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    4977 results match your criteria Immunoglobulin G Deficiency

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    Recombinant human hyaluronidase-facilitated subcutaneous immunoglobulin infusion in primary immunodeficiency diseases.
    Immunotherapy 2017 Sep 5. 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

    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

    Vitronectin Regulates the Fibrinolytic System during the Repair of Cerebral Cortex in Stab-Wounded Mice.
    J Neurotrauma 2017 Aug 7. 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

    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

    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

    Frequency and epitope specificity of anti-factor VIII C1 domain antibodies in acquired and congenital hemophilia A.
    Blood 2017 Aug 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

    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

    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

    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

    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

    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

    Complement drives glucosylceramide accumulation and tissue inflammation in Gaucher disease.
    Nature 2017 03 22;543(7643):108-112. Epub 2017 Feb 22.
    Division of Human Genetics, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio 45229, USA.
    Gaucher disease is caused by mutations in GBA1, which encodes the lysosomal enzyme glucocerebrosidase (GCase). GBA1 mutations drive extensive accumulation of glucosylceramide (GC) in multiple innate and adaptive immune cells in the spleen, liver, lung and bone marrow, often leading to chronic inflammation. The mechanisms that connect excess GC to tissue inflammation remain unknown. Read More

    Development of a complete human IgG monoclonal antibody to transferrin receptor 1 targeted for adult T-cell leukemia/lymphoma.
    Biochem Biophys Res Commun 2017 Mar 8;485(1):144-151. Epub 2017 Feb 8.
    Division of Tumor and Cellular Biochemistry, Department of Medical Science, Faculty of Medicine, University of Miyazaki, Japan. Electronic address:
    Iron is an essential nutrient for normal cell growth, and reprogramming of iron metabolism is essential to tumor cell survival and progression. HTLV-1-associated adult T-cell leukemia/lymphoma (ATLL) has no effective therapy and high levels of cell surface transferrin receptor 1 (TFR1) expression have been reported in ATLL by us and other groups. In this study, to develop a novel molecular-targeted therapy against TFR1 to modulate iron metabolism, we initially determined the expression pattern of several iron-related genes along with TFR1 and found that ATLL cells presented characteristic of an iron-deficiency state such as high expression of iron-regulatory protein 2 (IRP2) and low expression of its E3 ubiquitin-ligase, FBXL5. Read More

    Determination of Anti-Anisakis Simplex Antibodies and Relationship with αβ and γδ Lymphocyte Subpopulations in Patients with Crohn's Disease.
    Dig Dis Sci 2017 Apr 6;62(4):934-943. Epub 2017 Feb 6.
    Research Department, Arnau de Vilanova Hospital, c/San Clemente 12, 46015, Valencia, Spain.
    Background: The etiology of Crohn's disease (CD) is still unknown although new theories are based on defects in innate immunity. We have previously shown a decrease in γδ T cells in CD patients. Previous studies have shown a high prevalence of anti-A. Read More

    Anti-BlyS antibody reduces the immune reaction against enzyme and enhances the efficacy of enzyme replacement therapy in Fabry disease model mice.
    Clin Immunol 2017 May 2;178:56-63. Epub 2017 Feb 2.
    Division of Gene Therapy, Research Center for Medical Sciences, The Jikei University School of Medicine, Tokyo, Japan; Department of Pediatrics, The Jikei University School of Medicine, Tokyo, Japan. Electronic address:
    Formation of antibodies against a therapeutic enzyme is an important complication during enzyme replacement therapy (ERT) for lysosomal storage diseases. Fabry disease (FD) is caused by a deficiency of alpha-galactosidase (GLA), which results in the accumulation of globotriaosylceramide (GL-3). We have shown immune tolerance induction (ITI) during ERT in FD model mice by using an anti-B lymphocyte stimulator (anti-BlyS) antibody (belimumab). Read More

