Research Day

Chronic refractory Candida parapsilosis infectiong the pneumonectomy stump in a patient with Cystic Fibrosis

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Background: Cystic Fibrosis (CF) is a multisystem disorder causing cycle of infection and inflammation, recurrent respiratory infection, progressive bronchiectasis leading to respiratory failure. These patients are often exposed to multiple courses of antibiotics and risk multi-drug resistant organisms. Complication like CF-related diabetes and steroids uses increased the risk for opportunistic infections. Candida species cause localized and invasive infection in immunodeficient patients or patient with vascular access. Lower airway infection can cause lung function decline by airway obstruction. We present the case of a patient with previous pneumonectomy and chronic infection due to Candida parapsillosis of the airway stump causing progressive obstructive lung disease. He responded to long term Caspofungin and Fluconazole. Case Report: A 12 year old male with Cystic fibrosis diagnosed due to failure-to-thrive, CF with pancreatic insufficiency elevated sweat chloride of 94 meq/L, abnormal chest x-ray, and fecal elastase below 200 ìg/g. By age 6 months, patient had bronchiectasis, Sequentially had RML and RLL lobectomies at age 2 and to right completion pneumonectomy at age age 4. Patient remained stable for two years followed by recurrent pulmonary exacerbations and declining lung function. His right pneumonectomy at an early age cause poor lung growth and significant chest wall deformity and scoliosis. Comorbid conditions include chronic sinusitis, CF-related diabetes, and CF-related liver disease. His Chest CT showed fibrosis, nodules and cystic lesions on the remaining left lung. FEV1 decline from a baseline of 39% to a nadir of 25 % predicted. A bronchoscopy shows a whitish cheesy biofilm extending from the pneumonectomy stump to the left airways. Culture grew Candida parapsillosis . Patient had previously failed oral fluconazole, responded to Caspofungin for 4 months He was sent for transplant evaluation. However, his lung function significantly improved by the time of his evaluation. He was then switched to long term IV fluconazole which was discontinued due to rash. His lung function was 41% before discontinuation. Otherwise both drugs were well tolerated. Discussion: This case raise awareness of the risk for airway obstruction from lowered lung candida infection in patient with CF and particularly in patient with foreign body like a staple used for pneumonectomy. Proper Identification of candida species is important for management. References: 1. Pappas PG, et al (2016). "Executive Summary: Clinical Practice Guideline for the Management of Candidiasis: 2016 Update by the Infectious Diseases Society of America". Clin. Infect. Dis. 62 (4): 409–417

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