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    [Late diagnosis of WHIM sydrome].
    Medicina (B Aires) 2018 ;78(2):123-126
    Unidad Inmunología e Histocompatibilidad, Hospital Dr. Carlos G. Durand, Buenos Aires, Argentina.
    WHIM syndrome is a primary autosomal dominant immuno deficiency due to CXCR4 mutations characterized by mucocutaneous warts, hypogammaglobulinemia, recurrent bacterial infections and myelokathesis. Treatment consists in prophylactic antibiotics, immunoglobulin replacement and granulocyte or granulocyte/monocyte colony stimulating factors. We present the case of a 21 year old woman who showed leukopenia at 10 months of age and one year later multiple infections with hypogammaglobulinemia requiring intravenous immunoglobulin. Read More
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    The clinical and immunological features of patients with primary antibody deficiencies.
    Endocr Metab Immune Disord Drug Targets 2018 Apr 12. Epub 2018 Apr 12.
    Research Center for Immunodeficiencies, Children's Medical Center, Tehran University of Medical Sciences Tehran, 455. Iran.
    Background: Primary antibody deficiency (PAD) comprises a range of diseases from early to late terminal B cells defects and is associated with the various clinical complications.

    Methods: A total of 461 patients (311 males and 150 females) with PADs enrolled in the retrospective cohort study and for all patients' demographic information, clinical records and laboratory data were collected to investigate clinical complications.

    Results: The most prevalent first presentations of immunodeficiency were respiratory tract infections in 63. Read More
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    Role of serum immunoglobulins for predicting sarcoidosis outcome: A cohort study.
    PLoS One 2018 11;13(4):e0193122. Epub 2018 Apr 11.
    Internal Medicine Department, Rennes University Hospital, Rennes, France.
    Background: Sarcoidosis is a systemic granulomatous disease which carries variable outcomes. Serum protein electrophoresis is an easily accessible and routinely performed examination at diagnosis, in order to search for hypergammaglobulinemia, which is frequently found, and to rule out other granulomatous diseases such as common variable immunodeficiency. We aimed to assess the impact of baseline immunoglobulin level on the outcome of sarcoidosis. Read More
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    The Effect of Combination Antiretroviral Therapy Use Among HIV Positive Children on the Hazard of AIDS Using Calendar Year as an Instrumental Variable.
    Curr HIV Res 2018 Apr 9. Epub 2018 Apr 9.
    Pediatrics-Infectious Diseases, Stanford University, Stanford, CA . United States.
    Background: Instrumental variable (IV) analyses are a common causal inference technique used in the absence of randomized data. Combination antiretroviral therapy (cART) was first introduced in 1996 and calendar periods have been used as a proxy for cART use. However, cART use misclassification can bias IV analyses. Read More
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    Complications Associated with Underweight Primary Immunodeficiency Patients: Prevalence and Associations Within the USIDNET Registry.
    J Clin Immunol 2018 Apr 4. Epub 2018 Apr 4.
    Division of Allergy and Immunology, Children's Hospital of Philadelphia, Philadelphia, PA, USA.
    Purpose: The point prevalence of underweight status and obesity in primary immunodeficiency disease (PID) is unknown, despite the described associations between PID and weight loss and failure to thrive. The goal of this study is to estimate the prevalence of underweight status and obesity in PID patients and to investigate the associations between abnormal body weight and complications of PID.

