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    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

    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

    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

    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

    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

    Infectious diseases and immunological responses in adult subjects with lifetime untreated, congenital GH deficiency.
    Endocrine 2016 Oct 3;54(1):182-190. Epub 2016 Aug 3.
    Federal University of Sergipe, Division of Endocrinology, Aracaju, Sergipe, 49060-100, Brazil.
    Growth hormone is important for the development and function of the immune system, but there is controversy on whether growth hormone deficiency is associated to immune disorders. A model of isolated growth hormone deficiency may clarify if the lack of growth hormone is associated with increased susceptibility to infections, or with an altered responsiveness of the immune system. We have studied the frequency of infectious diseases and the immune function in adults with congenital, untreated isolated growth hormone deficiency. Read More

    Impact of Low Immunoglobulin G Levels on Disease Outcomes in Patients with Inflammatory Bowel Diseases.
    Dig Dis Sci 2016 Nov 12;61(11):3270-3277. Epub 2016 Sep 12.
    Center for Inflammatory Bowel Diseases, Digestive Disease and Surgery Institute-A31, The Cleveland Clinic Foundation, 9500 Euclid Ave., Cleveland, OH, 44195, USA.
    Background: Inflammatory bowel diseases (IBDs) are considered immune-mediated disorders with dysregulated innate and adaptive immunities. Secondary immunogloblin deficiency can occur in IBD and its impact on the disease course of IBD is not clear.

    Aims: We sought to determine associations between low IgG/G1 levels and poor clinical outcomes in IBD patients. Read More

    Deficiency in memory B cell compartment in a patient with infertility and recurrent pregnancy losses.
    J Reprod Immunol 2016 Nov 28;118:70-75. Epub 2016 Sep 28.
    Reproductive Medicine, Department of Obstetrics and Gynecology, Chicago Medical School at Rosalind Franklin University of Medicine and Science, Vernon Hills, IL, 60061, USA; Department of Microbiology and Immunology, Chicago Medical School at Rosalind Franklin University of Medicine and Science, North Chicago, IL, 60069, USA. Electronic address:
    Alterations in normal balance of B cell subsets have been reported in various rheumatic diseases. In this study, we report a woman with a history of recurrent pregnancy losses (RPL) and infertility who had low levels of memory B cells. A 35-year-old woman with a history of RPL and infertility was demonstrated to have increased peripheral blood CD19+ B cells with persistently low levels of memory B cell subsets. Read More

    'Pre-endoscopy point of care test (Simtomax- IgA/IgG-Deamidated Gliadin Peptide) for coeliac disease in iron deficiency anaemia: diagnostic accuracy and a cost saving economic model'.
    BMC Gastroenterol 2016 Sep 15;16:115. Epub 2016 Sep 15.
    Academic Department of Gastroenterology, Royal Hallamshire Hospital, Sheffield Teaching Hospitals, Sheffield, UK.
    Background: International guidelines recommend coeliac serology in iron deficiency anaemia, and duodenal biopsy for those tested positive to detect coeliac disease. However, pre-endoscopy serology is often unavailable, thus committing endoscopists to take routine duodenal biopsies. Some endoscopists consider duodenal biopsy mandatory in anaemia to exclude other pathologies. Read More

    Investigation of the Immunomodulatory effect of Berberis vulgaris on core-pulsed dendritic cell vaccine.
    BMC Complement Altern Med 2016 Aug 30;16(1):325. Epub 2016 Aug 30.
    Biomedical Technology, GEBRI, SRTA-city, Alexandria, Egypt.
    Background: Virus-induced dendritic cells (DCs) functional deficiency leads to sub-optimal initiation of adaptive immune responses and consequently chronic infection establishment. The present study reports an advanced hepatitis C virus (HCV) therapeutic vaccine model based on In vivo enrichment of DCs with barberry ethanolic crude extract (BCE) then pulsing them with HCV core protein.

