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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

    High-Resolution Sequencing of Viral Populations During Early SIV Infection Reveals Evolutionary Strategies for Rapid Escape from Emerging Env-Specific Antibody Responses.
    J Virol 2018 Jan 17. Epub 2018 Jan 17.
    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 env gene during early infection (≤ 29 weeks post-infection) in a cohort of four SIVmac251-infected rhesus macaques. Read More

    A difficult diagnosis of coeliac disease: Repeat duodenal histology increases diagnostic yield in patients with concomitant causes of villous atrophy.
    Arab J Gastroenterol 2018 Jan 8. 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

    Ten-year experience of transjugular intrahepatic portosystemic shunt for noncirrhotic portal hypertension.
    Eur J Gastroenterol Hepatol 2018 Jan 10. Epub 2018 Jan 10.
    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

    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

    [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

    Long-term follow up of IPEX syndrome patients after different therapeutic strategies: an international multicenter retrospective study.
    J Allergy Clin Immunol 2017 Dec 11. Epub 2017 Dec 11.
    Division of Stem Cell Transplantation and Regenerative Medicine, Department of Pediatrics, Stanford University School of Medicine, Stanford, CA, USA.
    Background: Immunedysregulation 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: To evaluate disease onset, progression and long-term outcome of the two main treatments in long-term IPEX survivors. Read More

    Elizabethkingia miricola as an opportunistic oral pathogen associated with superinfectious complications in humoral immunodeficiency: a case report.
    BMC Infect Dis 2017 Dec 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

    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

    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

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

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

    [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

    A novel de novo activating mutation in STAT3 identified in a patient with common variable immunodeficiency (CVID).
    Clin Immunol 2017 Nov 24. 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

    Liver transplantation in adults with liver disease due to common variable immunodeficiency leads to early recurrent disease and poor outcome.
    Liver Transpl 2017 Nov 20. Epub 2017 Nov 20.
    The Liver Unit, Addenbrooke's Hospital, Cambridge University Hospitals NHS Foundation Trust, Hills Road, Cambridge, CB2 0QQ, United Kingdom.
    Common variable immunodeficiency (CVID) is the commonest from 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

    Chronic Diarrhea in Common Variable Immunodeficiency: a Case Series and Review of the Literature.
    J Clin Immunol 2018 Jan 14;38(1):67-76. Epub 2017 Nov 14.
    Department of Translational Medical Sciences, Allergy and Clinical Immunology. Center for Basic and Clinical Immunology Research (CISI), University of Naples Federico II, Via S. Pansini 5, 80131, Naples, Italy.
    Common variable immunodeficiency (CVID) is a primary immunodeficiency characterized by reduced immunoglobulin serum levels and absent or impaired antibody production. Clinical manifestations, including infections, inflammatory and autoimmune diseases, and malignancies, also involve various segments of the gastrointestinal tract. Chronic diarrhea is one of the most common gastrointestinal symptoms and may cause a wide spectrum of potentially life-threatening conditions as malabsorption and protein-energy malnutrition. Read More

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

    Chronic meningoencephalitis caused by Echo virus 6 in a patient with common variable immunodeficiency : Successful treatment with pleconaril.
    Wien Klin Wochenschr 2018 Jan 7;130(1-2):70-72. Epub 2017 Nov 7.
    School of Medicine, University Hospital for Infectious Diseases "Dr. Fran Mihaljević", Department of Intensive Care Medicine and Neuroinfectology, University of Zagreb, Mirogojska cesta 8, Zagreb, Croatia.
    Chronic enteroviral meningoencephalitis, most commonly caused by echoviruses, can particularly be seen in agammaglobulinemic patients. In spite of the fact that no specific treatment for enteroviral infections exists, pleconaril is an antiviral drug reported to be efficient against enteroviral infections in infants and adults. We present a case of a 42-year-old male, previously diagnosed with common variable immunodeficiency, who presented with severe chronic meningoencephalitis caused by Echo virus 6 and was successfully treated with pleconaril. Read More

    Giardiasis mimicking celiac disease in a patient of common variable immunodeficiency.
    Trop Parasitol 2017 Jul-Dec;7(2):125-127
    Department of Pathology, All India Institute of Medical Sciences, Jodhpur, Rajasthan, India.
    An adolescent boy presented to pediatrics outpatient department with complaints of recurrent diarrhea, nausea, vomiting, and pedal edema since 3-4 months, with no relief even after taking treatment. His investigation revealed decreased serum IgA, IgG, and IgM levels with hypoproteinemia. Duodenal biopsy showed features of celiac disease (CD), but tissue transglutaminase IgA was negative. Read More

