Clinical and immunological characteristics of children with primary hypogammaglobulinemiae
Keywords:
primary hypohammaglobulinemia, children, bacterial infections, chronic bronchitis, serum immunoglobulins, subpopulations of lymphocytes, bronchiectasisAbstract
Objective: study of clinical and immunological indicators in children with primary hypogammaglobulinemia in order to determine treatment tactics.
Material and methods. 53 children with primary hypogammaglobulinemiae were examined. Anamnesis and data of immunological examination (levels of serum immunoglobulins IgA, IgM, IgG, subpopulations of lymphocytes) were collected from each child at the time before the beginning of immunoglobulin replacement therapy.
Results. The most common features of primary hypogammaglobulinemia were recurrent bacterial infections, observed in 44 children out of 53 (83%). Autoimmune diseases and allergic diseases were foundwith the same frequency, in 8 children (15.1%). Bronchoectatic disease occurred in 5 children (9.4%). The levels of serum IgG (1.77±2.61 g/l) and IgM (0.062±0.14 g/l), and level of B lymphocytes (0.27±0.22%, 13.7±14.1×109) were the lowest in the group of children with X-linked hypogammaglobulinemia, compared to other groups of children with primary hypogammaglobulinemia.
Conclusions. Therefore, in our study it was found that the most common clinical manifestations in children with primary hypogammaglobulinemia were recurrent bacterial infections, autoimmune and allergic pathology was rare. The development of bronchiectatic disease is associated with the presence of chronic bronchitis and recurrent pneumonia, late diagnosis of antibody deficiency and late beginning of regular immunoglobulin replacement therapy. Early diagnosis and timely correction of antibody deficiency play a key role in the prevention of the development of of bacterial infections and their complications.
The research was carried out in accordance with the principles of the Helsinki Declaration. The study protocol was approved by the Local Ethics Committee of an participating institution. The informed consent of the patient was obtained for conducting the studies.
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