Caudri Daan, Zitter David, Bronsveld Inez, Tiddens Harm
Department of Paediatric Pulmonology, Erasmus MC-Sophia Children's Hospital, Rotterdam, The Netherlands.
Telethon Kids Institute, The University of Western Australia, Subiaco, Australia.
Pediatr Pulmonol. 2017 Sep;52(9):1135-1141. doi: 10.1002/ppul.23739. Epub 2017 Jun 6.
Cystic Fibrosis (CF) lung disease is characterized by a marked heterogeneity. Sweat chloride-level is a functional marker of the CF Transmembrane Regulator (CFTR) protein and could be an important predictor of later disease severity.
In this retrospective analysis children from the Rotterdam CF clinic with available sweat chloride level at diagnosis and at least one routine spirometry-controlled volumetric chest CT scan in follow-up were included. CT scans were scored using the CF-CT scoring system (% of maximum). Associations between sweat chloride-levels and CF-CT scores were calculated using linear regression models, adjusting for age at sweat test and age at follow-up. Because structural lung damage develops over the course of many years, effect modification by the age at follow-up CT-scan was tested for by age-stratification.
In 59 children (30 male) sweat chloride was measured at diagnosis (median age 0.5 years, range 0-13) and later chest CT performed (median age 14 years, range 6-18). Sweat chloride was associated with significantly higher CT-CT total score, bronchiectasis score, and mucus plugging score. Stratification for age at follow-up in tertiles showed this association remained only in the oldest age group (range 15-18 years). In that subgroup associations were found with all but one of the CF-CT subscores, as well as with all tested lung functions parameters.
Sweat chloride-level is a significant predictor of CF lung disease severity as determined by chest CT and lung function. This association could only be demonstrated in children with follow-up to age 15 years and above.
囊性纤维化（CF）肺部疾病具有显著的异质性。汗液氯化物水平是CF跨膜调节因子（CFTR）蛋白的功能标志物，可能是后期疾病严重程度的重要预测指标。
在这项回顾性分析中，纳入了来自鹿特丹CF诊所的儿童，这些儿童在诊断时具有可用的汗液氯化物水平，并且在随访中至少有一次常规肺活量测定控制的容积胸部CT扫描。使用CF-CT评分系统（最大值的百分比）对CT扫描进行评分。使用线性回归模型计算汗液氯化物水平与CF-CT评分之间的关联，并对汗液测试时的年龄和随访时的年龄进行调整。由于肺部结构损伤是在多年的过程中发展的，因此通过年龄分层来测试随访CT扫描时年龄的效应修正。
59名儿童（30名男性）在诊断时测量了汗液氯化物（中位年龄0.5岁，范围0-13岁），随后进行了胸部CT检查（中位年龄14岁，范围6-18岁）。汗液氯化物与显著更高的CT-CT总分、支气管扩张评分和黏液阻塞评分相关。按随访时年龄三分位数分层显示，这种关联仅在年龄最大的年龄组（范围15-18岁）中存在。在该亚组中，除了一个CF-CT子评分外，还发现了与所有测试的肺功能参数的关联。
汗液氯化物水平是由胸部CT和肺功能确定的CF肺部疾病严重程度的重要预测指标。这种关联仅在随访至15岁及以上的儿童中得到证实。
