未登录In adult X-CGD patients, regulatory T cells are expanded while activated T cells display a NOX2-independent ROS increase
Cammarata, Ilenia; Pinna, Valeria; Pacella, Ilenia; Rotella, Ivano; Soresina, Annarosa; Badolato, Raffaele; Plebani, Alessandro; Pignata, Claudio; Cirillo, Emilia; Zicari, Anna Maria; Violi, Francesco; Carnevale, Roberto; Loffredo, Lorenzo; Piconese, Silvia .png)
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收藏Interim analysis: Open-label extension study of leniolisib for patients with APDSRao, V. Koneti; Kulm, Elaine; Sediva, Anna; Plebani, Alessandro; Schuetz, Catharina; Shcherbina, Anna; Dalm, Virgil A.; Trizzino, Antonino; Zharankova, Yulia; Webster, Sharon; Orpia, Alanvin; Koeurorholz, Julia; Lougaris, Vassilios; Rodina, Yulia; Radford, Kath; Bradt, Jason; Relan, Anurag; Holland, Steven M.; Lenardo, Michael J.; Uzel, Gulbu 分享 收藏 Activated phosphoinositide 3-kinase d syndrome: Update from the ESID Registry and comparison with other autoimmune-lymphoproliferative inborn errors of immunity活化的磷酸肌醇3-激酶d综合征: 来自ESID注册表的更新以及与其他自身免疫-淋巴增生先天性免疫错误的比较Maccari, Maria Elena; Wolkewitz, Martin; Schwab, Charlotte; Lorenzini, Tiziana; Leiding, Jennifer W.; Aladjdi, Nathalie; Abolhassani, Hassan; Abou-Chahla, Wadih; Aiuti, Alessandro; Azarnoush, Saba; Baris, Safa; Barlogis, Vincent; Barzaghi, Federica; Baumann, Ulrich; Bloomfield, Marketa; Bohynikova, Nadezda; Bodet, Damien; Boutboul, David; Bucciol, Giorgia; Buckland, MattheW S.; Burns, Siobhan O.; Cancrini, Caterina; Cath, Pascal; Cavazzana, Marina; Cheminant, Morgane; Chinello, Matteo; Ciznar, Peter; Coulter, Tanya I.; D'Aveni, Maud; Ekwall, Olov; Eric, Zelimir; Eren, Efrem; Fasth, Anders; Frange, Pierre; Fournier, Benjamin; Garcia-Prat, Marina; Gardembas, Martine; Geier, Christoph; Ghosh, Sujal; Goda, Vera; Hammarstrom, Lennar; Hauck, Fabian; Heeg, Maximilian; Heropolitanska-Pliszka, Edyta; Hilfanova, Anna; Jolles, Stephen; Karakoc-Aydiner, Elif; Kindle, Gerhard R.; Kiykim, Ayca; Klemann, Christian; Koletsi, Patra; Koltan, Sylwia; Kondratenko, Irina; Korholz, Julia; Kruger, Renate; Jeziorski, Eric; Levy, Romain; Le Guenno, Guillaume; Lefevre, Guillaume; Lougaris, Vassilios; Marzollo, Antonio; Mahlaoui, Nizar; Malphettes, Marion; Meinhardt, Andrea; Merlin, Etienne; Meyts, Isabelle; Milota, Tomas; Moreira, Fernando; Moshous, Despina; Mukhina, Anna; Neth, Olaf; Neubert, Jennifer; Neven, Benedicte; Nieters, Alexandra; Nove-Josserand, Raphaele; Oksenhendler, Eric; Ozen, Ahmet; Olbrich, Peter; Perlat, Antoinette; Pac, Malgorzata; Schmid, Jana Pachlopnik; Pacillo, Lucia; Parra-Martinez, Alba; Paschenko, Olga; Pellier, Isabelle; Sefer, Asena Pinar; Plebani, Alessandro; Plantaz, Dominique; Prader, Seraina; Raffray, Loic; Ritterbusch, Henrike; Riviere, Jacques G.; Rivalta, Beatrice; Rusch, Stephan; Sakovich, Inga; Savic, Sinisa; Scheible, Raphael; Schleinitz, Nicolas; Schuetz, Catharina; Schulz, Ansgar; Sediva, Anna; Semeraro, Michaela; Sharapova, Svetlana