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Selective IGA Deficiency

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https://www.readbyqxmd.com/read/29755476/transitional-b-cells-and-tlr9-responses-are-defective-in-selective-iga-deficiency
#1
Andri L Lemarquis, Helga K Einarsdottir, Rakel N Kristjansdottir, Ingileif Jonsdottir, Bjorn R Ludviksson
Selective IgA deficiency (IgAD) is the most common primary antibody deficiency in the western world with affected individuals suffering from an increased burden of autoimmunity, atopic diseases and infections. It has been shown that IgAD B cells can be induced with germinal center mimicking reactions to produce IgA. However, IgA is the most prevalent antibody in mucosal sites, where antigen-independent responses are important. Much interest has recently focused on the role of TLR9 in both naïve and mature B cell differentiation into IgA secreting plasma cells...
2018: Frontiers in Immunology
https://www.readbyqxmd.com/read/29651973/the-clinical-and-immunological-features-of-patients-with-primary-antibody-deficiencies
#2
Gholamreza Azizi, Yasser Bagheri, Marzieh Tavakol, Forough Askarimoghaddam, Kiyoomars Poorrostami, Hosein Rafiemanesh, Reza Yazdani, Fatemeh Kiaee, Sima Habibi, Kosar Abouhamzeh, Hamed Mohammadi, Mostafa Qorbani, Hassan Abolhassani, Asghar Aghamohammadi
BACKGROUND: Primary antibody deficiency (PAD) comprises a range of diseases from early to late terminal B cells defects and is associated with the various clinical complications. METHODS: A total of 461 patients (311 males and 150 females) with PADs enrolled in the retrospective cohort study and for all patients' demographic information, clinical records and laboratory data were collected to investigate clinical complications. RESULTS: The most prevalent first presentations of immunodeficiency were respiratory tract infections in 63...
April 12, 2018: Endocrine, Metabolic & Immune Disorders Drug Targets
https://www.readbyqxmd.com/read/29527861/possible-autoimmune-primary-ovarian-insufficiency-in-patients-with-selective-iga-deficiency
#3
Eli Magen, Atheer Masalha, Ekaterina Zueva, Daniel A Vardy
No abstract text is available yet for this article.
March 2018: Israel Medical Association Journal: IMAJ
https://www.readbyqxmd.com/read/29392049/sensorineural-hearing-loss-in-selective-immunglobulin-a-deficiency
#4
Erkan Eşki, Belgin Emine Usta, Suna Asilsoy, İsmail Yılmaz
Objective: To assess hearing functions in pediatric patients with selective immunoglobulin A (IgA) deficiency (SIGAD). Methods: Pure-tone audiometry, acoustic impedance, otoacoustic emission, and brainstem audiometric measurements were taken during a non-infectious period in 28 patients with SIGAD and 28 healthy children with normal otoscopic examination. The results of the hearing tests were compared between the two groups. Results: Two male patients and one female patient in the SIGAD group were found to have sensorineural hearing loss (SNHL)...
March 2017: Turkish Archives of Otorhinolaryngology
https://www.readbyqxmd.com/read/29379538/histologic-recovery-among-children-with-celiac-disease-on-a-gluten-free-diet-a-long-term-follow-up-single-center-experience
#5
Oana Belei, Andreea Dobrescu, Rodica Heredea, Emil Radu Iacob, Vlad David, Otilia Marginean
Introduction: Celiac disease (CD) is defined by gluten-induced immune-mediated enteropathy, affecting approximately 1% of the genetically predisposed population. The immunologic response to gluten causes characteristic intestinal alterations with gradual development. Histologic recovery of intestinal architecture was reported to occur within 6-12 months after starting a gluten-free diet, simultaneously with clinical remission. The aim of this study was to assess the rate and timing of histologic recovery among children with CD on a gluten-free diet, diagnosed and followed in an academic referral pediatric center during a 10-year period...
January 2018: Archives of Medical Science: AMS
https://www.readbyqxmd.com/read/29204088/terminally-differentiated-memory-t-cells-are-increased-in-patients-with-common-variable-immunodeficiency-and-selective-iga-deficiency
#6
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/28985731/screening-for-celiac-disease-in-poorly-controlled-type-2-diabetes-mellitus-worth-it-or-not
#7
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
#8
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)]...
