, Ljungman, Per, Chevallier, Patrice, Ringhoffer, Mark, Martin, Murray, Salmenniemi, Urpu, Poiré, Xavier, Lenhoff, Stig, Pioltelli, Pietro, Mordini, Nicola, Delforge, Michel, Garderet, Laurent, Schönland, Stefan, Yakoub-Agha, Ibrahim, and Kröger, Nicolaus

Published
2021
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77. Impfen bei Immundefizienz: Anwendungshinweise zu den von der Ständigen Impfkommission empfohlenen Impfungen. (III) Impfen bei hämatologischen und onkologischen Erkrankungen (antineoplastische Therapie, Stammzelltransplantation), Organtransplantation und Asplenie

Author

Laws, Hans-Jürgen, Baumann, Ulrich, Bogdan, Christian, Burchard, Gerd, Christopeit, Maximilian, Hecht, Jane, Heininger, Ulrich, Hilgendorf, Inken, Kern, Winfried, Kling, Kerstin, Kobbe, Guido, Külper, Wiebe, Lehrnbecher, Thomas, Meisel, Roland, Simon, Arne, Ullmann, Andrew, de Wit, Maike, and Zepp, Fred

Published
2020
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78. Outcomes of haploidentical transplants with PT-CY vs 10/10 MUD transplants with ATG in Germany

Author

Arslan, Aysenur, Labuhn, Svenja, Sala, Elisa, Ringhoffer, Mark, Schetelig, Johannes, Schröder, Thomas, Bug, Gesine, Franke, Georg-Nikolaus, Stelljes, Matthias, Dreger, Peter, Zeiser, Robert, Teschner, Daniel, Bethge, Wolfgang, Eder, Matthias, Edinger, Matthias, Amann, Elisa Maria, Neuchel, Christine, Schmid-Möglich, Amelie, Schmeller, Sandra, Beyersmann, Jan, Schrezenmeier, Hubert, Mytilineos, Joannis, Kröger, Nicolaus, Fürst, Daniel, Kröger, Nicolaus, Schroeder, Thomas, Stelljes, Matthias, Dreger, Peter, Zeiser, Robert, Bethge, Wolfgang, Teschner, Daniel, Edinger, Matthias, Platzbecker, Uwe, Elmaagacli, Ahmet, Hilgendorf, Inken, Burchert, Andreas, Stölzel, Friedrich, Verbeek, Mareike, Sala, Elisa, Tischer, Johanna, Schetelig, Johannes, Brecht, Arne, Kaufmann, Martin, Kimmich, Christoph, Bittenbring, Jörg Thomas, Wulf, Gerald, Blau, Igor Wolfgang, Winkler, Julia, Bug, Gesine, Jost, Edgar, Holderried, Tobias, Dürholt, Mareike, Scheid, Christof, Wagner-Drouet, Eva, Wolleschak, Denise, Ringhoffer, Mark, Kiehl, Michael, Wortmann, Friederike, Kobbe, Guido, Schroers, Roland, Krackhardt, Angela, Klein, Stefan, Müller, Lutz P., Schmid, Christoph, Kaun, Stephan, Wieschermann, Ute, Bartscht, Tobias, Meyer, Ralf Georg, Wölfl, Matthias, Bader, Peter, Niederland, Judith, Maucher, Jakob, Mauz-Körholz, Christine, Burkhardt, Birgit, Kentouche, Karim, Passweg, Jakob, and Ehlert, Karoline

Abstract

•Five-year OS and DFS were significantly lower in PT-CY Haplo transplantation than 10/10 MUD transplantation with ATG in a German cohort.•10/10 MUD transplantation with ATG provides higher GRFS and lower aGVHD, cGVHD, and nonrelapse mortality (NRM) than PT-CY Haplo in a German cohort.

Published
2024
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79. Autologous stem cell transplantation in T-cell/histiocyte-rich large B-cell lymphoma: EBMT Lymphoma Working Party study

Author

Renders, Simon, Ngoya, Maud, Finel, Herve, Rubio, Marie-Thérèse, Townsend, William, Schroers, Roland, Novak, Urban, Schaap, Nicolaas, Aljurf, Mahmoud, Helbig, Grzegorz, Collin, Matthew, Kobbe, Guido, Huynh, Anne, Pérez-Simón, José Antonio, Bloor, Adrian, Ghesquieres, Hervé, Sureda, Anna, Schmitz, Norbert, Glass, Bertram, and Dreger, Peter

Abstract

•Analysis of 201 patients with R/R THRLBCL vs 5453 with DLBCL undergoing auto-HCT reveals superior PFS, OS, and relapse incidence for THRLBCL.•Auto-HCT for R/R THRLBCL resulted in a 2-year PFS of 78% and 59% in DLBCL, making it attractive for consolidating chemosensitive disease.

