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Iga deficiency

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https://www.readbyqxmd.com/read/28941669/increased-ige-in-iga-deficiency
#1
EDITORIAL
Dennis K Ledford
No abstract text is available yet for this article.
September 20, 2017: Journal of Allergy and Clinical Immunology in Practice
https://www.readbyqxmd.com/read/28938254/antibiotic-prophylaxis-immunoglobulin-substitution-and-supportive-measures-prevent-infections-in-mecp2-duplication-syndrome
#2
Michael Bauer, Renate Krüger, Uwe Kölsch, Nadine Unterwalder, Christian Meisel, Volker Wahn, Horst von Bernuth
Respiratory infections are the main cause of early death in patients with MECP2 duplication syndrome. We report on a 20-year-old patient with MECP2 duplication syndrome, IgG2/IgG4/IgA/IgM-deficiency and polysaccharide-specific-antibody-deficiency, who had 46 episodes of pneumonia in his first 13 years of life. Immunoglobulin substitution, daily antibiotic prophylaxis with two agents and supportive measures reduced occurrence of pneumonia to 4 episodes in the following 6 years of life.
September 20, 2017: Pediatric Infectious Disease Journal
https://www.readbyqxmd.com/read/28932819/iga-and-igm-protein-primarily-drive-plasma-corona-induced-adhesion-reduction-of-plga-nanoparticles-in-human-blood-flow
#3
Daniel J Sobczynski, Omolola Eniola-Adefeso
The high abundance of immunoglobulins (Igs) in the plasma protein corona on poly(lactic-co-glycolic) acid (PLGA)-based vascular-targeted carriers (VTCs) has previously been shown to reduce their adhesion to activated endothelial cells (aECs) in human blood flow. However, the relative role of individual Ig classes (e.g., IgG, IgA, and IgM) in causing adhesion reduction remains largely unknown. Here, we characterized the influence of specific Ig classes in prescribing the binding efficiency of PLGA nano-sized VTCs in blood flow...
June 2017: Bioengineering & Translational Medicine
https://www.readbyqxmd.com/read/28932552/chylothorax-after-pediatric-cardiac-surgery-complicates-short-term-but-not-long-term-outcomes-a-propensity-matched-analysis
#4
Nikoletta R Czobor, György Roth, Zsolt Prodán, Daniel J Lex, Erzsébet Sápi, László Ablonczy, Mihály Gergely, Edgar A Székely, János Gál, Andrea Székely
BACKGROUND: The occurrence of postoperative chylothorax in children with congenital heart disease is a rare and serious complication in cardiac intensive care units (ICUs). The aim of our study was to identify the perioperative characteristics, treatment options, resource utilization and long term complications of patients having chylothorax after a pediatric cardiac surgery. METHODS: Patients were retrospectively assessed for the presence of chylothorax between January 2002 and December 2012 in a tertiary national cardiac center...
August 2017: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/28928744/ccctc-binding-factor-locks-premature-igh-germline-transcription-and-restrains-class-switch-recombination
#5
Ester Marina-Zárate, Arantxa Pérez-García, Almudena R Ramiro
In response to antigenic stimulation B cells undergo class switch recombination (CSR) at the immunoglobulin heavy chain (IgH) to replace the primary IgM/IgD isotypes by IgG, IgE, or IgA. CSR is initiated by activation-induced cytidine deaminase (AID) through the deamination of cytosine residues at the switch (S) regions of IgH. B cell stimulation promotes germline transcription (GLT) of specific S regions, a necessary event prior to CSR because it facilitates AID access to S regions. Here, we show that CCCTC-binding factor (CTCF)-deficient mice are severely impaired in the generation of germinal center B cells and plasma cells after immunization in vivo, most likely due to impaired cell survival...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28928736/selective-igm-deficiency-an-underestimated-primary-immunodeficiency
#6
REVIEW
Sudhir Gupta, Ankmalika Gupta
Although selective IgM deficiency (SIGMD) was described almost five decades ago, it was largely ignored as a primary immunodeficiency. SIGMD is defined as serum IgM levels below two SD of mean with normal serum IgG and IgA. It appears to be more common than originally realized. SIGMD is observed in both children and adults. Patients with SIGMD may be asymptomatic; however, approximately 80% of patients with SIGMD present with infections with bacteria, viruses, fungi, and protozoa. There is an increased frequency of allergic and autoimmune diseases in SIGMD...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28915952/iga-nephropathy-during-treatment-with-tnf-alpha-blockers-could-it-be-predicted
#7
Vito Di Lernia
Immunoglobulin A (IgA) nephropathy (IgAN) may sometimes be related to exposure to pharmacological agents, among which anti-Tumor Necrosis Factor (TNF)-alpha agents. The characteristic pathological feature is a deposition of IgA-containing immune complexes in vessel walls in the kidney mesangium. The link between TNF-alpha blockers and IgAN may be hypothesized examining diseases which share pathologic features. In this respect, idiopathic IgAN and Henoch Schonlein Purpura have been the object of studies revealing a pathogenetic role of aberrant glycosylation of IgA1 molecules...
