scholarly journals Differently expressed microRNA in response to the first Ig replacement therapy in common variable immunodeficiency patients

2020 ◽  
Vol 10 (1) ◽  
Author(s):  
Bruna De Felice ◽  
Ersilia Nigro ◽  
Rita Polito ◽  
Francesca Wanda Rossi ◽  
Antonio Pecoraro ◽  
...  
Keyword(s):  
Replacement Therapy ◽  
Common Variable Immunodeficiency ◽  
Antigen Processing ◽  
Serum Samples ◽  
Primary Immunodeficiency Disorder ◽  
Class I Mhc ◽  
Treatment Naïve ◽  
Delays In Diagnosis ◽  
First Time ◽  
Common Variable

AbstractCommon variable immunodeficiency (CVID) is a complex primary immunodeficiency disorder characterized by a high clinical and genetic heterogeneity. The molecular underlying causes of CVID are not still now clear and the delays in diagnosis and treatment worsen the prognosis of the patients. MicroRNAs are non-coding, endogenous small RNAs often deregulated in human diseases, such as autoimmune and other immune-based disorders. In the present study, we aimed to evaluate miRNAs associated with the CVID and, in particular, with the response to the first Ig replacement therapy. To this aim, we compared miRNA profile obtained by serum samples of treatment-naïve CVID patients before and 24 h after the first Ig replacement therapy. For the first time, using a microarray assay followed by an integrated bioinformatics/biostatistics analysis, we identified five microRNAs (hsa-miR-6742, hsa-miR-1825, hsa-miR-4769-3p, hsa-miR-1228-3p, hsa-miR-1972) differently modulated in CVID patients by Ig infusion. All of them were down-regulated, excepted miR-6742 which was up-regulated. The latter may be of particular interest, since its functions are related to pathways involving Class I MHC mediated antigen processing and adaptive as well as innate Immune System. In conclusion, this study shows for the first time the modulation of miRNAs involved in CVID patients after the first Ig replacement therapy. Further studies are needed to assess whether such miRNAs could represent novel potential biomarkers in management and therapy of CVID patients.

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

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.

Differences in Ig Replacement Therapy Dosing in Patients with Common Variable Immunodeficiency in Europe: Results from the ESID Database

2011 ◽  
Vol 127 (2) ◽  
pp. AB15-AB15 ◽  
Author(s):  
B. Gathmann ◽  
N. Mahlaoui ◽  
K. Warnatz ◽  
T.W. Kuijpers ◽  
S.S. Kilic ◽  
...  
Keyword(s):  
Replacement Therapy ◽  
Common Variable Immunodeficiency ◽  
Common Variable

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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