    Mechanisms of action of intravenous immunoglobulin.
    Transfus Apher Sci 2017 Feb 30;56(1):45-49. Epub 2016 Dec 30.
    Université Paris Descartes, Faculté de Médecine, Service de Médecine Interne, Centre de référence pour les vascularites nécrosantes et la sclérodermie systémique, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Paris, France; Institut Cochin, INSERM U1016, CNRS UMR 8104, Université Paris Descartes, Paris, France. Electronic address:
    Taking advantage of the "World Apheresis Association/Société Française d'Hémaphérèse" meeting held in Paris in April 2016, this article reviews the current knowledge on the mechanisms of action of intravenous immunoglobulins. Immunoglobulins are a plasma-derived drug, which have been initially used as a replacement therapy for patients with antibody deficiency. Since 1980 they have also been used for their anti-inflammatory and immunomodulating efficacy in auto-immune diseases. Read More

    Audit of provincial IVIG Request Forms and efficacy documentation in four Ontario tertiary care centres.
    Transfus Med 2017 Apr 31;27(2):122-131. Epub 2017 Jan 31.
    McMaster Centre for Transfusion Research, McMaster University, Hamilton, Ontario, Canada.
    Objective: Retrospective audit of IVIG Request Forms in four Ontario tertiary care centres: to determine the case mix of new IVIG requests, to authenticate information provided, and to determine documentation of clinical efficacy.

    Aims: To understand contributors to increases in IVIG utilisation and to determine whether IVIG is being used and monitored appropriately.

    Introduction: Intravenous immunoglobulin (IVIG) use in Canada is high compared with other developed countries. Read More

    Ataxia-telangiectasia: Immunodeficiency and survival.
    Clin Immunol 2017 May 24;178:45-55. Epub 2017 Jan 24.
    Department of Neurology - Pediatric Neurology, Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center, Nijmegen, The Netherlands.
    Ataxia-telangiectasia (AT) is a neurodegenerative disorder characterized by ataxia, telangiectasia, and immunodeficiency. An increased risk of malignancies and respiratory diseases dramatically reduce life expectancy. To better counsel families, develop individual follow-up programs, and select patients for therapeutic trials, more knowledge is needed on factors influencing survival. Read More

    Efficacy and Safety of Subcutaneous Belimumab in Systemic Lupus Erythematosus: A Fifty-Two-Week Randomized, Double-Blind, Placebo-Controlled Study.
    Arthritis Rheumatol 2017 May 7;69(5):1016-1027. Epub 2017 Apr 7.
    GlaxoSmithKline, Philadelphia, Pennsylvania.
    Objective: To assess the efficacy and safety of subcutaneous (SC) belimumab in patients with systemic lupus erythematosus (SLE).

    Methods: Patients with moderate-to-severe SLE (score of ≥8 on the Safety of Estrogens in Lupus Erythematosus National Assessment [SELENA] version of the SLE Disease Activity Index [SLEDAI]) were randomized 2:1 to receive weekly SC belimumab 200 mg or placebo by prefilled syringe in addition to standard SLE therapy for 52 weeks. The primary end point was the SLE Responder Index (SRI4) at week 52. Read More

    Humoral Reactivity of Renal Transplant-Waitlisted Patients to Cells From GGTA1/CMAH/B4GalNT2, and SLA Class I Knockout Pigs.
    Transplantation 2017 Apr;101(4):e86-e92
    1 Department of Surgery, University of Alabama at Birmingham, Birmingham, AL. 2 Department of Surgery, Indiana University, School of Medicine, Indianapolis, IN. 3 Transplant Surgery, University of Alabama at Birmingham, Birmingham, AL.
    Background: Antipig antibodies are a barrier to clinical xenotransplantation. We evaluated antibody binding of waitlisted renal transplant patients to 3 glycan knockout (KO) pig cells and class I swine leukocyte antigens (SLA).

