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Iga deficiency

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https://www.readbyqxmd.com/read/29204088/terminally-differentiated-memory-t-cells-are-increased-in-patients-with-common-variable-immunodeficiency-and-selective-iga-deficiency
#1
Jana Nechvatalova, Tomas Pavlik, Jiri Litzman, Marcela Vlkova
Introduction: Previous studies showed that several lymphocyte abnormalities seen in the most frequent symptomatic immunoglobulin deficiency, common variable immunodeficiency (CVID), were also observed in a genetically related asymptomatic disorder - selective IgA deficiency (IgAD). In this study we searched for abnormalities in the differentiation stages of T cells as well as for similarities of these abnormalities in CVID and IgAD patients. Material and methods: Using flow cytometry in 80 patients with IgAD, 48 patients with CVID, and 80 control persons we determined T-lymphocyte subsets: both CD4 and CD8 were divided into the naïve CD45RO-CD27+, early differentiated CD45RO+CD27+, late differentiated CD45RO+CD27- and fully differentiated effector CD45RO-CD27- memory T cells, as well as Treg cells, defined as CD4+CD25highCD127low T cells...
2017: Central-European Journal of Immunology
https://www.readbyqxmd.com/read/29202590/delayed-diagnosis-in-x-linked-agammaglobulinemia-and-its-relationship-to-the-occurrence-of-mutations-in-btk-non-kinase-domains
#2
Eduardo Carrillo-Tapia, Elizabeth García-García, Norma Estela Herrera-González, Marco Antonio Yamazaki-Nakashimada, Aidee Tamara Staines-Boone, Nora Hilda Segura-Mendez, Selma Cecilia Scheffler-Mendoza, Patricia O Farrill-Romanillos, Maria E Gonzalez-Serrano, Juan Carloa Rodriguez-Alba, Leopoldo Santos-Argumedo, Laura Berron-Ruiz, Alejandro Sanchez-Flores, Gabriela López-Herrera
BACKGROUND: X-linked agammaglobulinemia (XLA) is characterized by the absence of immunoglobulin and B cells. Patients suffer from recurrent bacterial infections from early childhood, and require lifelong immunoglobulin replacement therapy. Mutations in BTK (Bruton's Tyrosine Kinase) are associated with this phenotype. Some patients that present XLA do not show typical clinical symptoms, resulting in delayed diagnosis due to the lack of a severe phenotype. OBJECTIVE: This study presents a report of five XLA patients from four different families and attempts to determine a relationship between delayed diagnosis and the occurrence of BTK mutations...
December 5, 2017: Expert Review of Clinical Immunology
https://www.readbyqxmd.com/read/29194641/chemometric-analysis-of-minerals-in-gluten-free-products
#3
Anna Gliszczyńska-Świgło, Inga Klimczak, Iga Rybicka
BACKGROUND: Numerous studies indicate mineral deficiencies in people on a gluten-free (GF) diet. These deficiencies may indicate that GF products are less valuable source of minerals than gluten-containing products. In the study, the nutritional quality of fifty GF products is discussed taking into account the nutritional requirements for minerals expressed as % Recommended Daily Allowance (%RDA) or % Adequate Intake (%AI) for a model celiac patient. Analysed elements were: calcium, potassium, magnesium, sodium, copper, iron, manganese, and zinc...
November 30, 2017: Journal of the Science of Food and Agriculture
https://www.readbyqxmd.com/read/29190714/biomarkers-of-iga-vasculitis-nephritis-in-children
#4
Evangeline Pillebout, Agnès Jamin, Hamza Ayari, Pierre Housset, Melissa Pierre, Virginia Sauvaget, Denis Viglietti, Georges Deschenes, Renato C Monteiro, Laureline Berthelot
Henoch-Schönlein purpura is a systemic vasculitis characterized by IgA deposits, which target the skin, joints, and kidneys, among other organs. In children, prognosis is often good but little is known about biomarkers of pediatric nephritis. We hypothesized that biological markers, including cytokines, immunoglobulins, IgA-immune complexes, IgA glycosylation and neutrophil gelatinase-associated lipocalin (NGAL), may discriminate IgA vasculitis (IgAV) pediatric patients with renal involvement from those without renal involvement...
