common variable immunodeficiency

Summary

Summary: Heterogeneous group of immunodeficiency syndromes characterized by hypogammaglobulinemia of most isotypes, variable B-cell defects, and the presence of recurrent bacterial infections.

Top Publications

  1. pmc Morbidity and mortality in common variable immune deficiency over 4 decades
    Elena S Resnick
    Immunology Institute, Mount Sinai School of Medicine, New York, NY 10029, USA
    Blood 119:1650-7. 2012
  2. ncbi Thymic and bone marrow output in patients with common variable immunodeficiency
    Federico Serana
    Department of Biomedical Science and Biotechnology, University of Brescia, Brescia, Italy
    J Clin Immunol 31:540-9. 2011
  3. ncbi The EUROclass trial: defining subgroups in common variable immunodeficiency
    Claudia Wehr
    Department of Rheumatology and Clinical Immunology, University Clinic, Freiburg, Germany
    Blood 111:77-85. 2008
  4. ncbi Common variable immunodeficiency disorders: division into distinct clinical phenotypes
    Helen Chapel
    Department of Clinical Immunology, Nuffield Department of Medicine, University of Oxford, Oxford, United Kingdom
    Blood 112:277-86. 2008
  5. pmc Granulomatous disease in common variable immunodeficiency
    Omür Ardeniz
    Ege University Medical Faculty, Department of Internal Medicine, Division of Allergy and Clinical Immunology, Izmir, Turkey
    Clin Immunol 133:198-207. 2009
  6. ncbi Immune competence and switched memory B cells in common variable immunodeficiency
    Jimmy Ko
    Division of Clinical Immunology, Department of Medicine, Mount Sinai School of Medicine, 1425 Madison Avenue, Box 1089, New York, NY 10029, USA
    Clin Immunol 116:37-41. 2005
  7. ncbi Common variable immunodeficiency patient classification based on impaired B cell memory differentiation correlates with clinical aspects
    B Piqueras
    Laboratoire d Immunotogie Cellulaire, INSERM U543, Hopital Pitie Salpetriere, Paris, France
    J Clin Immunol 23:385-400. 2003
  8. ncbi Common variable immunodeficiency: a new look at an old disease
    Miguel A Park
    Division of Allergic Diseases, Department of Internal Medicine, Mayo Clinic, Rochester, MN, USA
    Lancet 372:489-502. 2008
  9. ncbi Infection outcomes in patients with common variable immunodeficiency disorders: relationship to immunoglobulin therapy over 22 years
    Mary Lucas
    Department of Clinical Immunology, Nuffield Department of Medicine, University of Oxford, Oxford, United Kingdom
    J Allergy Clin Immunol 125:1354-1360.e4. 2010
  10. ncbi Severe deficiency of switched memory B cells (CD27(+)IgM(-)IgD(-)) in subgroups of patients with common variable immunodeficiency: a new approach to classify a heterogeneous disease
    Klaus Warnatz
    Division of Rheumatology and Clinical Immunology, Department of Medicine, University Hospital of Freiburg, Germany
    Blood 99:1544-51. 2002

Detail Information

Publications315 found, 100 shown here

  1. pmc Morbidity and mortality in common variable immune deficiency over 4 decades
    Elena S Resnick
    Immunology Institute, Mount Sinai School of Medicine, New York, NY 10029, USA
    Blood 119:1650-7. 2012
    ....
  2. ncbi Thymic and bone marrow output in patients with common variable immunodeficiency
    Federico Serana
    Department of Biomedical Science and Biotechnology, University of Brescia, Brescia, Italy
    J Clin Immunol 31:540-9. 2011
    The study aims to obtain more information about the immune deficit of common variable immunodeficiency (CVID) patients.
  3. ncbi The EUROclass trial: defining subgroups in common variable immunodeficiency
    Claudia Wehr
    Department of Rheumatology and Clinical Immunology, University Clinic, Freiburg, Germany
    Blood 111:77-85. 2008
    The heterogeneity of common variable immunodeficiency (CVID) calls for a classification addressing pathogenic mechanisms as well as clinical relevance...
  4. ncbi Common variable immunodeficiency disorders: division into distinct clinical phenotypes
    Helen Chapel
    Department of Clinical Immunology, Nuffield Department of Medicine, University of Oxford, Oxford, United Kingdom
    Blood 112:277-86. 2008
    The European Common Variable Immunodeficiency Disorders registry was started in 1996 to define distinct clinical phenotypes and determine overlap within individual patients...
  5. pmc Granulomatous disease in common variable immunodeficiency
    Omür Ardeniz
    Ege University Medical Faculty, Department of Internal Medicine, Division of Allergy and Clinical Immunology, Izmir, Turkey
    Clin Immunol 133:198-207. 2009
    Granulomatous disease occurs in 8-22% of patients with common variable immunodeficiency (CVID). We examined the clinical and immunologic information of all 37 of 455 (8.1%) CVID subjects with this complication...
  6. ncbi Immune competence and switched memory B cells in common variable immunodeficiency
    Jimmy Ko
    Division of Clinical Immunology, Department of Medicine, Mount Sinai School of Medicine, 1425 Madison Avenue, Box 1089, New York, NY 10029, USA
    Clin Immunol 116:37-41. 2005
    b>Common variable immunodeficiency (CVID) is presumed to be a heterogenous group of disorders with potentially separate etiologies...
  7. ncbi Common variable immunodeficiency patient classification based on impaired B cell memory differentiation correlates with clinical aspects
    B Piqueras
    Laboratoire d Immunotogie Cellulaire, INSERM U543, Hopital Pitie Salpetriere, Paris, France
    J Clin Immunol 23:385-400. 2003
    b>Common variable immunodeficiency (CVID) is a very heterogeneous syndrome defined by impaired immunoglobulin production...
  8. ncbi Common variable immunodeficiency: a new look at an old disease
    Miguel A Park
    Division of Allergic Diseases, Department of Internal Medicine, Mayo Clinic, Rochester, MN, USA
    Lancet 372:489-502. 2008
    ..b>Common variable immunodeficiency (CVID) is the primary immunodeficiency most commonly encountered in clinical practice, and ..
  9. ncbi Infection outcomes in patients with common variable immunodeficiency disorders: relationship to immunoglobulin therapy over 22 years
    Mary Lucas
    Department of Clinical Immunology, Nuffield Department of Medicine, University of Oxford, Oxford, United Kingdom
    J Allergy Clin Immunol 125:1354-1360.e4. 2010
    b>Common variable immunodeficiency disorders (CVIDs) are the most common forms of symptomatic primary antibody failure in adults and children...
  10. ncbi Severe deficiency of switched memory B cells (CD27(+)IgM(-)IgD(-)) in subgroups of patients with common variable immunodeficiency: a new approach to classify a heterogeneous disease
    Klaus Warnatz
    Division of Rheumatology and Clinical Immunology, Department of Medicine, University Hospital of Freiburg, Germany
    Blood 99:1544-51. 2002
    Hypogammaglobulinemia is the hallmark of common variable immunodeficiency (CVID) syndrome, a heterogeneous disorder predisposing patients to recurrent bacterial infections...
