J Korean Pediatr Soc.  2000 Mar;43(3):444-449.

A Month-Old Infant with Chronic Granulomatous Disease Presented as Pulmonary Aspergillosis

Affiliations
  • 1Department of Pediatrics, College of Medicine, Ewha Womans University, Seoul, Korea.
  • 2Department of Pathology, College of Medicine, Ewha Womans University, Seoul, Korea.
  • 3Department of Microbiology, College of Medicine, Ewha Womans University, Seoul, Korea.
  • 4Department of Clinical Pathology, College of Medicine, Ewha Womans University, Seoul, Korea.
  • 5Department of Thoracic Surgery, College of Medicine, Ewha Womans University, Seoul, Korea.
  • 6Department of Radiology, College of Medicine, Ewha Womans University, Seoul, Korea.

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-gamma (INF-gamma) 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-gamma treatment.

Keyword

Chronic granulomatous disease; Pulmonary aspergillosis; INF-gamma; Activated NBT test

MeSH Terms

Abscess
Anemia
Anti-Bacterial Agents
Aspergillus
Aspergillus fumigatus
Drainage
Granuloma
Granulomatous Disease, Chronic*
Humans
Hypergammaglobulinemia
Infant*
Interferon-gamma
Leukocytosis
Lung
Myristic Acid
NADPH Oxidase
Nitroblue Tetrazolium
Phagocytes
Pulmonary Aspergillosis*
Superoxides
Suppuration
Thoracic Wall
Anti-Bacterial Agents
Interferon-gamma
Myristic Acid
NADPH Oxidase
Nitroblue Tetrazolium
Superoxides
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