September 2017: Medical Hypotheses
https://www.readbyqxmd.com/read/28882590/targeted-deletion-of-rankl-in-m-cell-inducer-cells-by-the-col6a1-cre-driver
#8
Kazuki Nagashima, Shinichiro Sawa, Takeshi Nitta, Alejandro Prados, Vasiliki Koliaraki, George Kollias, Tomoki Nakashima, Hiroshi Takayanagi
The gut-associated lymphoid tissues (GALTs), including Peyer's patches (PPs), cryptopatches (CPs) and isolated lymphoid follicles (ILFs), establish a host-microbe symbiosis by the promotion of immune reactions against gut microbes. Microfold cell inducer (MCi) cells in GALTs are the recently identified mesenchymal cells that express the cytokine RANKL and initiate bacteria-specific immunoglobulin A (IgA) production via induction of microfold (M) cell differentiation. In the previous study, the Twist2-Cre driver was utilized for gene deletion in mesenchymal cells including MCi cells...
September 5, 2017: Biochemical and Biophysical Research Communications
https://www.readbyqxmd.com/read/28881511/-role-of-igg-antibody-to-galactose-deficient-iga1-in-children-with-iga-nephropathy
#9
N Zhou, H Zhang, X R Liu, Q Sun, Q Meng, Z Chen, Y Shen
Objective: In order to learn the serum level of galactose-deficient IgA1 (GdIgA1), IgG antibody to galactose-deficient IgA1(GdIgA1-IgG) and the clinical role of them in IgA nephropathy(IgAN) children. Method: We compared blood levels of GdIgA1, GdIgA1-IgG in 33 children with IgAN, 38 children with other renal disease (including focal segmental glomerular nephritis, minimal change disease, Alport syndrome and thin basement membrane nephropathy) as disease controls, 35 healthy children as normal controls with enzyme-linked immunosorbent assay(ELISA)...
September 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/28852483/immunoglobulin-levels-and-infection-risk-with-rituximab-induction-for-anti-neutrophil-cytoplasmic-antibody-associated-vasculitis
#10
Shivani Shah, Khushleen Jaggi, Keiko Greenberg, Duvuru Geetha
BACKGROUND: Rituximab (RTX), a B cell-depleting anti-CD20 monoclonal antibody, is approved for treatment of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). Low immunoglobulin (Ig) levels have been observed surrounding RTX treatment. The association between the degree of Ig deficiency and infection risk is unclear in AAV patients. METHODS: AAV patients treated with RTX for remission induction at a single center (2005-15) with serum Ig measurements were included...
August 2017: Clinical Kidney Journal
https://www.readbyqxmd.com/read/28842185/early-diagnosis-of-pi3k%C3%AE-syndrome-in-a-2-years-old-girl-with-recurrent-otitis-and-enlarged-spleen
#11
F Saettini, M A Pelagatti, D Sala, D Moratto, S Giliani, R Badolato, A Biondi
Heterozygous gain of function mutations in the gene encoding p110δ subunit of PI3K have been recently associated with activated PI3K-δ syndrome (APDS), a novel combined immune deficiency characterized by recurrent sinopulmonary infections, lymphopenia, reduced class-switched memory B cells, lymphadenopathy, CMV and/or EBV viremia and EBV-related lymphoma. Here we report a dominant gain of function PIK3CD mutation (E1021K) in a patient presenting with recurrent otitis media, massive splenomegaly, and persistent EBV-viraemia...