  11. ncbi Human ICOS deficiency abrogates the germinal center reaction and provides a monogenic model for common variable immunodeficiency
    Klaus Warnatz
    Division of Rheumatology and Clinical Immunology, University Hospital, Freiburg, Germany
    Blood 107:3045-52. 2006
    ..patients revealed that this monogenic disease comprises the full clinical phenotype described for common variable immunodeficiency (CVID), including recurrent bacterial infections, adult as well as childhood onset, splenomegaly, ..
  12. ncbi Long-term follow-up and outcome of a large cohort of patients with common variable immunodeficiency
    Isabella Quinti
    Department of Clinical Immunology, University of Rome La Sapienza Rome, Rome, Italy
    J Clin Immunol 27:308-16. 2007
    b>Common Variable Immunodeficiency belongs to the group of rare diseases encompassing antibody deficiency syndromes of highly variable clinical presentation and outcome...
  13. ncbi Nodular regenerative hyperplasia: the main liver disease in patients with primary hypogammaglobulinemia and hepatic abnormalities
    Georgia Malamut
    Hepatology and Gastroenterology Department, Hopital Europeen Georges Pompidou, Paris, France
    J Hepatol 48:74-82. 2008
    ..Liver lesions associated with primary hypogammaglobulinemia have been poorly described. We aimed to assess the clinical, histological and immune features and outcome of hepatic injury in patients with primary hypogammaglobulinemia...
  14. pmc Efficacy and safety of rituximab in common variable immunodeficiency-associated immune cytopenias: a retrospective multicentre study on 33 patients
    Delphine Gobert
    Department of Internal Medicine, Hopital Henri Mondor, Creteil, Paris, France
    Br J Haematol 155:498-508. 2011
    Patients with common variable immunodeficiency (CVID) are at high risk of developing immune thrombocytopenia (ITP) and/or autoimmune haemolytic anaemia (AHA)...
  15. pmc Monocyte activation is a feature of common variable immunodeficiency irrespective of plasma lipopolysaccharide levels
    R R Barbosa
    Instituto de Medicina Molecular, Faculdade de Medicina, Universidade de Lisboa Serviço de Imunoalergologia, Hospital de Santa Maria, Centro Hospitalar Lisboa Norte, Lisboa, Portugal
    Clin Exp Immunol 169:263-72. 2012
    b>Common variable immunodeficiency disorders (CVID), the most frequent cause of symptomatic primary immunodeficiency, are defined by impaired antibody production...
  16. ncbi Hematologic complications of primary immune deficiencies
    C Cunningham-Rundles
    Department of Medicine, Mount Sinai Medical Center, New York, NY 10029, USA
    Blood Rev 16:61-4. 2002
    ..common in all the congenital immune diseases, they are particularly common in the antibody defects, common variable immunodeficiency and selective immunoglobulin A deficiency. In common variable immunodeficiency, ITP occurred in 7...
  17. pmc Chronic immune activation in common variable immunodeficiency (CVID) is associated with elevated serum levels of soluble CD14 and CD25 but not endotoxaemia
    J Litzman
    Department of Clinical Immunology and Allergology, Faculty of Medicine, Masaryk University and St Anne s University Hospital, Brno, Czech Republic
    Clin Exp Immunol 170:321-32. 2012
    b>Common variable immunodeficiency (CVID), the most frequent symptomatic immunoglobulin primary immunodeficiency, is associated with chronic T cell activation and reduced frequency of CD4(+) T cells...
  18. ncbi Association of IL-4 and IL-10 gene promoter polymorphisms with common variable immunodeficiency
    Nima Rezaei
    Immunology, Asthma and Allergy Research Institute, Children s Medical Center Hospital, Tehran University of Medical Sciences, Iran
    Immunobiology 215:81-7. 2010
    b>Common variable immunodeficiency (CVID) is a heterogeneous group of disorders, characterized by severe reduction in serum IgG and IgA with normal or low number of B-cells...
  19. ncbi Pulmonary complications of primary immunodeficiencies
    Rebecca H Buckley
    Departments of Pediatrics and Immunology, Duke University Medical Center, Durham, NC 27710, USA
    Paediatr Respir Rev 5:S225-33. 2004
    ..Finally, advances in treatment of the underlying diseases as well as their infectious complications will be covered...
  20. ncbi The outcome of patients with unclassified hypogammaglobulinemia in early childhood
    Necil Kutukculer
    Division of Pediatric Immunology, Department of Pediatrics, Faculty of Medicine, Ege University, Izmir, Turkey
    Pediatr Allergy Immunol 20:693-8. 2009
    ..three partial IgA deficiency, seven IgG subclass deficiency, two selective IgM deficiency and two common variable immunodeficiency (CVID) were diagnosed by long-term monitoring of immunoglobulin levels. Five (13...
  21. ncbi B cell receptor-mediated calcium signaling is impaired in B lymphocytes of type Ia patients with common variable immunodeficiency
    Christian Foerster
    Division of Rheumatology and Clinical Immunology, Centre of Chronic Immunodeficiency, University Medical Centre, Freiburg, Germany
    J Immunol 184:7305-13. 2010
    Several lines of evidence have demonstrated B cell intrinsic activation defects in patients with common variable immunodeficiency (CVID)...
  22. ncbi Diagnostic findings in 95 Finnish patients with common variable immunodeficiency
    L Kainulainen
    Department of Pediatrics, Turku University Hospital, Finland
    J Clin Immunol 21:145-9. 2001
    b>Common variable immunodeficiency is the most frequent of the primary hypogammaglobulinemias. It is manifested by a wide variety of clinical signs and symptoms...
  23. ncbi Treatment and outcome of autoimmune hematologic disease in common variable immunodeficiency (CVID)
    Julie Wang
    Division of Clinical Immunology, Mount Sinai Medical Center, New York, NY 10029, USA
    J Autoimmun 25:57-62. 2005
    b>Common variable immunodeficiency (CVID) is associated with autoimmunity, most commonly immune thrombocytopenic purpura (ITP) and autoimmune hemolytic anemia (AIHA)...
  24. ncbi Common variable immune deficiency and autoimmunity
    Daniel Brandt
    Division of Rheumatology, Allergy and Clinical Immunology, University of California at Davis School of Medicine, 451 E Health Sciences Drive, Suite 6510, Davis, CA 95616, USA
    Autoimmun Rev 5:465-70. 2006
    b>Common variable immunodeficiency (CVID) is a heterogeneous syndrome characterized by various degrees of hypogammaglobulinemia...
  25. pmc Circulating CD21low B cells in common variable immunodeficiency resemble tissue homing, innate-like B cells
    Mirzokhid Rakhmanov
    Centre of Chronic Immunodeficiency and Division of Rheumatology and Clinical Immunology, University Medical Center Freiburg, 79106 Freiburg, Germany
    Proc Natl Acad Sci U S A 106:13451-6. 2009
    ..Thus, a subgroup of patients with common variable immunodeficiency (CVID) presents with an extraordinary expansion of an unusual B cell population characterized by the ..
  26. pmc Memory B cells in common variable immunodeficiency: clinical associations and sex differences
    Silvia Sánchez-Ramón
    Department of Medicine, Pediatrics and Immunobiology Center, Mount Sinai Medical School, New York, NY 10029, USA
    Clin Immunol 128:314-21. 2008
    b>Common variable immunodeficiency (CVID) is a heterogeneous syndrome characterized by impaired antibody responses, recurrent infections, inflammatory, autoimmune and malignancy-related conditions...
