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"iga deficiency"

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
Sabina Chaudhary Hauge, Charlotte Kaestel Jensen, Leif Kofoed Nielsen, Ole B Pedersen, Erik Sørensen, Lise Wegner Thørner, Henrik Hjalgrim, Christian Erikstrup, Kaspar René Nielsen, Kathrine Agergård Kaspersen, Maria Didriksen, Morten Dziegiel, Henrik Ullum
The clinical importance of immunoglobulin A (IgA) deficiency in otherwise healthy individuals is not well described. We aimed to investigate the self-reported mental and physical health and the risk of infection in IgA-deficient blood donors compared to healthy control blood donors. Infectious events, recorded in public health registries either as prescriptions filled of any antimicrobial medicine or as hospital infections, were compared between 177 IgA-deficient blood donors and 1770 control blood donors. A subset of the IgA-deficient donors were further characterized by self-reported health (Short Form-12, n = 28) and circulating C-reactive protein (CRP) (n = 10)...
March 2018: APMIS: Acta Pathologica, Microbiologica, et Immunologica Scandinavica
Adrienne Sullivan, Ruth M Bland, Rosie Hague
Low or absent immunoglobulin A (IgA) levels are frequently found in children in whom immunodeficiency is not suspected. IgA deficiency is the most common primary immunodeficiency disorder in the UK affecting approximately 1 in 600 people. Isolated IgA deficiency is often identified coincidentally when investigating a child for conditions such as coeliac disease. The aim of this article is to provide a structured approach to the history, investigation and management of an isolated IgA deficiency.
February 23, 2018: Archives of Disease in Childhood. Education and Practice Edition
Clare Webb, Alan Norris, Katie Hands
We present the case of a 67-year-old man who suffered an acute anaphylactic reaction during red cell transfusion due to the presence of anti-IgA antibodies. The incidence and clinical relevance of anti-IgA antibodies in IgA deficiency is reviewed, and the wider investigation and management of acute transfusion reactions is also discussed. This case highlights the need to consider the potential risks of blood component transfusion against the purported benefit.
February 2018: Clinical Medicine: Journal of the Royal College of Physicians of London
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
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
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
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
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
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
Thomas R Welch
No abstract text is available yet for this article.
January 2018: Journal of Pediatrics
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
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
Dennis K Ledford
No abstract text is available yet for this article.
September 20, 2017: Journal of Allergy and Clinical Immunology in Practice
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
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
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
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
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
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
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