O.; Shcherbina, Anna; Slatter, Mary A.; Sogkas, Georgios; Soler-Palacin, Pere; Speckmann, Carsten; Stephan, Jean-Louis; Suarez, Felipe; Tommasini, Alberto; Truck, Johannes; Uhlmann, Annette; van Aerde, Koen J.; van Montfrans, Joris; von Bernuth, Horst; Warnatz, Klaus; Williams, Tony; Worth, Austen J. J.; Ip, Winnie; Picard, Capucine; Catherinot, Emilie; Nademi, Zohreh; Grimbacher, Bodo; Satter, Lisa R. Forbes; Kracker, Sven; Chandra, Anita; Condliffe, Alison M.; Ehl, Stephan 分享 收藏 CVID-Associated B Cell Activating Factor Receptor Variants Change Receptor Oligomerization, Ligand Binding, and Signaling ResponsesBlock, Violeta; Sevdali, Eirini; Recher, Mike; Abolhassani, Hassan; Hammarstrom, Lennart; Smulski, Cristian R.; Baronio, Manuela; Plebani, Alessandro; Proietti, Michele; Speletas, Matthaios; Warnatz, Klaus; Voll, Reinhard E.; Lougaris, Vassilios; Schneider, Pascal; Eibel, Hermann 分享 收藏 Exploration of a panel of urine biomarkers of kidney disease in two paediatric cohorts with Type 1 diabetes mellitus of differing durationZeni, Letizia; Norden, Anthony G. W.; Prandi, Elena; Canepa, Carolina; Burling, Keith; Simpson, Katherine; Felappi, Barbara; Plebani, Alessandro; Cancarini, Giovanni; Ferraro, Pietro Manuel; Fraser, Donald; Unwin, Robert J. 分享 收藏 The Impact of SARS-CoV-2 Infection in Patients with Inborn Errors of Immunity: the Experience of the Italian Primary Immunodeficiencies Network (IPINet)Giardino, Giuliana; Milito, Cinzia; Lougaris, Vassilios; Punziano, Alessandra; Carrabba, Maria; Cinetto, Francesco; Scarpa, Riccardo; Dellepiane, Rosa Maria; Ricci, Silvia; Rivalta, Beatrice; Conti, Francesca; Marzollo, Antonio; Firinu, Davide; Cirillo, Emilia; Lagnese, Gianluca; Cancrini, Caterina; Martire, Baldassare; Danieli, Maria Giovanna; Pession, Andrea; Vacca, Angelo; Azzari, Chiara; Fabio, Giovanna; Soresina, Annarosa; Agostini, Carlo; Spadaro, Giuseppe; Badolato, Raffaele; Cicalese, Maria Pia; Aiuti, Alessandro; Plebani, Alessandro; Quinti, Isabella; Pignata, Claudio 分享 收藏 DOCK8 functions as an adaptor that links TLR-MyD88 signaling to B cell activation (vol 13, 612, 2012)Jabara, Haifa H.; McDonald, Douglas R.; Janssen, Erin; Massaad, Michel J.; Ramesh, Narayanaswamy; Borzutzky, Arturo; Rauter, Ingrid; Benson, Halli; Schneider, Lynda; Baxi, Sachin; Recher, Mike; Notarangelo, Luigi D.; Wakim, Rima; Dbaibo, Ghassan; Dasouki, Majed; Al-Herz, Waleed; Barlan, Isil; Baris, Safa; Kutukculer, Necil; Ochs, Hans D.; Plebani, Alessandro; Kanariou, Maria; Lefranc, Gerard; Reisli, Ismail; Fitzgerald, Katherine A.; Golenbock, Douglas; Manis, John; Keles, Sevgi; Ceja, Reuben; Chatila, Talal A.; Geha, Raif S. 