August 31, 2017: Immunology Letters
https://www.readbyqxmd.com/read/28815356/dent-disease-in-poland-what-we-have-learned-so-far
#12
Marcin Zaniew, Małgorzata Mizerska-Wasiak, Iga Załuska-Leśniewska, Piotr Adamczyk, Katarzyna Kiliś-Pstrusińska, Adam Haliński, Jan Zawadzki, Beata S Lipska-Ziętkiewicz, Krzysztof Pawlaczyk, Przemysław Sikora, Michael Ludwig, Maria Szczepańska
PURPOSE: Dent disease (DD) is a rare tubulopathy characterized by proximal tubular dysfunction leading to chronic kidney disease (CKD). The aim of the study was to characterize patients with DD in Poland. METHODS: A retrospective analysis of a national cohort with genetically confirmed diagnosis. RESULTS: Of 24 males, all patients except one carried mutations in the CLCN5 gene; in one patient a mutation in the OCRL gene was disclosed. Molecular diagnosis was delayed 1 year on average (range 0-21 years)...
August 16, 2017: International Urology and Nephrology
https://www.readbyqxmd.com/read/28801359/ccr7-deficiency-allows-accelerated-clearance-of-chlamydia-from-the-female-reproductive-tract
#13
Lin-Xi Li, Jasmine C Labuda, Denise M Imai, Stephen M Griffey, Stephen J McSorley
Immune mechanisms responsible for pathogen clearance from the female reproductive tract (FRT) are incompletely defined; in particular, the contribution of lymphocyte trafficking to this process is unclear. CCR7-deficient mice have profoundly altered lymphocyte recirculation and display ectopic formation of lymphocyte aggregates within mucosal nonlymphoid tissues, including the FRT. In this study, we investigated how altered lymphocyte distribution in CCR7-deficient mice would affect host responses to Chlamydia muridarum within the reproductive tract...
October 1, 2017: Journal of Immunology: Official Journal of the American Association of Immunologists
https://www.readbyqxmd.com/read/28774562/defective-anti-polysaccharide-igg-vaccine-responses-in-iga-deficient-mice
#14
Yoichi Furuya, Girish S Kirimanjeswara, Sean Roberts, Rachael Racine, Jennifer Wilson-Welder, Alan M Sanfilippo, Sharon L Salmon, Dennis W Metzger
We report that IgA(-/-) mice exhibit specific defects in IgG antibody responses to various polysaccharide vaccines (Francisella tularensis LPS and Pneumovax), but not protein vaccines such as Fluzone. This defect further included responses to polysaccharide-protein conjugate vaccines (Prevnar and Haemophilus influenzae type b-tetanus toxoid vaccine). In agreement with these findings, IgA(-/-) mice were protected from pathogen challenge with protein- but not polysaccharide-based vaccines. Interestingly, after immunization with live bacteria, IgA(+/+) and IgA(-/-) mice were both resistant to lethal challenge and their IgG anti-polysaccharide antibody responses were comparable...
July 31, 2017: Vaccine
https://www.readbyqxmd.com/read/28768724/pd-l2-regulates-b-1-cell-antibody-production-against-phosphorylcholine-through-an-il-5-dependent-mechanism
#15
Jerome T McKay, Marcela A Haro, Christina A Daly, Rama D Yammani, Bing Pang, W Edward Swords, Karen M Haas
B-1 cells produce natural Abs which provide an integral first line of defense against pathogens while also performing important homeostatic housekeeping functions. In this study, we demonstrate that programmed cell death 1 ligand 2 (PD-L2) regulates the production of natural Abs against phosphorylcholine (PC). Naive PD-L2-deficient (PD-L2(-/-)) mice produced significantly more PC-reactive IgM and IgA. This afforded PD-L2(-/-) mice with selectively enhanced protection against PC-expressing nontypeable Haemophilus influenzae, but not PC-negative nontypeable Haemophilus influenzae, relative to wild-type mice...
