Results 31 to 40 of about 14,924 (223)

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

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

Secondary hypogammaglobulinemia in patients with multiple sclerosis on anti-CD20 therapy: Pathogenesis, risk of infection, and disease management

, 2023
•Hypogammaglobulinemia can occur with long-term anti-CD20 therapy in MS.•Mechanisms for anti-CD20 therapy-related hypogammaglobulinemia are poorly understood.•Infection risk may be increased in at-risk patients with low IgG.•Consensus guidelines for ...
James Stankiewicz, Kottil W. Rammohan, Rammohan, Kottil W., Alvarez, Enrique, Erin E. Longbrake, Stankiewicz, James, Carrie M. Hersh, Enrique Alvarez, Hersh, Carrie M., Longbrake, Erin E.   +9 more
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Tonsillar granuloma associated with hypogammaglobulinemia [PDF]

, 2020
Background Rare tonsillar granulomas may be caused for example by infections, malignancies or sarcoidosis. Granulomas also occur in inborn errors of immunity (IEI) such as common variable immunodeficiency (CVID) with B cell maturation defects and ...
Salo, Anna, Nissinen, Antti, Tastula, Mikko, Seppänen, Mikko R. J., Kuismin, Outi, Hautala, Timo, Åström, Pirjo, Laajala, Aleksi, Kauppila, Saila, Glumoff, Virpi, Tiitto, Leena   +10 more
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Hypogammaglobulinemia in Adolescents and Young Adults with Acute Lymphoblastic Leukemia

, 2020
Hypogammaglobulinemia is a poorly described complication of chemotherapy in adolescents and young adults (AYAs, 15–39 years) with acute lymphoblastic leukemia (ALL). The majority of AYAs treated on a Berlin–Frankfurt–Munster-based ALL regimen experienced
Nelson, Robert P., Althouse, Sandra K., Batra, Sandeep, Lange, Cassandra S., Rahrig, April   +4 more
core   +1 more source

Resistant Widespread Facial Impetigo in a Healthy Infant with Atopic Dermatitis [PDF]

Journal of Clinical and Diagnostic Research, 2018
Atopic Dermatitis (AD) or eczema is a chronic and relapsing, itchy skin inflammatory condition in infancy and childhood. The diagnosis is based on pruritus and an eczematous dermatitis with typical presentation.
Seyed Hesamedin Nabavizadeh, Ali Fazel, Soheila Alyasin, Mohammad Bahadoram, Gholamreza Houshmand   +4 more
doaj   +1 more source

Case Report: Kidney Transplantation in a Patient With Acquired Agammaglobulinemia and SLE. Issues and Challenges

Frontiers in Medicine, 2021
Lupus nephritis in the context of Systemic Lupus Erythematosus (SLE) is characterized by an unpredicted course with remissions and flare-ups. Among others, it remains a significant cause of end-stage kidney disease (ESKD) in relatively young patients ...
Paraskevi Pavlakou, Marios Papasotiriou, Theodoros Ntrinias, Alexandra Kourakli, Adamantia Bratsiakou, Dimitrios S. Goumenos, Evangelos Papachristou   +6 more
doaj   +1 more source

Vulnerability to Meningococcal Disease in Immunodeficiency Due to a Novel Pathogenic Missense Variant in NFKB1

Frontiers in Immunology, 2021
NF-κB1 deficiency is suggested to be the most common cause of common variable immunodeficiency (CVID). NFKB1 encodes for the p105 precursor protein of NF-κB1, which is converted into the active transcriptional subunit p50 through proteasomal processing ...
Manfred Anim, Manfred Anim, Georgios Sogkas, Georgios Sogkas, Gunnar Schmidt, Natalia Dubrowinskaja, Torsten Witte, Reinhold Ernst Schmidt, Reinhold Ernst Schmidt, Faranaz Atschekzei, Faranaz Atschekzei   +10 more
doaj   +1 more source

Evaluation of Hypogammaglobulinemia in Chronic Lymphocytic Leukemia Patients and Its Relation to Poor Prognostic Factors

, 2018
Aim:Chronic lymphocytic leukemia (CLL) is a heterogenous disease with variable clinical course. Rai staging system is used for at least 40 years to predict prognosis and need for treatment but more prognostic factors are needed.
Mesut Ayer, Deniz Özmen, Tayfun Elibol, Çağan M. Akay, Fatma Aylin Ayer   +4 more
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Oral manifestations in patients with hypogammaglobulinemia

, 2012
Objective. The overall objective of this study was to assess the oral manifestations and their association with immunologic status and health history, of individuals with hypogammaglobulinemia. Study Design.
Fernandes, Karin Sá, Gallottini, Marina Helena Cury, Kokron, Cristina Maria, Filho, Jorge Elias Kalil, Barros, Myrthes Anna Maragna Toledo   +4 more
core   +1 more source