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198 results on '"Bien E"'

1. Infectious complications in children with malignant bone tumors: a multicenter nationwide study

Author

Czyzewski K, Galazka P, Zalas-Wiecek P, Gryniewicz-Kwiatkowska O, Gietka A, Semczuk K, Chelmecka-Wiktorczyk L, Zak I, Salamonowicz M, Fraczkiewicz J, Zajac-Spychala O, Bien E, Plonowski M, Wawrykow P, Pierlejewski F, Gamrot Z, Malas Z, Stolpa W, Musial J, and Styczynski J

Subjects
infectious complications, risk factors analysis, Ewing sarcoma, osteosarcoma, malignant bone tumor, children, Infectious and parasitic diseases, RC109-216
Abstract

Krzysztof Czyzewski,1 Przemyslaw Galazka,2 Patrycja Zalas-Wiecek,3 Olga Gryniewicz-Kwiatkowska,4 Agnieszka Gietka,4 Katarzyna Semczuk,5 Liliana Chelmecka-Wiktorczyk,6 Iwona Zak,7 Malgorzata Salamonowicz,8 Jowita Fraczkiewicz,8 Olga Zajac-Spychala,9 Ewa Bien,10 Marcin Plonowski,11 Pawel Wawrykow,12 Filip Pierlejewski,13 Zuzanna Gamrot,14 Zofia Malas,15 Weronika Stolpa,16 Jakub Musial,17 Jan Styczynski11Department of Pediatric Hematology and Oncology, Collegium Medicum, Nicolaus Copernicus University Torun, Bydgoszcz, Poland; 2Department of General and Oncological Surgery for Children and Adolescents, Collegium Medicum, Nicolaus Copernicus University Torun, Bydgoszcz, Poland; 3Department of Microbiology, Collegium Medicum, Nicolaus Copernicus University Torun, Bydgoszcz, Poland; 4Department of Oncology, Children’s Memorial Health Institute, Warsaw, Poland; 5Department of Microbiology, Children’s Memorial Health Institute, Warsaw, Poland; 6Department of Pediatric Oncology and Hematology, University Children’s Hospital, Jagiellonian University, Collegium Medicum, Krakow, Poland; 7Department of Microbiology, University Children’s Hospital, Jagiellonian University, Collegium Medicum, Krakow, Poland; 8Department of Pediatric Stem Cell Transplantation, Hematology and Oncology, Medical University, Wroclaw, Poland; 9Department of Pediatric Oncology, Hematology and Transplantology, University of Medical Sciences, Poznan, Poland; 10Department of Pediatrics, Hematology and Oncology, Medical University, Gdansk, Poland; 11Department of Pediatric Oncology and Hematology, Medical University, Bialystok, Poland; 12Department of Pediatrics and Pediatric Oncology, Pomeranian Medical University, Szczecin, Poland; 13Department of Pediatric Oncology and Hematology, Medical University, Lodz, Poland; 14Division of Pediatric Hematology and Oncology, Chorzow City Hospital, Chorzow, Poland; 15Division of Pediatric Hematology and Oncology, Children Hospital, Olsztyn, Poland; 16Division of Pediatric Oncology, Hematology and Chemotherapy, Department of Pediatric, Silesian Medical University, Katowice, Poland; 17Department of Pediatric Oncohematology, Medical Faculty University of Rzeszow, Clinical Provincial Hospital No. 2, Rzeszow, PolandObjectives: The analysis of epidemiology, risk factors and outcome of infections in children with malignant bone tumors (MBT) undergoing chemotherapy.Methods: In this retrospective nationwide multicenter cross-sectional study, a total number of 126 children with MBT including 70 with Ewing sarcoma (ES) and 56 with osteosarcoma (OSA) were screened for infections over a period of 72 consecutive months.Results: The risk of infection was 7.15-fold higher in patients with ES as compared to the OSA group, especially concerning bacterial infections (4.1-fold increase risk). Bacterial infections occurred in 74.3% patients with ES and in 41.1% with OSA. The median time from diagnosis to first infection was 4.9 months. 33.0% of bacterial episodes were diagnosed as bloodstream (BSI), 31.1% as gastrointestinal tract, 30.1% as urinary tract infection. Infection-related mortality (IRM) from bacterial infection was 6% and 15% in ES and OSA patients, respectively. Cumulative incidence was 7.1% for invasive fungal disease and 6.3% for viral infections. The only significant risk factor for IRM was time to infection ≥5 months since the beginning of chemotherapy. All patients who have died from infection had BSI and were in neutropenia.Conclusions: Infections in the children with MBT in our study occurred with high frequency, especially in patients with ES. The most frequent were bacterial infections, while fungal and viral infections were episodic. Among the bacterial infections, bloodstream, urinary tract and gastrointestinal tract infections occurred with similar frequency. All deceased patients died due to BSI. Bacterial infection occurring ≥5 months since the beginning of chemotherapy was a risk factor for death.Keywords: infectious complications, risk factors analysis, Ewing sarcoma, osteosarcoma, malignant bone tumor, children

