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    4231 results match your criteria Immunoglobulin A Deficiency

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    IgM Augments Complement Bactericidal Activity with Serum from a Patient with a Novel CD79a Mutation.
    J Clin Immunol 2018 Jan 15. Epub 2018 Jan 15.
    Section Pediatric Infectious Diseases, Laboratory of Medical Immunology, Radboud Institute for Molecular Life Sciences, Radboud University Medical Center, Nijmegen, The Netherlands.
    Antibody replacement therapy for patients with antibody deficiencies contains only IgG. As a result, concurrent IgM and IgA deficiency present in a large proportion of antibody deficient patients persists. Especially patients with IgM deficiency remain at risk for recurrent infections of the gastrointestinal and respiratory tract. Read More

    Safety and tolerability of a novel, polyclonal human anti-MERS coronavirus antibody produced from transchromosomic cattle: a phase 1 randomised, double-blind, single-dose-escalation study.
    Lancet Infect Dis 2018 Jan 9. Epub 2018 Jan 9.
    National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, USA.
    Background: Middle East respiratory syndrome (MERS) is a severe respiratory illness with an overall mortality of 35%. There is no licensed or proven treatment. Passive immunotherapy approaches are being developed to prevent and treat several human medical conditions where alternative therapeutic options are absent. Read More

    Activation-induced cytidine deaminase deficiency accelerates autoimmune diabetes in NOD mice.
    JCI Insight 2018 Jan 11;3(1). Epub 2018 Jan 11.
    Section of Endocrinology, Department of Internal Medicine, Yale University School of Medicine, New Haven, Connecticut, USA.
    B cells play an important role in type 1 diabetes (T1D) development. However, the role of B cell activation-induced cytidine deaminase (AID) in diabetes development is not clear. We hypothesized that AID is important in the immunopathogenesis of T1D. Read More

    Bacterial-derived Neutrophilic Inflammation Drives Lung Remodeling in a Mouse Model of COPD.
    Am J Respir Cell Mol Biol 2018 Jan 9. Epub 2018 Jan 9.
    Vanderbilt University School of Medicine, Internal Medicine/Pulmonary and Critical Care Medicine, Nashville, Tennessee, United States ;
    Loss of secretory immunoglobulin A (SIgA) is common in the small airways of patients with chronic obstructive pulmonary disease (COPD) and may contribute to disease pathogenesis. Using mice that lack SIgA in the airways due to genetic deficiency of polymeric immunoglobulin receptor (pIgR-/- mice), we investigated the role of neutrophils in driving the fibrotic small airway wall remodeling and emphysema that develops spontaneously in these mice. By flow cytometry, we found an increase in the percentage of neutrophils among CD45+ cells in the lungs, as well as an increase in total neutrophils, in pIgR-/- mice compared to wild-type (WT) controls. Read More

    Celiac Disease in South Jordan.
    Pediatr Gastroenterol Hepatol Nutr 2017 Dec 22;20(4):222-226. Epub 2017 Dec 22.
    Department of Pediatrics, Faculty of Medicine, Jordan University of Science and Technology, Irbid, Jordan.
    Purpose: Celiac disease, an autoimmune enteropathy triggered by exposure to gluten, is not uncommon in South Jordan. However, its prevalence is underestimated due to lack of physician awareness of the diversity of disease presentation. The clinical spectrum includes classic gastrointestinal manifestations, as well as rickets, iron-deficiency anemia, short stature, elevated liver enzymes, and edema. Read More

    Prevalence of occult celiac disease in females with iron deficiency in the United States: an NHANES analysis.
    J Community Hosp Intern Med Perspect 2017 14;7(6):347-350. Epub 2017 Dec 14.
    Department of Internal Medicine, Hurley Medical Center, Michigan State University, College of Human Medicine, Flint, MI, USA.
    Aim: The prevalence of celiac disease (CD) in patients with iron deficiency (ID) is estimated at 0-6% in European countries. The prevalence of celiac disease in patients with iron deficiency in the USA is unknown. Given the treatable nature of gluten hypersensitivity, estimating the prevalence of CD in patients with ID can help to determine the need to screen these patients for occult CD. Read More

    Centrifugation-free washing: A novel approach for removing IgA from stored red blood cells.
    Am J Hematol 2017 Dec 29. Epub 2017 Dec 29.
    Department of Biomedical Engineering, University of Houston, Houston, TX 77204.
    Washed red blood cells (RBCs) are indicated for immunoglobulin A (IgA) deficient recipients. Centrifugation-based cell processors commonly used by hospital blood banks cannot consistently reduce IgA below the recommended levels, hence double washing is frequently required. Here we describe a prototype of a simple, portable, disposable system capable of washing stored RBCs without centrifugation, while reducing IgA below 0. Read More

