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Iga deficiency

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https://www.readbyqxmd.com/read/27920422/population-based-screening-for-selective-immunoglobulin-a-iga-deficiency-in-lithuanian-children-using-a-rapid-antibody-based-fingertip-test
#1
Vaidotas Urbonas, Jolita Sadauskaite, Rimante Cerkauskiene, Arvydas Kaminskas, Markku Mäki, Kalle Kurppa
BACKGROUND Selective immunoglobulin A (IgA) deficiency is the most common inherited immunodeficiency disorder worldwide. An early diagnosis is advocated because of the increased risk of infections, autoimmune diseases, and allergic reactions. We investigated the usefulness of a rapid point-of-care test in detecting for IgA deficiency in a population with a previously unknown prevalence. MATERIAL AND METHODS Altogether, 1000 children aged 11-13 years from randomly selected Lithuanian schools were enrolled. A point-of-care test with a fingertip sample was used to screen for the presence of IgA deficiency in children whose parents gave consent...
December 6, 2016: Medical Science Monitor: International Medical Journal of Experimental and Clinical Research
https://www.readbyqxmd.com/read/27920152/toll-like-receptor-9-stimulation-induces-aberrant-expression-of-a-proliferation-inducing-ligand-by-tonsillar-germinal-center-b-cells-in-iga-nephropathy
#2
Masahiro Muto, Benoit Manfroi, Hitoshi Suzuki, Kensuke Joh, Masaaki Nagai, Sachiko Wakai, Christian Righini, Masayuki Maiguma, Shozo Izui, Yasuhiko Tomino, Bertrand Huard, Yusuke Suzuki
The TNF family member a proliferation-inducing ligand (APRIL; also known as TNFSF13), produced by myeloid cells, participates in the generation and survival of antibody-producing plasma cells. We studied the potential role of APRIL in the pathogenesis of IgA nephropathy (IgAN). We found that a significant proportion of germinal centers (GCs) in tonsils of patients with IgAN contained cells aberrantly producing APRIL, contributing to an overall upregulation of tonsillar APRIL expression compared with that in tonsils of control patients with tonsillitis...
December 5, 2016: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/27898728/relationships-between-mucosal-antibodies-non-typeable-haemophilus-influenzae-nthi-infection-and-airway-inflammation-in-copd
#3
Karl J Staples, Stephen Taylor, Steve Thomas, Stephanie Leung, Karen Cox, Thierry G Pascal, Kristoffer Ostridge, Lindsay Welch, Andrew C Tuck, Stuart C Clarke, Andrew Gorringe, Tom M A Wilkinson
Non-typeable Haemophilus influenzae (NTHi) is a key pathogen in COPD, being associated with airway inflammation and risk of exacerbation. Why some patients are susceptible to colonisation is not understood. We hypothesised that this susceptibility may be due to a deficiency in mucosal humoral immunity. The aim of our study (NCT01701869) was to quantify the amount and specificity of antibodies against NTHi in the lungs and the associated risk of infection and inflammation in health and COPD. Phlebotomy, sputum induction and bronchoscopy were performed on 24 mild-to-moderate COPD patients and 8 age and smoking-matched controls...
2016: PloS One
https://www.readbyqxmd.com/read/27895168/cx3cr1-cell-mediated-salmonella-exclusion-protects-the-intestinal-mucosa-during-the-initial-stage-of-infection
#4
Angela L Man, Nadezhda Gicheva, Mari Regoli, Gary Rowley, Giovanna De Cunto, Nikolaus Wellner, Elizabeth Bassity, Massimo Gulisano, Eugenio Bertelli, Claudio Nicoletti
During Salmonella Typhimurium infection, intestinal CX3CR1(+) cells can either extend transepithelial cellular processes to sample luminal bacteria or, very early after infection, migrate into the intestinal lumen to capture bacteria. However, until now, the biological relevance of the intraluminal migration of CX3CR1(+) cells remained to be determined. We addressed this by using a combination of mouse strains differing in their ability to carry out CX3CR1-mediated sampling and intraluminal migration. We observed that the number of S...
