Chronic granulomatous disease presenting in childhood with Pseudomonas cepacia septicaemia.

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.