Persistent pleuritic chest pain in a patient with cystic fibrosis

A 14-year-old girl with cystic fibrosis (CF), homozygous for the Phe508del mutation, was electively admitted for optimisation of lung disease. Her CF was complicated by bronchiectasis, gastrostomy feeding, liver disease, and diabetes. Sputum cultures had identified Haemophilus parainfluenzae and Candida albicans in the previous 12 months, but she had never cultured Pseudomonas aeruginosa. She also had comorbid osteogenesis imperfecta (OI), type 3 phenotype, which was complicated by multiple long bone fractures including four rib fractures at birth. Her baseline forced expiratory volume in 1 s (FEV1) was 44% (0.78 L), forced vital capacity (FVC) 54% (1.06 L), and FEV1/FVC ratio 81% predicted. The combination of CF and OI had resulted in severe restrictive lung disease with nocturnal hypoventilation managed with bilevel noninvasive ventilation. Pleural effusion is rare in cystic fibrosis. Infection leading to pleural effusion is likely to be polymicrobial, including contributory fungal infection; microbiology of pleural fluid is commonly discordant with sputum. https://bit.ly/3MJXrhk

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