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Endocrine, Metabolic & Immune Disorders - Drug Targets

Editor-in-Chief

ISSN (Print): 1871-5303
ISSN (Online): 2212-3873

Research Article

Mannose-Binding Lectin Protein Deficiency Among Patients with Primary Immunodeficiency Disease Receiving IVIG Therapy

Author(s): Gholamreza Azizi, Fatemeh Kiaee, Somaye Yaslianifard, Hosein Rafiemanesh, Sara Mohammadikhajehdehi, Hamed Mohammadi, Seyed Sakineh Miresmaeeli, Leila H. Pour, Sina Abdolrahim Poor Heravi, Laleh Sharifi, Reza Yazdani, Hassan Abolhassani and Asghar Aghamohammadi*

Volume 18 , Issue 2 , 2018

Page: [175 - 183] Pages: 9

DOI: 10.2174/1871530317666171108111749

Price: $65

Abstract

Background: Primary immunodeficiencies (PIDs) are inherited disorders in which one or several components of the immune system are defective. Immunoglobulin replacement therapy is the mainstay of treatment for patients with impaired antibody production. However, recurrent infections would continue to occur in some patients due to the other high frequent concomitant defects, such as mannose-binding lectin (MBL) deficiency.

Methods: A total of 51 PID patients participated in this cross-sectional study. A detailed questionnaire was completed by interviewing patients in order to record demographic, clinical and laboratory data. The levels of MBL were determined in the serums of patients by a sandwich enzyme-linked immunosorbent assay (ELISA) technique.

Results: MBL deficiency was found in 29.4% of cases; 11.8% patients had mild, 3.9% patients had moderate and 13.7% patients had severe MBL deficiency. In patients with MBL deficiency, the rate of meningitis, sepsis, pneumonia, and otitis media was higher than patients with normal MBL levels. Immunoglobulin replacement therapy reduced the rate of infectious complications in PID patients; however, these reductions were more apparent in patients with normal MBL levels than patients with MBL deficiency.

Conclusion: Antibody deficient patients with a concomitant immune defect in MBL production have higher rates of recurrent infections despite receiving Immunoglobulin replacement therapy.

Keywords: Primary immunodeficiency, mannose-binding lectin, infection, IVIG therapy, polygenic disorder, primary antibody deficiency.

Graphical Abstract

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