Published
2019

2. Global effort to evacuate Ukrainian children with cancer and blood disorders who have been affected by war

Author

Agulnik, A, Kizyma, R, Salek, M, Wlodarski, M, Pogorelyy, M, Oszer, A, Yakimkova, T, Nogovitsyna, Y, Dutkiewicz, M, Dalle, J, Dirksen, U, Eggert, A, Fernandez-Teijeiro, A, Greiner, J, Kraal, K, Mueller, A, Sramkova, L, Zecca, M, Wise, P, Mlynarski, W, Avula, M, Adyrov, M, Berlanga, P, Blackwood, C, Bouffet, E, Czauderna, P, de Koning, L, dos Reis Farinha, N, Foster, W, Graetz, D, Gupta, S, Holter, W, Hough, R, Kliuchkivska, K, Kolenova, A, Kolodrubiec, J, Moreira, D, Mukkada, S, Mykychak, I, Raciborska, A, Salman, Z, Sopilnyak, A, Tyupa, S, Vinitsky, A, Wobst, N, Miller, B, Rasul, S, Rodriguez-Galindo, C, Alanbousi, I, Alexander, S, Apel, A, Bal, W, Balwierz, W, Basset-Salom, L, Bastardo Blanco, D, Bauer, K, Bayazitov, I, Bhakta, N, Bien, E, Bieniek, K, Blair, S, Bodak, K, Bordeianu, I, Braganca, J, Bucurenci, M, Budny, E, Budzyn, A, Bumgardner, C, Burditt, R, Burnside Clapp, V, Bykov, V, Canete, A, Carnelli, M, Cela, E, Cepowska, Z, Chaber, R, Cherner-Drieux, A, Chubata, M, Clough, H, Czernicka - Siwecka, J, Czyzewski, K, Dashchakovska, O, Dembowska-Baginska, B, Derwich, K, Dommett, R, Dorosh, O, Drabko, K, Dragomir, M, Dworzak, M, Dyma, S, Earl, J, English, M, Evseev, D, Farren, B, Fedyk, N, Ferneza, S, Fox Irwin, L, Galazkowski, R, Ganieva, G, Garanzha, V, Gelman, M, Godzinski, J, Goeres, A, Golban, R, Griksaitis, M, Hampel, M, Hastings, S, Heenen, D, Hill, M, Holiuk, I, Hutnik, L, Irga-Jaworska, N, Istomin, O, Janczar, S, Kacharian, A, Kalwak, K, Karolczyk, G, Karpenko, N, Katsubo, H, Kaznowska, B, Kentsis, A, Ketteler, P, Kienesberger, A, Kiselev, R, Kizyma, Z, Klymniuk, H, Kostiuk, Y, Kowalik, T, Kozlova, O, Kozubenko, V, Kramar, T, Krawczuk-Rybak, M, Kulemzina, I, Kurkowska, P, Kuzyk, A, Ladenstein, R, Laguna, P, Lassaletta, A, Lehmberg, K, Leontieva, O, Liashenko, S, Loizou, L, Lucchetta, S, Lupo, M, Lysytsia, L, Lysytsia, O, Machnik, K, Mainland, J, Matczak, K, Matysiak, M, Mayeur, P, Minervina, A, Mishkova, V, Mizia-Malarz, A, Morales La Madrid, A, Moreno, L, Moskvin, V, Muszynska-Roslan, K, Nelson, A, Ociepa, T, Oltolini, S, Onipko, N, Pappas, A, Patel, A, Patrahau, A, Pauley, J, Pavlenko, Y, Pavlovych, A, Peregud-Pogorzelski, J, Perek-Polnik, M, Perez, V, Perez-Martinez, A, Pikman, Y, Pitozzi, G, Portugal, R, Posternak, V, Prete, A, Pritchard-Jones, K, Radaelli, A, Reeves, T, Reinhardt, D, Reshetnyak, A, Rider, A, Rizzari, C, Rizzi, D, Rodriguez Hermosillo, K, Ronenko, O, Rostowska, A, Rudko, L, Sakaan, F, Sakhar, N, Savva, N, Scaccaglia, D, Schaeffer, E, Schneider, C, Scobie, N, Semeniuk, O, Shevchyk, R, Shuler, A, Shvets, S, Skoczen, S, Smeal, W, Sokolowski, I, Sonkin, A, Stepanjuk, A, Spota, A, Sterba, J, Styczynski, J, Svintsova, O, Synyuta, A, Szczepanski, T, Szczucinski, P, Szmyd, B, Tasso Cereceda, M, Teliuk, A, Tomanek, I, Topping, P, Torrent, M, Trelinska, J, Troyanovska, O, Trubnikova, E, Tsurkan, L, Tsymbalyuk-Voloshyn, I, Urasinski, T, Urbanek-Dadela, A, Vasilieva, N, Vasilyeva, A, Verdu-Amoros, J, Vilcu-Bajurean, N, Vinitsky, L, Volpe, G, Vorobel, O, Wachowiak, J, Wasiak, M, Wiedower, L, Wuenschel, L, Wysocki, M, Yurieva, M, Zagurska, A, Zakharenko, S, Zakharenko, A, Zapotochna, K, Zawitkowska, J, Agulnik A., Kizyma R., Salek M., Wlodarski M. W., Pogorelyy M., Oszer A., Yakimkova T., Nogovitsyna Y., Dutkiewicz M., Dalle J. -H., Dirksen U., Eggert A., Fernandez-Teijeiro A., Greiner J., Kraal K., Mueller A., Sramkova L., Zecca M., Wise P. H., Mlynarski W., Avula M., Adyrov M. V., Berlanga P., Blackwood C. A., Bouffet E., Czauderna P. S., de Koning L. A., dos Reis Farinha N. J., Foster W. B., Graetz D. E., Gupta S., Holter W., Hough R. E., Kliuchkivska K., Kolenova A., Kolodrubiec J., Moreira D. C., Mukkada S. T., Mykychak I., Raciborska A., Salman Z. S., Sopilnyak A., Tyupa S., Vinitsky A., Wobst N. M., Miller B. A., Rasul S. S., Rodriguez-Galindo C., Alanbousi I., Alexander S. W., Apel A., Bal W. A., Balwierz W. A., Basset-Salom L., Bastardo Blanco D., Bauer K. J., Bayazitov I. T., Bhakta N. H., Bien E. I., Bieniek K. A., Blair S. J., Bodak K. I., Bordeianu I. M., Braganca J. M., Bucurenci M. S., Budny E. B., Budzyn A., Bumgardner C. C., Burditt R. N., Burnside Clapp V. G., Bykov V., Canete A., Carnelli M., Cela E., Cepowska Z. P., Chaber R., Cherner-Drieux A., Chubata M., Clough H. M., Czernicka - Siwecka J., Czyzewski K., Dashchakovska O., Dembowska-Baginska B. M., Derwich K., Dommett R., Dorosh O., Drabko K. A., Dragomir M. D., Dworzak M., Dyma S., Earl J. D., English M. W., Evseev D. A., Farren B. S., Fedyk N., Ferneza S., Fox Irwin L. E., Galazkowski R. M., Ganieva G., Garanzha V., Gelman M. S., Godzinski J. K., Goeres A. F., Golban R., Griksaitis M. J., Hampel M. A., Hastings S. G., Heenen D. L., Hill M. C., Holiuk I., Hutnik L. M., Irga-Jaworska N., Istomin O., Janczar S. L., Kacharian A., Kalwak K., Karolczyk G. M., Karpenko N. M., Katsubo H., Kaznowska B. J., Kentsis A., Ketteler P., Kienesberger A., Kiselev R., Kizyma Z., Klymniuk H., Kostiuk Y., Kowalik T., Kozlova O., Kozubenko V., Kramar T., Krawczuk-Rybak M., Kulemzina I., Kurkowska P., Kuzyk A. S., Ladenstein R. L., Laguna P. J., Lassaletta A., Lehmberg K., Leontieva O., Liashenko S., Loizou L. G., Lucchetta S. A., Lupo M. W., Lysytsia L., Lysytsia O., Machnik K. A., Mainland J. A., Matczak K. E., Matysiak M. J., Mayeur P., Minervina A. A., Mishkova V., Mizia-Malarz A. J., Morales La Madrid A., Moreno L., Moskvin V. P., Muszynska-Roslan K. M., Nelson A. J., Ociepa T., Oltolini S., Onipko N., Pappas A., Patel A. B., Patrahau A., Pauley J. L., Pavlenko Y., Pavlovych A., Peregud-Pogorzelski J., Perek-Polnik M., Perez V., Perez-Martinez A., Pikman Y., Pitozzi G., Portugal R. G., Posternak V. V., Prete A., Pritchard-Jones K., Radaelli A., Reeves T., Reinhardt D., Reshetnyak A. V., Rider A. J., Rizzari C., Rizzi D., Rodriguez Hermosillo K. G., Ronenko O., Rostowska A. O., Rudko L., Sakaan F. M., Sakhar N., Savva N. N., Scaccaglia D., Schaeffer E. H., Schneider C. U., Scobie N., Semeniuk O., Shevchyk R., Shuler A. I., Shvets S., Skoczen S. P., Smeal W. J., Sokolowski I., Sonkin A. A., Stepanjuk A. I., Spota A., Sterba J., Styczynski J., Svintsova O., Synyuta A. V., Szczepanski T., Szczucinski P. K., Szmyd B. M., Tasso Cereceda M., Teliuk A., Tomanek I., Topping P., Torrent M., Trelinska J., Troyanovska O., Trubnikova E., Tsurkan L. G., Tsymbalyuk-Voloshyn I., Urasinski T. F., Urbanek-Dadela A., Vasilieva N., Vasilyeva A., Verdu-Amoros J., Vilcu-Bajurean N., Vinitsky L., Volpe G., Vorobel O., Wachowiak J. T., Wasiak M. S., Wiedower L. A., Wuenschel L. I., Wysocki M. S., Yurieva M., Zagurska A., Zakharenko