Published
2024
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81. Correction to: A Multi-center, Open-Label, Single-Arm Trial to Evaluate the Efficacy, Pharmacokinetics, and Safety and Tolerability of IGSC 20% in Subjects with Primary Immunodeficiency

Author

Santamaria, Manuel, Neth, Olaf, Douglass, Jo A., Krivan, Gergely, Kobbe, Robin, Bernatowska, Ewa, Grigoriadou, Sofia, Bethune, Claire, Chandra, Anita, Horneff, Gerd, Borte, Michael, Sonnenschein, Anja, Kralickova, Pavlina, Ramón, Silvia Sánchez, Langguth, Daman D., Gonzalez‑Granado, Luis Ignacio, Alsina, Laia, Querolt, Montse, Griffin, Rhonda, Hames, Carrie, Mondou, Elsa, Price, Jeffrey, Sanz, Ana, and Lin, Jiang

Published
2022
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82. Real-world experience of CPX-351 as first-line treatment for patients with acute myeloid leukemia

Author

Rautenberg, Christina, Stölzel, Friedrich, Röllig, Christoph, Stelljes, Matthias, Gaidzik, Verena, Lauseker, Michael, Kriege, Oliver, Verbeek, Mareike, Unglaub, Julia Marie, Thol, Felicitas, Krause, Stefan W., Hänel, Mathias, Neuerburg, Charlotte, Vucinic, Vladan, Jehn, Christian-Friedrich, Severmann, Julia, Wass, Maxi, Fransecky, Lars, Chemnitz, Jens, Holtick, Udo, Schäfer-Eckart, Kerstin, Schröder, Josephine, Kraus, Sabrina, Krüger, William, Kaiser, Ulrich, Scholl, Sebastian, Koch, Kathrin, Henning, Lea, Kobbe, Guido, Haas, Rainer, Alakel, Nael, Röhnert, Maximilian-Alexander, Sockel, Katja, Hanoun, Maher, Platzbecker, Uwe, Holderried, Tobias A. W., Morgner, Anke, Heuser, Michael, Sauer, Tim, Götze, Katharina S., Wagner-Drouet, Eva, Döhner, Konstanze, Döhner, Hartmut, Schliemann, Christoph, Schetelig, Johannes, Bornhäuser, Martin, Germing, Ulrich, Schroeder, Thomas, and Middeke, Jan Moritz

Published
2021
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84. Erratum zu: Impfen bei Immundefizienz: Anwendungshinweise zu den von der Ständigen Impfkommission empfohlenen Impfungen. (III) Impfen bei hämatologischen und onkologischen Erkrankungen (antineoplastische Therapie, Stammzelltransplantation), Organtransplantation und Asplenie

Author

Laws, Hans-Jürgen, Baumann, Ulrich, Bogdan, Christian, Burchard, Gerd, Christopeit, Maximilian, Hecht, Jane, Heininger, Ulrich, Hilgendorf, Inken, Kern, Winfried, Kling, Kerstin, Kobbe, Guido, Külper, Wiebe, Lehrnbecher, Thomas, Meisel, Roland, Simon, Arne, Ullmann, Andrew, de Wit, Maike, and Zepp, Fred

Published
2020
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90. Results from a multicenter, noninterventional registry study for multiple myeloma patients who received stem cell mobilization regimens with and without plerixafor

Author

Morris, Curly, Chabannon, Christian, Masszi, Tamas, Russell, Nigel, Nahi, Hareth, Kobbe, Guido, Krejci, Marta, Auner, Holger W., Pohlreich, David, Hayden, Patrick, Basak, Grzegorz W., Lenhoff, Stig, Schaap, Nicolaas, van Biezen, Anja, Knol, Cora, Iacobelli, Simona, Liu, Qianying, Celanovic, Marina, Garderet, Laurent, and Kröger, Nicolaus

Published
2020
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95. The relationship of sleep with temperature and metabolic rate in a hibernating primate.

Author

Krystal, Andrew, Krystal, AD, Schopler, B, Kobbe, S, Williams, C, Rakatondrainibe, H, Yoder, AD, and Klopfer, P

Abstract

STUDY OBJECTIVES: It has long been suspected that sleep is important for regulating body temperature and metabolic-rate. Hibernation, a state of acute hypothermia and reduced metabolic-rate, offers a promising system for investigating those relationships.