November 28, 2016: Journal of Immunology: Official Journal of the American Association of Immunologists
https://www.readbyqxmd.com/read/27893677/impact-of-iron-deficiency-anemia-on-the-function-of-the-immune-system-in-children
#5
Tamer Hasan Hassan, Mohamed Ahmed Badr, Nehad Ahmed Karam, Marwa Zkaria, Hosam Fathy El Saadany, Doaa Mohamed Abdel Rahman, Doaa Abdallah Shahbah, Salah Mohamed Al Morshedy, Manar Fathy, Asmaa Mohamed Hosni Esh, Amal Mohamed Selim
The importance of iron deficiency as a public health problem is based ultimately on the seriousness of its consequences on health. The most extensively investigated consequences of iron deficiency involve work performance and immune function. The significance of the effects on work performance is generally accepted. In contrast, data on the influence of iron deficiency on immune function are often perceived as being confusing and contradictory.We aimed to evaluate the effect of iron deficiency anemia on humoral, cellular, nonspecific immunity, and also the effect on the cytokines that are the key factors of many immunologic steps...
November 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27889723/vitamin-d-supplementation-for-treatment-and-prevention-of-pneumonia-in-under-five-children-a-randomized-double-blind-placebo-controlled-trial
#6
Piyush Gupta, Pooja Dewan, Dheeraj Shah, Nisha Sharma, Nidhi Bedi, Iqbal R Kaur, Ajay Kumar Bansal, S V Madhu
OBJECTIVE: To evaluate the efficacy of single oral mega-dose of Vitamin D3 for treatment and prevention of pneumonia in under-five children. DESIGN: Randomized, double blind, placebo-controlled trial. SETTING: Tertiary-care hospital. PARTICIPANTS: 324 children (of 980 assessed) between 6 mo-5 y age (median (IQR): 12 (7,19.8) mo) with WHO-defined severe pneumonia. Of these, 126 (39%) were vitamin D deficient (serum 25(OH)D <12 ng/mL)...
November 15, 2016: Indian Pediatrics
https://www.readbyqxmd.com/read/27886121/sodium-butyrate-reduces-colitogenic-immunoglobulin-a-coated-bacteria-and-modifies-the-composition-of-microbiota-in-il-10-deficient-mice
#7
Tenghui Zhang, Chao Ding, Mingli Zhao, Xujie Dai, Jianbo Yang, Yi Li, Lili Gu, Yao Wei, Jianfeng Gong, Weiming Zhu, Ning Li, Jieshou Li
High levels of immunoglobulin A (IgA)-coated bacteria may have a role in driving inflammatory bowel disease (IBD). We therefore investigated the effect of sodium butyrate on microbiota in IBD prone interleukin (IL)-10(-/-) mice. At 8 weeks of age, mice were allocated into three groups (n = 4/group): normal (C57BL/6), IL-10(-/-), and IL-10(-/-) treated with sodium butyrate (100 mM). Severity of colitis, inflammatory cytokine and short-chain fatty acid (SCFA) concentration in proximal colon contents, the percentage of IgA-coated bacteria and microbiota composition by 16S ribosomal RNA assessment of stool were measured after 4 weeks of treatment...
November 24, 2016: Nutrients
https://www.readbyqxmd.com/read/27884168/ataxia-telangiectasia-a-review
#8
REVIEW
Cynthia Rothblum-Oviatt, Jennifer Wright, Maureen A Lefton-Greif, Sharon A McGrath-Morrow, Thomas O Crawford, Howard M Lederman
DEFINITION OF THE DISEASE: Ataxia telangiectasia (A-T) is an autosomal recessive disorder primarily characterized by cerebellar degeneration, telangiectasia, immunodeficiency, cancer susceptibility and radiation sensitivity. A-T is often referred to as a genome instability or DNA damage response syndrome. EPIDEMIOLOGY: The world-wide prevalence of A-T is estimated to be between 1 in 40,000 and 1 in 100,000 live births. CLINICAL DESCRIPTION: A-T is a complex disorder with substantial variability in the severity of features between affected individuals, and at different ages...
