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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
1   Zentrum für Kinder- und Jugendmedizin, Universitätsklinik Freiburg (Direktor: Prof. Dr. med. Andrea Superti-Furga)
,
Bodo Grimbacher
2   Abteilung Rheumatologie und Klinische Immunologie, Medizinische Universitätsklinik Freiburg (Direktor: Prof. Dr. med. Hans-Hartmut Peter)
,
Klaus Warnatz
2   Abteilung Rheumatologie und Klinische Immunologie, Medizinische Universitätsklinik Freiburg (Direktor: Prof. Dr. med. Hans-Hartmut Peter)
,
Hermann M. Wolf
3   Immunologische Tagesklinik und Biomedizinisches Institut Wien, Österreich (Leitung: Univ.-Prof. Dr. med. Martha Eibl)
› Author Affiliations
Further Information

Publication History

Eingegangen: 13 December 2007

angenommen: 03 January 2008

Publication Date:
27 January 2018 (online)

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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
 

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