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3. Diagnostic evaluation of paediatric autoimmune lymphoproliferative immunodeficiencies (ALPID): a prospective cohort study

Author

Hägele, Pauline, Staus, Paulina, Scheible, Raphael, Uhlmann, Annette, Heeg, Maximilian, Klemann, Christian, Maccari, Maria Elena, Ritterbusch, Henrike, Armstrong, Martin, Cutcutache, Ioana, Elliott, Katherine S, von Bernuth, Horst, Leahy, Timothy Ronan, Leyh, Jörg, Holzinger, Dirk, Lehmberg, Kai, Svec, Peter, Masjosthusmann, Katja, Hambleton, Sophie, Jakob, Marcus, Sparber-Sauer, Monika, Kager, Leo, Puzik, Alexander, Wolkewitz, Martin, Lorenz, Myriam Ricarda, Schwarz, Klaus, Speckmann, Carsten, Rensing-Ehl, Anne, Ehl, Stephan, Abinun, Mario, Abrahamsen, Tore, Albert, Michael, Almalky, Mohamed, Altaf, Sadaf, Babayeva, Royala, Bakhtiar, Shahrzad, Baris, Safa, Baumann, Ulrich, Becker, Martina, Berger, Thomas, Biebl, Ariane, Bielack, Stefan, Biskup, Saskia, Bismarck, Philipp, Bode, Sebastian, Borchers, Regine, Boztug, Carl Friedrich, Brockmann, Knut, Bruwier, Annelyse, Buchholz, Bernd, Cant, Andrew, Castro, Carla N, Classen, Carl, Claviez, Alexander, Crazzolara, Roman, Cuntz, Franziska, Dąbrowska-Leonik, Nel, Derichs, Ute, Dückers, Gregor, Eberl, Wolfgang, Ebetsberger-Dachs, Georg, Erlacher, Miriam, Fabre, Alexandre, Faletti, Laura, Farmand, Susan, Figueiredo, Antonio, Fischer, Marco, Flaadt, Tim, Full, Hermann, Gambineri, Eleonora, Girschick, Hermann, Goldacker, Sigune, Grimbacher, Bodo, Groß, Miriam, Gruhn, Andrew J, Gungoren, Ezgi, Haberfellner, Florian, Hague, Rosie, Hauch, Holger, Hauck, Fabian, Heine, Sabine, Holzinger, Dirk, Huisman, Elise, Jakovljevic, Gordana, James, Beki, Janda, Ales, Janda, Małgorzata, Jones, Neil, Kaiser-Labusch, Petra, Kentouche, Karim, Knight, Julian, Knirsch, Stephanie, Kontny, Udo, Körholz, Julia, Krenn, Ezgi Yalcin, Kuehnle, Ingrid, Kühne, Thomas, Lee-Dimroth, Jae-Yun, Lehmann, Hartwig, Leipold, Michael H., Meinhardt, Andrea, Mönkemöller, Kirsten, Morbach, Henner, Mücke, Urs, Nathrath, Michaela, Naumann-Bartsch, Nora, Neth, Olaf, Niemeyer, Charlotte, Olbrich, Peter, Ostró, Róbert, Owens, Stephen, Pac, Malgorzata, Pachlopnik Schmid, Rita, Page, Markus G., Pekrun, Arnulf, Prader, Seraina, Proietti, Michele, Puzik, Alexander, Rajacic, Nada, Rothoeft, Tobias, Ryan, Freimut H., Salou, Sarah, Salzer, Elisabeth, Savic, Sinisa, Schilling, Antonio E., Schmid, Jana, Schönberger, Stefan, Schuetz, Catharina, Schuez-Havupalo, Tore G., Schulte, Björn, Schulz, Ansgar, Schuster, Volker, Seidel, Markus, Siepermann, Kathrin, Smisek, Petr, Soomann, Maarja, Stiefel, Martina, Storck, Simone, Strahm, Brigitte, Streiter, Elise J, Teltschik, Charlotte M., Thalhammer, Julian, Tippelt, Stephan, Toskov, Vasil, Trück, Johannes, Vieth, Simon, Wegehaupt, Oliver, and Wiesel, Thomas

Abstract

Lymphoproliferation and autoimmune cytopenias characterise autoimmune lymphoproliferative syndrome. Other conditions sharing these manifestations have been termed autoimmune lymphoproliferative syndrome-like diseases, although they are frequently more severe. The aim of this study was to define the genetic, clinical, and immunological features of these disorders to improve their diagnostic classification.

Published
2024
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4. Factors associated with severity in invasive community-acquired Staphylococcus aureus infections in children: a prospective European multicentre study

Author

Gijón, M., Bellusci, M., Petraitiene, B., Noguera-Julian, A., Zilinskaite, V., Sanchez Moreno, P., Saavedra-Lozano, J., Glikman, D., Daskalaki, M., Kaiser-Labusch, P., Falup-Pecurariu, O., Montagnani, C., Prieto, L., Gené, A., Trumpulyte, G., Kulecnikova, I., Lepe, J.A., Cercenado, E., Kudinsky, R., Makri, A., Huppertz, H.I., Bleotu, L., Cocchi, P., García-Hierro, P., Vitkauskiene, A., Fortuny, C., Zukovskaja, V., Neth, O., Santos, M., Rokney, A., Petra, M., Lixandru, R., Galli, L., Guillén, S., Chaves, F., and Rojo Conejo, P.

Published
2016
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5. Tuberculosis Disease in Children and Adolescents on Therapy With Antitumor Necrosis Factor-ɑ Agents: A Collaborative, Multicenter Paediatric Tuberculosis Network European Trials Group (ptbnet) Study

Author

Noguera-Julian A., Calzada-Hernandez J., Brinkmann F., Roy R. B., Bilogortseva O., Buettcher M., Carvalho I., Chechenyeva V., Falcon L., Goetzinger F., Guerrero-Laleona C., Hoffmann P., Jelusic M., Niehues T., Ozere I., Shackley F., Suciliene E., Welch S. B., Scholvinck E. H., Ritz N., Tebruegge M., Curtis N., Villanueva P., Marais B., Britton P., Clark J., Pichler J., Zschocke A., Bogyi M., Dreesman A., Mouchet F., Velizarova S., Pavic I., Nygaard U., Pulsen A., Kontturi A., Salo E., Chadelat K., Kruger R., Tee S., Ahrens F., Barker M., Zimmermann T., Schulze-Sturm U., Kaiser-Labusch P., Tsolia M., Ghanaie O. M., Buonsenso D., Lo Vecchio A., Ivaskeviciene I., Vilc V., Smyrnaios A., Arbore A. S., Starshinova A., Solovic I., Krivec U., Aldeco M., Espiau M., Soriano-Arandes A., Neth O., Santiago B., Gomez-Pastrana D., Blazquez D., Bustillo M., Perez-Porcuna T. M., Cilleruelo M. J., Kotz K., Bennet R., Relly C., Niederer-Loher A., Rochat I., Pavskyi S., Riordan A., Doherty C., Bamford A., Shingadia D., Emonts M., Ferreras-Antolin L., McMaster P., Moriarty P., Noguera-Julian, A., Calzada-Hernandez, J., Brinkmann, F., Roy, R. B., Bilogortseva, O., Buettcher, M., Carvalho, I., Chechenyeva, V., Falcon, L., Goetzinger, F., Guerrero-Laleona, C., Hoffmann, P., Jelusic, M., Niehues, T., Ozere, I., Shackley, F., Suciliene, E., Welch, S. B., Scholvinck, E. H., Ritz, N., Tebruegge, M., Curtis, N., Villanueva, P., Marais, B., Britton, P., Clark, J., Pichler, J., Zschocke, A., Bogyi, M., Dreesman, A., Mouchet, F., Velizarova, S., Pavic, I., Nygaard, U., Pulsen, A., Kontturi, A., Salo, E., Chadelat, K., Kruger, R., Tee, S., Ahrens, F., Barker, M., Zimmermann, T., Schulze-Sturm, U., Kaiser-Labusch, P., Tsolia, M., Ghanaie, O. M., Buonsenso, D., Lo Vecchio, A., Ivaskeviciene, I., Vilc, V., Smyrnaios, A., Arbore, A. S., Starshinova, A., Solovic, I., Krivec, U., Aldeco, M., Espiau, M., Soriano-Arandes, A., Neth, O., Santiago, B., Gomez-Pastrana, D., Blazquez, D., Bustillo, M., Perez-Porcuna, T. M., Cilleruelo, M. J., Kotz, K., Bennet, R., Relly, C., Niederer-Loher, A., Rochat, I., Pavskyi, S., Riordan, A., Doherty, C., Bamford, A., Shingadia, D., Emonts, M., Ferreras-Antolin, L., Mcmaster, P., and Moriarty, P.

Subjects
reactivation, Disease, anti-TNF-alpha, 0302 clinical medicine, Medicine, children, 030212 general & internal medicine, JUVENILE IDIOPATHIC ARTHRITIS, Child, Anti–TNF-alpha, RISK, Latent tuberculosis, GAMMA RELEASE ASSAYS, Miliary tuberculosi, SERIOUS INFECTION, Infectious Diseases, tuberculosis, anti–TNF-alpha, medicine.drug, miliary tuberculosis, Microbiology (medical), Adult, medicine.medical_specialty, Miliary tuberculosis, Tuberculosis, Adolescent, CORTICOSTEROIDS, Tuberculin, DIAGNOSIS, CLASSIFICATION, 03 medical and health sciences, Necrosis, Latent Tuberculosis, Internal medicine, SURVEILLANCE, INFLIXIMAB, Humans, Retrospective Studies, 030203 arthritis & rheumatology, business.industry, Tuberculin Test, Tumor Necrosis Factor-alpha, Retrospective cohort study, medicine.disease, FACTOR INHIBITORS, Infliximab, Clinical research, business, Interferon-gamma Release Tests
Abstract

Background In adults, anti–tumor necrosis factor-α (TNF-α) therapy is associated with progression of latent tuberculosis (TB) infection (LTBI) to TB disease, but pediatric data are limited. Methods Retrospective multicenter study within the Paediatric Tuberculosis Network European Trials Group, capturing patients Results Sixty-six tertiary healthcare institutions providing care for children with TB participated. Nineteen cases were identified: Crohn’s disease (n = 8; 42%) and juvenile idiopathic arthritis (n = 6; 32%) were the commonest underlying conditions. Immune-based TB screening (tuberculin skin test and/or interferon-γ release assay) was performed in 15 patients before commencing anti–TNF-α therapy but only identified 1 LTBI case; 13 patients were already receiving immunosuppressants at the time of screening. The median interval between starting anti–TNF-α therapy and TB diagnosis was 13.1 (IQR, 7.1–20.3) months. All cases presented with severe disease, predominantly miliary TB (n = 14; 78%). One case was diagnosed postmortem. TB was microbiologically confirmed in 15 cases (79%). The median duration of anti-TB treatment was 50 (IQR, 46–66) weeks. Five of 15 (33%) cases who had completed TB treatment had long-term sequelae. Conclusions LTBI screening is frequently false-negative in this patient population, likely due to immunosuppressants impairing test performance. Therefore, patients with immune-mediated diseases should be screened for LTBI at the point of diagnosis, before commencing immunosuppressive medication. Children on anti–TNF-α therapy are prone to severe TB disease and significant long-term morbidity. Those observations underscore the need for robust LTBI screening programs in this high-risk patient population, even in low-TB-prevalence settings.