  27. pmc Update in understanding common variable immunodeficiency disorders (CVIDs) and the management of patients with these conditions
    Helen Chapel
    Department of Clinical Immunology, Oxford Radcliffe Hospitals, Oxford, UK
    Br J Haematol 145:709-27. 2009
    The common variable immunodeficiency disorders are a mixed group of heterogeneous conditions linked by lack of immunoglobulin production and primary antibody failure...
  28. ncbi The loss of IgM memory B cells correlates with clinical disease in common variable immunodeficiency
    Rita Carsetti
    Research Center, Ospedale Pediatrico Bambino Gesu, Rome, Italy
    J Allergy Clin Immunol 115:412-7. 2005
    ..tract infections caused by encapsulated bacteria might cause permanent organ damage in patients with common variable immunodeficiency (CVID)...
  29. ncbi Effectiveness of immunoglobulin replacement therapy on clinical outcome in patients with primary antibody deficiencies: results from a multicenter prospective cohort study
    Isabella Quinti
    Department of Clinical Immunology, Sapienza University of Rome, Viale dell Universita 37, 00185, Rome, Italy
    J Clin Immunol 31:315-22. 2011
    ....
  30. pmc Genome-wide association identifies diverse causes of common variable immunodeficiency
    Jordan S Orange
    Division of Allergy and Immunology, Children s Hospital of Philadelphia Research Institute, Children s Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, PA, USA
    J Allergy Clin Immunol 127:1360-7.e6. 2011
    b>Common variable immunodeficiency (CVID) is a heterogeneous immune defect characterized by hypogammaglobulinemia, failure of specific antibody production, susceptibility to infections, and an array of comorbidities.
  31. pmc Autoimmunity in common variable immunodeficiency
    Shradha Agarwal
    Mount Sinai Medical Center, New York, NY 10029, USA
    Curr Allergy Asthma Rep 9:347-52. 2009
    b>Common variable immunodeficiency (CVID) is the most common clinically significant primary immune defect...
  32. ncbi Homozygous loss of ICOS is associated with adult-onset common variable immunodeficiency
    Bodo Grimbacher
    Division of Rheumatology and Clinical Immunology, Medical School, University of Freiburg, Hugstetterstr 55, 79106 Freiburg, Germany
    Nat Immunol 4:261-8. 2003
    No genetic defect is known to cause common variable immunodeficiency (CVID), a heterogeneous human disorder leading to adult-onset panhypogammaglobulinemia...
  33. ncbi Expansion of CD19(hi)CD21(lo/neg) B cells in common variable immunodeficiency (CVID) patients with autoimmune cytopenia
    Klaus Warnatz
    Division of Rheumatology and Clinical Immunology, Dept of Medicine, University Hospital Freiburg, Germany
    Immunobiology 206:502-13. 2002
    b>Common variable immunodeficiency (CVID) is characterized by a severe hypogammaglobulinemia...
  34. pmc Granulomatous disease: distinguishing primary antibody disease from sarcoidosis
    D F Arnold
    Department of Clinical Immunology, John Radcliffe Hospital, Headley Way, Oxford OX3 9DU, UK
    Clin Immunol 128:18-22. 2008
  35. pmc Changes in B cell immunophenotype in common variable immunodeficiency: cause or effect - is bronchiectasis indicative of undiagnosed immunodeficiency?
    P Bright
    Immunology Department, Barts Health NHS Trust, London, UK
    Clin Exp Immunol 171:195-200. 2013
    b>Common variable immunodeficiency (CVID) is the most common severe primary immunodeficiency, but the pathology of this condition is poorly understood...
  36. pmc Efficiency of immunoglobulin G replacement therapy in common variable immunodeficiency: correlations with clinical phenotype and polymorphism of the neonatal Fc receptor
    V Gouilleux-Gruart
    CNRS UMR 7292, Universite Francois Rabelais, CHRU de Tours, Tours, France
    Clin Exp Immunol 171:186-94. 2013
    Treatment of common variable immunodeficiency disorders (CVID) is based on replacement therapy using intravenous (i.v.) or subcutaneous (s.c.) immunoglobulin (Ig)G. Interindividual variation of IgG dose is common...
  37. ncbi Practice parameter for the diagnosis and management of primary immunodeficiency
    Francisco A Bonilla
    Department of Medicine, Children s Hospital Boston, Harvard Medical School, Boston, Massachusetts, USA
    Ann Allergy Asthma Immunol 94:S1-63. 2005
  38. ncbi Late-onset combined immune deficiency: a subset of common variable immunodeficiency with severe T cell defect
    Marion Malphettes
    Departement d Immunologie, Hopital Saint Louis, Assistance Publique Hopitaux de Paris, Paris, France
    Clin Infect Dis 49:1329-38. 2009
    b>Common variable immunodeficiency (CVID) is a primary immune deficiency defined by defective antibody production. In most series, a small proportion of patients present with opportunistic infections (OIs).
  39. ncbi Common variable immunodeficiency (CVID): exploring the multiple dimensions of a heterogeneous disease
    Ulrich Salzer
    Centre of Chronic Immunodeficiency, University Medical Centre Freiburg, Germany
    Ann N Y Acad Sci 1250:41-9. 2012
    b>Common variable immunodeficiency (CVID) represents a large heterogeneous group of antibody deficiency syndromes associated with a plethora of clinical features and as yet largely undefined molecular causes...
  40. pmc Perspectives on common variable immune deficiency
    Joon H Park
    Department of Medicine and the Immunology Institute, Mount Sinai School of Medicine, New York, New York, USA
    Ann N Y Acad Sci 1246:41-9. 2011
    b>Common variable immunodeficiency (CVID) is considered to be a collection of genetic immune defects with complex inheritance patterns...
  41. pmc Abnormal liver function in common variable immunodeficiency disorders due to nodular regenerative hyperplasia
    C Ward
    Department of Clinical Immunology, Nuffield Department of Medicine, University of Oxford, Oxford, UK
    Clin Exp Immunol 153:331-7. 2008
    Patients with common variable immunodeficiency disorders are monitored for liver function test abnormalities. A proportion of patients develop deranged liver function and some also develop hepatomegaly...
  42. ncbi The enteropathy associated with common variable immunodeficiency: the delineated frontiers with celiac disease
    Georgia Malamut
    Universite Paris Descartes, France
    Am J Gastroenterol 105:2262-75. 2010
    The enteropathy associated with common variable immunodeficiency (CVID) is poorly characterized, and its possible relationships with well-defined causes of enteropathy, such as celiac sprue (CS), remain debated...
  43. ncbi Increased activation of protein kinase A type I contributes to the T cell deficiency in common variable immunodeficiency
    P Aukrust
    Research Institute for Internal Medicine, Medical Department A, Rikshospitalet, Oslo, Norway
    J Immunol 162:1178-85. 1999
    The molecular mechanisms underlying the T cell dysfunction often present in common variable immunodeficiency (CVI) are not established...
