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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.

journal of nippon medical school

Usefulness of Bronchoalveolar Lavage for the Diagnosis and Treatment of Refractory Pneumonia in a Patient with Kostmann Syndrome, a Severe Congenital Neutropenia.