Results 21 to 30 of about 19,540 (227)

The Natural History of Untreated Primary Hypogammaglobulinemia in Adults: Implications for the Diagnosis and Treatment of Common Variable Immunodeficiency Disorders (CVID)

Frontiers in Immunology, 2019
Background: Adults with primary hypogammaglobulinemia are frequently encountered by clinicians. Where IgG levels are markedly decreased, most patients are treated with subcutaneous or intravenous immunoglobulin (SCIG/IVIG), because of the presumed risk ...
Rohan Ameratunga, Rohan Ameratunga, Yeri Ahn, Richard Steele, See-Tarn Woon   +4 more
doaj   +1 more source

Impaired B-Cell Differentiation in a Patient With STAT1 Gain-of-Function Mutation

Frontiers in Immunology, 2020
Hypogammaglobulinemia is a rare complication of STAT1 gain-of-function (GOF) mutations. We report an adult patient diagnosed with hypogammaglobulinemia caused by B-cell depletion during the treatment of disseminated cryptococcosis.
Kazuki Nemoto, Toshinori Kawanami, Takayuki Hoshina, Masataka Ishimura, Kei Yamasaki, Satoshi Okada, Hirokazu Kanegane, Kazuhiro Yatera, Koichi Kusuhara   +8 more
doaj   +1 more source

Primary vs. Secondary Antibody Deficiency: Clinical Features and Infection Outcomes of Immunoglobulin Replacement [PDF]

, 2014
Secondary antibody deficiency can occur as a result of haematological malignancies or certain medications, but not much is known about the clinical and immunological features of this group of patients as a whole. Here we describe a cohort of 167 patients
AA Bousfiha, B Gathmann, B Gathmann, B Piqueras, B Seymour, C Casulo, C Wehr, DF Florescu, E Becerra, E Castigli, ES Resnick, FA Bonilla, G Gunther, H Chapel, H Chapel, H Griffiths, Hilary J. Longhurst, HM Chapel, I De La Torre, I Quinti, J Blore, J Shortt, JA Freeman, JD Edgar, John Dempster, JS Orange, JS Orange, K Warnatz, K Warnatz, L Mouthon, Lorena Lorenzo, M Lucas, M Makatsori, M Yomota, Matthaios Speletas, Matthew Buckland, ME Conley, ME Rose, MJ Boumans, N Ozaras, N Venhoff, NH Yip, P Bright, P McLaughlin, P Wood, R Herriot, S Baris, S Bernatsky, S Molica, Sai S. Duraisingham, Sofia Grigoriadou, SS Duraisingham, T Kawano, U Salzer, W Al-Herz, W Strober   +55 more
core   +3 more sources

Common variable immunodeficiency and idiopathic primary hypogammaglobulinemia: two different conditions within the same disease spectrum

Haematologica, 2013
Patients with hypogammaglobulinemia who do not fulfill all the classical diagnostic criteria for common variable immunodeficiency (reduction of two immunoglobulin isotypes and a reduced response to vaccination) constitute a diagnostic and therapeutic ...
Gertjan J. Driessen, Virgil A.S.H. Dalm, P. Martin van Hagen, H. Anne Grashoff, Nico G. Hartwig, Annemarie M. C. van Rossum, Adilia Warris, Esther de Vries, Barbara H. Barendregt, Ingrid Pico, Sandra Posthumus, Menno C. van Zelm, Jacques J.M. van Dongen, Mirjam van der Burg   +13 more
doaj   +1 more source

Mulibrey nanism and immunological complications: a comprehensive case report and literature review

Frontiers in Immunology, 2023
IntroductionMulibrey nanism (MUL) is a rare disorder caused by TRIM37 gene variants characterized by growth failure, dysmorphic features, congestive heart failure (CHF), and an increased risk of Wilms’ tumor.
Andrea Gazzin, Andrea Gazzin, Francesca Pala, Marita Bosticardo, Julie Niemela, Jennifer Stoddard, Eleonora Biasin, Paola Quarello, Diana Carli, Diana Carli, Francesca Ferroni, Ottavia M. Delmonte, Davide Montin, Sergio D. Rosenzweig, Francesco Licciardi, Luigi D. Notarangelo   +15 more
doaj   +1 more source

A computational pipeline for the diagnosis of CVID patients [PDF]

