Clinical and immunological characteristics of children with primary immunodeficiencies
DOI:
https://doi.org/10.15574/PP.2024.98.70Keywords:
primary immunodeficiency, replacement therapy, children, serum immunoglobulins, lymphocyte subpopulations, bacterial infections, bronchiectasisAbstract
Aim - to study of clinical and immunological indicators in children with primary immunodeficiencies to determine vaccination tactics.
Materials and methods. 51 children with primary immunodeficiency diseases (PID), who made up the main group of the study, were examined. Of the 51 children with PID, 22 children had preserved antibody-forming function, and 29 children had a deficiency of antibody formation, and they received replacement immunoglobulin therapy. Immunological laboratory data (subpopulations of lymphocytes, levels of IgA, IgM and IgG in blood serum) were compared with healthy children in the control group.
Results. Recurrent bacterial infections were most common in children with primary immunodeficiency: otitis media in 34/51 (66.7%), pneumonia in 33/51 (64.7%), bronchitis in 32/51 (62.7%), and sinusitis in 19/51 (37.3%). Bronchiectatic disease occurred in 5 (9.8%) children. Severe invasive infections such as sepsis occurred in 4 (7.8%) children and osteomyelitis in 4 (7.8%) children. In the group of children with PID on immunoglobulin replacement therapy, the levels of serum immunoglobulins IgA (0.798±1.341 g/l), IgM (0.98±1.44 g/l) and IgG (0.99±1.44 g/l) and B-lymphocytes (8.7±7.5%, 302.8±291.1×109/l) were the lowest in comparison with the group of children with PID with preserved or partially preserved function of antibody formation and the control group.
Conclusions. Recurrent bacterial infections are the most common clinical manifestation in children with primary immunodeficiency. Some patients developed bronchiectasis as a result of frequent bacterial infections of the bronchopulmonary system and an untimely diagnosis of antibody deficiency. Timely and appropriate replacement therapy and early correction of antibody deficiency can minimize the progression of many infections and complications in such patients, including vaccine-directed infections. Children with PID with preserved or partially preserved antibody production are expected to have a suboptimal response to primary immunization and require additional doses of vaccine to achieve a long-lasting response to vaccination.
The research was carried out in accordance with the principles of the Declaration of Helsinki. The research protocol was approved by the Institution's Local Ethics Committee. Informed consent of parents and children was obtained for conducting research.
The author declares no conflict of interest.
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