S. S., Zakharenko A. V., Zapotochna K., Zawitkowska J. E., Agulnik, A, Kizyma, R, Salek, M, Wlodarski, M, Pogorelyy, M, Oszer, A, Yakimkova, T, Nogovitsyna, Y, Dutkiewicz, M, Dalle, J, Dirksen, U, Eggert, A, Fernandez-Teijeiro, A, Greiner, J, Kraal, K, Mueller, A, Sramkova, L, Zecca, M, Wise, P, Mlynarski, W, Avula, M, Adyrov, M, Berlanga, P, Blackwood, C, Bouffet, E, Czauderna, P, de Koning, L, dos Reis Farinha, N, Foster, W, Graetz, D, Gupta, S, Holter, W, Hough, R, Kliuchkivska, K, Kolenova, A, Kolodrubiec, J, Moreira, D, Mukkada, S, Mykychak, I, Raciborska, A, Salman, Z, Sopilnyak, A, Tyupa, S, Vinitsky, A, Wobst, N, Miller, B, Rasul, S, Rodriguez-Galindo, C, Alanbousi, I, Alexander, S, Apel, A, Bal, W, Balwierz, W, Basset-Salom, L, Bastardo Blanco, D, Bauer, K, Bayazitov, I, Bhakta, N, Bien, E, Bieniek, K, Blair, S, Bodak, K, Bordeianu, I, Braganca, J, Bucurenci, M, Budny, E, Budzyn, A, Bumgardner, C, Burditt, R, Burnside Clapp, V, Bykov, V, Canete, A, Carnelli, M, Cela, E, Cepowska, Z, Chaber, R, Cherner-Drieux, A, Chubata, M, Clough, H, Czernicka - Siwecka, J, Czyzewski, K, Dashchakovska, O, Dembowska-Baginska, B, Derwich, K, Dommett, R, Dorosh, O, Drabko, K, Dragomir, M, Dworzak, M, Dyma, S, Earl, J, English, M, Evseev, D, Farren, B, Fedyk, N, Ferneza, S, Fox Irwin, L, Galazkowski, R, Ganieva, G, Garanzha, V, Gelman, M, Godzinski, J, Goeres, A, Golban, R, Griksaitis, M, Hampel, M, Hastings, S, Heenen, D, Hill, M, Holiuk, I, Hutnik, L, Irga-Jaworska, N, Istomin, O, Janczar, S, Kacharian, A, Kalwak, K, Karolczyk, G, Karpenko, N, Katsubo, H, Kaznowska, B, Kentsis, A, Ketteler, P, Kienesberger, A, Kiselev, R, Kizyma, Z, Klymniuk, H, Kostiuk, Y, Kowalik, T, Kozlova, O, Kozubenko, V, Kramar, T, Krawczuk-Rybak, M, Kulemzina, I, Kurkowska, P, Kuzyk, A, Ladenstein, R, Laguna, P, Lassaletta, A, Lehmberg, K, Leontieva, O, Liashenko, S, Loizou, L, Lucchetta, S, Lupo, M, Lysytsia, L, Lysytsia, O, Machnik, K, Mainland, J, Matczak, K, Matysiak, M, Mayeur, P, Minervina, A, Mishkova, V, Mizia-Malarz, A, Morales La Madrid, A, Moreno, L, Moskvin, V, Muszynska-Roslan, K, Nelson, A, Ociepa, T, Oltolini, S, Onipko, N, Pappas, A, Patel, A, Patrahau, A, Pauley, J, Pavlenko, Y, Pavlovych, A, Peregud-Pogorzelski, J, Perek-Polnik, M, Perez, V, Perez-Martinez, A, Pikman, Y, Pitozzi, G, Portugal, R, Posternak, V, Prete, A, Pritchard-Jones, K, Radaelli, A, Reeves, T, Reinhardt, D, Reshetnyak, A, Rider, A, Rizzari, C, Rizzi, D, Rodriguez Hermosillo, K, Ronenko, O, Rostowska, A, Rudko, L, Sakaan, F, Sakhar, N, Savva, N, Scaccaglia, D, Schaeffer, E, Schneider, C, Scobie, N, Semeniuk, O, Shevchyk, R, Shuler, A, Shvets, S, Skoczen, S, Smeal, W, Sokolowski, I, Sonkin, A, Stepanjuk, A, Spota, A, Sterba, J, Styczynski, J, Svintsova, O, Synyuta, A, Szczepanski, T, Szczucinski, P, Szmyd, B, Tasso Cereceda, M, Teliuk, A, Tomanek, I, Topping, P, Torrent, M, Trelinska, J, Troyanovska, O, Trubnikova, E, Tsurkan, L, Tsymbalyuk-Voloshyn, I, Urasinski, T, Urbanek-Dadela, A, Vasilieva, N, Vasilyeva, A, Verdu-Amoros, J, Vilcu-Bajurean, N, Vinitsky, L, Volpe, G, Vorobel, O, Wachowiak, J, Wasiak, M, Wiedower, L, Wuenschel, L, Wysocki, M, Yurieva, M, Zagurska, A, Zakharenko, S, Zakharenko, A, Zapotochna, K, Zawitkowska, J, Agulnik A., Kizyma R., Salek M., Wlodarski M. W., Pogorelyy M., Oszer A., Yakimkova T., Nogovitsyna Y., Dutkiewicz M., Dalle J. -H., Dirksen U., Eggert A., Fernandez-Teijeiro A., Greiner J., Kraal K., Mueller A., Sramkova L., Zecca M., Wise P. H., Mlynarski W., Avula M., Adyrov M. V., Berlanga P., Blackwood C. A., Bouffet E., Czauderna P. S., de Koning L. A., dos Reis Farinha N. J., Foster W. B., Graetz D. E., Gupta S., Holter W., Hough R. E., Kliuchkivska K., Kolenova A., Kolodrubiec J., Moreira D. C., Mukkada S. T., Mykychak I., Raciborska A., Salman Z. S., Sopilnyak A., Tyupa S., Vinitsky A., Wobst N. M., Miller B. A., Rasul S. S., Rodriguez-Galindo C., Alanbousi I., Alexander S. W., Apel A., Bal W. A., Balwierz W. A., Basset-Salom L., Bastardo Blanco D., Bauer K. J., Bayazitov I. T., Bhakta N. H., Bien E. I., Bieniek K. A., Blair S. J., Bodak K. I., Bordeianu I. M., Braganca J. M., Bucurenci M. S., Budny E. B., Budzyn A., Bumgardner C. C., Burditt R. N., Burnside Clapp V. G., Bykov V., Canete A., Carnelli M., Cela E., Cepowska Z. P., Chaber R., Cherner-Drieux A., Chubata M., Clough H. M., Czernicka - Siwecka J., Czyzewski K., Dashchakovska O., Dembowska-Baginska B. M., Derwich K., Dommett R., Dorosh O., Drabko K. A., Dragomir M. D., Dworzak M., Dyma S., Earl J. D., English M. W., Evseev D. A., Farren B. S., Fedyk N., Ferneza S., Fox Irwin L. E., Galazkowski R. M., Ganieva G., Garanzha V., Gelman M. S., Godzinski J. K., Goeres A. F., Golban R., Griksaitis M. J., Hampel M. A., Hastings S. G., Heenen D. L., Hill M. C., Holiuk I., Hutnik L. M., Irga-Jaworska N., Istomin O., Janczar S. L., Kacharian A., Kalwak K., Karolczyk G. M., Karpenko N. M., Katsubo H., Kaznowska B. J., Kentsis A., Ketteler P., Kienesberger A., Kiselev R., Kizyma Z., Klymniuk H., Kostiuk Y., Kowalik T., Kozlova O., Kozubenko V., Kramar T., Krawczuk-Rybak M., Kulemzina I., Kurkowska P., Kuzyk A. S., Ladenstein R. L., Laguna P. J., Lassaletta A., Lehmberg K., Leontieva O., Liashenko S., Loizou L. G., Lucchetta S. A., Lupo M. W., Lysytsia L., Lysytsia O., Machnik K. A., Mainland J. A., Matczak K. E., Matysiak M. J., Mayeur P., Minervina A. A., Mishkova V., Mizia-Malarz A. J., Morales La Madrid A., Moreno L., Moskvin V. P., Muszynska-Roslan K. M., Nelson A. J., Ociepa T., Oltolini S., Onipko N., Pappas A., Patel A. B., Patrahau A., Pauley J. L., Pavlenko Y., Pavlovych A., Peregud-Pogorzelski J., Perek-Polnik M., Perez V., Perez-Martinez A., Pikman Y., Pitozzi G., Portugal R. G., Posternak V. V., Prete A., Pritchard-Jones K., Radaelli A., Reeves T., Reinhardt D., Reshetnyak A. V., Rider A. J., Rizzari C., Rizzi D., Rodriguez Hermosillo K. G., Ronenko O., Rostowska A. O., Rudko L., Sakaan F. M., Sakhar N., Savva N. N., Scaccaglia D., Schaeffer E. H., Schneider C. U., Scobie N., Semeniuk O., Shevchyk R., Shuler A. I., Shvets S., Skoczen S. P., Smeal W. J., Sokolowski I., Sonkin A. A., Stepanjuk A. I., Spota A., Sterba J., Styczynski J., Svintsova O., Synyuta A. V., Szczepanski T., Szczucinski P. K., Szmyd B. M., Tasso Cereceda M., Teliuk A., Tomanek I., Topping P., Torrent M., Trelinska J., Troyanovska O., Trubnikova E., Tsurkan L. G., Tsymbalyuk-Voloshyn I., Urasinski T. F., Urbanek-Dadela A., Vasilieva N., Vasilyeva A., Verdu-Amoros J., Vilcu-Bajurean N., Vinitsky L., Volpe G., Vorobel O., Wachowiak J. T., Wasiak M. S., Wiedower L. A., Wuenschel L. I., Wysocki M. S., Yurieva M., Zagurska A., Zakharenko S. S., Zakharenko A. V., Zapotochna K., and Zawitkowska J. E.