2017: PloS One
https://www.readbyqxmd.com/read/29186424/nalt-m-cells-are-important-for-immune-induction-for-the-common-mucosal-immune-system
#5
Yasuhiro Date, Masashi Ebisawa, Shinji Fukuda, Hideaki Shima, Yuuki Obata, Daisuke Takahashi, Tamotsu Kato, Misaho Hanazato, Gaku Nakato, Ifor R Williams, Koji Hase, Hiroshi Ohno
Nasopharynx-associated lymphoid tissue (NALT) is one of the major constituents of the mucosa-associated lymphoid tissue (MALT), and has the ability to induce antigen-specific immune responses. However, the molecular mechanisms responsible for antigen uptake from the nasal cavity into the NALT remain largely unknown. Immunohistochemical analysis showed that CCL9 and CCL20 were co-localized with GP2 in the epithelium covering NALT, suggesting the existence of M cells in NALT. In analogy with the reduced number of Peyer's patch M cells in CCR6-deficient mice, the number of NALT M cells was drastically decreased in CCR6-deficient mice compared to the wild-type mice...
November 27, 2017: International Immunology
https://www.readbyqxmd.com/read/29132233/prospects-for-modulating-the-cd40-cd40l-pathway-in-the-therapy-of-the-hyper-igm-syndrome
#6
Xiangxue Meng, Bin Yang, Wen-Chen Suen
The critical role of the CD40/CD40L pathway in B-cell proliferation, immunoglobulin (Ig) isotype switching and germinal center formation has been studied and described extensively in previous literature. Interruption of the CD40/CD40L signal causes hyper-IgM (HIGM) syndrome, which has been classified and recognized as a group of rare inherited immune deficiency disorders. Defects in CD40 and CD40L interactions or in downstream signaling molecules, including activation-induced cytidine deaminase, uracyl-DNA-glycosylase, NF-κB and DNA repair enzymes, result in an increased level of serum IgM and a significantly decreased or absent level of IgA, IgG and IgE that is accompanied by severe recurrent infections and autoimmune diseases...
January 1, 2017: Innate Immunity
https://www.readbyqxmd.com/read/29128760/vitamin-d-deficiency-in-children-with-recurrent-respiratory-infections-with-or-without-immunoglobulin-deficiency
#7
Nel Dąbrowska-Leonik, Ewa Bernatowska, Małgorzata Pac, Wiktor Filipiuk, Jan Mulawka, Barbara Pietrucha, Edyta Heropolitańska-Pliszka, Katarzyna Bernat-Sitarz, Beata Wolska-Kuśnierz, Bożena Mikołuć
PURPOSE: The objective of this study was to evaluate thevitamin D concentration in patients with recurrent respiratory infections with or without immunoglobulin G, A or M (IgG, IgA, IgM) deficiency, and to find a correlation between the vitamin D concentration and the response to hepatitis B vaccination. MATERIALS AND METHOD: The study involved 730 patients with recurrent respiratory infections. The concentration of 25-hydroxyvitamin D (25(OH)D), immunoglobulins G, A and M, anti-HBs was determined...
November 9, 2017: Advances in Medical Sciences
https://www.readbyqxmd.com/read/29126311/value-of-biomarkers-for-predicting-immunoglobulin-a-vasculitis-nephritis-outcome-in-an-adult-prospective-cohort
#8
Laureline Berthelot, Agnès Jamin, Denis Viglietti, Jonathan M Chemouny, Hamza Ayari, Melissa Pierre, Pierre Housset, Virginia Sauvaget, Margarita Hurtado-Nedelec, François Vrtovsnik, Eric Daugas, Renato C Monteiro, Evangeline Pillebout
Background: Henoch-Schönlein purpura, more recently renamed immunoglobulin A vasculitis (IgAV), is a systemic vasculitis characterized by IgA deposits. The current markers used to assess IgAV inaccurately evaluate the risk of nephritis occurrence and its long-term outcomes. The current study assessed biomarkers of nephritis outcomes. Methods: This French multicentre prospective study enrolled 85 adult patients at the time of disease onset. Patients were assessed for clinical and biological parameters and re-examined after 1 year...