    A Severe Anaphylactic Reaction Associated with IgM-Class Anti-Human IgG Antibodies in a Hyper-IgM Syndrome Type 2 Patient.
    J Clin Immunol 2018 Jan 19;38(1):144-148. Epub 2017 Dec 19.
    Department of Pediatrics, National Defense Medical College, 3-2 Namiki, Tokorozawa, Saitama, 359-0042, Japan.
    Purpose: A 42-year-old man with hyper-IgM syndrome type 2 caused by activation-induced cytidine deaminase (AID) deficiency developed a severe anaphylactic reaction to intravenous immunoglobulin. The purpose of this study was to clarify the cause of the anaphylactic reaction of the patient.

    Methods: We measured IgM-class anti-human IgG and anti-human IgA antibodies in his serum by sandwich enzyme-linked immunosorbent assay (ELISA). Read More

    [Myasthenia gravis, Graves-Basedow disease and other autoimmune diseases in patient with diabetes type 1 - APS-3 case report, therapeutic complications].
    Pediatr Endocrinol Diabetes Metab 2017 ;23(3):159-164
    Department of Children's Diabetology , School of Medicine in Katowice, Medical University of Silesia in Katowice.
    Diabetes type 1(T1D) is the most frequent form of diabetes in children and young people, which essence is autoimmune destruction of pancreatic B cells islet. Co-occurrence of other autoimmune diseases is observed in children with T1D, the most often are: Hashimoto disease or coeliac disease. We report the case of the patient, who presents coincidence of T1D with other rare autoimmune diseases such as: Graves - Basedow disease, myasthenia gravis, vitiligo and IgA deficiency. Read More

    [Clinical therapeutic effect on bronchial asthma in children of different body constitutions treated with sanfu pingchuan plaster and sanjiu zhichuan plaster].
    Zhongguo Zhen Jiu 2017 Apr;37(4):386-390
    Administration Office, Hebei Baoding Children Hospital, Baoding 071000, China.
    Objective: To observe the difference in the clinical therapeutic effects on bronchial asthma in children of different body constitutions treated with sanfu pingchuan plaster and sanjiu zhichuan plaster.

    Methods: One hundred and twenty-two children of bronchial asthma were divided into three groups according to TCM body constitutions, 42 cases in the qi deficiency constitution group, 40 cases in the yang deficiency constitution group and 40 cases in the phlegm damp constitution group. From 2011 to 2013, the acupoint plaster was applied to all of the children in the three groups during the dog days and the third nine-day period after the winter solstice each year. Read More

    Terminally differentiated memory T cells are increased in patients with common variable immunodeficiency and selective IgA deficiency.
    Cent Eur J Immunol 2017 30;42(3):244-251. Epub 2017 Oct 30.
    Department of Clinical Immunology and Allergology, St Anne's University Hospital, and Faculty of Medicine, Masaryk University, Brno, Czech Republic.
    Introduction: Previous studies showed that several lymphocyte abnormalities seen in the most frequent symptomatic immunoglobulin deficiency, common variable immunodeficiency (CVID), were also observed in a genetically related asymptomatic disorder - selective IgA deficiency (IgAD). In this study we searched for abnormalities in the differentiation stages of T cells as well as for similarities of these abnormalities in CVID and IgAD patients.

    Material And Methods: Using flow cytometry in 80 patients with IgAD, 48 patients with CVID, and 80 control persons we determined T-lymphocyte subsets: both CD4 and CD8 were divided into the naïve CD45RO-CD27+, early differentiated CD45RO+CD27+, late differentiated CD45RO+CD27- and fully differentiated effector CD45RO-CD27- memory T cells, as well as Treg cells, defined as CD4+CD25highCD127low T cells. Read More

    Delayed diagnosis in X-linked agammaglobulinemia and its relationship to the occurrence of mutations in BTK non-kinase domains.
    Expert Rev Clin Immunol 2018 Jan 11;14(1):83-93. Epub 2017 Dec 11.
    a Unidad de Investigación en Inmunodeficiencias , Instituto Nacional de Pediatría, SSA , Ciudad de México , Mexico.
    Background: X-linked agammaglobulinemia (XLA) is characterized by the absence of immunoglobulin and B cells. Patients suffer from recurrent bacterial infections from early childhood, and require lifelong immunoglobulin replacement therapy. Mutations in BTK (Bruton's Tyrosine Kinase) are associated with this phenotype. Read More