    Methods: Using the US Immunodeficiency Network (USIDNET), we performed a retrospective analysis of 653 pediatric (age 2 to 20 years) and 514 adult (age > 20) patient records with information on patient body mass index (BMI). Read More
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    Evaluation of the TLR negative regulatory network in CVID patients.
    Genes Immun 2018 Apr 5. Epub 2018 Apr 5.
    Immunology Research Center (IRC), Institute of Immunology and Infectious Diseases, Iran University of Medical Sciences, Tehran, Iran.
    Common variable immunodeficiency (CVID), a clinically symptomatic primary immunodeficiency disease (PID), is characterized by hypogammaglobulinemia leading to recurrent infections and various complications. Recently, some defects in the signaling of TLRs have been identified in CVID patients which led us to investigate the expression of TLR4 and 9 negative regulatory molecules and their upregulation status following their activation. Using TaqMan real-time PCR, SOCS1, TNFAIP3, RFN216, and IRAK-M transcripts among peripheral blood mononuclear cells (PBMCs) were measured with/without TLR4 and 9 activations. Read More
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    [Successful cord blood transplantation in a patient with adult-onset common variable immunodeficiency].
    Rinsho Ketsueki 2018 ;59(3):293-299
    Department of Hematology/Oncology, Wakayama Medical University.
    Common variable immunodeficiency (CVID) is the most frequently diagnosed congenital immunodeficiency and is characterized by dysfunctional antibody production. It often occurs at the age of ≥10 years. Here we reported a case of a 46-year-old man confirmed with adult-onset CVID. Read More
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    Intravenous immunoglobulin (IVIG) efficiency in women with common variable immunodeficiency (CVID) decreases significantly during pregnancy.
    J Matern Fetal Neonatal Med 2018 Apr 3:1-5. Epub 2018 Apr 3.
    c Department of Comprehensive Reproductive Medicine , Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University , Tokyo , Japan.
    Immunoglobulin replacement therapy, including intravenous immunoglobulin (IVIG), is essential for pregnant women with common variable immunodeficiency (CVID) since it prevents infection and improves the health of the newborn. There are no established IVIG treatment protocols for pregnant women with CVID, and the relationship between IVIG treatment and maternal serum IgG changes during pregnancy remains unclear. Therefore, we reviewed the medical charts of four CVID patients, including one receiving subcutaneous immunoglobulin (SCIG), for IVIG dose and frequency, maternal serum IgG changes, obstetrical findings, and perinatal outcomes. Read More
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    Phenotypic characterization of patients with rheumatologic manifestations of common variable immunodeficiency.
    Semin Arthritis Rheum 2018 Feb 23. Epub 2018 Feb 23.
    Divisions of Rheumatology and Allergy and Clinical Immunology, Johns Hopkins University School of Medicine, Baltimore, MD.
    Patients with common variable immunodeficiency (CVID) have a higher incidence of rheumatologic disorders. To delineate this clinical association, we investigated the phenotypic features of patients with CVID affected by these conditions.

    Methods: We conducted a retrospective analysis of 870 pediatric and adult patients with CVID included in the United States Immunodeficiency Network (USIDNET) registry. Read More
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    Rheumatologic complications in a cohort of 227 patients with common variable immunodeficiency.
    Scand J Immunol 2018 May;87(5):e12663
    Research Center for Immunodeficiencies, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran.
    Common variable immunodeficiency (CVID) is the most prevalent symptomatic type of human primary immunodeficiency diseases (PID). Clinically, CVID is characterized by increased susceptibility to infections and a wide variety of autoimmune and rheumatologic disorders. All patients with CVID registered in Iranian PID Registry (IPIDR) were enrolled in this retrospective cohort study. Read More
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    Urinary tract blastomycosis diagnosed by urine cytology.
    Diagn Cytopathol 2018 Mar 23. Epub 2018 Mar 23.
    Department of Laboratory Medicine and Pathology, University of Minnesota Medical School, Minneapolis, Minnesota.
    Urinary tract blastomycosis is an uncommon manifestation of disseminated Blastomyces infection. Here, we report a 50-year-old male with common variable immunodeficiency who presented with urinary symptoms and a renal mass concerning for a kidney neoplasm. Urine cytology revealed typical broad-based budding yeasts with thick-walled refractile capsules, leading to diagnosis of urinary tract blastomycosis. Read More
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    Common variable immunodeficiency presenting in a man with recurrent pneumonia caused by .
    BMJ Case Rep 2018 Mar 20;2018. Epub 2018 Mar 20.
    Department of Internal Medicine, Berkshire Medical Center, Pittsfield, Massachusetts, USA.
    Common variable immunodeficiency (CVID) refers to a group of disorders where differentiation and maturation of B cells into plasma cells are affected, leading to decreased or defective immunoglobulin production and subsequent immunodeficiency. Symptoms may present at any age between 5 and 72 years, although more severe forms often manifest earlier in life. Milder forms may not be detected. Read More
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    The Significance of B-cell Subsets in Patients with Unclassified Hypogammaglobulinemia and Association with Intravenous Immunoglobulin Replacement Requirement.
    Iran J Immunol 2018 Mar;15(1):1-13
    Department of Pediatric Allergy and Immunology, Dr. Behcet Uz Children's Hospital, Izmir, Turkey.
    Background: Patients with unclassified hypogammaglobulinemia (UCH) constitute a diagnostic and therapeutic dilemma, because information concerning the clinical and immunological characteristics of these patients is insufficient.

