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Iga deficiency

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https://www.readbyqxmd.com/read/28527887/mst1-deficiency-promotes-b-cell-responses-by-cd4-t-cell-derived-il-4-resulting-in-hypergammaglobulinemia
#1
Eunchong Park, Myun Soo Kim, Ju Han Song, Kyung Min Cho, Kyung-Hye Roh, Rana Lee, Tae Sung Kim
MST1 deficiency causes T and B cell lymphopenia, resulting in combined immunodeficiency. However, MST1-deficient patients also exhibit autoimmune-like symptoms such as hypergammaglobulinemia and autoantibody production. Recent studies have shown that the autoimmune responses observed in MST1-deficient patients were most likely attributable to defective regulatory T (Treg) cells instead of intrinsic signals in MST1-lacking B cells. Nevertheless, it is not determined how MST1 deficiency in T cells breaks B cell tolerance and causes systemic autoimmune-like phenotypes...
May 17, 2017: Biochemical and Biophysical Research Communications
https://www.readbyqxmd.com/read/28500690/tnf-receptor-associated-periodic-syndrome-traps-mimicking-chronic-spontaneous-urticaria
#2
S Aurich, J C Simon, R Treudler
Tumor necrosis factor (TNF) receptor-associated periodic syndrome (TRAPS) is a rare (about one per million) autosomal dominant autoinflammatory disease being typically characterized by recurrent fever episodes accompanied by variable gastrointestinal, musculoskeletal, neurological, lymphoid, urogenital and skin symptoms (table 1) [1-3]. We report on a 21 year old non atopic male with prediagnosed IgA deficiency, Marfan syndrome and hypothyroidism who first presented in our university allergy centre with three year history of antihistamine resistant, only slightly itching urticarial rash being accompanied by fatigue...
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
#3
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/28487087/recurrent-respiratory-tract-infections-rrti-in-the-elderly-a-late-onset-mild-immunodeficiency
#4
Esther van de Vosse, Monique M van Ostaijen-Ten Dam, René Vermaire, Els M Verhard, Jacqueline L Waaijer, Jaap A Bakker, Sandra T Bernards, Hermann Eibel, Maarten J van Tol, Jaap T van Dissel, Margje H Haverkamp
Elderly with late-onset recurrent respiratory tract infections (RRTI) often have specific anti-polysaccharide antibody deficiency (SPAD). We hypothesized that late-onset RRTI is caused by mild immunodeficiencies, such as SPAD, that remain hidden through adult life. We analyzed seventeen elderly RRTI patients and matched controls. We determined lymphocyte subsets, expression of BAFF receptors, serum immunoglobulins, complement pathways, Pneumovax-23 vaccination response and genetic variations in BAFFR and MBL2...
May 6, 2017: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/28480529/acne-conglobata-in-a-long-term-survivor-with-trisomy-13-accompanied-by-selective-igm-deficiency
#5
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/28472507/a-case-report-of-hypoglycemia-and-hypogammaglobulinemia-david-syndrome-in-a-patient-with-a-novel-nfkb2-mutation
#6
Rayhan A Lal, Laura K Bachrach, Andrew R Hoffman, Jingga Inlora, Shannon Rego, Michael P Snyder, David B Lewis
Context: DAVID syndrome (Deficient Anterior pituitary with Variable Immune Deficiency) is a rare disorder in which children present with symptomatic ACTH deficiency preceded by hypogammaglobulinemia from B-cell dysfunction with recurrent infections, termed common variable immunodeficiency (CVID). Subsequent whole exome sequencing studies have revealed germline heterozygous C-terminal mutations of NFKB2 as either a cause of DAVID syndrome or of CVID without clinical hypopituitarism. However, to the best of our knowledge there have been no cases in which the endocrinopathy has presented in the absence of a prior clinical history of CVID...
May 3, 2017: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28468886/human-sera-collected-during-1979-2010-possess-blocking-antibody-titers-to-pandemic-gii-4-noroviruses-isolated-during-3-decades
#7
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 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 last 4 decades.In total 268 sera were investigated for blocking titers (BT50 values) of virus-like particles (VLPs) to pig gastric mucin (PGM) using 4 VLPs that represent different GII.4 norovirus variants identified between 1987-2012...
