European Journal of Pediatrics

, Volume 152, Issue 11, pp 896–899

The management of chronic granulomatous disease

  • A. Fischer
  • A. W. Segal
  • R. Seger
  • R. S. Weening
Immunology/Allergology

DOI: 10.1007/BF01957525

Cite this article as:
Fischer, A., Segal, A.W., Seger, R. et al. Eur J Pediatr (1993) 152: 896. doi:10.1007/BF01957525

Abstract

Chronic granulomatous disease (CGD) is a primary immunodeficiency disease which results from absence of the NADPH oxidase in the professional phagocytic cells [13] neutrophils, monocytes, macrophages and eosinophils. Deficiency of this oxidase renders the patient liable to infection by bacteria and fungi, and, as the name of the disease suggests, to chronic granulomatous inflammation. These patients present with a great variety of infections and other complications of their disease, which often tax the clinical and therapeutic skill of the doctors responsible for their care. Collectively we look after, or advise on the management of, over 100 of these subjects, and have developed experience in the diagnosis and management of the infections and other clinical problems they present. We thought that it might be timely to provide guidelines for their management based upon this experience. The numbers of patients are still relatively small, and the clinical presentations very varied, so it is immpossible to provide clear statistical proof of the veracity of this advice. It does, however, reflect the working practise of the physicians caring for many of these patients in Europe.

Key words

Neutrophil Inflammation Immunodeficiency Granuloma Chronic granulomatous disease Infection Antibiotics Interferon Therapy 

Abbreviations

CGD

chronic granulomatous disease

NADPH

nicotinamide adenine dinucleotide phosphate; reduced form

NBT

nitroblue tetrazolium

Copyright information

© Springer-Verlag 1993

Authors and Affiliations

  • A. Fischer
    • 1
  • A. W. Segal
    • 2
  • R. Seger
    • 3
  • R. S. Weening
    • 4
  1. 1.Department of ImmunologyNecker HospitalParisFrance
  2. 2.Department of MedicineUniversity College LondonLondonUK
  3. 3.Ast. Immunology/HaematologieUniversitäts-KinderklinikZurichSwitzerland
  4. 4.Department of Paediatrics, Academic Medical CentreUniversity of AmsterdamThe Netherlands