Angeborene Antikörpermangelsyndrome („B-Zell-Defekte“)

Stephan Ehl; Bodo Grimbacher; Klaus Warnatz; Hermann M. Wolf

doi:10.1055/s-0038-1630891

Kinder- und Jugendmedizin, Inhaltsverzeichnis

Kinder- und Jugendmedizin 2008; 08(03): 151-158
DOI: 10.1055/s-0038-1630891

Immundefekte

Schattauer GmbH

Angeborene Antikörpermangelsyndrome („B-Zell-Defekte“)

Primary antibody deficiency syndromes

Stephan Ehl

¹Zentrum für Kinder- und Jugendmedizin, Universitätsklinik Freiburg (Direktor: Prof. Dr. med. Andrea Superti-Furga)

,

Bodo Grimbacher

²Abteilung Rheumatologie und Klinische Immunologie, Medizinische Universitätsklinik Freiburg (Direktor: Prof. Dr. med. Hans-Hartmut Peter)

,

Klaus Warnatz

²Abteilung Rheumatologie und Klinische Immunologie, Medizinische Universitätsklinik Freiburg (Direktor: Prof. Dr. med. Hans-Hartmut Peter)

,

Hermann M. Wolf

³Immunologische Tagesklinik und Biomedizinisches Institut Wien, Österreich (Leitung: Univ.-Prof. Dr. med. Martha Eibl)

› Institutsangaben

Abstract

Zusammenfassung

Patienten jedweden Alters sollten bei Vorliegen einer pathologischen Infektanfälligkeit (z. B. häufige bakterielle Infektionen des Respirationstraktes) auf das Vorliegen eines angeborenen Antikörpermangelsyndroms untersucht werden. Die frühe Diagnose einer primären Antikörperdefizienz ist entscheidend für die Prognose der betroffenen Patienten, da nur durch frühzeitigen Beginn einer adäquat dosierten Immunglobulin-Substitutionstherapie die Häufigkeit infektiöser Episoden reduziert und damit eine annähernd normale Lebensqualität ermöglicht wird. Durch frühe Diagnose und Therapie wird insbesondere die Entwicklung von Langzeitfolgen wie chronisch-obstruktiven Lungenerkrankungen verhindert.

Summary

Patients at any age with undue susceptibility to infections (e. g. recurrent bacterial infections of the upper and lower respiratory tract) should be investigated for possible primary antibody deficiency syndrome. Early diagnosis of inborn defects of antibody production is pivotal for the prognosis of the patients, as the frequency and severity of infectious episodes and their complications can only be reduced by early initiation and sufficient dosage of immunoglobulin substitution therapy. Therapy can be provided by intravenous infusions or subcutaneous home treatment. Late diagnosis and delayed treatment of the severe antibody deficiency syndromes such as CVID results in substantial morbidity and considerable mortality.

Schlüsselwörter

Antikörpermangelsyndrom - Antikörperdefizienz - Hypogammaglobulinämie

Keywords

Immunodeficiency - antibody deficiency - hypogammaglobulinemia

Volltext

Referenzen

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