May 3, 2017: Journal of Virology
https://www.readbyqxmd.com/read/28466499/implementation-of-national-institute-for-health-and-care-excellence-nice-guidance-to-measure-iga-with-all-coeliac-screens-can-an-affordable-solution-be-devised
#8
Dylan James Mac Lochlainn, Ravina Hira-Kazal, Helen Varney, John Maher
There has been a dramatic increase in requests for coeliac disease (CD) serological screening using IgA 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 since 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...
May 2, 2017: Clinical and Experimental Immunology
https://www.readbyqxmd.com/read/28458713/syndrome-differentiation-of-iga-nephropathy-based-on-clinicopathological-parameters-a-decision-tree-model
#9
Yanghui Gu, Yu Wang, Chunlan Ji, Ping Fan, Zhiren He, Tao Wang, Xusheng Liu, Chuan Zou
Background. IgA nephropathy is the most common cause of primary glomerulonephritis in China, and Traditional Chinese Medicine (TCM) is a vital treatment strategy. However, not all doctors prescribing TCM medicine have adequate knowledge to classify the syndrome accurately. Aim. To explore the feasibility of differentiation of TCM syndrome types among IgA nephropathy patients based on clinicopathological parameters. Materials and Methods. The cross-sectional study enrolled 464 biopsy-proven IgA nephropathy adult patients from 2010 to 2016...
2017: Evidence-based Complementary and Alternative Medicine: ECAM
https://www.readbyqxmd.com/read/28437323/lack-of-utility-of-anti-ttg-igg-to-diagnose-celiac-disease-when-anti-ttg-iga-is-negative
#10
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
https://www.readbyqxmd.com/read/28431078/effect-of-threonine-on-secretory-immune-system-using-a-chicken-intestinal-ex%C3%A2-vivo-model-with-lipopolysaccharide-challenge
#11
Q Zhang, X Chen, S D Eicher, K M Ajuwon, T J Applegate
Secretory IgA (sIgA) and its transcytosis receptor, polymeric immunoglobulin receptor (pIgR), along with mucus, form the first lines of intestinal defense. Threonine (Thr) is a major component of intestinal mucins and IgA, which are highly secreted under lipopolysaccharide (LPS) induced inflammation. In the current study, the effect of Thr on the secretory immune system was determined in an ex vivo chicken ileal explant model. Results showed that a 2-hour Thr-deprivation of culture medium induced a compensatory increase in the mRNA expression of interleukin-8 (IL-8), mucin 2 ( ), and IgA during LPS challenge, and this increase was suppressed with Thr addition to the media ( P  ≤ 0...
April 18, 2017: Poultry Science
https://www.readbyqxmd.com/read/28425252/allergic-and-autoimmune-disorders-in-families-with-selective-iga-deficiency
#12
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/28414063/hypersensitivity-transfusion-reactions-due-to-iga-deficiency-are-rare-according-to-french-hemovigilance-data
#13
Charles Tacquard, Karim Boudjedir, Monique Carlier, Jean-Yves Muller, Philippe Gomis, Paul Michel Mertes
In an analysis of a large French hemovigilance database (2004-2014), hypersensitivity transfusion reactions (HTRs) due to IgA deficiency were extremely rare with an frequency far lower than reported so far. In depth investigation of HTRs for IgA deficiency should be limited to moderate to severe recurrent reactions.
April 13, 2017: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/28413046/heat-stress-decreases-expression-of-the-cytokines-avian-%C3%AE-defensins-4-and-6-and-toll-like-receptor-2-in-broiler-chickens-infected-with-salmonella-enteritidis
#14
W M Quinteiro-Filho, A S Calefi, D S G Cruz, T P A Aloia, A Zager, C S Astolfi-Ferreira, J A Piantino Ferreira, S Sharif, J Palermo-Neto
A high ambient temperature is a highly relevant stressor in poultry production. Heat stress (HS) has been reported to reduce animal welfare, performance indices and increase Salmonella susceptibility. Salmonella spp. are major zoonotic pathogen that cause over 1 billion of human infections worldwide annually. Therefore, the current study was designed to analyze the effect of heat stress on Salmonella infection in chickens through modulation of the immune responses. Salmonella Enteritidis was inoculated via gavage at one day of age (10(6)cfu/mL)...
