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Iga deficiency

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https://www.readbyqxmd.com/read/29679061/the-role-of-macrophage-migration-inhibitory-factor-in-the-function-of-intestinal-barrier
#1
Milica Vujicic, Tamara Saksida, Sanja Despotovic, Svetlana Sokovic Bajic, Ivana Lalić, Ivan Koprivica, Dragica Gajic, Natasa Golic, Maja Tolinacki, Ivana Stojanovic
Macrophage migration inhibitory factor (MIF) is a multifunctional protein that is involved in the development of gut-related inflammation. To investigate the role of MIF in the function of the intestinal barrier, we have explored intestinal permeability and gut-associated immune response in MIF-deficient (MIF-KO) mice. The absence of MIF provoked impairment of tight and adherens epithelial junctions in the colon through the disturbance of E-cadherin, zonula occludens-1, occludin and claudin-2 expression, which lead to the increase of intestinal barrier permeability...
April 20, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29671115/utility-of-dna-rna-protein-and-functional-approaches-to-solve-cryptic-immunodeficiencies
#2
Margot A Cousin, Matthew J Smith, Ashley N Sigafoos, Jay J Jin, Marine I Murphree, Nicole J Boczek, Patrick R Blackburn, Gavin R Oliver, Ross A Aleff, Karl J Clark, Eric D Wieben, Avni Y Joshi, Pavel N Pichurin, Roshini S Abraham, Eric W Klee
PURPOSE: We report a female infant identified by newborn screening for severe combined immunodeficiencies (NBS SCID) with T cell lymphopenia (TCL). The patient had persistently elevated alpha-fetoprotein (AFP) with IgA deficiency, and elevated IgM. Gene sequencing for a SCID panel was uninformative. We sought to determine the cause of the immunodeficiency in this infant. METHODS: We performed whole-exome sequencing (WES) on the patient and parents to identify a genetic diagnosis...
April 18, 2018: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/29659363/-late-diagnosis-of-whim-sydrome
#3
María V Paolini, Silvia Danielian, Emma Prieto, María Fernanda Tami, Matías M Oleastro, Diego S Fernández Romero
WHIM syndrome is a primary autosomal dominant immuno deficiency due to CXCR4 mutations characterized by mucocutaneous warts, hypogammaglobulinemia, recurrent bacterial infections and myelokathesis. Treatment consists in prophylactic antibiotics, immunoglobulin replacement and granulocyte or granulocyte/monocyte colony stimulating factors. We present the case of a 21 year old woman who showed leukopenia at 10 months of age and one year later multiple infections with hypogammaglobulinemia requiring intravenous immunoglobulin...
2018: Medicina
https://www.readbyqxmd.com/read/29651973/the-clinical-and-immunological-features-of-patients-with-primary-antibody-deficiencies
#4
Gholamreza Azizi, Yasser Bagheri, Marzieh Tavakol, Forough Askarimoghaddam, Kiyoomars Poorrostami, Hosein Rafiemanesh, Reza Yazdani, Fatemeh Kiaee, Sima Habibi, Kosar Abouhamzeh, Hamed Mohammadi, Mostafa Qorbani, Hassan Abolhassani, Asghar Aghamohammadi
BACKGROUND: Primary antibody deficiency (PAD) comprises a range of diseases from early to late terminal B cells defects and is associated with the various clinical complications. METHODS: A total of 461 patients (311 males and 150 females) with PADs enrolled in the retrospective cohort study and for all patients' demographic information, clinical records and laboratory data were collected to investigate clinical complications. RESULTS: The most prevalent first presentations of immunodeficiency were respiratory tract infections in 63...
April 12, 2018: Endocrine, Metabolic & Immune Disorders Drug Targets
https://www.readbyqxmd.com/read/29649230/lower-serum-iga-is-associated-with-copd-exacerbation-risk-in-spiromics
#5
Nirupama Putcha, Gabriel G Paul, Antoine Azar, Robert A Wise, Wanda K O'Neal, Mark T Dransfield, Prescott G Woodruff, Jeffrey L Curtis, Alejandro P Comellas, M Bradley Drummond, Allison A Lambert, Laura M Paulin, Ashraf Fawzy, Richard E Kanner, Robert Paine, MeiLan K Han, Fernando J Martinez, Russell P Bowler, R Graham Barr, Nadia N Hansel
BACKGROUND: Decreased but measurable serum IgA levels (≤70 mg/dL) have been associated with risk for infections in some populations, but are unstudied in COPD. This study tested the hypothesis that subnormal serum IgA levels would be associated with exacerbation risk in COPD. METHODS: Data were analyzed from 1,049 COPD participants from the observational cohort study SPIROMICS (535 (51%) women; mean age 66.1 (SD 7.8), 338 (32%) current smokers) who had baseline serum IgA measured using the Myriad RBM biomarker discovery platform...
