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Iga deficiency

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https://www.readbyqxmd.com/read/28332870/changes-in-the-level-of-immunoglobulins-and-cd4-cd8-ratio-in-young-and-aged-mice-with-estradiol-deficiency
#1
Ming-Jun Shao, Yi-Jun Zhu, Ying-Er Qiu, Min Hu, Yun-Qin He
Studies demonstrated that deficiency in 17β-estradiol (E2) in postmenopausal women influences their immune system. However, few studies have reported alterations in immunologic presentation during nonnatural menopause in young females. Here we compared the differences in immune response between young C57BL/6N mice with surgical or medical variectomy and aged C57BL/6N mice with the common feature of E2 deficiency following Con A stimulation. We observed inverted CD4/CD8 ratios in the aged group and apparent reduced production of serum immunoglobin (Ig)G, IgA, and IgM in the surgical group, whereas changes in immune parameters in the medical group were moderate...
April 2017: Immunological Investigations
https://www.readbyqxmd.com/read/28331568/lithophagia-as-a-clue-for-celiac-disease-a-case-report-and-literature-review
#2
Mosayeb Shahryar, Iraj Shahramian, Seyed Mohsen Dehghani, NoorMohammad Noori, Maryam Ataollahi
Lithophagia is a type of pica that might be resulted from Iron Deficiency Anemia (IDA) which is the frequent presenting signs of Celiac Disease (CD). A 5-year-old child with a two year history of the lithophagia with a, refractory IDA, abdominal distention and constipation. The child did not grow well and had failure to thrive. With suspicion to CD, TTg IgA level was measured and due to an incearse of TTg IgA level the patients were undergone esophagogastrodeudonoscpy and jejunal biopsy. The biopsy showed severe villous atrophy and an increase in limphoplasma cells...
2017: Gastroenterology and Hepatology From Bed to Bench
https://www.readbyqxmd.com/read/28295247/targeting-c3a-c5a-receptors-inhibits-human-mesangial-cell-proliferation-and-alleviates-iga-nephropathy-in-mice
#3
Ying Zhang, Xianli Yan, Ting Zhao, Qihe Xu, Qi Peng, Ruimin Hu, Songxia Quan, Yali Zhou, Guolan Xing
Complement activation has a deep pathogenic influence in IgA nephropathy (IgAN). C3a and C5a, small cleavage fragments generated by complement activation, are key mediators of inflammation. The fragments exert broad pro-inflammatory effects by binding to specific receptors (C3aR and C5aR, respectively). However, no studies thus far have investigated the effects of C3a, C5a and their receptors on IgAN. We observed that C3aR and C5aR antagonists repressed IgA-induced cell proliferation and IL-6 and MCP-1 production in cultured human mesangial cells (HMCs)...
March 15, 2017: Clinical and Experimental Immunology
https://www.readbyqxmd.com/read/28293726/inflammation-in-iga-nephropathy
#4
Thomas Rauen, Jürgen Floege
Immunoglobulin A nephropathy (IgAN) is the most frequently occurring primary glomerulonephritis in Caucasian and Asian populations. Nonetheless, therapeutic recommendations are based on weak evidence, large controlled trials are scarce and, in particular, the additional value of immunosuppression beyond comprehensive supportive measures is not well-established. The use of immunosuppressants is supported by experimental insights into IgAN pathogenesis that suggest an autoimmune component in disease development...
March 14, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28289675/humoral-immunodeficiency-with-hypotonia-feeding-difficulties-enteropathy-and-mild-eczema-caused-by-a-classical-foxp3-mutation
#5
Paul Tuijnenburg, Eloy Cuadrado, Annet M Bosch, Angelika Kindermann, Machiel H Jansen, Marielle Alders, Ester M M van Leeuwen, Taco W Kuijpers
We describe here the case of a boy who presented with pulmonary infections, feeding difficulties due to velopharyngeal insufficiency and gastroesophageal reflux, myopathy, and hypotonia soon after birth. Later, he was also found to have an elevated immunoglobulin (Ig) E and mild eczema and was diagnosed with inflammatory bowel disease. Further immunological screening at the age of 7 years showed low B and NK cell numbers but normal CD4(+) and CD8(+) T cells and notably, normal numbers of CD4(+) regulatory T (Treg) cells...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/28255003/prognostic-value-of-serum-biomarkers-of-autoimmunity-for-recurrence-of-iga-nephropathy-after-kidney-transplantation
#6
Francois Berthoux, Hitoshi Suzuki, Hesham Mohey, Nicolas Maillard, Christophe Mariat, Jan Novak, Bruce A Julian
A prognostic biomarker for IgA nephropathy (IgAN) recurrence after renal transplant is lacking. We followed 96 consecutive first renal transplant recipients with native kidney IgAN (79 men; 92 deceased donors; mean age =48.1 years old) on calcineurin inhibitor-based immunosuppression over 10 years for death, allograft failure, and clinicopathologic recurrence (CPR; clinically evident and biopsy proven). Using time-dependent Cox regression analysis and receiver operating characteristic curves, we assessed prognostic significance of levels of galactose-deficient IgA1 (Gd-IgA1; autoantigen) and Gd-IgA1-specific IgG and IgA autoantibodies in serum obtained at time of transplant or native kidney IgAN diagnosis (30 patients only)...
