Early markers of cystic fibrosis structural lung disease: follow-up of the ACFBAL cohort

Abstract

Little is known about early predictors of later cystic fibrosis (CF) structural lung disease. This study examined early predictors of progressive structural lung abnormalities in children who completed the Australasian CF Bronchoalveolar Lavage (ACFBAL) clinical trial at age 5-years and participated in an observational follow-up study (CF-FAB).Eight Australian and New Zealand CF centres participated in CF-FAB and provided follow-up chest computed-tomography (CT) scans for children who had completed the ACFBAL study with baseline scans at age 5-years. CT scans were annotated using PRAGMA-CF scoring. Ordinal regression analysis and linear regression were used to investigate associations between PRAGMA-CF (Perth-Rotterdam Annotated Grid Morphometric Analysis for CF) outcomes at follow-up and variables measured during the ACFBAL study.99 out of 157 ACFBAL children (mean±sd age 13±1.5 years) participated in the CF-FAB study. The probability of bronchiectasis at follow-up increased with airway disease severity on the baseline CT scan. In multiple regression (retaining factors at p<0.05) the extent of bronchiectasis at follow-up was associated with baseline atelectasis (OR 7.2, 95% CI 2.4-22; p≤ 0.001), bronchoalveolar lavage (BAL) log 2 interleukin (IL)-8 (OR 1.2, 95% CI 1.05-1.5; p=0.010) and body mass index z-score (OR 0.49, 95% CI 0.24-1.00; p=0.05) at age 5 years. Percentage trapped air at follow-up was associated with BAL log 2 IL-8 (coefficient 1.3, 95% CI 0.57-2.1; p<0.001) at age 5 years.The extent of airway disease, atelectasis, airway inflammation and poor nutritional status in early childhood are risk factors for progressive structural lung disease in adolescence. (Copyright ©ERS 2020.)following investigators constitute the ACFBAL Study Investigators Group:; following investigators constitute the CF FAB Study Investigators Group:; Additional contributions: We are indebted to all current and former clinical and research staff from Queensland Children's Hospital, Brisbane:. Language: English. Date Revised: 20210621. Date Created: 20200118. Date Completed: 20210621. Update Code: 20220301. Publication Type: Journal Article, Observational Study, Research Support, Non-U.S. Gov't. Journal ID: 8803460. Publication Model: Electronic-Print. Cited Medium: Internet. NLM ISO Abbr: Eur Respir J. Comment: Comment in: Eur Respir J. 2020 Apr 3;55(4):. (PMID: 32245773). Linking ISSN: 09031936. Subset: MEDLINE; Date of Electronic Publication: 2020 Apr 03. Current Imprints: Publication: Sheffield, United Kingdom : European Respiratory Society; Original Imprints: Publication: Copenhagen : Published jointly by the Society and Munksgaard, 1988-Investigator: CE Wainwright; K Grimwood; J Cheney; N George; JB Carlin; CF Robertson; S Vidmar; R Carzino; M Moodie; DS Armstrong; PJ Cooper; AJ Martin; B Whitehead; CA Byrnes; HAWM Tiddens; CE Wainwright; K Grimwood; PD Sly; HAWM Tiddens; G Rogers; RJ Massie; CF Robertson; PJ Cooper; CA Byrnes; S Vidmar; AJ Martin; B Whitehead; D Armstrong; JB Carlin; P Wark; N Gailer; N Smith; C Moss; K Jess; P Yarrow; M McArthur; S Forbes; H Selvadurai; S Ranganathan; P Robinson; N Zajakovski; MC Erasmus; EV Wiel. <br />