September 15, 2017: Journal of Immunology: Official Journal of the American Association of Immunologists
https://www.readbyqxmd.com/read/28755066/two-sides-of-the-same-coin-pediatric-onset-and-adult-onset-common-variable-immune-deficiency
#16
Lauren A Sanchez, Solrun Melkorka Maggadottir, Matthew S Pantell, Patricia Lugar, Charlotte Cunningham Rundles, Kathleen E Sullivan
PURPOSE: Common variable immunodeficiency (CVID) is a complex, heterogeneous immunodeficiency characterized by hypogammaglobulinemia, recurrent infections, and poor antibody response to vaccination. While antibiotics and immunoglobulin prophylaxis have significantly reduced infectious complications, non-infectious complications of autoimmunity, inflammatory lung disease, enteropathy, and malignancy remain of great concern. Previous studies have suggested that CVID patients diagnosed in childhood are more severely affected by these complications than adults diagnosed later in life...
July 28, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28746740/ccr6-promotes-steady-state-intestinal-mononuclear-phagocyte-association-with-the-intestinal-epithelium-imprinting-and-immune-surveillance
#17
Keely G McDonald, Leroy W Wheeler, Jeremiah R McDole, Shannon Joerger, Jenny K Gustafsson, Devesha H Kulkarni, Kathryn A Knoop, Ifor R Williams, Mark J Miller, Rodney D Newberry
The intestinal lamina propria (LP) contains antigen presenting cells with features of dendritic cells (DCs) and macrophages, collectively referred to as mononuclear phagocytes (MNPs). Association of MNPs with the epithelium is implied to play an important role in multiple facets of intestinal immunity including imprinting MNPs with the ability to induce IgA production, inducing the expression of gut homing molecules on T cells, facilitating the capture of luminal antigens and microbes, and subsequent immune responses in the mesenteric lymph node (MLN)...
July 26, 2017: Immunology
https://www.readbyqxmd.com/read/28744287/ccr6-deficiency-impairs-iga-production-and-dysregulates-antimicrobial-peptide-production-altering-the-intestinal-flora
#18
Ya-Lin Lin, Peng-Peng Ip, Fang Liao
Intestinal immunity exists as a complex relationship among immune cells, epithelial cells, and microbiota. CCR6 and its ligand-CCL20 are highly expressed in intestinal mucosal tissues, such as Peyer's patches (PPs) and isolated lymphoid follicles (ILFs). In this study, we investigated the role of the CCR6-CCL20 axis in intestinal immunity under homeostatic conditions. CCR6 deficiency intrinsically affects germinal center reactions in PPs, leading to impairments in IgA class switching, IgA affinity, and IgA memory B cell production and positioning in PPs, suggesting an important role for CCR6 in T-cell-dependent IgA generation...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28738401/serum-vitamins-and-minerals-at-diagnosis-and-follow-up-in-children-with-celiac-disease
#19
Vini Deora, Nicole Aylward, AbdulRazaq Sokoro, Wael El-Matary
OBJECTIVES: Children with celiac disease (CD) may experience deficiencies of several micronutrients. The objectives of the present study were to determine the prevalence of micronutrient deficiencies in children with CD at diagnosis, 6 months, and 18 months after the start of a gluten-free diet (GFD), and examine any correlation between micronutrient deficiencies, serum tissue transglutaminase (TtG) immunoglobulin A (IgA) antibody titers, and the degree of mucosal damage at diagnosis...
August 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28737147/brucella-causing-liver-abscess-in-a-child-with-selective-iga-deficiency
#20
Devdeep Mukherjee, Priyankar Pal, Ritabrata Kundu
BACKGROUND: Brucella has been known to cause pyrexia of unknown origin. CASE CHARACTERISTICS: 9-year-old boy with fever and abdominal pain; multiple abscesses within the liver on ultrasonography. OBSERVATIONS: IgM Antibodies against Brucella were raised in his serum sample, and Brucella serum agglutination test was positive. Immunological work-up suggested selective IgA deficiency. Reduction in size following treatment with trimethoprim-sulphamethoxazole, amikacin and doxycycline...
July 15, 2017: Indian Pediatrics
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