CFTR-related bronchiectasis is typically characterized by the early-age onset of symptoms, diffuse bronchiectasis with predominant upper lobe distribution on CT scan, and early presence of Staphylococcus aureus (S. aureus), Pseudomonas aeruginosa (P. aeruginosa) or Burkholdera cepacia (B. cepacia) in respiratory cultures. The gene discussed is CFTR; the disease is bronchiectasis.