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    5041 results match your criteria Immunoglobulin G Deficiency

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    Adaptive Returns of Deficient Systemic Plasma Immunoglobulin G Levels as Rehabilitation Biomarker After Emergency Colectomy for Fulminant Ulcerative Colitis.
    Clin Med Insights Gastroenterol 2017 13;10:1179552217746692. Epub 2017 Dec 13.
    Department of Surgery, Vanderbilt University School of Medicine, Nashville, TN, USA.
    Restorative proctocolectomy (RPC) with ileal pouch-anal anastomosis (IPAA) is the standard surgical treatment for ulcerative colitis (UC). Emergency colectomies are performed for fulminant colitis (ie, toxic megacolon, profuse bleeding, perforation, or sepsis). The RPC and IPAA involve manipulation of the proximal ileum, which may influence the essential physiological function of gut-associated lymphoid tissues. Read More

    [Retinoic Acid Prevents Dendritic Cells from Inducing Novel Inflammatory T Cells That Produce Abundant Interleukin-13].
    Yakugaku Zasshi 2017 ;137(12):1491-1496
    Laboratory of Immunology, Kagawa School of Pharmaceutical Sciences, Tokushima Bunri University.
     Vitamin A (VA) plays critical roles in gut homeostasis. Dendritic cells in mesenteric lymph nodes (MLN-DCs) can metabolize VA to retinoic acid (RA), thereby inducing gut-tropic lymphocytes and enhancing peripheral differentiation of regulatory T cells expressing forkhead box P3. We found that MLN-DCs from VA-deficient mice induced a distinct inflammatory T helper type 2 (Th2)-cell subset that produced abundant interleukin-13 (IL-13) and expressed receptors for homing to skin and inflammatory sites but not to the intestine. Read More

    [EFFICACY OF IVIG TREATMENT IN BRONCHIECTASIS ASSOCIATED WITH IGG SUBCLASS DEFICIENCY].
    Harefuah 2017 Nov;156(11):705-709
    Pulmonary Institute, Rabin Medical Center, Beilinson Campus, Petach Tikva, Israel, affiliated with Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
    Introduction: Bronchiectasis is characterized by an abnormal dilatation of the bronchi leading to a chronic inflammatory process, airway blockage and impaired clearance of secretions. The damage to the airways is usually progressive and is the result of several pathogenic processes. In the past, healing of infections (especially pulmonary tuberculosis) was the main cause of airway dilatation and progression of chronic inflammation. Read More

    mTOR intersects antibody-inducing signals from TACI in marginal zone B cells.
    Nat Commun 2017 Nov 13;8(1):1462. Epub 2017 Nov 13.
    Program for Inflammatory and Cardiovascular Disorders, Institut Hospital del Mar d'Investigacions Mèdiques (IMIM), Barcelona, 08003, Spain.
    Mechanistic target of rapamycin (mTOR) enhances immunity in addition to orchestrating metabolism. Here we show that mTOR coordinates immunometabolic reconfiguration of marginal zone (MZ) B cells, a pre-activated lymphocyte subset that mounts antibody responses to T-cell-independent antigens through a Toll-like receptor (TLR)-amplified pathway involving transmembrane activator and CAML interactor (TACI). This receptor interacts with mTOR via the TLR adapter MyD88. 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

    IgG1 Is Required for Optimal Protection after Immunization with the Purified Porin OmpD from Salmonella Typhimurium.
    J Immunol 2017 Dec 10;199(12):4103-4109. Epub 2017 Nov 10.
    Institute of Immunology and Immunotherapy, University of Birmingham, Birmingham B15 2TT, United Kingdom;
    In mice, the IgG subclass induced after Ag encounter can reflect the nature of the Ag. Th2 Ags such as alum-precipitated proteins and helminths induce IgG1, whereas Th1 Ags, such as Salmonella Typhimurium, predominantly induce IgG2a. The contribution of different IgG isotypes to protection against bacteria such as S. 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

    Broad Susceptibility of Nucleolar Proteins and Autoantigens to Complement C1 Protease Degradation.
    J Immunol 2017 Dec 25;199(12):3981-3990. Epub 2017 Oct 25.
    Department of Microbiology and Immunology, Yong Loo Lin School of Medicine and Immunology Programme, National University of Singapore, Singapore 117597, Singapore; and
    Anti-nuclear autoantibodies, which frequently target the nucleoli, are pathogenic hallmarks of systemic lupus erythematosus (SLE). Although the causes of these Abs remain broad and ill-defined, a genetic deficiency in C1 complex (C1qC1r2C1s2) or C4 is able to induce these Abs. Considering a recent finding that, in dead cells, nucleoli were targeted by C1q and two nucleolar autoantigens were degraded by C1r/C1s proteases, we considered that C1 could help protect against antinuclear autoimmunity by broadly degrading nucleolar proteins or autoantigens. Read More

