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

[ Vol. 18 , Issue. 2 ]

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*   Pages 175 - 183 ( 9 )

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.

Affiliation:

Research Center for Immunodeficiencies, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Research Center for Immunodeficiencies, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Department of Microbiology and Immunology, School of Medicine, Alborz University of Medical Sciences, Karaj, Students' Research Committee, School of Public Health, Shahid Beheshti University of Medical Sciences, Tehran, Research Center for Immunodeficiencies, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Department of Immunology, School of Medicine, Tabriz University of Medical Sciences, Tabriz, Research Center for Immunodeficiencies, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Research Center for Immunodeficiencies, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Research Center for Immunodeficiencies, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Research Center for Immunodeficiencies, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Research Center for Immunodeficiencies, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Research Center for Immunodeficiencies, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Research Center for Immunodeficiencies, Children's Medical Center, Tehran University of Medical Sciences, Tehran

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