    Celiac Disease and Gluten Sensitivity.
    Prim Care 2017 Dec;44(4):693-707
    Department of Family Medicine, University of Virginia, PO Box 800729, Charlottesville, VA 22908-0543, USA.
    Celiac disease is an immune-mediated enteropathy triggered by gluten that affects genetically predisposed individuals, typically causing intestinal symptoms and malabsorption. Diagnosis requires stepwise evaluation with anti-tissue transglutaminase IgA and histologic analysis of the small bowel. Strict adherence to a gluten-free diet is the primary treatment. Read More

    Prospects for modulating the CD40/CD40L pathway in the therapy of the hyper-IgM syndrome.
    Innate Immun 2018 Jan 13;24(1):4-10. Epub 2017 Nov 13.
    1 Sunshine Lake Pharma Co., Ltd, Dongguan 523867, PR China.
    The critical role of the CD40/CD40L pathway in B-cell proliferation, immunoglobulin (Ig) isotype switching and germinal center formation has been studied and described extensively in previous literature. Interruption of the CD40/CD40L signal causes hyper-IgM (HIGM) syndrome, which has been classified and recognized as a group of rare inherited immune deficiency disorders. Defects in CD40 and CD40L interactions or in downstream signaling molecules, including activation-induced cytidine deaminase, uracyl-DNA-glycosylase, NF-κB and DNA repair enzymes, result in an increased level of serum IgM and a significantly decreased or absent level of IgA, IgG and IgE that is accompanied by severe recurrent infections and autoimmune diseases. Read More

    Vitamin D deficiency in children with recurrent respiratory infections, with or without immunoglobulin deficiency.
    Adv Med Sci 2017 Nov 9;63(1):173-178. Epub 2017 Nov 9.
    Department of Paediatrics, Rheumatology, Immunology and Metabolic Bone Diseases, The Medical University of Białystok, Białystok, Poland.
    Purpose: The objective of this study was to evaluate thevitamin D concentration in patients with recurrent respiratory infections with or without immunoglobulin G, A or M (IgG, IgA, IgM) deficiency, and to find a correlation between the vitamin D concentration and the response to hepatitis B vaccination.

    Materials And Method: The study involved 730 patients with recurrent respiratory infections. The concentration of 25-hydroxyvitamin D (25(OH)D), immunoglobulins G, A and M, anti-HBs was determined. Read More

    The Clinical Utility of Measuring IgG Subclass Immunoglobulins During Immunological Investigation for Suspected Primary Antibody Deficiencies.
    Lab Med 2017 Nov;48(4):314-325
    The Binding Site Group Limited, Edgbaston, Birmingham.
    Measurement of IgG subclass concentrations is a standard laboratory test run as part of a panel to investigate the suspicion of antibody deficiency. The assessment is clinically important when total IgG is within the normal age-specific reference range. The measurement is useful for diagnosis of IgG subclass deficiency, to aid the diagnosis of specific antibody deficiency, as a supporting test for the diagnosis of common variable immunodeficiency, as well as for risk stratification of patients with low IgA. Read More

    Epistatic interactions between mutations of TACI (TNFRSF13B) and TCF3 result in a severe primary immunodeficiency disorder and systemic lupus erythematosus.
    Clin Transl Immunology 2017 Oct 20;6(10):e159. Epub 2017 Oct 20.
    Department of Immunology, Walter and Eliza Hall Institute of Medical Research, Parkville, VIC, Australia.
    Common variable immunodeficiency disorders (CVID) are a group of primary immunodeficiencies where monogenetic causes account for only a fraction of cases. On this evidence, CVID is potentially polygenic and epistatic although there are, as yet, no examples to support this hypothesis. We have identified a non-consanguineous family, who carry the C104R (c. Read More

    Endogenous Calcitriol Synthesis Controls the Humoral IgE Response in Mice.
    J Immunol 2017 Dec 6;199(12):3952-3958. Epub 2017 Nov 6.
    Klinik für Dermatologie, Venerologie und Allergologie, Allergie-Centrum-Charité, Campus Charité Mitte, Charité - Universitätsmedizin Berlin, D-10117 Berlin, Germany;
    The vitamin D receptor participates in the control of IgE class-switch recombination in B cells. The physiologic vitamin D receptor agonist, 1,25(OH)2D3 (calcitriol), is synthesized by the essential enzyme 25-hydroxyvitamin D3-1α-hydroxylase (CYP27B1), which can be expressed by activated immune cells. The role of endogenous calcitriol synthesis for the regulation of IgE has not been proven. Read More

    Autoantibodies against complement components in systemic lupus erythematosus - role in the pathogenesis and clinical manifestations.
    Lupus 2017 Dec 18;26(14):1550-1555. Epub 2017 May 18.
    2 Department of Chemistry, Biochemistry, Physiology and Pathophysiology, Sofia University "St. Kliment Ohridski", Bulgaria.
    Many complement structures and a number of additional factors, i.e. autoantibodies, receptors, hormones and cytokines, are implicated in the complex pathogenesis of systemic lupus erythematosus. Read More