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

    Practical Approach to the Flattened Duodenal Biopsy.
    Surg Pathol Clin 2017 Dec 23;10(4):823-839. Epub 2017 Sep 23.
    Department of Pathology, Mayo Clinic, Hilton 11, 200 First Street Southwest, Rochester, MN 55902, USA. Electronic address:
    Celiac disease features duodenal intraepithelial lymphocytosis with or without villous atrophy. Lymphocytosis without villous atrophy will be proven to represent celiac disease in 10% to 20% of cases. The differential diagnosis is broad: Helicobacter pylori gastritis, NSAID injury and bacterial overgrowth are considerations. Read More

    Modulation of the Interleukin-21 Pathway with Interleukin-4 Distinguishes Common Variable Immunodeficiency Patients with More Non-infectious Clinical Complications.
    J Clin Immunol 2018 Jan 4;38(1):45-55. Epub 2017 Nov 4.
    Division of Allergy and Immunology, Department of Paediatrics, McGill University Health Centre, Montreal, Quebec, Canada.
    Purpose: Common variable immunodeficiency (CVID) is characterized by hypogammaglobulinemia and clinical manifestations such as infections, autoimmunity, and malignancy. We sought to determine if responsiveness to interleukin-21 (IL-21), a key cytokine for B cell differentiation, correlates with distinct clinical phenotypes in CVID.

    Methods: CVID subjects were recruited through the Canadian Primary Immunodeficiency Evaluative Survey registry. Read More

    Faecal microbiota transplantation in patients with Clostridium difficile and significant comorbidities as well as in patients with new indications: A case series.
    World J Gastroenterol 2017 Oct;23(39):7174-7184
    Department of Gastroenterology, Helsinki University Hospital, Helsinki 00029, Finland.
    Fecal microbiota transplantation (FMT) is effective in recurrent Clostridium difficile infection (rCDI). Knowledge of the safety and efficacy of FMT treatment in immune deficient patients is scarce. FMT has been suggested as a potential method for an increasing number of new indications besides rCDI. Read More

    Disseminated histoplasmosis in a patient with common variable immunodeficiency: A coincidence or the result of T cell defects?
    IDCases 2017 18;10:105-107. Epub 2017 Oct 18.
    Department of Medicine - Divisions of Hospital Medicine, Infectious Diseases and Critical Care, University of Missouri, One Hospital Dr, Columbia, MO 65212, United States.
    Background: In endemic regions, histoplasmosis is often seen in hosts with defective cell mediated immunity. We report a case of disseminated histoplasmosis in a patient with common variable immunodeficiency (CVID), a disorder mainly characterized by B cell defects.

    Case: A 35 year old male with CVID developed fever, headache, dyspnea and pancytopenia within few weeks of swimming in the Tennessee River. Read More

    Skewed Invariant Natural Killer T (iNKT) Cells, Impaired iNKT:B Cell Help and Decreased SAP Expression in Blood Lymphocytes from Patients with Common Variable Immunodeficiency.
    Scand J Immunol 2017 Sep;86(3):171-178
    Grupo de Inmunodeficiencias Primarias, Facultad de Medicina, Universidad de Antioquia, Medellín, Colombia.
    Common variable immunodeficiency (CVID) is a syndrome with predominantly defective B cell function. However, abnormalities in the number and function of other lymphocyte subpopulations in peripheral blood (PB) have been described in most patients. We have analysed the distribution of iNKT cell subpopulations in the PB of CVID patients and the ability of these cells to provide in vitro cognate B cell help. Read More

    Autoimmune Cytopenias and Associated Conditions in CVID: a Report From the USIDNET Registry.
    J Clin Immunol 2018 Jan 28;38(1):28-34. Epub 2017 Oct 28.
    Weill Cornell Medical College, 505 East 70th street, New York, NY, 10021, USA.
    Purpose: Autoimmune cytopenia is frequently a presenting manifestation of common variable immune deficiency (CVID). Studies characterizing the CVID phenotype associated with autoimmune cytopenias have mostly been limited to large referral centers. Here, we report prevalence of autoimmune cytopenias in CVID from the USIDNET Registry and compare the demographics and clinical features of patients with and without this complication. Read More

    High prevalence of pain among adult HIV-infected patients at University of Gondar Hospital, Northwest Ethiopia.
    J Pain Res 2017 13;10:2461-2469. Epub 2017 Oct 13.
    Community Health Unit, College of Medicine and Health Science, University of Gondar, Gondar, Ethiopia.
    Background: HIV/AIDS are pressing global health problems. Pain is a common symptom reported by patients living with HIV/AIDS. The exact cause of pain in HIV patients has not been thoroughly described, but it may, due to a symptom of HIV itself, result from opportunistic infections, as a side effect of antiretroviral drugs, concurrent neoplasia or other causes. Read More

