Issue 2/2021
Content (25 Articles)
In Memoriam: Asghar Aghamohammadi (May 30, 1951–November 14, 2020)
Nima Rezaei
Immune Dysfunction in Mendelian Disorders of POLA1 Deficiency
Petro Starokadomskyy, Andrea Escala Perez-Reyes, Ezra Burstein
When Screening for Severe Combined Immunodeficiency (SCID) with T Cell Receptor Excision Circles Is Not SCID: a Case-Based Review
David Buchbinder, Jolan E. Walter, Manish J. Butte, Wan-Yin Chan, Maria Chitty Lopez, Victoria R. Dimitriades, Morna J. Dorsey, Diane J. Nugent, Jennifer M. Puck, Jasjit Singh, Cathleen A. Collins
Tocilizumab for Severe Worsening COVID-19 Pneumonia: a Propensity Score Analysis
Mathilde Roumier, Romain Paule, Alexandre Vallée, Julien Rohmer, Marie Ballester, Anne-Laure Brun, Charles Cerf, Marie-Laure Chabi, Thierry Chinet, Marie-Alice Colombier, Eric Farfour, Erwan Fourn, Guillaume Géri, David Khau, Ibrahim Marroun, Matthieu Ponsoye, Antoine Roux, Hélène Salvator, Yoland Schoindre, Anne-Gaëlle Si Larbi, Colas Tchérakian, Marc Vasse, Anne Verrat, Benjamin Zuber, Louis-Jean Couderc, Jean-Emmanuel Kahn, Matthieu Groh, Félix Ackermann
The Impact of Immunosuppression and Autoimmune Disease on Severe Outcomes in Patients Hospitalized with COVID-19
Enric Monreal, Susana Sainz de la Maza, Jose Ignacio Fernández-Velasco, Elena Natera-Villalba, Claudia Geraldine Rita, Fernando Rodríguez-Jorge, Álvaro Beltrán-Corbellini, Ignacio Iturrieta-Zuazo, Enrique Rodríguez de Santiago, Mercedes Espiño, Ana de Andrés, Jesús Fortún, Esther Barbero, Mónica Vázquez, Milagros Fernández Lucas, Luis Manzano, Beatriz Montero-Errasquín, Lucienne Costa-Frossard, Jaime Masjuan, Luisa María Villar
A Case–Control Study of the 2019 Influenza Vaccine and Incidence of COVID-19 Among Healthcare Workers
Nilofar Massoudi, Babak Mohit
N-Glycan Modification in Covid-19 Pathophysiology: In vitro Structural Changes with Limited Functional Effects
Cristiane J. Nunes-Santos, Hye Sun Kuehn, Sergio D. Rosenzweig
Impact of SARS-CoV-2 Pandemic on Patients with Primary Immunodeficiency
Samaneh Delavari, Hassan Abolhassani, Farhad Abolnezhadian, Fateme Babaha, Sara Iranparast, Hamid Ahanchian, Nasrin Moazzen, Mohammad Nabavi, Saba Arshi, Morteza Fallahpour, Mohammad Hassan Bemanian, Sima Shokri, Tooba Momen, Mahnaz Sadeghi-Shabestari, Rasol Molatefi, Afshin Shirkani, Ahmad Vosughimotlagh, Molood Safarirad, Meisam Sharifzadeh, Salar Pashangzadeh, Fereshte Salami, Paniz Shirmast, Arezou Rezaei, Tannaz Moeini Shad, Minoo Mohraz, Nima Rezaei, Lennart Hammarström, Reza Yazdani, Asghar Aghamohamamdi
Severe COVID-19 in Patients with B Cell Alymphocytosis and Response to Convalescent Plasma Therapy
Jonathan London, David Boutboul, Karine Lacombe, France Pirenne, Beate Heym, Valérie Zeller, Antoine Baudet, Amani Ouedrani, Alice Bérezné
Targeted Proteomics Reveals Inflammatory Pathways that Classify Immune Dysregulation in Common Variable Immunodeficiency
Roos-Marijn Berbers, Julia Drylewicz, Pauline M. Ellerbroek, Joris M. van Montfrans, Virgil A. S. H. Dalm, P. Martin van Hagen, Baerbel Keller, Klaus Warnatz, Annick van de Ven, Jaap M. van Laar, Stefan Nierkens, Helen L. Leavis
Infection Phenotypes Among Patients with Primary Antibody Deficiency Mined from a US Patient Registry
Grant Pickett, Tina Motazedi, Carleigh Kutac, Gina Cahill, Charlotte Cunnigham-Rundles, Ramsay L. Fuleihan, Kathleen E. Sullivan, Nicholas L. Rider
Immunoglobulin Replacement Therapy Versus Antibiotic Prophylaxis as Treatment for Incomplete Primary Antibody Deficiency
Bas M. Smits, Ilona Kleine Budde, Esther de Vries, Ineke J. M. ten Berge, Robbert G. M. Bredius, Marcel van Deuren, Jaap T. van Dissel, Pauline M. Ellerbroek, Michiel van der Flier, P. Martin van Hagen, Chris Nieuwhof, Bram Rutgers, Lieke E. A. M. Sanders, Anna Simon, Taco W. Kuijpers, Joris M. van Montfrans
Primary Immunodeficiencies in India: Molecular Diagnosis and the Role of Next-Generation Sequencing