    Successful discontinuation of immunoglobulin G replacement at age 10 in a patient with immunoglobulin G2 deficiency.
    SAGE Open Med Case Rep 2017 11;5:2050313X17736421. Epub 2017 Oct 11.
    Department of Pediatrics, Keio University School of Medicine, Tokyo, Japan.
    Context: Immunoglobulin G2 deficiency that persists beyond the age of 6 years is likely to be permanent.

    Case Report: We report on a young Japanese female, diagnosed as having immunoglobulin G2 deficiency and low anti-pneumococcal immunoglobulin G2 antibody levels when 3 years old, with a subsequent medical history of frequent respiratory infections and asthma. Monthly intravenous immunoglobulin replacement therapy was started at 4 years of age. Read More

    Recombinant human hyaluronidase-facilitated subcutaneous immunoglobulin infusion in primary immunodeficiency diseases.
    Immunotherapy 2017 Sep 5;9(12):1035-1050. Epub 2017 Sep 5.
    Allergy Partners of North Texas, Medical City Children's Hospital, Dallas, TX, USA.
    Most primary immunodeficiency diseases (PIDDs) resulting in antibody deficiency require intravenous or subcutaneous immunoglobulin G (SCIG) replacement therapy. The flow and distribution of SCIG to the vasculature is impeded by the glycosaminoglycan hyaluronan in the extracellular matrix, which limits the infusion rate and volume per site, necessitating frequent infusions and multiple infusion sites. Hyaluronidase depolymerizes hyaluronan and is a spreading factor for injectable biologics. Read More

    Unusual loss of chymosin in mammalian lineages parallels neo-natal immune transfer strategies.
    Mol Phylogenet Evol 2017 Nov 26;116:78-86. Epub 2017 Aug 26.
    CIIMAR - Interdisciplinary Centre of Marine and Environmental Research, U. Porto - University of Porto, Porto, Portugal; Department of Biology, Faculty of Sciences, U. Porto - University of Porto, Portugal. Electronic address:
    Gene duplication and loss are powerful drivers of evolutionary change. The role of loss in phenotypic diversification is notably illustrated by the variable enzymatic repertoire involved in vertebrate protein digestion. Among these we find the pepsin family of aspartic proteinases, including chymosin (Cmy). Read More

    Involvement of pentraxin-3 in anti-neutrophil cytoplasmic antibody production induced by aluminum salt adjuvant.
    Clin Exp Rheumatol 2017 Sep-Oct;35(5):735-738. Epub 2017 Aug 28.
    Department of Nephrology, Faculty of Medicine, University of Tsukuba, Japan.
    Objectives: Pentraxin 3 (PTX3) is a multifunctional soluble factor. PTX3 can be involved in the regulation of vasculitis and is expressed in the cytoplasm of neutrophils. As anti-neutrophil cytoplasmic antibody (ANCA) is recognised as a cause of vasculitis, we aimed to discover the role of PTX3 in ANCA production in vivo. Read More

    Single Nucleotide Polymorphisms of the High Affinity IgG Receptor FcγRI Reduce Immune Complex Binding and Downstream Effector Functions.
    J Immunol 2017 Oct 16;199(7):2432-2439. Epub 2017 Aug 16.
    Laboratory of Translational Immunology, University Medical Center Utrecht, 3584 CX Utrecht, the Netherlands
    Binding of IgG Abs to FcγRs on immune cells induces FcγR cross-linking that leads to cellular effector functions, such as phagocytosis, Ab-dependent cellular cytotoxicity, and cytokine release. However, polymorphisms in low affinity FcγRs have been associated with altered avidity toward IgG, thereby substantially impacting clinical outcomes of multimodular therapy when targeting cancer or autoimmune diseases with mAbs as well as the frequency and severity of autoimmune diseases. In this context, we investigated the consequences of three nonsynonymous single nucleotide polymorphisms (SNPs) for the high affinity receptor for IgG, FcγRI. Read More

