Staphylokokkeninfektionen bei Patienten mit primären Immundefekten

Horst von Bernuth; Stephan Ehl

doi:10.1055/s-0038-1628954

Kinder- und Jugendmedizin, Inhaltsverzeichnis

Kinder- und Jugendmedizin 2008; 08(02): 93-100
DOI: 10.1055/s-0038-1628954

Infektiologie

Schattauer GmbH

Staphylokokkeninfektionen bei Patienten mit primären Immundefekten

Staphylococcal disease in children with primary immunodeficiencies

Autor*innen

Horst von Bernuth

¹Laboratoire de génétique humaine des maladies infectieuses, Université René Descartes – INSERM U550, Faculté de Médecine Necker, Paris (Direktor: Dr. Laurent Abel)
Stephan Ehl

³Zentrum für Kinder- und Jugendmedizin, Universtitätsklinikum Freiburg (Direktor: Prof. Dr. med. Andrea Superti-Furga)

Abstract

Zusammenfassung

Schwere, invasive und rezidivierende Staphylokokkeninfektionen können ein Hinweis für einen angeborenen Immundefekt sein. Insbesondere angeborene Störungen der Anzahl der neutrophilen Granulozyten, der Phagozytenaktivierung, der Leukozytenadhäsion, der Neutrophilen- Granula, des intrazellulären Abtötens phagozytierter Erreger und Hyper-IgE-Syndrome gehen mit einer erhöhten Anfälligkeit für Staphylokokkeninfektionen einher. Bei jeder schweren invasiven Staphylokokkeninfektion im Kindesund insbesondere im Säuglingsalter sollte daher auch an einen angeborenen Immundefekt gedacht werden. Ein rationales Vorgehen, das durch Anamnese, klinischen Befund und orientierende Labordiagnostik entscheiden hilft, ob ein Patient mit Staphylokokkeninfektion an einem auf Immundefekte spezialisierten Zentrum vorgestellt und betreut werden muss, wird vorgestellt.

Summary

Severe, invasive and recurrent staphylococcal disease can be a warning sign for inborn immunodeficiencies. In particular defects in neutrophil numbers, in the capacity of phagocytes to become activated, in leucocyte adhesion, in specific neutrophilic granules, in intracellular killing and Hyper-IgE-syndromes are accompanied with an increased susceptibility for staphylococcal disease. Severe invasive staphylococcal disease in children and in particular in newborns and infants should therefore raise the suspicion for an inborn immunodeficiency. A rationale, based on history, clinical status and basic laboratory tests, is presented in order to decide whether a patient with staphylococcal disease has to be referred to and taken care of by a specialised center for immunodeficiencies.

Schlüsselwörter

Staphylokokkeninfektionen - angeborene Immundefekte - Phagozyten - Granulozyten - angeborene Immunität

Keywords

Staphylococcal disease - primary immunodeficiencies - phagocytes - granulocytes - innate immunity

Volltext

Referenzen

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