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"iga deficiency"

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https://www.readbyqxmd.com/read/29335801/igm-augments-complement-bactericidal-activity-with-serum-from-a-patient-with-a-novel-cd79a-mutation
#1
Jeroen D Langereis, Stefanie S Henriet, Saskia Kuipers, Corry M R Weemaes, Mirjam van der Burg, Marien I de Jonge, Michiel van der Flier
Antibody replacement therapy for patients with antibody deficiencies contains only IgG. As a result, concurrent IgM and IgA deficiency present in a large proportion of antibody deficient patients persists. Especially patients with IgM deficiency remain at risk for recurrent infections of the gastrointestinal and respiratory tract. The lack of IgM in the current IgG replacement therapy is likely to contribute to the persistence of these mucosal infections because this antibody class is especially important for complement activation on the mucosal surface...
January 15, 2018: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/29329957/safety-and-tolerability-of-a-novel-polyclonal-human-anti-mers-coronavirus-antibody-produced-from-transchromosomic-cattle-a-phase-1-randomised-double-blind-single-dose-escalation-study
#2
John H Beigel, Jocelyn Voell, Parag Kumar, Kanakatte Raviprakash, Hua Wu, Jin-An Jiao, Eddie Sullivan, Thomas Luke, Richard T Davey
BACKGROUND: Middle East respiratory syndrome (MERS) is a severe respiratory illness with an overall mortality of 35%. There is no licensed or proven treatment. Passive immunotherapy approaches are being developed to prevent and treat several human medical conditions where alternative therapeutic options are absent. We report the safety of a fully human polyclonal IgG antibody (SAB-301) produced from the hyperimmune plasma of transchromosomic cattle immunised with a MERS coronavirus vaccine...
January 9, 2018: Lancet Infectious Diseases
https://www.readbyqxmd.com/read/29302503/celiac-disease-in-south-jordan
#3
Eyad Altamimi
Purpose: Celiac disease, an autoimmune enteropathy triggered by exposure to gluten, is not uncommon in South Jordan. However, its prevalence is underestimated due to lack of physician awareness of the diversity of disease presentation. The clinical spectrum includes classic gastrointestinal manifestations, as well as rickets, iron-deficiency anemia, short stature, elevated liver enzymes, and edema. Our goal was to evaluate celiac disease presentation in clinically diagnosed children. Methods: Retrospective study included all children diagnosed with celiac disease between September 2009 and September 2015...
December 2017: Pediatric Gastroenterology, Hepatology & Nutrition
https://www.readbyqxmd.com/read/29285804/centrifugation-free-washing-a-novel-approach-for-removing-iga-from-stored-red-blood-cells
#4
Eszter Vörös, Nathaniel Z Piety, Briony C Strachan, Madeleine Lu, Sergey S Shevkoplyas
Washed red blood cells (RBCs) are indicated for immunoglobulin A (IgA) deficient recipients. Centrifugation-based cell processors commonly used by hospital blood banks cannot consistently reduce IgA below the recommended levels, hence double washing is frequently required. Here we describe a prototype of a simple, portable, disposable system capable of washing stored RBCs without centrifugation, while reducing IgA below 0.05 mg/dL in a single run. Samples from RBC units (n = 8, leukoreduced, 4-6 weeks storage duration) were diluted with normal saline to a hematocrit of 10%, and then washed using either the prototype washing system, or via conventional centrifugation...
December 29, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/29246330/50-years-ago-in-the-journal-of-pediatrics-comment-on-iga-deficiency-and-susceptibility-to-infection
#5
Thomas R Welch
No abstract text is available yet for this article.
January 2018: Journal of Pediatrics
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/29114388/epistatic-interactions-between-mutations-of-taci-tnfrsf13b-and-tcf3-result-in-a-severe-primary-immunodeficiency-disorder-and-systemic-lupus-erythematosus
#7
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/28941669/increased-ige-in-iga-deficiency
#8
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/28932552/chylothorax-after-pediatric-cardiac-surgery-complicates-short-term-but-not-long-term-outcomes-a-propensity-matched-analysis
#9
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/28774562/defective-anti-polysaccharide-igg-vaccine-responses-in-iga-deficient-mice
#10
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/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/28691461/oral-manifestations-of-selective-iga-deficiency-review-and-case-report
#12
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
#13
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
#14
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/28551598/novel-variant-of-common-variable-immunodeficiency
#15
Eileen Conaway
A 57-year-old woman with frequent respiratory infections was initially diagnosed with IgG subclass deficiency based on low levels of IgG subclasses 2 and 3. Three years later, she progressed to having IgA deficiency as well. With a normal total IgG level, she does not meet criteria for common variable immunodeficiency (CVID). This may represent a variant of CVID. This also highlights the importance of immunoglobulin subclass estimation in patients where immunodeficiency is suspected clinically. She is being treated with rotational antibiotics the first 10 days of every month, monthly intravenous immunoglobulin (IVIG) infusion and osteopathic manipulation one to two times per month...