    Objective: To evaluate B-cell subsets in cases with UCH and common variable immunodeficiency (CVID) and their association with treatment requirement in UCH patients.

    Methods: The study included 41 UCH, 25 CVID, and 36 healthy individuals between the ages of 4-18 years. Read More
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    Implanted ports in adults with primary immunodeficiency.
    J Vasc Access 2018 Mar 1:1129729818757966. Epub 2018 Mar 1.
    1 Department of Medicine, Brookwood Medical Center, Birmingham, AL, USA.
    Background: We sought to learn more about the utility and safety of implanted ports for monthly immunoglobulin G infusions in adults with primary immune deficiency.

    Methods: We reviewed charts of adults who were referred to a single practice during the interval 2006-2016 for evaluation and management of frequent or severe upper and lower respiratory tract and other infections, subnormal total immunoglobulin G or immunoglobulin G subclasses, and suboptimal responses to polyvalent pneumococcal polysaccharide vaccinations; were diagnosed to have primary immune deficiency; and were advised to undergo immunoglobulin G therapy.

    Results: Of 606 patients, 20 (19 women, 1 man; 16 immunoglobulin G subclass deficiency, 4 common variable immunodeficiency; 3. Read More
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    Sirolimus as an alternative treatment in patients with granulomatous-lymphocytic lung disease and humoral immunodeficiency with impaired regulatory T cells.
    Pediatr Allergy Immunol 2018 Mar 12. Epub 2018 Mar 12.
    Allergy and Clinical Immunology Department, Institut de Recerca Pediàtrica Hospital Sant Joan de Déu, Esplugues de Llobregat, Spain.
    Background: One of the most frequent non-infectious complications of humoral immunodeficiencies with a CVID-like pattern is a particular form of inflammatory lung disease which is called granulomatous-lymphocytic interstitial lung disease (GLILD). Its development worsens patient prognosis, with a significant decrease in survival. Currently, there are no unified guidelines regarding its management, and different combinations of immunosuppressants have been used with variable success. Read More
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    Phenotypic and genotypic characterization of inflammatory bowel disease in children under six years of age in China.
    World J Gastroenterol 2018 Mar;24(9):1035-1045
    Department of Gastroenterology, Children's Hospital, Zhejiang University School of Medicine, Hangzhou 310052, Zhejiang Province, China.
    Aim: To analyze clinical differences between monogenic and nonmonogenic very-early-onset inflammatory bowel disease (VEO-IBD) and to characterize monogenic IBD phenotypically and genotypically genetic testing.