Arun Kumar Arunachalam, Madhavi Maddali, Fouzia N. Aboobacker, Anu Korula, Biju George, Vikram Mathews, Eunice Sindhuvi Edison
Reduced Intensity Bone Marrow Transplantation with Post-Transplant Cyclophosphamide for Pediatric Inherited Immune Deficiencies and Bone Marrow Failure Syndromes
Orly R. Klein, Samantha Bapty, Howard M. Lederman, M. Elizabeth M. Younger, Elias T. Zambidis, Richard J. Jones, Kenneth R. Cooke, Heather J. Symons
Primary Cutaneous Aspergillosis in a Patient with CARD9 Deficiency and Aspergillus Susceptibility of Card9 Knockout Mice
Yi Zhang, Chen Huang, Yinggai Song, Yubo Ma, Zhe Wan, Xuejun Zhu, Xiaowen Wang, Ruoyu Li
Identification of Critical Transcriptomic Signaling Pathways in Patients with H Syndrome and Rosai-Dorfman Disease
Samuel Lara-Reyna, James A. Poulter, Elton J.R. Vasconcelos, Mark Kacar, Michael F. McDermott, Reuben Tooze, Rainer Doffinger, Sinisa Savic
Safety and Tolerability of Subcutaneous IgPro20 at High Infusion Parameters in Patients with Primary Immunodeficiency: Findings from the Pump-Assisted Administration Cohorts of the HILO Study
John T. Anderson, Vincent R. Bonagura, Juthaporn Cowan, Connie Hsu, S. Shahzad Mustafa, Niraj C. Patel, John M. Routes, Panida Sriaroon, Donald C. Vinh, Jutta H. Hofmann, Michaela Praus, Mikhail A. Rojavin
Novel Compound Heterozygous Mutations in ZAP70 Leading to a SCID Phenotype with Normal Downstream In vitro Signaling
Kelsey Kaman, Monique Abrams, Kerry Dobbs, Luigi D. Notarangelo, Ottavia M. Delmonte, Boaz Palterer, Sung-Yun Pai, Alicia Johnston
A Novel RAC2 Variant Presenting as Severe Combined Immunodeficiency
Heather Stern, Agnes Donkó, Teresa Shapiro, Amy P. Hsu, Thomas L. Leto, Steven M. Holland, Doerthe Adriana Andreae
Eculizumab-Responsive Adult Onset Protein Losing Enteropathy, Caused by Germline CD55-Deficiency and Complicated by Aggressive Angiosarcoma
David Hagin, Dror Lahav, Tal Freund, Sivan Shamai, Eli Brazowski, Sigal Fishman, Alina Kurolap, Hagit Baris Feldman, Mordechai Shohat, Ophira Salomon
Complete Absence of CD3γ Protein Expression Is Responsible for Combined Immunodeficiency with Autoimmunity Rather than SCID
Ottavia M. Delmonte, Jared H. Rowe, Adam K. Dobbs, Boaz Palterer, Riccardo Castagnoli, Luigi D. Notarangelo
Infection Due to Serratia sp. in Chronic Granulomatous Disease—Is the Incidence Low in Tropical Countries?
Dharmagat Bhattarai, Aman Gupta, Pandiarajan Vignesh, Hitender Rao, Archana Angrup, Amit Rawat
BCG-osis and Hematopoietic Cell Transplant for Primary Immunodeficiencies
Anthony Sabulski, Stella M. Davies, Grant Paulsen, Rebecca Marsh, Sharat Chandra
Functional Asplenia and Specific Polysaccharide Antibody Deficiency in a Girl with SAVI
Else M. Bijker, Berenice Rösler, Esther Hoppenreijs, Stefanie Henriet, Michiel van der Flier
Correction to: Infections in Infants with SCID: Isolation, Infection Screening and Prophylaxis in PIDTC Centers
Morna Dorsey, Nicola A. M. Wright, Natalia S. Chaimowitz, Blachy J. Dávila Saldaña, Holly Miller, Michael D. Keller, Monica S. Thakar, Ami J. Shah, Rolla Abu-Arja, Jeffrey Andolina, Victor Aquino, J. L. Barnum, Jeffrey J. Bednarski, Monica Bhatia, Francisco A. Bonilla, Manish J. Butte, Nancy J. Bunin, Lauri M. Burroughs, Sharat Chandra, Sonali Chaudhury, Karin Chen, Hey Chong, Geoff Cuvelier, Jignesh Dalal, Magee L. DeFelice, Kenneth B. DeSantes, Lisa R. Forbes, Alfred Gillio, Fred Goldman, Avni Y. Joshi, Neena Kapoor, Alan P. Knutsen, Lisa Kobrynski, Jay A. Lieberman, Jennifer W. Leiding, Benjamin Oshrine, Kiran P. Patel, Susan Prockop, Troy C. Quigg, Ralph Quinones, Kirk R. Schultz, Christine Seroogy, David Shyr, Subhadra Siegel, Angela R. Smith, Troy R. Torgerson, Mark T. Vander Lugt, Lolie C. Yu, Morton J. Cowan, Rebecca H. Buckley, Christopher C. Dvorak, Linda M. Griffith, Elie Haddad, Donald B. Kohn, Brent Logan, Luigi D. Notarangelo, Sung-Yun Pai, Jennifer Puck, Michael A. Pulsipher, Jennifer Heimall