    Serum carnitine and acyl-carnitine in patients with meningitis due to tick-borne encephalitis virus infection.
    Adv Clin Exp Med 2017 Mar-Apr;26(2):277-280
    Department of Infectious Diseases and Neuroinfections, Medical University of Bialystok, Poland.
    Background: Hard ticks are the main vectors of tick-borne encephalitis virus (TBEV). Free carnitine (FC) and acylcarnitines (AC) have the basic role in β-oxidation as well as the modulation of immune and nervous system. Homeostasis of carnitines in the TBE patients was not studied so far. Read More

    High-resolution physicochemical characterization of different intravenous immunoglobulin products.
    PLoS One 2017 31;12(7):e0181251. Epub 2017 Jul 31.
    Research, Momenta Pharmaceuticals, Cambridge, Massachusetts, United States of America.
    Intravenous immunoglobulin (IVIg) is a complex mixture drug comprising diverse immunoglobulins and non-IgG proteins purified from the plasma of thousands of healthy donors. Approved IVIg products on the market differ regarding source of plasma, isolation process, and formulation. These products are used widely, and often interchangeably, for the treatment of immunodeficiency and autoimmune and inflammatory diseases, but their mechanisms of action in different indications are not well understood. Read More

    Complement regulator CD59 prevents peripheral organ injury in rats made seropositive for neuromyelitis optica immunoglobulin G.
    Acta Neuropathol Commun 2017 Jul 27;5(1):57. Epub 2017 Jul 27.
    Departments of Medicine and Physiology, University of California, 1246 Health Sciences East Tower, San Francisco, CA, 94143-0521, USA.
    Pathogenesis in aquaporin-4 immunoglobulin G (AQP4-IgG) seropositive neuromyelitis optica spectrum disorders (herein called NMO) involves complement-dependent cytotoxicity initiated by AQP4-IgG binding to astrocyte AQP4. We recently reported that rats lacking complement inhibitor protein CD59 were highly susceptible to development of NMO pathology in brain and spinal cord following direct AQP4-IgG administration (Yao and Verkman, Acta Neuropath Commun 2017, 5:15). Here, we report evidence that CD59 is responsible for protection of peripheral, AQP4-expressing tissues in seropositive NMO. 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

    Anti-Neu5Gc and anti-non-Neu5Gc antibodies in healthy humans.
    PLoS One 2017 17;12(7):e0180768. Epub 2017 Jul 17.
    Thomas E. Starzl Transplantation Institute, University of Pittsburgh, Pittsburgh, PA, United States of America.
    Our group previously investigated the levels of anti-Gal and anti-nonGal IgM and IgG in a cohort of 75 healthy humans of various backgrounds, and found some significant differences related to factors such as age, gender, ABO blood group, diet, vaccination history, and geographic location during childhood. We have now expanded our cohort (n = 84) to investigate the levels of anti-Neu5Gc and anti-nonGal/nonNeu5Gc antibodies in healthy humans. Anti-nonGal and anti-nonGal/nonNeu5Gc human IgM and IgG binding to pRBCs and pAECs from GTKO/CD46 and GTKO/CD46/Neu5GcKO pigs were measured by flow cytometry. Read More

    Screening protocols to monitor respiratory status in primary immunodeficiency disease: findings from a European survey and subclinical infection working group.
    Clin Exp Immunol 2017 Nov 25;190(2):226-234. Epub 2017 Aug 25.
    Jeroen Bosch Academy, Jeroen Bosch Hospital, 's-Hertogenbosch, the Netherlands.
    Many patients with primary immunodeficiency (PID) who have antibody deficiency develop progressive lung disease due to underlying subclinical infection and inflammation. To understand how these patients are monitored we conducted a retrospective survey based on patient records of 13 PID centres across Europe, regarding the care of 1061 adult and 178 paediatric patients with PID on immunoglobulin (Ig) G replacement. The most common diagnosis was common variable immunodeficiency in adults (75%) and hypogammaglobulinaemia in children (39%). Read More

