Usefulness of bronchoalveolar lavage for the diagnosis and treatment of refractory pneumonia in a patient with Kostmann syndrome, a severe congenital neutropenia.

An 11‑year‑old girl with Kostmann syndrome developed refractory pneumonia. Culture of oral discharge throat‑swab specimens and blood could not identity the causative organism and systemic antimicrobial therapy failed to achieve improvement. We then performed diagnostic bronchoalveolar lavage（BAL）and culture of BAL fluid（BALF）yielded Pseudomonas aeruginosa. Therapeutic BAL using gentamicin produced a striking improvement of her pneumonia. Conclusion: In immunocompromised children with pneumonia BAL helps to identify the causative organism. If the patient is unresponsive to systemic antimicrobial therapy BAL using antimicrobial agents is also worth trying.（J Nippon Med Sch 2001; 68: 340―343）