    Immunoglobulin A and liver diseases.
    J Gastroenterol 2017 Oct 26. Epub 2017 Oct 26.
    Department of Medicine, University of California, San Diego, MC0063, 9500 Gilman Drive, La Jolla, San Diego, CA, 92093, USA.
    Immunoglobulin A (IgA) is a major immunoglobulin isotype in the gut and plays a role in maintenance of gut homeostasis. Secretory IgA (SIgA) has multiple functions in the gut, such as to regulate microbiota composition, to protect intestinal epithelium from pathogenic microorganisms, and to help for immune-system development. The liver is the front-line organ that receives gut-derived products through the portal vein, implying that the liver could be severely affected by a disrupted intestinal homeostasis. Read More

    Dual Roles of Glutathione in Ecdysone Biosynthesis and Antioxidant Function During Larval Development in Drosophila.
    Genetics 2017 Dec 11;207(4):1519-1532. Epub 2017 Oct 11.
    Faculty of Life and Environmental Sciences, University of Tsukuba, Ibaraki 305-8572, Japan
    Ecdysteroids, including the biologically active hormone 20-hydroxyecdysone (20E), play essential roles in controlling many developmental and physiological events in insects. Ecdysteroid biosynthesis is achieved by a series of specialized enzymes encoded by the Halloween genes. Recently, a new class of Halloween gene, noppera-bo (nobo), encoding a glutathione S-transferase (GST) in dipteran and lepidopteran species, has been identified and characterized. Read More

    Screening for celiac disease in poorly controlled type 2 diabetes mellitus: worth it or not?
    BMC Endocr Disord 2017 Oct 6;17(1):62. Epub 2017 Oct 6.
    Department of Endocrinology and Metabolism, Diskapi Training and Research Hospital, Ankara, Turkey.
    Background: Recent studies have demonstrated that immune factors might have a role in the pathophysiology of insulin resistance and type 2 diabetes mellitus (T2DM). Inappropriate glycemic control in patients with T2DM is an important risk factor for the occurrence of diabetes complications. The prevalence of celiac disease (CD) is high in type 1 diabetes mellitus however, there are scarce data about its prevalence in T2DM. Read More

    Truly selective primary IgM deficiency is probably very rare.
    Clin Exp Immunol 2018 Feb 27;191(2):203-211. Epub 2017 Oct 27.
    Department of Tranzo, Tilburg University, Tilburg, the Netherlands.
    Isolated decreased serum-immunoglobulin (Ig)M has been associated with severe and/or recurrent infections, atopy and autoimmunity. However, the reported high prevalence of clinical problems in IgM-deficient patients may reflect the skewed tertiary centre population studied so far. Also, many papers on IgM deficiency have included patients with more abnormalities than simply IgM-deficiency. Read More

    Antibiotic Prophylaxis, Immunoglobulin Substitution and Supportive Measures Prevent Infections in MECP2 Duplication Syndrome.
    Pediatr Infect Dis J 2017 Sep 20. Epub 2017 Sep 20.
    1Pediatric Pneumology, Immunology and Intensive Care Medicine, Charité University Medicine Berlin, Germany 2Labor Berlin GmbH, Department of Immunology, Berlin, Germany 3Brandenburg Center for Regenerative Therapies (BCRT), Charité University Medicine Berlin, Germany 4Sozialpädiatrisches Zentrum, Charité University Medicine Berlin, Germany.
    Respiratory infections are the main cause of early death in patients with MECP2 duplication syndrome. We report on a 20-year-old patient with MECP2 duplication syndrome, IgG2/IgG4/IgA/IgM-deficiency and polysaccharide-specific-antibody-deficiency, who had 46 episodes of pneumonia in his first 13 years of life. Immunoglobulin substitution, daily antibiotic prophylaxis with two agents and supportive measures reduced occurrence of pneumonia to 4 episodes in the following 6 years of life. Read More

    Chylothorax after pediatric cardiac surgery complicates short-term but not long-term outcomes-a propensity matched analysis.
    J Thorac Dis 2017 Aug;9(8):2466-2475
    Department of Anesthesiology and Pediatric Intensive Care, Gottsegen György Hungarian Institute of Cardiology, Budapest, Hungary.
    Background: The occurrence of postoperative chylothorax in children with congenital heart disease is a rare and serious complication in cardiac intensive care units (ICUs). The aim of our study was to identify the perioperative characteristics, treatment options, resource utilization and long term complications of patients having chylothorax after a pediatric cardiac surgery.

