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Pseudomonas cepacia pneumonia in a child with chronic granulomatous disease and selective IgA deficiency
Abstract:
A 6 1/2 year-old boy with chronic granulomatous disease (CGD) and selective IgA deficiency developed a chronic progressive pneumonia which failed to respond to several conventional combinations of antimicrobial therapy. On lung biopsy, Pseudomonas cepacia was obtained in pure culture, sensitive to chloramphenicol, tetracycline, kanamycin and nalidixic acid. With specific therapy, he slowly recovered. P. cepacia has not been previously described as a cause of persistent pneumonia in immunodeficient children. The occurrence of CGD and selective IgA deficiency together is a very rare combination of immunodeficiencies.
Insights
A rare combination of immunodeficiencies, chronic granulomatous disease (CGD) and selective IgA deficiency, predisposed a child to persistent pneumonia caused by Pseudomonas cepacia, a previously undescribed pathogen in such cases.
Area of Science:
- Immunology
- Pediatric Infectious Diseases
- Microbiology
Background:
- Chronic granulomatous disease (CGD) and selective IgA deficiency are rare immunodeficiencies.
- Children with immunodeficiencies are susceptible to opportunistic infections.
Observation:
- A 6.5-year-old boy with CGD and selective IgA deficiency presented with chronic pneumonia.
- The pneumonia was refractory to multiple conventional antimicrobial therapies.
Findings:
- Lung biopsy identified Pseudomonas cepacia as the causative agent in pure culture.
- P. cepacia isolates were sensitive to chloramphenicol, tetracycline, kanamycin, and nalidixic acid.
- Targeted antimicrobial therapy led to the patient's slow recovery.
Implications:
- Pseudomonas cepacia is identified as a novel cause of persistent pneumonia in immunocompromised children.
- This case highlights the importance of considering unusual pathogens in refractory infections in patients with combined immunodeficiencies.
- The rare co-occurrence of CGD and selective IgA deficiency warrants further investigation regarding shared predispositions or interactions.