    Methods: A retrospective analysis of children aged 0 to 6 years diagnosed with VEO-IBD in a tertiary hospital in southern China from 2005 to 2017 was performed. Clinical data for VEO-IBD patients were collected, and genetic characteristics were analyzed using whole exome sequencing or target gene panel sequencing. Read More
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    Pharmacokinetics of a novel human intravenous immunoglobulin 10% in patients with primary immunodeficiency diseases: Analysis of a phase III, multicentre, prospective, open-label study.
    Eur J Pharm Sci 2018 Mar 6;118:80-86. Epub 2018 Mar 6.
    Division of Pediatric Allergy/Immunology, John H. Stroger, Jr. Hospital of Cook County, 1969 W. Ogden Ave., Chicago, IL 60612, USA; Rush University Medical Center, 1653 W. Congress Parkway, Chicago, IL 60612, USA. Electronic address:
    Intravenous immunoglobulin (IVIG) therapy is commonly used to treat patients with primary antibody deficiency. This prospective, open-label, non-randomised, multicentre, phase III trial investigated the pharmacokinetics of a new 10% liquid IVIG product (panzyga®; Octapharma) in 51 patients aged 2-75 years with common variable immunodeficiency (n = 43) or X-linked agammaglobulinaemia (n = 8). Patients were treated with IVIG 10% every 3 (n = 21) or 4 weeks (n = 30) at a dose of 200-800 mg/kg for 12 months. Read More
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    [Primary antibody deficiency syndromes].
    Ugeskr Laeger 2018 Feb;180(9)
    Primary antibody deficiency syndromes (PAD) are a group of primary immunodeficiencies (PID) characterized by reduced production of immunoglobulins and recurrent respiratory tract infections. PAD varies from rare but life-threatening agammaglobulinaemias to frequent but often asymptomatic conditions such as selective immunoglobulin(Ig)A deficiency or IgG subclass deficiency. Common variable immunodeficiency is the clinically most important PAD. Read More
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    Evaluation of infectious and non-infectious complications in patients with primary immunodeficiency.
    Cent Eur J Immunol 2017 30;42(4):336-341. Epub 2017 Dec 30.
    Research Center for Immunodeficiencies, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran.
    Introduction: Primary immunodeficiency diseases (PIDs) are a heterogeneous group of genetic immune disorders. PID patients suffer from a variety of complications. The aim of this study was to determine the infectious and non-infectious complications among PID patients. Read More
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    Loss-of-function nuclear factor κB subunit 1 (NFKB1) variants are the most common monogenic cause of common variable immunodeficiency in Europeans.
    J Allergy Clin Immunol 2018 Mar 2. Epub 2018 Mar 2.
    NIHR BioResource-Rare Diseases, Cambridge Biomedical Campus, Cambridge, United Kingdom.
    Background: The genetic cause of primary immunodeficiency disease (PID) carries prognostic information.

    Objective: We conducted a whole-genome sequencing study assessing a large proportion of the NIHR BioResource-Rare Diseases cohort.