November 25, 2016: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/27870872/aberrant-iga1-glycosylation-in-iga-nephropathy-a-systematic-review
#9
Qiang Sun, Zhenhai Zhang, Hong Zhang, Xiaorong Liu
OBJECTIVE: Galactose-deficient IgA1 was evaluated in patients with IgA nephropathy(IgAN) and controls in order to determine the predictive value of galactose-deficient IgA1 in cases of IgA nephropathy. METHODS: PubMed, EMBASE, Cochrane central register of controlled trials, CNKI, CBM disc, and VIP database were searched to identify eligible studies that evaluated a difference in aberrant IgA1 glycosylation in IgAN patients compared with controls. A meta-analysis was conducted to evaluate the impact of galactose-deficient IgA1(Gd-IgA1) levels in different groups...
2016: PloS One
https://www.readbyqxmd.com/read/27858203/enhanced-fucosylation-of-ga1-in-the-digestive-tracts-of-x-ray-irradiated-mice
#10
Masao Iwamori, Kyoko Tanaka, Shigeki Adachi, Daisuke Aoki, Taisei Nomura
In mice at 4 days after X-ray-irradiation at 0.5 Gy/min for 16 min, the tissue weights of immune organs, i.e., thymus and spleen, were decreased due to injury to lymphocytes by the X-rays. The resulting immunosuppressive condition allowed the growth of lactobacilli, i.e., L. murinus, which contained LacβTH-DG and possessed the ability to induce transcription of the fucosyltransferase gene for synthesis of FGA1. LacβTH-DG was detected in the jejunal and ileal contents of X-ray-irradiated mice, but not in those of control mice, whereas LacTetH-DG of L...
November 18, 2016: Glycoconjugate Journal
https://www.readbyqxmd.com/read/27853628/tir-domain-containing-adapter-inducing-interferon-%C3%AE-trif-regulates-cxcr5-t-helper-cells-in-the-intestine
#11
Saravana Kanagavelu, Claudia Flores, Shinichiro Hagiwara, Jose Ruiz, Jinhee Hyun, Ei E Cho, Frank Sun, Laura Romero, David Q Shih, Masayuki Fukata
OBJECTIVE: Establishing an effective humoral immunity is an important host defense mechanism in intestinal mucosa. T follicular helper (Tfh) cells are a spectrum of CXCR5 expressing T helper cells that induce antigen-specific B cell differentiation. Because the differentiation of T helper cells is largely regulated by innate immunity, we addressed whether TRIF signaling regulates Tfh cell differentiation and its ability to trigger humoral immune responses in the intestine. METHOD: CD4(+)CXCR5(+) T cells, B cells, and plasma cells in the Peyer's patches (PPs) of WT and TRIF-deficient (Trif(LPS2)) mice were analyzed by flow cytometry at the baseline, 9 days post primary infection, and 7 days post-secondary infection with Y...
October 2016: Journal of Clinical & Cellular Immunology
https://www.readbyqxmd.com/read/27821627/a-randomized-controlled-trial-of-rituximab-in-iga-nephropathy-with-proteinuria-and-renal-dysfunction
#12
Richard A Lafayette, Pietro A Canetta, Brad H Rovin, Gerald B Appel, Jan Novak, Karl A Nath, Sanjeev Sethi, James A Tumlin, Kshama Mehta, Marie Hogan, Stephen Erickson, Bruce A Julian, Nelson Leung, Felicity T Enders, Rhubell Brown, Barbora Knoppova, Stacy Hall, Fernando C Fervenza
IgA nephropathy frequently leads to progressive CKD. Although interest surrounds use of immunosuppressive agents added to standard therapy, several recent studies have questioned efficacy of these agents. Depleting antibody-producing B cells potentially offers a new therapy. In this open label, multicenter study conducted over 1-year follow-up, we randomized 34 adult patients with biopsy-proven IgA nephropathy and proteinuria >1 g/d, maintained on angiotensin-converting enzyme inhibitors or angiotensin receptor blockers with well controlled BP and eGFR<90 ml/min per 1...