April 2017: Veterinary Immunology and Immunopathology
https://www.readbyqxmd.com/read/28409678/a-hairline-crack-in-the-levee-focal-secretory-iga-deficiency-as-a-first-step-toward-emphysema
#15
Jeffrey L Curtis
No abstract text is available yet for this article.
April 15, 2017: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/28386703/erratum-to-low-iga-and-igm-is-associated-with-a-higher-prevalence-of-bronchiectasis-in-primary-antibody-deficiency
#16
John P Hodkinson, Catherine Bangs, Andrea Wartenberg-Demand, Artur Bauhofer, Patrick Langohr, Matthew S Buckland, David Guzman, Patrick F K Yong, Sorena Kiani-Alikhan
No abstract text is available yet for this article.
May 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28350935/screening-for-celiac-disease-evidence-report-and-systematic-review-for-the-us-preventive-services-task-force
#17
REVIEW
Roger Chou, Christina Bougatsos, Ian Blazina, Katherine Mackey, Sara Grusing, Shelley Selph
Importance: Silent or subclinical celiac disease may result in potentially avoidable adverse health consequences. Objective: To review the evidence on benefits and harms of screening for celiac disease in asymptomatic adults, adolescents, and children 3 years and older for the US Preventive Services Task Force. Data Sources: Ovid MEDLINE, Cochrane Central Register of Controlled Trials, and Cochrane Database of Systematic Reviews, searched to June 14, 2016...
March 28, 2017: JAMA: the Journal of the American Medical Association
https://www.readbyqxmd.com/read/28350748/short-article-mortality-and-differential-diagnoses-of-villous-atrophy-without-coeliac-antibodies
#18
Annalisa Schiepatti, Federico Biagi, Giacomo Fraternale, Claudia Vattiato, Davide Balduzzi, Simona Agazzi, Claudia Alpini, Catherine Klersy, Gino R Corazza
OBJECTIVE: Villous atrophy (VA) of the small bowel is mainly related to coeliac disease (CD), whose diagnosis is made on the basis of positive endomysial/tissue transglutaminase antibodies while on a gluten-containing diet in the vast majority of patients. However, VA can also occur in other conditions whose epidemiology is little known. Our aim was to study the epidemiology and clinical features of these rare enteropathies. PATIENTS AND METHODS: Clinical and laboratory data of all the patients with VA directly diagnosed in our centre in the last 15 years were collected and statistically analysed...
May 2017: European Journal of Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/28340830/a-novel-biomarker-for-post-transplant-recurrent-iga-nephropathy
#19
S Temurhan, S U Akgul, Y Caliskan, A S Artan, C Kekik, H Yazici, E Demir, B Caliskan, A Turkmen, F S Oguz, M S Sever
BACKGROUND: The serum levels of galactose-deficient immunoglobulin (Ig)A1 (Gd-IgA1) represent the most promising candidate biomarker for IgA nephropathy (IgAN). The aim of this study was to evaluate the serum levels of Gd-IgA1 as a novel noninvasive biomarker for post-transplant IgAN recurrence. METHODS: Serum Gd-IgA1 levels of 18 patients with recurrent IgAN were compared with control renal transplant recipients (n = 23) with non-recurrent IgAN and control non-transplant IgAN patients (n = 44) and healthy relatives (n = 11)...
April 2017: Transplantation Proceedings
https://www.readbyqxmd.com/read/28337237/relationship-between-helicobacter-pylori-infection-and-celiac-disease-a-cross-sectional-study-and-a-brief-review-of-the-literature
#20
Sebahat Basyigit, Oktay Unsal, Metin Uzman, Ferdane Sapmaz, Ozlem Ceylan Dogan, Ayse Kefeli, Zeliha Asilturk, Abdullah Ozgur Yeniova, Yasar Nazligul
INTRODUCTION: Whether Helicobacter pylori triggers celiac disease (CD) or protects against CD is currently the subject of research. In the literature, there are epidemiologic studies that have reported conflicting results regarding the association between H. pylori and CD. AIM: To compare the prevalence of CD autoantibody positivity and the levels of CD autoantibodies between H. pylori-positive and H. pylori-negative subjects. MATERIAL AND METHODS: This study was prospectively designed and included 240 dyspeptic patients who underwent upper gastrointestinal endoscopy with gastric and duodenal biopsies...
2017: Przegla̜d Gastroenterologiczny
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