    Methods: Peripheral blood mononuclear cells from SLA identical wild type (WT), α1, 3-galactosyltransferase (GGTA1) KO, GGTA1/ cytidine monophosphate-N-acetylneuraminic acid hydroxylase (CMAH) KO, and GGTA1/ CMAH /b1,4 N-acetylgalactosaminyl transferase (B4GalNT2) KO pigs were screened for human antibody binding using flow cytometric crossmatch (FCXM). Read More

    Cathepsin K Deficiency Ameliorates Systemic Lupus Erythematosus-like Manifestations in Fas(lpr) Mice.
    J Immunol 2017 Mar 16;198(5):1846-1854. Epub 2017 Jan 16.
    Department of Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, MA 02115;
    Cysteinyl cathepsin K (CatK) is expressed in osteoclasts to mediate bone resorption, but is also inducible under inflammatory conditions. Fas(lpr) mice on a C57BL/6 background develop spontaneous systemic lupus erythematosus-like manifestations. Although normal mouse kidneys expressed negligible CatK, those from Fas(lpr) mice showed elevated CatK expression in the glomeruli and tubulointerstitial space. Read More

    Intravenous Immunoglobulin in the Treatment of Primary Immunodeficiency Diseases.
    Pediatr Ann 2017 Jan;46(1):e8-e12
    Intravenous immunoglobulin (IVIG) has been used as antibody replacement therapy in primary immunodeficiency diseases (PIDDs) for more than 50 years. Its role as a therapeutic agent has expanded over the past couple of decades as its anti-inflammatory and immune-modulatory mechanisms of action have been elucidated. It is now used "off-label" to treat other autoimmune diseases. Read More

    A New RNA-Based Adjuvant Enhances Virus-Specific Vaccine Responses by Locally Triggering TLR- and RLH-Dependent Effects.
    J Immunol 2017 Feb 11;198(4):1595-1605. Epub 2017 Jan 11.
    Institute for Experimental Infection Research, TWINCORE, Centre for Experimental and Clinical Infection Research, a Joint Venture between the Hannover Medical School and the Helmholtz Centre for Infection Research, D-30625 Hannover, Germany;
    Among innovative adjuvants conferring a Th1-shift, RNAdjuvant is a promising candidate. This adjuvant consists of a 547-nt uncapped noncoding ssRNA containing polyU repeats that is stabilized by a cationic carrier peptide. Whereas vaccination of mice with an influenza subunit vaccine induced moderate virus-specific IgG1, vaccination together with RNAdjuvant significantly enhanced this IgG1 and additionally promoted the formation of IgG2b/c, which is indicative of Th1 responses. Read More

    Rapid infusions of human normal immunoglobulin 50g/l are safe and well tolerated in immunodeficiencies and immune thrombocytopenia.
    Int Immunopharmacol 2017 Mar 7;44:38-42. Epub 2017 Jan 7.
    Department of Molecular Medicine, "Sapienza" University of Rome, Italy.
    Intravenous immunoglobulin (IVIg) is accepted as an effective and well-tolerated treatment for primary and secondary immunodeficiencies (ID) and immune thrombocytopenia (ITP). Adverse reactions of IVIg are usually mild, comprising transient flu-like symptoms, change in blood pressure and tachycardia. However IVIg therapy can be burdensome for both patients and healthcare facilities, since the infusion may take up to 4h to administer. Read More

    Frequent Infections, Hypotonia, and Anemia in a Breastfed Infant.
    J Pediatr Hematol Oncol 2017 Mar;39(2):141-142
    *Pediatrics Department, Hedi Chaker Hospital †Faculty of Medicine of Sfax ‡Hematology Laboratory, Habib Bourguiba Hospital, Sfax, Tunisia.
    Vitamin B12 deficiency may be responsible of serious hematologic and neurodevelopmental abnormalities. We report the case of an infant who was hospitalized because of recurrent infections, failure to thrive, hypotonia, and weakness. He was 8 months old and had been exclusively breastfed. Read More