Published
2022

3. EP06.49: Prenatal detection of large complicated lympho‐venous malformation: effective and safe maternal sirolimus treatment.

Author

Mazur, N.K., Klosowska, A., Styczewska, M., Gluszkiewicz, L., Krawczyk, M.A., Adamski, P., Matwiejczyk, L., Domzalska‐Popadiuk, I., Grzybowska, M.E., Wydra, D.G., and Bien, E.

Subjects
FETAL ultrasonic imaging, CORD blood, CESAREAN section, LYMPHATIC abnormalities, RAPAMYCIN
Abstract

This article discusses the use of prenatal sirolimus treatment for congenital lympho-venous malformations (CLVMs), which are rare vascular anomalies that can be life-threatening. The study reports a case of a massive CLVM detected during second trimester screening, which led to the decision to administer off-label maternal oral sirolimus. The treatment was continued until delivery, and the child has continued sirolimus postnatally with a reduction in the CLVM. The article highlights the effectiveness and safety of sirolimus treatment for high-risk CLVMs and emphasizes the importance of interdisciplinary cooperation in providing this therapy. [Extracted from the article]

Published
2024
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8. Inequalities in diagnosis and registration of pediatric very rare tumors: a European study on pleuropulmonary blastoma

Author

Grigoletto, V., primary, Tagarelli, A., additional, Sparber-Sauer, M., additional, Koscielniak, E., additional, Orbach, D., additional, Duplan, M., additional, Stachowicz-Stencel, T., additional, Bien, E., additional, López-Almaraz, R., additional, Ben-Ami, T., additional, Pourtsidis, A., additional, Österlundh, G., additional, Mazanek, P., additional, Zsiros, J., additional, Farinha, N., additional, Ferrari, A., additional, and Bisogno, G., additional

Published
2020
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9. Defining and listing very rare cancers of paediatric age: consensus of the Joint Action on Rare Cancers in cooperation with the European Cooperative Study Group for Pediatric Rare Tumors

Author

Ferrari, A, Brecht, I, Gatta, G, Schneider, D, Orbach, D, Cecchetto, G, Godzinski, J, Reguerre, Y, Bien, E, Stachowicz-Stencel, T, Ost, M, Magni, C, Kearns, P, Vassal, G, Massimino, M, Biondi, A, Bisogno, G, Trama, A, Brecht, IB, Schneider, DT, Stachowicz-Stencel, Te, Ferrari, A, Brecht, I, Gatta, G, Schneider, D, Orbach, D, Cecchetto, G, Godzinski, J, Reguerre, Y, Bien, E, Stachowicz-Stencel, T, Ost, M, Magni, C, Kearns, P, Vassal, G, Massimino, M, Biondi, A, Bisogno, G, Trama, A, Brecht, IB, Schneider, DT, and Stachowicz-Stencel, Te

Abstract

Although all tumours are rare in childhood, there are some particularly rare paediatric cancers which have not benefited from advances made by the international paediatric oncology network. To establish a shared definition and produce a list of these entities, the European Union Joint Action on Rare Cancers (JARC) promoted a consensus effort. The definition was based on the incidence rates estimated using the information network on rare cancers (RARECAREnet) database, pooling data from 94 population-based cancer registries and 27 countries. The RARECAREnet list of cancers was used to estimate the incidence rates. This list groups cancers by combining the International Classification of Diseases for Oncology, third edition, morphology and topography codes. According to the consensus, very rare paediatric cancers were identified as those with an annual incidence <2/1000000 and corresponded to 11% of all cancers in patients aged 0–14 years. Two subgroups were identified: tumour types typical of childhood (i.e. hepatoblastoma, pleuropulmonary blastoma, pancreatoblastoma) and those typical of adult age (i.e. carcinomas, melanoma). The threshold of 2/1000000 could also be adopted in populations aged 0–19 years: in this case, three tumour types had an incidence rate which was >2/1000000 (i.e. thyroid and testicular cancers and skin melanoma), but the consensus experts considered them as ‘very rare’ according to their clinical needs (e.g. shortage of knowledge and clinical expertise as the other rare paediatric cancers). The JARC consensus produced a definition and a list of very rare paediatric cancers which may represent a starting point for prioritising research on these tumours, based on data and patients’ clinical needs.