2018: PloS One
https://www.readbyqxmd.com/read/29623817/restless-legs-syndrome-in-patients-infected-with-helicobacter-pylori
#6
Farzaneh Rezvani, Mohammad Sayadnasiri, Omid Rezaei
Objective Although the etiology of restless legs syndrome (RLS) is yet to be discovered, some studies have mentioned the effect of infectious diseases in occurrence of RLS. This study aims to determine the association between common Helicobacter pylori (H. pylori) infection and occurrence of RLS. Methods In this descriptive analytical cross-sectional study, patients with RLS who were referred to the Internal Medicine Clinic of Fayazbakhsh Hospital in Tehran from September to December 2016 were evaluated. Patients with underlying diseases that would lead to secondary RLS were excluded from the study...
April 6, 2018: Neurological Research
https://www.readbyqxmd.com/read/29619862/characteristics-of-patients-with-coexisting-iga-nephropathy-and-membranous-nephropathy
#7
Pei Chen, Su-Fang Shi, Zhen Qu, Na Zhao, Xin-Fang Xie, Ji-Cheng Lv, Li-Jun Liu, Hong Zhang
BACKGROUND: Coexistence of IgA nephropathy (IgAN) and membranous nephropathy (MN) in the same patient is rare. Few studies have reported the clinical and pathological features of patients with combined IgAN and MN (IgAN-MN). METHODS: The clinico-pathological features, levels of galactose-deficient IgA1 (Gd-IgA1) and autoantibodies against M-type transmembrane phospholipase A2 receptor (anti-PLA2 R) in sera were compared among IgAN-MN, IgAN, and MN patients. RESULTS: Twenty-six patients with biopsy-proven IgAN-MN were enrolled...
November 2018: Renal Failure
https://www.readbyqxmd.com/read/29606255/recent-advances-in-the-physiopathology-of-iga-nephropathy
#8
Renato C Monteiro
Immunoglobulin A nephropathy or Berger's disease is the most common type of primary glomerulonephritis, which is characterized by IgA1-containing immune-deposits in the glomerular mesangium. Microscopic haematuria and proteinuria are the most common presentations. Mesangial cell proliferation with IgA deposition is found on renal biopsy. Mechanims of the disease implicate at least four key molecules have been implicated in immune complex formation: galactose-deficient IgA1, autoantibodies anti-galactose-deficient-IgA1, soluble CD89 (Fc receptor for IgA) and the CD71 mesangial IgA receptor (transferrin receptor)...
April 2018: Néphrologie & Thérapeutique
https://www.readbyqxmd.com/read/29594459/iron-storage-disease-hemochromatosis-and-hepcidin-response-to-iron-load-in-two-species-of-pteropodid-fruit-bats-relative-to-the-common-vampire-bat
#9
Iga M Stasiak, Dale A Smith, Tomas Ganz, Graham J Crawshaw, Jutta D Hammermueller, Dorothee Bienzle, Brandon N Lillie
Hepcidin is the key regulator of iron homeostasis in the body. Iron storage disease (hemochromatosis) is a frequent cause of liver disease and mortality in captive Egyptian fruit bats (Rousettus aegyptiacus), but reasons underlying this condition are unknown. Hereditary hemochromatosis in humans is due to deficiency of hepcidin or resistance to the action of hepcidin. Here, we investigated the role of hepcidin in iron metabolism in one species of pteropodid bat that is prone to iron storage disease [Egyptian fruit bat (with and without hemochromatosis)], one species of pteropodid bat where iron storage disease is rare [straw-colored fruit bat (Eidolon helvum)], and one species of bat with a natural diet very high in iron, in which iron storage disease is not reported [common vampire bat (Desmodus rotundus)]...
March 29, 2018: Journal of Comparative Physiology. B, Biochemical, Systemic, and Environmental Physiology
https://www.readbyqxmd.com/read/29567905/diminished-cd40l-expression-on-t-cells-in-a-case-of-disseminated-cryptococcosis
#10
Supreet Kumar Mohanty, Deepshi Thakral, Devika Gupta, Prabin Kumar, Dipendra Kumar Mitra
X-linked hyperimmunoglobulin M (HIGM) syndrome may increase the susceptibility of patients to disseminated cryptococcal infections primarily due to CD40L deficiency that causes defective cross talk between T- and B-cells, thus preventing class switching. In HIGM syndrome, serum IgM levels are elevated with severe reduction in serum immunoglobulin G (IgG) and IgA levels. In addition, the expression of CD40L (CD154) on in vitro-activated T-cells is severely reduced or absent. Here, we describe a rare, and perhaps, the first reported case in India of a 3-year-old male child with X-linked HIGM immunodeficiency syndrome who developed disseminated Cryptococcosis...
