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Chronic granulomatous disease presenting in childhood with Pseudomonas cepacia septicaemia
D E Lacy1, D A Spencer, A Goldstein
1Children's Hospital, Middleway, Birmingham, U.K.
The Journal of Infection
|November 1, 1993
Insights
Two children with severe infections died from Pseudomonas cepacia septicaemia. Doctors later diagnosed both with Chronic Granulomatous Disease (CGD), a condition that impairs the immune system.
Area of Science:
- Pediatrics
- Infectious Diseases
- Immunology
Background:
- Pseudomonas cepacia infections can be severe in children.
- Chronic Granulomatous Disease (CGD) is a primary immunodeficiency affecting phagocyte function.
Observation:
- Two pediatric patients presented with fever, hepatosplenomegaly, and ascites.
- Both children developed fatal Pseudomonas cepacia septicaemia despite aggressive medical management.
Findings:
- Subsequent diagnosis in both cases revealed Chronic Granulomatous Disease (CGD).
- This suggests a potential link between CGD and severe P. cepacia infections.
Implications:
- Clinicians should consider CGD in children with unexplained P. cepacia infections.
- Early diagnosis of CGD can guide management of recurrent or severe infections.
Abstract:
Two children who presented with fever, enlarged liver and spleen and ascites were found to have Pseudomonas cepacia septicaemia which proved fatal despite appropriate antibiotics and maximum supportive care. Chronic granulomatous disease of childhood (CGD) was subsequently diagnosed in both children. The possibility of CGD needs to be considered in any child with unexplained P. cepacia infection.