    Update on the use of immunoglobulin in human disease: A review of evidence.
    J Allergy Clin Immunol 2017 Mar 29;139(3S):S1-S46. Epub 2016 Dec 29.
    Department of Pediatrics, Division of Allergy & Immunology, University of South Florida, Morsani College of Medicine, Johns Hopkins All Children's Hospital, St Petersburg, Fla.
    Human immunoglobulin preparations for intravenous or subcutaneous administration are the cornerstone of treatment in patients with primary immunodeficiency diseases affecting the humoral immune system. Intravenous preparations have a number of important uses in the treatment of other diseases in humans as well, some for which acceptable treatment alternatives do not exist. We provide an update of the evidence-based guideline on immunoglobulin therapy, last published in 2006. Read More

    Effect of Continuous B Cell Depletion With Rituximab on Pathogenic Autoantibodies and Total IgG Levels in Antineutrophil Cytoplasmic Antibody-Associated Vasculitis.
    Arthritis Rheumatol 2017 May 31;69(5):1045-1053. Epub 2017 Mar 31.
    Massachusetts General Hospital, Boston.
    Objective: To evaluate the effect of rituximab on pathogenic autoantibodies and total Ig levels, and to identify serious adverse events in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) treated with continuous B cell depletion.

    Methods: We conducted a retrospective analysis of 239 patients with AAV treated with rituximab-induced continuous B cell depletion. Two treatment cohorts were analyzed: an induction group (n = 52) and a maintenance group (n = 237). Read More

    When to initiate immunoglobulin replacement therapy (IGRT) in antibody deficiency: a practical approach.
    Clin Exp Immunol 2017 Jun 30;188(3):333-341. Epub 2017 Jan 30.
    Department of Clinical Immunology and Allergology, St Annes's University Hospital, Faculty of Medicine, Masaryk University, Brno, Czech Republic.
    Primary antibody deficiencies (PAD) constitute the majority of all primary immunodeficiency diseases (PID) and immunoglobulin replacement forms the mainstay of therapy for many patients in this category. Secondary antibody deficiencies (SAD) represent a larger and expanding number of patients resulting from the use of a wide range of immunosuppressive therapies, in particular those targeting B cells, and may also result from renal or gastrointestinal immunoglobulin losses. While there are clear similarities between primary and secondary antibody deficiencies, there are also significant differences. Read More

    Acquired Dysfibrinogenemia Caused by Autoantibody Inhibiting Fibrin Polymerization in a Patient with MELAS Syndrome and Bleeding Tendency.
    Ann Clin Lab Sci 2016 Dec;46(6):696-700
    Department of Laboratory Medicine, Seoul National University Hospital, Seoul, Korea
    We present a case of acquired dysfibrinogenemia caused by an autoantibody that inhibited fibrin polymerization in a patient previously diagnosed with MELAS (mitochondrial myopathy, encephalopathy, lactic acidosis, stroke-like episodes). The patient showed prolonged PT, aPTT, and thrombin time. There was no factor deficiency but fibrinogen antigen and activity were decreased. Read More

    Intravenous and subcutaneous immunoglobulin G replacement therapy.
    Allergy Asthma Proc 2016 Nov;37(6):426-431
    Clinical Immunology Program, Boston Childrens Hospital, Boston, Massachusetts, USA.
    Human polyclonal immunoglobulin G (IgG) for therapeutic use has been available for decades. This drug was developed for treatment of antibody deficiency (replacement therapy), although its use has expanded into many anti-inflammatory and immunomodulatory applications in recent years. This review focuses on IgG prescribing for replacement therapy. Read More