    Methods: Patients were retrospectively assessed for the presence of chylothorax between January 2002 and December 2012 in a tertiary national cardiac center. Read More

    CCCTC-Binding Factor Locks Premature IgH Germline Transcription and Restrains Class Switch Recombination.
    Front Immunol 2017 4;8:1076. Epub 2017 Sep 4.
    B Lymphocyte Biology Laboratory, Fundacion Centro Nacional de Investigaciones Cardiovasculares Carlos III, Madrid, Spain.
    In response to antigenic stimulation B cells undergo class switch recombination (CSR) at the immunoglobulin heavy chain (IgH) to replace the primary IgM/IgD isotypes by IgG, IgE, or IgA. CSR is initiated by activation-induced cytidine deaminase (AID) through the deamination of cytosine residues at the switch (S) regions of IgH. B cell stimulation promotes germline transcription (GLT) of specific S regions, a necessary event prior to CSR because it facilitates AID access to S regions. Read More

    Selective IgM Deficiency-An Underestimated Primary Immunodeficiency.
    Front Immunol 2017 5;8:1056. Epub 2017 Sep 5.
    Program in Primary Immunodeficiency and Aging, Division of Basic and Clinical Immunology, University of California at Irvine, Irvine, CA, United States.
    Although selective IgM deficiency (SIGMD) was described almost five decades ago, it was largely ignored as a primary immunodeficiency. SIGMD is defined as serum IgM levels below two SD of mean with normal serum IgG and IgA. It appears to be more common than originally realized. Read More

    Targeted deletion of RANKL in M cell inducer cells by the Col6a1-Cre driver.
    Biochem Biophys Res Commun 2017 11 5;493(1):437-443. Epub 2017 Sep 5.
    Department of Immunology, Graduate School of Medicine and Faculty of Medicine, The University of Tokyo, Hongo 7-3-1, Bunkyo-ku, Tokyo, 113-0033, Japan. Electronic address:
    The gut-associated lymphoid tissues (GALTs), including Peyer's patches (PPs), cryptopatches (CPs) and isolated lymphoid follicles (ILFs), establish a host-microbe symbiosis by the promotion of immune reactions against gut microbes. Microfold cell inducer (MCi) cells in GALTs are the recently identified mesenchymal cells that express the cytokine RANKL and initiate bacteria-specific immunoglobulin A (IgA) production via induction of microfold (M) cell differentiation. In the previous study, the Twist2-Cre driver was utilized for gene deletion in mesenchymal cells including MCi cells. Read More

    Immunoglobulin levels and infection risk with rituximab induction for anti-neutrophil cytoplasmic antibody-associated vasculitis.
    Clin Kidney J 2017 Aug 12;10(4):470-474. Epub 2017 Apr 12.
    Department of Nephrology, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
    Background: Rituximab (RTX), a B cell-depleting anti-CD20 monoclonal antibody, is approved for treatment of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). Low immunoglobulin (Ig) levels have been observed surrounding RTX treatment. The association between the degree of Ig deficiency and infection risk is unclear in AAV patients. Read More

    Early diagnosis of PI3Kδ syndrome in a 2 years old girl with recurrent otitis and enlarged spleen.
    Immunol Lett 2017 Oct 31;190:279-281. Epub 2017 Aug 31.
    Fondazione Monza e Brianza per il Bambino e la sua Mamma, Ospedale San Gerardo, Monza, Italy; Università degli Studi di Milano-Bicocca, Monza, Italy.
    Heterozygous gain of function mutations in the gene encoding p110δ subunit of PI3K have been recently associated with activated PI3K-δ syndrome (APDS), a novel combined immune deficiency characterized by recurrent sinopulmonary infections, lymphopenia, reduced class-switched memory B cells, lymphadenopathy, CMV and/or EBV viremia and EBV-related lymphoma. Here we report a dominant gain of function PIK3CD mutation (E1021K) in a patient presenting with recurrent otitis media, massive splenomegaly, and persistent EBV-viraemia. The immunological studies showed low IgA level, but normal IgM, IgG, and normal antibody response to diphtheria and tetanus toxoid vaccination. Read More

    CCR7 Deficiency Allows Accelerated Clearance of Chlamydia from the Female Reproductive Tract.
    J Immunol 2017 Oct 11;199(7):2547-2554. Epub 2017 Aug 11.
    Center for Comparative Medicine, Department of Anatomy, Physiology, and Cell Biology, School of Veterinary Medicine, University of California, Davis, Davis, CA 95616; and.
    Immune mechanisms responsible for pathogen clearance from the female reproductive tract (FRT) are incompletely defined; in particular, the contribution of lymphocyte trafficking to this process is unclear. CCR7-deficient mice have profoundly altered lymphocyte recirculation and display ectopic formation of lymphocyte aggregates within mucosal nonlymphoid tissues, including the FRT. In this study, we investigated how altered lymphocyte distribution in CCR7-deficient mice would affect host responses to Chlamydia muridarum within the reproductive tract. Read More

