Increased Serum Levels of Soluble CD30 in Patients with Common Variable Immunodeficiency and Its Clinical Implications

2007 ◽  
Vol 28 (1) ◽  
pp. 78-84 ◽  
Author(s):  
Nima Rezaei ◽  
Mostafa Haji-Molla-Hoseini ◽  
Asghar Aghamohammadi ◽  
Ali Akbar Pourfathollah ◽  
Mina Moghtadaie ◽  
...  
Keyword(s):  
Common Variable Immunodeficiency ◽  
Serum Levels ◽  
Clinical Implications ◽  
Soluble Cd30 ◽  
Common Variable

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 Serum B cell activating factor and interleukin 10 levels in common variable immunodeficiency: Relationship with clinical findings

2019 ◽  
Vol 76 (1) ◽  
pp. 42-49
Author(s):  
Radovan Mijanovic ◽  
Sladjana Andrejevic ◽  
Vladimir Jurisic ◽  
Branka Bonaci-Nikolic
Keyword(s):  
Autoimmune Diseases ◽  
B Cell ◽  
Common Variable Immunodeficiency ◽  
Healthy Subjects ◽  
Serum Levels ◽  
Pulmonary Diseases ◽  
Recurrent Infections ◽  
B Cell Activating Factor ◽  
Chronic Pulmonary Diseases ◽  
Common Variable

Background/Aim. Common variable immunodeficiency (CVID) is an immunologically and clinically heterogeneous disorder. Disturbed cytokine production is implicated in dysfunctional immune response. The aim of this study was to investigated B-cell activating factor (BAFF) and interleukin (IL)- 10 levels in CVID patients. Methods. The study included 28 CVID patients diagnosed and followed during a 20-year period (mean follow-up 14.5 years). Control groups consisted of 4 patients with X-linked agammaglobulinemia (XLA) and 21 healthy subjects. According to clinical characteristics, the CVID patients were divided into four groups which partly overlap: chronic pulmonary diseases (n = 21), splenomegaly (n = 13), autoimmune diseases (n = 9) and patients with recurrent infections despite regular intravenous immunoglobulin (IVIg) substitution (n = 4). The serum levels of BAFF and IL-10 were measured by commercial ELISA. Results. The BAFF levels were found to be higher in all CVID patients compared to the healthy controls (p < 0.01). The most significant differences were observed in the patients with pulmonary diseases and splenomegaly (p < 0.0001). Also, concentrations of IL-10 were higher in all CVID patients in comparison with the XLA patients (p < 0.05) and healthy subjects (p < 0.01). A statistically significant positive correlation (r = 0.86; p < 0.01) was found between the levels of BAFF and IL-10 in the CVID patients with autoimmune diseases. We demonstrated that the CVID patients with chronic pulmonary diseases had higher levels of IL-10, while the CVID patients with recurrent infections had higher BAFF concentrations in comparison to the patients without these features (p < 0.05). Conclusion. In spite of the limited number of patients, this is the first report from Serbia, examining the serum levels of BAFF and IL-10 in the CVID patients. Our study showed significantly increased concentrations of serum BAFF and IL-10 in the patients with CVID compared to the healthy subjects. Further studies are needed to confirm our findings that the BAFF levels are more pronounced in patients with recurrent infections while IL-10 levels are higher in patients with chronic pulmonary diseases.

scholarly journals Features of the cytokine profile in humoral primary immunodeficiency patients

2015 ◽  
Vol 12 (1) ◽  
pp. 34-37
Author(s):  
L P Sizyakina ◽  
I I Andreeva ◽  
E A Antonova
Keyword(s):  
Primary Immunodeficiency ◽  
Common Variable Immunodeficiency ◽  
Serum Levels ◽  
Cytokine Profile ◽  
Common Variable

The results of cytokines serum levels in patients with infectious and autoimmune phenotypes of X-linked agammaglobulinemia (X-AGG) and common variable immunodeficiency (CVID) are presented. In patients with varying nature of the primary agammaglobulinemia similar cytokine changes were estimated.

scholarly journals Gut Microbiome and Common Variable Immunodeficiency: Few Certainties and Many Outstanding Questions

2021 ◽  
Vol 12 ◽  
Author(s):  
Gilda Varricchi ◽  
Remo Poto ◽  
Gianluca Ianiro ◽  
Alessandra Punziano ◽  
Gianni Marone ◽  
...  
Keyword(s):  
Gut Microbiota ◽  
Common Variable Immunodeficiency ◽  
Cell Activation ◽  
Immune Cell ◽  
Serum Levels ◽  
Therapeutic Interventions ◽  
Polygenic Inheritance ◽  
Alpha And Beta Diversity ◽  
Inflammatory Conditions ◽  
Common Variable

