Granulocyte-macrophage colony stimulating factor does not improve neutrophil oxidative metabolism in a patient with variant X-linked chronic granulomatous disease

Eur J Pediatr. 1991 Jun;150(8):575-8. doi: 10.1007/BF02072210.

Abstract

Variant X-linked chronic granulomatous disease (CGD) is characterised by a decreased but still measurable respiratory burst and cytochrome b content of phagocytes resulting in a clinically milder form of the disease. We examined the in vivo effect of recombinant human granulocyte-macrophage colony stimulating factor (rh-GM-CSF) on the neutrophil functions of a patient treated for liver abscess. The number of white blood cells was markedly increased at the highest dose of GM-CSF injected (30 micrograms/kg per day). This was mainly due to a large increase in eosinophils and to a lesser extent in neutrophils. No change in the deficient neutrophil respiratory burst nitroblue tetrazolium (NBT)-reduction, superoxide (O2-)-production and cytochrome b content was observed during 6 weeks of therapy with increasing doses of GM-CSF. No significant clinical improvement of the liver abscess was observed during treatment with GM-CSF.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Child
  • Cytochrome b Group / analysis
  • Genetic Linkage
  • Granulocyte-Macrophage Colony-Stimulating Factor / pharmacology*
  • Granulocyte-Macrophage Colony-Stimulating Factor / therapeutic use
  • Granulomatous Disease, Chronic / complications
  • Granulomatous Disease, Chronic / genetics
  • Granulomatous Disease, Chronic / metabolism*
  • Humans
  • Leukocyte Count
  • Liver Abscess / drug therapy
  • Liver Abscess / etiology
  • Male
  • NADPH Oxidases*
  • Neutrophils / chemistry
  • Neutrophils / drug effects*
  • Neutrophils / metabolism*
  • Oxidation-Reduction
  • Superoxides / metabolism
  • X Chromosome

Substances

  • Cytochrome b Group
  • Superoxides
  • Granulocyte-Macrophage Colony-Stimulating Factor
  • cytochrome b558
  • NADPH Oxidases