Služba za alergologijo, revmatologijo in klinično imunologijo, Pediatrična klinika, Univerzitetni klinični center Ljubljana, Ljubljana, Slovenija
Primary immunodeficiency is a consequence of incorrect functioning of any part of the immune system. Hypogammaglobulinaemia is a disease where a patient has a low level of immunoglobulins and antibodies in the blood. Immunoglobulins are proteins responsible for defence against infections. Patients are exposed to frequent, mostly severe respiratory and gastrointestinal infections. Permanent organ damage can result from frequent infections. To prevent such outcomes it is important that the disease is diagnosed and the infection treated with substitute immunoglobulin therapy as soon as possible. The aim of the treatment is avoidance of infections and the prevention of chronic organ damage.
Key words: Hypogammaglobulinemia, immunoglobulin, replacement therapy, intravenous immunoglobulins, subcutaneous immunoglobulins.