    Methods: In the predominantly European study population of principally sporadic unrelated PID cases (n = 846), a novel Bayesian method identified nuclear factor κB subunit 1 (NFKB1) as one of the genes most strongly associated with PID, and the association was explained by 16 novel heterozygous truncating, missense, and gene deletion variants. Read More
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    Low Serum IgE Is a Sensitive and Specific Marker for Common Variable Immunodeficiency (CVID).
    J Clin Immunol 2018 Feb 17. Epub 2018 Feb 17.
    Department of Medicine, University of Virginia, Box 801355, Charlottesville, VA, 22908, USA.
    Although small prior studies have suggested that IgE can be low in common variable immunodeficiency (CVID), the workup for patients with recurrent infections and suspected hypogammaglobulinemia does not include the routine measurement of serum IgE. We sought to test the hypothesis that low/undetectable serum IgE is characteristic of CVID by comparing the frequency of low/undetectable serum IgE in healthy controls and patients with CVID. We measured total serum IgE in a large multi-center cohort of patients with CVID (n = 354) and compared this to large population-based cohorts of children and adults. Read More
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    Clinical Implications of Digenic Inheritance and Epistasis in Primary Immunodeficiency Disorders.
    Front Immunol 2017 26;8:1965. Epub 2018 Jan 26.
    School of Biological Sciences, University of Auckland, Auckland, New Zealand.
    The existence of epistasis in humans was first predicted by Bateson in 1909. Epistasis describes the non-linear, synergistic interaction of two or more genetic loci, which can substantially modify disease severity or result in entirely new phenotypes. The concept has remained controversial in human genetics because of the lack of well-characterized examples. Read More
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    Proline-serine-threonine phosphatase interacting protein 1 (PSTPIP1) controls immune synapse stability in human T cells.
    J Allergy Clin Immunol 2018 Feb 9. Epub 2018 Feb 9.
    Department of Pediatric Immunology and Laboratory of Translational Immunology, Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht, The Netherlands. Electronic address:
    Background: Proline-serine-threonine phosphatase interacting protein 1 (PSTPIP1) is a cytosolic adaptor protein involved with T-cell activation, differentiation, and migration. On cognate T-cell contact, PSTPIP1 is recruited to surface-expressed CD2, where it regulates F-actin remodeling. An immune synapse (IS) is thereby rapidly formed, consisting of T-cell receptor clusters surrounded by a ring of adhesion molecules, including CD2. Read More
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    Human NF-κB1 Haploinsufficiency and Epstein-Barr Virus-Induced Disease-Molecular Mechanisms and Consequences.
    Front Immunol 2017 18;8:1978. Epub 2018 Jan 18.
    Ludwig Boltzmann Institute for Rare and Undiagnosed Diseases, Vienna, Austria.
    Nuclear factor kappa-light-chain-enhancer of activated B cells 1 (NF-κB1)-related human primary immune deficiencies have initially been characterized as defining a subgroup of common variable immunodeficiencies (CVIDs), representing intrinsic B-cell disorders with antibody deficiency and recurrent infections of various kind. Recent evidence indicates that NF-κB1 haploinsufficiency underlies a variable type of combined immunodeficiency (CID) affecting both B and T lymphocyte compartments, with a broadened spectrum of disease manifestations, including Epstein-Barr virus (EBV)-induced lymphoproliferative disease and immediate life-threatening consequences. As part of this review series focused on EBV-related primary immunodeficiencies, we discuss the current clinical and molecular understanding of monoallelic germline mutations with special focus on the emerging context of EBV-associated disease. Read More
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    Common Variable Immunodeficiency and Gastric Malignancies.
    Int J Mol Sci 2018 Feb 2;19(2). Epub 2018 Feb 2.
    Department of Biomedical Sciences and Human Oncology, Unit of Internal Medicine, University of Bari Medical School, 70124 Bari, Italy.
    Common variable immunodeficiency (CVID) is an immunodeficiency disorder with a high incidence of gastrointestinal manifestations and an increased risk of gastric carcinoma and lymphoma. This review discusses the latest advancements into the immunological, clinical and diagnostic aspects of gastric malignancies in patients with CVID. The exact molecular pathways underlying the relationships between CVID and gastric malignancies remain poorly understood. Read More
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    Antibodies against Pneumococcal Capsular Polysaccharides and Natural Anti-Galactosyl (Alpha-Gal) in Patients with Humoral Immunodeficiencies.
    J Immunol Res 2017 17;2017:7304658. Epub 2017 Dec 17.
    Department of Clinical Immunology and Allergy, Faculty of Medicine, Masaryk University, St. Anne's University Hospital, Brno, Czech Republic.
    Humoral deficiencies represent a broad group of disorders. The aim of the study was to compare the levels of antibodies against pneumococcal capsular polysaccharides (anti-PCP) and natural anti-galactosyl (anti-Gal) antibodies in (1) patients with chronic lymphocytic leukaemia (CLL), (2) patients with common variable immunodeficiency (CVID), and (3) a healthy population and to explore their diagnostic and prognostic potential. Serum immunoglobulin levels and levels of anti-Gal IgG, IgA, and IgM and anti-PCP IgG and IgG2 were determined in 59 CLL patients, 30 CVID patients, and 67 healthy controls. Read More
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    An Activating Janus Kinase-3 Mutation Is Associated with Cytotoxic T Lymphocyte Antigen-4-Dependent Immune Dysregulation Syndrome.
    Front Immunol 2017 15;8:1824. Epub 2017 Dec 15.
    Center for Chronic Immunodeficiency, University Medical Center Freiburg, Freiburg, Germany.
    Heterozygous mutations in the cytotoxic T lymphocyte antigen-4 (CTLA-4) are associated with lymphadenopathy, autoimmunity, immune dysregulation, and hypogammaglobulinemia in about 70% of the carriers. So far, the incomplete penetrance of CTLA-4 haploinsufficiency has been attributed to unknown genetic modifiers, epigenetic changes, or environmental effects. We sought to identify potential genetic modifiers in a family with differential clinical penetrance of CTLA-4 haploinsufficiency. Read More
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    Common Variable Immunodeficiency Non-Infectious Disease Endotypes Redefined Using Unbiased Network Clustering in Large Electronic Datasets.
    Front Immunol 2017 9;8:1740. Epub 2018 Jan 9.
    Massachusetts General Hospital, Boston, MA, United States.
    Common variable immunodeficiency (CVID) is increasingly recognized for its association with autoimmune and inflammatory complications. Despite recent advances in immunophenotypic and genetic discovery, clinical care of CVID remains limited by our inability to accurately model risk for non-infectious disease development. Herein, we demonstrate the utility of unbiased network clustering as a novel method to analyze inter-relationships between non-infectious disease outcomes in CVID using databases at the United States Immunodeficiency Network (USIDNET), the centralized immunodeficiency registry of the United States, and Partners, a tertiary care network in Boston, MA, USA, with a shared electronic medical record amenable to natural language processing. Read More
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    Autoimmune hepatitis as a complication of common variable immunodeficiency.
    Rev Esp Enferm Dig 2018 Mar;110(3):212-213
    Unidad de Gestión Clínica de Aparato Digestivo, Hospital Universitario Virgen del Rocío, España.
    In common variable immunodeficiency (CVID) there is a deregulation of the immune system, which frequently leads to an increased risk of infections, but also to autoimmunity phenomena. Autoimmune hepatitis may develop at any time of CVID's evolution, but it is difficult to diagnose due to the frequent absence of autoantibodies and low levels of IgG. Early diagnosis is important because targeted treatment may allow disease improvement. Read More
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    Purinergic signaling in B cells.
    Acta Biochim Pol 2018 23;65(1):1-7. Epub 2018 Jan 23.
    Department of Molecular Medicine, Medical University of Gdansk, Gdańsk, Poland.
    Adenosine and adenosine triphosphate are involved in purinergic signaling which plays an important role in control of the immune system. Much data have been obtained regarding impact of purinergic signaling on dendritic cells, macrophages, monocytes and T lymphocytes, however less attention has been paid to purinergic regulation of B cells. This review summarizes present knowledge on ATP- and Ado-dependent signaling in B lymphocytes. Read More
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    Reversible Suppression of Lymphoproliferation and Thrombocytopenia with Rapamycin in a Patient with Common Variable Immunodeficiency.
    J Clin Immunol 2018 Feb 19;38(2):159-162. Epub 2018 Jan 19.
    Immunology Division, Garvan Institute of Medical Research, Sydney, Australia.
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    The circulating T helper subsets and regulatory T cells in patients with common variable immunodeficiency with no known monogenic disease.
    J Investig Allergol Clin Immunol 2018 Jan 18. Epub 2018 Jan 18.
    Research Center for Immunodeficiencies, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran.
    Background: Common variable immunodeficiency (CVID) is the most common symptomatic primary immunodeficiency (PID), characterized by heterogeneous clinical manifestations and defects in B- and T- cells. In the present study, we investigated the T helper (Th) cell subsets and regulatory T (Treg) cells, and their related cytokines and transcription factors in the CVID patients with no definite genetic diagnosis.