Published
2019

23. Diagnosis and Treatment of Renal Cell Carcinoma in Children: A Report from the Polish Pediatric Rare Tumor Study Group

Author

Stachowicz-Stencel, T., primary, Bien, E., additional, Balcerska, A., additional, Godzinski, J., additional, Synakiewicz, A., additional, Perek-Polnik, M., additional, Kurylak, A., additional, Pietras, W., additional, Kuzmicz, M., additional, Mizia-Malarz, A., additional, Rybczynska, A., additional, and Nurzynska-Flak, J., additional

Published
2011
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28. Performance Expectancy, Effort Expectancy, Social Influence and Facilitating Conditions are Factors that Influence Rural Health Workers in the Use of Wireless Access for Health and Perception of Behavior of their Pregnant Patients

Author

Bien Eli Nillos

Subjects
health information technology, public health, behavioral science, unified theory of acceptance and use of technology, descriptive design, philippines, Social Sciences, Education
Abstract

Technology has been integrated into various levels at the rural health systems by both government and non-government agencies. The study aimed to identify the factors influencing adoption of Wireless Access for Health (WAH) by rural health workers and how it influences the perception of health workers regarding the health-seeking behavior of their clients. Some selected rural health workers from 11 Philippine municipalities responded to a survey adopted from the Unified Theory of Acceptance and Use of Technology (UTAUT) questionnaire. Correlation analysis and one-way analysis of variance were used to test the relationships between the variables. Results showed that to establish high adoption of WAH among rural health workers, interventions must ignite appreciation among health workers as regard to the importance and relevance of the technology to their work (performance expectancy). Support from the health managers and local chief executives of the municipalities, both administrative (social influence) and logistical (facilitating conditions) are necessary for them to adopt the technology. There is also perceived increase in facilities-based deliveries and deliveries by skilled birth attendants in the municipalities after installation of WAH.

Published
2016
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29. Serum level of soluble interleukin-2 receptor correlates with the clinical course and activity of Wilms’ tumour and soft tissue sarcomas in children

Author

Bien, E., Balcerska, A., and Kuchta, G.

Abstract

Wilms’ tumour (WT) and soft tissue sarcomas (SA) in children lack reliable biochemical markers. This study was carried out to determine the clinical significance of serum soluble interleukin-2 receptor (sIL-2R) in the diagnostics and treatment monitoring of children with WT and SA. The study included 48 children: ten with WT, eight with SA and 30 healthy controls. The sIL-2R levels (ELISA) and rates of elevated sIL-2R values were estimated prospectively at diagnosis and in complete remission during treatment and after therapy. As the dependence on age was determined, the levels of sIL-2R were expressed as multiplications of the upper value of the normal range for a particular age (xN). Median pretreatment levels of sIL-2R in patients exceeded those of healthy controls (1.79 xN for WT and 1.53 for SA vs. 0.61 for controls; p<0.001) as did the rates of elevated sIL-2R values (80% of WT and 87.5% of SA patients vs. 0% of controls). Good response to therapy was paralleled by a significant decline of pretreatment sIL-2R levels and its elevated rates. Thus, sIL-2R determination may be of some value in the diagnostics and treatment monitoring of childhood WT and SA.

Published
2007
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32. Curcuminoids and ω-3 fatty acids with anti-oxidants potentiate cytotoxicity of natural killer cells against pancreatic ductal adenocarcinoma cells and inhibit interferon γ production

Author

Milan eFiala, Ramesh eHalder, Anasheh eAlmasi, Bien eSagong, Jessica eLeung, and Anahid eJewett

Subjects
Curcumin, Pancreatic Cancer, Natural Killer cells, Interferon-γ, ω-3 fatty acids, Physiology, QP1-981
Abstract

Pancreatic cancer has a poor prognosis attributed in part to immune suppression and deactivation of natural killer (NK) cells. Curcuminoids have a potential for improving the therapy of pancreatic cancer given promising results in cancer models and a clinical trial, but their oral absorption is limited. Our objective in this study is to show curcuminoid anti-oncogenic effects alone and together with human NK cells. We tested curcuminoids in an emulsion of ω-3 fatty acids and anti-oxidants (Smartfish) regarding their direct cytocidal effect and enhancement of the cytocidal activity of NK cells in pancreatic ductal adenocarcinoma (PDAC) cells (Mia Paca 2 and L3.6). Curcuminoids (at > 10 microM) with ω-3 fatty acids and anti-oxidants or with the lipidic mediator resolvin D1 (RvD1) (26 nM) induced high caspase-3 activity in PDAC cells. Importantly, curcuminoids with ω-3 fatty acids and anti-oxidants or with RvD1 significantly potentiated NK cell cytocidal function and protected them against degradation. In a co-culture of cancer cells with NK cells, interferon-γ ( IFN-γ) production by NK cells was not altered by ω-3 fatty acids with anti-oxidants or by RvD1 but was inhibited by curcuminoids. The inhibition was not eliminated by ω-3 fatty acids or RvD1 but was relieved by removing curcuminoids after adding NK cells. In conclusion, curcuminoids with ω-3 fatty acids and anti-oxidants or with RvD1 have increased cytotoxic activity on PDAC cells alone and with NK cells. The effects of curcuminoids with ω-3 fatty acids and anti-oxidants on pancreatic cancer will be investigated in a mouse model with humanized immune system.

Published
2015
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33. Pediatric Mesothelioma Tumors: The European Expert Group Contribution

Author

Orbach, D., Andre, N., Brecht, I., Lopez Almaraz, R., Ben Ami, T., Wermersch, S., Carton, M., Virgone, C., Bisogno, G., Schneider, D., Bajciova, V., Reguerre, Y., Galateau-Salle, F., Teresa Stachowicz-Stencel, Dvir, R., Rees, H., Bien, E., Ferrari, A., and Ben-Arush, M.

