keyword
https://read.qxmd.com/read/38474381/gene-signature-of-regulatory-t-cells-isolated-from-children-with-selective-iga-deficiency-and-common-variable-immunodeficiency
#1
JOURNAL ARTICLE
Magdalena Rutkowska-Zapała, Agnieszka Grabowska-Gurgul, Marzena Lenart, Anna Szaflarska, Anna Kluczewska, Monika Mach-Tomalska, Monika Baj-Krzyworzeka, Maciej Siedlar
Selective IgA deficiency (SIgAD) is the most common form and common variable immunodeficiency (CVID) is the most symptomatic form of predominant antibody deficiency. Despite differences in the clinical picture, a similar genetic background is suggested. A common feature of both disorders is the occurrence of autoimmune conditions. Regulatory T cells (Tregs ) are the major immune cell type that maintains autoimmune tolerance. As the different types of abnormalities of Treg cells have been associated with autoimmune disorders in primary immunodeficiency (PID) patients, in our study we aimed to analyze the gene expression profiles of Treg cells in CVID and SIgAD patients compared to age-matched healthy controls...
February 27, 2024: Cells
https://read.qxmd.com/read/38427721/mast-cells-help-organize-the-peyer-s-patch-niche-for-induction-of-iga-responses
#2
JOURNAL ARTICLE
Marco De Giovanni, Vivasvan S Vykunta, Adi Biram, Kevin Y Chen, Hanna Taglinao, Jinping An, Dean Sheppard, Helena Paidassi, Jason G Cyster
Peyer's patches (PPs) are lymphoid structures situated adjacent to the intestinal epithelium that support B cell responses that give rise to many intestinal IgA-secreting cells. Induction of isotype switching to IgA in PPs requires interactions between B cells and TGFβ-activating conventional dendritic cells type 2 (cDC2s) in the subepithelial dome (SED). However, the mechanisms promoting cDC2 positioning in the SED are unclear. Here, we found that PP cDC2s express GPR35, a receptor that promotes cell migration in response to various metabolites, including 5-hydroxyindoleacetic acid (5-HIAA)...
March 2024: Science Immunology
https://read.qxmd.com/read/38427309/examining-the-association-between-serum-galactose-deficient-iga1-and-primary-iga-nephropathy-a-systematic-review-and-meta-analysis
#3
REVIEW
Pedro Alves Soares Vaz de Castro, Arthur Aguiar Amaral, Mariana Godinho Almeida, Haresh Selvaskandan, Jonathan Barratt, Ana Cristina Simões E Silva
BACKGROUND: IgA nephropathy (IgAN) is a common primary glomerular disease. The O-glycosylation status of IgA1 plays a crucial role in disease pathophysiology. The level of poorly-O-galactosylated IgA1, or galactose-deficient IgA1 (Gd-IgA1), has also been identified as a potential biomarker in IgAN. We sought to examine the value of serum Gd-IgA1 as a biomarker in IgAN, by investigating its association with clinical, laboratory, and histopathological features of IgAN. METHODS: The review followed Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) recommendations and was registered in PROSPERO (CRD42021287423)...
March 1, 2024: Journal of Nephrology
https://read.qxmd.com/read/38306460/clinical-and-experimental-treatment-of-primary-humoral-immunodeficiencies
#4
JOURNAL ARTICLE
Anna Szaflarska, Marzena Lenart, Magdalena Rutkowska-Zapała, Maciej Siedlar
Selective IgA deficiency (sIgAD) and Common Variable Immunodeficiency (CVID) and Transient Hypogammaglobulinemia of Infancy (THI) are the most frequent forms of primary antibody deficiencies. Difficulties in initial diagnosis, especially in the early childhood, the familiar occurrence of these diseases, as well as the possibility of progression to each other suggest common cellular and molecular patomechanism and a similar genetic background. In this review, we discuss both similarities and differences of these three humoral immunodeficiencies, focusing on current and novel therapeutic approaches...
