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A5667 - Necrotizing Pneumonia in a Patient with Newly Diagnosed Chronic Granulomatous Disease
Author Block: D. Cerrone, R. M. Kato; Division of Pediatric Pulmonology and Sleep Medicine, Children's Hospital Los Angeles, Los Angeles, CA, United States.
A previously healthy 5 year old female presented to the emergency room in the setting of worsened cough, fatigue, and fever. She was hypoxemic with tachypnea and respiratory distress. She had recently been adopted from China and had evidence of poor weight gain since arrival but did not a have a history of pulmonary disease. A chest radiograph in the emergency department revealed complete opacification of the left hemithorax and serologic evaluation revealed leukocytosis with bandemia. A Chest CT was obtained which revealed necrotizing pneumonia involving the entirety of the left lung with scattered abscesses and pneumatoceles. She was treated with broad spectrum antibiotic coverage and her hypoxemia improved by her second day in the hospital. In the setting of failure to thrive and necrotizing pneumonia she underwent an immunodeficiency workup which revealed a NBT of 0% and abnormal DHR (dihydrorhodamine flow cytometry) thus consistent with the diagnosis of Chronic Granulomatous Disease (CGD). For further characterization a bronchoscopy and lung biopsy were obtained ten days into antibiotic treatment. The BAL culture returned positive for a filamentous fungus. During her lung biopsy the lung parenchyma was noted to be dense with concern for fibrosis throughout all lobes. The biopsy revealed fragments of organizing granulation tissue with fibrosis. Her post-op course was complicated by development of a left pleural effusion which resolved spontaneously. She required around the clock supplemental oxygen post-operatively. She was subsequently discharged with close pulmonology and immunology follow-up. In clinic supplemental oxygen was discontinued. She has since been able to play and keep up with her peers with little evidence of limitation. Repeat chest CTs have been obtained in the outpatient setting and have revealed significant interval improvement with stable pneumatoceles. The case highlights the relative plasticity of the lung and the surprisingly favorable prognosis shared among patients with severe cases of necrotizing pneumonia.