36. Infectious complications in children treated for Non-Hodgkin lymphomas - Results of a multicenter nationwide study

Author

Zajac-Spychala, O., Wachowiak, J., Czyzewski, K., Dziedzic, M., Wysocki, M., Zalas-Wiecek, P., Szmydki-Baran, A., Hutnik, L., Matysiak, M., Malas, Z., Badowska, W., Gryniewicz-Kwiatkowska, O., Czajnska-Deptula, A., Kulicka, E., Dembowska-Baginska, B., Semczuk, K., Dzierzanowska-Fangrat, K., Bartnik, M., Ociepa, T., Urasinski, T., Fraczkiewicz, J., Salamonowicz, M., Kazanowska, B., Wrobel, G., Chybicka, A., Irga-Jaworska, N., Bien, E., Drozynska, E., Chelmecka-Wiktorczyk, L., Balwierz, W., Zak, I., Pierlejewski, F., Mlynarski, W., Urbanek-Dadela, A., Karolczyk, G., Stolpa, W., Sobol-Milejska, G., Plonowski, M., Maryna Krawczuk-Rybak, Musial, J., Chaber, R., Gamrot-Pyka, Z., Woszczyk, M., Tomaszewska, R., Szczepanski, T., Kowalczyk, J., and Styczynski, J.

37. Clinical and Histological Analysis of Children and Adolescents with Ovarian Germ Cell Tumours in Poland

Author

Adamkiewicz-Drozynska, E., Polczynska, K., Stefanowicz, J., Jezierska, M., Izycka-Swieszewska, E., Rapala, M., Bilska, K., Chelmecka-Hanusiewicz, L., Debski, R., Karolczyk, G., Leszczynskas, E., Mizia-Malarz, A., Nurzynska-Flak, J., Rosinska, B., Wachowiak-Szajdak, K., Malinowska, I., Trelinska, J., Wawrykow, P., Woszczyk, M., Pobudejska, A., Wieckowska, J., Balwierz, W., Dembowska-Baginska, B., Kowalczyk, J., Krawczuk-Rybak, M., Michał Matysiak, Mlynarski, W., Sobol-Milejska, G., Szczepanski, T., Peregud-Pogorzelski, J., Urasinski, T., Wachowiak, J., Wysocki, M., Chybicka, A., and Bien, E.

46. Neurofibromatosis Type 1 (Recklinghausen's Disease)

Author

Dambrosia Jm, Myers S, M. A. Zasloff, Bien E, Anita Pikus, Roswell Eldridge, Muriel I. Kaiser-Kupfer, Sandra Schlesinger, Dilys M. Parry, and Denckla Mb

Subjects
medicine.medical_specialty, Intelligence quotient, business.industry, Poison control, Cognition, Audiology, medicine.disease, Central nervous system disease, McNemar's test, medicine, Physical therapy, Specific Learning Disorder, Sibling, Neurofibromatosis, business
Abstract

• Neurologic and cognitive function in neurofibromatosis type 1 (NF1) were assessed in a controlled pilot study of 13 pairs of siblings aged 6 to 27 years. One subject in each pair was affected with NF1, and the other, the control subject, was unaffected. Subjects with evidence of focal central nervous system disease were excluded. The 13 subjects with NF1 had no excess of mental retardation, attention-deficit disorder, or specific learning disorders (using Wilcoxon's Signed Rank Test and McNemar's Test for Symmetry). These subjects, however, had significantly higher scores for subtle neurologic abnormalities (21 vs 6) and significantly lower full-scale IQ scores (94 vs 105) than their unaffected siblings. The IQ scores of the affected subjects were not clustered at the lower end of the scale but showed a slight downward shift in distribution compared with those of their siblings. In addition, a visual-spatial orientation deficit was present in eight of nine affected subjects so evaluated. The findings suggest that subjects with NF1 have a widespread alteration of the brain during development that manifests as one or more specific types of neuropsychologic deficits. ( AJDC . 1989;143:833-837)

Published
1989
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47. Global effort to evacuate Ukrainian children with cancer and blood disorders who have been affected by war