March 2, 2017: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/28250126/respiratory-syncytial-virus-rsv-vaccine-vectored-by-a-stable-chimeric-and-replication-deficient-sendai-virus-protects-mice-without-inducing-enhanced-disease
#7
Marian Alexander Wiegand, Gianni Gori-Savellini, Claudia Gandolfo, Guido Papa, Christine Kaufmann, Eva Felder, Alessandro Ginori, Maria Giulia Disanto, Donatella Spina, Maria Grazia Cusi
Respiratory syncytial virus (RSV) is a major cause of severe respiratory infections in children and elderly people and no marketed vaccine exists. In this study, we generated and analyzed a subunit vaccine against RSV based on a novel genome replication-deficient Sendai virus (SeV) vector. We inserted the RSV F protein, known as a genetically stable antigen, into our vector in a specific way to optimize the vaccine features. Exchanging the ectodomain of the SeV F protein with its counterpart from RSV, we created a chimeric vectored vaccine that contains the RSV F protein as an essential structural component...
March 1, 2017: Journal of Virology
https://www.readbyqxmd.com/read/28224999/allograft-inflammatory-factor-1-is-a-regulator-of-transcytosis-in-m-cells
#8
Sari Kishikawa, Shintaro Sato, Satoshi Kaneto, Shigeo Uchino, Shinichi Kohsaka, Seiji Nakamura, Hiroshi Kiyono
M cells in follicle-associated epithelium (FAE) are specialized antigen-sampling cells that take up intestinal luminal antigens. Transcription factor Spi-B regulates M-cell maturation, but the molecules that promote transcytosis within M cells are not fully identified. Here we show that mouse allograft inflammatory factor 1 (Aif1) is expressed by M cells and contributes to M-cell transcytosis. FAE in Aif1(-/-) mice has suppressed uptake of particles and commensal bacteria, compared with wild-type mice. Translocation of Yersinia enterocolitica, but not of Salmonella enterica serovar Typhimurium, leading to the generation of antigen-specific IgA antibodies, is also diminished in Aif1-deficient mice...
February 22, 2017: Nature Communications
https://www.readbyqxmd.com/read/28223728/levothyroxine-therapy-and-impaired-clearance-are-the-strongest-contributors-to-small-intestinal-bacterial-overgrowth-results-of-a-retrospective-cohort-study
#9
Thorsten Brechmann, Andre Sperlbaum, Wolff Schmiegel
AIM: To identify a set of contributors, and weight and rank them on a pathophysiological basis. METHODS: Patients who have undergone a lactulose or glucose hydrogen breath test to rule out small intestinal bacterial overgrowth (SIBO) for various clinical symptoms, including diarrhoea, weight loss, abdominal pain, cramping or bloating, were seen as eligible for inclusion in a retrospective single-centre study. Clinical data such as co-morbidities, medication, laboratory parameters and other possible risk factors have been identified from the electronic data system...
February 7, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/28212436/antibody-deficiency-in-patients-with-frequent-exacerbations-of-chronic-obstructive-pulmonary-disease-copd
#10
Brian N McCullagh, Alejandro P Comellas, Zuhair K Ballas, John D Newell, M Bridget Zimmerman, Antoine E Azar
Chronic Obstructive Pulmonary Disease is the third leading cause of death in the US, and is associated with periodic exacerbations, which account for the largest proportion of health care utilization, and lead to significant morbidity, mortality, and worsening lung function. A subset of patients with COPD have frequent exacerbations, occurring 2 or more times per year. Despite many interventions to reduce COPD exacerbations, there is a significant lack of knowledge in regards to their mechanisms and predisposing factors...
