Results 1 to 10 of about 15,405 (142)

Inborn Errors of Immunity and Cancer [PDF]

Biology, 2021
Inborn Errors of Immunity (IEI) are a heterogeneous group of disorders characterized by a defect in the function of at least one, and often more, components of the immune system.
Alessandra Tiri, Riccardo Masetti, Francesca Conti, Anna Tignanelli, Elena Turrini, Patrizia Bertolini, Susanna Esposito, Andrea Pession   +7 more
doaj   +3 more sources

Granulomatous inflammation in inborn errors of immunity [PDF]

Frontiers in Pediatrics, 2023
Granulomas have been defined as inflammatory infiltrates formed by recruitment of macrophages and T cells. The three-dimensional spherical structure typically consists of a central core of tissue resident macrophages which may merge into multinucleated ...
Keith A. Sacco, Andrea Gazzin, Luigi D. Notarangelo, Ottavia M. Delmonte   +3 more
doaj   +3 more sources

Autoimmunity and inborn errors of immunity: a complex coexistence [PDF]

Frontiers in Immunology
Inborn errors of immunity (IEI) and autoimmune diseases represent two major consequences of immune system dysregulation. Although recurrent infections are a hallmark of IEI, autoimmune manifestations are also highly prevalent in affected patients ...
Ahamada Elamine, Ibtihal Benhsaien, Ibtihal Benhsaien, Fatima Ailal, Fatima Ailal, Mohammed Hbibi, Mohammed Fahi, Mohammed Fahi, Ahmed Aziz Bousfiha, Ahmed Aziz Bousfiha, Jalila EL Bakkouri, Jalila EL Bakkouri, Jalila EL Bakkouri   +12 more
doaj   +2 more sources

Thrombocytopenia in patients with inborn errors of immunity [PDF]

BMC Immunology
Background Inborn errors of immunity (IEI) are inherited defects of innate or adaptive immune system. Thrombocytopenia is a significant multifactorial complication in IEI patients leading to severe clinical consequences including coagulative disorders ...
Saba Fekrvand, Maryam Mohtashami, Negin Sanadgol, Helia Salehi, Najmeh Nameh Goshay Fard, Ehsan Khoshnezhad Afkham, Zahra Chavoshzadeh, Nima Parvaneh, Seyed Alireza Mahdaviani, Samin Sharafian, Sahar Barzamini, Hamid Ahanchian, Arash Kalantari, Alireza Shafiei, Marzieh Tavakol, Farhad Abolnezhadian, Mina Kianmanesh Rad, Gholamreza Hassanpour, Taher Cheraghi, Amir Salehi Farid, Samaneh Delavari, Hassan Abolhassani, Nima Rezaei, Reza Yazdani   +23 more
doaj   +2 more sources

Lymphoproliferation in Inborn Errors of Immunity: Mechanisms, Manifestations and Clinical Management, with a Focus on ALPID [PDF]

ImmunoTargets and Therapy
Giulia Gualtiero,1– 3,* Helena Buso,1,4,* Eva C Coopmans,2,3 Cinzia Milito,5,6 Davide Firinu,7 Virgil ASH Dalm,2,3 Francesco Cinetto1,4 1Department of Medicine (DIMED), University of Padua, Padua, Italy; 2Department of Internal Medicine ...
Gualtiero G, Buso H, Coopmans EC, Milito C, Firinu D, Dalm VA, Cinetto F   +6 more
doaj   +2 more sources

Intronic branchpoint-to-acceptor variants underlying inborn errors of immunityIntronic branchpoint-to-acceptor variants [PDF]

Journal of Human Immunity
We describe seven intronic pathogenic variants underlying inborn errors of immunity.
Najiba Alioua, Nathalie Lambert, Mathilde Puel, Sylvain Hanein, Paul Bastard, Mathieu Fusaro, Marie Jaffray, Bernardita Medel, Lydia Khellaf, Yoann Seeleuthner, Mélodie Perin, Corinne Jacques, Marlène Pasquet, Laura Olivier, Fernando Sepulveda, Tom Le Voyer, Aurélie Cobat, Patrick Nitschké, Lionel Galicier, Nicolas Schleinitz, Eric Oksenhendler, Marion Malphettes, Bénédicte Neven, Despina Moshous, Felipe Suarez, Claire Fieschi, Jean-Laurent Casanova, Geneviève de Saint Basile, Guillaume Dorval, Capucine Picard, Jacinta Bustamante, Peng Zhang, Jérémie Rosain   +32 more
doaj   +2 more sources

An ACE2 Triple Decoy that neutralizes SARS-CoV-2 shows enhanced affinity for virus variants

Scientific Reports, 2021
The SARS-CoV-2 variants replacing the first wave strain pose an increased threat by their potential ability to escape pre-existing humoral protection. An angiotensin converting enzyme 2 (ACE2) decoy that competes with endogenous ACE2 for binding of the ...
Shiho Tanaka, Gard Nelson, C. Anders Olson, Oleksandr Buzko, Wendy Higashide, Annie Shin, Marcos Gonzalez, Justin Taft, Roosheel Patel, Sofija Buta, Ashley Richardson, Dusan Bogunovic, Patricia Spilman, Kayvan Niazi, Shahrooz Rabizadeh, Patrick Soon-Shiong   +15 more
doaj   +1 more source

Endocrinopathies in Inborn Errors of Immunity

Frontiers in Immunology, 2021
Inborn errors of immunity (IEI), caused by hereditary or genetic defects, are a group of more than 400 disorders, in which the immune system, including lymphocytes, neutrophils, macrophages, and complements, does not function properly.
Kei Takasawa, Hirokazu Kanegane, Kenichi Kashimada, Tomohiro Morio   +3 more
doaj   +1 more source

Failure of immunological competence: when to suspect?

Jornal de Pediatria, 2021
Objectives: To draw physicians’ attention to the different warning signs of diseases of inborn errors of immunity. Data sources: A non-systematic review of the literature was carried out in the PubMed, LILACS, and SciELO databases, in addition to ...
Fernanda Pinto-Mariz
doaj   +1 more source

Nature and nurture: understanding phenotypic variation in inborn errors of immunity

Frontiers in Cellular and Infection Microbiology, 2023
The overall disease burden of pediatric infection is high, with widely varying clinical outcomes including death. Among the most vulnerable children, those with inborn errors of immunity, reduced penetrance and variable expressivity are common but poorly
Morgan Similuk, Taco Kuijpers
doaj   +1 more source