Author

Asya Agulnik, Roman Kizyma, Marta Salek, Marcin W Wlodarski, Mikhail Pogorelyy, Aleksandra Oszer, Taisiya Yakimkova, Yuliya Nogovitsyna, Malgorzata Dutkiewicz, Jean-Hugues Dalle, Uta Dirksen, Angelika Eggert, Ana Fernández-Teijeiro, Jeanette Greiner, Kathelijne Kraal, Alexandra Mueller, Lucie Sramkova, Marco Zecca, Paul H Wise, Wojciech Mlynarski, Meghana Avula, Mykhaylo V Adyrov, Pablo Berlanga, Christopher Andrew Blackwood, Eric Bouffet, Piotr Stefan Czauderna, Linda A de Koning, Nuno Jorge dos Reis Farinha, Whitney Baer Foster, Dylan Elizabeth Graetz, Sumit Gupta, Wolfgang Holter, Rachael Emma Hough, Khrystyna Kliuchkivska, Alexandra Kolenova, Julia Kołodrubiec, Daniel C Moreira, Sheena Teresa Mukkada, Iryna Mykychak, Anna Raciborska, Zeena S Salman, Andriy Sopilnyak, Sergiy Tyupa, Anna Vinitsky, Natalia Margarete Wobst, Beth Anne Miller, Suheir Subhi Rasul, Carlos Rodriguez-Galindo, Inna Alanbousi, Sarah Weeks Alexander, Anna Apel, Wioletta Anna Bal, Walentyna Aniela Balwierz, Luisa Basset-Salom, Daniel Bastardo Blanco, Karolina Jadwiga Bauer, Ildar T Bayazitov, Nickhill Hitesh Bhakta, Ewa Iwona Bien, Katarzyna Anna Bieniek, Sally Jane Blair, Khrystyna Ihorivna Bodak, Irina Michael Bordeianu, Joao Maria Braganca, Mihaela Silvia Bucurenci, Elżbieta Beata Budny, Andrii Budzyn, Christopher Carl Bumgardner, Raina Nichole Burditt, Victoria Grace Burnside Clapp, Viacheslav Bykov, Adela Cañete, Monica Carnelli, Elena Cela, Zuzanna Paulina Cepowska, Radoslaw Chaber, Anna Cherner-Drieux, Mariya Chubata, Heidi M Clough, Jolanta Czernicka - Siwecka, Krzysztof Czyzewski, Olha Dashchakovska, Bozenna Malgorzata Dembowska-Baginska, Katarzyna Derwich, Rachel Dommett, Olha Dorosh, Katarzyna Anna Drabko, Monica Desiree Dragomir, Michael Dworzak, Sergii Dyma, Julian Darocus Earl, Martin William English, Dmitry A Evseev, Becky S Farren, Nataliia Fedyk, Severyn Ferneza, Leeanna Elizabeth Fox Irwin, Robert Maciej Gałązkowski, Galyna Ganieva, Vasylyna Garanzha, Marina S Gelman, Jan Krzysztof Godzinski, Anne Francoise Goeres, Rodica Golban, Michael J Griksaitis, Michal Andrzej Hampel, Sara Grace Hastings, Delphine Liliane Heenen, Marcela C Hill, Igor Holiuk, Lukasz Marek Hutnik, Ninela Irga-Jaworska, Oleksandr Istomin, Szymon Lech Janczar, Arman Kacharian, Krzysztof Kalwak, Grażyna Malgorzata Karolczyk, Nataliia Mikolaivna Karpenko, Halyna Katsubo, Bernarda Jadwiga Kaznowska, Alex Kentsis, Petra Ketteler, Anita Kienesberger, Roman Kiselev, Zoryana Kizyma, Hryhorii Klymniuk, Yuliia Kostiuk, Tomasz Kowalik, Olena Kozlova, Vladyslav Kozubenko, Tetyana Kramar, Maryna Krawczuk-Rybak, Irina Kulemzina, Paulina Kurkowska, Andriy S Kuzyk, Ruth Lydia Ladenstein, Pawel Jozef Laguna, Alvaro Lassaletta, Kai Lehmberg, Oksana Leontieva, Serhii Liashenko, Loizos G Loizou, Sonia Anna Lucchetta, Matthew William Lupo, Lesya Lysytsia, Oleksandr Lysytsia, Katarzyna Anna Machnik, Jeff A Mainland, Katarzyna Ewa Matczak, Michal Jacek Matysiak, Pierre Mayeur, Anastasia A Minervina, Volha Mishkova, Agnieszka Joanna Mizia-Malarz, Andres Morales La Madrid, Lucas Moreno, Vadim P Moskvin, Katarzyna Maria Muszyńska-Rosłan, Akoya Janae Nelson, Tomasz Ociepa, Stefano Oltolini, Nataliia Onipko, Andrew Pappas, Amit B Patel, Alina Patrahau, Jennifer L Pauley, Yehor Pavlenko, Andrij Pavlovych, Jarosław Peregud-Pogorzelski, Marta Perek-Polnik, Vanesa Perez, Antonio Perez-Martinez, Yana Pikman, Graziano Pitozzi, Rui Gentil Portugal, Victoria Vita Posternak, Arcangelo Prete, Kathy Pritchard-Jones, Alessandra Radaelli, Tegan Reeves, Dirk Reinhardt, Andrey V Reshetnyak, Andrew Jacob Rider, Carmelo Rizzari, Damiano Rizzi, Karen Gabriela Rodriguez Hermosillo, Olena Ronenko, Aneta Olga Rostowska, Liudmyla Rudko, Firas Mohamed Sakaan, Nadezhda Sakhar, Natallia N Savva, Davide Scaccaglia, Elizabeth Hawthorne Schaeffer, Carina Ursula Schneider, Nicole Scobie, Olena Semeniuk, Roksoliana Shevchyk, Ana I Shuler, Stanislav Shvets, Szymon Pawel Skoczen, William John Smeal, Igor Sokolowski, Anna A Sonkin, Alla Ivanivna Stepanjuk, Andrea Spota, Jaroslav Sterba, Jan Styczynski, Olha Svintsova, Andriy V Synyuta, Tomasz Szczepanski, Paweł Kukiz Szczucinski, Bartosz Miroslaw Szmyd, Maria Tasso Cereceda, Alina Teliuk, Iwona Tomanek, Phoebe Topping, Montserrat Torrent, Joanna Trelińska, Olha Troyanovska, Elena Trubnikova, Lyudmila G Tsurkan, Iryna Tsymbalyuk-Voloshyn, Tomasz Franciszek Urasinski, Agnieszka Urbanek-Dadela, Nataliia Vasilieva, Aksana Vasilyeva, Jaime Verdú-Amorós, Natalia Vilcu-Bajurean, Leo Vinitsky, Giovanni Volpe, Oksana Vorobel, Jacek Tadeusz Wachowiak, Marcin Slawomir Wasiak, Lance Allan Wiedower, Lena Isolde Wuenschel, Mariusz Stanislaw Wysocki, Marina Yurieva, Anastasiia Zagurska, Stanislav S Zakharenko, Aelita V Zakharenko, Khrystyna Zapotochna, Joanna Emilia Zawitkowska, Agulnik, A, Kizyma, R, Salek, M, Wlodarski, M, Pogorelyy, M, Oszer, A, Yakimkova, T, Nogovitsyna, Y, Dutkiewicz, M, Dalle, J, Dirksen, U, Eggert, A, Fernandez-Teijeiro, A, Greiner, J, Kraal, K, Mueller, A, Sramkova, L, Zecca, M, Wise, P, Mlynarski, W, Avula, M, Adyrov, M, Berlanga, P, Blackwood, C, Bouffet, E, Czauderna, P, de Koning, L, dos Reis Farinha, N, Foster, W, Graetz, D, Gupta, S, Holter, W, Hough, R, Kliuchkivska, K, Kolenova, A, Kolodrubiec, J, Moreira, D, Mukkada, S, Mykychak, I, Raciborska, A, Salman, Z, Sopilnyak, A, Tyupa, S, Vinitsky, A, Wobst, N, Miller, B, Rasul, S, Rodriguez-Galindo, C, Alanbousi, I, Alexander, S, Apel, A, Bal, W, Balwierz, W, Basset-Salom, L, Bastardo Blanco, D, Bauer, K, Bayazitov, I, Bhakta, N, Bien, E, Bieniek, K, Blair, S, Bodak, K, Bordeianu, I, Braganca, J, Bucurenci, M, Budny, E, Budzyn, A, Bumgardner, C, Burditt, R, Burnside Clapp, V, Bykov, V, Canete, A, Carnelli, M, Cela, E, Cepowska, Z, Chaber, R, Cherner-Drieux, A, Chubata, M, Clough, H, Czernicka - Siwecka, J, Czyzewski, K, Dashchakovska, O, Dembowska-Baginska, B, Derwich, K, Dommett, R, Dorosh, O, Drabko, K, Dragomir, M, Dworzak, M, Dyma, S, Earl, J, English, M, Evseev, D, Farren, B, Fedyk, N, Ferneza, S, Fox Irwin, L, Galazkowski, R, Ganieva, G, Garanzha, V, Gelman, M, Godzinski, J, Goeres, A, Golban, R, Griksaitis, M, Hampel, M, Hastings, S, Heenen, D, Hill, M, Holiuk, I, Hutnik, L, Irga-Jaworska, N, Istomin, O, Janczar, S, Kacharian, A, Kalwak, K, Karolczyk, G, Karpenko, N, Katsubo, H, Kaznowska, B, Kentsis, A, Ketteler, P, Kienesberger, A, Kiselev, R, Kizyma, Z, Klymniuk, H, Kostiuk, Y, Kowalik, T, Kozlova, O, Kozubenko, V, Kramar, T, Krawczuk-Rybak, M, Kulemzina, I, Kurkowska, P, Kuzyk, A, Ladenstein, R, Laguna, P, Lassaletta, A, Lehmberg, K, Leontieva, O, Liashenko, S, Loizou, L, Lucchetta, S, Lupo, M, Lysytsia, L, Lysytsia, O, Machnik, K, Mainland, J, Matczak, K, Matysiak, M, Mayeur, P, Minervina, A, Mishkova, V, Mizia-Malarz, A, Morales La Madrid, A, Moreno, L, Moskvin, V, Muszynska-Roslan, K, Nelson, A, Ociepa, T, Oltolini, S, Onipko, N, Pappas, A, Patel, A, Patrahau, A, Pauley, J, Pavlenko, Y, Pavlovych, A, Peregud-Pogorzelski, J, Perek-Polnik, M, Perez, V, Perez-Martinez, A, Pikman, Y, Pitozzi, G, Portugal, R, Posternak, V, Prete, A, Pritchard-Jones, K, Radaelli, A, Reeves, T, Reinhardt, D, Reshetnyak, A, Rider, A, Rizzari, C, Rizzi, D, Rodriguez Hermosillo, K, Ronenko, O, Rostowska, A, Rudko, L, Sakaan, F, Sakhar, N, Savva, N, Scaccaglia, D, Schaeffer, E, Schneider, C, Scobie, N, Semeniuk, O, Shevchyk, R, Shuler, A, Shvets, S, Skoczen, S, Smeal, W, Sokolowski, I, Sonkin, A, Stepanjuk, A, Spota, A, Sterba, J, Styczynski, J, Svintsova, O, Synyuta, A, Szczepanski, T, Szczucinski, P, Szmyd, B, Tasso Cereceda, M, Teliuk, A, Tomanek, I, Topping, P, Torrent, M, Trelinska, J, Troyanovska, O, Trubnikova, E, Tsurkan, L, Tsymbalyuk-Voloshyn, I, Urasinski, T, Urbanek-Dadela, A, Vasilieva, N, Vasilyeva, A, Verdu-Amoros, J, Vilcu-Bajurean, N, Vinitsky, L, Volpe, G, Vorobel, O, Wachowiak, J, Wasiak, M, Wiedower, L, Wuenschel, L, Wysocki, M, Yurieva, M, Zagurska, A, Zakharenko, S, Zakharenko, A, Zapotochna, K, and Zawitkowska, J

