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) log2 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 log2 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. In children with cystic fibrosis, airways disease severity on chest computed tomography at age 5 years increased the risk of bronchiectasis in adolescence and its extent was predicted by poorer nutrition, airway inflammation, and atelectasis http://bit.ly/2Nnk8LW

中文翻译：

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囊性纤维化结构性肺病的早期标志物：ACFBAL 队列的随访

关于晚期囊性纤维化 (CF) 结构性肺病的早期预测因素知之甚少。本研究检查了在 5 岁时完成澳大利亚 CF 支气管肺泡灌洗 (ACFBAL) 临床试验并参与观察性随访研究 (CF-FAB) 的儿童进行性肺结构异常的早期预测因素。八个澳大利亚和新西兰 CF 中心参与了 CF-FAB，并为完成 ACFBAL 研究并在 5 岁时进行基线扫描的儿童提供后续胸部计算机断层扫描 (CT) 扫描。CT 扫描使用 PRAGMA-CF 评分进行注释。序数回归分析和线性回归用于研究 PRAGMA-CF（珀斯-鹿特丹 CF 注释网格形态测量分析）结果与 ACFBAL 研究期间测量的变量之间的关联。157 名 ACFBAL 儿童中有 99 名（平均±sd 年龄 13±1.5 岁）参加了 CF-FAB 研究。随访时支气管扩张的可能性随着基线 CT 扫描上气道疾病的严重程度而增加。在多元回归（p<0.05 的保留因素）中，随访时支气管扩张的程度与基线肺不张（OR 7.2，95% CI 2.4–22；p≤ 0.001）、支气管肺泡灌洗液 (BAL) log2 白介素 (IL) 相关-8 (OR 1.2, 95% CI 1.05–1.5; p=0.010) 和体重指数 z 分数 (OR 0.49, 95% CI 0.24–1.00; p=0.05) 在 5 岁时。随访时截留空气的百分比与 5 岁时的 BAL log2 IL-8（系数 1.3，95% CI 0.57–2.1；p<0.001）相关。儿童早期气道疾病的程度、肺不张、气道炎症和营养不良是青春期进行性结构性肺病的危险因素。