    Methods: DCs were enriched by BCE intravenous injection in BALB/c mice. Read More

    Immunoglobulin G Subclass Deficiencies in Adult Patients with Chronic Airway Diseases.
    J Korean Med Sci 2016 Oct;31(10):1560-5
    Division of Pulmonary, Allergy, and Critical Care Medicine, Department of Medicine, Hallym University Medical Center, Anyang, Korea.
    Immunoglobulin G subclass deficiency (IgGSCD) is a relatively common primary immunodeficiency disease (PI) in adults. The biological significance of IgGSCD in patients with chronic airway diseases is controversial. We conducted a retrospective study to characterize the clinical features of IgGSCD in this population. Read More

    Helicobacter pylori infection and iron deficiency in patients with coronary artery disease.
    Cell Mol Biol (Noisy-le-grand) 2016 Jul 31;62(8):8-14. Epub 2016 Jul 31.
    University of Alberta Department of Medical Genetics, Faculty of Medicine and Dentistry Edmonton Canada.
    The aim of this study was to investigate whether impact of the seropositivity to Helicobacter pylori (H pylori) infection on ferritin and iron levels is an independent risk factor for atherosclerosis in patients with cardiovascular disease. The anti H pylori IgG, IgA levels, serum ferritin and iron concentration of 86 patients with cardiovascular disease and 64 participants free of cardiovascular disease as control subjects were determined by ELISA assay. The results of present study showed that seropositivity to H pylori IgG and IgA levels of coronary artery disease (CAD) patients was higher than controls and CAD patients with negative anti H pylori IgG and IgA significantly. Read More

    Prospective evaluation of Streptococcus pneumoniae serum antibodies in patients with primary immunodeficiency on regular intravenous immunoglobulin treatment.
    Allergol Immunopathol (Madr) 2017 Jan - Feb;45(1):55-62. Epub 2016 Jul 29.
    Division of Pediatric Infectious Diseases, Department of Pediatrics, Universidade Federal de São Paulo, São Paulo, SP, Brazil. Electronic address:
    Background: This is a prospective study that assessed pneumococcal antibody levels in PID patients under intravenous immunoglobulin (IVIG) treatment using different brands.

    Methods: Twenty-one patients receiving regular IVIG every 28 days were invited to participate: 12 with common variable immunodeficiency, six with X-linked agammaglobulinaemia and three with hyper-IgM syndrome. One blood sample was collected from each patient just prior to IVIG administration at a three-month time interval during one year. Read More

    Venous Thromboembolism After Knee Arthroscopy in Undiagnosed Familial Thrombophilia.
    Orthopedics 2016 Nov 27;39(6):e1052-e1057. Epub 2016 Jul 27.
    Venous thromboembolism is uncommon after knee arthroscopy, and there are no guidelines for thromboprophylaxis in elective routine knee arthroscopy. Preoperative evaluation of common thrombophilias should provide guidance for postarthroscopy thromboprophylaxis in otherwise healthy patients who are at high risk for venous thromboembolism. This study assessed 10 patients with venous thromboembolism after total hip or knee arthroplasty. Read More

    Immunoglobulin A deficiency following treatment with lamotrigine.
    Brain Dev 2016 Nov 7;38(10):947-949. Epub 2016 Jul 7.
    Department of Pediatrics, Graduate School of Medical and Dental Sciences, Kagoshima University, Kagoshima, Japan.
    Lamotrigine (LTG) is an anti-epileptic drug and mood-stabilizing agent, whose adverse effects include skin rash and dizziness. Interactions with the immune system are rare, and only a few cases linking hypogammaglobulinemia to LTG treatment have been previously described. In this report, we describe a case in which a patient developed hypogammaglobulinemia, and a subsequent immunoglobulin A (IgA) deficiency, following LTG treatment. Read More

    [Seroprevalence of human parvovirus B19 in children with fever and rash in the North of Tunisia].
    Bull Soc Pathol Exot 2016 Aug 7;109(3):165-71. Epub 2016 Jul 7.
    Unité de recherche UR12ES01, faculté de médecine de Tunis, université de Tunis El-Manar, Tunis, Tunisie.
    The aim of the study is to evaluate the prevalence of specific antibodies anti-human parvovirus B19 (PVB19) immunoglobulin M (IgM) and IgG in children with fever and rash. This study involved 257 children aged from 7 months to 15 years with febrile rash unrelated to measles and rubella (seronegative for IgM). The sera were examined by immunoenzymatic assay. Read More