February 2, 2024: Clinical and Experimental Immunology
https://read.qxmd.com/read/38291108/new-orphan-disease-therapies-from-the-proteome-of-industrial-plasma-processing-waste-a-treatment-for-aceruloplasminemia
#5
JOURNAL ARTICLE
Alan Zanardi, Ilaria Nardini, Sara Raia, Antonio Conti, Barbara Ferrini, Patrizia D'Adamo, Enrica Gilberti, Giuseppe DePalma, Sara Belloli, Cristina Monterisi, Angela Coliva, Paolo Rainone, Rosa Maria Moresco, Filippo Mori, Giada Zurlo, Carla Scali, Letizia Natali, Annalisa Pancanti, Pierangelo Giovacchini, Giulio Magherini, Greta Tovani, Laura Salvini, Vittoria Cicaloni, Cristina Tinti, Laura Tinti, Daniele Lana, Giada Magni, Maria Grazia Giovannini, Alessandro Gringeri, Andrea Caricasole, Massimo Alessio
Plasma-derived therapeutic proteins are produced through an industrial fractionation process where proteins are purified from individual intermediates, some of which remain unused and are discarded. Relatively few plasma-derived proteins are exploited clinically, with most of available plasma being directed towards the manufacture of immunoglobulin and albumin. Although the plasma proteome provides opportunities to develop novel protein replacement therapies, particularly for rare diseases, the high cost of plasma together with small patient populations impact negatively on the development of plasma-derived orphan drugs...
January 30, 2024: Communications Biology
https://read.qxmd.com/read/38271880/influence-of-pre-b-cell-receptor-deficiency-on-the-immunoglobulin-repertoires-in-peripheral-blood-b-cells-before-and-after-immunization
#6
JOURNAL ARTICLE
Takeyuki Shimizu, Lin Sun, Kazuo Ohnishi
During B cell development, pre-B cell receptor (pre-BCR), comprising the immunoglobulin heavy chain (HC) and surrogate light chain (SLC), plays a crucial role. The expression of pre-BCR serves as a certification of HC quality, confirming its ability to associate with the SLC and light chain (LC). In mice lacking SLC, the absence of this quality control mechanism leads to a distorted repertoire of HCs in the spleen and bone marrow. In this study, we conducted a comparative analysis of the immunoglobulin gene repertoire in peripheral blood cells of both wild-type mice and pre-BCR-deficient mice...
January 24, 2024: Molecular Immunology
https://read.qxmd.com/read/38231436/clinical-and-treatment-history-of-patients-with-partial-digeorge-syndrome-and-autoimmune-cytopenia-at-multiple-centers
#7
JOURNAL ARTICLE
Priya K Patel, Michell Lozano Chinga, Melis Yilmaz, Sonia Joychan, Boglarka Ujhazi, Maryssa Ellison, Sumai Gordon, Daime Nieves, Krisztian Csomos, Don Eslin, Zeinab A Afify, Jessica Meznarich, John Bohnsack, Kelly Walkovich, Markus G Seidel, Svetlana Sharapova, Oksana Boyarchyk, Elena Latysheva, Irina Tuzankina, Ahmad B Shaker, Irmel Ayala, Panida Sriaroon, Emma Westermann-Clark, Jolan E Walter
BACKGROUND: Patients with partial DiGeorge syndrome (pDGS) can present with immune dysregulation, the most common being autoimmune cytopenia (AIC). There is a lack of consensus on the approach to type, combination, and timing of therapies for AIC in pDGS. Recognition of immune dysregulation early in pDGS clinical course may help individualize treatment and prevent adverse outcomes from chronic immune dysregulation. OBJECTIVES: Objectives of this study were to characterize the natural history, immune phenotype, and biomarkers in pDGS with AIC...