Published
2019

6. Characteristics and treatment regimens across ERS SHARP severe asthma registries

Author

van Bragt, JJMH, Adcock, IM, Bel, EHD, Braunstahl, GJ, ten Brinke, A, Busby, J, Canonica, GW, Cao, H, Chung, KF, Csoma, Z, Dahlen, B, Davin, E, Hansen, S, Heffler, E, Horvath, I, Korn, S, Kots, M, Kuna, P, Kwon, N, Louis, R, Plaza, V, Porsbjerg, C, Ramos-Barbon, D, Richards, LB, Skrgat, S, Sont, JK, Vijverberg, SJH, Weersink, EJM, Yasinska, V, Wagers, SS, Djukanovic, R, Maitland-van der Zee, AH, Abenhardt, B, Adler, J, Alfonso, R, Ali, R, Alkameh, S, Sanchez, CA, Alvares, L, Anderson, G, Assing, K, Ayre, S, Becker, J, Bergmann, K, Bieksiene, K, Bjerring, N, Blasi, F, Bloemen, P, Blum, H, Boing, S, Bonavia, M, Bossios, A, Bourdin, A, Brons, A, Brusselle, G, Buis, J, Caiaffa, M, Calabrese, C, Camiciottoli, G, Caruso, C, Martinez, MC, Centanni, S, Serrano, CC, Corsico, A, Cosmi, L, Costantino, M, Costello, R, Crimi, N, Dahlen, S, D'Amato, M, Davies, D, Piqueras, FDGC, Decarlo, G, Deimling, A, Del Giacco, S, Campos, RD, Djandji, M, Doberer, D, Dupont, L, Dyett, K, Edelbaher, N, Edelmann, M, Ehmann, R, Ekberg-Jansson, A, Farsi, A, Favero, E, Feimer, J, Fletcher, M, Foschino, B, Frankemolle, B, Gaga, M, Gappa, M, de Pedro, JG, Rivero, JG, Gasplmayr, M, Gebhardt, R, Geldmacher, H, Geltner, C, Gerstlauer, M, Gibson, T, Giuseppe, G, Gogoll, C, Grimm-Sachs, V, Grisle, I, Grun, B, Grunewaldt, A, Guarnieri, G, Blanco, JG, Hamelmann, E, Hamerlijnck, D, Hammers-Reinhard, A, Hanon, S, Harzheim, D, Heaney, L, Hellmich, S, Herden, M, Hering, T, Herth, F, Hilberg, O, Howarth, P, Hubatsch, M, Humbert, M, Husemann, K, Idzko, M, Jackson, D, Jandl, M, Jaumont, X, Joos, G, Jost, M, Juch, M, Kabesch, M, Kaiser-Labusch, P, Kardos, P, Kassner, F, Keeley, T, Kerr, W, Kirschner, J, Klimek, L, Koca, M, Koczulla, R, Koerner-Rettberg, C, Kopac, P, Kronsbein, J, Lipinska, IK, Langer, M, Langeveld, B, Lantz, A, Lazarinis, N, Lazic, Z, Lehtimaki, L, Leuppi, J, Lombardi, C, Lommatzsch, M, Lopez-Vina, A, Luca, R, Ludviksdottir, D, Luttecke-Hecht, C, Macchia, L, Magni, T, Rivera, CM, Mastoridis, P, Mazza, F, Menzella, F, Menzies-Gow, A, Michils, A, Mihaltan, F, Milanese, M, Milger-Kneidinger, K, Molinska, J, Montagna, I, Montuschi, P, Mulleneisen, N, Esquerre, MM, Nanzer-Kelly, A, Nenasheva, N, Neurohr, C, Nucera, E, Otker, J, Oud, K, Paggiaro, P, Parente, R, Parkinson, J, Passalacqua, G, Patberg, N, Patella, V, Patino, O, Paulsson, T, Peche, R, Pelaia, G, Peress, E, de Llano, LP, Pfeffer, P, Pfister, P, Pilette, C, Sierra, CP, Pini, L, Powitz, F, Ranger, T, Rasmussen, L, Rasmussen, K, Rezelj, M, Ricciardi, L, Ricciardolo, F, Ridolo, E, Rijssenbeek-Nouwens, L, Rolla, G, Ribate, DR, Rudiger, S, Safioti, G, Sandstrom, T, Santus, P, Sauer, R, Schauerte, G, Schipmann, R, Schleich, F, Schmid, J, Schmidt, F, Schmidt, O, Schmitz, M, Schrag, T, Schroer, S, Schultz, K, Schulz, C, Scichilone, N, Sedlak, V, Selb, J, Senna, G, Sergejeva, S, Pariente, JS, Sichau, M, Simona, D, Singer, A, Skowasch, D, Smeenk, F, Smith, S, Solidoro, P, Spadaro, G, Spanevello, A, Stefansdottir, M, Steinmetz, K, Steiss, J, Stephan, M, Stieglitz, S, Suhling, H, Taube, C, Yavuz, ST, Tudoric, N, Ulrik, C, van de Ven, M, van den Elshout, F, Van Dyke, M, Van Nederveen-Bendien, S, van Veen, I, Vandenplas, O, Velthove, K, Vianello, A, Vogelberg, C, Wallen-Nielsen, E, Weersink, EJ, Wisskirchen, T, Yacoub, M, Yancey, S, Zappa, M, Zielen, S, Zimmermann, C, Zimmermann, R, Graduate School, AII - Inflammatory diseases, APH - Personalized Medicine, Pulmonology, Paediatric Pulmonology, van Bragt, J. J. M. H., Adcock, I. M., Bel, E. H. D., Braunstahl, G. -J., ten Brinke, A., Busby, J., Canonica, G. W., Cao, H., Chung, K. F., Csoma, Z., Dahlen, B., Davin, E., Hansen, S., Heffler, E., Horvath, I., Korn, S., Kots, M., Kuna, P., Kwon, N., Louis, R., Plaza, V., Porsbjerg, C., Ramos-Barbon, D., Richards, L. B., Skrgat, S., Sont, J. K., Vijverberg, S. J. H., Weersink, E. J. M., Yasinska, V., Wagers, S. S., Djukanovic, R., Maitland-Van der Zee, A. H., Abenhardt, B., Adler, J., Alfonso, R., Ali, R., Alkameh, S., Almonacid Sanchez, C., Alvares, L., Anderson, G., Assing, K., Ayre, S., Becker, J., Bergmann, K., Bieksiene, K., Bjerring, N., Blasi, F., Bloemen, P., Blum, H., Boing, S., Bonavia, M., Bossios, A., Bourdin, A., Brons, A., Brusselle, G., Buis, J., Caiaffa, M., Calabrese, C., Camiciottoli, G., Caruso, C., Castilla Martinez, M., Centanni, S., Cisneros Serrano, C., Corsico, A., Cosmi, L., Costantino, M., Costello, R., Crimi, N., Dahlen, S., D'Amato, M., Davies, D., de Borja Garcia-Cosio Piqueras, F., Decarlo, G., Deimling, A., Del Giacco, S., Diaz Campos, R., Djandji, M., Doberer, D., Dupont, L., Dyett, K., Edelbaher, N., Edelmann, M., Ehmann, R., Ekberg-Jansson, A., Farsi, A., Favero, E., Feimer, J., Fletcher, M., Foschino, B., Frankemolle, B., Gaga, M., Gappa, M., Garcia de Pedro, J., Garcia Rivero, J., Gasplmayr, M., Gebhardt, R., Geldmacher, H., Geltner, C., Gerstlauer, M., Gibson, T., Giuseppe, G., Gogoll, C., Grimm-Sachs, V., Grisle, I., Grun, B., Grunewaldt, A., Guarnieri, G., Gullon Blanco, J., Hamelmann, E., Hamerlijnck, D., Hammers-Reinhard, A., Hanon, S., Harzheim, D., Heaney, L., Hellmich, S., Herden, M., Hering, T., Herth, F., Hilberg, O., Howarth, P., Hubatsch, M., Humbert, M., Husemann, K., Idzko, M., Jackson, D., Jandl, M., Jaumont, X., Joos, G., Jost, M., Juch, M., Kabesch, M., Kaiser-Labusch, P., Kardos, P., Kassner, F., Keeley, T., Kerr, W., Kirschner, J., Klimek, L., Koca, M., Koczulla, R., Koerner-Rettberg, C., Kopac, P., Kronsbein, J., Kuprys Lipinska, I., Langer, M., Langeveld, B., Lantz, A., Lazarinis, N., Lazic, Z., Lehtimaki, L., Leuppi, J., Lombardi, C., Lommatzsch, M., Lopez-Vina, A., Luca, R., Ludviksdottir, D., Luttecke-Hecht, C., Macchia, L., Magni, T., Martinez Rivera, C., Mastoridis, P., Mazza, F., Menzella, F., Menzies-Gow, A., Michils, A., Mihalthan, F., Milanese, M., Milger-Kneidinger, K., Molinska, J., Montagna, I., Montuschi, P., Mulleneisen, N., Munoz Esquerre, M., Nanzer-Kelly, A., Nenasheva, N., Neurohr, C., Nucera, E., Otker, J., Oud, K., Paggiaro, P., Parente, R., Parkinson, J., Passalacqua, G., Patberg, N., Patella, V., Patino, O., Paulsson, T., Peche, R., Pelaia, G., Peress, E., Perez de Llano, L., Pfeffer, P., Pfister, P., Pilette, C., Pinedo Sierra, C., Pini, L., Powitz, F., Ranger, T., Rasmussen, L., Rasmussen, K., Rezelj, M., Ricciardi, L., Ricciardolo, F., Ridolo, E., Rijssenbeek-Nouwens, L., Rolla, G., Romero Ribate, D., Rudiger, S., Safioti, G., Sandstrom, T., Santus, P., Sauer, R., Schauerte, G., Schipmann, R., Schleich, F., Schmid, J., Schmidt, F., Schmidt, O., Schmitz, M., Schrag, T., Schroer, S., Schultz, K., Schulz, C., Scichilone, N., Sedlak, V., Selb, J., Senna, G., Sergejeva, S., Serrano Pariente, J., Sichau, M., Simona, D., Singer, A., Skowasch, D., Smeenk, F., Smith, S., Solidoro, P., Spadaro, G., Spanevello, A., Stefansdottir, M., Steinmetz, K., Steiss, J., Stephan, M., Stieglitz, S., Suhling, H., Taube, C., Tolga Yavuz, S., Tudoric, N., Ulrik, C., van de Ven, M., van den Elshout, F., van Dyke, M., van Nederveen-Bendien, S., van Veen, I., Vandenplas, O., Velthove, K., Vianello, A., Vogelberg, C., Wallen-Nielsen, E., Wisskirchen, T., Yacoub, M., Yancey, S., Zappa, M., Zielen, S., Zimmermann, C., Zimmermann, R., UCL - SSS/IREC/PNEU - Pôle de Pneumologie, ORL et Dermatologie, UCL - (MGD) Service de pneumologie, van Bragt J.J.M.H., Adcock I.M., Bel E.H.D., Braunstahl G.-J., ten Brinke A., Busby J., Canonica G.W., Cao H., Chung K.F., Csoma Z., Dahlen B., Davin E., Hansen S., Heffler E., Horvath I., Korn S., Kots M., Kuna P., Kwon N., Louis R., Plaza V., Porsbjerg C., Ramos-Barbon D., Richards L.B., Skrgat S., Sont J.K., Vijverberg S.J.H., Weersink E.J.M., Yasinska V., Wagers S.S., Djukanovic R., Maitland-Van der Zee A.H., Abenhardt B., Adler J., Alfonso R., Ali R., Alkameh S., Almonacid Sanchez C., Alvares L., Anderson G., Assing K., Ayre S., Becker J., Bergmann K., Bieksiene K., Bjerring N., Blasi F., Bloemen P., Blum H., Boing S., Bonavia M., Bossios A., Bourdin A., Brons A., Brusselle G., Buis J., Caiaffa M., Calabrese C., Camiciottoli G., Caruso C., Castilla Martinez M., Centanni S., Cisneros Serrano C., Corsico A., Cosmi L., Costantino M., Costello R., Crimi N., Dahlen S., D'Amato M., Davies D., de Borja Garcia-Cosio Piqueras F., Decarlo G., Deimling A., Del Giacco S., Diaz Campos R., Djandji M., Doberer D., Dupont L., Dyett K., Edelbaher N., Edelmann M., Ehmann R., Ekberg-Jansson A., Farsi A., Favero E., Feimer J., Fletcher M., Foschino B., Frankemolle B., Gaga M., Gappa M., Garcia de Pedro J., Garcia Rivero J., Gasplmayr M., Gebhardt R., Geldmacher H., Geltner C., Gerstlauer M., Gibson T., Giuseppe G., Gogoll C., Grimm-Sachs V., Grisle I., Grun B., Grunewaldt A., Guarnieri G., Gullon Blanco J., Hamelmann E., Hamerlijnck D., Hammers-Reinhard A., Hanon S., Harzheim D., Heaney L., Hellmich S., Herden M., Hering T., Herth F., Hilberg O., Howarth P., Hubatsch M., Humbert M., Husemann K., Idzko M., Jackson D., Jandl M., Jaumont X., Joos G., Jost M., Juch M., Kabesch M., Kaiser-Labusch P., Kardos P., Kassner F., Keeley T., Kerr W., Kirschner J., Klimek L., Koca M., Koczulla R., Koerner-Rettberg C., Kopac P., Kronsbein J., Kuprys Lipinska I., Langer M., Langeveld B., Lantz A., Lazarinis N., Lazic Z., Lehtimaki L., Leuppi J., Lombardi C., Lommatzsch M., Lopez-Vina A., Luca R., Ludviksdottir D., Luttecke-Hecht C., Macchia L., Magni T., Martinez Rivera C., Mastoridis P., Mazza F., Menzella F., Menzies-Gow A., Michils A., Mihalthan F., Milanese M., Milger-Kneidinger K., Molinska J., Montagna I., Montuschi P., Mulleneisen N., Munoz Esquerre M., Nanzer-Kelly A., Nenasheva N., Neurohr C., Nucera E., Otker J., Oud K., Paggiaro P., Parente R., Parkinson J., Passalacqua G., Patberg N., Patella V., Patino O., Paulsson T., Peche R., Pelaia G., Peress E., Perez de Llano L., Pfeffer P., Pfister P., Pilette C., Pinedo Sierra C., Pini L., Powitz F., Ranger T., Rasmussen L., Rasmussen K., Rezelj M., Ricciardi L., Ricciardolo F., Ridolo E., Rijssenbeek-Nouwens L., Rolla G., Romero Ribate D., Rudiger S., Safioti G., Sandstrom T., Santus P., Sauer R., Schauerte G., Schipmann R., Schleich F., Schmid J., Schmidt F., Schmidt O., Schmitz M., Schrag T., Schroer S., Schultz K., Schulz C., Scichilone N., Sedlak V., Selb J., Senna G., Sergejeva S., Serrano Pariente J., Sichau M., Simona D., Singer A., Skowasch D., Smeenk F., Smith S., Solidoro P., Spadaro G., Spanevello A., Stefansdottir M., Steinmetz K., Steiss J., Stephan M., Stieglitz S., Suhling H., Taube C., Tolga Yavuz S., Tudoric N., Ulrik C., van de Ven M., van den Elshout F., van Dyke M., van Nederveen-Bendien S., van Veen I., Vandenplas O., Velthove K., Vianello A., Vogelberg C., Wallen-Nielsen E., Wisskirchen T., Yacoub M., Yancey S., Zappa M., Zielen S., Zimmermann C., Zimmermann R., Amsterdam UMC, National Heart and Lung Institute [London] (NHLI), Imperial College London-Royal Brompton and Harefield NHS Foundation Trust, Department of Medical Microbiology and Infection Control, Franciscus Gasthuis & Vlietland, Kleiweg 500, 3045 PM, Rotterdam, The Netherlands., Medical Centre Leeuwarden, Queen's University [Belfast] (QUB), Humanitas University [Milan] (Hunimed), Novartis Pharmaceuticals Corporation, East Hanover, NJ, USA, Korányi National Institute of Pulmonology (OKPI), Karolinska University Hospital [Stockholm], The European Lung Foundation (ELF), Bispebjerg and Frederiksberg Hospitals, Humanitas Clinical and Research Center [Rozzano, Milan, Italy], University Medical Center of the Johannes Gutenberg-University Mainz, Chiesi Farmaceutici, Medical University of Łódź (MUL), GlaxoSmithKline, Brentford, Middlesex, Centre Hospitalier Universitaire de Liège (CHU-Liège), Hospital de la Santa Creu i Sant Pau, Copenhagen University Hospital, Respiratory and Allergic Diseases [Golnik, Slovenia], University Clinic of Respiratory and Allergic Diseases Golnik, Leiden University Medical Center (LUMC), Biosci Consulting, University Hospital Southampton NHS Foundation Trust, SHARP Clinical Research, Hôpital Arnaud de Villeneuve [CHRU Montpellier], and Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier)