Subjects
Neoplasms, Medizin, Ethnicity, cancer, Humans, war, MED/38 - PEDIATRIA GENERALE E SPECIALISTICA, Hematology, Child, Hematologic Diseases, blood disorder
Published
2022
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48. Defining and listing very rare cancers of paediatric age: consensus of the Joint Action on Rare Cancers in cooperation with the European Cooperative Study Group for Pediatric Rare Tumors

Author

Daniel Orbach, Maura Massimino, Ewa Bien, Annalisa Trama, Gianni Bisogno, Gilles Vassal, Gemma Gatta, Pamela Kearns, Ines B Brecht, Michael C. Ost, Yves Reguerre, Teresa Stachowicz-Stencel, Jan Godzinski, Chiara Magni, Giovanni Cecchetto, Andrea Ferrari, Dominik T. Schneider, Andrea Biondi, Ferrari, A, Brecht, I, Gatta, G, Schneider, D, Orbach, D, Cecchetto, G, Godzinski, J, Reguerre, Y, Bien, E, Stachowicz-Stencel, T, Ost, M, Magni, C, Kearns, P, Vassal, G, Massimino, M, Biondi, A, Bisogno, G, and Trama, A

Subjects
0301 basic medicine, Cancer Research, Pediatrics, medicine.medical_specialty, Consensus, Adolescent, Cancer registrie, Cancer registries, Incidence, Very rare paediatric cancers, Oncology, Population, Consensu, Listing (computer), 03 medical and health sciences, Rare Diseases, 0302 clinical medicine, Neoplasms, Humans, Medicine, media_common.cataloged_instance, Registries, Paediatric age, European union, Child, education, media_common, education.field_of_study, business.industry, Incidence (epidemiology), Cancer, Very rare paediatric cancer, medicine.disease, Europe, Joint action, 030104 developmental biology, 030220 oncology & carcinogenesis, business, International Classification of Diseases for Oncology
Abstract

Although all tumours are rare in childhood, there are some particularly rare paediatric cancers which have not benefited from advances made by the international paediatric oncology network. To establish a shared definition and produce a list of these entities, the European Union Joint Action on Rare Cancers (JARC) promoted a consensus effort. The definition was based on the incidence rates estimated using the information network on rare cancers (RARECAREnet) database, pooling data from 94 population-based cancer registries and 27 countries. The RARECAREnet list of cancers was used to estimate the incidence rates. This list groups cancers by combining the International Classification of Diseases for Oncology, third edition, morphology and topography codes. According to the consensus, very rare paediatric cancers were identified as those with an annual incidence 2/1000000 (i.e. thyroid and testicular cancers and skin melanoma), but the consensus experts considered them as ‘very rare’ according to their clinical needs (e.g. shortage of knowledge and clinical expertise as the other rare paediatric cancers). The JARC consensus produced a definition and a list of very rare paediatric cancers which may represent a starting point for prioritising research on these tumours, based on data and patients’ clinical needs.

Published
2019
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49. Efficacy of anti PD-1 therapy in children and adolescent melanoma patients (MELCAYA study).

Author

Mandalà M, Ferrari A, Brecht IB, Suijkerbuijk KP, Maschke L, Giannarelli D, Indini A, Ubaldi M, Pecci G, Atkinson V, Helgadottir H, Chiaravalli S, Benannoune N, Robert C, Teterycz P, Rutkowski P, Puig S, Madonna G, Kebudi R, Grynberg S, Arantes LM, Bien E, Krawczyk M, Pasquale MD, Dierselhuis MP, Massi D, Long GV, Ascierto PA, and Eggermont AMM

Subjects
Humans, Adolescent, Child, Male, Female, Child, Preschool, Retrospective Studies, Skin Neoplasms drug therapy, Skin Neoplasms pathology, Skin Neoplasms mortality, Progression-Free Survival, Antineoplastic Agents, Immunological therapeutic use, Antineoplastic Agents, Immunological adverse effects, Melanoma drug therapy, Melanoma mortality, Melanoma pathology, Immune Checkpoint Inhibitors therapeutic use, Immune Checkpoint Inhibitors adverse effects, Programmed Cell Death 1 Receptor antagonists & inhibitors
Abstract

Background: Data on the efficacy and safety of anti PD-1 antibodies in children and adolescents (CA) with melanoma are lacking. The aim of this study was to determine outcomes of CA melanoma patients receiving anti PD-1 antibodies., Methods: Melanoma patients ≤18 years treated with anti PD-1 were retrospectively retrieved from 15 academic centers. Information on histopathological diagnosis, surgical treatment, systemic therapy, objective response rate (ORR), safety profile was collected. Progression-free survival (PFS) and overall survival (OS) were assessed by Kaplan-Meier method., Results: Between April 2016 and March 2024, 99 patients treated with systemic therapy were retrieved, 81 treated with anti PD-1 therapy. Median age was 14 years (range 2-18 years), 37 pts were ≤12 yrs. Overall, 38 CA patients received anti PD-1 in adjuvant setting, and the 3-year PFS and OS were 70.6 % and 81.1 %, respectively. Two patients received anti-PD-1 based neoadjuvant treatment, both had a pathologic complete response and remain disease free. Fifty-six received a systemic therapy for advanced disease and among them, 43 received anti PD-1-based therapy for advanced disease in 1st line, while 12 and 5 pts received a 2nd and 3rd line, respectively. Among patients receiving a 1st line therapy with anti PD-1 monotherapy the ORR was 25 %, and the 3-year OS was 34 %. Toxicities were consistent with previous studies in adult melanoma patients., Conclusions: Our study provides the first evidence of efficacy of anti PD-1 in CA melanoma patients and supports the use of anti PD-1 therapy in pts ≤18 years, included those <12 years., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 The Authors. Published by Elsevier Ltd.. All rights reserved.)

Published
2024
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