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Cystic fibrosis with Burkholderia infection — SCE Respiratory MCQ

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HardRare/MiscellaneousCystic fibrosis with Burkholderia infectionSCE Respiratory

A 42-year-old woman with cystic fibrosis has increased sputum volume, weight loss and declining FEV1. Sputum cultures repeatedly grow Burkholderia cepacia complex. She is being assessed for lung transplantation. What is the most appropriate management implication?

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Correct answer: D — Discuss infection status with the transplant centre because it affects risk stratification

Burkholderia cepacia complex infection in cystic fibrosis has major prognostic and transplant-assessment implications, so the transplant team must be involved. It should not be ignored because transmissibility and post-transplant outcomes vary by genomovar and clinical course. Amoxicillin, enzyme changes and omalizumab do not address this infection risk.

Reference: CF Trust Standards of Care; ISHLT Lung Transplant guidance