Subjects
Severe asthma, Pediatrics, MESH: Registries, MESH: Asthma, Cross-sectional study, Respiratory System, Medizin, [SDV.MHEP.PSR]Life Sciences [q-bio]/Human health and pathology/Pulmonology and respiratory tract, 0302 clinical medicine, MESH: Belgium, Belgium, Medicine research, Anti-Asthmatic Agents, Registries, 030212 general & internal medicine, [SDV.IMM.ALL]Life Sciences [q-bio]/Immunology/Allergology, 10. No inequality, 11 Medical and Health Sciences, Netherlands, 2. Zero hunger, education.field_of_study, SHARP CRC, MESH: Administration, Inhalation, MESH: Anti-Asthmatic Agents, 3. Good health, Europe, Italy, MESH: Poland, MESH: Sweden, medicine.drug, Pulmonary and Respiratory Medicine, medicine.medical_specialty, MESH: Hungary, Population, Investigació mèdica, Settore MED/10 - Malattie Dell'Apparato Respiratorio, 03 medical and health sciences, MESH: Cross-Sectional Studies, Administration, Inhalation, MESH: Spain, medicine, Humans, education, Asma, Retrospective Studies, Asthma, Sweden, Hungary, MESH: Humans, business.industry, Settore MED/09 - MEDICINA INTERNA, MESH: Italy, MESH: Retrospective Studies, Retrospective cohort study, Original Articles, asthma, medicine.disease, Clinical trial, Cross-Sectional Studies, Clinical research, 030228 respiratory system, Spain, MESH: Netherlands, MESH: Europe, Poland, business, Body mass index, Mepolizumab
Abstract

Little is known about the characteristics and treatments of patients with severe asthma across Europe, but both are likely to vary. This is the first study in the European Respiratory Society Severe Heterogeneous Asthma Research collaboration, Patient-centred (SHARP) Clinical Research Collaboration and it is designed to explore these variations. Therefore, we aimed to compare characteristics of patients in European severe asthma registries and treatments before starting biologicals.This was a cross-sectional retrospective analysis of aggregated data from 11 national severe asthma registries that joined SHARP with established patient databases.Analysis of data from 3236 patients showed many differences in characteristics and lifestyle factors. Current smokers ranged from 0% (Poland and Sweden) to 9.5% (Belgium), mean body mass index ranged from 26.2 (Italy) to 30.6 kg·m−2 (the UK) and the largest difference in mean pre-bronchodilator forced expiratory volume in 1 s % predicted was 20.9% (the Netherlands versus Hungary). Before starting biologicals patients were treated differently between countries: mean inhaled corticosteroid dose ranged from 700 to 1335 µg·day−1 between those from Slovenia versus Poland when starting anti-interleukin (IL)-5 antibody and from 772 to 1344 µg·day−1 in those starting anti-IgE (Slovenia versus Spain). Maintenance oral corticosteroid use ranged from 21.0% (Belgium) to 63.0% (Sweden) and from 9.1% (Denmark) to 56.1% (the UK) in patients starting anti-IL-5 and anti-IgE, respectively.The severe asthmatic population in Europe is heterogeneous and differs in both clinical characteristics and treatment, often appearing not to comply with the current European Respiratory Society/American Thoracic Society guidelines definition of severe asthma. Treatment regimens before starting biologicals were different from inclusion criteria in clinical trials and varied between countries.

Published
2019
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8. Global Review of the Age Distribution of Rotavirus Disease in Children Aged <5 Years Before the Introduction of Rotavirus Vaccination

Author

Hasso-Agopsowicz, Mateusz, Ladva, Nanji, Lopman, Benjamin, Sanderson, Colin, Cohen, Adam, Tate, Jacqueline, Riveros, Ximena, Henao-Restrepo, Ana Maria, Clark, Andrew E., Ladva, Chandresh Nanji, Alkorta, M, Atchison, C, Banajeh, S, Becker-Dreps, S, Benhafid, M, Bhandari, N, Bodhidatta, L, Braeckman, T, Bwogi, J, de Cassia Compagnoli Carmona, R, Cilla, G., Contreras-Roldan, I, Coulson, B, Cunliffe, N, Dagan, R, Givon, N, Degiuseppe, J, Dhiman, S, Dian, Z, Díaz, J., Dutta, S, Krishnan, T, Manna, B, Fletcher-Lartey, S, Fu, C, Gendrel, D., Ghenghesh, K., Gonzalez Mago, G, De Grazia, S, Grimwood, K, Groome, M, Haque, A., Heininger, U, Houpt, E, Iturriza-Gómara, M., Hungerford, D, Jarquin, C, McCracken, J, Contreras, I, Cordon-Rosales, C, Kaiser-Labusch, P, Kang, G, Kar, S, Kiulia, N, Kotloff, K, Latipov, R, Linhares, A, Lorrot, M., Mandile, M, Mast, C, Mathew, M., Matinon-Torres, F, Matthijnssens, J, Mladenova, Z., Monini, M, Montes, M, Arana, A, Motamedifar, M, Najafi, A, Nelson, T., Nokes, J, Ntoumi, F, Numazaki, K, O’Reilly, C, Ochoa, T, Page, N, Page, A., Langendorf, C, Podkolzin, A, Quach, C, Racz, M, de Rougemont, Alexis, Ruiz-Palacios, G, Saha, S, Satter, S, Soares, L, Sudarmo, S, Shigemura, K, Shirakawa, T, Athiyyah, A, Tagbo, B, Tarr, P, Klein, E., Denno, D, Turner, A., Uzoma, E, Vatosoa, R, Wandera, E, Wikswo, M, Payne, D, Yhu-Chering, H, Yoshikawa, T, Sugata, K, Yuan, Q, Liying, L, Zaman, K., Zhou, X -N, Zhang, S -X, Xu, Wei-Jiang, Serhan, Fatima, Nakamura, Tomoka, Antoni, Sébastien, Agócs, Mary, Murray, Jillian, Cherian, Thomas, Mwenda, Jason, Weldegebriel, Goitom, Biey, Joseph, Cheikh, Dah, Teleb, Nadia, Rahman, Hossam Abdel, Ahmed, Hinda, Daniels, Danni, Videbaek, Dovile, Wasley, Annemarie, Singh, Simarjit, de Oliveira, Lucia, Rey-Benito, Gloria, Sanwogou, N Jennifer, Liyanage, Jayantha, Wijesinghe, Pushpa Ranjan, Batmunkh, Nyambat, Grabovac, Varja, Fox, Kimberley, Paladin, Fem Julia, Henschke, Nicholas, Queensland University of Technology [Brisbane] (QUT), Microbiology Service, Hospital Donostia, Grupo de Investigación en Energía y Medioambiente [Bucaramanga]] (GIEMA), Universidad Industrial de Santander [Bucaramanga] (UIS), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), University of Tripoli - University Al Fateh, Visva Bharati University, Enteric Virus Unit, Virus Reference Department, Centre for Infections, Health Protection Agency, Department of Virology, National Reference Laboratory of Enteroviruses, National Center of Infectious and Parasitic Diseases [Sofia, Bulgarie] (NCIPD), Friction Stir Laboratory, Brigham Young University (BYU), UFR des Sciences de Santé (Université de Bourgogne), Université de Bourgogne (UB), Centre National de Référence des virus entériques [CHU de Dijon] (CNR virus entériques), Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), Laboratoire de sérologie-virologie (CHU de Dijon), Procédés Alimentaires et Microbiologiques [Dijon] (PAM), Université Bourgogne Franche-Comté [COMUE] (UBFC)-Université de Bourgogne (UB)-AgroSup Dijon - Institut National Supérieur des Sciences Agronomiques, de l'Alimentation et de l'Environnement, Institut de biologie moléculaire des plantes (IBMP), Centre National de la Recherche Scientifique (CNRS)-Université de Strasbourg (UNISTRA), School of Social and Community Medicine [Bristol], University of Bristol [Bristol], Vaccine Preventable Disease and Immunization, Organisation Mondiale de la Santé / World Health Organization Office (OMS / WHO), and University of Stavanger