    Unexpectedly High Prevalence of Common Variable Immunodeficiency in Finland.
    Front Immunol 2017 28;8:1190. Epub 2017 Sep 28.
    Adult Immunodeficiency Unit, Infectious Diseases, Inflammation Center, University of Helsinki, Helsinki University Hospital, Helsinki, Finland.
    Background: Common variable immunodeficiency (CVID) is the most common primary immunodeficiency. Prevalence varies greatly between countries and studies. Most diagnostic criteria include hypogammaglobulinemia and impaired vaccine response. Read More

    Review: Diagnosing Common Variable Immunodeficiency Disorder in the Era of Genome Sequencing.
    Clin Rev Allergy Immunol 2017 Oct 14. Epub 2017 Oct 14.
    Auckland Hospital, Park Rd, Grafton, Auckland, 1010, New Zealand.
    Common variable immunodeficiency disorders (CVID) are an enigmatic group of often heritable conditions, which may manifest for the first time in early childhood or as late as the eighth decade of life. In the last 5 years, next generation sequencing (NGS) has revolutionised identification of genetic disorders. However, despite the best efforts of researchers around the globe, CVID conditions have been slow to yield their molecular secrets. Read More

    Patients with common variable immunodeficiency paradoxically have increased rates of autoimmune disorders.
    BMJ Case Rep 2017 Oct 13;2017. Epub 2017 Oct 13.
    Department of Infectious Diseases, University of Vermont Medical Center and Larner College of Medicine, Boston, Massachusetts, USA.
    Common variable immunodeficiency (CVID), characterised by disordered B cell function, is one of the most common primary immunodeficiency disorders. Patients with CVID are at lifelong risk of recurrent infections, particularly of the respiratory and gastrointestinal tracts. Paradoxically, given their immunocompromised state, patients with CVID are also at significantly increased risk of autoimmune disorders, which are seen in almost 25% of cases. Read More

    Autoimmunity in a cohort of 471 patients with primary antibody deficiencies.
    Expert Rev Clin Immunol 2017 Nov 11;13(11):1099-1106. Epub 2017 Oct 11.
    c Research Center for Immunodeficiencies, Children's Medical Center , Tehran University of Medical Sciences , Tehran , Iran.
    Objectives: The aim of this study was to evaluate the frequency of autoimmunity in primary antibody deficiency (PAD).

    Methods: A total of 471 patients with PADs enrolled in this retrospective cohort study. For all patients' demographic information, clinical records and laboratory data were collected to investigate autoimmune complications. Read More

    Prolonged Excretion of Poliovirus among Individuals with Primary Immunodeficiency Disorder: An Analysis of the World Health Organization Registry.
    Front Immunol 2017 25;8:1103. Epub 2017 Sep 25.
    World Health Organization, Geneva, Switzerland.
    Individuals with primary immunodeficiency disorder may excrete poliovirus for extended periods and will constitute the only remaining reservoir of virus after eradication and withdrawal of oral poliovirus vaccine. Here, we analyzed the epidemiology of prolonged and chronic immunodeficiency-related vaccine-derived poliovirus cases in a registry maintained by the World Health Organization, to identify risk factors and determine the length of excretion. Between 1962 and 2016, there were 101 cases, with 94/101 (93%) prolonged excretors and 7/101 (7%) chronic excretors. Read More

    Dysregulation of Innate Lymphoid Cells in Common Variable Immunodeficiency.
    Curr Allergy Asthma Rep 2017 Oct 5;17(11):77. Epub 2017 Oct 5.
    Division of Clinical Immunology, Department of Medicine, Icahn School of Medicine at Mount Sinai, One Gustave L. Levy Place, Box 1089, New York, NY, 10029, USA.
    Common variable immunodeficiency (CVID) is the most prevalent symptomatic primary immune deficiency. With widespread use of immunoglobulin replacement therapy, non-infectious complications, such as autoimmunity, chronic intestinal inflammation, and lung disease, have replaced infections as the major cause of morbidity and mortality in this immune deficiency. The pathogenic mechanisms that underlie the development of these complications in CVID are not known; however, there have been numerous associated laboratory findings. Read More