    Vasculitis update: pathogenesis and biomarkers.
    Pediatr Nephrol 2018 Feb 7;33(2):187-198. Epub 2017 Aug 7.
    Infection, Inflammation, and Immunology Section, University College London Great Ormond Street Institute of Child Health, 30 Guilford Street, London, WC1N1EH, UK.
    Better understanding of the pathogenesis and treatment of primary systemic vasculitides (PSV) has led to the development of many potentially clinically relevant biomarkers. Genome-wide association studies have highlighted that MHC class II polymorphisms may influence the development of particular anti-neutrophil cytoplasmic antibody (ANCA) serotypes, but not the clinical phenotype of ANCA-associated vasculitis (AAV). Although ANCAs are overall poor biomarkers of disease activity, they may be useful for the prediction of flares of renal and/or pulmonary vasculitis. Read More

    Defective anti-polysaccharide IgG vaccine responses in IgA deficient mice.
    Vaccine 2017 Sep 31;35(37):4997-5005. Epub 2017 Jul 31.
    Department of Immunology and Microbial Disease, Albany Medical College, Albany, NY 12208, USA. Electronic address:
    We report that IgA-/- mice exhibit specific defects in IgG antibody responses to various polysaccharide vaccines (Francisella tularensis LPS and Pneumovax), but not protein vaccines such as Fluzone. This defect further included responses to polysaccharide-protein conjugate vaccines (Prevnar and Haemophilus influenzae type b-tetanus toxoid vaccine). In agreement with these findings, IgA-/- mice were protected from pathogen challenge with protein- but not polysaccharide-based vaccines. Read More

    Two Sides of the Same Coin: Pediatric-Onset and Adult-Onset Common Variable Immune Deficiency.
    J Clin Immunol 2017 Aug 28;37(6):592-602. Epub 2017 Jul 28.
    Division of Allergy and Immunology, Children's Hospital of Philadelphia, ARC 1216, 3615 Civic Center Blvd., Philadelphia, PA, 19104, USA.
    Purpose: Common variable immunodeficiency (CVID) is a complex, heterogeneous immunodeficiency characterized by hypogammaglobulinemia, recurrent infections, and poor antibody response to vaccination. While antibiotics and immunoglobulin prophylaxis have significantly reduced infectious complications, non-infectious complications of autoimmunity, inflammatory lung disease, enteropathy, and malignancy remain of great concern. Previous studies have suggested that CVID patients diagnosed in childhood are more severely affected by these complications than adults diagnosed later in life. Read More

    CCR6 Deficiency Impairs IgA Production and Dysregulates Antimicrobial Peptide Production, Altering the Intestinal Flora.
    Front Immunol 2017 11;8:805. Epub 2017 Jul 11.
    Taiwan International Graduate Program in Molecular Medicine, National Yang-Ming University and Academia Sinica, Taipei, Taiwan.
    Intestinal immunity exists as a complex relationship among immune cells, epithelial cells, and microbiota. CCR6 and its ligand-CCL20 are highly expressed in intestinal mucosal tissues, such as Peyer's patches (PPs) and isolated lymphoid follicles (ILFs). In this study, we investigated the role of the CCR6-CCL20 axis in intestinal immunity under homeostatic conditions. Read More

    Serum Vitamins and Minerals at Diagnosis and Follow-up in Children With Celiac Disease.
    J Pediatr Gastroenterol Nutr 2017 Aug;65(2):185-189
    *Section of Pediatric Gastroenterology, Department of Pediatrics and Child Health, Max Rady College of Medicine, Rady Faculty of Health Sciences†Departments of Internal Medicine and Pathology, Max Rady College of Medicine, University of Manitoba‡Children's Hospital Research Institute of Manitoba, Winnipeg, Manitoba, Canada§Department of Pediatrics, University of Alexandria, Egypt.
    Objectives: Children with celiac disease (CD) may experience deficiencies of several micronutrients. The objectives of the present study were to determine the prevalence of micronutrient deficiencies in children with CD at diagnosis, 6 months, and 18 months after the start of a gluten-free diet (GFD), and examine any correlation between micronutrient deficiencies, serum tissue transglutaminase (TtG) immunoglobulin A (IgA) antibody titers, and the degree of mucosal damage at diagnosis.

