Pediatr Allergy Respir Dis.  1997 Mar;7(1):92-100.

2 Cases of X-linked Chronic Granulomatous Disease Diagnosed by Flow Cytometry using 2'7'-dichIorofIuorescein diacetate

• Lee JJ
• Shim JY
• Hong SJ
• Park CJ
• Chi HS

Abstract

Chronic granulomatous disease is a group of rare X-1inked or autosomal recessive genetic disorders due to the NADPH oxidase defect, which causes the defect in neutrophil respiratory burst function, the recurrent bacterial and fungal infection, and the granulomatous lesion. This disease can be diagnosed by measuring the decreased neutrophil respiratory burst activity in patient who shows X-linked recessive or autosomal recessive inheritance and the recurrent infection. To measure the neutrophil respiratory burst activity, NBT(nitroblue tetrazolium dye) test has been used. Recently, it has become possible to diagnose the chronic granulomatous disease patient as we1l as the carder simply and rapidly with the f1ow cytometry which measures hydrogen peroxide within the neutrophil, using 2' 7' -dichlorofluorescein diacetate(DCF-DA). We diagnosed two boys with history of recurrent infections and their carrier mothers as X-linked chronic granulomatous disease by f1ow cytometry using DCF-DA, and could follow up neutrophil respiratory burst activity after the administration of interferon gamma(INF-gamma) to these patients.

Keyword

Chronic granulomatous disease; 2'7'-dichlorofluorescein diacetate; Flow cyto-metry; Interferon-gamma

MeSH Terms

Flow Cytometry*
Follow-Up Studies
Granulomatous Disease, Chronic*
Humans
Hydrogen Peroxide
Interferon-gamma
Interferons
Mothers
NADPH Oxidase
Neutrophils
Respiratory Burst
Wills
Hydrogen Peroxide
Interferon-gamma
Interferons
NADPH Oxidase