    A monocyte-TNF-endothelial activation axis in sickle transgenic mice: Therapeutic benefit from TNF blockade.
    Am J Hematol 2017 Nov 29;92(11):1119-1130. Epub 2017 Jul 29.
    Division of Hematology-Oncology-Transplantation, Department of Medicine, University of Minnesota Medical School, Minneapolis, Minnesota.
    Elaboration of tumor necrosis factor (TNF) is a very early event in development of ischemia/reperfusion injury pathophysiology. Therefore, TNF may be a prominent mediator of endothelial cell and vascular wall dysfunction in sickle cell anemia, a hypothesis we addressed using NY1DD, S+SAntilles , and SS-BERK sickle transgenic mice. Transfusion experiments revealed participation of abnormally activated blood monocytes exerting an endothelial activating effect, dependent upon Egr-1 in both vessel wall and blood cells, and upon NFκB(p50) in a blood cell only. Read More

    A multicentre, prospective, non-randomized, sequential, open-label trial to demonstrate the bioequivalence between intravenous immunoglobulin new generation (IGNG) and standard IV immunoglobulin (IVIG) in adult patients with primary immunodeficiency (PID).
    Rev Med Interne 2017 Sep 3;38(9):578-584. Epub 2017 Jul 3.
    LFB BIOMEDICAMENTS, Immunology Therapeutic Unit, Courtabœuf, France.
    Objectives: To demonstrate the bioequivalence between 2 intravenous immunoglobulin (IVIG) preparations, TEGELINE® and ClairYg®, a ready-to-use 5% IVIG, in primary immunodeficiency (PID). Secondary objectives were to assess the efficacy, safety and pharmacokinetics of ClairYg®.

    Methods: Twenty-two adult PID patients receiving stable doses of TEGELINE® (5% lyophilized IVIG) were switched to ClairYg® for 6 months. Read More

    Vitronectin Regulates the Fibrinolytic System during the Repair of Cerebral Cortex in Stab-Wounded Mice.
    J Neurotrauma 2017 Nov 7;34(22):3183-3191. Epub 2017 Aug 7.
    1 Graduate School of Humanities and Sciences, Ochanomizu University , Otsuka, Bunkyo-ku, Tokyo, Japan .
    Vitronectin (VN), one of the serum proteins, is known to be involved in the regulation of blood coagulation, fibrinolysis, and cell migration. It has been proposed that the regulation of fibrinolysis by VN promotes the blood-brain barrier (BBB) recovery from brain injuries such as traumatic injury and subarachnoid hemorrhage. The effects of VN on fibrinolysis in the injured brain remain unclear, however. Read More

    Report of an unsual case of anophthalmia and craniofacial cleft in a newborn with Toxoplasma gondii congenital infection.
    BMC Infect Dis 2017 Jul 3;17(1):459. Epub 2017 Jul 3.
    Laboratory Inmunología Experimental, Instituto Nacional de Pediatría (INP), Secretaría de Salud, Torre de Investigación, Av. Insurgentes Sur 3700-C, Col Insurgentes Cuicuilco, 04530, Ciudad de México, DF, Mexico.
    Background: We present one unusual case of anophthalmia and craniofacial cleft, probably due to congenital toxoplasmosis only.

    Case Presentation: A two-month-old male had a twin in utero who disappeared between the 7th and the 14th week of gestation. At birth, the baby presented anophthalmia and craniofacial cleft, and no sign compatible with genetic or exposition/deficiency problems, like the Wolf-Hirschhorn syndrome or maternal vitamin A deficiency. Read More

    A COMPARISON OF RAPID DIAGNOSTIC TESTING (BY PLASMODIUM LACTATE DEHYDROGENASE), AND QUANTITATIVE BUFFY COAT TECHNIQUE IN MALARIA DIAGNOSIS IN CHILDREN.
    Afr J Infect Dis 2017 8;11(2):31-38. Epub 2017 Jun 8.
    Adjunct Professor, Department of Paediatrics, College of Medicine, University of Ibadan, University College Hospital, Ibadan.
    Background: The World Health Organization (WHO) considers early and rapid diagnosis as one of the strategies to control malaria. This study compared the performance of Quantitative Buffy Coat (QBC) test and the Plasmodium lactate dehydrogenase (pLDH) rapid diagnostic test (RDT) with microscopy as the gold standard.

