Pediatria pre prax 5/2010
Primary immunodeficiencies with predominantly antibody disorders
Recurrent infections of different nature are very common in pediatric practice. In most cases there will be found no immune disorders, but the risk of late diagnosis of primary immunodeficiency and development of permanent organ damage allow to think about this possibility in early diagnostic steps. The paper discusses pathogenesis, clinical presentation, diagnosis and therapy of most common primary antibody production defects. Presented are clinical warning signs, age-specific reference values for total IgG, IgA and IgM, IgG subclass levels and protective antibody levels against most relevant vaccines.
Keywords: primary immunodeficiency, hypogammaglobulinemia, X linked agammaglobulinemia, hyper IgM syndrome, selective IgA deficiency, common variable immunodeficiency.
