title: [Primary antibody deficiency syndrome. Improved life expectancy and immunoglobulin G substitution].
abstract: Recurrent pyogenic infections are the leading clinical symptom in patients with antibody deficiency syndromes. In accordance with the WHO-classification at least nine different diseases are differentiated. In some cases diagnosis can be made simply by determining the immunoglobulin serum levels. Some diseases, such as Ig-heavy chain gene deletions, are clearly characterized by molecular biological methods, while other terms, such as common variable immunodeficiency, cover a collection of different diseases with changes predominantly of B-cell functions. The main features of the antibody deficiency syndromes are described. In most cases adequate therapy can be achieved by IgG replacement therapy.
authors.affiliation: Kinderklinik, Abteilung II, Universität Ulm.
authors.forename: P
authors.initials: P
authors.lastname: Bartmann
mesh_terms: D006801:Humans; D017098:IgA Deficiency; D007074:Immunoglobulin G / Q000627:therapeutic use; D007136:Immunoglobulins / Q000302:isolation & purification; D007153:Immunologic Deficiency Syndromes / Q000145:classification / Q000175:diagnosis* / Q000401:mortality; D011379:Prognosis
chemical_list: D007074:Immunoglobulin G; D007136:Immunoglobulins
vernacular_title: Primäre Antikörpermangelsyndrome. Verbesserte Lebenserwartung und Immunoglobulin-G-Substitution.