scholarly journals Major approaches in early diagnostics of common variable immunodeficiency in adults in Moscow

F1000Research ◽  
2012 ◽  
Vol 1 ◽  
pp. 46 ◽  
Author(s):  
Alexander V Karaulov ◽  
Irina V Sidorenko ◽  
Anna S Kapustina
Keyword(s):  
Common Variable Immunodeficiency ◽  
Structural Damage ◽  
Clinical Signs ◽  
Clinical Manifestations ◽  
Immunoglobulin Therapy ◽  
Early Diagnostics ◽  
Intravenous Immunoglobulin Therapy ◽  
Early Start ◽  
Treatment Concepts ◽  
Common Variable

Common variable immunodeficiency (CVID) is a primary immunological disease characterized predominantly by hypogammaglobulinemia. The main clinical manifestations are severe recurrent infections that often lead to structural damage of affected organs. The early start of adequate intravenous immunoglobulin therapy has significantly improved the prognosis of this serious disorder. Patients with CVID are also predisposed to autoimmune and lymphoproliferative complications. This article deals with the features of this primary immunodeficiency in adults. Clinical manifestations, immunological features and treatment concepts were gathered during 21 years of observation of such patients in Moscow. The authors suggest early predictive clinical signs of CVID in adults.

scholarly journals Proteomic Analysis of Sera from Common Variable Immunodeficiency Patients Undergoing Replacement Intravenous Immunoglobulin Therapy

2011 ◽  
Vol 2011 ◽  
pp. 1-10 ◽  
Author(s):  
Giuseppe Spadaro ◽  
Concetta D'Orio ◽  
Arturo Genovese ◽  
Antonella Galeotafiore ◽  
Chiara D'Ambrosio ◽  
...  
Keyword(s):  
Common Variable Immunodeficiency ◽  
Serum Proteins ◽  
Immunoglobulin Therapy ◽  
Normal Subjects ◽  
Serum Levels ◽  
Serum Samples ◽  
Intravenous Immunoglobulin Therapy ◽  
Proteomic Approach ◽  
Adults And Children ◽  
Common Variable

Common variable immunodeficiency is the most common form of symptomatic primary antibody failure in adults and children. Replacement immunoglobulin is the standard treatment of these patients. By using a differential proteomic approach based on 2D-DIGE, we examined serum samples from normal donors and from matched, naive, and immunoglobulin-treated patients. The results highlighted regulated expression of serum proteins in naive patients. Among the identified proteins, clusterin/ApoJ serum levels were lower in naive patients, compared to normal subjects. This finding was validated in a wider collection of samples from newly enrolled patients. The establishment of a cellular system, based on a human hepatocyte cell line HuH7, allowed to ascertain a potential role in the regulation ofCLUgene expression by immunoglobulins.

scholarly journals Common variable immunodeficiency – case report

2013 ◽  
Vol 3 (2) ◽  
pp. 170-172
Author(s):  
Emina Vukas ◽  
Aida Dizdarević ◽  
Senka Mesihović - Dinarević ◽  
Adisa Čengić
Keyword(s):  
T Cells ◽  
Dendritic Cells ◽  
Case Report ◽  
Immune System ◽  
Primary Immunodeficiency ◽  
Common Variable Immunodeficiency ◽  
Clinical Picture ◽  
Clinical Signs ◽  
Common Variable

Common variable immunodeficiency (CVID) or acquired hypogammaglobulinemia is the type of primary immunodeficiency. Deregulation of the immune system, leading to hypogammaglobulinemia, defective activation and proliferation of T cells and dendritic cells, and malfunction of the cytokines are observed in CVID. The clinical picture of CVID varies, any organ or system can be affected, therefore the diagnosis is often difficult and delayed and sometimes is not always possible. This article describes a twelve years old boy with all the clinical signs of immunodeficiency, as confi rmed by laboratory. The main treatment consists of life-long immunoglobulin substitution in intravenous or subcutaneous form.

scholarly journals The many faces of common variable immunodeficiency

Hematology ◽  
2012 ◽  
Vol 2012 (1) ◽  
pp. 301-305 ◽  
Author(s):  
Charlotte Cunningham-Rundles
Keyword(s):  
Common Variable Immunodeficiency ◽  
Clinical Manifestations ◽  
Chronic Infections ◽  
Patient Registries ◽  
Biologically Relevant ◽  
Inflammatory Conditions ◽  
Large Patient ◽  
Immune Defect ◽  
Autoimmune Cytopenias ◽  
Common Variable

Abstract Common variable immunodeficiency (CVID) is a rare immune deficiency characterized by low levels of serum IgG, IgA, and/or IgM, with a loss of Ab production. The diagnosis is most commonly made in adults between the ages of 20 and 40 years, but both children and much older adults can be found to have this immune defect. The range of clinical manifestations is broad, including acute and chronic infections, inflammatory and autoimmune diseases, and an increased incidence of cancer and lymphoma. For all of these reasons, the disease phenotype is both heterogeneous and complex. In the past few years, data from large patient registries have revealed that both selected laboratory markers and clinical phenotyping may aid in separating groups of subjects into biologically relevant categories. CVID consists of 2 phenotypes, 1 in which infections are the characteristic and another in which impressive inflammatory and/or hematologic complications also develop, including lymphadenopathy, splenomegaly, autoimmune cytopenias, enteropathy, and/or and granulomatous disease. These phenotypes appear to be stable, are related to immunologic and inflammatory markers, and are predictive of outcomes. This review outlines current understanding about this syndrome based on studies of large cohorts, highlighting the evaluation and treatment of complications and, in particular, the autoimmune and inflammatory conditions that affect these patients.

scholarly journals Association of clinical manifestations and B cell subsets with Freiburg and Paris classifications in patients with Common Variable Immunodeficiency (CVID).

2015 ◽  
Vol 6 ◽  
Author(s):  
Berrón-Ruiz Laura ◽  
Vargas-Hernández Alexander ◽  
Segura-Méndez Nora ◽  
López-Herrera Gabriela ◽  
Mogica-Martínez Dolores ◽  
...  
Keyword(s):  
B Cell ◽  
Common Variable Immunodeficiency ◽  
Clinical Manifestations ◽  
B Cell Subsets ◽  
Common Variable

scholarly journals Recurrent Campylobacter jejuni Infection in an Immunodeficient Patient Treated with Repeated Faecal Microbiota Transplant (FMT)—A Case Report

2022 ◽  
Vol 14 (1) ◽  
pp. 56-62
Author(s):  
Blair Merrick ◽  
Aravind Gokul Tamilarasan ◽  
Raphael Luber ◽  
Patrick F. K. Yong ◽  
Kuldeep Cheent ◽  
...  
Keyword(s):  
Case Report ◽  
Campylobacter Jejuni ◽  
Successful Treatment ◽  
Common Variable Immunodeficiency ◽  
Immunoglobulin Therapy ◽  
Faecal Microbiota ◽  
Limited Evidence ◽  
Campylobacter Infection ◽  
Common Variable

There is limited evidence to guide successful treatment of recurrent Campylobacter infection in patients with common variable immunodeficiency (CVID) already managed on regular immunoglobulin therapy. The role of faecal microbiota transplant (FMT) is uncertain. We report a case of recurrent Campylobacter jejuni infection in a patient with CVID treated with repeated FMT with 18 months of symptom resolution prior to relapse.

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