    Methods: Children (<17 years) with CD had their serum vitamins, minerals, and anti-TtG IgA antibodies measured at diagnosis, 6 and 18 months after starting a GFD. Read More

    Brucella Causing Liver Abscess in a Child with Selective IgA Deficiency.
    Indian Pediatr 2017 Jul;54(7):595-596
    Department of Pediatric Medicine, Institute of Child Health, Kolkata, West Bengal, India. Correspondence to: Dr Devdeep Mukherjee, Uttara Cooperative Housing Society, 13, Broad Street, Kolkata 700 019, West Bengal, India.
    Background: Brucella has been known to cause pyrexia of unknown origin.

    Case Characteristics: 9-year-old boy with fever and abdominal pain; multiple abscesses within the liver on ultrasonography.

    Observations: IgM Antibodies against Brucella were raised in his serum sample, and Brucella serum agglutination test was positive. Read More

    Selective IgM Deficiency: Clinical and Laboratory Features of 17 Patients and a Review of the Literature.
    J Clin Immunol 2017 Aug 21;37(6):559-574. Epub 2017 Jul 21.
    Department of Clinical Immunology and Allergy, St. Anne's University Hospital in Brno, Pekarska 53, 65691, Brno, Czech Republic.
    Purpose: Primary selective IgM deficiency (sIgMD) is a primary immunodeficiency with unclear pathogenesis and a low number of published cases.

    Methods: We reviewed clinical and laboratory manifestations of 17 sIgMD patients. Serum IgM, IgG, and its subclasses, IgA, IgE, antibodies against tetanus toxoid, pneumococcal polysaccharides and Haemophilus influenzae type b, isohemagglutinins, and T and B lymphocyte subsets, expressions of IgM on B cells and B lymphocyte production of IgM were compared with previously reported case reports and a small series of patients, which included 81 subjects in total. Read More

    Cernunnos deficiency associated with BCG adenitis and autoimmunity: First case from the national Iranian registry and review of the literature.
    Clin Immunol 2017 Oct 17;183:201-206. Epub 2017 Jul 17.
    Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Science, Tehran, Iran. Electronic address:
    Non-homologous end-joining (NHEJ) is a pathway that repairs double-strand breaks (DSB) in DNA and plays a vital role in V(D)J recombination of immunoglobulin genes. Cernunnos is a DNA repair factor that is involved in nonhomologous end-joining (NHEJ) process. Impairment in Cernunnos leads to a genetic disease characterized by neural disorders, immunodeficiency and increased radiosensitivity. Read More

    X-Linked Agammaglobulinaemia: Outcomes in the modern era.
    Clin Immunol 2017 Oct 17;183:54-62. Epub 2017 Jul 17.
    Institute of Cellular Medicine, Newcastle University, 4th Floor, William Leech Building, Medical School, Framlington Place, Newcastle upon Tyne NE2 4HH, United Kingdom.
    Colonel Ogden Bruton reported X-Linked Agammaglobulinaemia in 1952 and treated the child with replacement immunoglobulin therapy. Over 60years later, the treatment for XLA has largely remained unchanged. Replacement immunoglobulin lacks the isotypes IgA and IgM, leading to concerns that patients continue to experience recurrent sinopulmonary tract infections and be at increased risk of bronchiectasis. Read More

    Mannose-Binding Lectin Levels Could Predict Prognosis in IgA Nephropathy.
    J Am Soc Nephrol 2017 Nov 11;28(11):3175-3181. Epub 2017 Jul 11.
    Renal Division, Department of Medicine, Peking University First Hospital, Beijing, China.
    IgA nephropathy (IgAN) is characterized by infections followed by episodic gross hematuria. Deficiency of mannose-binding lectin (MBL) is associated with recurrent infection in many diseases, but controversy exists regarding the role of MBL in IgAN. Here, we measured MBL2 variants and MBL levels in 749 patients with IgAN and 489 healthy controls. Read More

    Oral manifestations of selective IgA-deficiency: review and case-report.
    J Biol Regul Homeost Agents 2017 Apr-Jun;31(2 Suppl 1):113-117
    Department of Surgical and Morphological Sciences, University of Insubria, Ospedale di Circolo Macchi Foundation, Unit of Oral Pathology, Varese, Italy.
    Immunoglobulin A deficiency is the most common primary immunodeficiency defined as decreased serum level of IgA (less than 7 mg/dl) in the presence of normal levels of other immunoglobulin isotypes. Most individuals with IgA deficiency are asymptomatic and identified coincidentally. However, some patients may present with recurrent infections, allergic disorders and autoimmune manifestations, such as diabetes mellitus, Graves disease and celiac disease. Read More