    Lupus anticoagulant-hypoprothrombinemia syndrome and catastrophic antiphospholipid syndrome in a patient with antidomain I antibodies.
    Blood Coagul Fibrinolysis 2016 Jul;27(5):580-2
    aNancy University Hospital, Vascular Medicine Division and Regional Competence, Center for Rare Vascular And Systemic Autoimmune DiseasesbDijon University Hospital, Haematology laboratory, Nancy, FrancecBiochemistry, Cardiovascular Research Institute Maastricht (CARIM), Maastricht, University, Maastricht, the NetherlandsdNancy University Hospital, Nuclear Medecine & Nancyclotep Experimental Imaging, PlatformeUniversité de LorrainefInserm, UMR_S 1116gNancy University Hospital, Department of Critical Care Medicine, Nancy, France.
    Lupus anticoagulant-hypoprothrombinemia syndrome is a rare condition characterized by the association of acquired factor II deficiency and lupus anticoagulant. Contrary to classical antiphospholipid syndrome, it may cause severe life-threatening bleeding (89% of published cases). We report a patient, positive for antidomain I antibodies, with initially primary lupus anticoagulant-hypoprothrombinemia syndrome without previous clinical manifestation or underlying systemic disease. Read More

    Complement and Antibody-mediated Enhancement of Red Blood Cell Invasion and Growth of Malaria Parasites.
    EBioMedicine 2016 Jul 14;9:207-16. Epub 2016 May 14.
    Department of Microbiology and Immunology, Pennsylvania State University, College of Medicine, 500 University Drive, Hershey, PA 17033, United States; Department of Medicine, Division of Infectious Diseases and Epidemiology, Pennsylvania State University, College of Medicine, 500 University Drive, Hershey, PA 17033, United States. Electronic address:
    Plasmodium falciparum malaria is a deadly pathogen. The invasion of red blood cells (RBCs) by merozoites is a target for vaccine development. Although anti-merozoite antibodies can block invasion in vitro, there is no efficacy in vivo. Read More

    Deficiency of cold-inducible ribonucleic acid-binding protein reduces renal injury after ischemia-reperfusion.
    Surgery 2016 Aug 3;160(2):473-83. Epub 2016 Jun 3.
    Department of Surgery, Hofstra Northwell School of Medicine, Manhasset, NY; Center for Translational Research, The Feinstein Institute for Medical Research, Manhasset, NY. Electronic address:
    Background: Renal ischemia-reperfusion injury, commonly caused by major operation and shock, leads to acute kidney injury and is associated with high morbidity and mortality. Cold-inducible ribonucleic acid-binding protein, a cold shock protein, has recently been identified as a damage-associated molecular pattern. We hypothesized that cold-inducible ribonucleic acid-binding protein exacerbates severity of injury in renal ischemia-reperfusion. Read More

    Response to pneumococcal polysaccharide vaccine in children with asthma, and children with recurrent respiratory infections, and healthy children.
    Allergol Immunopathol (Madr) 2016 Jul-Aug;44(4):376-81. Epub 2016 May 30.
    Public Health School University of Chile, Santiago de Chile, Chile.
    Background: To analyse specific immune response to the 23-valent pneumococcal polysaccharide vaccine by measuring pneumococcal antibodies in children with asthma and with respiratory recurrent infection (RRI) as compared to healthy children.

    Methods: The study included 60 children, divided into three groups: 20 with asthma, 20 with RRI, and 20 healthy controls. Post-vaccination specific IgG antibodies against 10 pneumococcal serotypes (S1, S3, S4, S5, S6B, S9V, S14, S18C, S19F, and S23F) contained in the 23-valent pneumococcal polysaccharide vaccine (PPV) were measured. Read More