January 2018: Indian Journal of Pathology & Microbiology
https://www.readbyqxmd.com/read/29562371/the-frequency-of-celiac-disease-in-children-with-autoimmune-thyroiditis
#11
Y Sahin, O Evliyaoglu, T Erkan, F C Cokugras, O Ercan, T Kutlu
BACKGROUND AND AIMS: Although the presence of autoimmune thyroiditis (AT) in celiac disease (CD) has been well documented among adults, CD in AT has been less reported in children. We aimed to investigate the frequency of CD in children with AT. Materials and Methods : This prospective study was carried out from October 2015 to August 2016 and included 66 patients with AT. Firstly, total IgA and tissue transglutaminase antibody (tTG) IgA levels were measured. Those with increased level of tTG IgA were tested for anti-endomysium IgA antibodies (EMA)...
January 2018: Acta Gastro-enterologica Belgica
https://www.readbyqxmd.com/read/29561985/review-of-celiac-disease-presentation-in-a-pediatric-tertiary-centre
#12
Gracinda Nogueira Oliveira, Rajiv Mohan, Andrew Fagbemi
BACKGROUND: Celiac disease is an immune-mediated disorder with a multiform presentation and therefore a challenging diagnosis. OBJECTIVE: Our purpose is to identify the epidemiological, clinical, laboratory and histologic characteristics of children with celiac disease at diagnosis and on follow-up. METHODS: Children with previously established or newly diagnosed celiac disease, admitted in a tertiary centre in a two-year period (2014-2016) were recruited...
January 2018: Arquivos de Gastroenterologia
https://www.readbyqxmd.com/read/29561521/fdg-avid-focal-liver-reaction-from-proton-therapy-in-a-patient-with-primary-esophageal-adenocarcinoma
#13
Hena S Ahmed, Austin R Pantel, James M Metz, John P Plastaras, Michael D Farwell
A 25-year-old man with IgA deficiency was treated with 2 months of chemotherapy and proton therapy for gastroesophageal junction adenocarcinoma. Restaging PET/CT 18 days posttherapy demonstrated 2 new foci of increased FDG uptake in the left hepatic lobe, which were favored to represent radiation injury as opposed to new metastases. Follow-up MRI with contrast 2 weeks later demonstrated hypoenhancement and T1/T2 hypointensity in the liver, without restricted diffusion, which correlated with the dominant FDG-avid focus...
March 20, 2018: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/29551516/interleukin-6-is-essential-for-glomerular-immunoglobulin-a-deposition-and-the-development-of-renal-pathology-in-cd37-deficient-mice
#14
Angelique L W M M Rops, Erik Jansen, Alie van der Schaaf, Elmar Pieterse, Nils Rother, Julia Hofstra, Henry B P M Dijkman, Anne-Els van de Logt, Jack Wetzels, Johan van der Vlag, Annemiek B van Spriel
Immunoglobulin A (IgA) nephropathy (IgAN), the most common glomerulonephritis worldwide, is characterized by IgA depositions in the kidney. Deficiency of CD37, a leukocyte-specific tetraspanin, leads to spontaneous development of renal pathology resembling IgAN. However, the underlying molecular mechanism has not been resolved. Here we found that CD37 expression on B cells of patients with IgAN was significantly decreased compared to B cells of healthy donors. Circulating interleukin (IL)-6 levels, but not tumor necrosis factor-α or IL-10, were elevated in Cd37-/- mice compared to wild-type mice after lipopolysaccharide treatment...
March 15, 2018: Kidney International
https://www.readbyqxmd.com/read/29529610/glucocorticoids-reduce-aberrant-o-glycosylation-of-iga1-in-iga-nephropathy-patients
#15
Petr Kosztyu, Martin Hill, Jana Jemelkova, Lydie Czernekova, Leona Raskova Kafkova, Miroslav Hruby, Karel Matousovic, Karel Vondrak, Josef Zadrazil, Ivan Sterzl, Jiri Mestecky, Milan Raska
BACKGROUND/AIMS: IgA nephropathy is associated with aberrant O-glycosylation of IgA1, which is recognized by autoantibodies leading to the formation of circulating immune complexes. Some of them, after deposition into kidney mesangium, trigger glomerular injury. In patients with active disease nonresponding to angiotensin-converting enzyme inhibitors or angiotensin II blockers, corticosteroids are recommended. METHODS: The relationship between the corticosteroid therapy and serum levels of IgA, aberrantly O-glycosylated IgA1, IgA-containing immune complexes and their mesangioproliferative activity was analyzed in IgA nephropathy patients and disease and healthy controls...