January 17, 2024: Journal of Clinical Immunology
https://read.qxmd.com/read/38138434/uhplc-ms-based-metabolomics-reveal-the-potential-mechanism-of-armillaria-mellea-acid-polysaccharide-in-and-its-effects-on-cyclophosphamide-induced-immunosuppressed-mice
#8
JOURNAL ARTICLE
Ying Li, Qingqing Li, Huazhou Niu, Hui Li, Lili Jiao, Wei Wu
Armillaria mellea (Vahl) P. Kumm is commonly used for food and pharmaceutical supplements due to its immune regulatory function, and polysaccharides are one of its main components. The aim of this research is to study the immunological activity of the purified acidic polysaccharide fraction, namely, AMPA, isolated from Armillaria mellea crude polysaccharide (AMP). In this study, a combination of the immune activity of mouse macrophages in vitro and serum metabonomics in vivo was used to comprehensively explore the cell viability and metabolic changes in immune-deficient mice in the AMPA intervention, with the aim of elucidating the potential mechanisms of AMPA in the treatment of immunodeficiency...
December 5, 2023: Molecules: a Journal of Synthetic Chemistry and Natural Product Chemistry
https://read.qxmd.com/read/38106572/evaluating-progression-risk-in-patients-with-immunoglobulin-a-nephropathy
#9
REVIEW
Daniel C Cattran, Jürgen Floege, Rosanna Coppo
The highly variable rate of decline in kidney function in patients with immunoglobulin A nephropathy (IgAN) provides a major clinical challenge. Predicting which patients will progress to kidney failure, and how quickly, is difficult. Multiple novel therapies are likely to be approved in the short-term, but clinicians lack the tools to identify patients most likely to benefit from specific treatments at the right time. Noninvasive and validated markers for selecting at-risk patients and longitudinal monitoring are urgently needed...
December 2023: KI Reports
https://read.qxmd.com/read/38068276/reply-to-%C3%A3-zdemir-%C3%A3-allergic-disease-with-selective-iga-deficiency-comment-on-cinicola-et-al-the-allergic-phenotype-of-children-and-adolescents-with-selective-iga-deficiency-a-longitudinal-monocentric-study-j-clin-med-2022-11-5705
#10
JOURNAL ARTICLE
Bianca Laura Cinicola, Giulia Brindisi, Martina Capponi, Alessandra Gori, Lorenzo Loffredo, Giovanna De Castro, Caterina Anania, Alberto Spalice, Cristiana Alessia Guido, Cinzia Milito, Marzia Duse, Isabella Quinti, Federica Pulvirenti, Anna Maria Zicari
We carefully read the correspondence [...].
November 22, 2023: Journal of Clinical Medicine
https://read.qxmd.com/read/38005969/covid-19-vaccination-coverage-and-factors-influencing-vaccine-hesitancy-among-patients-with-inborn-errors-of-immunity-in-latvia-a-mixed-methods-study
#11
JOURNAL ARTICLE
Zane Lucane, Mirdza Kursite, Kristaps Sablinskis, Linda Gailite, Natalja Kurjane
BACKGROUND: The European Society for Immunodeficiencies recommends that all patients with inborn errors of immunity (IEI) without contraindications should receive SARS-CoV-2 vaccination. The aim of this study was to investigate the reasons that discourage IEI patients from receiving the recommended vaccination and to assess vaccination coverage among IEI patients in Latvia. METHODS: In this multicenter mixed-methods study, the vaccination status of all patients with IEI within two tertiary centers in Latvia was reviewed using electronic health records...
October 25, 2023: Vaccines
https://read.qxmd.com/read/37959169/allergic-disease-with-selective-iga-deficiency-comment-on-cinicola-et-al-the-allergic-phenotype-of-children-and-adolescents-with-selective-iga-deficiency-a-longitudinal-monocentric-study-j-clin-med-2022-11-5705
#12
JOURNAL ARTICLE
Öner Özdemir
I read the article titled ' The Allergic Phenotype of Children and Adolescents with Selective IgA Deficiency: A Longitudinal Monocentric Study ' by Cinicola et al. with great interest [...].