    Materials And Methods: The study involved children ages 0-5 years who presented with a history of fever at the University College Hospital, Ibadan, Nigeria. Read More

    Importance of neonatal immunoglobulin transfer for hippocampal development and behaviour in the newborn pig.
    PLoS One 2017 28;12(6):e0180002. Epub 2017 Jun 28.
    Department of Biology, Lund University, Lund, Sweden.
    Neurological disorders are among the main clinical problems affecting preterm children and often result in the development of communication and learning disabilities later in life. Several factors are of importance for brain development, however the role of immunoglobulins (passive immunity transfer) has not yet been investigated. Piglets are born agammaglobulinemic, as a result of the lack of transfer of maternal immunoglobulins in utero, thus, they serve as an ideal model to mimic the condition of immunoglobulin deficiency in preterm infants. Read More

    Absence of γ-Chain in Keratinocytes Alters Chemokine Secretion, Resulting in Reduced Immune Cell Recruitment.
    J Invest Dermatol 2017 Oct 17;137(10):2120-2130. Epub 2017 Jun 17.
    Institute of Immunity and Transplantation, University College London, London, UK; Department of Immunology, Royal Free Hospital Foundation Trust, London, UK. Electronic address:
    Loss-of-function mutations in the common gamma (γc) chain cytokine receptor subunit give rise to severe combined immunodeficiency characterized by lack of T and natural killer cells and infant death from infection. Hematopoietic stem cell transplantation or gene therapy offer a cure, but despite successful replacement of lymphoid immune lineages, a long-term risk of severe cutaneous human papilloma virus infections persists, possibly related to persistent γc-deficiency in other cell types. Here we show that keratinocytes, the only cell type directly infected by human papilloma virus, express functional γc and its co-receptors. Read More

    Hypersensitivity pneumonitis onset and severity is regulated by CD103 dendritic cell expression.
    PLoS One 2017 19;12(6):e0179678. Epub 2017 Jun 19.
    Institut Universitaire de Cardiologie et de Pneumologie de Québec, Université Laval, Quebec, Quebec, Canada.
    Background: Pulmonary dendritic cells drive lung responses to foreign antigens, including Saccharopolyspora rectivirgula, a causative agent of hypersensitivity pneumonitis. While the airway inflammatory mechanisms involved in hypersensitivity pneumonitis are well described, the mechanisms leading to the break in homeostasis and hypersensitivity pneumonitis onset are not well-described, and could involve CD103+ dendritic cells, which are found at baseline and during inflammatory responses in the lung. However, recent demonstration of the ability of CD103+ dendritic cells to induce inflammatory responses starkly contrasts with their classically described role as regulatory cells. Read More

    1,25-dihydroxyvitamin D3 -induced dendritic cells suppress experimental autoimmune encephalomyelitis by increasing proportions of the regulatory lymphocytes and reducing T helper type 1 and type 17 cells.
    Immunology 2017 Nov 10;152(3):414-424. Epub 2017 Jul 10.
    Department of Neurology and Neuroscience Centre, The First Hospital of Jilin University, Changchun, China.
    Dendritic cells (DCs), a bridge for innate and adaptive immune responses, play a key role in the development of multiple sclerosis (MS) and experimental autoimmune encephalomyelitis (EAE), an animal model for MS. Administration of tolerogenic DCs has been used as an immunotherapy in autoimmune diseases. Deficiency of vitamin D is an environmental risk factor of MS. Read More

    Measurement of the IgG2 response to Pneumococcal capsular polysaccharides may identify an antibody deficiency in individuals referred for immunological investigation.
    J Immunoassay Immunochem 2017 14;38(5):514-522. Epub 2017 Jun 14.
    a The Binding Site Group Limited , Edgbaston , Birmingham.
    IgG2 is the most efficient subclass for providing protection against pneumococcal pathogens. We hypothesised that some individuals may be unable to mount an effective pneumococcal capsular polysaccharide (PCP) IgG2 response despite having a normal PCP IgG concentration (PCP IgG2 deficient). The median pre-vaccination PCP IgG2 concentration was significantly lower in individuals referred for immunological investigation compared to healthy controls (2. Read More

    A Nonadjuvanted IgG2a Monoclonal Antibody against Nucleosomes Elicits Potent T Cell-Dependent, Idiotype-Specific IgG1 Responses and Glomerular IgG1/IgG2a Deposits in Normal Mice.
    J Immunol 2017 Jul 7;199(2):489-500. Epub 2017 Jun 7.
    Institute of Pathology, Oslo University Hospital, University of Oslo, 0372 Oslo, Norway.
    Idiotypes (Ids) are unique epitopes of Ab V regions and can trigger anti-Id immune responses, but immunization with several nonadjuvanted isologous IgG mAbs has induced tolerance to their Ids. We immunized non-lupus-prone mice with 11 allotype "a" of IgG2a (IgG2aa) and 4 IgG2c nonadjuvanted, isologous mAbs purified from serum-free medium. Of five IgG2aa mAbs with specificity for nucleosomes, the repeating histone-DNA subunit of chromatin, four elicited an IgG1 anti-mAb response and one mAb was nonimmunogenic. Read More