March 6, 2018: Kidney & Blood Pressure Research
https://www.readbyqxmd.com/read/29527861/possible-autoimmune-primary-ovarian-insufficiency-in-patients-with-selective-iga-deficiency
#16
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
https://www.readbyqxmd.com/read/29511864/secondary-antibody-deficiency-a-complication-of-anti-cd20-therapy-for-neuroinflammation
#17
E C Tallantyre, D H Whittam, S Jolles, D Paling, C Constantinesecu, N P Robertson, A Jacob
B-cell depleting anti-CD20 monoclonal antibody therapies are being increasingly used as long-term maintenance therapy for neuroinflammatory disease compared to many non-neurological diseases where they are used as remission-inducing agents. While hypogammaglobulinaemia is known to occur in over half of patients treated with medium to long-term B-cell-depleting therapy (in our cohort IgG 38, IgM 56 and IgA 18%), the risk of infections it poses seems to be under-recognised. Here, we report five cases of serious infections associated with hypogammaglobulinaemia occurring in patients receiving rituximab for neuromyelitis optica spectrum disorders...
March 6, 2018: Journal of Neurology
https://www.readbyqxmd.com/read/29511621/robust-dna-repair-in-paxx-deficient-mammalian-cells
#18
Alisa Dewan, Mengtan Xing, Marie Benner Lundbæk, Raquel Gago-Fuentes, Carole Beck, Per Arne Aas, Nina-Beate Liabakk, Siri Sæterstad, Khac Thanh Phong Chau, Bodil Merete Kavli, Valentyn Oksenych
To ensure genome stability, mammalian cells employ several DNA repair pathways. Nonhomologous DNA end joining (NHEJ) is the DNA repair process that fixes double-strand breaks throughout the cell cycle. NHEJ is involved in the development of B and T lymphocytes through its function in V(D)J recombination and class switch recombination (CSR). NHEJ consists of several core and accessory factors, including Ku70, Ku80, XRCC4, DNA ligase 4, DNA-PKcs, Artemis, and XLF. Paralog of XRCC4 and XLF (PAXX) is the recently described accessory NHEJ factor that structurally resembles XRCC4 and XLF and interacts with Ku70/Ku80...
March 2018: FEBS Open Bio
https://www.readbyqxmd.com/read/29506643/-diagnosis-and-treatment-of-primary-antibody-deficiency-syndromes
#19
Carsten Schade Larsen, Terese L Katzenstein
Primary antibody deficiencies (PAD) make up more than half of primary immunodeficiencies. PAD is characterized by low levels of one or more immunoglobulin (Ig) classes or impaired vaccine response. Recurrent infections are the predominant presenting symptoms, but autoimmune disorders are also frequent. Onset of symptoms is often after the age of six. Screening for PAD with measurement of the levels of serum IgG, IgM, and IgA is simple and can be done in general practice. Replacement therapy with Ig is the cornerstone in treatment of PAD and reduces the frequency of infections and mortality...
February 26, 2018: Ugeskrift for Laeger
https://www.readbyqxmd.com/read/29503733/clinicopathological-and-immunological-characteristics-and-outcome-of-concomitant-coeliac-disease-and-non-alcoholic-fatty-liver-disease-in-adults-a-large-prospective-longitudinal-study
#20
Sanaa Kamal, Khaled K Aldossari, Dhalia Ghoraba, Sara Mahmoud Abdelhakam, Amgad H Kamal, Mohamad Bedewi, Leila Nabegh, Khaled Bahnasy, Tamer Hafez
Objective: Concomitant non-alcoholic fatty liver disease (NAFLD) and coeliac disease (CD) have not been adequately studied. This study investigated the frequency of CD among NAFLD patients and the clinicopathological and immunological patterns and outcome of concomitant NAFLD and CD. Design: This prospective longitudinal study screened patients with NAFLD for CD (tissue transglutaminase antibodies (TTGA); anti-TTGA and antiendomysial antibodies (EMA)). Patients with concomitant NAFLD and CD and patients with either NAFLD or CD were enrolled and followed...
2018: BMJ Open Gastroenterology
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