Berita Kesehatan
Structural determinants of long term functional outcomes in young children with cystic fibrosis
Senin, 04 Jan 2021 15:09:40


Background Accelerated lung function decline in individuals with cystic fibrosis (CF) starts in adolescence with respiratory complications being the most common cause of death in later life. Factors contributing to lung function decline are not well understood, in particular its relationship with structural lung disease in early childhood. Detection and management of structural lung disease could be an important step in improving outcomes in CF patients.

Methods Annual chest computed tomography (CT) scans were available from 2005 to 2016 as a part of AREST CF cohort for children aged 3-months to 6-years. Annual spirometry measurements were available for 89.77% of the cohort (n=167 children at age 5–6 years) from ages 5 to 15 years through outpatient clinics at Perth Children's Hospital and The Royal Children's Hospital in Melbourne. (n=697 measurements, age 9.3 (2.1) years).

Findings Children with a total CT score at 5–6 years above the median were more likely to have abnormal FEV1 (aHR 2.67 (1.06, 6.72) p=0.037) during the next 10 years compared to those below the median chest CT score. The extent of all structural abnormalities except bronchial wall thickening were associated with lower FEV1 Z scores. Mucus plugging and trapped air were the most predictive sub-score (adjusted mean change −0.17 (−0.26, −0.07) p<0.001 and −0.09 (−0.14, −0.04) p<0.001 respectively).

Interpretation Chest CT identifies children at an early age who have adverse long-term outcomes. The prevention of structural lung damage should be a goal of early intervention and can be usefully assessed with chest CT. In an era of therapeutics that might alter disease trajectories, chest CT could provide an early readout of likely long-term success.


This manuscript has recently been accepted for publication in the European Respiratory Journal. It is published here in its accepted form prior to copyediting and typesetting by our production team. After these production processes are complete and the authors have approved the resulting proofs, the article will move to the latest issue of the ERJ online. Please open or download the PDF to view this article.

Conflict of interest: Dr. Turkovic has nothing to disclose.

Conflict of interest: Dr. Caudri has nothing to disclose.

Conflict of interest: Dr. Rosenow reports grants from National Health and Medical Research Council, during the conduct of the study; In addition, Dr. Rosenow has a patent PCT/AU2016/000079 issued to Australian Patent Office.

Conflict of interest: Dr. Breuer has nothing to disclose.

Conflict of interest: Dr. Murray has nothing to disclose.

Conflict of interest: Dr. Tiddens reports other from Roche, other from Novartis, grants from CFF, grants from Vertex, grants from Chiesi, grants from NCFS outside the submitted work; In addition, Dr. Tiddens has a patent Vectura licensed, and a patent PRAGMA-CF scoring system issued and I am heading the Erasmus MC-Sophia Children's Hospital core laboratory LungAnalysis.

Conflict of interest: Dr. Ramanauskas has nothing to disclose.

Conflict of interest: Dr. Ranganathan has nothing to disclose.

Conflict of interest: Dr. Hall has nothing to disclose.

Conflict of interest: Dr. Stick has nothing to disclose.

  • Received April 16, 2019.
  • Accepted February 12, 2020.