    Effect of Immunosuppressive Drugs on the Changes of Serum Galactose-Deficient IgA1 in Patients with IgA Nephropathy.
    PLoS One 2016 8;11(12):e0166830. Epub 2016 Dec 8.
    Department of Infection, Immunity & Inflammation, University of Leicester, Leicester, United Kingdom.
    Galactose-deficient IgA1 (Gd-IgA1) and IgA-IgG complexes are known to play an important role in the pathogenesis of IgA nephropathy (IgAN). We aimed therefore to determine the impact of immunosuppression on the serum levels of Gd-IgA1, total IgA1 and IgA-IgG complexes in IgAN patients. In a retrospective study, serum samples from IgAN patients collected before transplantation (t0) and at 3- and 6-month posttransplant (t3 & t6) were used to measure the levels of Gd-IgA1, total IgA1 and IgA-IgG complexes. Read More

    Shift from intravenous or 16% subcutaneous replacement therapy to 20% subcutaneous immunoglobulin in patients with primary antibody deficiencies.
    Int J Immunopathol Pharmacol 2017 Mar 7;30(1):73-82. Epub 2016 Dec 7.
    1 Anna Meyer Children's Hospital, University of Florence, Florence, Italy.
    In patients with primary antibody deficiencies, subcutaneous administration of IgG (SCIG) replacement is effective, safe, well-tolerated, and can be self-administered at home. A new SCIG replacement at 20% concentration (Hizentra(®)) has been developed and has replaced Vivaglobin(®) (SCIG 16%). An observational prospective multi-centric open-label study, with retrospective comparison was conducted in 15 Italian centers, in order to investigate whether and to what extent switching to Hizentra(®) would affect frequency of infusions, number of infusion sites, patients' satisfaction, and tolerability in patients previously treated with Vivaglobin(®) or intravenous immunoglobulins (IVIG). Read More

    Relationships between Mucosal Antibodies, Non-Typeable Haemophilus influenzae (NTHi) Infection and Airway Inflammation in COPD.
    PLoS One 2016 29;11(11):e0167250. Epub 2016 Nov 29.
    Clinical & Experimental Sciences, University of Southampton Faculty of Medicine, Southampton General Hospital, Tremona Road, Southampton, United Kingdom.
    Non-typeable Haemophilus influenzae (NTHi) is a key pathogen in COPD, being associated with airway inflammation and risk of exacerbation. Why some patients are susceptible to colonisation is not understood. We hypothesised that this susceptibility may be due to a deficiency in mucosal humoral immunity. Read More

    Divergent clinical outcomes of alpha-glucosidase enzyme replacement therapy in two siblings with infantile-onset Pompe disease treated in the symptomatic or pre-symptomatic state.
    Mol Genet Metab Rep 2016 Dec 18;9:98-105. Epub 2016 Nov 18.
    Showa University Northern Yokohama Hospital, Children Medical Center, Yokohama, Kanagawa, Japan.
    Pompe disease is an autosomal recessive, lysosomal glycogen storage disease caused by acid α-glucosidase deficiency. Infantile-onset Pompe disease (IOPD) is the most severe form and is characterized by cardiomyopathy, respiratory distress, hepatomegaly, and skeletal muscle weakness. Untreated, IOPD generally results in death within the first year of life. Read More

    Protective Immunity Elicited by Oral Immunization of Mice with Salmonella enterica Serovar Typhimurium Braun Lipoprotein (Lpp) and Acetyltransferase (MsbB) Mutants.
    Front Cell Infect Microbiol 2016 10;6:148. Epub 2016 Nov 10.
    Department of Microbiology and Immunology, University of Texas Medical BranchGalveston, TX, USA; Institute for Human Infections and Immunity, University of Texas Medical BranchGalveston, TX, USA; Sealy Center for Vaccine Development and World Health Organisation Collaborating Center for Vaccine Research, University of Texas Medical BranchGalveston, TX, USA; Center for Biodefense and Emerging Infectious Diseases, University of Texas Medical BranchGalveston, TX, USA.
    We evaluated the extent of attenuation and immunogenicity of the ΔlppAB and ΔlppAB ΔmsbB mutants of Salmonella enterica serovar Typhimurium when delivered to mice by the oral route. These mutants were deleted either for the Braun lipoprotein genes (lppA and lppB) or in combination with the msbB gene, which encodes an acetyltransferase required for lipid A modification of lipopolysaccharide. Both the mutants were attenuated (100% animal survival) and triggered robust innate and adaptive immune responses. Read More

