Recurrent pneumonitis due to Pseudomonas cepacia. An unexpected phagocyte dysfunction.

A PATIENT with necrotizing pneumonitis due to Pseudomonas cepacia (EO-I) was described previously.' Two years later at age 21 years, this patient again had severe pneumonia, and Ps. cepacia was isolated from bronchial washings. The patient's phagocytes ingested but failed to kill Ps. cepacia and several other organisms. The phagocyte dysfunction was indistinguishable from that found in chronic granulomatous disease of childhood (CGD). Most patients with CGD have frequent infections, have multiple granulomatous abscesses beginning early in life, and have a short life span. In the present case, in sharp contrast to this pattern, the clinical course of the patient has been benign.

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