2017: PloS One
https://www.readbyqxmd.com/read/28209808/galactosylation-of-iga1-is-associated-with-common-variation-in-c1galt1
#11
Daniel P Gale, Karen Molyneux, David Wimbury, Patricia Higgins, Adam P Levine, Ben Caplin, Anna Ferlin, Peiran Yin, Christopher P Nelson, Horia Stanescu, Nilesh J Samani, Robert Kleta, Xueqing Yu, Jonathan Barratt
IgA nephropathy (IgAN), an important cause of kidney failure, is characterized by glomerular IgA deposition and is associated with changes in O-glycosylation of the IgA1 molecule. Here, we sought to identify genetic factors contributing to levels of galactose-deficient IgA1 (Gd-IgA1) in white and Chinese populations. Gd-IgA1 levels were elevated in IgAN patients compared with ethnically matched healthy subjects and correlated with evidence of disease progression. White patients with IgAN exhibited significantly higher Gd-IgA1 levels than did Chinese patients...
February 16, 2017: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/28188711/-clinical-symptoms-in-iga-deficiency
#12
Flavio Augusto De Oliveira-Serra, Tainá Mosca, Maria da Conceição Santos de Menezes, Wilma Carvalho-Neves Forte
BACKGROUND: IgA deficiency is the most common primary immunodeficiency. Early diagnosis and clinical follow-up may improve the quality of life of patients with IgA deficiency. To this end, IgA deficiency should be further studied and better understood on its clinical manifestations. OBJECTIVE: To determine IgA deficiency clinical manifestations. METHODS: Cross-sectional, retrospective, exploratory study, where the medical records of 39 patients with IgA deficiency were analyzed...
January 2017: Revista Alergia Mexico: Organo Oficial de la Sociedad Mexicana de Alergia e Inmunología, A.C
https://www.readbyqxmd.com/read/28187132/gwas-for-serum-galactose-deficient-iga1-implicates-critical-genes-of-the-o-glycosylation-pathway
#13
Krzysztof Kiryluk, Yifu Li, Zina Moldoveanu, Hitoshi Suzuki, Colin Reily, Ping Hou, Jingyuan Xie, Nikol Mladkova, Sindhuri Prakash, Clara Fischman, Samantha Shapiro, Robert A LeDesma, Drew Bradbury, Iuliana Ionita-Laza, Frank Eitner, Thomas Rauen, Nicolas Maillard, Francois Berthoux, Jürgen Floege, Nan Chen, Hong Zhang, Francesco Scolari, Robert J Wyatt, Bruce A Julian, Ali G Gharavi, Jan Novak
Aberrant O-glycosylation of serum immunoglobulin A1 (IgA1) represents a heritable pathogenic defect in IgA nephropathy, the most common form of glomerulonephritis worldwide, but specific genetic factors involved in its determination are not known. We performed a quantitative GWAS for serum levels of galactose-deficient IgA1 (Gd-IgA1) in 2,633 subjects of European and East Asian ancestry and discovered two genome-wide significant loci, in C1GALT1 (rs13226913, P = 3.2 x 10-11) and C1GALT1C1 (rs5910940, P = 2...
February 2017: PLoS Genetics
https://www.readbyqxmd.com/read/28179509/iga-coated-e-coli-enriched-in-crohn-s-disease-spondyloarthritis-promote-th17-dependent-inflammation
#14
Monica Viladomiu, Charles Kivolowitz, Ahmed Abdulhamid, Belgin Dogan, Daniel Victorio, Jim G Castellanos, Viola Woo, Fei Teng, Nhan L Tran, Andrew Sczesnak, Christina Chai, Myunghoo Kim, Gretchen E Diehl, Nadim J Ajami, Joseph F Petrosino, Xi K Zhou, Sergio Schwartzman, Lisa A Mandl, Meira Abramowitz, Vinita Jacob, Brian Bosworth, Adam Steinlauf, Ellen J Scherl, Hsin-Jung Joyce Wu, Kenneth W Simpson, Randy S Longman
Peripheral spondyloarthritis (SpA) is a common extraintestinal manifestation in patients with active inflammatory bowel disease (IBD) characterized by inflammatory enthesitis, dactylitis, or synovitis of nonaxial joints. However, a mechanistic understanding of the link between intestinal inflammation and SpA has yet to emerge. We evaluated and functionally characterized the fecal microbiome of IBD patients with or without peripheral SpA. Coupling the sorting of immunoglobulin A (IgA)-coated microbiota with 16S ribosomal RNA-based analysis (IgA-seq) revealed a selective enrichment in IgA-coated Escherichia coli in patients with Crohn's disease-associated SpA (CD-SpA) compared to CD alone...