分享 收藏 Progressive Depletion of B and T Lymphocytes in Patients with Ataxia Telangiectasia: Results of the Italian Primary Immunodeficiency Network共济失调性毛细血管扩张症患者的B和T淋巴细胞逐渐耗竭: 意大利原发性免疫缺陷网络的结果Cirillo, Emilia; Polizzi, Agata; Soresina, Annarosa; Prencipe, Rosaria; Giardino, Giuliana; Cancrini, Caterina; Finocchi, Andrea; Rivalta, Beatrice; Dellepiane, Rosa M.; Baselli, Lucia A.; Montin, Davide; Trizzino, Antonino; Consolini, Rita; Azzari, Chiara; Ricci, Silvia; Lodi, Lorenzo; Quinti, Isabella; Milito, Cinzia; Leonardi, Lucia; Duse, Marzia; Carrabba, Maria; Fabio, Giovanna; Bertolini, Patrizia; Coccia, Paola; D'Alba, Irene; Pession, Andrea; Conti, Francesca; Zecca, Marco; Lunardi, Claudio; Lo Bianco, Manuela; Presti, Santiago; Sciuto, Laura; Micheli, Roberto; Bruzzese, Dario; Lougaris, Vassilios; Badolato, Raffaele; Plebani, Alessandro; Chessa, Luciana; Pignata, Claudio 分享 收藏 Immunological Evaluation of Patients Affected with Jacobsen Syndrome Reveals Profound Not Age-Related Lymphocyte AlterationsBaronio, Manuela; Saettini, Francesco; Gazzurelli, Luisa; Rossi, Stefano; Marzollo, Antonio; Ricci, Silvia; Zama, Daniele; Palterer, Boaz; Clementina, Canessa; Lorenzo, Lodi; Chiarini, Marco; Sottini, Alessandra; Imberti, Luisa; Gorio, Chiara; Rossini, Linda; Badolato, Raffaele; Plebani, Alessandro; Moratto, Daniele; Lougaris, Vassilios 分享 收藏 Combined immunodeficiency with autoimmunity caused by a homozygous missense mutation in inhibitor of nuclear factor κB kinase alpha (IKKα)Bainter, Wayne; Lougaris, Vassilios; Wallace, Jacqueline G.; Badran, Yousef; Hoyos-Bachiloglu, Rodrigo; Peters, Zachary; Wilkie, Hazel; Das, Mrinmoy; Janssen, Erin; Beano, Abdallah; Ben Farhat, Khaoula; Kam, Christy; Bercich, Luisa; Incardona, Paolo; Villanacci, Vincenzo; Bondioni, Maria Pia; Meini, Antonella; Baronio, Manuela; Abarzua, Phammela; Parolini, Silvia; Tabellini, Giovanna; Maio, Stefano; Schmidt, Birgitta; Goldsmith, Jeffrey D.; Murphy, George; Hollander, Georg; Plebani, Alessandro; Chou, Janet; Geha, Raif S. 分享 收藏 Emergence of Letermovir-resistant HCMV UL56 mutant during rescue treatment in a liver transplant recipient with ganciclovir-resistant infection HCMV: a case reportPaolucci, Stefania; Campanini, Giulia; Cassaniti, Irene; Tebaldi, Alessandra; Novazzi, Federica; Fratini, Alice; Meini, Antonella; Girelli, Federica; Palumbo, Laura; Plebani, Alessandro; Baldanti, Fausto 分享 收藏 Simple Measurement of IgA Predicts Immunity and Mortality in Ataxia-TelangiectasiaIgA的简单测量可预测共济失调-毛细血管扩张症的免疫力和死亡率Zielen, Stefan; Duecker, Ruth Pia; Woelke, Sandra; Donath, Helena; Bakhtiar, Sharhzad; Buecker, Aileen; Kreyenberg, Hermann; Huenecke, Sabine; Bader, Peter; Mahlaoui, Nizar; Ehl, Stephan; El-Helou, Sabine M.; Pietrucha, Barbara; Plebani, Alessandro; van der Flier, Michiel; van Aerde, Koen; Kilic, Sara S.; Reda, Shereen M.; Kostyuchenko, Larysa; McDermott, Elizabeth; Galal, Nermeen; Pignata, Claudio; Perez, Juan Luis Santos; Laws, Hans-Juergen; Niehues, Tim; Kutukculer, Necil; Seidel, Markus G.; Marques, Laura; Ciznar, Peter; Edgar, John David M.; Soler-Palacin, Pere; von Bernuth, Horst; Krueger, Renate; Meyts, Isabelle; Baumann, Ulrich; Kanariou, Maria; Grimbacher, Bodo; Hauck, Fabian; Graf, Dagmar; Granado, Luis Ignacio Gonzalez; Prader, Seraina; Reisli, Ismail; Slatter, Mary; Rodriguez-Gallego, Carlos; Arkwright, Peter