May 27, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28500690/tumour-necrosis-factor-receptor-associated-periodic-syndrome-mimicking-chronic-spontaneous-urticaria
#16
LETTER
S Aurich, J C Simon, R Treudler
No abstract text is available yet for this article.
May 13, 2017: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/28496534/collagenous-gastritis-in-a-young-female-with-iga-deficiency
#17
Muhammad S Anwar, Ali Aamar, Ali Marhaba, Jagmohan S Sidhu
Collagenous gastritis, without colonic involvement, is exceptionally rare. It is not known to be associated with IgA deficiency and scleroderma. This is the first report of this type of association. We present a 26-year-old white female with a past medical history of gastroesophageal reflux disease and scleroderma. She was evaluated for complaints of abdominal pain and diarrhea. Esophagogastroduodenoscopy showed gastritis and duodenitis. Colonoscopy was normal. The histopathological report showed collagenous gastritis and focal lymphocytic duodenitis...
April 2017: Gastroenterology Research
https://www.readbyqxmd.com/read/28468886/human-sera-collected-between-1979-and-2010-possess-blocking-antibody-titers-to-pandemic-gii-4-noroviruses-isolated-over-three-decades
#18
Sumit Sharma, Beatrice Carlsson, Rita Czakó, Sirkka Vene, Mats Haglund, Johnny Ludvigsson, Göran Larson, Lennart Hammarström, Stanislav V Sosnovtsev, Robert L Atmar, Kim Y Green, Mary K Estes, Lennart Svensson
The emergence of pandemic GII.4 norovirus (NoV) strains has been proposed to occur due to changes in receptor usage and thereby to lead to immune evasion. To address this hypothesis, we measured the ability of human sera collected between 1979 and 2010 to block glycan binding of four pandemic GII.4 noroviruses isolated in the last 4 decades. In total, 268 sera were investigated for 50% blocking titer (BT50) values of virus-like particles (VLPs) against pig gastric mucin (PGM) using 4 VLPs that represent different GII...
July 15, 2017: Journal of Virology
https://www.readbyqxmd.com/read/28466499/implementation-of-national-institute-for-health-and-care-excellence-nice-guidance-to-measure-immunoglobulin-a-with-all-coeliac-screens-can-an-affordable-solution-be-devised
#19
D J Mac Lochlainn, R Hira-Kazal, H Varney, J Maher
There has been a dramatic increase in requests for coeliac disease (CD) serological screening using immunoglobulin (Ig)A tissue transglutaminase antibodies (IgA-tTG). Recently, the UK National Institute for Health and Care Excellence has revised its guidance, recommending that total IgA should also be measured in all samples. This is justified, as false-negative results may occur with IgA deficiency. However, implementation of this guidance will incur considerable expense. Tests that measure IgA-tTG antibodies can detect IgA deficiency, indicated by low background signal...
September 2017: Clinical and Experimental Immunology
https://www.readbyqxmd.com/read/28437323/lack-of-utility-of-anti-ttg-igg-to-diagnose-celiac-disease-when-anti-ttg-iga-is-negative
#20
Imad Absah, Abdul R Rishi, Rami Gebrail, Melissa R Snyder, Joseph A Murray
OBJECTIVES: Guidelines for diagnosing celiac disease (CD) recommend initial testing with a highly sensitive serologic test for anti-tissue transglutaminase immunoglobulin A antibodies (tTG IgA). When the probability of CD is high, IgA deficiency should be considered. The 2 approaches to address this include measuring "both tTG IgA and tTG IgG" or measuring "total IgA." We aim to assess the utility of an isolated positive tTG IgG result in diagnosing CD. METHODS: We conducted a retrospective review of patients undergoing serologic testing for CD from January 1997 to June 2014...
May 2017: Journal of Pediatric Gastroenterology and Nutrition
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