    Casual or Causal? Two Unique Cases of Hodgkin's Lymphoma: A Case Report and Literature Review.
    Am J Case Rep 2017 May 19;18:553-557. Epub 2017 May 19.
    Department of Internal Medicine, Abington Hospital Jefferson Health, Abington, PA, USA.
    BACKGROUND Immunosuppressive diseases and therapies have long been connected to risk of malignancies, especially lymphoma. With some diseases and drugs, the association is well established but the data is mostly anecdotal because of the rarity of the situation. CASE REPORT We present 2 rare cases. Read More

    Frequency and epitope specificity of anti-factor VIII C1 domain antibodies in acquired and congenital hemophilia A.
    Blood 2017 08 15;130(6):808-816. Epub 2017 May 15.
    Department of Pediatrics, Clinical and Molecular Hemostasis, Frankfurt University Hospital, Frankfurt am Main, Germany.
    Several studies showed that neutralizing anti-factor VIII (anti-fVIII) antibodies (inhibitors) in patients with acquired hemophilia A (AHA) and congenital hemophilia A (HA) are primarily directed to the A2 and C2 domains. In this study, the frequency and epitope specificity of anti-C1 antibodies were analyzed in acquired and congenital hemophilia inhibitor patients (n = 178). The domain specificity of antibodies was studied by homolog-scanning mutagenesis (HSM) with single human domain human/porcine fVIII proteins and antibody binding to human A2, C1, and C2 domains presented as human serum albumin (HSA) fusion proteins. Read More

    Self-reactive VH4-34-expressing IgG B cells recognize commensal bacteria.
    J Exp Med 2017 Jul 12;214(7):1991-2003. Epub 2017 May 12.
    Department of Immunobiology, Yale University School of Medicine, New Haven, CT 06510
    The germline immunoglobulin (Ig) variable heavy chain 4-34 (VH4-34) gene segment encodes in humans intrinsically self-reactive antibodies that recognize I/i carbohydrates expressed by erythrocytes with a specific motif in their framework region 1 (FWR1). VH4-34-expressing clones are common in the naive B cell repertoire but are rarely found in IgG memory B cells from healthy individuals. In contrast, CD27+IgG+ B cells from patients genetically deficient for IRAK4 or MYD88, which mediate the function of Toll-like receptors (TLRs) except TLR3, contained VH4-34-expressing clones and showed decreased somatic hypermutation frequencies. Read More

    A Case Report of Hypoglycemia and Hypogammaglobulinemia: DAVID Syndrome in a Patient With a Novel NFKB2 Mutation.
    J Clin Endocrinol Metab 2017 Jul;102(7):2127-2130
    Division of Allergy, Immunology & Rheumatology, Department of Pediatrics, Stanford University School of Medicine, Stanford, California 94305.
    Context: Deficient anterior pituitary with variable immune deficiency (DAVID) syndrome is a rare disorder in which children present with symptomatic adrenocorticotropic hormone (ACTH) deficiency preceded by hypogammaglobulinemia from B-cell dysfunction with recurrent infections, called common variable immunodeficiency (CVID). Subsequent whole exome sequencing studies have revealed germline heterozygous C-terminal mutations of NFKB2 as a cause of DAVID syndrome or of CVID without clinical hypopituitarism. However, to the best of our knowledge there have been no cases in which the endocrinopathy has presented in the absence of a prior clinical history of CVID. Read More

    Implementation of National Institute for Health and Care Excellence (NICE) guidance to measure immunoglobulin A with all coeliac screens: can an affordable solution be devised?
    Clin Exp Immunol 2017 Sep 19;189(3):352-358. Epub 2017 May 19.
    Department of Immunology, Eastbourne Hospital, Eastbourne, East Sussex, UK.
    There has been a dramatic increase in requests for coeliac disease (CD) serological screening using immunoglobulin (Ig)A tissue transglutaminase antibodies (IgA-tTG). Recently, the UK National Institute for Health and Care Excellence has revised its guidance, recommending that total IgA should also be measured in all samples. This is justified, as false-negative results may occur with IgA deficiency. Read More

