Characterisation of Pseudomonas aeruginosa from lung explant of a patient with primary ciliary dyskinesia. PCD

Background/Aims Primary ciliary dyskinesia (PCD) is a rare inherited disease characterized by abnormal ciliary function, leading to compromised mucociliary clearance and persistent bacterial infection of the upper and lower airways. Consequently, patients develop chronic productive cough and bronchiectasis. Akin to cystic fibrosis, Pseudomonas aeruginosa is a common chronic bacterial infection associated which often persists despite aggressive antibiotic intervention. In severe cases, complete lung transplant is the only viable option. However, while the genotypic and phenotypic characteristics of P. aeruginosa is known to vary considerably and reflect pathophysiology, variation within an individual is poorly understood. Here we report a longitudinal, microbiological investigation of an individual with PCD, who eventually underwent a complete lung transplant.