Bronchiectasis due to Cystic Fibrosis

29 year old male with a history of Cystic Fibrosis presented with fever, chills, productive cough and hemoptysis. His chest XR revealed bilateral pulmonary consolidations. The patient was started on IV antibiotics including Meropenem and Tobramycin.

Cystic Fibrosis is an autosomal recessive disease secondary to a defect in a cystic fibrosis transmembrane conductance regulator protein (CFTR). The clinical manifestations are primarily due to the production of thick, viscous secretions in the lungs, pancreas, liver, intestines and reproductive tract.

Pulmonary manifestations often include obstructive lung disease with chronic bronchitis and bronchiectasis (see image above with arrows pointing to the bronchiectasis). The colonization with Pseudomonas Aeruginosa is very common in these patients.

Other manifestations of Cystic Fibrosis may include:
Exocrine pancreas insufficiency leading to malabsorption
Focal biliary cirrhosis
Infertility