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 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.

Monogenic Primary Immunodeficiency Disorder Associated with Common Variable Immunodeficiency and Autoimmunity

2020 ◽  
Vol 181 (9) ◽  
pp. 706-714 ◽  
Author(s):  
Mohammad Hossein Asgardoon ◽  
Gholamreza Azizi ◽  
Reza Yazdani ◽  
Mahsa Sohani ◽  
Salar Pashangzadeh ◽  
...  
Keyword(s):  
Primary Immunodeficiency ◽  
Common Variable Immunodeficiency ◽  
Primary Immunodeficiency Disorder ◽  
Immunodeficiency Disorder ◽  
Common Variable

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 P081 The structure of the proteomic profile and gut microbiota in patients with primary immunodeficiency, common variable immunodeficiency and inflammatory bowel disease

2020 ◽  
Vol 14 (Supplement_1) ◽  
pp. S175-S176
Author(s):  
A A Iakovlev ◽  
A Volkov ◽  
O Bashtovaya ◽  
I Shcherbakova ◽  
T Ghazudin ◽  
...  
Keyword(s):  
Gut Microbiota ◽  
Primary Immunodeficiency ◽  
Common Variable Immunodeficiency ◽  
Fold Increase ◽  
Control Group ◽  
Microbiological Analysis ◽  
Colon Mucosa ◽  
Hydrogen Breath Test ◽  
E Coli ◽  
Common Variable

Abstract Background Infringement of intestinal microbiota in patients with primary immunodeficiency (PID), common variable immunodeficiency (CVID) and ulcerative colitis (UC) continues to be studied. The aim of the research is a comprehensive study of the structure of gut microbiota and mucosal protein profile (MPP) in patients with PID, CVID and UC in different phases of the disease course. Methods The research included 15 patients with PID, 22 CVID and 46 UC (28 with relapse and 18 with persistent remission of the disease), control group—20 healthy volunteers. Estimation of gut microbiota was performed by bacteriological seeding, hydrogen breath test (HBT) with lactulose. Content of short-chain fatty acids (SCFAs) and microbial lipid markers (MLM) in faeces and colon mucosa was determined by gas–liquid chromatographic and gas chromatography–mass spectrometry (GC–MS). MPP was based on isoelectric focusing techniques (SDS–PAGE, 2DGE). Mass spectrograms were obtained using MALDI-TOF-MS/MS (Bruker, USA). Results HBT showed a 4.5-, 8- and 11-fold increase in hydrogen production on a 150th-minute study. SCFA showed a 6-, 9- and 11-fold decrease in propionic and butyric acids, mainly in patients with PID and UC: 0.2 + 0.1, 0.14 + 0.03 and 0.04 + 0.02 mg/g, respectively. Microbiological analysis showed a decrease in titers of E. coli, bifido- and lactobacilli on average 4.6 + 0.8 Lg. The conditionally pathogenic microflora was represented by E. coli, lactose-negative strains (n = 51,105/g) and haemolytic strains (n = 34,104/g, Proteus spp. (n = 48,105/g), Staphylococcus spp. (n = 29,105–6/g), Candida (n = 51,104–6/g) and Clostridium spp. (n = 38,104/g) in stool culture. MLN GS-MS results showed a 5.5-, 3- and 6-fold increase in total bacterial load in patients with PID, CVID and UC, respectively, which was represented by resident anaerobic microflora: Streptococcus mutants, Clostridium difficile, Candida albicans. Results of MPP (detection rate of 75% or more) of the colon mucosa in patients with PID were detected: 1, 2, 4 okkludin, kininogen 1, interleukin-1B, interleukin 8, B2-glycoprotein, heat shock protein 27, in patients with CVID—translational elongation factor, apolipoprotein C-III. In patients with UC—NF-kB, alipoprotein C-III, TNF-α, interleukin-2 and 8 were presented. Conclusion In patients with PID, CVID and UC markers of colon excess growth and significant decrease in SCFA are recorded during the relapse period. MPP in patients with PID, CVID and UC was characterised by proteins characteristic of inflammation, apoptosis, proliferation and proteins reflecting the activity of the autoimmune process. Patients with UC in the remission have recovery trend of gut microbiota, production of SCFA, disappearance of specific components of altered MPP.

scholarly journals Common variable immunodeficiency and isosporiasis: first report case

2012 ◽  
Vol 45 (6) ◽  
pp. 768-769 ◽  
Author(s):  
Gisele Barbosa e Silva ◽  
Karla Pereira Fernandes ◽  
Gesmar Rodrigues Silva Segundo
Keyword(s):  
Primary Immunodeficiency ◽  
Common Variable Immunodeficiency ◽  
Giardia Lamblia ◽  
Opportunistic Infections ◽  
Severe Case ◽  
Negative Patient ◽  
Immunological Status ◽  
Laboratory Investigations ◽  
Common Variable ◽  
Hiv Negative

We report a severe case of diarrhea in a 62-year-old female HIV-negative patient from whom Giardia lamblia and Isospora belli were isolated. Because unusual and opportunistic infections should be considered as criteria for further analysis of immunological status, laboratory investigations led to a diagnosis of common variable immunodeficiency (CVID). This is the first reported case of isosporiasis in a patient with CVID and illustrates the importance of being aware of a possible link, particularly in relation to primary immunodeficiency.

scholarly journals A Young Girl with Repeated Episodes of Pneumonia and Intermittednt Diarrhoea - The Primary Immunodeficiency Syndrome

2013 ◽  
Vol 22 (1) ◽  
pp. 24-26
Author(s):  
Abdullah Al-Mamun ◽  
Samim Hasan ◽  
Syed Ahmed Abdullah ◽  
Ahmed Riyad Hossain ◽  
Safiul Alam ◽  
...  
Keyword(s):  
Replacement Therapy ◽  
Primary Immunodeficiency ◽  
Common Variable Immunodeficiency ◽  
Young Girl ◽  
Organ Specific ◽  
Relative Rarity ◽  
Immunoglobulin Replacement ◽  
Immunodeficiency Syndrome ◽  
Work Up ◽  
Common Variable

A description is given of a case of a young girl who presented with repeated episodes of pneumonia since childhood. In addition, she had episodes of intermittent diarrhoea. Her investigative work-up revealed a deficiency of serum immunoglobulins. The profile was suggestive of common variable immunodeficiency. Because of a relative rarity of this disease, it is often misdiagnosed earlier. Late diagnosis and delayed institution of immunoglobulin replacement therapy results in increased morbidity with a wide variety of organ-specific complications and increased mortality. DOI: http://dx.doi.org/10.3329/bjmed.v22i1.13597 Bangladesh J Medicine 2011; 22: 24-26

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