    Global TLR2 and 4 deficiency in mice impacts bone resorption, inflammatory markers and atherosclerosis to polymicrobial infection.
    Mol Oral Microbiol 2016 May 25. Epub 2016 May 25.
    Department of Periodontology, College of Dentistry, University of Florida, Gainesville, FL, USA.
    Toll-like-receptors (TLRs) play a significant role in the generation of a specific innate immune response against invading pathogens. TLR2 and TLR4 signaling contributes to infection-induced inflammation in periodontal disease (PD) and atherosclerosis. Observational studies point towards a relationship between PD and atherosclerosis, but the role of TLR2 and TLR4 in the recognition of multiple oral pathogens and their modulation of host response leading to atherosclerosis are not clear. Read More

    [Understanding primary immunodeficiencies: usefulness of a register].
    Rev Med Suisse 2016 Apr;12(513):708, 710-2
    Primary Immunodeficiency Diseases (PID) comprise inborn defects of the immune system which are and therefore difficult to study For this reason, the European Society for ImmunoDeficiencies (ESID) has set up an internet-based international patient and research database which integrates research data with more detailed clinical information. These disorders are not only found in children, but also in adults resulting in a wide range of clinical manifestations. Primary immunodeficiency adults are much less known and may remain undiagnosed. Read More

    Gingival Inflammation and Aggressive Periodontitis in a Child with a Specific Antibody Deficiency.
    Dent Update 2016 Mar;43(2):130-2, 135-6
    Exuberant gingival inflammation accompanied by periodontitis is a rare finding in a very young child and may indicate a defect in the host response. Affected children should be referred to appropriate specialists to establish a definitive diagnosis. A 5-year-old girl presented with persistent gingival inflammation and periodontal destruction. Read More

    Selective Subnormal IgG1 in 54 Adult Index Patients with Frequent or Severe Bacterial Respiratory Tract Infections.
    J Immunol Res 2016 31;2016:1405950. Epub 2016 Mar 31.
    Southern Iron Disorders Center, Birmingham, AL 35209, USA; Department of Microbiology, University of Alabama at Birmingham, Birmingham, AL 35294, USA.
    We characterized 54 adult index patients with reports of frequent or severe bacterial respiratory tract infections at diagnosis of selective subnormal IgG1. Mean age was 50 ± 13 (SD) y; 87.0% were women. Read More

    Use of subcutaneous immunoglobulin in primary immune deficiencies.
    Turk Pediatri Ars 2016 Mar 1;51(1):8-14. Epub 2016 Mar 1.
    Clinic of Pediatric Allergy and Immunology, Marmara University Pendik Training and Research Hospital, İstanbul, Turkey.
    Aim: Immunoglobulin replacement therapy is required to reduce the frequency and severity of infections in patients with primary antibody deficiencies. Immunoglobulin G (IgG) can be administered intramuscularly, intravenously or subcutaneously. We aimed to evaluate the efficacy, dose adjustment and adverse events in subcutaneous immunoglobulin therapy by retrospectively presenting the records of 16 patients who received subcutaneous immunoglobulin therapy. Read More

    Characterization of a hypoallergenic wheat line lacking ω-5 gliadin.
    Allergol Int 2016 Oct 18;65(4):400-405. Epub 2016 Apr 18.
    Department of Dermatology, Shimane University Faculty of Medicine, Shimane, Japan.
    Background: There is no curative treatment for wheat-dependent exercise-induced anaphylaxis (WDEIA). ω-5 Gliadin is one of the dominant allergens affecting WDEIA patients. The use of ω-5 gliadin-free wheat flour in the regular diet is considered one of the prophylactic approaches against the elicitation of allergic symptoms and sensitization to ω-5 gliadin. Read More

    Health-Related Quality of Life and Health Resource Utilization in Patients with Primary Immunodeficiency Disease Prior to and Following 12 Months of Immunoglobulin G Treatment.
    J Clin Immunol 2016 Jul 18;36(5):450-61. Epub 2016 Apr 18.
    Baxalta US, Inc, Cambridge, MA, USA.
    Purpose: Health-related quality of life (HRQOL) has not been examined in patients with predominant antibody deficiency both pre- and post-immunoglobulin G (IgG) treatment initiation. HRQOL and health resource utilization (HRU) were assessed in newly diagnosed patients with primary immunodeficiency disease (PIDD) pre- and 12 months post-IgG treatment initiation.