Subjects
rotavirus gastroenteritis, age distribution, rotavirus, [SDV.MHEP.MI]Life Sciences [q-bio]/Human health and pathology/Infectious diseases, vaccine, [SDV.MP.VIR]Life Sciences [q-bio]/Microbiology and Parasitology/Virology
Abstract

International audience; We sought datasets with granular age distributions of rotavirus-positive disease presentations among children

Published
2019
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9. Characteristics and treatment regimens across ERS SHARP severe asthma registries

Author

van Bragt, JJMH, Adcock, IM, Bel, EHD, Braunstahl, G-J, ten Brinke, A, Busby, J, Canonica, GW, Cao, H, Chung, KF, Csoma, Z, Dahlen, B, Davin, E, Hansen, S, Heffler, E, Horvath, I, Korn, S, Kots, M, Kuna, P, Kwon, N, Louis, R, Plaza, V, Porsbjerg, C, Ramos-Barbon, D, Richards, LB, Skrgat, S, Sont, JK, Vijverberg, SJH, Weersink, EJM, Yasinska, V, Wagers, SS, Djukanovic, R, Maitland-van der Zee, AH, Abenhardt, B, Adler, J, Alfonso, R, Ali, R, Alkameh, S, Almonacid Sanchez, C, Alvares, L, Anderson, G, Assing, K, Ayre, S, Becker, J, Bergmann, K, Bieksiene, K, Bjerring, N, Blasi, F, Bloemen, P, Blum, H, Boeing, S, Bonavia, M, Bossios, A, Bourdin, A, Brons, A, Brusselle, G, Buis, J, Caiaffa, M, Calabrese, C, Camiciottoli, G, Caruso, C, Castilla Martinez, M, Centanni, S, Cisneros Serrano, C, Corsico, A, Cosmi, L, Costantino, M, Costello, R, Crimi, N, Dahlen, S, D'Amato, M, Davies, D, Garcia-Cosio Piqueras, FDB, Decarlo, G, Deimling, A, Del Giacco, S, Diaz Campos, R, Djandji, M, Doberer, D, Dupont, L, Dyett, K, Edelbaher, N, Edelmann, M, Ehmann, R, Ekberg-Jansson, A, Farsi, A, Favero, E, Feimer, J, Fletcher, M, Foschino, B, Frankemolle, B, Gaga, M, Gappa, M, Garcia de Pedro, J, Garcia Rivero, J, Gasplmayr, M, Gebhardt, R, Geldmacher, H, Geltner, C, Gerstlauer, M, Gibson, T, Giuseppe, G, Gogoll, C, Grimm-Sachs, V, Grisle, I, Gruen, B, Gruenewaldt, A, Guarnieri, G, Gullon Blanco, J, Hamelmann, E, Hamerlijnck, D, Hammers-Reinhard, A, Hanon, S, Harzheim, D, Heaney, L, Hellmich, S, Herden, M, Hering, T, Herth, F, Hilberg, O, Howarth, P, Hubatsch, M, Humbert, M, Husemann, K, Idzko, M, Jackson, D, Jandl, M, Jaumont, X, Joos, G, Joest, M, Juech, M, Kabesch, M, Kaiser-Labusch, P, Kardos, P, Kaessner, F, Keeley, T, Kerr, W, Kirschner, J, Klimek, L, Koca, M, Koczulla, R, Koerner-Rettberg, C, Kopac, P, Kronsbein, J, Lipinska, IK, Langer, M, Langeveld, B, Lantz, A, Lazarinis, N, Lazic, Z, Lehtimaki, L, Leuppi, J, Lombardi, C, Lommatzsch, M, Lopez-Vina, A, Luca, R, Ludviksdottir, D, Luettecke-Hecht, C, Macchia, L, Magni, T, Martinez Rivera, C, Mastoridis, P, Mazza, F, Menzella, F, Menzies-Gow, A, Michils, A, Mihaltan, F, Milanese, M, Milger-Kneidinger, K, Molinska, J, Montagna, I, Montuschi, P, Muelleneisen, N, Munoz Esquerre, M, Nanzer-Kelly, A, Nenasheva, N, Neurohr, C, Nucera, E, Otker, J, Oud, K, Paggiaro, P, Parente, R, Parkinson, J, Passalacqua, G, Patberg, N, Patella, V, Patino, O, Paulsson, T, Peche, R, Pelaia, G, Peress, E, Perez de Llano, L, Pfeffer, P, Pfister, P, Pilette, C, Pinedo Sierra, C, Pini, L, Powitz, F, Ranger, T, Rasmussen, L, Rasmussen, K, Rezelj, M, Ricciardi, L, Ricciardolo, F, Ridolo, E, Rijssenbeek-Nouwens, L, Rolla, G, Romero Ribate, D, Ruediger, S, Safioti, G, Sandstrom, T, Santus, P, Sauer, R, Schauerte, G, Schipmann, R, Schleich, F, Schmid, J, Schmidt, F, Schmidt, O, Schmitz, M, Schrag, T, Schroeer, S, Schultz, K, Schulz, C, Scichilone, N, Sedlak, V, Selb, J, Senna, G, Sergejeva, S, Serrano Pariente, J, Sichau, M, Simona, D, Singer, A, Skowasch, D, Smeenk, F, Smith, S, Solidoro, P, Spadaro, G, Spanevello, A, Stefansdottir, M, Steinmetz, K, Steiss, J, Stephan, M, Stieglitz, S, Suhling, H, Taube, C, Yavuz, ST, Tudoric, N, Ulrik, C, van de Ven, M, van den Elshout, F, Van Dyke, M, Van Nederveen-Bendien, S, van Veen, I, Vandenplas, O, Velthove, K, Vianello, A, Vogelberg, C, Wallen-Nielsen, E, Weersink, EJ, Wisskirchen, T, Yacoub, M, Yancey, S, Zappa, M, Zielen, S, Zimmermann, C, Zimmermann, R, van Bragt, JJMH, Adcock, IM, Bel, EHD, Braunstahl, G-J, ten Brinke, A, Busby, J, Canonica, GW, Cao, H, Chung, KF, Csoma, Z, Dahlen, B, Davin, E, Hansen, S, Heffler, E, Horvath, I, Korn, S, Kots, M, Kuna, P, Kwon, N, Louis, R, Plaza, V, Porsbjerg, C, Ramos-Barbon, D, Richards, LB, Skrgat, S, Sont, JK, Vijverberg, SJH, Weersink, EJM, Yasinska, V, Wagers, SS, Djukanovic, R, Maitland-van der Zee, AH, Abenhardt, B, Adler, J, Alfonso, R, Ali, R, Alkameh, S, Almonacid Sanchez, C, Alvares, L, Anderson, G, Assing, K, Ayre, S, Becker, J, Bergmann, K, Bieksiene, K, Bjerring, N, Blasi, F, Bloemen, P, Blum, H, Boeing, S, Bonavia, M, Bossios, A, Bourdin, A, Brons, A, Brusselle, G, Buis, J, Caiaffa, M, Calabrese, C, Camiciottoli, G, Caruso, C, Castilla Martinez, M, Centanni, S, Cisneros Serrano, C, Corsico, A, Cosmi, L, Costantino, M, Costello, R, Crimi, N, Dahlen, S, D'Amato, M, Davies, D, Garcia-Cosio Piqueras, FDB, Decarlo, G, Deimling, A, Del Giacco, S, Diaz Campos, R, Djandji, M, Doberer, D, Dupont, L, Dyett, K, Edelbaher, N, Edelmann, M, Ehmann, R, Ekberg-Jansson, A, Farsi, A, Favero, E, Feimer, J, Fletcher, M, Foschino, B, Frankemolle, B, Gaga, M, Gappa, M, Garcia de Pedro, J, Garcia Rivero, J, Gasplmayr, M, Gebhardt, R, Geldmacher, H, Geltner, C, Gerstlauer, M, Gibson, T, Giuseppe, G, Gogoll, C, Grimm-Sachs, V, Grisle, I, Gruen, B, Gruenewaldt, A, Guarnieri, G, Gullon Blanco, J, Hamelmann, E, Hamerlijnck, D, Hammers-Reinhard, A, Hanon, S, Harzheim, D, Heaney, L, Hellmich, S, Herden, M, Hering, T, Herth, F, Hilberg, O, Howarth, P, Hubatsch, M, Humbert, M, Husemann, K, Idzko, M, Jackson, D, Jandl, M, Jaumont, X, Joos, G, Joest, M, Juech, M, Kabesch, M, Kaiser-Labusch, P, Kardos, P, Kaessner, F, Keeley, T, Kerr, W, Kirschner, J, Klimek, L, Koca, M, Koczulla, R, Koerner-Rettberg, C, Kopac, P, Kronsbein, J, Lipinska, IK, Langer, M, Langeveld, B, Lantz, A, Lazarinis, N, Lazic, Z, Lehtimaki, L, Leuppi, J, Lombardi, C, Lommatzsch, M, Lopez-Vina, A, Luca, R, Ludviksdottir, D, Luettecke-Hecht, C, Macchia, L, Magni, T, Martinez Rivera, C, Mastoridis, P, Mazza, F, Menzella, F, Menzies-Gow, A, Michils, A, Mihaltan, F, Milanese, M, Milger-Kneidinger, K, Molinska, J, Montagna, I, Montuschi, P, Muelleneisen, N, Munoz Esquerre, M, Nanzer-Kelly, A, Nenasheva, N, Neurohr, C, Nucera, E, Otker, J, Oud, K, Paggiaro, P, Parente, R, Parkinson, J, Passalacqua, G, Patberg, N, Patella, V, Patino, O, Paulsson, T, Peche, R, Pelaia, G, Peress, E, Perez de Llano, L, Pfeffer, P, Pfister, P, Pilette, C, Pinedo Sierra, C, Pini, L, Powitz, F, Ranger, T, Rasmussen, L, Rasmussen, K, Rezelj, M, Ricciardi, L, Ricciardolo, F, Ridolo, E, Rijssenbeek-Nouwens, L, Rolla, G, Romero Ribate, D, Ruediger, S, Safioti, G, Sandstrom, T, Santus, P, Sauer, R, Schauerte, G, Schipmann, R, Schleich, F, Schmid, J, Schmidt, F, Schmidt, O, Schmitz, M, Schrag, T, Schroeer, S, Schultz, K, Schulz, C, Scichilone, N, Sedlak, V, Selb, J, Senna, G, Sergejeva, S, Serrano Pariente, J, Sichau, M, Simona, D, Singer, A, Skowasch, D, Smeenk, F, Smith, S, Solidoro, P, Spadaro, G, Spanevello, A, Stefansdottir, M, Steinmetz, K, Steiss, J, Stephan, M, Stieglitz, S, Suhling, H, Taube, C, Yavuz, ST, Tudoric, N, Ulrik, C, van de Ven, M, van den Elshout, F, Van Dyke, M, Van Nederveen-Bendien, S, van Veen, I, Vandenplas, O, Velthove, K, Vianello, A, Vogelberg, C, Wallen-Nielsen, E, Weersink, EJ, Wisskirchen, T, Yacoub, M, Yancey, S, Zappa, M, Zielen, S, Zimmermann, C, and Zimmermann, R

Abstract

Little is known about the characteristics and treatments of patients with severe asthma across Europe, but both are likely to vary. This is the first study in the European Respiratory Society Severe Heterogeneous Asthma Research collaboration, Patient-centred (SHARP) Clinical Research Collaboration and it is designed to explore these variations. Therefore, we aimed to compare characteristics of patients in European severe asthma registries and treatments before starting biologicals.This was a cross-sectional retrospective analysis of aggregated data from 11 national severe asthma registries that joined SHARP with established patient databases.Analysis of data from 3236 patients showed many differences in characteristics and lifestyle factors. Current smokers ranged from 0% (Poland and Sweden) to 9.5% (Belgium), mean body mass index ranged from 26.2 (Italy) to 30.6 kg·m-2 (the UK) and the largest difference in mean pre-bronchodilator forced expiratory volume in 1 s % predicted was 20.9% (the Netherlands versus Hungary). Before starting biologicals patients were treated differently between countries: mean inhaled corticosteroid dose ranged from 700 to 1335 µg·day-1 between those from Slovenia versus Poland when starting anti-interleukin (IL)-5 antibody and from 772 to 1344 µg·day-1 in those starting anti-IgE (Slovenia versus Spain). Maintenance oral corticosteroid use ranged from 21.0% (Belgium) to 63.0% (Sweden) and from 9.1% (Denmark) to 56.1% (the UK) in patients starting anti-IL-5 and anti-IgE, respectively.The severe asthmatic population in Europe is heterogeneous and differs in both clinical characteristics and treatment, often appearing not to comply with the current European Respiratory Society/American Thoracic Society guidelines definition of severe asthma. Treatment regimens before starting biologicals were different from inclusion criteria in clinical trials and varied between countries.