  44. ncbi B-cell replication history and somatic hypermutation status identify distinct pathophysiologic backgrounds in common variable immunodeficiency
    Gertjan J Driessen
    Department of Pediatric Infectious Disease and Immunology, Erasmus Medical Center, University Medical Center Rotterdam, The Netherlands
    Blood 118:6814-23. 2011
    b>Common variable immunodeficiency disorder (CVID) is the most prevalent form of primary idiopathic hypogammaglobulinemia. Identification of genetic defects in CVID is hampered by clinical and immunologic heterogeneity...
  45. pmc Assessment of thymic output in common variable immunodeficiency patients by evaluation of T cell receptor excision circles
    V Guazzi
    Division of Allergy and Clinical Immunology, University of Rome La Sapienza, Rome, Italy
    Clin Exp Immunol 129:346-53. 2002
    b>Common variable immunodeficiency (CVID) is a heterogeneous syndrome characterized by repeated infections and hypogammaglobulinaemia...
  46. ncbi Flowcytometric phenotyping of common variable immunodeficiency
    Klaus Warnatz
    Division of Rheumatology and Clinical Immunology, University Medical Center Freiburg, Freiburg, Germany
    Cytometry B Clin Cytom 74:261-71. 2008
    b>Common variable immunodeficiency (CVID) comprises heterogeneous antibody deficiency disorders. To classify this heterogeneous syndrome, clinical as well as immunologic parameters have been combined...
  47. ncbi Common variable immunodeficiency: 20-yr experience at a single centre
    M Pilar Llobet
    Paediatric Immunodeficiencies and Immunology Units, Vall d Hebron Hospital, Barcelona, Spain
    Pediatr Allergy Immunol 20:113-8. 2009
    b>Common variable immunodeficiency (CVID) is the most common symptomatic primary immunodeficiency...
  48. pmc TLR-mediated B cell defects and IFN-α in common variable immunodeficiency
    Joyce E Yu
    Department of Medicine, Mount Sinai Medical Center, New York, NY 10029, USA
    J Clin Immunol 32:50-60. 2012
    ..IFN-α also upregulated TLR7 and TLR9 mRNA expression comparable to normal levels in B cells of group 2 subjects, indicating that the loss of IFN-α could be a significant component of the B-cell defect for these subjects...
  49. ncbi T and B lymphocyte abnormalities in bone marrow biopsies of common variable immunodeficiency
    Manuella L Gomes Ochtrop
    Department of Rheumatology and Clinical Immunology, University Medical Centre, Freiburg, Germany
    Blood 118:309-18. 2011
    In common variable immunodeficiency (CVID) defects in early stages of B-cell development, bone marrow (BM) plasma cells and T lymphocytes have not been studied systematically...
  50. pmc Review of gastric cancer risk factors in patients with common variable immunodeficiency disorders, resulting in a proposal for a surveillance programme
    F Dhalla
    Core medical trainee, Imperial NHS Trust, London Deanery Immunoallergology Department, Santa Maria Hospital, Lisbon, Portugal
    Clin Exp Immunol 165:1-7. 2011
    b>Common variable immunodeficiency disorders (CVIDs) are the most frequent symptomatic primary immunodeficiencies in adults...
  51. ncbi [Autoimmune disease in primary antibody deficiencies]
    E Sarmiento
    Unidad de Inmunología Clínica, Servicio de Inmunologia, Hospital General Universitario Gregorio Maranon, Dr Esquerdo 46, 28007 Madrid, Spain
    Allergol Immunopathol (Madr) 33:69-73. 2005
    ..Many immune deficiency syndromes, mainly humoral defects, are associated with autoimmune disorders. In the present study, we describe the frequency of autoimmune disorders in these patients...
  52. ncbi Defective Vav expression and impaired F-actin reorganization in a subset of patients with common variable immunodeficiency characterized by T-cell defects
    Silvia Rossi Paccani
    Department of Evolutionary Biology, University of Siena, Via Aldo Moro 2, 53100 Siena, Italy
    Blood 106:626-34. 2005
    b>Common variable immunodeficiency (CVID) is a primary immune disorder characterized by impaired antibody production, which is in many instances secondary to defective T-cell function (T-CVID)...
  53. ncbi TNF and lymphotoxin-alpha polymorphisms associated with common variable immunodeficiency: role in the pathogenesis of granulomatous disease
    C G Mullighan
    Nuffield Department of Surgery, Churchill Hospital, Headington, Oxford, United Kingdom
    J Immunol 159:6236-41. 1997
    A subgroup of common variable immunodeficiency (CVID) patients have distinct clinical features, particularly granulomata splenomegaly, characteristic blood lymphocyte phenotype, and elevated circulating TNF levels...
  54. pmc Do ribosomopathies explain some cases of common variable immunodeficiency?
    S Khan
    Department of Immunology, Frimley Park Hospital NHS Foundation Trust, Portsmouth Road, Frimley, Camberley, Surrey, UK
    Clin Exp Immunol 163:96-103. 2011
    The considerable clinical heterogeneity of patients with common variable immunodeficiency disorders (CVID) shares some similarity with bone-marrow failure disorders such as Diamond-Blackfan anaemia (DBA) and Shwachman-Diamond syndrome (..
  55. ncbi Common variable immunodeficiency at the end of a prospering decade: towards novel gene defects and beyond
    Hermann Eibel
    Centre of Chronic Immunodeficiency, University Medical Centre Freiburg, Freiburg, Germany
    Curr Opin Allergy Clin Immunol 10:526-33. 2010
    Patients with a primary antibody deficiency of unknown cause are usually allotted the diagnosis of common variable immunodeficiency (CVID), thus creating a genetically, immunologically, and clinically highly heterogeneous study population,..
  56. ncbi Clinical and immunological overlap between autoimmune lymphoproliferative syndrome and common variable immunodeficiency
    A Rensing-Ehl
    Centre of Chronic Immunodeficiency, University Medical Centre, Freiburg, Germany
    Clin Immunol 137:357-65. 2010
    ..with low IgG and susceptibility to infection revealing a significant overlap between ALPS and common variable immunodeficiency (CVID)...
  57. ncbi Pediatric common variable immunodeficiency: immunologic and phenotypic associations with switched memory B cells
    Pierre L Yong
    Department of Medicine, Philadelphia Veterans Affairs Medical Center, Philadelphia, PA 19104, USA
    Pediatr Allergy Immunol 21:852-8. 2010
    Recent studies suggest that patients with common variable immunodeficiency (CVID) and low numbers of switched memory B cells have lower IgG levels and higher rates of autoimmune disease, splenomegaly, and granulomatous disease; however, ..
  58. ncbi Defective calcium signaling and disrupted CD20-B-cell receptor dissociation in patients with common variable immunodeficiency disorders
    Annick A J M van de Ven
    Department of Pediatric Immunology and Infectious Diseases, University Medical Center Utrecht and Wilhelmina Children s Hospital, Utrecht, The Netherlands
    J Allergy Clin Immunol 129:755-761.e7. 2012
    B cells of patients with common variable immunodeficiency (CVID) disorders display impairment in production of immunoglobulin class-switched antibodies, which is possibly contributed to by defects in early B-cell activation...
  59. pmc Role for Msh5 in the regulation of Ig class switch recombination
    Hideharu Sekine
    Medical University of South Carolina, Charleston, SC 29425, USA
    Proc Natl Acad Sci U S A 104:7193-8. 2007
    ....