November 7, 2016: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/27806690/vitamin-d-deficiency-may-predict-a-poorer-outcome-of-iga-nephropathy
#13
Xiao-Hua Li, Xin-Ping Huang, Ling Pan, Cheng-Yu Wang, Ju Qin, Feng-Wei Nong, Yu-Zhen Luo, Yue Wu, Yu-Ming Huang, Xi Peng, Zhen-Hua Yang, Yun-Hua Liao
BACKGROUND: Experimental studies showed that 25-hydroxy-vitamin D [25(OH)D] deficiency (defined as 25-hydroxy-vitamin D < 15 ng/ml) has been associated with CKD progression. Patients with IgA nephropathy have an exceptionally high rate of severe 25(OH)D deficiency; however, it is not known whether this deficiency is a risk factor for progression of IgA nephropathy. We conducted this study to investigate the relationship between the plasma level of 25(OH)D and certain clinical parameters and renal histologic lesions in the patients with IgA nephropathy, and to evaluate whether the 25(OH)D level could be a good prognostic marker for IgA nephropathy progression...
November 2, 2016: BMC Nephrology
https://www.readbyqxmd.com/read/27805618/eosinophils-are-required-to-suppress-th2-responses-in-peyer-s-patches-during-intestinal-infection-by-nematodes
#14
J Strandmark, S Steinfelder, C Berek, A A Kühl, S Rausch, S Hartmann
Infections with enteric nematodes result in systemic type 2 helper T (Th2) responses, expansion of immunoglobulin (Ig)G1 antibodies, and eosinophilia. Eosinophils have a supportive role in mucosal Th2 induction during airway hyperreactivity. Whether eosinophils affect the local T-cell and antibody response in the gut-associated lymphoid tissue during enteric infections is unknown. We infected eosinophil-deficient ΔdblGATA-1 mice with the Th2-inducing small intestinal nematode Heligmosomoides polygyrus and found that parasite fecundity was decreased in the absence of eosinophils...
November 2, 2016: Mucosal Immunology
https://www.readbyqxmd.com/read/27800605/b-cell-activation-with-cd40l-or-cpg-measures-the-function-of-b-cell-subsets-and-identifies-specific-defects-in-immunodeficient-patients
#15
Emiliano Marasco, Chiara Farroni, Simona Cascioli, Valentina Marcellini, Marco Scarsella, Ezio Giorda, Eva Piano Mortari, Lucia Leonardi, Alessia Scarselli, Diletta Valentini, Caterina Cancrini, Marzia Duse, Ola Grimsholm, Rita Carsetti
Around 65% of primary immunodeficiencies are antibody deficiencies. Functional tests are useful tools to study B-cell functions in vitro. However, no accepted guidelines for performing and evaluating functional tests have been issued yet. Here, we report our experience on the study of B-cell functions in infancy and throughout childhood. We show that T-independent stimulation with CpG measures proliferation and differentiation potential of memory B cells. Switched memory B cells respond better than IgM memory B cells...
November 1, 2016: European Journal of Immunology
https://www.readbyqxmd.com/read/27799660/toward-noninvasive-diagnosis-of-iga-nephropathy-a-pilot-urinary-metabolomic-and-proteomic-study
#16
Michaela Neprasova, Dita Maixnerova, Jan Novak, Colin Reily, Bruce A Julian, Jan Boron, Petr Novotny, Miloslav Suchanek, Vladimir Tesar, Petr Kacer
IgA nephropathy is diagnosed by renal biopsy, an invasive procedure with a risk of significant complications. Noninvasive approaches are needed for possible diagnostic purposes and especially for monitoring disease activity or responses to treatment. In this pilot project, we assessed the utility of urine samples as source of biomarkers of IgA nephropathy. We used spot urine specimens from 19 healthy controls, 11 patients with IgA nephropathy, and 8 renal-disease controls collected on day of renal biopsy. Urine samples were analyzed using untargeted metabolomic and targeted proteomic analyses by several experimental techniques: liquid chromatography coupled with mass spectrometry, immunomagnetic isolation of target proteins coupled with quantitation by mass spectrometry, and protein arrays...