, 2019
Common variable immunodeficiency (CVID) is one of the most frequently diagnosed primary antibody deficiencies (PADs), a group of disorders characterized by a decrease in one or more immunoglobulin (sub) classes and/or impaired antibody responses caused ...
Bogaert, Delfien, Dullaers, Melissa, Emmaneel, Annelies, Haerynck, Filomeen, Saeys, Yvan, Tavernier, Simon, Van Gassen, Sofie   +6 more
core   +2 more sources

In-depth blood immune profiling of Good syndrome patients

Frontiers in Immunology, 2023
IntroductionGood syndrome (GS) is a rare adult-onset immunodeficiency first described in 1954. It is characterized by the coexistence of a thymoma and hypogammaglobulinemia, associated with an increased susceptibility to infections and autoimmunity.
Alba Torres-Valle, Alba Torres-Valle, Alba Torres-Valle, Larraitz Aragon, Susana L. Silva, Susana L. Silva, Cristina Serrano, Miguel Marcos, Miguel Marcos, Miguel Marcos, Josefa Melero, Carolien Bonroy, Carolien Bonroy, Pedro Pablo Arenas-Caro, David Monzon Casado, Pedro Mikel Requejo Olaizola, Jana Neirinck, Jana Neirinck, Mattias Hofmans, Mattias Hofmans, Sonia de Arriba, María Jara, María Jara, María Jara, Carlos Prieto, Ana E. Sousa, Álvaro Prada, Jacques J. M. van Dongen, Jacques J. M. van Dongen, Jacques J. M. van Dongen, Martín Pérez-Andrés, Martín Pérez-Andrés, Martín Pérez-Andrés, Martín Pérez-Andrés, Alberto Orfao, Alberto Orfao, Alberto Orfao, Alberto Orfao   +37 more
doaj   +1 more source

IgA and IgG hypogammaglobulinemia in Waldenström’s macroglobulinemia

Haematologica, 2010
Background Hypogammaglobulinemia is common in Waldenström’s macroglobulinemia. The etiology of this finding remains unclear, but it has been speculated to be based on tumor-induced suppression of the ‘uninvolved’ immunoglobulin productionDesign and ...
Zachary R. Hunter, Robert J. Manning, Christine Hanzis, Bryan T. Ciccarelli, Leukothea Ioakimidis, Christopher J. Patterson, Megan C. Lewicki, Hsuiyi Tseng, Ping Gong, Xia Liu, Yangsheng Zhou, Guang Yang, Jenny Sun, Lian Xu, Patricia Sheehy, Massimo Morra, Steven P. Treon   +16 more
doaj   +1 more source

Subcutaneous Immunoglobulin Replacement Therapy with Hizentra® is Safe and Effective in Children Less Than 5 Years of Age. [PDF]

, 2015
BACKGROUND:Hizentra® (IGSC 20%) is a 20% liquid IgG product approved for subcutaneous administration in adults and children 2 years of age and older who have primary immunodeficiency disease (PIDD). There is limited information about the use of IGSC 20 %
Atkinson, Thomas Prescott, Bonilla, Francisco A, Dorsey, Morna, Gallagher, Joel L, Haddad, Elie, Heimall, Jennifer, Kobrynski, Lisa, Morris, David, Ochs, Hans D, Patel, Niraj C, Wahlstrom, Justin   +10 more
core   +2 more sources

Human Inborn Errors of Immunity: 2019 Update of the IUIS Phenotypical Classification. [PDF]

, 2020
Since 2013, the International Union of Immunological Societies (IUIS) expert committee (EC) on Inborn Errors of Immunity (IEI) has published an updated phenotypic classification of IEI, which accompanies and complements their genotypic classification ...
Ailal, Fatima, Al-Herz, Waleed, Bousfiha, Aziz, Casanova, Jean-Laurent, Chatila, Talal, Cunningham-Rundles, Charlotte, Etzioni, Amos, Franco, Jose Luis, Holland, Steven M, Jeddane, Leila, Klein, Christoph, Morio, Tomohiro, Ochs, Hans D, Oksenhendler, Eric, Picard, Capucine, Puck, Jennifer, Sullivan, Kathleen E, Tangye, Stuart G, Torgerson, Troy R   +18 more
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