Familial Case of Hereditary Agammaglobulinemia Bruton Type
https://doi.org/10.31550/1727-2378-2023-22-7-62-65
Abstract
Aim. To update the information about hereditary agammoglobulinemia, to describe a clinical case.
Key Points. The frequency of hereditary agammaglobulinemia varies from 1:100,000 to 1:200,000. The cause of this disease is a gene mutation on the long arm of the X chromosome that codes for B-cell tyrosine kinase. The disease is characterized by a sharp decrease in all classes of serum immunoglobulins and the number of circulating B - lymphocytes. Hereditary agammaglobulinemia manifested by repeated bacterial infections during the first two years of life. Patients require lifelong replacement therapy with intravenous immunoglobulins.
In this article we included 3 own clinical observations.
Conclusion. The quality of life and prognosis of the patients with Bruton's disease depends on the early detection of the disease and the quality of medical care provided
About the Authors
L. Yu. Popova
Orenburg State Medical University, Russian Ministry of Health
Russian Federation
Russian Federation
6 Sovetskaya St., Orenburg, 460000
G. D. Alemanova
Orenburg State Medical University, Russian Ministry of Health
Russian Federation
Russian Federation
6 Sovetskaya St., Orenburg, 460000
E. A. Zlodeeva
Orenburg State Medical University, Russian Ministry of Health
Russian Federation
Russian Federation
6 Sovetskaya St., Orenburg, 460000
O. V. Kirichenko
Orenburg State Medical University, Russian Ministry of Health
Russian Federation
Russian Federation
6 Sovetskaya St., Orenburg, 460000
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Review
For citations:
Popova L.Yu., Alemanova G.D., Zlodeeva E.A., Kirichenko O.V. Familial Case of Hereditary Agammaglobulinemia Bruton Type. Title. 2023;22(7):62-65. (In Russ.) https://doi.org/10.31550/1727-2378-2023-22-7-62-65
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