  60. pmc Lymphoproliferative disease in antibody deficiency: a multi-centre study
    M M Gompels
    Immunology and Immunogenetics, Southmead Hospital, North Bristol NHS Trust, Bristol, UK
    Clin Exp Immunol 134:314-20. 2003
    ..Of these patients, 29 had common variable immunodeficiency, one X-linked agammaglobulinaemia, one X-linked immunoglobulin deficiency of uncertain cause and ..
  61. pmc Translational mini-review series on immunodeficiency: molecular defects in common variable immunodeficiency
    C Bacchelli
    Molecular Immunology Unit, Institute of Child Health, London, UK
    Clin Exp Immunol 149:401-9. 2007
    b>Common variable immunodeficiency (CVID) is a primary immunodeficiency that typically affects adults and is characterized by abnormalities of quantative and qualitative humoral function that are heterogeneous in their immunological profile ..
  62. ncbi Quality of life in common variable immunodeficiency requiring intravenous immunoglobulin therapy
    Haig Tcheurekdjian
    Department of Medicine, University Hospitals of Cleveland, Cleveland, Ohio, USA
    Ann Allergy Asthma Immunol 93:160-5. 2004
    ..There are no studies of patients with primary immunodeficiency states receiving intravenous immunoglobulin (IVIG) therapy that assess health-related quality of life (HRQOL) using a well-standardized and reproducible method...
  63. pmc Cancer risk among patients with IgA deficiency or common variable immunodeficiency and their relatives: a combined Danish and Swedish study
    L Mellemkjaer
    Institute of Cancer Epidemiology, Danish Cancer Society, Copenhagen, Denmark
    Clin Exp Immunol 130:495-500. 2002
    The extremely high risk reported for some types of cancer among patients with common variable immunodeficiency (CVID) is based on a limited number of investigations...
  64. ncbi Altered spectrum of somatic hypermutation in common variable immunodeficiency disease characteristic of defective repair of mutations
    Bhargavi Duvvuri
    The School of Kinesiology and Health Science, Faculty of Health, York University, Toronto, Ontario, Canada, M3J 1P3
    Immunogenetics 63:1-11. 2011
    Pathogenic common variable immunodeficiency diseases (CVID) are genetic, usually inherited diseases for which a limited number of genetic defects have been implicated...
  65. ncbi Common variable immune deficiency: respiratory manifestations, pulmonary function and high-resolution CT scan findings
    K M Thickett
    Adult Cystic Fibrosis Unit, Department of Respiratory Medicine, Birmingham Heartlands Hospital, Birmingham, UK
    QJM 95:655-62. 2002
    ..Aim: To investigate respiratory complications, lung function and high-resolution computerized tomography scan (HRCT) findings and mortality in 47 patients with CVID...
  66. pmc Measurement of peripheral B cell subpopulations in common variable immunodeficiency (CVID) using a whole blood method
    B L Ferry
    Department of Clinical Immunology, The Churchill Hospital, Oxford Radcliffe Hospital NHS Trust, Oxford, UK
    Clin Exp Immunol 140:532-9. 2005
    ..B cells and increased percentages of undifferentiated B cells in peripheral blood of patients with common variable immunodeficiency (CVID)...
  67. ncbi TACI mutation in common variable immunodeficiency and IgA deficiency
    Rima Rachid
    Division of Immunology, Children s Hospital, Boston, 300 Longwood Avenue, Boston, MA 02115, USA
    Curr Allergy Asthma Rep 6:357-62. 2006
    b>Common variable immunodeficiency (CVID) is a heterogeneous primary immunodeficiency disease...
  68. ncbi Lymphocyte characteristics in children with common variable immunodeficiency
    Annick A J M van de Ven
    Department of Pediatric Immunology and Infectious Diseases, Wilhelmina Children s Hospital, Utrecht, The Netherlands
    Clin Immunol 135:63-71. 2010
    The diagnosis of common variable immunodeficiency (CVID) is reserved for patients who suffer from undefined B cell dysfunction. Division of the CVID population into subgroups enables research for underlying disease causes...
  69. ncbi Gastrointestinal tract pathology in patients with common variable immunodeficiency (CVID): a clinicopathologic study and review
    Jason A Daniels
    Department of Pathology, Johns Hopkins Hospital, Baltimore, MD 21231, USA
    Am J Surg Pathol 31:1800-12. 2007
    b>Common variable immunodeficiency (CVID) is characterized by a host of gastrointestinal (GI) lesions that can mimic other conditions.
  70. pmc How I treat common variable immune deficiency
    Charlotte Cunningham-Rundles
    Department of Medicine, Mount Sinai School of Medicine, Mount Sinai Medical Center, 1425 Madison Ave, New York, NY 10029, USA
    Blood 116:7-15. 2010
    b>Common variable immunodeficiency is a rare immune deficiency, characterized by low levels of serum immunoglobulin G, A, and/or M with loss of antibody production...
  71. pmc Association between hepatitis C virus and very-low-density lipoprotein (VLDL)/LDL analyzed in iodixanol density gradients
    Søren U Nielsen
    Liver Research Group, School of Clinical Medical Sciences, University of Newcastle, England
    J Virol 80:2418-28. 2006
    ..Immunoprecipitation analysis showed that ApoB remained associated with HCV after treatment of serum with deoxycholic acid or NP-40, whereas ApoE was removed from HCV with these detergents...
  72. ncbi Granulomatous disease in common variable immunodeficiency
    Yoshikazu Morimoto
    Department of Medicine, National Jewish Medical and Research Center, 1400 Jackson Street, Denver, CO 80206, USA
    Curr Allergy Asthma Rep 5:370-5. 2005
    b>Common variable immunodeficiency (CVID) is a primary immunodeficiency of unknown etiology characterized by low levels of immunoglobulin (Ig)G, failure to make specific antibodies in response to infection or immunization, and variable T-..
  73. pmc Primary B-cell deficiencies reveal a link between human IL-17-producing CD4 T-cell homeostasis and B-cell differentiation
    Rita R Barbosa
    Instituto de Medicina Molecular, Faculdade de Medicina, Universidade de Lisboa, Lisboa, Portugal
    PLoS ONE 6:e22848. 2011
    ..b>Common Variable Immunodeficiency Disorders (CVID), defined by defects in B-cell differentiation into plasma and memory B cells, are ..
  74. ncbi Unravelling the complexity of T cell abnormalities in common variable immunodeficiency
    Antonello Giovannetti
    Department of Clinical Medicine, Division of Allergy and Clinical Immunology, La Sapienza University, Viale dell Universita 37, 00185 Rome, Italy
    J Immunol 178:3932-43. 2007
    ..several phenotypic and functional parameters of T cell-mediated immunity in a large series of common variable immunodeficiency (CVID) patients...
  75. pmc Autoimmune manifestations in common variable immunodeficiency
    C Cunningham-Rundles
    Mount Sinai School of Medicine, 1425 Madison Avenue, New York City 10029, USA
    J Clin Immunol 28:S42-5. 2008
    ..While the pathogenesis of autoreactivity is unknown for CVID subjects in general, and to a greater extent in those with autoimmunity, there is a loss of switched memory B cells...
