A clinical case of autosomal recessive agammaglobulinemia with B-cell deficiency
https://doi.org/10.53529/2500-1175-2023-4-51-55
Abstract
Background. Primary agammaglobulinemia is the result of specific changes in B-cells that lead to low antibody production. A preliminary diagnosis is established if there is a history of frequent bacterial infections (otitis media, sinusitis, skin abscesses), including severe course, in some cases caused by opportunistic flora and atypical mycobacteria; low levels of immunoglobulins. The main symptoms of primary immunodeficiency in a child from this clinical example were frequent recidivating bronchial obstruction with the development of pneumonia.
Presentation of the clinical case.The publication presents a clinical case of autosomal recessive agammaglobulinemia with B-cell deficiency in a child of 2 years, 7 months. During the follow-up period from 4 months to 2 months, 7 months, the child had 3 episodes of pneumonia, 3 episodes of purulent otitis media. The child repeatedly underwent inpatient treatment, where he received broad-spectrum antibiotics as treatment. Based on the examination (IgA (0.02 g/l), IgG (0.3 g/l), IgM (0.07 g/l) and the absence of CD19+ cells), the diagnosis of “Primary immunodeficiency, agammaglobulinemia” was made, which was subsequently confirmed by the RDC of Moscow. From the moment of diagnosis, the child receives intravenous immunoglobulins at a dose of 7.5 g. and antibacterial therapy.
Conclusion. Early recognition and diagnosis of these conditions is crucial to improve outcomes and prevent complications.
Keywords
Supporting agencies: The authors express their gratitude to E.A. Filipicheva, an allergist and immunologist at GBUZ RM “CRCH”, for her help in collecting information during the preparation of the manuscript of the article.
About the Authors
E. V. Negodnova
N. P. Ogarev Mordovia State University
Mozambique
Mozambique
Elena Valerievna Negodnova - Senior lecturer of the Department of Immunology, Microbiology and Virology with a course in Сlinical immunology and Allergology, allergist-immunologist
68 Bolshevistskaya Str., Saransk, 430005
M. S. Iskandyarova
N. P. Ogarev Mordovia State University
Russian Federation
Russian Federation
Maria Sergeevna Iskandyarova - Senior lecturer of the Department of Immunology, Microbiology and Virology with a course in Сlinical immunology and Allergology, allergist-immunologist
68 Bolshevistskaya Str., Saransk, 430005
E. N. Tyagusheva
N. P. Ogarev Mordovia State University
Russian Federation
Russian Federation
Evgenia Nikolaevna Tyagusheva - 4th year student
68 Bolshevistskaya Str., Saransk, 430005
O. A. Radaeva
N. P. Ogarev Mordovia State University
Russian Federation
Russian Federation
Olga Aleksandrovna Radaeva - Doctor of Sci., head of the Department of Immunology, Microbiology and Virology with a course in clinical Immunology and Allergology, allergist-immunologist
68 Bolshevistskaya Str., Saransk, 430005
G. V. Fominova
N. P. Ogarev Mordovia State University
Russian Federation
Russian Federation
Galina Vladimirovna Fominova - Cand. of Sci., Associate professor of the Department of Obstetrics and Gynecology named after Professor A. P. Marusov
68 Bolshevistskaya Str., Saransk, 430005
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Review
For citations:
Negodnova E.V., Iskandyarova M.S., Tyagusheva E.N., Radaeva O.A., Fominova G.V. A clinical case of autosomal recessive agammaglobulinemia with B-cell deficiency. Allergology and Immunology in Paediatrics. 2023;(4):51-55. https://doi.org/10.53529/2500-1175-2023-4-51-55
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