February 8, 2017: Science Translational Medicine
https://www.readbyqxmd.com/read/28176232/celiac-disease-in-children-with-severe-acute-malnutrition-sam-a-hospital-based-study
#15
Neetu Beniwal, Gaurav Ameta, Chandra Kumar Chahar
OBJECTIVES: To evaluate the prevalence and clinical features of Celiac disease among children with severe acute malnutrition (SAM). METHODS: This prospective observational study was conducted in PBM Children Hospital, Bikaner from July 2012 through December 2013. All consecutively admitted children with SAM were recruited. All subjects were screened for Celiac disease by serological test for IgA-anti tissue Transglutaminase (IgA tTG) antibodies. All seropositive children underwent upper gastrointestinal endoscopy for small bowel biopsy for the confirmation...
February 8, 2017: Indian Journal of Pediatrics
https://www.readbyqxmd.com/read/28168577/determination-of-anti-anisakis-simplex-antibodies-and-relationship-with-%C3%AE-%C3%AE-and-%C3%AE-%C3%AE-lymphocyte-subpopulations-in-patients-with-crohn-s-disease
#16
C Benet-Campos, C Cuéllar, C García-Ballesteros, V Zamora, R Gil-Borrás, I Catalán-Serra, F López-Chuliá, J C Andreu-Ballester
BACKGROUND: The etiology of Crohn's disease (CD) is still unknown although new theories are based on defects in innate immunity. We have previously shown a decrease in γδ T cells in CD patients. Previous studies have shown a high prevalence of anti-A. simplex immunoglobulins in CD patients. The diminution of γδ T cells in the peripheral blood and intestinal mucosa of CD patients may create a state of immunosuppression that would facilitate A. simplex infection. AIMS: To study the antibody responses to Anisakis antigens in Crohn's disease patients and its relationship with αβ and γδ T cell subsets...
February 6, 2017: Digestive Diseases and Sciences
https://www.readbyqxmd.com/read/28162005/the-clinical-significance-of-complete-class-switching-defect-in-ataxia-telangiectasia-patients
#17
Saleh Ghiasy, Leila Parvaneh, Gholamreza Azizi, Ghazal Sadri, Majid Zaki Dizaji, Hassan Abolhassani, Asghar Aghamohammadi
OBJECTIVES: Ataxia telangiectasia (AT) is a primary immunodeficiency associated with recurrent infections. We aimed to investigate clinical and immunological classification in AT patients who suffer from a different spectrum of humoral immune defects. METHODS: AT patients were categorized according to the ability of class switching and patients with hyper IgM (HIgM) profile were defined as class switching defect (CSD). RESULTS: Serum immunoglobulin profile in 66 AT patients showed normal immunoglobulin level (22...
February 4, 2017: Expert Review of Clinical Immunology
https://www.readbyqxmd.com/read/28130498/myeloid-atg16l1-facilitates-host-bacteria-interactions-in-maintaining-intestinal-homeostasis
#18
Hong Zhang, Libo Zheng, Dermot P B McGovern, Ariel M Hamill, Ryan Ichikawa, Yoshitake Kanazawa, Justin Luu, Kotaro Kumagai, Marianne Cilluffo, Masayuki Fukata, Stephan R Targan, David M Underhill, Xiaolan Zhang, David Q Shih
Intact ATG16L1 plays an essential role in Paneth cell function and intestinal homeostasis. However, the functional consequences of ATG16L1 deficiency in myeloid cells, particularly macrophages, are not fully characterized. We generated mice with Atg16l1 deficiency in myeloid and dendritic cells and showed that mice with myeloid Atg16l1 deficiency had exacerbated colitis in two acute and one chronic model of colitis with increased proinflammatory to anti-inflammatory macrophage ratios, production of proinflammatory cytokines, and numbers of IgA-coated intestinal microbes...
March 1, 2017: Journal of Immunology: Official Journal of the American Association of Immunologists
https://www.readbyqxmd.com/read/28090187/recurrent-intestinal-obstruction-in-a-patient-with-selective-iga-deficiency
#19
Chee-Kin Hui
A 32 year old woman presented with acute onset of abdominal pain and fever. An urgent computerised tomography (CT) of the whole abdomen showed dilated loop at the terminal ileum in the right lower abdomen with thickening of the wall and oedema. The CT was suggestive of distal small bowel obstruction at the ileum with surrounding wall oedema. Multiple biopsies taken from the terminal ileum and colon on colonoscopy were all unremarkable. She represented one-year later with a recurrence of intestinal obstruction...
November 2016: Malaysian Journal of Medical Sciences: MJMS
https://www.readbyqxmd.com/read/28079608/mortality-and-differential-diagnoses-of-villous-atrophy-without-coeliac-antibodies
#20
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...
January 11, 2017: European Journal of Gastroenterology & Hepatology
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