    Disease burden for patients with primary immunodeficiency diseases identified at reference hospitals in Guanajuato, Mexico.
    PLoS One 2017 27;12(4):e0175867. Epub 2017 Apr 27.
    Department of Immuno-Allergology Pediatrics, Hospital Pediátrico de León, León Guanajuato, México.
    Background: In addition to the deleterious effect on health, there is considerable economic and psychosocial morbidity associated with primary immunodeficiency diseases (PID). Also, the cost of a late diagnosis frequently results in a heavy disease burden on the patient. The objective of this study was to collect and analyze data on patients with PID in the state of Guanajuato in Mexico, to indirectly estimate the burden of the disease. Read More

    Acquired hemophilia A: a review of recent data and new therapeutic options.
    Hematology 2017 Oct 25;22(9):514-520. Epub 2017 Apr 25.
    a Italian National Blood Centre, National Institute of Health , Rome , Italy.
    Objectives: Acquired hemophilia A (AHA) is a rare, but potentially life-threatening, bleeding disorder caused by an autoantibody against factor VIII that interferes with its coagulant function.

    Methods: We performed a narrative review focusing on the diagnostic aspects of AHA and on the current treatment strategies with particular regard to new data and therapeutic developments.

    Results: The management of this severe hemorrhagic disorder is based on the control of bleeding with the use of bypassing agents and on the utilization of a variety of immunosuppressant agents with the goal of eliminating the autoantibody permanently. Read More

    The lack of BTK does not impair monocytes and polymorphonuclear cells functions in X-linked agammaglobulinemia under treatment with intravenous immunoglobulin replacement.
    PLoS One 2017 19;12(4):e0175961. Epub 2017 Apr 19.
    Department of Molecular Medicine, Sapienza University of Rome, Roma, Italy.
    The lack of BTK in X-linked agammaglobulinemia (XLA) patients does not affect monocytes and polymorphonuclear cells (PMN) phenotype and functions. In this study, we show that XLA patients had an increased frequency of the intermediate monocytes subset and that BTK-deficient monocytes and PMN had a normal expression of receptors involved in the activation and cellular responses. We demonstrate that BTK is not required for migration, phagocytosis and the production of reactive oxygen species (ROS) following engagement of FC gamma receptors (FcγR). Read More

    Heat stress decreases expression of the cytokines, avian β-defensins 4 and 6 and Toll-like receptor 2 in broiler chickens infected with Salmonella Enteritidis.
    Vet Immunol Immunopathol 2017 Apr 27;186:19-28. Epub 2017 Feb 27.
    Neuroimmunomodulation Research Group, Department of Pathology, School of Veterinary Medicine, University of São Paulo, São Paulo, SP, Brazil.
    A high ambient temperature is a highly relevant stressor in poultry production. Heat stress (HS) has been reported to reduce animal welfare, performance indices and increase Salmonella susceptibility. Salmonella spp. Read More

    The Pneumocell-study: Vaccination of IgG1- and IgG2-deficient patients with Prevnar13.
    Vaccine 2017 May 11;35(20):2654-2660. Epub 2017 Apr 11.
    Department of Laboratory Medicine, Division of Clinical Microbiology, Sweden. Electronic address:
    Background: Patients with IgG-deficiency often suffer from repeated bacterial infections with S. pneumoniae. Since there is a lack of knowledge regarding whether IgG-deficient patients would benefit from conjugate pneumococcal vaccination, we set out to evaluate the effect of Prevnar13 vaccination in IgG1- and/or IgG2-deficient patients. Read More

    Megakaryocytes compensate for Kit insufficiency in murine arthritis.
    J Clin Invest 2017 May 4;127(5):1714-1724. Epub 2017 Apr 4.
    The growth factor receptor Kit is involved in hematopoietic and nonhematopoietic development. Mice bearing Kit defects lack mast cells; however, strains bearing different Kit alleles exhibit diverse phenotypes. Herein, we investigated factors underlying differential sensitivity to IgG-mediated arthritis in 2 mast cell-deficient murine lines: KitWsh/Wsh, which develops robust arthritis, and KitW/Wv, which does not. Read More

    Patient Characteristics and Individualization of Biologic Therapy.
    Immunol Allergy Clin North Am 2017 May 1;37(2):261-281. Epub 2017 Mar 1.
    Division of Allergy and Immunology, New Jersey Medical School, Newark, NJ, USA. Electronic address:
    Progress in the understanding of disease processes has provided additional therapeutic targets, best exemplified by the increasing role of biologics in the clinical armamentarium. This article provides a focused review of current treatment paradigms and pathophysiology for asthma, atopic dermatitis, urticaria, as well as C1 inhibitor deficiency. It elucidates the populations in which biologics were studied for the aforementioned disease states, emphasizing characteristics to consider when selecting therapy. Read More