Published
2013

97. High-performing university students use AI more often and more effectively.

Author

Clark, Abby, Mistretta, Kathleen, Chambers, Emily, Terry, Sandy, Marshall, Bryan, Cardon, Peter, Fowler, Brad, Kobbe, Eric, and Risvold, Ward

Subjects
MANAGEMENT information systems, ARTIFICIAL intelligence, LEARNING, EDUCATION ethics, CHATGPT, EDUCATIONAL technology
Abstract

This study examines the significance of artificial intelligence (AI) in the realm of education, with a specific focus on its potential as a support mechanism and a driving force in educational contexts. The ongoing discourse surrounding the impact of AI technologies on student learning outcomes and the evolution of educational approaches is gaining momentum due to the rapid progress in this field. The primary objective of this research is to illuminate this crucial subject matter by delving into the diverse advantages and disadvantages of integrating AI into educational environments. By utilizing a blend of quantitative and qualitative research methodologies, the study assesses the current utilization of AI tools in classrooms and their efficacy in enriching learning processes. The outcomes seek to present an impartial viewpoint on the capacities and constraints of AI, providing valuable insights for educators, policymakers, and experts in educational technology. [ABSTRACT FROM AUTHOR]

Published
2024
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98. Prevalence of infectious diseases, immunity to vaccine-preventable diseases and chronic medical conditions among Ukrainian refugees in Germany – A cross sectional study from the German Network University Medicine (NUM).

Author

Brinkmann, Folke, Friedrichs, Anette, Behrens, Georg MN, Behrens, Pia, Berner, Reinhard, Caliebe, Amke, Denkinger, Claudia M., Giesbrecht, Katharina, Gussew, Alexander, Hoffmann, Anna Theresa, Hojenski, Leonhard, Hovardovska, Olga, Dopfer-Jablonka, Alexandra, Kaasch, Achim J., Kobbe, Robin, Kraus, Monika, Lindner, Andreas, Maier, Christoph, Mitrov, Lazar, and Nauck, Matthias

Abstract

Vulnerability to infectious diseases in refugees is dependent on country of origin, flight routes, and conditions. Information on specific medical needs of different groups of refugees is lacking. We assessed the prevalence of infectious diseases, immunity to vaccine-preventable diseases, and chronic medical conditions in children, adolescents, and adult refugees from Ukraine who arrived in Germany in 2022. Using different media, we recruited Ukrainian refugees at 13 sites between 9–12/2022. An antigen test for acute respiratory syndrome coronavirus type 2 (SARS-CoV-2) infection, serologies for a range of vaccine-preventable diseases, as well as interferon gamma release assays (IGRAs) for tuberculosis (TB), and SARS-CoV-2 were performed. We assessed personal and family history of chronic medical conditions, infectious diseases, vaccination status, and conditions during migration. Overall, 1793 refugees (1401 adults and 392 children/adolescents) were included. Most participants were females (n = 1307; 72·3%) and from Eastern or Southern Ukraine. TB IGRA was positive in 13% (n = 184) of the adults and in 2% (n = 7) of the children. Serology-based immunological response was insufficient in approximately 21% (360/1793) of the participants for measles, 32% (572/1793) for diphtheria, and 74% (1289/1793) for hepatitis B. We show evidence of low serological response to vaccine-preventable infections and increased LTBI prevalence in Ukrainian refugees. These findings should be integrated into guidelines for screening and treatment of infectious diseases in migrants and refugees in Germany and Europe. Furthermore, low immunity for vaccine-preventable diseases in Ukrainians independent of their refugee status, calls for tailor-made communication efforts. [ABSTRACT FROM AUTHOR]

Published
2024
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99. Late treatment-related mortality versus competing causes of death after allogeneic transplantation for myelodysplastic syndromes and secondary acute myeloid leukemia

Author

Schetelig, Johannes, de Wreede, Liesbeth C., van Gelder, Michel, Koster, Linda, Finke, Jürgen, Niederwieser, Dietger, Beelen, Dietrich, Mufti, G. J., Platzbecker, Uwe, Ganser, Arnold, Heidenreich, Silke, Maertens, Johan, Socié, Gerard, Brecht, Arne, Stelljes, Matthias, Kobbe, Guido, Volin, Liisa, Nagler, Arnon, Vitek, Antonin, Luft, Thomas, Ljungman, Per, Yakoub-Agha, Ibrahim, Robin, Marie, and Kröger, Nicolaus

Published
2019
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100. HIV-Infektion im Kindesalter – Update

Author

Wintergerst, Uwe, Baumann, Ulrich, v. Both, Ulrich, Buchholz, Bernd, Feiterna-Sperling, Cornelia, Förster-Waldl, Elisabeth, Kobbe, Robin, Königs, Christoph, Maritz, Elke, Neubert, Jennifer, Niehues, Tim, Notheis, Gundula, and Paioni, Paolo

Published
2019
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