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Aspergillus osteomyelitis in a child who has p67-phox-deficient chronic granulomatous disease
1Department of Pediatrics, Kurume University School of Medicine, Japan.
Abstract:
Here we describe Aspergillus osteomyelitis of the tibia in a 9-year-old boy who has an autosomal recessive form of chronic granulomatous disease (CGD). The patient showed a p67-phagocyte oxidase (phox) deficiency, which is rare type of CGD in Japan. The initial treatment which consisted of surgical debridement and antibiotic therapy with amphotericin B (AMPH), did not control the infection. Aspergillus fumigatus (A. fumigatus) pure isolated from drainage fluid and necrotic bone tissue demonstrated less susceptible to antifungal agents, including AMPH, fluconazole and flucytosine. Recombinant interferon gamma was then administrated, and it was effective in controlling the course of severe invasive aspergillosis. This report indicates the use of interferon gamma might be helpful in control for Aspergillus osteomyelitis of the tibia in a child with CGD demonstrated p67-phox deficiency refractory to conventional therapy with AMPH.
Insights
A rare form of chronic granulomatous disease (CGD) in a child led to Aspergillus osteomyelitis. Interferon gamma effectively treated the infection when conventional therapies failed.
Area of Science:
- Infectious Diseases
- Immunology
- Pediatrics
Background:
- Chronic Granulomatous Disease (CGD) is a primary immunodeficiency disorder characterized by impaired phagocyte function.
- Autosomal recessive forms of CGD, such as p67-phox deficiency, are rare and can lead to severe, invasive infections.
- Aspergillus osteomyelitis is a serious bone infection, particularly challenging in immunocompromised patients.
Observation:
- A 9-year-old boy with a rare p67-phox deficiency (a type of CGD) developed Aspergillus osteomyelitis of the tibia.
- Initial treatment with surgical debridement and amphotericin B was ineffective.
- The isolated Aspergillus fumigatus strain showed reduced susceptibility to conventional antifungal agents.
Findings:
- Recombinant interferon gamma administration was initiated for the severe invasive aspergillosis.
- Interferon gamma proved effective in controlling the Aspergillus osteomyelitis.
- This case highlights a rare p67-phox deficiency in a Japanese CGD patient.
Implications:
- Interferon gamma may be a valuable therapeutic option for Aspergillus osteomyelitis in CGD patients with p67-phox deficiency.
- Refractory invasive fungal infections in CGD warrant consideration of novel treatment strategies.
- Early diagnosis and tailored treatment are crucial for managing invasive aspergillosis in pediatric CGD.