    Long-term Clinical Outcome of Antibody Replacement Therapy in Humoral Immunodeficient Adults With Respiratory Tract Infections.
    EBioMedicine 2017 Apr 21;18:254-260. Epub 2017 Mar 21.
    Department of Medical Microbiology and Immunology, St. Antonius Hospital, Nieuwegein, The Netherlands; Department of Science, University College Roosevelt, Middelburg, The Netherlands.
    In severe humoral immunodeficiency the indication for antibody replacement therapy (ART) is clear, and supported by several large studies. However, for milder forms of humoral immunodeficiency, the indication for ART is less clear. This is a retrospective cohort study of 87 adults with recurrent respiratory tract infections who received ART. Read More

    Clinical Profile, Dosing, and Quality-of-Life Outcomes in Primary Immune Deficiency Patients Treated at Home with Immunoglobulin G: Data from the IDEaL Patient Registry.
    J Manag Care Spec Pharm 2017 Apr;23(4):400-406
    1 Coram/CVS specialty infusion services, Denver, Colorado.
    Background: Patients with primary immune deficiency (PID) often require immunoglobulin G (IgG, commonly referred to as Ig) replacement therapy to prevent infections and associated comorbidities. Ig therapy can be given either through intravenous or subcutaneous routes, and both can be done in the home setting. There is limited information available on the real-world diagnosis, management, and outcomes of this patient population, given the variable disease presentation and treatment options. Read More

    Biochemical characterization and stability of immune globulin intravenous 10% liquid (Panzyga®).
    Biologicals 2017 Jan 27;45:33-38. Epub 2016 Oct 27.
    Octapharma Pharmazeutika Produktionsgesellschaft m.b.H., Vienna, Austria.
    Panzyga® is a new glycine-formulated immune globulin intravenous 10% liquid for the treatment of patients suffering from immunodeficiencies and autoimmune diseases. Panzyga® is a high purity, native and functional IgG product with an IgG subclass distribution equivalent to normal plasma. The levels of hemagglutinins and accompanying plasma proteins (including IgA and IgM) are low. Read More

    Changes in the Level of Immunoglobulins and CD4/CD8 Ratio in Young and Aged Mice with Estradiol Deficiency.
    Immunol Invest 2017 Apr 7;46(3):305-313. Epub 2017 Feb 7.
    a Department of Obstetrics and Gynecology , Jinhua Municipal Central Hospital , Zhejiang Province , People's Republic of China.
    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. Read More

    Serial Serum Immunoglobulin G (IgG) Trough Levels in Patients with X-linked Agammaglobulinemia on Replacement Therapy with Intravenous Immunoglobulin: Its Correlation with Infections in Indian Children.
    J Clin Immunol 2017 Apr 21;37(3):311-318. Epub 2017 Mar 21.
    Division of Basic and Clinical Immunology, University of California, Irvine, CA, USA.
    Patients with primary antibody deficiency (PAD) are being increasingly diagnosed in the developing world. However, care of these children continues to remain suboptimal due to financial and social constraints. Immunoglobulin (Ig) trough level is an important predicting factor for infections in children on replacement immunoglobulin therapy. Read More

    Evaluation of the Safety, Tolerability, and Pharmacokinetics of Gammaplex® 10% Versus Gammaplex® 5% in Subjects with Primary Immunodeficiency.
    J Clin Immunol 2017 Apr 18;37(3):301-310. Epub 2017 Mar 18.
    Section of Allergy and Immunology, Department of Immunology and Microbiology, Rush University Medical Center, Chicago, IL, USA.
    Purpose: This phase 3, multicenter, open-label, randomized, two-period, crossover bioequivalence trial evaluated the safety, tolerability, and pharmacokinetics of intravenous immunoglobulins (IVIGs) Gammaplex 5% and Gammaplex 10% in 33 adults and 15 children with primary immunodeficiency diseases (PIDs).

    Methods: Eligible adults received five Gammaplex 5% infusions followed by five Gammaplex 10% infusions, or vice versa, stratified by a 21- or 28-day dosing regimen. Pediatric subjects received five Gammaplex 10% infusions only. Read More

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