    Somatic Hypermutation Defects in Common Variable Immune Deficiency.
    Curr Allergy Asthma Rep 2017 Oct 5;17(11):76. Epub 2017 Oct 5.
    Laboratorio de Inmunología Oncológica, Instituto de Medicina Experimental (IMEX) - Consejo Nacional de Investigaciones Científicas y Técnicas (CONICET), Academia Nacional de Medicina (ANM), Buenos Aires, Argentina.
    Common variable immunodeficiency (CVID) is a heterogeneous disorder characterized by impaired antibody production and recurrent infections. In the last 20 years, several groups have reported that B cells from CVID patients have an impaired somatic hypermutation (SHM). The reported frequency of this defect among CVID patient cohorts is highly variable and so is the methodology used to evaluate this process. Read More

    Influenza Vaccination in Patients with Common Variable Immunodeficiency (CVID).
    Curr Allergy Asthma Rep 2017 Oct 5;17(11):78. Epub 2017 Oct 5.
    Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Institute of Medical Immunology, Augustenburger Platz 1, 13353, Berlin, Germany.
    Purpose Of Review: Vaccination against influenza in patients with primary antibody deficiency is recommended. Common variable immunodeficiency (CVID) is the most frequent and clinically relevant antibody deficiency disease and is by definition characterized by an impaired vaccination response. The purpose of this review is to present the current knowledge of humoral and cellular vaccine response to influenza in CVID patients. Read More

    TLR9 stimulation of B-cells induces transcription of p53 and prevents spontaneous and irradiation-induced cell death independent of DNA damage responses. Implications for Common variable immunodeficiency.
    PLoS One 2017 3;12(10):e0185708. Epub 2017 Oct 3.
    Department of Molecular Medicine, Institute of Basic Medical Sciences, University of Oslo, Oslo, Norway.
    In the present study, we address the important issue of whether B-cells protected from irradiation-induced cell death, may survive with elevated levels of DNA damage. If so, such cells would be at higher risk of gaining mutations and undergoing malignant transformation. We show that stimulation of B-cells with the TLR9 ligands CpG-oligodeoxynucleotides (CpG-ODN) prevents spontaneous and irradiation-induced death of normal peripheral blood B-cells, and of B-cells from patients diagnosed with Common variable immunodeficiency (CVID). Read More

    Multiple Presentations of LRBA Deficiency: a Single-Center Experience.
    J Clin Immunol 2017 Nov 27;37(8):790-800. Epub 2017 Sep 27.
    Department of Pediatric Allergy and Immunology, Ankara University School of Medicine, Cebeci, 06590, Ankara, Turkey.
    Introduction: LPS-responsive beige-like anchor protein (LRBA) deficiency is a primary immunodeficiency categorized as common variable immunodeficiency associated with autoimmune manifestations and inflammatory bowel diseases; however, the clinical spectrum has been extended. Here, we present our cohort of Turkish LRBA-deficient patients from a single center, demonstrating a diversity of clinical manifestations.

    Method: Seven affected individuals from five families were assessed retrospectively in this study. Read More

    Evans Syndrome as First Manifestation of Primary Immunodeficiency in Clinical Practice.
    J Pediatr Hematol Oncol 2017 Oct;39(7):490-494
    Departments of *Allergy and Clinical Immunology ‡Hematology and Oncology, Hospital Sant Joan de Dèu, Institut de Recerca Pediàtrica Hospital Sant Joan de Dèu, Esplugues de Llobregat §Immunology Department, Hospital Clinic-IDIBAPS, Universitat de Barcelona †Functional Unit of Clinical Immunology SJD-Clinic, Barcelona, Spain.
    Background: Evans syndrome (ES) is a rare immune disorder in children, manifested by simultaneous or sequential autoimmune cytopenias (ACs) of unknown cause and having a chronic course with periods of exacerbation and remission. Some primary immunodeficiencies (PIDs) may present with autoimmune manifestations without infections, masking suspicion of them. The PIDs that can typically manifest as ES are autoimmune lymphoproliferative syndrome and common variable immunodeficiency (CVID). Read More

    Preferential Reduction of Circulating Innate Lymphoid Cells Type 2 in Patients with Common Variable Immunodeficiency with Secondary Complications Is Part of a Broader Immune Dysregulation.
    J Clin Immunol 2017 Nov 21;37(8):759-769. Epub 2017 Sep 21.
    Center for Chronic Immunodeficiency (CCI), Medical Center-University of Freiburg, Faculty of Medicine, University of Freiburg, Breisacherstr. 115, 79106, Freiburg, Germany.
    Purpose: Over a third of patients with common variable immunodeficiency (CVID) suffer from secondary complications like inflammatory organ disease, autoimmune manifestations, or lymphoproliferation contributing to increased morbidity and mortality in affected patients. Innate lymphoid cells (ILCs) have emerging roles in setting the milieu for physiological, but also pathological, immune responses and inflammation. We therefore sought to correlate the recently identified disturbed homeostasis of ILCs with alterations of the adaptive immune system in complex CVID patients (CVIDc). Read More