October 24, 2023: Journal of Clinical Medicine
https://read.qxmd.com/read/37894426/exploring-the-significance-of-immune-checkpoints-and-ebv-reactivation-in-antibody-deficiencies-with-near-normal-immunoglobulin-levels-or-hyperimmunoglobulinemia
#13
JOURNAL ARTICLE
Paulina Mertowska, Sebastian Mertowski, Konrad Smolak, Marcin Pasiarski, Jolanta Smok-Kalwat, Stanisław Góźdź, Ewelina Grywalska
This study delves into the intricate landscape of primary immunodeficiencies, with a particular focus on antibody deficiencies characterized by near-normal immunoglobulin levels or hyperimmunoglobulinemia. Contrary to the conventional focus on genetic dysregulation, these studies investigate the key roles of immune checkpoints, such as PD-1/PD-L1, CTLA-4/CD86, and CD200R/CD200, on selected subpopulations of T and B lymphocytes and their serum concentrations of soluble forms in patients recruited for the studies in healthy volunteers...
October 19, 2023: Cancers
https://read.qxmd.com/read/37863316/the-association-between-malignancy-immunodeficiency-and-atopy-in-ige-deficient-patients
#14
JOURNAL ARTICLE
Ariela Agress, Yasmine Oprea, Shusmita Roy, Carolyn Strauch, David Rosenstreich, Denisa Ferastraoaru
BACKGROUND: Studies show that IgE deficient patients (IgE<2.5 kU/L) have a high prevalence of malignancy, but relevant clinical and laboratory characteristics associated with this susceptibility have never been well characterized. OBJECTIVE: To evaluate if there is an association between a malignancy diagnosis and other immunological parameters (atopy or other immune abnormalities) in IgE deficient patients. METHODS: We retrospectively analyzed medical records of 408 IgE deficient adults seen at our institution between 2005-2020...
October 18, 2023: Journal of Allergy and Clinical Immunology in Practice
https://read.qxmd.com/read/37850675/dental-treatment-under-general-anesthesia-with-nasal-intubation-in-a-patient-with-selective-immunoglobulin-a-deficiency
#15
JOURNAL ARTICLE
Yuho Sakuma, Mika Ogawa, Chie Nakagawa, Kodai Momota, Emi Kaji, Kingo Matsumura, Saori Morinaga, Kentaro Nogami, Mizuko Ikeda
Immunoglobulin A (IgA) deficiency is one of the most common immune disorders characterized by increased susceptibility to infections, especially involving the respiratory tract and mucosal surfaces of the mouth, gingiva, and nasal sinus. Because dental surgery and general anesthesia may pose an increased risk for systemic infections, management of IgA-deficient patients requires caution during dental procedures and intubated general anesthesia. We report a 5-year-old female patient with IgA deficiency who underwent extraction of 18 deciduous teeth under general anesthesia...
September 1, 2023: Anesthesia Progress
https://read.qxmd.com/read/37809154/guardians-of-immunity-advances-in-primary-immunodeficiency-disorders-and-management
#16
REVIEW
Nikhil Chowdary Peddi, Sravya Vuppalapati, Himabindu Sreenivasulu, Sudheer Kumar Muppalla, Apeksha Reddy Pulliahgaru
Primary immunodeficiency disorders (PIDs) are a heterogeneous group of genetic conditions profoundly impacting immune function. The investigation spans various PID categories, offering insights into their distinct pathogenic mechanisms and clinical manifestations. Within the adaptive immune system, B-cell, T-cell, and combined immunodeficiencies are dissected, emphasizing their critical roles in orchestrating effective immune responses. In the realm of the innate immune system, focus is directed toward phagocytes and complement deficiencies, underscoring the pivotal roles of these components in initial defense against infections...