    Methods: Adults (age ≥18 years) completed the 36-item Short Form Health Survey, version 2; pediatric patients (PP)/caregivers completed the Pediatric Quality of Life Inventory (PedsQL). Read More

    Treatment with anti-IL-6 receptor antibody prevented increase in serum hepcidin levels and improved anemia in mice inoculated with IL-6-producing lung carcinoma cells.
    BMC Cancer 2016 Apr 11;16:270. Epub 2016 Apr 11.
    Product Research Department, Chugai Pharmaceutical Co., Ltd., 200 Kajiwara, Kamakura, Kanagawa, 247-8530, Japan.
    Background: Hepcidin, a key regulator of iron metabolism, is produced mainly by interleukin-6 (IL-6) during inflammation. A mechanism linking cancer-related anemia and IL-6 through hepcidin production is suggested. To clarify the hypothesis that overproduction of IL-6 elevates hepcidin levels and contributes to the development of cancer-related anemia, we evaluated anti-IL-6 receptor antibody treatment of cancer-related anemia in an IL-6-producing human lung cancer xenograft model. Read More

    IVIG in autoimmune disease - Potential next generation biologics.
    Autoimmun Rev 2016 Aug 25;15(8):781-5. Epub 2016 Mar 25.
    CSL Behring AG, Research, Switzerland.
    Polyclonal plasma-derived IgG is a mainstay therapeutic of immunodeficiency disorders as well as of various inflammatory autoimmune diseases. In immunodeficiency the primary function of IVIG/SCIG is to replace missing antibody specificities, consequently a diverse Fab-based repertoire is critical for efficacy. Attempts to capture the Ig repertoire and express it as a recombinant IVIG product are currently ongoing. Read More

    Isotypic analysis of antibodies against activated Factor VII in patients with Factor VII deficiency using the x-MAP technology.
    Thromb Res 2016 May 23;141:22-7. Epub 2016 Feb 23.
    Department of Biological Hematology, Hospital Saint-Eloi, Montpellier, France. Electronic address:
    While the immune response to hemophilic factors in hemophilia has been widely studied, little is known about the development of anti-Factor VII (FVII) antibodies in FVII deficiency. We developed a robust technique based on the x-MAP technology to detect the presence of antibodies against FVII and characterize their isotype and validated this method using blood samples from 100 patients with FVII deficiency (median FVII clotting activity [FVII:C]: 6%) and 95 healthy controls. Anti-FVII antibodies were detected in patients but also in some controls, although the concentration of total immunoglobulin G (IgGt) and IgG1 and IgG4 subclasses was significantly different between groups. Read More

    Humoral deficiency in three paediatric patients with genetic diseases.
    Allergol Immunopathol (Madr) 2016 May-Jun;44(3):257-62. Epub 2016 Mar 2.
    Pediatric Allergy and Clinical Immunology Department, Hospital Sant Joan de Déu-Universitat de Barcelona, Barcelona, Spain; Functional Unit of Immunology, Hospital Sant Joan de Déu-Universitat de Barcelona, Barcelona, Spain. Electronic address:
    Background: Primary immunodeficiencies (PID) represent a heterogeneous group of genetic disorders characterised by poor or absent function in one or more components of the immune system. Humoral or antibody immunodeficiencies are the most common form of PID, of which common variable immunodeficiency (CVID) is the most frequent symptomatic form. CVID is usually characterised by hypogammaglobulinaemia with poor antibody specificity, and an increased susceptibility to infections, autoimmunity and lymphoproliferation. Read More

    Simultaneous detection of IgA and IgG antibodies against tissue transglutaminase: The preferred pre-biopsy test in childhood celiac disease.
    Scand J Clin Lab Invest 2016 29;76(3):208-16. Epub 2016 Feb 29.
    a Departments of Women's and Children's Health , Uppsala University , Uppsala , Sweden ;
    Objectives: IgA antibodies against tissue transglutaminase (anti-TG2) is a reliable marker of celiac disease (CD). However, IgA-deficient patients are not identified and young children may lack IgA anti-TG2. Combined detection of IgA and IgG (IgA/IgG) against deamidated gliadin peptides (DGP) has shown a high diagnostic performance for untreated CD. Read More

    Deficiency of Adenosine Deaminase 2 Causes Antibody Deficiency.
    J Clin Immunol 2016 Apr 27;36(3):179-86. Epub 2016 Feb 27.
    CCI-Center for Chronic Immunodeficiency, Universitätsklinikum Freiburg, Engesserstraße 4, 79108, Freiburg, Germany.
    Purpose: Determining the monogenic cause of antibody deficiency and immune dysregulation in a non-consanguineous family with healthy parents, two affected children, and one unaffected child.