    Methods: The study population comprised 13 CVID patients and 13 healthy controls (HC). Read More
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    High-Resolution Sequencing of Viral Populations during Early Simian Immunodeficiency Virus Infection Reveals Evolutionary Strategies for Rapid Escape from Emerging Env-Specific Antibody Responses.
    J Virol 2018 04 14;92(7). Epub 2018 Mar 14.
    Department of Biology, Boston College, Chestnut Hill, Massachusetts, USA
    Primate lentiviruses, including the human and simian immunodeficiency viruses (HIV and SIV), produce infections marked by persistent, ongoing viral replication. This occurs despite the presence of virus-specific adaptive immune responses, including antibodies targeting the viral envelope glycoprotein (Env), and evolution of antibody-escape variants is a well-documented feature of lentiviral infection. Here, we examined the evolutionary dynamics of the SIV gene during early infection (≤29 weeks postinfection) in a cohort of four SIV251-infected rhesus macaques. Read More
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    A difficult diagnosis of coeliac disease: Repeat duodenal histology increases diagnostic yield in patients with concomitant causes of villous atrophy.
    Arab J Gastroenterol 2017 Dec 8;18(4):241-244. Epub 2018 Jan 8.
    Internal Medicine Section, DIBIMIS, University of Palermo, Italy.
    Villous atrophy in absence of coeliac disease (CD)-specific antibodies represents a diagnostic dilemma. We report a case of a woman with anaemia, weight loss and diarrhoea with an initial diagnosis of seronegative CD and a histological documented villous atrophy who did not improve on gluten-free diet due to the concomitant presence of common variable immunodeficiency (CVID) and Giardia lamblia infection. This case report confirms that CD diagnosis in CVID patients is difficult; the combination of anti-endomysial antibodies (EmA-IgA), anti-tissue transglutaminase antibodies (tTG-IgAb) antibodies and total IgA is obligatory in basic diagnostic of CD but in CVID are negative. Read More
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    Ten-year experience of transjugular intrahepatic portosystemic shunt for noncirrhotic portal hypertension.
    Eur J Gastroenterol Hepatol 2018 May;30(5):557-562
    Departments of Hepatology.
    Background: Transjugular intrahepatic portosystemic shunt (TIPS) is considered to be well suited for the treatment of noncirrhotic portal hypertension (NCPHT) because of a usually severe portal hypertension (PHT) and a mild liver failure, but very less data are available.