November 3, 2017: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/29126302/the-clinical-utility-of-measuring-igg-subclass-immunoglobulins-during-immunological-investigation-for-suspected-primary-antibody-deficiencies
#9
Antony R Parker, Markus Skold, David B Ramsden, J Gonzalo Ocejo-Vinyals, Marcos López-Hoyos, Stephen Harding
Measurement of IgG subclass concentrations is a standard laboratory test run as part of a panel to investigate the suspicion of antibody deficiency. The assessment is clinically important when total IgG is within the normal age-specific reference range. The measurement is useful for diagnosis of IgG subclass deficiency, to aid the diagnosis of specific antibody deficiency, as a supporting test for the diagnosis of common variable immunodeficiency, as well as for risk stratification of patients with low IgA...
November 8, 2017: Laboratory Medicine
https://www.readbyqxmd.com/read/29114388/epistatic-interactions-between-mutations-of-taci-tnfrsf13b-and-tcf3-result-in-a-severe-primary-immunodeficiency-disorder-and-systemic-lupus-erythematosus
#10
Rohan Ameratunga, Wikke Koopmans, See-Tarn Woon, Euphemia Leung, Klaus Lehnert, Charlotte A Slade, Jessica C Tempany, Anselm Enders, Richard Steele, Peter Browett, Philip D Hodgkin, Vanessa L Bryant
Common variable immunodeficiency disorders (CVID) are a group of primary immunodeficiencies where monogenetic causes account for only a fraction of cases. On this evidence, CVID is potentially polygenic and epistatic although there are, as yet, no examples to support this hypothesis. We have identified a non-consanguineous family, who carry the C104R (c.310T>C) mutation of the Transmembrane Activator Calcium-modulator and cyclophilin ligand Interactor (TACI, TNFRSF13B) gene. Variants in TNFRSF13B/TACI are identified in up to 10% of CVID patients, and are associated with, but not solely causative of CVID...
October 2017: Clinical & Translational Immunology
https://www.readbyqxmd.com/read/29109124/endogenous-calcitriol-synthesis-controls-the-humoral-ige-response-in-mice
#11
Juliane Lindner, Sebastian Rausch, Sandra Treptow, Kerstin Geldmeyer-Hilt, Tina Krause, René St-Arnaud, Alice Arabian, Andreas Radbruch, Susanne Hartmann, Margitta Worm, Guido Heine
The vitamin D receptor participates in the control of IgE class-switch recombination in B cells. The physiologic vitamin D receptor agonist, 1,25(OH)2D3 (calcitriol), is synthesized by the essential enzyme 25-hydroxyvitamin D3-1α-hydroxylase (CYP27B1), which can be expressed by activated immune cells. The role of endogenous calcitriol synthesis for the regulation of IgE has not been proven. In this study, we investigated IgE-responses in Cyp27b1-knockout (KO) mice following sensitization to OVA or intestinal infection with Heligmosomoides polygyrus Specific Igs and plasmablasts were determined by ELISA and ELISpot, Cyp27b1 expression was measured by quantitative PCR...
November 6, 2017: Journal of Immunology: Official Journal of the American Association of Immunologists
https://www.readbyqxmd.com/read/29099932/the-role-of-b-cells-in-carriage-and-clearance-of-mycoplasma-pneumoniae-from-the-respiratory-tract-of-mice
#12
Patrick M Meyer Sauteur, Ruben C A de Groot, Silvia C Estevão, Theo Hoogenboezem, Adrianus C J M de Bruijn, Marcel Sluijter, Marjolein J W de Bruijn, Ismé M De Kleer, Rien van Haperen, Judith M A van den Brand, Debby Bogaert, Pieter L A Fraaij, Cornelis Vink, Rudi W Hendriks, Janneke N Samsom, Wendy W J Unger, Annemarie M C van Rossum
Carriage of Mycoplasma pneumoniae (Mp) in the nasopharynx is considered a prerequisite for pulmonary infection. Interestingly, Mp carriage is also detected after infection. While B cells are known to be involved in pulmonary Mp clearance, their role in Mp carriage is unknown. We here show in a mouse model that Mp persists in the nose after pulmonary infection, similar to humans. Infection of mice enhanced Mp-specific immunoglobulin (Ig) M and IgG levels in serum and bronchoalveolar lavage fluid. However, nasal washes only contained elevated Mp-specific IgA...