September 2023: Curēus
https://read.qxmd.com/read/37797893/interim-analysis-open-label-extension-study-of-leniolisib-for-patients-with-apds
#17
JOURNAL ARTICLE
V Koneti Rao, Elaine Kulm, Anna Šedivá, Alessandro Plebani, Catharina Schuetz, Anna Shcherbina, Virgil A Dalm, Antonino Trizzino, Yulia Zharankova, Sharon Webster, Alanvin Orpia, Julia Körholz, Vassilios Lougaris, Yulia Rodina, Kath Radford, Jason Bradt, Anurag Relan, Steven M Holland, Michael J Lenardo, Gulbu Uzel
BACKGROUND: Activated phosphoinositide 3-kinase delta (PI3Kδ) syndrome (APDS; or p110δ-activating mutations causing senescent T cells, lymphadenopathy, and immunodeficiency) is an inborn error of immunity caused by PI3Kδ hyperactivity. Resultant immune deficiency and dysregulation lead to recurrent sinopulmonary infections, herpes viremia, autoimmunity, and lymphoproliferation. OBJECTIVE: Leniolisib, a selective PI3Kδ inhibitor, demonstrated favorable impact on immune cell subsets and lymphoproliferation over placebo in patients with APDS over 12 weeks...
October 4, 2023: Journal of Allergy and Clinical Immunology
https://read.qxmd.com/read/37728524/clinical-and-immunological-phenotypes-of-selective-igm-deficiency-in-children-results-from-a-multicenter-study
#18
MULTICENTER STUDY
Riccardo Castagnoli, Ivan Taietti, Martina Votto, Matteo Naso, Maria De Filippo, Alessia Marseglia, Lorenza Montagna, Mara De Amici, Maria Antonietta Avanzini, Daniela Montagna, Gian Luigi Marseglia, Amelia Licari
BACKGROUND: A few studies assessed the clinical and immunological features of selective IgM deficiency (SIgMD), especially in the pediatric age. We aimed to characterize the clinical and immunological phenotypes of a cohort of pediatric patients with SIgMD according to the different diagnostic criteria available. METHODS: In this multicenter study, we evaluated pediatric SIgMD patients diagnosed at the Pediatric Clinic in Pavia, Italy, or through the Italian Primary Immunodeficiency NETwork (IPINET) and monitored changes in their diagnosis over a time frame that ranges from several months to several years...
September 2023: Pediatric Allergy and Immunology
https://read.qxmd.com/read/37715890/clinical-validation-of-a-primary-antibody-deficiency-screening-algorithm-for-primary-care
#19
JOURNAL ARTICLE
Marianne A Messelink, Paco M J Welsing, Giovanna Devercelli, Jan Willem N Marsden, Helen L Leavis
PURPOSE: The diagnostic delay of primary antibody deficiencies (PADs) is associated with increased morbidity, mortality, and healthcare costs. Therefore, a screening algorithm was previously developed for the early detection of patients at risk of PAD in primary care. We aimed to clinically validate and optimize the PAD screening algorithm by applying it to a primary care database in the Netherlands. METHODS: The algorithm was applied to a data set of 61,172 electronic health records (EHRs)...
November 2023: Journal of Clinical Immunology
https://read.qxmd.com/read/37702233/immunological-evaluation-of-pediatric-patients-with-polyautoimmunity
#20
JOURNAL ARTICLE
Fatemeh Sadat Mahdavi, Marzieh Tavakol, Fatemeh Aghamahdi, Homa Sadri, Zahra Chavoshzadeh, Mahnaz Jamee, Shahab Noorian, Mohammad Reza Alaei, Pooya Ashkevari, Juan-Manuel Anaya, Hassan Abolhassani, Hans D Ochs, Gholamreza Azizi
BACKGROUND: Autoimmunity can be the first or predominant manifestation in patients with primary immunodeficiency disorder, also referred to as inborn errors of immunity (IEI). This study aims to evaluate the immune status of pediatric patients with polyautoimmunity to identify those with underlying immune defects. METHODS: In this cross-sectional study, pediatric patients with polyautoimmunity including at least one confirmed autoimmune endocrine disease were enrolled...
September 12, 2023: Endocrine, Metabolic & Immune Disorders Drug Targets
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