Published
2020

10. Lungenrundherde: 3 Fälle

Author

Wiehl, M, Jablonka, K, and Kaiser-Labusch, P

Subjects
ddc: 610, 610 Medical sciences, Medicine
Abstract

Einleitung: Schattengebende Lungenrundherde im Röntgen Thorax sind in der Pädiatrie relativ selten und in der internistischen Medizin meist Ausdruck von Metastasen. Wir berichten über 3 Fälle einer „Kugelpneumonie“. Als Folge einer bakteriellen Entzündung des Lungenparenchyms[zum vollständigen Text gelangen Sie über die oben angegebene URL], 67. Jahrestagung der Norddeutschen Gesellschaft für Kinder- und Jugendmedizin (NDGKJ)

Published
2018
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11. Das Guillain-Barré-Syndrom im Kleinkindalter – eine seltene Differentialdiagnose bei Laufverweigerung

Author

Winner, K, Kaiser-Labusch, P, and Huppertz, HI

Subjects
ddc: 610, 610 Medical sciences, Medicine
Abstract

Das Guillain-Barré-Syndrom ist eine idiopathische Polyneuritis der spinalen Nervenwurzeln und peripheren Nerven, die mit einer Inzidenz von 1-2/100.000 in der Allgemeinbevölkerung selten ist. Da der Altersgipfel dieser Erkrankung im 2.-3. Lebensjahrzehnt liegt, sind Fälle im Kleinkindalter[zum vollständigen Text gelangen Sie über die oben angegebene URL], 67. Jahrestagung der Norddeutschen Gesellschaft für Kinder- und Jugendmedizin (NDGKJ)

Published
2018
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19. Pediatric Community-Acquired Bone and Joint Staphylococcus AureusInfections In Europe

Author

Gijón, Manuel, Bellusci, Marcello, Petraitiene, Birute, Noguera-Julian, Antoni, Glikman, Daniel, Saavedra-Lozano, Jesús, Neth, Olaf, Daskalaki, Maria, Zilinskaite, Virginija, Kaiser-Labusch, Petra, Prieto, Luis, and Rojo, Pablo

Abstract

To analyze host and pathogen factors related to disease severity of community-acquired bone and joint infections in children, a cohort of pediatric patients was prospectively recruited from 13 centers in 7 European countries. A total of 85 children were included, 11 (13%) had a severe infection. Panton-Valentine leukocidin–positive isolates were 17%, and 6% of the isolates were methicillin-resistant Staphylococcus aureus. Multivariate analysis identified Panton-Valentine leukocidin presence (adjusted odds ratio, 12.6; P= 0.01) as the only factor independently associated with severe outcome, regardless of methicillin resistance.

Published
2020
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20. The German PID-net registry

Author

El-Helou, S. M., Biegner, A. K., Bode, S., Ehl, S., Heeg, M., Ritterbusch, H., Rusch, S., Schmitt, R., Warnatz, K., Baerlecken, N. T., Baumann, U., Beider, R., Ernst, D., Gerschmann, S., Klemann, C., Mielke, G., Schmidt, R. E., Schuermann, G., Viemann, D., Albert, M., Eichinger, A., Eisl, E., Hauck, F., Klein, C., Sollinger, F., von Bernuth, H., Hanitsch, L., Krueger, R., Scheibenbogen, C., Avila, A., Borte, M., Borte, S., Fasshauer, M., Hauenherm, A., Kellner, N., Mueller, H., Uelzen, A., Bader, P., Bakhtiar, S., Hess, U., Lee, J. Y., Schubert, R., Voss, S., Zielen, S., Bienemann, K., Lankisch, P., Laws, H. J., Neubert, J., Dueckers, G., Lamers, B., Langemeyer, V., Niehues, T., Hoenig, M., Schulz, A., Schwarz, K., Steinmann, S., Graf, D., Haase, G., Liese, J. G., Morbach, H., Schwaneck, E., Tony, H. P., Foell, D., Hellige, A., Masjosthusmann, K., Mohr, M., Wittkowski, H., Fischer, J. C., Hedrich, C. M., Roesen-Wolff, A., Roesler, J., Behrends, U., Rieber, N., Schauer, U., Boesecke, C., Dilloo, D., Engelhardt, A., Huelsmann, B., Rockstroh, J., Scholtes, C., Schoenberger, S., Wasmuth, J. C., Handgretinger, R., Henes, J., Holzer, U., Kanz, L., Ankermann, T., von Bismarck, P., Schreiber, S., Zeuner, R., Huppertz, H. I., Kaiser-Labusch, P., Greil, J., Jakoby, D., Kulozik, A. E., Graf, N., Heine, S., Garwer, B., Kobbe, R., Lehmberg, K., Mueller, I., Herrmann, F., Horneff, G., Klein, A., Peitz, J., Schmidt, N., Apel, K., Bielack, S., Gross-Wieltsch, U., Deutz, P., Lassay, L., Kamitz, D., Stienen, A., Tenbrock, K., Wagner, N., Classen, C. F., Weiss, M., Bernbeck, B., Brummel, B., Lara-Villacanas, E., Muenstermann, E., Schneider, D., Tietsch, N., Westkemper, M., Naumann-Bartsch, N., Metzler, M., Sobik, B., Kuehnle, I., Kullmann, S., Kramm, C., Girschick, H., Elbe, S., Specker, C., Vinnemeier-Laubenthal, L., Haenicke, H., Schulz, C., Schweigerer, L., Mueller, T. G., Kindle, G., Grimbacher, B., El-Helou, S. M., Biegner, A. K., Bode, S., Ehl, S., Heeg, M., Ritterbusch, H., Rusch, S., Schmitt, R., Warnatz, K., Baerlecken, N. T., Baumann, U., Beider, R., Ernst, D., Gerschmann, S., Klemann, C., Mielke, G., Schmidt, R. E., Schuermann, G., Viemann, D., Albert, M., Eichinger, A., Eisl, E., Hauck, F., Klein, C., Sollinger, F., von Bernuth, H., Hanitsch, L., Krueger, R., Scheibenbogen, C., Avila, A., Borte, M., Borte, S., Fasshauer, M., Hauenherm, A., Kellner, N., Mueller, H., Uelzen, A., Bader, P., Bakhtiar, S., Hess, U., Lee, J. Y., Schubert, R., Voss, S., Zielen, S., Bienemann, K., Lankisch, P., Laws, H. J., Neubert, J., Dueckers, G., Lamers, B., Langemeyer, V., Niehues, T., Hoenig, M., Schulz, A., Schwarz, K., Steinmann, S., Graf, D., Haase, G., Liese, J. G., Morbach, H., Schwaneck, E., Tony, H. P., Foell, D., Hellige, A., Masjosthusmann, K., Mohr, M., Wittkowski, H., Fischer, J. C., Hedrich, C. M., Roesen-Wolff, A., Roesler, J., Behrends, U., Rieber, N., Schauer, U., Boesecke, C., Dilloo, D., Engelhardt, A., Huelsmann, B., Rockstroh, J., Scholtes, C., Schoenberger, S., Wasmuth, J. C., Handgretinger, R., Henes, J., Holzer, U., Kanz, L., Ankermann, T., von Bismarck, P., Schreiber, S., Zeuner, R., Huppertz, H. I., Kaiser-Labusch, P., Greil, J., Jakoby, D., Kulozik, A. E., Graf, N., Heine, S., Garwer, B., Kobbe, R., Lehmberg, K., Mueller, I., Herrmann, F., Horneff, G., Klein, A., Peitz, J., Schmidt, N., Apel, K., Bielack, S., Gross-Wieltsch, U., Deutz, P., Lassay, L., Kamitz, D., Stienen, A., Tenbrock, K., Wagner, N., Classen, C. F., Weiss, M., Bernbeck, B., Brummel, B., Lara-Villacanas, E., Muenstermann, E., Schneider, D., Tietsch, N., Westkemper, M., Naumann-Bartsch, N., Metzler, M., Sobik, B., Kuehnle, I., Kullmann, S., Kramm, C., Girschick, H., Elbe, S., Specker, C., Vinnemeier-Laubenthal, L., Haenicke, H., Schulz, C., Schweigerer, L., Mueller, T. G., Kindle, G., and Grimbacher, B.

Published
2017

24. Impaired IL-23–dependent induction of IFN-γ underlies mycobacterial disease in patients with inherited TYK2 deficiency

Author

Ogishi, Masato, Arias, Andrés Augusto, Yang, Rui, Han, Ji Eun, Zhang, Peng, Rinchai, Darawan, Halpern, Joshua, Mulwa, Jeanette, Keating, Narelle, Chrabieh, Maya, Lainé, Candice, Seeleuthner, Yoann, Ramírez-Alejo, Noé, Nekooie-Marnany, Nioosha, Guennoun, Andrea, Muller-Fleckenstein, Ingrid, Fleckenstein, Bernhard, Kilic, Sara S., Minegishi, Yoshiyuki, Ehl, Stephan, Kaiser-Labusch, Petra, Kendir-Demirkol, Yasemin, Rozenberg, Flore, Errami, Abderrahmane, Zhang, Shen-Ying, Zhang, Qian, Bohlen, Jonathan, Philippot, Quentin, Puel, Anne, Jouanguy, Emmanuelle, Pourmoghaddas, Zahra, Bakhtiar, Shahrzad, Willasch, Andre M., Horneff, Gerd, Llanora, Genevieve, Shek, Lynette P., Chai, Louis Y.A., Tay, Sen Hee, Rahimi, Hamid H., Mahdaviani, Seyed Alireza, Nepesov, Serdar, Bousfiha, Aziz A., Erdeniz, Emine Hafize, Karbuz, Adem, Marr, Nico, Navarrete, Carmen, Adeli, Mehdi, Hammarstrom, Lennart, Abolhassani, Hassan, Parvaneh, Nima, Al Muhsen, Saleh, Alosaimi, Mohammed F., Alsohime, Fahad, Nourizadeh, Maryam, Moin, Mostafa, Arnaout, Rand, Alshareef, Saad, El-Baghdadi, Jamila, Genel, Ferah, Sherkat, Roya, Kiykim, Ayça, Yücel, Esra, Keles, Sevgi, Bustamante, Jacinta, Abel, Laurent, Casanova, Jean-Laurent, and Boisson-Dupuis, Stéphanie

Abstract

Human cells homozygous for rare loss-of-expression (LOE) TYK2 alleles have impaired, but not abolished, cellular responses to IFN-α/β (underlying viral diseases in the patients) and to IL-12 and IL-23 (underlying mycobacterial diseases). Cells homozygous for the common P1104A TYK2 allele have selectively impaired responses to IL-23 (underlying isolated mycobacterial disease). We report three new forms of TYK2 deficiency in six patients from five families homozygous for rare TYK2 alleles (R864C, G996R, G634E, or G1010D) or compound heterozygous for P1104A and a rare allele (A928V). All these missense alleles encode detectable proteins. The R864C and G1010D alleles are hypomorphic and loss-of-function (LOF), respectively, across signaling pathways. By contrast, hypomorphic G996R, G634E, and A928V mutations selectively impair responses to IL-23, like P1104A. Impairment of the IL-23–dependent induction of IFN-γ is the only mechanism of mycobacterial disease common to patients with complete TYK2 deficiency with or without TYK2 expression, partial TYK2 deficiency across signaling pathways, or rare or common partial TYK2 deficiency specific for IL-23 signaling.