November 1, 2017: Journal of Infectious Diseases
https://www.readbyqxmd.com/read/29092674/autoantibodies-against-complement-components-in-systemic-lupus-erythematosus-role-in-the-pathogenesis-and-clinical-manifestations
#13
M H Hristova, V S Stoyanova
Many complement structures and a number of additional factors, i.e. autoantibodies, receptors, hormones and cytokines, are implicated in the complex pathogenesis of systemic lupus erythematosus. Genetic defects in the complement as well as functional deficiency due to antibodies against its components lead to different pathological conditions, usually clinically presented. Among them hypocomplementemic urticarial vasculitis, different types of glomerulonephritis as dense deposit disease, IgA nephropathy, atypical haemolytic uremic syndrome and lupus nephritis are very common...
December 2017: Lupus
https://www.readbyqxmd.com/read/29075899/immunoglobulin-a-and-liver-diseases
#14
REVIEW
Tatsuo Inamine, Bernd Schnabl
Immunoglobulin A (IgA) is a major immunoglobulin isotype in the gut and plays a role in maintenance of gut homeostasis. Secretory IgA (SIgA) has multiple functions in the gut, such as to regulate microbiota composition, to protect intestinal epithelium from pathogenic microorganisms, and to help for immune-system development. The liver is the front-line organ that receives gut-derived products through the portal vein, implying that the liver could be severely affected by a disrupted intestinal homeostasis. Indeed, some liver diseases like alcoholic liver disease are associated with an altered composition of gut microbiota and increased blood endotoxin levels...
October 26, 2017: Journal of Gastroenterology
https://www.readbyqxmd.com/read/29037908/new-insights-in-the-pathogenesis-of-immunoglobulin-a-vasculitis-henoch-sch%C3%A3-nlein-purpura
#15
REVIEW
Marieke H Heineke, Aranka V Ballering, Agnès Jamin, Sanae Ben Mkaddem, Renato C Monteiro, Marjolein Van Egmond
Immunoglobulin A vasculitis (IgAV), also referred to as Henoch-Schönlein purpura, is the most common form of childhood vasculitis. The pathogenesis of IgAV is still largely unknown. The disease is characterized by IgA1-immune deposits, complement factors and neutrophil infiltration, which is accompanied with vascular inflammation. Incidence of IgAV is twice as high during fall and winter, suggesting an environmental trigger associated to climate. Symptoms can resolve without intervention, but some patients develop glomerulonephritis with features similar to IgA nephropathy that include hematuria, proteinuria and IgA deposition in the glomerulus...
October 14, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/29021278/dual-roles-of-glutathione-in-ecdysone-biosynthesis-and-antioxidant-function-during-the-larval-development-in-drosophila
#16
Sora Enya, Chikana Yamamoto, Hajime Mizuno, Tsuyoshi Esaki, Hsin-Kuang Lin, Masatoshi Iga, Kana Morohashi, Yota Hirano, Hiroshi Kataoka, Tsutomu Masujima, Yuko Shimada-Niwa, Ryusuke Niwa
Ecdysteroids, including the biologically active hormone 20-hydroxyecdysone (20E), play essential roles in controlling many developmental and physiological events in insects. Ecdysteroid biosynthesis is achieved by a series of specialized enzymes encoded in the Halloween genes. Recently, a new class of Halloween gene, noppera-bo (nobo), encoding a glutathione S-transferase (GST) in dipteran and lepidopteran species, has been identified and characterized. GSTs are well known to conjugate substrates with the reduced form of glutathione (GSH), a bioactive tripeptide composed of glutamate, cysteine, and glycine...
