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Iga deficiency

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https://www.readbyqxmd.com/read/28636500/the-association-of-rs1047763-and-rs1008898-of-c1galt1-with-iga-nephropathy-risk-a-global-meta-analysis
#1
Ruili Nie, Guixue Cheng, Jin Zhang, Yu Dong, Chen Wang, Jianhua Liu, Xiaosong Qin
IgA nephropathy (IgAN) is a globally common primary glomerulonephritis characterized by an elevated level of serum IgA and immune complex deposition in the mesangial area. In the serum of patients with IgAN, the hinge region of IgA1 immunoglobulin contains aberrantly glycosylated O-glycans deficient in galactose, which is normally added to the core 1 O-glycan structure by core 1 synthase, glycoprotein-N-acetylgalactosamine 3-beta-galactosyltransferase 1 (C1GALT1), the key enzyme in the process of glycosylation...
June 2017: Monoclonal Antibodies in Immunodiagnosis and Immunotherapy
https://www.readbyqxmd.com/read/28632732/fructo-oligosaccharides-and-intestinal-barrier-function-in-a-methionine-choline-deficient-mouse-model-of-nonalcoholic-steatohepatitis
#2
Kotaro Matsumoto, Mayuko Ichimura, Koichi Tsuneyama, Yuki Moritoki, Hiromichi Tsunashima, Katsuhisa Omagari, Masumi Hara, Ichiro Yasuda, Hiroshi Miyakawa, Kentaro Kikuchi
Impairments in intestinal barrier function, epithelial mucins, and tight junction proteins have been reported to be associated with nonalcoholic steatohepatitis. Prebiotic fructo-oligosaccharides restore balance in the gastrointestinal microbiome. This study was conducted to determine the effects of dietary fructo-oligosaccharides on intestinal barrier function and steatohepatitis in methionine-choline-deficient mice. Three groups of 12-week-old male C57BL/6J mice were studied for 3 weeks; specifically, mice were fed a methionine-choline-deficient diet, a methionine-choline-deficient diet plus 5% fructo-oligosaccharides in water, or a normal control diet...
2017: PloS One
https://www.readbyqxmd.com/read/28617178/concentrations-of-pneumococcal-iga-and-igm-are-compromised-in-some-individuals-with-antibody-deficiencies
#3
Ainara Echeverría de Carlos, Ricardo Gómez de la Torre, Enrique García Carus, Luis Caminal Montero, Jose Bernardino Díaz López, Hector Suárez Casado, Luis Molinos Matin, Lourdes Tricas Aizpún, Stephen Harding, Antony R Parker
The response to pneumococcal vaccination is assessed by measurement of antigen specific IgG only and is compromised in a number of antibody deficiencies. We measured the concentrations of Pneumococcal IgA and IgM in individuals with both normal and abnormal pneumococcal capsular polysaccharide (PCP) IgG concentrations. A higher number of individuals had abnormal pre vaccination IgA and IgM concentrations below the lower limit of the normal range compared to the control group. Post vaccination a lower number of individuals had IgA and IgM concentrations below the upper limit of the normal range compared to the control group...
June 15, 2017: Journal of Immunoassay & Immunochemistry
https://www.readbyqxmd.com/read/28588580/specific-antibody-deficiency-controversies-in-diagnosis-and-management
#4
REVIEW
Elena Perez, Francisco A Bonilla, Jordan S Orange, Mark Ballow
Specific antibody deficiency (SAD) is a primary immunodeficiency disease characterized by normal immunoglobulins (Igs), IgA, IgM, total IgG, and IgG subclass levels, but with recurrent infection and diminished antibody responses to polysaccharide antigens following vaccination. There is a lack of consensus regarding the diagnosis and treatment of SAD, and its clinical significance is not well understood. Here, we discuss current evidence and challenges regarding the diagnosis and treatment of SAD. SAD is normally diagnosed by determining protective titers in response to the 23-valent pneumococcal polysaccharide vaccine...
2017: Frontiers in 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
#5
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/28552941/ccl20-secreted-from-iga1-stimulated-human-mesangial-cells-recruits-inflammatory-th17-cells-in-iga-nephropathy
#6
Guoyuan Lu, Xiaopan Zhang, Lei Shen, Qing Qiao, Yuan Li, Jieqiong Sun, Jinping Zhang
IgA nephropathy (IgAN) is the most common primary glomerulonephritis characterized by human mesangial cells (HMC) proliferation and extracellular matrix expansion associated with immune deposits consisting of galactose-deficient IgA1. However, how IgA1 contributes to IgAN has yet to be completely elucidated. In this study, the expression profile of chemokines was more altered in IgA1-treated HMC than in the control group. CCL20 was significantly higher either in the serum of IgAN patients or in IgA1-treated HMC...
2017: PloS One
https://www.readbyqxmd.com/read/28552877/gluten-free-flours-from-different-raw-materials-as-the-source-of-vitamin-b1-b2-b3-and-b6
#7
Iga Rybicka, Anna Gliszczynska-Swiglo
Gluten-free (GF) products are those with a natural absence or acceptable level (<20 mg/kg) of gluten. They should be a part of a diet for people with gluten-related disorders, like celiac disease. Recently the popularity of a gluten-free diet (GFD) has risen extremely, because a lot of healthy individuals exclude gluten from their menus. According to the literature data on nutritional deficiencies in the GFD, this trend seems to be risky. This paper describes the nutritional value of 14 flours from different GF raw materials from the aspect of B-group vitamin content (B1, B2, B3, B6)...
2017: Journal of Nutritional Science and Vitaminology
https://www.readbyqxmd.com/read/28551598/novel-variant-of-common-variable-immunodeficiency
#8
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/28527887/mst1-deficiency-promotes-b-cell-responses-by-cd4-t-cell-derived-il-4-resulting-in-hypergammaglobulinemia
#9
Eunchong Park, Myun Soo Kim, Ju Han Song, 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...
July 15, 2017: Biochemical and Biophysical Research Communications
https://www.readbyqxmd.com/read/28500690/tumour-necrosis-factor-receptor-associated-periodic-syndrome-mimicking-chronic-spontaneous-urticaria
#10
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
#11
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
#12
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...
July 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
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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