  76. ncbi Common variable immune deficiency (CVID) presenting as an autoimmune disease: role of memory B cells
    Bret R Haymore
    Department of Allergy and Immunology, Walter Reed Army Medical Center, Washington, DC, USA
    Autoimmun Rev 7:309-12. 2008
    b>Common variable immunodeficiency (CVID) is a clinically heterogeneous disorder. Most often patients present with recurrent sinopulmonary infections, although it may present with autoimmune manifestations...
  77. ncbi Infections in 252 patients with common variable immunodeficiency
    Eric Oksenhendler
    Department of Clinical Immunology, Hopital Saint Louis, Paris, France
    Clin Infect Dis 46:1547-54. 2008
    b>Common variable immunodeficiency is characterized by recurrent infections and defective immunoglobulin production.
  78. ncbi Granulomatous-lymphocytic lung disease shortens survival in common variable immunodeficiency
    Christopher A Bates
    Department of Medicine, University of Colorado Health Sciences Center, Denver, CO 80206, USA
    J Allergy Clin Immunol 114:415-21. 2004
    b>Common variable immunodeficiency (CVID) is a primary immunodeficiency characterized by low levels of serum immunoglobulins and an inability to make specific antibodies.
  79. ncbi Memory switched B cell percentage and not serum immunoglobulin concentration is associated with clinical complications in children and adults with specific antibody deficiency and common variable immunodeficiency
    Hana Alachkar
    Department of Immunology, Hope Hospital, Manchester, UK
    Clin Immunol 120:310-8. 2006
    ..humoral immunodeficiencies are arbitrarily classified into specific antibody deficiency (SAD) or common variable immunodeficiency (CVID), this distinction does not accurately predict the risk of the bronchiectasis, one of the major ..
  80. ncbi Reduced frequency of CD4(+)CD25(HIGH)FOXP3(+) cells and diminished FOXP3 expression in patients with Common Variable Immunodeficiency: a link to autoimmunity?
    J Genre
    Department of Immunology, Biomedical Sciences Institute, University of Sao Paulo, Brazil
    Clin Immunol 132:215-21. 2009
    b>Common Variable Immunodeficiency (CVID) is a primary immunodeficiency disease characterized by defective immunoglobulin production and often associated with autoimmunity...
  81. ncbi Autoimmune thrombocytopenic purpura and common variable immunodeficiency: analysis of 21 cases and review of the literature
    Marc Michel
    Department of Internal Medicine, Hopital Henri Mondor, Assistance Publique Hopitaux de Paris, Creteil, France
    Medicine (Baltimore) 83:254-63. 2004
    ..the main characteristics and outcome of autoimmune thrombocytopenic purpura (AITP) in patients with common variable immunodeficiency (CVID), we analyzed data from 21 patients and reviewed additional cases from the literature...
  82. ncbi B-cell and T-cell phenotypes in CVID patients correlate with the clinical phenotype of the disease
    Gael Mouillot
    Immunology Laboratory, INSERM UMR S945, CIB Pitié Salpêtrière, Assistance Publique Hopitaux de Paris, Paris, France
    J Clin Immunol 30:746-55. 2010
    b>Common variable immunodeficiency (CVID) is a heterogeneous disorder characterized by recurrent infections and defective immunoglobulin production.
  83. ncbi Profiling of polychromatic flow cytometry data on B-cells reveals patients' clusters in common variable immunodeficiency
    Tomas Kalina
    Department of Pediatric Hematology and Oncology, 2nd Faculty of Medicine, Charles University Prague and University Hospital Motol, Prague, Czech Republic
    Cytometry A 75:902-9. 2009
    The aim of this study was to find an objective computational approach for phenotype analysis of common variable immunodeficiency (CVID) patients that describes all differences in the six-color space and to form groups of patients using ..
  84. ncbi Common variable immunodeficiency: association between memory B cells and lung diseases
    Drahomira Detkova
    Immunology Unit, University Hospital Vall d Hebron, Barcelona, Spain
    Chest 131:1883-9. 2007
    Malabsorption syndrome often develops in patients with common variable immunodeficiency (CVID). Why structural damages appear in some CVID patients and not in others is not fully understood...
  85. ncbi Autoimmunity in common variable immunodeficiency: correlation with lymphocyte phenotype in the French DEFI study
    Julien Boileau
    Department of Clinical Immunology and Internal Medicine, Hôpitaux Universitaires de Strasbourg et Université de Strasbourg, CNRS UPR9021, Strasbourg, France
    J Autoimmun 36:25-32. 2011
    b>Common variable immunodeficiency (CVID) is the most frequent clinically expressed primary immunodeficiency in adults and is characterized by primary defective immunoglobulin production...
  86. ncbi Frequency of Treg cells is reduced in CVID patients with autoimmunity and splenomegaly and is associated with expanded CD21lo B lymphocytes
    Gururaj Arumugakani
    Department of Clinical Immunology, Beckett Wing, St James s University Hospital, Leeds, UK
    J Clin Immunol 30:292-300. 2010
    b>Common variable immunodeficiency is a heterogeneous antibody deficiency syndrome with autoimmune and inflammatory complications in a significant proportion of patients...
  87. ncbi Immunoglobulin therapy to control lung damage in patients with common variable immunodeficiency
    Javier De Gracia
    Department of Pneumology, Hospital Universitari Vall d Hebron, Roger de Flor 235 bajos 2 a, 08025 Barcelona, Spain
    Int Immunopharmacol 4:745-53. 2004
    Lung damage progression is the most frequent condition in patients with common variable immunodeficiency (CVID). Appropriate immunoglobulin dose adjustments and follow-up guidelines to evaluate this have not been well established.
  88. pmc TACI mutations and disease susceptibility in patients with common variable immunodeficiency
    A E J Poodt
    Department of Pediatrics, Jeroen Bosch Hospital, s Hertogenbosch, The Netherlands
    Clin Exp Immunol 156:35-9. 2009
    The most prevalent primary immunodeficiency is common variable immunodeficiency (CVID). Mutations have been described in four genes, ICOS, CD19, BAFF-R and TNFRSF13B (encoding TACI), together associated with 10-15% of CVID cases...
  89. ncbi Novel mutations in a Japanese patient with CD19 deficiency
    H Kanegane
    Department of Pediatrics, Graduate School of Medicine, University of Toyama, Toyama, Japan
    Genes Immun 8:663-70. 2007
    b>Common variable immunodeficiency (CVID) is a heterogeneous disorder characterized by recurrent bacterial infections, hypogammaglobulinemia and low to normal numbers of circulating B cells...
  90. ncbi Common variable immunodeficiency: crossroads between infections, inflammation and autoimmunity
    Simone Baldovino
    Centro Universitario di Ricerche di Immunologia Clinica, Immunopatologia e Documentazione su Malattie Rare CMID, Universita di Torino, Ospedale G Bosco, Turin, Italy
    Autoimmun Rev 12:796-801. 2013
    b>Common variable immunodeficiency is a collection of diseases characterized by primary hypogammaglobulinemia. The causes of CVID are extremely heterogeneous and may affect virtually every pathway linked to B cell development and function...