D.; Bethune, Claire; Deripapa, Elena; Sharapova, Svetlana O.; Lehmberg, Kai; Davies, E. Graham; Schuetz, Catharina; Kindle, Gerhard; Schubert, Ralf 分享 收藏 Inherited human c-Rel deficiency disrupts myeloid and lymphoid immunity to multiple infectious agentsLevy, Romain; Langlais, David; Beziat, Vivien; Rapaport, Franck; Rao, Geetha; Lazarov, Tomi; Bourgey, Mathieu; Zhou, Yu J.; Briand, Coralie; Moriya, Kunihiko; Ailal, Fatima; Avery, Danielle T.; Markle, Janet; Lim, Ai Ing; Ogishi, Masato; Yang, Rui; Pelham, Simon; Emam, Mehdi; Migaud, Melanie; Deswarte, Caroline; Habib, Tanwir; Saraiva, Luis R.; Moussa, Eman A.; Guennoun, Andrea; Boisson, Bertrand; Belkaya, Serkan; Martinez-Barricarte, Ruben; Rosain, Jeremie; Belkadi, Aziz; Breton, Sylvain; Payne, Kathryn; Benhsaien, Ibtihal; Plebani, Alessandro; Lougaris, Vassilios; Di Santo, James P.; Neven, Benedicte; Abel, Laurent; Ma, Cindy S.; Bousfiha, Ahmed Aziz; Marr, Nico; Bustamante, Jacinta; Liu, Kang; Gros, Philippe; Geissmann, Frederic; Tangye, Stuart G.; Casanova, Jean-Laurent; Puel, Anne 分享 收藏 Known and potential molecules associated with altered B cell development leading to predominantly antibody deficienciesAmirifar, Parisa; Yazdani, Reza; Azizi, Gholamreza; Ranjouri, Mohammad Reza; Durandy, Anne; Plebani, Alessandro; Lougaris, Vassilios; Hammarstrom, Lennart; Aghamohammadi, Asghar; Abolhassani, Hassan 分享 收藏 A CVID-associated variant in the ciliogenesis protein CCDC28B disrupts immune synapse assemblyCapitani, Nagaja; Onnis, Anna; Finetti, Francesca; Cassioli, Chiara; Plebani, Alessandro; Brunetti, Jlenia; Troilo, Arianna; D'Elios, Sofia; Baronio, Manuela; Gazzurelli, Luisa; Della Bella, Chiara; Billadeau, Daniel D.; D'Elios, Mario Milco; Lougaris, Vassilios; Baldari, Cosima T. 分享 收藏 Clinical outcome, incidence, and SARS-CoV-2 infection-fatality rates in Italian patients with inborn errors of immunityMilito, Cinzia; Lougaris, Vassilios; Giardino, Giuliana; Punziano, Alessandra; Vultaggio, Alessandra; Carrabba, Maria; Cinetto, Francesco; Scarpa, Riccardo; Delle Piane, Rosa Maria; Baselli, Lucia; Ricci, Silvia; Rivalta, Beatrice; Conti, Francesca; Marasco, Carolina; Marzollo, Antonio; Firinu, Davide; Pulvirenti, Federica; Lagnese, Gianluca; Vivarelli, Emanuele; Cancrin, Caterina; Martire, Baldassare; Danieli, Maria Giovanna; Pession, Andrea; Vacca, Angelo; Azzari, Chiara; Fabio, Giovanna; Matucci, Andrea; Soresina, Anna Rosa; Agostini, Carlo; Spadaro, Giuseppe; Badolato, Raffaele; Cicalese, Maria Pia; Aiuti, Alessandro; Pleban, Alessandro; Pignata, Claudio; Quinti, Isabella 分享 收藏 Granulomatous Lymphocytic Interstitial Lung Disease (GLILD) in Common Variable Immunodeficiency (CVID): A Multicenter Retrospective Study of Patients From Italian PID Referral CentersCinetto, Francesco; Scarpa, Riccardo; Carrabba, Maria; Firinu, Davide; Lougaris, Vassilios; Buso, Helena; Garzi, Giulia; Gianese, Sabrina; Soccodato, Valentina; Punziano, Alessandra; Lagnese, Gianluca; Tessarin, Giulio; Costanzo, Giulia; Landini, Nicholas; Vio, Stefania; Bondioni, Maria