2 Cases of X-linked Chronic Granulomatous Disease Diagnosed by Flow Cytometry using 2'7'-dichIorofIuorescein diacetate.
- Author:
Jung Joo LEE
1
;
Jung Yeon SHIM
;
Soo Jong HONG
;
Chan Jeoung PARK
;
Hyun Sook CHI
Author Information
1. Department of Pediatrics, College of Medicine, University of Ulsan, Asan Medical Center, Seoul, Korea.
- Publication Type:Case Report
- Keywords:
Chronic granulomatous disease;
2'7'-dichlorofluorescein diacetate;
Flow cyto-metry;
Interferon-gamma
- MeSH:
Flow Cytometry*;
Follow-Up Studies;
Granulomatous Disease, Chronic*;
Humans;
Hydrogen Peroxide;
Interferon-gamma;
Interferons;
Mothers;
NADPH Oxidase;
Neutrophils;
Respiratory Burst;
Wills
- From:Pediatric Allergy and Respiratory Disease
1997;7(1):92-100
- CountryRepublic of Korea
- Language:Korean
-
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.