    [Clinical and immunological profile of 15 Moroccan patients with Hyper IgM syndrome].
    Pan Afr Med J 2017 19;26:212. Epub 2017 Apr 19.
    Laboratoire d'Immunologie Clinique, Inflammation et Allergie, Faculté de Médecine et Pharmacie, Université Hassan II, Casablanca, Maroc.
    Hyper IgM syndrome is a well known genetic (primary) immunodeficiency disorder which was first described in 1961. It is caused by B lymphocyte deficiency characterized by normal or elevated serum IgM levels and low or zero levels of IgG, IgA, IgE resulting from isotype-switching deficiency. Clinical manifestations are dominated by recurrent infections, especially involving the digestive tube of the ENT sphere and the lungs. Read More

    Autoimmunity and allergy control in adults submitted to complete thymectomy early in infancy.
    PLoS One 2017 7;12(7):e0180385. Epub 2017 Jul 7.
    Instituto de Medicina Molecular, Faculdade de Medicina, Universidade de Lisboa. Lisboa, Portugal.
    The contribution of the decline in thymic activity for the emergence of autoimmunity is still debatable. Immune-competent adults submitted to complete thymectomy early in life provide a unique model to address this question. We applied here strict criteria to identify adults lacking thymic activity based on sjTREC levels, to exclude thymic rebound and/or ectopic thymuses. Read More

    A unified pathogenesis for kidney diseases, including genetic diseases and cancers, by the protein-homeostasis-system hypothesis.
    Kidney Res Clin Pract 2017 Jun 30;36(2):132-144. Epub 2017 Jun 30.
    Department of Pediatrics, College of Medicine, The Catholic University of Korea, Seoul, Korea.
    Every cell of an organism is separated and protected by a cell membrane. It is proposed that harmony between intercellular communication and the health of an organism is controlled by a system, designated the protein-homeostasis-system (PHS). Kidneys consist of a variety of types of renal cells, each with its own characteristic cell-receptor interactions and producing characteristic proteins. Read More

    Vitamin D-deficiency and sex-specific dysregulation of placental inflammation.
    J Steroid Biochem Mol Biol 2017 Jul 1. Epub 2017 Jul 1.
    Dept of Orthopaedic Surgery, David Geffen School of Medicine at UCLA, Los Angeles, CA, 90095, USA; Institute of Metabolism and Systems Research, The University of Birmingham, Birmingham B15 2TT, UK; Centre for Endocrinology, Diabetes and Metabolism, Birmingham Health Partners, Birmingham B15 2TH, UK. Electronic address:
    To investigate an immunomodulatory role for vitamin D in pregnancy we used mice raised on vitamin D-sufficient (SUFF), or -deficient (DEF) diets. At embryonic day 14, pregnant mice received intraperitoneal injection of lipopolysaccharide (LPS) or vehicle for 24h, with age-matched non-pregnant mice as controls. In non-pregnant mice, 6 serum analytes (IL-1β, IL-18, MDC/CCL22, MIP-1α/CCL3, EGF, IgA) were lower in DEF mice. Read More

    Early Postnatal Secondhand Smoke Exposure Disrupts Bacterial Clearance and Abolishes Immune Responses in Muco-Obstructive Lung Disease.
    J Immunol 2017 Aug 30;199(3):1170-1183. Epub 2017 Jun 30.
    Department of Comparative Biomedical Sciences, School of Veterinary Medicine, Louisiana State University, Baton Rouge, LA 70803;
    Secondhand smoke (SHS) exposure has been linked to the worsening of ongoing lung diseases. However, whether SHS exposure affects the manifestation and natural history of imminent pediatric muco-obstructive airway diseases such as cystic fibrosis remains unclear. To address these questions, we exposed Scnn1b transgenic (Scnn1b-Tg+) mice to SHS from postnatal day (PND) 3-21 and lung phenotypes were examined at PND22. Read More

    Immunological phenotype of the murine Lrba knockout.
    Immunol Cell Biol 2017 Oct 25;95(9):789-802. Epub 2017 Jul 25.
    Center for Chronic Immunodeficiency (CCI), Medical Center-Faculty of Medicine, University of Freiburg, Freiburg, Germany.
    Biallelic mutations in the human lipopolysaccharide responsive beige-like anchor (LRBA) gene lead to a primary immunodeficiency known as LRBA deficiency, characterized by a broad range of clinical manifestations including autoimmunity, organomegaly, hypogammaglobulinemia and recurrent infections. Considering the phenotypic heterogeneity in patients and the severity of the disease, our aim was to assess the role of LRBA in immune cells and to understand the underlying pathomechanisms through the study of a Lrba knockout (Lrba-/-) mouse model. LRBA-deficient mice did not show severe clinical or immunological signs of disease, either at steady state under specific-pathogen-free conditions, after vaccination with T-dependent and T-independent antigens, or in the context of acute infections with lymphocytic choriomeningitis virus (LCMV) or Salmonella Typhimurium. Read More

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