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  Authority of Polish Society of Allergology

vol 19. no 4. December 2014  

 Review articles
Primary antibody deficiencies in children
Aleksandra Szczawińska-Popłonyk

Antibody biosynthesis defects are the most prevalent category of primary
immune deficiencies of diverse immunopathology, clinical manifestation
and genetic background. Hence, hypogammaglobulinemia may be a
hallmark of mild or even asymptomatic IgA or IgG subclass deficiencies,
as well as a sign of a severe impairment of humoral immunity occurring
in agammaglobulinemia or in common variable immune deficiency. The
defective synthesis of antigen-specific antibodies, which are indispensable
for an effective adaptive immune response to infections, is a fundamental
pathomechanism which leads to clinical manifestations. The age
of the patient, modifying genetic factors as well as environmental conditions
may constitute further determinants of the clinical course of the
disease and pose the risk of complications such as allergy, autoimmunity
and lymphoproliferation in children affected with antibody production
defects. The supplementation treatment with intravenous or subcutaneous
polyvalent immunoglobulins provides antigen-specific antibodies
and is the basic therapeutic option, effective in prevention, reduction of
a number and alleviation of clinical course of infections.

keywords: immunoglobuliny, dzieci, agammaglobulinemia, pospolity,zmienny niedobór odporności, immunoglobulins, children, agammaglobulinemia, common variable immunodeficiency

pages: from 195 to 201

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