Published
2022
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25. Functional flow cytometry of monocytes for routine diagnosis of innate primary immunodeficiencies.

Author

Ammann, Sandra, Fuchs, Sebastian, Martin-Martin, Lydia, Castro, Carla N., Spielberger, Benedikt, Klemann, Christian, Elling, Roland, Heeg, Maximilian, Speckmann, Carsten, Hainmann, Ina, Kaiser-Labusch, Petra, Horneff, Gerd, Thalhammer, Julian, Bredius, Robbert G., Stadt, Udo zur, Lehmberg, Kai, Fuchs, Ilka, von Spee-Mayer, Caroline, Henneke, Philipp, and Ehl, Stephan

Published
2020
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26. Randomization of Left-Right Asymmetry and Congenital Heart Defects

Author

Nöthe-Menchen, Tabea, Wallmeier, Julia, Pennekamp, Petra, Höben, Inga M., Olbrich, Heike, Loges, Niki T., Raidt, Johanna, Dougherty, Gerard W., Hjeij, Rim, Dworniczak, Bernd, Omran, Heymut, Amirav, Israel, Biebach, Luisa, Fabricius, Dorit, Griese, Matthias, Große-Onnebrink, Jörg, Häffner, Karsten, Hector, Andreas, Jung, Andreas, Kaiser-Labusch, Petra, Kaiser, Thomas, Keßler, Christina, Kitz, Richard, Knowles, Michael R., Koerner-Rettberg, Cordula, Kristoffersson, Ulf, Leigh, Margaret W., Mertsch, Pontus, Mischo, Bernhard, Nielsen, Kim G., Poeta, Marco, Rietschel, Ernst, Roth, Samra, Santamaria, Francesca, Schmalstieg, Christian, Schmidts, Miriam, Schwarz, Carsten, Schwerk, Nicolaus, Seithe, Horst, Tebbe, Johannes, Werner, Claudius, and Zariwala, Maimoona A.

Abstract

Supplemental Digital Content is available in the text.

Published
2019
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27. Initial presenting manifestations in 16,486 patients with inborn errors of immunity include infections and noninfectious manifestations

Author

Tim Niehues, Catherine Waruiru, Conleth Feighery, Uwe Schauer, Virginie Courteille, Kai Lehmberg, Ingo Müller, I. Esteves, Henner Morbach, Michael Borte, Patrick Hundsdoerfer, Klaus Schwarz, Ewelina Gowin, Alessandro Aiuti, Andreas Holbro, Federica Barzaghi, João Farela Neves, Dagmar Graf, Hannah Tamary, Veneta Milenova, Benedikt Boetticher, Eleonora Gambineri, Vera Goda, Alia Eldash, Jan-Christian Wasmuth, Fabio Candotti, Svetlana O. Sharapova, Markus Metzler, Juergen Brunner, Anna Hilfanova, Brindusa Ruxandra Capilna, Pere Soler-Palacín, Arnau Antolí, Horst von Bernuth, Vassilios Lougaris, Maria Carrabba, Bernd H. Belohradsky, Julian Thalhammer, Nathalie de Vergnes, Peter Olbrich, Peter Kopač, Leif G. Hanitsch, Alexandra Nieters, Filomeen Haerynck, Juliana Gabzdilova, Sezin Aydemir, Rabab El Hawary, Patrick F.K. Yong, Maria Giovanna Danieli, Alberto Tommasini, Sandra Steinmann, Ulrich Baumann, Figen Dogu, Elisabeth Förster-Waldl, Carolina Marasco, Donato Amodio, Lorenzo Lodi, Xavier Solanich, Caterina Cancrini, Brigita Sitkauskiene, Torsten Witte, Clementina Vanessa, Nima Rezaei, Jean-Christophe Goffard, Kirsten Wittke, Emmanouil Liatsis, Helen Baxendale, Susana L. Silva, Bodo Grimbacher, Henrike Ritterbusch, Evangelia Farmaki, Safa Meshaal, Sujal Ghosh, Larysa Kostyuchenko, David Edgar, Simone Cesaro, R Zeuner, Nerea Salmón Rodríguez, Isabella Quinti, Stephan Ehl, Pauline Brosselin, Joerg C. Henes, Pilar Llobet Agulló, Rosa Maria Dellepiane, Andrea Meinhardt, Marina Kojić, Georgios Sogkas, Stephan Borte, Catharina Schuetz, Suheyla Ocak, Karin Marschall, Lukas M. Gasteiger, Stefan Raffac, Sofia Tantou, Sadia Noorani, Matthaios Speletas, Philippe Randrianomenjanahary, Ursula Holzer, Ayca Kiykim, Johannes G. Liese, Angelo Vacca, Gisela Fecker, Ekrem Unal, Koen J. van Aerde, Alba Parra-Martínez, Kaan Boztug, Sophie Stiehler, Sybille Landwehr-Kenzel, Claudio Pignata, Jennifer Neubert, Janine Reichenbach, Shahnaz Parvin, Sarah Goddard, Andrea Schroll, Dirk Holzinger, Asghar Aghamohammadi, Hassan Abolhassani, Johannes Trück, Estela Paz-Artal, Shereen M. Reda, Anna Shcherbina, Maria Raptaki, Jaroslava Orosova, Beata Wolska-Kuśnierz, Tessa Kerre, Gerrit Ahrenstorf, Ben Zion Garty, Dirk Foell, Benjamin Becker, Ulrike F. Demel, Androniki Kapousouzi, Abraham Rutgers, Klaus Warnatz, Gemma Rocamora Blanch, Stephan Rusch, Luis M. Allende, Dalia Abd Elaziz, Safa Baris, Jorisvan Montfrans, Dominik T. Schneider, Raphael Scheible, Juana Gil-Herrera, Gerhard Kindle, Annarosa Soresina, Giovanna Fabio, Uwe Wintergerst, Emilia Faria, Maria Fasshauer, Silvia Ricci, Aisha Elmarsafy, Barbara Pietrucha, Carsten Speckmann, Nizar Mahlaoui, Ulrich Heininger, Isabelle Meyts, Matthew Buckland, Efimia Papadopoulou-Alataki, Robin Kobbe, A Herwadkar, Sebastian F. N. Bode, Ali Sobh, László Maródi, Baldassarre Martire, Chiara Azzari, Maximilian Heeg, Katja Masjosthusmann, Michael H. Albert, Matteo Chinello, Juan Luis Santos-Pérez, Aarnoud Huissoon, Tanya I. Coulter, Hendrik Schulze-Koops, Norbert Graf, Radwa Alkady, Jolanta Bernatoniene, Seraina Prader, Alenka Gagro, Joachim Roesler, Taco W. Kuijpers, Ewa Więsik-Szewczyk, Maria Elena Maccari, Conrad Ferdinand Lippert, Miriam González-Amores, Johannes Dirks, Daniel E Pleguezuelo, Christof M. Kramm, Anders Fasth, Volker Schuster, Olov Ekwall, Nikolaus Rieber, Javier Carbone, Petra Kaiser-Labusch, Diana Ernst, Lucia Augusta Baselli, Luis Ignacio Gonzalez-Granado, Maria Kanariou, Stefanie S. V. Henriet, Sigune Goldacker, Kerstin Felgentreff, Oana Joean, Fine Roosens, Fabian Hauck, Eva C. Schwaneck, Milos Jesenak, Manfred Hoenig, Lenka Kapustova, Christoph Boesecke, Alain Fischer, Sara Pereira da Silva, Julia Körholz, Ansgar Schulz, Carolynne Schwarze-Zander, Mikko Seppänen, Nermeen Galal, Nora Naumann-Bartsch, Tomaz Garcez, Peter Ciznar, Klara M. Posfay-Barbe, Zelimir Pavle Eric, Reinhold E. Schmidt, Hermann J. Girschick, Sabine Heine, Anika-Kerstin Biegner, Annick A. J. M. van de Ven, Stefan Schreiber, J. Merlijn van den Berg, Nurit Assia Batzir, Alexandra Jablonka, Kim Stol, Gregor Dückers, Antonios G.A. Kolios, Ioannis Kakkas, Christian Klemann, Marina N. Guseva, Sofia Grigoriadou, Elif Karakoc-Aydiner, Antonio Marzollo, Peter D. Arkwright, Urs C. Steiner, Sara Sebnem Kilic, Romina Dieli-Crimi, Gergely Kriván, Monika Sparber-Sauer, Marco Cazzaniga, Fulvio Porta, Paraskevi Maggina, Tomas Milota, Robbert G. M. Bredius, Martine Pergent, Klaus Tenbrock, Jana Pachlopnik Schmid, Florentia Dimitriou, Cathal Laurence Steele, Helen Bourne, Anna Bobcakova, Gerd Horneff, Judith Potjewijd, Marc Schmalzing, Tobias Ankermann, Paul Ryan, Oksana Boyarchuk, Necil Kutukculer, Carl Friedrich Classen, Zita Chovancová, Moira Thomas, Cinzia Milito, Michaela Bitzenhofer-Grüber, Faranaz Atschekzei, Eva Hlaváčková, Viviana Moschese, Julie Smet, Hans-Hartmut Peter, Carla Teixeira, Sabine M El-Helou, Suzanne de Kruijf Bazen, Helmut Wittkowski, Donate Jakoby, Marina Garcia-Prat, Esther de Vries, Richard Herriot, Sven Kracker, Alessandro Plebani, Lisa Göschl, Laura Hora Marques, Anna Sediva, Jiri Litzman, Mark M. Gompels, Renate Krüger, Şefika İlknur Kökçü Karadağ, Nadine Binder, Anna Szaflarska, Peter Jandus, Lisa Ibberson, Johann Greil, Ulf Schulze-Sturm, Mehtap Sirin, Aydan Ikinciogullari, Edyta Heropolitańska-Pliszka, Michael E. Weiss, Alla Skapenko, Lukas Wisgrill, Hana Alachkar, Uta Behrends, Silvia Sánchez-Ramón, Maria N. Hatzistilianou, Otilia Petrovicova, Darko Richter, Zoreh Nademi, Jürgen K. Rockstroh, Sohilla Lotfy, Markus G. Seidel, Timothy Ronan Leahy, Audra Blažienė, Translational Immunology Groningen (TRIGR), Paediatric Infectious Diseases / Rheumatology / Immunology, AII - Inflammatory diseases, ARD - Amsterdam Reproduction and Development, University of Zurich, Ehl, Stephan, Thalhammer, J., Kindle, G., Nieters, A., Rusch, S., Seppanen, M. R. J., Fischer, A., Grimbacher, B., Edgar, D., Buckland, M., Mahlaoui, N., Ehl, S., Boztug, K., Brunner, J., Demel, U. F., Forster-Waldl, E., Gasteiger, L. M., Goschl, L., Kojic, M., Schroll, A., Seidel, M. G., Wintergerst, U., Wisgrill, L., Sharapova, S. O., Goffard, J. -C., Kerre, T., Meyts, I., Roosens, F., Smet, J., Haerynck, F., Eric, Z. P., Milenova, V., Gagro, A., Richter, D., Chovancova, Z., Hlavackova, E., Litzman, J., Milota, T., Sediva, A., Elaziz, D. A., Alkady, R. S., El Sayed El Hawary, R., Eldash, A. S., Galal, N., Lotfy, S., Meshaal, S. S., Reda, S. M., Sobh, A., Elmarsafy, A., Brosselin, P., Courteille, V., De Vergnes, N., Kracker, S., Pergent, M., Randrianomenjanahary, P., Ahrenstorf, G., Albert, M. H., Ankermann, T., Atschekzei, F., Baumann, U., Becker, B. C., Behrends, U., Belohradsky, B. H., Biegner, A. -K., Binder, N., Bode, S. F. N., Boesecke, C., Boetticher, B., Borte, M., Borte, S., Classen, C. F., Dirks, J., Duckers, G., El-Helou, S., Ernst, D., Fasshauer, M., Fecker, G., Felgentreff, K., Foell, D., Ghosh, S., Girschick, H. J., Goldacker, S., Graf, N., Graf, D., Greil, J., Hanitsch, L. G., Hauck, F., Heeg, M., Heine, S. I., Henes, J. C., Hoenig, M., Holzer, U., Holzinger, D., Horneff, G., Hundsdoerfer, P., Jablonka, A., Jakoby, D., Joean, O., Kaiser-Labusch, P., Klemann, C., Kobbe, R., Korholz, J., Kramm, C. M., Kruger, R., Landwehr-Kenzel, S., Lehmberg, K., Liese, J. G., Lippert, C. F., Maccari, M. E., Masjosthusmann, K., Meinhardt, A., Metzler, M., Morbach, H., Muller, I., Naumann-Bartsch, N., Neubert, J., Niehues, T., Peter, H. -H., Rieber, N., Ritterbusch, H., Rockstroh, J. K., Roesler, J., Schauer, U., Scheible, R., Schmalzing, M., Schmidt, R. E., Schneider, D. T., Schreiber, S., Schuetz, C., Schulz, A., Schulze-Koops, H., Schulze-Sturm, U., Schuster, V., Schwaneck, E. C., Schwarz, K., Schwarze-Zander, C., Sirin, M., Skapenko, A., Sogkas, G., Sparber-Sauer, M., Speckmann, C., Steinmann, S., Stiehler, S., Tenbrock, K., von Bernuth, H., Warnatz, K., Wasmuth, J. -C., Weiss, M., Witte, T., Wittke, K., Wittkowski, H., Zeuner, R. A., Farmaki, E., Hatzistilianou, M. N., Kakkas, I., Kanariou, M. G., Kapousouzi, A., Liatsis, E., Maggina, P., Papadopoulou-Alataki, E., Raptaki, M., Speletas, M., Tantou, S., Goda, V., Krivan, G., Marodi, L., Abolhassani, H., Aghamohammadi, A., Rezaei, N., Feighery, C., Leahy, T. R., Ryan, P., Batzir, N. A., Garty, B. Z., Tamary, H., Aiuti, A., Amodio, D., Azzari, C., Barzaghi, F., Baselli, L. A., Cancrini, C., Carrabba, M., Cazzaniga, M., Cesaro, S., Chinello, M., Danieli, M. G., Dellepiane, R. M., Fabio, G., Gambineri, E., Lodi, L., Lougaris, V., Marasco, C., Martire, B., Marzollo, A., Milito, C., Moschese, V., Pignata, C., Plebani, A., Porta, F., Quinti, I., Ricci, S., Soresina, A., Tommasini, A., Vacca, A., Vanessa, C., Blaziene, A., Sitkauskiene, B., Gowin, E., Heropolitanska-Pliszka, E., Pietrucha, B., Szaflarska, A., Wiesik-Szewczyk, E., Wolska-Kusnierz, B., Esteves, I., Faria, E., Marques, L. H., Neves, J. F., Silva, S. L., Teixeira, C., Pereira da Silva, S., Capilna, B. R., Guseva, M. N., Shcherbina, A., Bobcakova, A., Ciznar, P., Gabzdilova, J., Jesenak, M., Kapustova, L., Orosova, J., Petrovicova, O., Raffac, S., Kopac, P., Allende, L. M., Antoli, A., Blanch, G. R., Carbone, J., Dieli-Crimi, R., Garcia-Prat, M., Gil-Herrera, J., Gonzalez-Granado, L. I., Agullo, P. L., Olbrich, P., Parra-Martinez, A., Paz-Artal, E., Pleguezuelo, D. E., Rodriguez, N. S., Sanchez-Ramon, S., Santos-Perez, J. L., Solanich, X., Soler-Palacin, P., Gonzalez-Amores, M., Ekwall, O., Fasth, A., Bitzenhofer-Gruber, M., Candotti, F., Dimitriou, F., Heininger, U., Holbro, A., Jandus, P., Kolios, A. G. A., Marschall, K., Schmid, J. P., Posfay-Barbe, K. M., Prader, S., Reichenbach, J., Steiner, U. C., Truck, J., Bredius, R. G., de Kruijf- Bazen, S., de Vries, E., Henriet, S. S. V., Kuijpers, T. W., Potjewijd, J., Rutgers, A., Stol, K., van Aerde, K. J., Van den Berg, J. M., van de Ven, A. A. J. M., Montfrans, J., Aydemir, S., Baris, S., Dogu, F., Ikinciogullari, A., Karakoc-Aydiner, E., Kilic, S. S., Kiykim, A., Kokcu Karadag, S. I., Kutukculer, N., Ocak, S., Unal, E., Boyarchuk, O., Hilfanova, A., Kostyuchenko, L. V., Alachkar, H., Arkwright, P. D., Baxendale, H. E., Bernatoniene, J., Coulter, T. I., Garcez, T., Goddard, S., Gompels, M. M., Grigoriadou, S., Herriot, R., Herwadkar, A., Huissoon, A., Ibberson, L., Nademi, Z., Noorani, S., Parvin, S., Steele, C. L., Thomas, M., Waruiru, C., Yong, P. F. K., and Bourne, H.