  91. ncbi The C76R transmembrane activator and calcium modulator cyclophilin ligand interactor mutation disrupts antibody production and B-cell homeostasis in heterozygous and homozygous mice
    Chiara Bacchelli
    Centre for Immunodeficiency, Molecular Immunology Unit, UCL Institute of Child Health, London, United Kingdom
    J Allergy Clin Immunol 127:1253-9.e13. 2011
    ..and calcium modulator cyclophilin ligand interactor (TACI), are found in 10% of patients with common variable immunodeficiency. However, the most commonly detected mutation is the heterozygous change C104R, which is also found ..
  92. ncbi Preparations of intravenous immunoglobulins diminish the number and proinflammatory response of CD14+CD16++ monocytes in common variable immunodeficiency (CVID) patients
    Maciej Siedlar
    Department of Clinical Immunology, Polish American Institute of Pediatrics, Jagiellonian University Medical College, Krakow, Poland
    Clin Immunol 139:122-32. 2011
    ..Our data suggest that reduction in the number of CD14(+)CD16(++) monocytes and the blockade of their cytokine production via triggering CD32B can contribute to the anti-inflammatory action of IVIG...
  93. ncbi Telomere-dependent replicative senescence of B and T cells from patients with type 1a common variable immunodeficiency
    Marcella Visentini
    Department of Clinical Immunology, Sapienza University of Rome, Rome, Italy
    Eur J Immunol 41:854-62. 2011
    A subset of patients with common variable immunodeficiency (CVID), group 1a of the Freiburg classification, is characterized by increased B cells expressing low levels of CD21 (CD21(low) ), lymphoproliferation and autoimmunity...
  94. pmc Germline mutations in NFKB2 implicate the noncanonical NF-κB pathway in the pathogenesis of common variable immunodeficiency
    Karin Chen
    Department of Pediatrics, Division of Allergy, Immunology and Rheumatology, University of Utah, Salt Lake City, UT 84108, USA Electronic address
    Am J Hum Genet 93:812-24. 2013
    b>Common variable immunodeficiency (CVID) is a heterogeneous disorder characterized by antibody deficiency, poor humoral response to antigens, and recurrent infections...
  95. pmc Defective maturation of dendritic cells in common variable immunodeficiency
    T H Scott-Taylor
    Department of Immunology, Royal Free and University College Medical School, London, UK
    Clin Exp Immunol 145:420-7. 2006
    Monocyte-derived dendritic cells (MdDCs) from many patients with common variable immunodeficiency (CVID) have been shown recently to have reduced expression of surface molecules associated with maturity...
  96. pmc Response of refractory immune thrombocytopenic purpura in a patient with common variable immunodeficiency to treatment with rituximab
    T M El-Shanawany
    Department of Immunology, University Hospital of Wales, Cardiff, UK
    J Clin Pathol 60:715-6. 2007
  97. ncbi Infliximab treatment for severe granulomatous disease in common variable immunodeficiency: a case report and review of the literature
    Akaluck Thatayatikom
    Division of Immunology Rheumatology, Department of Pediatrics, St Louis Children s Hospital and Washington University School of Medicine, St Louis, Missouri 63110, USA
    Ann Allergy Asthma Immunol 95:293-300. 2005
    Granulomatous disease resembling sarcoidosis is a well-described condition associated with common variable immunodeficiency (CVID). Its treatment remains problematic, and new therapeutic options are needed.
  98. pmc Analysis of families with common variable immunodeficiency (CVID) and IgA deficiency suggests linkage of CVID to chromosome 16q
    Alejandro A Schäffer
    Department of Health and Human Services, National Center for Biotechnology Information, National Library of Medicine, National Institutes of Health, Bldg 38A Rm 6S608 8600 Rockville Pike, Bethesda, MD 20894, USA
    Hum Genet 118:725-9. 2006
    b>Common variable immunodeficiency (CVID) is an antibody deficiency syndrome that often co-occurs in families with selective IgA deficiency (IgAD). Vorechovský et al...
  99. pmc Characterization of immunologic defects in patients with common variable immunodeficiency (CVID) with intestinal disease
    Shradha Agarwal
    Division of Clinical Immunology, Mount Sinai School of Medicine, New York, New York 10029, USA
    Inflamm Bowel Dis 17:251-9. 2011
    b>Common variable immunodeficiency (CVID) is a heterogeneous disorder commonly presenting with recurrent sinopulmonary infections...
  100. ncbi Efficacy of intravenous immunoglobulin in the prevention of pneumonia in patients with common variable immunodeficiency
    Paula Jane Busse
    Division of Clinical Immunology, Department of Medicine, The Mount Sinai Medical Center, New York, NY, USA
    J Allergy Clin Immunol 109:1001-4. 2002
    b>Common variable immunodeficiency (CVID) is a primary immune disorder characterized by antibody deficiency and a decrease in serum IgG and IgA, IgM, or both levels at least 2 SDs below the mean for age and not attributed to other known ..
  101. ncbi Impaired nutritional status in common variable immunodeficiency patients correlates with reduced levels of serum IgA and of circulating CD4+ T lymphocytes
    M Muscaritoli
    Department of Clinical Medicine, University La Sapienza, Rome, Italy
    Eur J Clin Invest 31:544-9. 2001
    b>Common variable immunodeficiency (CVI) is a primary defect of the immune system. Infections, persistent diarrhoea and malabsorption may result in malnutrition, which may in turn contribute to increased morbidity...

Research Grants31

  1. APRIL-TACI: Role in mucosal IgA and human IgA deficiency
    Emanuela Castigli; Fiscal Year: 2006
    ..Identification of genes mutated in IgA deficient patients is critical for devising therapies that boost their IgA antibody response. ..
  2. Training program in hematologic and oncologic diseases
    Yi Zheng; Fiscal Year: 2012
    ..lymphoproliferative disorder, severe combined immunodeficiencies, Wiskott-Aldrich syndrome, and common variable immunodeficiency, molecular gene therapy strategies treating Fanconi anemia, storage diseases, and sickle cell anemia, ..
  3. Uncovering co-stimulatory T cell help defects in common variable immunodeficiency
    Elena E Perez; Fiscal Year: 2010
    b>Common variable immunodeficiency is a well described primary immunodeficiency characterized by low serum immunoglobulin concentrations, defective specific antibody production and increased susceptibility to bacterial infections...
  4. HLA*B Associated Genes and Memory B cells in patients with CVID
    Harry Schroeder; Fiscal Year: 2009
    b>Common variable immunodeficiency (CVID) is a clinical diagnosis given to patients who suffer with an unexplained deficiencies of serum immunoglobulins. The presenting complaint for most CVID patients is recurrent sinopulmonary infections...
  5. Characterization of Immune Deficiency in Congenital Bone Marrow Failure Syndromes
    Troy R Torgerson; Fiscal Year: 2012
    ..the following specific aims: 1) Determine the incidence of telomere shortening in patients with Common Variable Immunodeficiency (CVID) and Combined Immune Deficiency (CID), and 2) Evaluate B cell development and maturation in ..
  6. HLA Region and KIR Genomics in Common Variable Immune Deficiency
    HARRY WILLIAM SCHROEDER; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): Common variable immunodeficiency (CVID) is a clinical diagnosis given to patients who suffer with unexplained deficiencies of serum immunoglobulins...
  7. Elucidating the Genetic Basis of Common Variable Immune Deficiency
    Karl Voelkerding; Fiscal Year: 2012
    DESCRIPTION (provided by applicant): Common variable immunodeficiency (CVID), which is the most common primary immunodeficiency disease is a heterogeneous group of diseases characterized by defective immunoglobulin production that leads ..