    Roles of Aluminum Hydroxide and Monophosphoryl Lipid A Adjuvants in Overcoming CD4+ T Cell Deficiency To Induce Isotype-Switched IgG Antibody Responses and Protection by T-Dependent Influenza Vaccine.
    J Immunol 2017 Jan 23;198(1):279-291. Epub 2016 Nov 23.
    Center for Inflammation, Immunity and Infection, Institute for Biomedical Sciences, Georgia State University, Atlanta, GA 30303; and
    Vaccine adjuvant effects in the CD4-deficient condition largely remain unknown. We investigated the roles of combined monophosphoryl lipid A (MPL) and aluminum hydroxide (Alum) adjuvant (MPL+Alum) in inducing immunity after immunization of CD4 knockout (CD4KO) and wild-type (WT) mice with T-dependent influenza vaccine. MPL+Alum adjuvant mediated IgG isotype-switched Abs, IgG-secreting cell responses, and protection in CD4KO mice, which were comparable to those in WT mice. Read More

    Developing an Inactivated Rotavirus Vaccine and Evaluating the Immunogenicity Against a Commercially Available Attenuated Rotavirus Vaccine Using a Mice Animal Model.
    Viral Immunol 2016 Dec 18;29(10):565-571. Epub 2016 Nov 18.
    1 Research and Development Sector, Egyptian Company for Production of Vaccines, Sera and Drugs-EgyVac (VACSERA Holding Company) , Giza, Egypt .
    There is a high demand for public immunization against Rotavirus (RV), especially in Africa. In Africa, the attenuated RV vaccination is contraindicated in patients with immune diseases and nutrition deficiency. Therefore, the inactivated RV vaccine (IRVV) could be an alternative. Read More

    One-Year Tuberculosis Risk in Rheumatoid Arthritis Patients Starting Their First Tumor Necrosis Factor Inhibitor Therapy from 2008 to 2012 in Taiwan: A Nationwide Population-Based Cohort Study.
    PLoS One 2016 10;11(11):e0166339. Epub 2016 Nov 10.
    Division of Allergy, Immunology and Rheumatology, Department of Internal Medicine, Taichung Veterans General Hospital, Taichung, Taiwan.
    Objective: To investigate the risk of tuberculosis (TB) among rheumatoid arthritis (RA) patients within 1 year after initiation of tumor necrosis factor inhibitor (TNFi) therapy from 2008 to 2012.

    Methods: We used the 2003-2013 Taiwanese National Health Insurance Research Database to identify RA patients who started any RA-related medical therapy from 2008 to 2012. Those who initiated etanercept or adalimumab therapy during 2008-2012 were selected as the TNFi group and those who never received biologic disease-modifying anti-rheumatic drug therapy were identified as the comparison group after excluding the patients who had a history of TB or human immunodeficiency virus infection/acquired immune deficiency syndrome. Read More

