Chronic granulomatous disease (CGD) and complete myeloperoxidase deficiency both yield strongly reduced dihydrorhodamine 123 test signals but can be easily discerned in routine testing for CGD.

@article{Mauch2007ChronicGD,
  title={Chronic granulomatous disease (CGD) and complete myeloperoxidase deficiency both yield strongly reduced dihydrorhodamine 123 test signals but can be easily discerned in routine testing for CGD.},
  author={Lysann Mauch and Andreas Lun and Maurice R G O'gorman and John S Harris and Ilka Schulze and Arturo Zychlinsky and Tobias A Fuchs and Uta Oelschlaegel and Sebastian Brenner and Dolphe Kutter and Angela Roesen-Wolff and Joachim Roesler},
  journal={Clinical chemistry},
  year={2007},
  volume={53 5},
  pages={890-6}
}
BACKGROUND The flow cytometric dihydrorhodamine 123 (DHR) assay is used as a screening test for chronic granulomatous disease (CGD), but complete myeloperoxidase (MPO) deficiency can also lead to a strongly decreased DHR signal. Our aim was to devise simple laboratory methods to differentiate MPO deficiency (false positive for CGD) and NADPH oxidase abnormalities (true CGD). METHODS We measured NADPH-oxidase and MPO activity in neutrophils from MPO-deficient patients, CGD patients, NADPH… CONTINUE READING

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Inborn metabolic errors of white blood cells: myeloperoxidase deficiency of neutrophils and monocytes physiopathological consequences

  • D. Kutter
  • Klinicka Biochemie Metabolismus
  • 2003
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