Journal of the Korean Pediatric Society 2000;43(3):444-449.
Published online March 15, 2000.
A Month-Old Infant with Chronic Granulomatous Disease Presented as Pulmonary Aspergillosis
SY Bae1, JH Jung1, KH Kim1, YM Hong1, HS Koo2, JY Seoh3, KS Hong4, ES Kang4, YS Won5, JH Yoo6
1Departments of Pediatrics, College of Medicine, Ewha Womans University, Seoul, Korea
2Departments of Pathology, College of Medicine, Ewha Womans University, Seoul, Korea
3Departments of Microbiology, College of Medicine, Ewha Womans University, Seoul, Korea
4Departments of Clinical Pathology, College of Medicine, Ewha Womans University, Seoul, Korea
5Departments of Thoracic Surgery, College of Medicine, Ewha Womans University, Seoul, Korea
6Departments of Radiology, College of Medicine, Ewha Womans University, Seoul, Korea
Aspergillus 폐렴으로 발현한 생후 1개월 남아의 만성 육아종 질환
배상영1, 정지혜1, 김경효1, 홍영미1, 구혜수2, 서주영3, 홍기숙4, 강은숙4, 원용순5, 유정현6
1이화여자대학교 의과대학 소아과학교실
2이화여자대학교 의과대학 해부병리학교실
3이화여자대학교 의과대학 미생물학교실
4이화여자대학교 의과대학 임상병리학과교실
5이화여자대학교 의과대학 흉부외과교실
6이화여자대학교 의과대학 방사선과학교실
Abstract
Chronic granulomatous disease(CGD) is an uncommon inherited disorder in which phagocytic cells fails to produce antimicrobial superoxide(O2-) due to NADPH oxidase deficiency. This disease can be easily screened by PMA(phobol myristate acetate)-activated nitroblue tetrazolium(NBT) test. Also the disease has features of chronic pyogenic infection, granuloma formation, anemia, hypergammaglobulinemia, and leukocytosis. Treatment uses antibiotics sensitive to specific organism, surgical drainage of abscess and antifungal drugs for fungal infection. Recently, interferon-γ(INF-γ) has been used as treatment and prevention. We experienced a case of CGD with pulmonary aspergillosis in a month-old infant. It was confirmed by activated NBT test. Aspergillus was observed in his lung tissue and Aspergillus fumigatus was cultured from pus of the chest wall. His symptoms improved after antifungal and INF-γ treatment.
Key Words: Chronic granulomatous disease, Pulmonary aspergillosis, INF-γ, Activated NBT test


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