    Patients And Methods: Records of patients referred for TIPS between 2004 and 2015 for NCPHT were reviewed. No patient should have clinical or biological or histological features of cirrhosis. Read More
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    Intra cranial granulomatous disease in common variable immunodeficiency: Case series and review of the literature.
    Semin Arthritis Rheum 2017 Oct 20. Epub 2017 Oct 20.
    Division of Rheumatology, Cleveland Clinic, Cleveland, OH.
    Background/purpose: Common variable immunodeficiency (CVID) is typically characterized by hypogammaglobulinemia and often but not always recurrent infections. Paradoxically, 8-22% of patients with CVID develop granulomatous disease. Granulomata have been described in many organs including the lungs, skin, liver, spleen, kidneys, eyes, lymph nodes, and intestines. Read More
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    [Common immunodeficiency variable in adults].
    Rev Alerg Mex 2017 Oct-Dec;64(4):452-462
    Instituto Mexicano del Seguro Social, Centro Médico Nacional Siglo XXI, Hospital de Especialidades, Servicio de Alergia e Inmunología Clínica, Ciudad de México, México.
    Primary immunodeficiencies (PIDs) are low-incidence diseases caused by defects in genes involved in the development, maintenance, and regulation of the immune system. Common variable immunodeficiency (CVID) is the most common symptomatic immunodeficiency of adulthood. It has an approximate prevalence of 1 in 25 000-50 000 in the general population, with a delay in diagnosis between 6-7 years. Read More
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    Long-term follow-up of IPEX syndrome patients after different therapeutic strategies: An international multicenter retrospective study.
    J Allergy Clin Immunol 2018 Mar 11;141(3):1036-1049.e5. Epub 2017 Dec 11.
    Division of Stem Cell Transplantation and Regenerative Medicine, Department of Pediatrics, Stanford University School of Medicine, Stanford, Calif. Electronic address:
    Background: Immunodysregulation polyendocrinopathy enteropathy x-linked (IPEX) syndrome is a monogenic autoimmune disease caused by FOXP3 mutations. Because it is a rare disease, the natural history and response to treatments, including allogeneic hematopoietic stem cell transplantation (HSCT) and immunosuppression (IS), have not been thoroughly examined.

    Objective: This analysis sought to evaluate disease onset, progression, and long-term outcome of the 2 main treatments in long-term IPEX survivors. Read More
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    Elizabethkingia miricola as an opportunistic oral pathogen associated with superinfectious complications in humoral immunodeficiency: a case report.
    BMC Infect Dis 2017 12 12;17(1):763. Epub 2017 Dec 12.
    Hirszfeld Institute of Immunology and Experimental Therapy, Polish Academy of Sciences, Rudolfa Weigla 12, 53-114, Wroclaw, Poland.
    Background: Elizabethkingia miricola is a rare Gram-negative bacterium found in water and clinical specimens. Typical culturing methods often misidentify Elizabethkingia spp. as Flavobacterium or Chryseobacterium. Read More
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    Initial intravenous immunoglobulin doses should be based on adjusted body weight in obese patients with primary immunodeficiency disorders.
    Allergy Asthma Clin Immunol 2017 6;13:47. Epub 2017 Dec 6.
    Department of Clinical Immunology, Auckland Hospital, Park Rd, Grafton 1010, Auckland, New Zealand.
    Background: Immunoglobulin therapy plays a critical role in the treatment of immunodeficiency disorders as well as autoimmune and inflammatory conditions. In immunodeficient patients, there has been controversy whether initial loading doses of intravenous (IVIG) should be based on actual body weight or a calculated parameter such as adjusted body weight in obese patients.