February 2018: Clinical and Experimental Immunology
https://www.readbyqxmd.com/read/28928736/selective-igm-deficiency-an-underestimated-primary-immunodeficiency
#9
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/28768724/pd-l2-regulates-b-1-cell-antibody-production-against-phosphorylcholine-through-an-il-5-dependent-mechanism
#10
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/28737147/brucella-causing-liver-abscess-in-a-child-with-selective-iga-deficiency
#11
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
https://www.readbyqxmd.com/read/28730517/selective-igm-deficiency-clinical-and-laboratory-features-of-17-patients-and-a-review-of-the-literature
#12
Zita Chovancova, Pavlina Kralickova, Alena Pejchalova, Marketa Bloomfield, Jana Nechvatalova, Marcela Vlkova, Jiri Litzman
PURPOSE: Primary selective IgM deficiency (sIgMD) is a primary immunodeficiency with unclear pathogenesis and a low number of published cases. METHODS: We reviewed clinical and laboratory manifestations of 17 sIgMD patients. Serum IgM, IgG, and its subclasses, IgA, IgE, antibodies against tetanus toxoid, pneumococcal polysaccharides and Haemophilus influenzae type b, isohemagglutinins, and T and B lymphocyte subsets, expressions of IgM on B cells and B lymphocyte production of IgM were compared with previously reported case reports and a small series of patients, which included 81 subjects in total...
August 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28691461/oral-manifestations-of-selective-iga-deficiency-review-and-case-report
#13
L Azzi, F Croveri, R Vinci, V Maurino, A Boggio, D Mantegazza, D Farronato, A Tagliabue, J Silvestre-Rangil, L Tettamanti
Immunoglobulin A deficiency is the most common primary immunodeficiency defined as decreased serum level of IgA (less than 7 mg/dl) in the presence of normal levels of other immunoglobulin isotypes. Most individuals with IgA deficiency are asymptomatic and identified coincidentally. However, some patients may present with recurrent infections, allergic disorders and autoimmune manifestations, such as diabetes mellitus, Graves disease and celiac disease. The international literature has not produced any kind of review yet about intra-oral manifestations of selective IgA-deficiency...
April 2017: Journal of Biological Regulators and Homeostatic Agents
https://www.readbyqxmd.com/read/28651547/autoantibodies-against-baff-april-or-il21-an-alternative-pathogenesis-for-antibody-deficiencies
#14
Marian-Christopher Pott, Natalie Frede, Jennifer Wanders, Lennart Hammarström, Erik-Oliver Glocker, Cristina Glocker, Fariba Tahami, Bodo Grimbacher
BACKGROUND: The ability of anti-cytokine antibodies to play a disease-causing role in the pathogenesis of immunodeficiencies is widely accepted. The aim of this study was to investigate whether autoantibodies against BAFF (important B cell survival signal), APRIL (important plasma cell survival signal), or Interleukin-21 (important cytokine for immunoglobulin class switch) present an alternative mechanism for the development of the following primary antibody deficiencies (PADs): common variable immune deficiency (CVID) or selective IgA deficiency (sIgAD)...
June 26, 2017: BMC Immunology
https://www.readbyqxmd.com/read/28579988/an-expanded-role-for-hla-genes-hla-b-encodes-a-microrna-that-regulates-iga-and-other-immune-response-transcripts
#15
Nilesh Chitnis, Peter M Clark, Malek Kamoun, Catherine Stolle, F Brad Johnson, Dimitri S Monos
We describe a novel functional role for the HLA-B locus mediated by its intron-encoded microRNA (miRNA), miR-6891-5p. We show that in vitro inhibition of miR-6891-5p impacts the expression of nearly 200 transcripts within the B-lymphoblastoid cell line (B-LCL) COX, affecting a large number of metabolic pathways, including various immune response networks. The top affected transcripts following miR-6891-5p inhibition are those encoding the heavy chain of IgA. We identified a conserved miR-6891-5p target site on the 3'UTR of both immunoglobulin heavy chain alpha 1 and 2 (IGHA1 and IGHA2) transcripts and demonstrated that this miRNA modulates the expression of IGHA1 and IGHA2...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28480529/acne-conglobata-in-a-long-term-survivor-with-trisomy-13-accompanied-by-selective-igm-deficiency
#16
Chiyoko N Inoue, Yoshiko Tanaka, Nobuko Tabata
Trisomy 13 (T13) is a congenital chromosomal disorder that is usually fatal within 2 years of birth, and only a few patients have been reported to reach adolescence. Here, we report a male long-term survivor of T13, currently 15 years of age, with a several-year history of extensive acne conglobata (AC) with abscesses on the face and neck. Methicillin-resistant Staphylococcus aureus was consistently isolated from the pustular lesions. Serum IgM levels were extremely low at 10 mg/dl. There were no abnormalities in neutrophil and total B cell number, or in serum IgA and IgG levels...