Subjects
0301 basic medicine, Male, Pediatrics, syndromic, Sex Factor, Disease, registry, medicine.disease_cause, Cohort Studies, 0302 clinical medicine, Primary Immunodeficiency Disease, inborn error of immunity, Immunology and Allergy, warning signs, Age Factor, Registries, Family history, presenting symptom, Child, Primary immunodeficiency, Granuloma, autoimmune, immune dysregulation, inflammatory, Adult, Autoimmune Diseases, Female, Humans, Infections, Lymphoproliferative Disorders, Middle Aged, Primary Immunodeficiency Diseases, Sex Factors, Age Factors, 10177 Dermatology Clinic, Infections/epidemiology, 3. Good health, Settore MED/02, Warning signs, Lymphoproliferative Disorder, 2723 Immunology and Allergy, Infection, Human, medicine.medical_specialty, Immunology, 610 Medicine & health, Malignancy, primary immunodeficiency, Autoimmune Disease, 03 medical and health sciences, Immunity, Autoimmune Diseases/epidemiology, medicine, 2403 Immunology, business.industry, warning sign, Common variable immunodeficiency, Granuloma/epidemiology, Immune dysregulation, medicine.disease, Primary Immunodeficiency Diseases/epidemiology, 030104 developmental biology, Lymphoproliferative Disorders/epidemiology, Cohort Studie, business, 030215 immunology
Abstract

BACKGROUND: Inborn errors of immunity (IEI) are rare diseases, which makes diagnosis a challenge. A better description of the initial presenting manifestations should improve awareness and avoid diagnostic delay. Although increased infection susceptibility is a well-known initial IEI manifestation, less is known about the frequency of other presenting manifestations.OBJECTIVE: We sought to analyze age-related initial presenting manifestations of IEI including different IEI disease cohorts.METHODS: We analyzed data on 16,486 patients of the European Society for Immunodeficiencies Registry. Patients with autoinflammatory diseases were excluded because of the limited number registered.RESULTS: Overall, 68% of patients initially presented with infections only, 9% with immune dysregulation only, and 9% with a combination of both. Syndromic features were the presenting feature in 12%, 4% had laboratory abnormalities only, 1.5% were diagnosed because of family history only, and 0.8% presented with malignancy. Two-third of patients with IEI presented before the age of 6 years, but a quarter of patients developed initial symptoms only as adults. Immune dysregulation was most frequently recognized as an initial IEI manifestation between age 6 and 25 years, with male predominance until age 10 years, shifting to female predominance after age 40 years. Infections were most prevalent as a first manifestation in patients presenting after age 30 years.CONCLUSIONS: An exclusive focus on infection-centered warning signs would have missed around 25% of patients with IEI who initially present with other manifestations.

Published
2021

28. Case Report: Rubella Virus-Induced Cutaneous Granulomas in Two Pediatric Patients With DNA Double Strand Breakage Repair Disorders - Outcome After Hematopoietic Stem Cell Transplantation.

Author

Baumann U, Schulte JH, Groß JP, Beier R, Ludwig M, Wahn V, Hofmann J, Maecker-Kolhoff B, Sauer M, Kaiser-Labusch P, Karimian N, Blume-Peytavi U, Ghoreschi F, Ott H, Perelygina L, Klemann C, Blankenstein O, von Bernuth H, and Krüger R

Subjects
Child, Granuloma genetics, Humans, Infant, Newborn, Rubella virus genetics, Ataxia Telangiectasia genetics, DNA Repair-Deficiency Disorders complications, Hematopoietic Stem Cell Transplantation adverse effects, Immunologic Deficiency Syndromes genetics
Abstract

We report two patients with DNA repair disorders (Artemis deficiency, Ataxia telangiectasia) with destructive skin granulomas, presumably triggered by live-attenuated rubella vaccinations. Both patients showed reduced naïve T cells. Rapid resolution of skin lesions was observed following hematopoietic stem cell transplantation. However, the patient with AT died due to complications of severe hepatic veno-occlusive disease 6 month after HSCT. Dried blood spots obtained after birth were available from this patient and showed absent T-cell receptor excision circles (TRECs). Therefore, newborn screening may help to prevent patients with moderate T-cell deficiency from receiving live-attenuated rubella vaccine potentially causing granulomas., Competing Interests: Authors JH and HB were employed by Labor Berlin GmbH The remaining authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 Baumann, Schulte, Groß, Beier, Ludwig, Wahn, Hofmann, Maecker-Kolhoff, Sauer, Kaiser-Labusch, Karimian, Blume-Peytavi, Ghoreschi, Ott, Perelygina, Klemann, Blankenstein, von Bernuth and Krüger.)

Published
2022
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29. The expansion of human T-bet high CD21 low B cells is T cell dependent.

Author

Keller B, Strohmeier V, Harder I, Unger S, Payne KJ, Andrieux G, Boerries M, Felixberger PT, Landry JJM, Nieters A, Rensing-Ehl A, Salzer U, Frede N, Usadel S, Elling R, Speckmann C, Hainmann I, Ralph E, Gilmour K, Wentink MWJ, van der Burg M, Kuehn HS, Rosenzweig SD, Kölsch U, von Bernuth H, Kaiser-Labusch P, Gothe F, Hambleton S, Vlagea AD, Garcia Garcia A, Alsina L, Markelj G, Avcin T, Vasconcelos J, Guedes M, Ding JY, Ku CL, Shadur B, Avery DT, Venhoff N, Thiel J, Becker H, Erazo-Borrás L, Trujillo-Vargas CM, Franco JL, Fieschi C, Okada S, Gray PE, Uzel G, Casanova JL, Fliegauf M, Grimbacher B, Eibel H, Ehl S, Voll RE, Rizzi M, Stepensky P, Benes V, Ma CS, Bossen C, Tangye SG, and Warnatz K

Subjects
Adult, Cohort Studies, Female, Humans, Male, Middle Aged, B-Lymphocytes immunology, Receptors, Complement 3d immunology, T-Box Domain Proteins immunology, T-Lymphocytes immunology
Abstract

Accumulation of human CD21 low B cells in peripheral blood is a hallmark of chronic activation of the adaptive immune system in certain infections and autoimmune disorders. The molecular pathways underpinning the development, function, and fate of these CD21 low B cells remain incompletely characterized. Here, combined transcriptomic and chromatin accessibility analyses supported a prominent role for the transcription factor T-bet in the transcriptional regulation of these T-bet high CD21 low B cells. Investigating essential signals for generating these cells in vitro established that B cell receptor (BCR)/interferon-γ receptor (IFNγR) costimulation induced the highest levels of T-bet expression and enabled their differentiation during cell cultures with Toll-like receptor (TLR) ligand or CD40L/interleukin-21 (IL-21) stimulation. Low proportions of CD21 low B cells in peripheral blood from patients with defined inborn errors of immunity (IEI), because of mutations affecting canonical NF-κB, CD40, and IL-21 receptor or IL-12/IFNγ/IFNγ receptor/signal transducer and activator of transcription 1 (STAT1) signaling, substantiated the essential roles of BCR- and certain T cell–derived signals in the in vivo expansion of T-bet high CD21 low B cells. Disturbed TLR signaling due to MyD88 or IRAK4 deficiency was not associated with reduced CD21 low B cell proportions. The expansion of human T-bet high CD21 low B cells correlated with an expansion of circulating T follicular helper 1 (cTfh1) and T peripheral helper (Tph) cells, identifying potential sources of CD40L, IL-21, and IFNγ signals. Thus, we identified important pathways to target autoreactive T-bet high CD21 low B cells in human autoimmune conditions, where these cells are linked to pathogenesis and disease progression.