    Case Presentation: I describe a patient with Common Variable Immunodeficiency disorder (CVID) who underwent bariatric surgery for morbid obesity. Read More
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    Immunological Rare Diseases.
    Adv Exp Med Biol 2017 ;1031:497-509
    Department of Clinical and Biological Sciences, Turin University, Turin, Italy.
    The immune system is delegated to defend the body from attacks from outside or inside. Many diseases can affect immune system reducing its ability to defend self or inducing an abnormal response against external or internal antigens. Rare diseases affecting immune system present some issue in common with other rare diseases and some peculiarities due to the huge variability in the disease's expression. Read More
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    Terminally differentiated memory T cells are increased in patients with common variable immunodeficiency and selective IgA deficiency.
    Cent Eur J Immunol 2017 30;42(3):244-251. Epub 2017 Oct 30.
    Department of Clinical Immunology and Allergology, St Anne's University Hospital, and Faculty of Medicine, Masaryk University, Brno, Czech Republic.
    Introduction: Previous studies showed that several lymphocyte abnormalities seen in the most frequent symptomatic immunoglobulin deficiency, common variable immunodeficiency (CVID), were also observed in a genetically related asymptomatic disorder - selective IgA deficiency (IgAD). In this study we searched for abnormalities in the differentiation stages of T cells as well as for similarities of these abnormalities in CVID and IgAD patients.

    Material And Methods: Using flow cytometry in 80 patients with IgAD, 48 patients with CVID, and 80 control persons we determined T-lymphocyte subsets: both CD4 and CD8 were divided into the naïve CD45ROCD27, early differentiated CD45ROCD27, late differentiated CD45ROCD27 and fully differentiated effector CD45ROCD27 memory T cells, as well as Treg cells, defined as CD4CD25highCD127low T cells. Read More
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    [Common variable immune deficiency lately revealed by gastrointestinal problems: about a case].
    Pan Afr Med J 2017 20;28:48. Epub 2017 Sep 20.
    Service d'Hépato-Gastro-Entérologie, CHU Ibn Rochd, Casablanca, Maroc.
    Common Variable Immune Deficiency (CVID) is rare. It is a constitutional deficit of humoral immunity characterized by recurrent bacterial infections and by increased frequency of tumors, autoimmune or granulomatous diseases. Gastrointestinal manifestations are very variable and sometimes reveal common variable immune deficiency. Read More
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    A novel de novo activating mutation in STAT3 identified in a patient with common variable immunodeficiency (CVID).
    Clin Immunol 2018 Feb 24;187:132-136. Epub 2017 Nov 24.
    Institute of Biomedical and Clinical Science, University of Exeter, Exeter, UK.
    Common variable immunodeficiency (CVID) is characterised by repeated infection associated with primary acquired hypogammaglobulinemia. CVID frequently has a complex aetiology but, in certain cases, it has a monogenic cause. Recently, variants within the gene encoding the transcription factor STAT3 were implicated in monogenic CVID. Read More
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    Liver transplantation in adults with liver disease due to common variable immunodeficiency leads to early recurrent disease and poor outcome.
    Liver Transpl 2018 Feb;24(2):171-181
    The Liver Unit, Addenbrooke's Hospital, Cambridge University Hospitals National Health Service Foundation Trust, Cambridge, United Kingdom.
    Common variable immunodeficiency (CVID) is the most common form of primary immunodeficiency characterized by antibody deficiency, recurrent bacterial infections, and autoimmunity. Advanced chronic liver disease occurs in a subset of patients with CVID and manifests with various histological features, such as nodular regenerative hyperplasia, inflammation, fibrosis, and cholangiopathy. We present a case series characterizing the outcomes in adult patients transplanted for primary CVID-related liver disease. Read More
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