    Practical Considerations for Self-Administration of Subcutaneous Immunoglobulin G Utilizing Recombinant Human Hyaluronidase, an Advanced Method of Subcutaneous Administration: A Nurse's Perspective.
    J Infus Nurs 2016 Nov/Dec;39(6):359-368
    Allergy Partners of North Texas Research, Dallas, Texas (Ms Miars); Division of Basic and Clinical Immunology, University of California, Irvine, Irvine, California (Dr Tran); and Baxalta US Inc, now part of Shire, Knoxville, Tennessee (Ms Duff). Linda K. Miars, LVN, is affiliated with Allergy Partners of North Texas Research in Dallas, Texas. Her interests in the field of infusion therapy include patient education and management of adverse reaction to immunoglobulin G (IgG) administered subcutaneously (IGSC) and IgG administered intravenously (IGIV). Michelle Tran, DNP, FNP-C, is affiliated with the University of California, Irvine in Irvine, California. Her interests in the field of infusion therapy include the management of IGIV adverse reactions and barriers to weight loss in patients with primary immunodeficiency diseases. Kimberly Duff, BSN, RN, has been a manager of clinical education in global medical affairs for Shire for the past 5 years. She has 31 years of experience in nursing, with the last 17 in the field of immunology. She provides clinical expertise to support the development of new products and helps others understand the patient experience. Ms Miars and Dr Tran have no conflicts of interest to report. Ms Duff is an employee of Shire. Editorial support was provided by BlueMomentum, a division of Ashfield Healthcare Communication (a UDG Healthcare plc company) and was funded by Shire.
    An approved subcutaneous infusion of immunoglobulin G using recombinant human hyaluronidase (IGHy) allows adult patients with primary immunodeficiency disease to self-administer every 3 to 4 weeks using 1 to 2 subcutaneous infusion site(s). This article reviews the practical considerations for nurses to simplify patient education and training. Key considerations include pump choice and parameters, ancillary supplies, and technique. Read More

    Antibodies against Hepatitis A and Hepatitis B Virus in Intravenous Immunoglobulin Products.
    J Korean Med Sci 2016 Dec;31(12):1937-1942
    Center for Vaccine Evaluation and Study, Medical Research Institute, Ewha Womans University School of Medicine, Seoul, Korea.
    The worldwide seroprevalence of hepatitis A virus (HAV) and hepatitis B virus (HBV) has changed over the last two decades, indicating a declining incidence of HAV and HBV infections. Therefore, vaccinations against HAV and HBV are recommended for unimmunized people before traveling to an endemic area. Unfortunately, primary antibody deficiency (PAD) patients can only obtain humoral immunity through intravenous immunoglobulin G (IVIG) replacement and not from vaccination because of a defect in antibody production. Read More

    Helicobacter pylori infection and its related factors in junior high school students in Nagano Prefecture, Japan.
    Helicobacter 2017 Apr 27;22(2). Epub 2016 Oct 27.
    Department of Public Health, Aichi Medical University School of Medicine, Nagakute, Japan.
    Background: There have been few reports on Helicobacter pylori (H. pylori) infection in asymptomatic Japanese children and adolescents. We hypothesized that the prevalence of H. Read More

    Functional antibodies to Haemophilus influenzae type B, Neisseria meningitidis, and Streptococcus pneumoniae contained in intravenous immunoglobulin products.
    Transfusion 2017 Jan 23;57(1):157-165. Epub 2016 Oct 23.
    Center for Vaccine Evaluation and Study, Medical Research Institute, Seoul, Republic of Korea.
    Background: Intravenous immunoglobulin G (IVIG) replacement therapy is used to prevent invasive infections in patients with primary antibody deficiency (PAD). However, few studies have functionally evaluated specific antibodies against encapsulated bacteria that cause invasive infection in patients with PAD. In this study, functional antibodies against Haemophilus influenzae type b (Hib), Streptococcus pneumoniae (pneumococci), and Neisseria meningitidis (meningococci) in IVIG therapy were evaluated. Read More

    Tolerability and safety of Octagam® (IVIG): a post-authorization safety analysis of four non-interventional phase IV trials
.
    Int J Clin Pharmacol Ther 2016 Nov;54(11):847-855
    Objective: To evaluate the tolerability and safety of Octagam® 5% and 10% across all indications, ages, and treatment regimens, using data from four non-interventional post-authorization safety studies (PASS); this analysis was performed following changes in the preparation of raw material used to manufacture Octagam.

    Methods: All four studies included in- and out-patients prescribed Octagam for treatment of their medical condition. Physicians used case report forms to document baseline demographics, Octagam treatment details, and data on the efficacy of Octagam, and recorded all adverse drug reactions (ADRs) and other safety data. Read More

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