October 11, 2017: Genetics
https://www.readbyqxmd.com/read/28985731/screening-for-celiac-disease-in-poorly-controlled-type-2-diabetes-mellitus-worth-it-or-not
#17
Muhammed Kizilgul, Ozgur Ozcelik, Selvihan Beysel, Hakan Akinci, Seyfullah Kan, Bekir Ucan, Mahmut Apaydin, Erman Cakal
BACKGROUND: Recent studies have demonstrated that immune factors might have a role in the pathophysiology of insulin resistance and type 2 diabetes mellitus (T2DM). Inappropriate glycemic control in patients with T2DM is an important risk factor for the occurrence of diabetes complications. The prevalence of celiac disease (CD) is high in type 1 diabetes mellitus however, there are scarce data about its prevalence in T2DM. Our aim was to investigate the prevalence of celiac disease among insulin-using type 2 diabetes patients with inappropriate glycemic control...
October 6, 2017: BMC Endocrine Disorders
https://www.readbyqxmd.com/read/28984901/truly-selective-primary-igm-deficiency-is-probably-very-rare
#18
L M A Janssen, T Macken, M C W Creemers, J F M Pruijt, J J J Eijk, E de Vries
Isolated decreased serum-immunoglobulin (Ig)M has been associated with severe and/or recurrent infections, atopy and autoimmunity. However, the reported high prevalence of clinical problems in IgM-deficient patients may reflect the skewed tertiary centre population studied so far. Also, many papers on IgM deficiency have included patients with more abnormalities than simply IgM-deficiency. We studied truly selective primary IgM deficiency according to the diagnostic criteria of the European Society for Immunodeficiencies (ESID) (true sIgMdef) by reviewing the literature (261 patients with primary decreased serum-IgM in 46 papers) and analysing retrospectively all patients with decreased serum-IgM in a large teaching hospital in 's-Hertogenbosch, the Netherlands [1 July 2005-23 March 2016; n = 8049 IgM < 0·4 g/l; n = 2064 solitary (IgG+IgA normal/IgM < age-matched reference)]...
October 6, 2017: Clinical and Experimental Immunology
https://www.readbyqxmd.com/read/28969604/pathogenic-role-of-glycan-specific-igg-antibodies-in-iga-nephropathy
#19
Yan-Feng Zhao, Li Zhu, Li-Jun Liu, Su-Fang Shi, Ji-Cheng Lv, Hong Zhang
BACKGROUND: Accumulating evidences proved the important roles of circulating IgA1-containing immune complexes (cIgA1) in IgA nephropathy (IgAN). Galactose-deficient IgA1 (Gd-IgA1) and glycan-specific IgG antibody have been identified as major components in cIgA1. Before, Gd-IgA1 was reported as a vital factor in IgAN, partly via of its pathogenic role to induce mesangial cells activation. However, we still lack direct evidences to clarify the biological effect of glycan-specific IgG antibody in IgAN...
September 29, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/28966241/effect-of-minerals-on-intestinal-iga-production-using-deep-sea-water-drinks
#20
Hisashi Shiraishi, Maho Fujino, Naoki Shirakawa, Nanao Ishida, Hiroki Funato, Ayumu Hirata, Noriaki Abe, Michiro Iizuka, Kohei Jobu, Junko Yokota, Mitsuhiko Miyamura
Minerals are essential for life, as they are a vital part of protein constituents, enzyme cofactors, and other components in living organisms. Deep sea water is characterized by its cleanliness and stable low temperature, and its possible health- and medical benefits are being studied. However, no study has yet evaluated the physical properties of the numerous commercially available deep sea water products, which have varying water sources and production methods. We analyzed these products' mineral content and investigated their effect on living organism, focusing on immune functions, and investigated the relation between physiological immunoactivities and mineral intake...
2017: Biological & Pharmaceutical Bulletin
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