Common variable immunodeficiency (CVID) is the most common symptomatic primary antibody immunodeficiency, characterized by reduced serum levels of IgG, IgA, and/or IgM. The vast majority of CVID patients have polygenic inheritance. Immune dysfunction in CVID can frequently involve the gastrointestinal tract and lung. Few studies have started to investigate the gut microbiota profile in CVID patients. Overall, the results suggest that in CVID patients there is a reduction of alpha and beta diversity compared to controls. In addition, these patients can exhibit increased plasma levels of lipopolysaccharide (LPS) and markers (sCD14 and sCD25) of systemic immune cell activation. CVID patients with enteropathy exhibit decreased IgA expression in duodenal tissue. Mouse models for CVID unsatisfactorily recapitulate the polygenic causes of human CVID. The molecular pathways by which gut microbiota contribute to systemic inflammation and possibly tumorigenesis in CVID patients remain poorly understood. Several fundamental questions concerning the relationships between gut microbiota and the development of chronic inflammatory conditions, autoimmune disorders or cancer in CVID patients remain unanswered. Moreover, it is unknown whether it is possible to modify the microbiome and the outcome of CVID patients through specific therapeutic interventions.

scholarly journals High serum levels of BAFF, APRIL, and TACI in common variable immunodeficiency

2007 ◽  
Vol 124 (2) ◽  
pp. 182-189 ◽  
Author(s):  
Adina K. Knight ◽  
Lin Radigan ◽  
Thomas Marron ◽  
Allison Langs ◽  
Li Zhang ◽  
...  
Keyword(s):  
Common Variable Immunodeficiency ◽  
Serum Levels ◽  
High Serum ◽  
Common Variable

scholarly journals NEUROENDOCRINE TUMOR IN A CHILD WITH COMMON VARIABLE IMMUNODEFICIENCY

2020 ◽  
Vol 38 ◽  
Author(s):  
Pedro de Souza Lucarelli Antunes ◽  
Heloísa Gabriel Tersariol ◽  
Mainã Marques Belém Veiga ◽  
Maria Conceição Santos de Menezes ◽  
Fabíola Del Carlo Bernardi ◽  
...  
Keyword(s):  
Neuroendocrine Tumor ◽  
Common Variable Immunodeficiency ◽  
Pediatric Patients ◽  
Medical Literature ◽  
Clinical Manifestations ◽  
Serum Levels ◽  
Immunological Investigation ◽  
One Year ◽  
To Receive ◽  
Common Variable

ABSTRACT Objective: To report a case of a child with primary immunodeficiency who at eight years developed digestive symptoms, culminating with the diagnosis of a neuroendocrine tumor at ten years of age. Case description: One-year-old boy began to present recurrent pneumonias in different pulmonary lobes. At four years of age, an immunological investigation showed a decrease in IgG and IgA serum levels. After the exclusion of other causes of hypogammaglobinemia, he was diagnosed with a Common Variable Immunodeficiency and started to receive monthly replacement of human immunoglobulin. The patient evolved well, but at 8 years of age began with epigastrium pain and, at 10 years, chronic persistent diarrhea and weight loss. After investigation, a neuroendocrine tumor was diagnosed, which had a rapid progressive evolution to death. Comments: Medical literature has highlighted the presence of gastric tumors in adults with Common Variable Immunodeficiency, emphasizing the importance of early diagnosis and the investigation of digestive neoplasms. Up to now there is no description of neuroendocrine tumor in pediatric patients with Common Variable Immunodeficiency. We believe that the hypothesis of digestive neoplasm is important in children with Common Variable Immunodeficiency and with clinical manifestations similar to the case described here in the attempt to improve the prognosis for pediatric patients.

Low Prevalence of Selective IgA Deficiency in Infected Children Born to HIV-Seropositive Mothers: An In Vivo Model for Speculation on Selective IgA Deficiency Pathogenesis

2008 ◽  
Vol 21 (4) ◽  
pp. 1035-1039
Author(s):  
E. Chiappini ◽  
L. Galli ◽  
P.A. Tovo ◽  
C. Gabiano ◽  
C. Lisi ◽  
...  
Keyword(s):  
Hiv Infection ◽  
Common Variable Immunodeficiency ◽  
Serum Levels ◽  
Iga Deficiency ◽  
In Vivo Model ◽  
Selective Iga Deficiency ◽  
Perinatal Hiv ◽  
Common Variable

Anecdotal reports of restored immunoglobulin production in individuals with common variable immunodeficiency after acquiring HIV infection suggest that perturbation of the immune system occurring during HIV infection may force some underlying functional defects. These findings raise intriguing questions about the pathogenesis of common variable immunodeficiency. No study has investigated the possible influence of HIV infection on the development of selective IgA deficiency, a primary immunologic defect genetically related to common variable immunodeficiency. IgA serum levels were evaluated in a large cohort of children born to HIV-infected mothers from 1985 to 2006. To avoid differences possibly due to different follow-up durations we considered only infected and non-infected children aged over 4 years at last-follow-up. The study included 1,157 non-infected children and 964 infected children, aged ≥ 4 years at last-follow-up and with availability of two or more serum IgA determinations over six months of age. No child displayed immunoglobulin values compatible with diagnosis of common variable immunodeficiency. However, 0/964 infected children vs. 5/1157 non-infected children had selective IgA deficiency (P=0.048). It may be speculated that several immunological alterations, occurring simultaneously in perinatal HIV infection, surpass the functional defect exhibited in some children with selective IgA deficiency. Our data may shed light on the pathogenesis of selective IgA deficiency.

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