    Hypogammaglobulinemia in children: a warning sign to look deeply?
    APMIS 2017 Oct;125(10):902-909
    Department of Pediatrics, Escola Paulista de Medicina, Universidade Federal de São Paulo-UNIFESP, São Paulo, Brazil.
    This study investigated phenotypic and functional characteristics of lymphocytes in children with common variable immunodeficiency (CVID) and unclassified hypogammaglobulinemia (UH), as well as B-cell subsets in non-consanguineous parents. Blood samples of 30 children, CVID (n = 9), UH (n = 9), healthy donors HD (n = 12), and 19 adults (parents and controls) were labeled by a combination of surface markers to identify CD4, CD8 T-cell and B-cell subpopulations. T-cell cytokine production in children was analyzed in vitro after stimulation with phytohemagglutinin (PHA) and tetanus toxoid. Read More

    Role of Apoptosis in the Pathogenesis of Common Variable Immunodeficiency (CVID).
    Endocr Metab Immune Disord Drug Targets 2017 Nov;17(4):332-340
    bResearch Center for Immunodeficiencies, Pediatrics Center of Excellence, Children`s Medical Center, Tehran University of Medical Science, Tehran, Iran.
    Background: Common variable immunodeficiency (CVID) is a heterogeneous immune deficiency characterized by hypogammaglobulinemia. Since B cell maturation and differentiation is defective in this disorder, we evaluated apoptosis in B cells of patients with CVID compared with healthy donors (HD).

    Methods: Determination of peripheral blood B-cell subsets in CVID and HDs, was performed using flow cytometry. Read More

    Passive blood anaphylaxis: subcutaneous immunoglobulins are a cause of ongoing passive anaphylactic reaction.
    Allergy Asthma Clin Immunol 2017 15;13:41. Epub 2017 Sep 15.
    Department of Immunology of Infectious Diseases, Hirszfeld Institute of Immunology and Experimental Therapy, Polish Academy of Sciences, POBox 1818, 50-385 Wrocław-46, Poland.
    Background: Allergic, especially anaphylactic, reactions during immunoglobulin replacement therapy are rare, but their pathophysiology and classification remain ambiguous. Recent findings show positive results of skin tests with commercially available immunoglobulins, but target antigens and responsible compounds of the tested immunoglobulins have not been strictly identified.

    Case Description And Findings: Four adult patients with recently diagnosed common variable immunodeficiency qualified for standard subcutaneous immunoglobulin replacement therapy regimen. Read More

    Intravenous Immunoglobulin Monotherapy for Granulomatous Lymphocytic Interstitial Lung Disease in Common Variable Immunodeficiency.
    Intern Med 2017 Nov 15;56(21):2899-2902. Epub 2017 Sep 15.
    Department of Pathology, Japanese Red Cross Medical Center, Japan.
    Common variable immunodeficiency (CVID) is a heterogeneous subset of immunodeficiency disorders. Recurrent bacterial infection is the main feature of CVID, but various non-infectious complications can occur. A 42-year-old woman presented with cough and abnormal chest X-ray shadows. Read More

    The Lung in Dysregulated States of Humoral Immunity.
    Respiration 2017 15;94(5):389-404. Epub 2017 Sep 15.
    Université Paris 13, Sorbonne-Paris-Cité, EA2363, Paris, France.
    In common variable immunodeficiency, lung manifestations are related to different mechanisms: recurrent pneumonias due to encapsulated bacteria responsible for diffuse bronchiectasis, diffuse infiltrative pneumonia with various patterns, and lymphomas, mostly B cell extranodal non-Hodgkin type. The diagnosis relies on significant serum Ig deficiency and the exclusion of any primary or secondary cause. Histopathology may be needed. Read More

    Predictors of granulomatous lymphocytic interstitial lung disease in common variable immunodeficiency: Methodological issues.
    Ann Allergy Asthma Immunol 2017 Sep;119(3):296
    Managerial Epidemiology Research Center, Department of Public Health, School of Nursing and Midwifery, Maragheh University of Medical Sciences, Maragheh, Iran; Department of Epidemiology and Biostatistics, School of Public Health, Tehran University of Medical Sciences, Tehran, Iran. Electronic address:

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