Pia; Consonni, Dario; Marasco, Carolina; Del Giacco, Stefano; Rattazzi, Marcello; Vacca, Angelo; Plebani, Alessandro; Fabio, Giovanna; Spadaro, Giuseppe; Agostini, Carlo; Quinti, Isabella; Milito, Cinzia 分享 收藏 Coronavirus disease 2019 in patients with inborn errors of immunity: An international study先天性免疫缺陷患者的冠状病毒疾病2019: 一项国际研究Meyts, Isabelle; Bucciol, Giorgia; Quinti, Isabella; Neven, Benedicte; Fischer, Alain; Seoane, Elena; Lopez-Grandos, Eduardo; Gianelli, Carla; Robles-Marhuenda, Angel; Jeandel, Pierre-Yves; Paillard, Catherine; Sankaran, Vijay G.; Demirdag, Yesim Yilmaz; Lougaris, Vassilios; Aiuti, Alessandro; Plebani, Alessandro; Milito, Cinzia; Dalm, Virgil A. S. H.; Guevara-Hoyer, Kissy; Sanchez-Ramon, Silvia; Bezrodnik, Liliana; Barzaghi, Federica; Ignacio Gonzalez-Granado, Luis; Hayman, Grant R.; Uzel, Gulbu; Mendonca, Leonardo Oliveira; Agostini, Carlo; Spadaro, Giuseppe; Badolato, Raffaele; Soresina, Annarosa; Vermeulen, Francois; Bosteels, Cedric; Lambrecht, Bart N.; Keller, Michael; Mustillo, Peter J.; Abraham, Roshini S.; Gupta, Sudhir; Ozen, Ahmet; Karakoc-Aydiner, Elif; Baris, Safa; Freeman, Alexandra F.; Yamazaki-Nakashimada, Marco; Scheffler-Mendoza, Selma; Espinosa-Padilla, Sara; Gennery, Andrew R.; Jolles, Stephen; Espinosa, Yazmin; Poli, M. Cecilia; Fieschi, Claire; Hauck, Fabian; Cunningham-Rundles, Charlotte; Mahlaoui, Nizar; Warnatz, Klaus; Sullivan, Kathleen E.; Tangye, Stuart G. 分享 收藏 Modeling, optimization, and comparable efficacy of T cell and hematopoietic stem cell gene editing for treating hyper-IgM syndromeVavassori, Valentina; Mercuri, Elisabetta; Marcovecchio, Genni E.; Castiello, Maria C.; Schiroli, Giulia; Albano, Luisa; Margulies, Carrie; Buquicchio, Frank; Fontana, Elena; Beretta, Stefano; Merelli, Ivan; Cappelleri, Andrea; Rancoita, Paola M., V; Lougaris, Vassilios; Plebani, Alessandro; Kanariou, Maria; Lankester, Arjan; Ferrua, Francesca; Scanziani, Eugenio; Cotta-Ramusino, Cecilia; Villa, Anna; Naldini, Luigi; Genovese, Pietro 分享 收藏 Consensus of the Italian Primary Immunodeficiency Network on transition management from pediatric to adult care in patients affected with childhood-onset inborn errors of immunity意大利初级免疫缺陷网络关于儿童期先天性免疫缺陷患者从儿科到成人护理过渡管理的共识Cirillo, Emilia; Giardino, Giuliana; Ricci, Silvia; Moschese, Viviana; Lougaris, Vassilios; Conti, Francesca; Azzari, Chiara; Barzaghi, Federica; Canessa, Clementina; Martire, Baldassarre; Badolato, Raffaele; Dotta, Laura; Soresina, Annarosa; Cancrini, Caterina; Finocchi, Andrea; Montin, Davide; Romano, Roberta; Amodio, Donato; Ferrua, Francesca; Tommasini, Alberto; Baselli, Lucia Augusta; Dellepiane, Rosa Maria; Polizzi, Agata; Chessa, Luciana; Marzollo, Antonio; Cicalese, Maria Pia; Putti, Maria Caterina; Pession, Andrea; Aiuti, Alessandro; Locatelli, Franco; Plebani, Alessandro; Pignata, Claudio 分享 收藏 
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