    Methods: Whole Exome Sequencing (WES) was performed in the index family. WES results were confirmed by Sanger Sequencing. Read More

    Quantitative Evidence of Wear-Off Effect at the End of the Intravenous IgG (IVIG) Dosing Cycle in Primary Immunodeficiency.
    J Clin Immunol 2016 Apr 24;36(3):210-9. Epub 2016 Feb 24.
    Clinical Research and Development, CSL Behring GmbH, Marburg, Germany.
    Purpose: Intravenous IgG (IVIG) treatment wear-off is commonly experienced by patients, who report increased susceptibility to infection, and decreased quality of life towards the end of their 3- or 4-week dosing cycle, when serum IgG levels approach their trough. We quantified IVIG wear-off in terms of treatment efficacy and patient well-being.

    Methods: Data were collected from patients enrolled in three Phase III trials of Sandoglobulin NF Liquid or Privigen, treated every 3- or 4- weeks. Read More

    Age-dependent increase of blood-brain barrier permeability and neuron-binding autoantibodies in S100B knockout mice.
    Brain Res 2016 Apr 22;1637:154-67. Epub 2016 Feb 22.
    Graduate School of Biomedical Sciences, Rowan University, Stratford, NJ 08084, USA; Department of Cell Biology, Rowan School of Osteopathic Medicine, Stratford, NJ 08084, USA. Electronic address:
    S100B is a calcium-sensor protein that impacts multiple signal transduction pathways. It is widely considered to be an important biomarker for several neuronal diseases as well as blood-brain barrier (BBB) breakdown. In this report, we demonstrate a BBB deficiency in mice that lack S100B through detection of leaked Immunoglobulin G (IgG) in the brain parenchyma. Read More

    Immunogenicity of Renal Microvascular Endothelial Cells From Genetically Modified Pigs.
    Transplantation 2016 Mar;100(3):533-7
    1 Department of Surgery, Indiana University School of Medicine, Indianapolis, IN.2 Department of Surgery, Indiana University Health, Indianapolis, IN.3 IU Health Transplant Institute, Indianapolis, IN.
    Background: Disrupting the porcine GGTA1 and CMAH genes [double knockout (DKO)] that produce the gal-α(1,3)-gal and N-glycolylneuraminic acid xenoantigens reduces human antibody binding to porcine peripheral blood mononuclear cells. It is important to examine rejection pathways at an organ-specific level. The object of this study is to evaluate the human preformed antibody reactivity against DKO renal microvascular endothelial cells (RMEC) in vitro. Read More

    A Comparative Study of Intravenous Immunoglobulin and Subcutaneous Immunoglobulin in Adult Patients with Primary Immunodeficiency Diseases: A Systematic Review and Meta-Analysis.
    Expert Rev Clin Immunol 2016 9;12(5):595-602. Epub 2016 Mar 9.
    d Health management and economics research center , Iran University of Medical Sciences , Tehran , Iran.
    Subcutaneous immunoglobulin (SCIG) is a new therapeutic procedure for patients with primary immunodeficiency (PI). This research is a systematic review of studies on the efficacy and safety of intravenous immunoglobulin (IVIG) and SCIG in adult patients with PI. This study includes a systematic review of cohorts and randomized clinical trials (24 articles) from 5 databases with no time limits. Read More