Published
2021
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30. Pediatric Community-Acquired Bone and Joint Staphylococcus Aureus Infections In Europe: Severe Infections are Associated to Panton-Valentine Leucocidin Presence.

Author

Gijón M, Bellusci M, Petraitiene B, Noguera-Julian A, Glikman D, Saavedra-Lozano J, Neth O, Daskalaki M, Zilinskaite V, Kaiser-Labusch P, Prieto L, and Rojo P

Subjects
Adolescent, Arthritis, Infectious microbiology, Bone and Bones microbiology, Child, Child, Preschool, Europe epidemiology, Humans, Infant, Infant, Newborn, Joints microbiology, Methicillin-Resistant Staphylococcus aureus pathogenicity, Prospective Studies, Severity of Illness Index, Staphylococcal Infections transmission, Staphylococcus aureus genetics, Staphylococcus aureus pathogenicity, Virulence Factors, Arthritis, Infectious epidemiology, Bacterial Toxins genetics, Community-Acquired Infections epidemiology, Community-Acquired Infections microbiology, Exotoxins genetics, Leukocidins genetics, Staphylococcal Infections epidemiology
Abstract

To analyze host and pathogen factors related to disease severity of community-acquired bone and joint infections in children, a cohort of pediatric patients was prospectively recruited from 13 centers in 7 European countries. A total of 85 children were included, 11 (13%) had a severe infection. Panton-Valentine leukocidin-positive isolates were 17%, and 6% of the isolates were methicillin-resistant Staphylococcus aureus. Multivariate analysis identified Panton-Valentine leukocidin presence (adjusted odds ratio, 12.6; P = 0.01) as the only factor independently associated with severe outcome, regardless of methicillin resistance.

Published
2020
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31. Risk Factors for Complicated Lymphadenitis Caused by Nontuberculous Mycobacteria in Children.

Author

Kuntz M, Kohlfürst DS, Feiterna-Sperling C, Krüger R, Baumann U, Buchtala L, Elling R, Grote V, Hübner J, Hufnagel M, Kaiser-Labusch P, Liese J, Otto EM, Rose MA, Schneider C, Schuster V, Seidl M, Sommerburg O, Vogel M, von Bernuth H, Weiß M, Zimmermann T, Nieters A, Zenz W, and Henneke P

Subjects
Adolescent, Age Factors, Austria epidemiology, Child, Child, Preschool, Female, Germany epidemiology, Humans, Infant, Infant, Newborn, Lymphadenitis microbiology, Male, Mycobacterium Infections, Nontuberculous microbiology, Registries, Retrospective Studies, Risk Factors, Seasons, Sex Factors, Lymphadenitis epidemiology, Mycobacterium Infections, Nontuberculous epidemiology, Nontuberculous Mycobacteria isolation & purification
Abstract

Nontuberculous mycobacteria (NTM) are an emerging cause of infections, including chronic lymphadenitis in children. To identify risk factors for NTM lymphadenitis, particularly complicated disease, we collected epidemiologic, clinical, and microbiological data on 138 cases of NTM lymphadenitis in children across 13 centers in Germany and Austria. We assessed lifestyle factors but did not identify specific risk behaviors. We noted that more cases of NTM lymphadenitis occurred during cold months than during warm months. Moreover, we noted female sex and age <5.5 years as potential risk factors. Complete extirpation of the affected lymph node appeared to be the best therapeutic measure. We integrated the study data to develop a simple risk score to predict unfavorable clinical outcomes for NTM lymphadenitis.

Published
2020
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32. The German National Registry of Primary Immunodeficiencies (2012-2017).

Author

El-Helou SM, Biegner AK, Bode S, Ehl SR, Heeg M, Maccari ME, Ritterbusch H, Speckmann C, Rusch S, Scheible R, Warnatz K, Atschekzei F, Beider R, Ernst D, Gerschmann S, Jablonka A, Mielke G, Schmidt RE, Schürmann G, Sogkas G, Baumann UH, Klemann C, Viemann D, von Bernuth H, Krüger R, Hanitsch LG, Scheibenbogen CM, Wittke K, Albert MH, Eichinger A, Hauck F, Klein C, Rack-Hoch A, Sollinger FM, Avila A, Borte M, Borte S, Fasshauer M, Hauenherm A, Kellner N, Müller AH, Ülzen A, Bader P, Bakhtiar S, Lee JY, Heß U, Schubert R, Wölke S, Zielen S, Ghosh S, Laws HJ, Neubert J, Oommen PT, Hönig M, Schulz A, Steinmann S, Schwarz K, Dückers G, Lamers B, Langemeyer V, Niehues T, Shai S, Graf D, Müglich C, Schmalzing MT, Schwaneck EC, Tony HP, Dirks J, Haase G, Liese JG, Morbach H, Foell D, Hellige A, Wittkowski H, Masjosthusmann K, Mohr M, Geberzahn L, Hedrich CM, Müller C, Rösen-Wolff A, Roesler J, Zimmermann A, Behrends U, Rieber N, Schauer U, Handgretinger R, Holzer U, Henes J, Kanz L, Boesecke C, Rockstroh JK, Schwarze-Zander C, Wasmuth JC, Dilloo D, Hülsmann B, Schönberger S, Schreiber S, Zeuner R, Ankermann T, von Bismarck P, Huppertz HI, Kaiser-Labusch P, Greil J, Jakoby D, Kulozik AE, Metzler M, Naumann-Bartsch N, Sobik B, Graf N, Heine S, Kobbe R, Lehmberg K, Müller I, Herrmann F, Horneff G, Klein A, Peitz J, Schmidt N, Bielack S, Groß-Wieltsch U, Classen CF, Klasen J, Deutz P, Kamitz D, Lassay L, Tenbrock K, Wagner N, Bernbeck B, Brummel B, Lara-Villacanas E, Münstermann E, Schneider DT, Tietsch N, Westkemper M, Weiß M, Kramm C, Kühnle I, Kullmann S, Girschick H, Specker C, Vinnemeier-Laubenthal E, Haenicke H, Schulz C, Schweigerer L, Müller TG, Stiefel M, Belohradsky BH, Soetedjo V, Kindle G, and Grimbacher B

Subjects
Adolescent, Adult, Aged, Aged, 80 and over, Child, Child, Preschool, Delayed Diagnosis, Female, Genetic Therapy, Germany epidemiology, Hematopoietic Stem Cell Transplantation, Humans, Immunoglobulins therapeutic use, Infant, Infant, Newborn, Male, Middle Aged, Prevalence, Registries, Young Adult, Immunologic Deficiency Syndromes diagnosis, Immunologic Deficiency Syndromes epidemiology, Immunologic Deficiency Syndromes genetics, Immunologic Deficiency Syndromes therapy
Abstract

Introduction: The German PID-NET registry was founded in 2009, serving as the first national registry of patients with primary immunodeficiencies (PID) in Germany. It is part of the European Society for Immunodeficiencies (ESID) registry. The primary purpose of the registry is to gather data on the epidemiology, diagnostic delay, diagnosis, and treatment of PIDs. Methods: Clinical and laboratory data was collected from 2,453 patients from 36 German PID centres in an online registry. Data was analysed with the software Stata® and Excel. Results: The minimum prevalence of PID in Germany is 2.72 per 100,000 inhabitants. Among patients aged 1-25, there was a clear predominance of males. The median age of living patients ranged between 7 and 40 years, depending on the respective PID. Predominantly antibody disorders were the most prevalent group with 57% of all 2,453 PID patients (including 728 CVID patients). A gene defect was identified in 36% of patients. Familial cases were observed in 21% of patients. The age of onset for presenting symptoms ranged from birth to late adulthood (range 0-88 years). Presenting symptoms comprised infections (74%) and immune dysregulation (22%). Ninety-three patients were diagnosed without prior clinical symptoms. Regarding the general and clinical diagnostic delay, no PID had undergone a slight decrease within the last decade. However, both, SCID and hyper IgE- syndrome showed a substantial improvement in shortening the time between onset of symptoms and genetic diagnosis. Regarding treatment, 49% of all patients received immunoglobulin G (IgG) substitution (70%-subcutaneous; 29%-intravenous; 1%-unknown). Three-hundred patients underwent at least one hematopoietic stem cell transplantation (HSCT). Five patients had gene therapy. Conclusion: The German PID-NET registry is a precious tool for physicians, researchers, the pharmaceutical industry, politicians, and ultimately the patients, for whom the outcomes will eventually lead to a more timely diagnosis and better treatment.

Published
2019
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33. Tyrosine kinase 2 is not limiting human antiviral type III interferon responses.

Author

Fuchs S, Kaiser-Labusch P, Bank J, Ammann S, Kolb-Kokocinski A, Edelbusch C, Omran H, and Ehl S

Subjects
Animals, Cell Line, Child, Disease Susceptibility immunology, Disease Susceptibility virology, Eczema etiology, Eczema immunology, Humans, Immunoglobulin E blood, Interferon-gamma immunology, Interferon-gamma metabolism, Interleukin-10 genetics, Interleukin-10 immunology, Interleukin-6 genetics, Interleukin-6 immunology, Mice, Mutation, Receptors, Cytokine immunology, Receptors, Interleukin-10 genetics, Receptors, Interleukin-10 immunology, Signal Transduction immunology, Skin pathology, TYK2 Kinase genetics, TYK2 Kinase immunology, Interferons immunology, Job Syndrome immunology, Killer Cells, Natural immunology, TYK2 Kinase deficiency, TYK2 Kinase metabolism
Abstract

Tyrosine kinase 2 (TYK2) associates with interferon (IFN) alpha receptor, IL-10 receptor (IL-10R) beta and other cytokine receptor subunits for signal transduction, in response to various cytokines, including type-I and type-III IFNs, IL-6, IL-10, IL-12 and IL-23. Data on TYK2 dependence on cytokine responses and in vivo consequences of TYK2 deficiency are inconsistent. We investigated a TYK2 deficient patient, presenting with eczema, skin abscesses, respiratory infections and IgE levels >1000 U/mL, without viral or mycobacterial infections and a corresponding cellular model to analyze the role of TYK2 in type-III IFN mediated responses and NK-cell function. We established a novel simple diagnostic monocyte assay to show that the mutation completely abolishes the IFN-α mediated antiviral response. It also partly reduces IL-10 but not IL-6 mediated signaling associated with reduced IL-10Rβ expression. However, we found almost normal type-III IFN signaling associated with minimal impairment of virus control in a TYK2 deficient human cell line. Contrary to observations in TYK2 deficient mice, NK-cell phenotype and function, including IL-12/IL-18 mediated responses, were normal in the patient. Thus, preserved type-III IFN responses and normal NK-cell function may contribute to antiviral protection in TYK2 deficiency leading to a surprisingly mild human phenotype., (© 2016 WILEY-VCH Verlag GmbH & Co. KGaA, Weinheim.)

Published
2016
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