2016: Disease Markers
https://www.readbyqxmd.com/read/27795213/-clinic-of-humoral-primary-immunodeficiencies-in-adults-experience-in-a-tertiary-hospital
#17
Julio César Cambray-Gutiérrez, Diana Andrea Herrera-Sánchez, Lizbeth Blancas-Galicia, Patricia María O'Farrill-Romanillos
BACKGROUND: Primary immunodeficiencies (PID) are characterized by alteration of the components of the immune system. Humoral deficiencies represent 50%. The most common are selective IgA deficiency, Bruton agammaglobulinemia, and common variable immunodeficiency (CVID). OBJECTIVE: To describe the epidemiological and clinical characteristics of adults with humoral PID, cared for in a Primary Humoral Immunodeficiencies Clinic. METHODS: A descriptive cross-sectional study that included a year of analysis, including 35 patients with humoral PID, 31 with CVID, and 4 with Bruton agammaglobulinemia...
October 2016: Revista Alergia Mexico: Organo Oficial de la Sociedad Mexicana de Alergia e Inmunología, A.C
https://www.readbyqxmd.com/read/27790582/salivary-iga-versus-hiv-and-dental-caries
#18
Pradip Kumar Mandal, Malay Mitra, Sonu Acharya, Chiranjit Ghosh, Susant Mohanty, Subrata Saha
INTRODUCTION: The inter-relationship of Human Immunodeficiency Virus (HIV) infection and dental caries as well as Salivary Immunoglobulin-A (S-IgA) level appear to remain under explored while a manual and electronic search of the literature was made. Hence, the present study was undertaken to assess the relationship of S-IgA and dental caries status in HIV positive children. AIM: The aim of this study was to find out the relationship of S-IgA antibody with dental caries by measuring the concentration of IgA in saliva of HIV positive and negative children and determine the dental caries status in HIV positive and HIV negative children, which may help in treatment planning and prevention of the same...
September 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/27789066/novel-aicda-mutation-in-a-case-of-autosomal-recessive-hyper-igm-syndrome-growth-hormone-deficiency-and-autoimmunity
#19
A Fazel, S Kashef, S Aleyasin, S Harsini, Z Karamizadeh, S Zoghi, S K Flores, K Boztug, N Rezaei
BACKGROUND: The Hyper-immunoglobulin M syndromes (HIGM) are a heterogeneous group of genetic disorders, which have been rarely reported to be associated with growth hormone deficiency (GHD). METHODS AND RESULTS: A nine-year-old girl with recurrent urinary tract infections, diarrhoea, sinopulmonary infections, and failure to thrive since the age of six months had normal CD3+, CD4+, CD8+T lymphocytes, and CD19+B lymphocytes and natural killer (NK) cells, but extremely elevated IgM and significantly decreased IgG and IgA...
October 24, 2016: Allergologia et Immunopathologia
https://www.readbyqxmd.com/read/27776452/the-long-and-winding-road-to-iga-deficiency-causes-and-consequences
#20
D T Laura Vo Ngoc, Lizette Krist, Frans J van Overveld, Ger T Rijkers
The most common humoral immunodeficiency is IgA deficiency. One of the first papers addressing the cellular and molecular mechanisms underlying IgA deficiency indicated that immature IgA-positive B-lymphocytes are present in these patients. This suggests that the genetic background for IgA is still intact and that class switching can take place. At this moment, it cannot be ruled out that genetic as well as environmental factors are involved. Areas covered: A clinical presentation, the biological functions of IgA, and the management of IgA deficiency are reviewed...
October 25, 2016: Expert Review of Clinical Immunology
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