  8. Molecular Genetics of HLA and Disease
    Stephen Hauser; Fiscal Year: 2009
    ..The focus is on Multiple Sclerosis, Rheumatoid Arthritis, IgA Deficiency, Common Variable Immunodeficiency, Myasthenia Gravis, Systemic Lupus Erythematosus and Ulcerative Colitis...
  9. Epigenetics: A Novel Approach in Primary Immunodeficiencies
    MARIA JULIA BEVILAQUA FELIPPE; Fiscal Year: 2010
    DESCRIPTION (Provided by the applicant) Abstract: Common variable immunodeficiency (CVID) is the most frequent clinically relevant primary immunodeficiency in humans...
  10. Anti-IL-12p40 Treatment of CVID Enteropathy: Gene Expression/Microbiota Analysis
    Warren Strober; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): Common variable immunodeficiency (CVID) is the most common symptomatic primary antibody deficient syndrome and is characterized by decreased levels of serum immunoglobulins and recurrent sinopulmonary ..
  11. Lymphoproliferative Disorders in Primary Immunodeficiencies
    JOHN MICHAEL ROUTES; Fiscal Year: 2012
    We demonstrated that patients with common variable immunodeficiency (CVID) and granulomatous and lymphocytic interstitial lung disease (GLILD) are at high risk for the development of B cell lymphomas and early mortality...
  12. Primary Immuno-Deficiencies Affecting Specific Stages of the Immune Response
    CORNELIS P TERHORST; Fiscal Year: 2013
    ..to study the role of single genes in the pathogenesis of X-linked Lymphoproliferative syndrome (XLP), Common variable immunodeficiency (CVID), Omenn syndrome and Severe Combined Immunodeficiencies (SCID) in an application, entitled: "..
  13. DNA Repair Defects in IgA Deficiency
    REUBEN HARRIS; Fiscal Year: 2009
    ..IgAD may develop over time into a more severe form of antibody deficiency, common variable immunodeficiency (CVID), and the two diseases can even co-exist in different members of the same family suggesting ..
  14. A novel model for the study of primary immunodeficiency: the equine CVID
    MARIA FLAMINIO; Fiscal Year: 2009
    DESCRIPTION (provided by applicant): Common variable immunodeficiency (CVID) in horses is a natural primary immunologic condition characterized by late-onset B cell lymphopenia or depletion, hypo- or agammaglobulinemia, and recurrent ..
  15. Immunodeficiencies and Autoimmune Diseases (a Scientific Colloquium)
    Noel Rose; Fiscal Year: 2009
    ..For example, approximately 20 % of patients with common variable immunodeficiency (CVID), a B cell defect, develop an autoimmune condition such as idiopathic thrombocytopenia (ITP), ..
  16. EFFECTS OF PEG-IL-2 IN PRIMARY IMMUNODEFICIENCY DISEASE
    Charlotte Cunningham Rundles; Fiscal Year: 1991
    ..Since many patients with common variable immunodeficiency have reduced IL-2 production and substantial T cell defects which correctable in vitro by the ..
  17. INTERDISCIPLINARY RESEARCH OF IMMUNOLOGIC DISEASES
    John Fahey; Fiscal Year: 1992
    ..Diseases included are: inflammatory bowel diseases (Role of in vivo primed CTL in immune disorders), common variable immunodeficiency and bone marrow transplantation (Retinoids/lymphokines enhance defective human B cells), AIDS (B cell ..
  18. Plasmacytoid dendritic cells, IFN-alpha, Th-1 response
    Frederick Siegal; Fiscal Year: 2004
    ..Through the use of multivariate statistical analysis, the influence of functional, IFN-producing pDC on Th-1 immune responses will be defined. ..
  19. STRUCTURE/FUNCTION ANALYSIS OF JAK3 IN HUMAN SCID
    JOSEPH ROBERTS; Fiscal Year: 2001
    ..mutations will result in attenuated immune deficits manifest as combined immunodeficiency (CID) or common variable immunodeficiency (CVID)...
  20. 2003 CIS Summer School
    Charlotte Cunningham Rundles; Fiscal Year: 2003
    ..The participants will be drawn from training programs, hospitals and institutions that are in North, Central or South America. Only fellows from the United States will receive funding made possible through the NIH grant. ..
  21. MECHANISMS OF IL-2 IMMUNE ENHANCEMENT IN HUMANS
    Charlotte Cunningham Rundles; Fiscal Year: 2001
    ..the mechanisms by which IL-2 enhances T cell immunity in a primary immunodeficiency disease, common variable immunodeficiency (CVI)...
  22. 2002 Summer School in Primary Immunodeficiency Disorders
    Charlotte Cunningham Rundles; Fiscal Year: 2002
    ..Fellows participating in the course will be reimbursed for their travel expenses. No registration fee will be charged for the fellows participating in the program. ..
  23. Primary Immune Deficiency Consortium Conference
    Charlotte Cunningham Rundles; Fiscal Year: 2008
    ..Principle topics will focus on: Diagnosis of Immunodeficiency; Genetics, Immune Reconstruction, and the Use of Standard and Novel Therapies. ..
  24. Primary Immunne Deficiency Consortium Conference
    Charlotte Cunningham Rundles; Fiscal Year: 2005
    ..Principle topics will focus on: Antibody Deficiency; Combined Immune Defects; Neutrophil Disorders; Laboratory Evaluation of Primary Immunodeficiency; Immune Reconstiution, and the Use of Standard and Novel Therapies. ..
  25. LYMPHOKINE REGULATION OF HUMAN B CELL MATURATION
    Lloyd Mayer; Fiscal Year: 1991
    ..distinct stages of B cell maturation (with their associated changes) and aid in the understanding of disorders of B cell maturation such as common variable immunodeficiency (CVI), chronic lymphocytic leukemia (CLL) and autoimmunity.
  26. TNF-Induced Apoptosis of Lymphocytes in Aged Humans
    Sudhir Gupta; Fiscal Year: 2004
    ..These studied should mechanism (s) for increased TNF-a-induced apoptosis during aging. ..
  27. THE ROLE OF DENDRITIC CELLS IN IMMUNITY TO FIV INFECTION
    WENDY SPRAGUE; Fiscal Year: 2005
    ..Results of these DC-based vaccine studies will provide useful proof-of-principle information regarding the induction of protective immunity immunodeficiency virus infections. ..
  28. IMPACT OF HIV-1 GENOTYPE ON THERAPY RESPONSE IN CHILDREN
    John W Sleasman; Fiscal Year: 2010
    ..Specific studies will evaluate: [A.] thymic output, [B.] post thymic T cell activation and differentiation, and [C.] functional immune response to neoantigen between VS/IS and VF/IS outcome groups. ..
  29. 12th International Workshop on Ataxia-Telangiectasia and ATM
    Richard Gatti; Fiscal Year: 2006
    ..unreadable] [unreadable] [unreadable]..
  30. 13th International Workshop on Ataxia-Telangiectasia and ATM
    Richard Gatti; Fiscal Year: 2008
    ..We also hope to encourage new young investigators to both clinical and basic A-T/ATM research. [unreadable] [unreadable] [unreadable]..