    [Thrombotic Thrombocytopenic Purpura --Pathophysiology and Assays of ADAMTS13 Activity].
    Rinsho Byori 2015 Oct;63(10):1228-36
    Thrombotic thrombocytopenic purpura (TTP) is a life-threatening disorder classified with a type of thrombotic microangiopathy (TMA). TTP is caused by a deficiency of von Willebrand factor-cleaving protease called ADAMTS13 (a disintegrin-like and metalloprotease with a thrombospondin type1 motif 13). Low ADAMTS13 levels result in increased ultra-large von Willebrand factor multimers (UL-VWFM), which induce platelet adhesion and thrombosis. Read More

    Dyslipidemia-associated alterations in B cell subpopulation frequency and phenotype during experimental atherosclerosis.
    Atherosclerosis 2016 Apr 29;247:118-26. Epub 2015 Dec 29.
    Grupo de Ciencias Básicas, Universidad CES, Medellín, Colombia. Electronic address:
    Lymphocytes, the cellular effectors of adaptive immunity, are involved in the chronic inflammatory process known as atherosclerosis. Proatherogenic and atheroprotective properties have been ascribed to B cells. However, information regarding the role of B cells during atherosclerosis is scarce. Read More

    Loss of Fancc Impairs Antibody-Secreting Cell Differentiation in Mice through Deregulating the Wnt Signaling Pathway.
    J Immunol 2016 Apr 19;196(7):2986-94. Epub 2016 Feb 19.
    Division of Experimental Hematology and Cancer Biology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH 45229
    Fanconi anemia (FA) is characterized by a progressive bone marrow failure and an increased incidence of cancer. FA patients have high susceptibility to immune-related complications such as infection and posttransplant graft-versus-host disease. In this study, we investigated the effect of FA deficiency in B cell function using the Fancc mouse model. Read More

    The Formation of Microthrombi in Parenchymal Microvessels after Traumatic Brain Injury Is Independent of Coagulation Factor XI.
    J Neurotrauma 2016 Sep 17;33(17):1634-44. Epub 2016 Feb 17.
    1 Department of Neurodegeneration, Royal College of Surgeons in Ireland (RCSI) , Dublin, Ireland .
    Microthrombus formation and bleeding worsen the outcome after traumatic brain injury (TBI). The aim of the current study was to characterize these processes in the brain parenchyma after experimental TBI and to determine the involvement of coagulation factor XI (FXI). C57BL/6 mice (n = 101) and FXI-deficient mice (n = 15) were subjected to controlled cortical impact (CCI). Read More

    The Challenge of Immunoglobulin-G Subclass Deficiency and Specific Polysaccharide Antibody Deficiency--a Dutch Pediatric Cohort Study.
    J Clin Immunol 2016 Feb 4;36(2):141-8. Epub 2016 Feb 4.
    Department of Pediatrics, Jeroen Bosch Hospital, 's-Hertogenbosch, The Netherlands.
    Purpose: Immunoglobulin(Ig)G-subclass deficiency and specific polysaccharide antibody deficiency (SPAD) are among the most frequent causes of recurrent respiratory infections in children. Little is known about their prevalence, clinical presentation and prognosis. No study has been published in a Western-European nor in a mainly non-tertiary cohort until now. Read More

    Arq Gastroenterol 2015 Dec;52(4):278-82
    School of Medicine, Shahid Sadoughi University of Medical Sciences, Yazd, Iran.
    Background: Celiac disease is an immune-mediated enteropathy due to a permanent sensitivity to gluten in genetically susceptible people. Iron-deficiency anemia is the most widely experienced anemia in humans. Iron-deficiency anemia additionally is a common extra intestinal manifestation of celiac disease. Read More

    Factor V Deficiency in Korean Patients: Clinical and Laboratory Features, Treatment, and Outcome.
    J Korean Med Sci 2016 Feb 25;31(2):208-13. Epub 2016 Jan 25.
    Department of Hematology-Oncology, Inha University Hospital and School of Medicine, Incheon, Korea.
    Due to rarity of factor V (FV) deficiency, there have been only a few case reports in Korea. We retrospectively analysed the clinical-laboratory features of FV deficiency in 10 Korean patients. Between January 1987 and December 2013, 10 case reports published in a Korean journal or proceedings of Korea Society on Thrombosis and Hemostasis were reviewed. Read More

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