May 7, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28425252/allergic-and-autoimmune-disorders-in-families-with-selective-iga-deficiency
#17
Mustafa Erkoçoğlu, Ayşe Metin, Ayşenur Kaya, Celal Özcan, Ayşegül Akan, Ersoy Civelek, Murat Çapanoğlu, Tayfur Giniş, Can Naci Kocabaş
BACKGROUND/AIM: IgA deficiency is the most common human primary immunodeficiency. The prevalence of allergic disorders and autoimmunity is thought to be increased in selective IgA deficiency (sIgAD). However, it is currently unclear if these disorders coincide within these families. We aimed to evaluate the frequency of allergic and autoimmune disorders in children with sIgAD and their first-degree relatives (FDRs). MATERIALS AND METHODS: The study included 81 children diagnosed with sIgAD and 274 of their FDRs...
April 18, 2017: Turkish Journal of Medical Sciences
https://www.readbyqxmd.com/read/28212436/antibody-deficiency-in-patients-with-frequent-exacerbations-of-chronic-obstructive-pulmonary-disease-copd
#18
Brian N McCullagh, Alejandro P Comellas, Zuhair K Ballas, John D Newell, M Bridget Zimmerman, Antoine E Azar
Chronic Obstructive Pulmonary Disease is the third leading cause of death in the US, and is associated with periodic exacerbations, which account for the largest proportion of health care utilization, and lead to significant morbidity, mortality, and worsening lung function. A subset of patients with COPD have frequent exacerbations, occurring 2 or more times per year. Despite many interventions to reduce COPD exacerbations, there is a significant lack of knowledge in regards to their mechanisms and predisposing factors...
2017: PloS One
https://www.readbyqxmd.com/read/28179509/iga-coated-e-coli-enriched-in-crohn-s-disease-spondyloarthritis-promote-t-h-17-dependent-inflammation
#19
Monica Viladomiu, Charles Kivolowitz, Ahmed Abdulhamid, Belgin Dogan, Daniel Victorio, Jim G Castellanos, Viola Woo, Fei Teng, Nhan L Tran, Andrew Sczesnak, Christina Chai, Myunghoo Kim, Gretchen E Diehl, Nadim J Ajami, Joseph F Petrosino, Xi K Zhou, Sergio Schwartzman, Lisa A Mandl, Meira Abramowitz, Vinita Jacob, Brian Bosworth, Adam Steinlauf, Ellen J Scherl, Hsin-Jung Joyce Wu, Kenneth W Simpson, Randy S Longman
Peripheral spondyloarthritis (SpA) is a common extraintestinal manifestation in patients with active inflammatory bowel disease (IBD) characterized by inflammatory enthesitis, dactylitis, or synovitis of nonaxial joints. However, a mechanistic understanding of the link between intestinal inflammation and SpA has yet to emerge. We evaluated and functionally characterized the fecal microbiome of IBD patients with or without peripheral SpA. Coupling the sorting of immunoglobulin A (IgA)-coated microbiota with 16 S ribosomal RNA-based analysis (IgA-seq) revealed a selective enrichment in IgA-coated Escherichia coli in patients with Crohn's disease-associated SpA (CD-SpA) compared to CD alone...
February 8, 2017: Science Translational Medicine
https://www.readbyqxmd.com/read/28090187/recurrent-intestinal-obstruction-in-a-patient-with-selective-iga-deficiency
#20
Chee-Kin Hui
A 32 year old woman presented with acute onset of abdominal pain and fever. An urgent computerised tomography (CT) of the whole abdomen showed dilated loop at the terminal ileum in the right lower abdomen with thickening of the wall and oedema. The CT was suggestive of distal small bowel obstruction at the ileum with surrounding wall oedema. Multiple biopsies taken from the terminal ileum and colon on colonoscopy were all unremarkable. She represented one-year later with a recurrence of intestinal obstruction...
November 2016: Malaysian Journal of Medical Sciences: MJMS
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