异常的学龄前肺部清除指数（LCI）反映了临床状态，并预测了儿童期后期囊性纤维化的肺活量测定值较低。
Abnormal preschool Lung Clearance Index (LCI) reflects clinical status and predicts lower spirometry later in childhood in cystic fibrosis.
机构信息
Department of Respiratory Medicine, The Children's Hospital at Westmead (CHW), Sydney, Australia.
Sydney Medical School, University of Sydney, Australia.
出版信息
J Cyst Fibros. 2019 Sep;18(5):721-727. doi: 10.1016/j.jcf.2019.02.007. Epub 2019 Mar 1.
BACKGROUND
Clinical and prognostic value of preschool Multiple Breath Washout (MBW) remains unclear.
METHODS
Initial MBW results (Exhalyzer® D, EcoMedics AG) in preschool Cystic Fibrosis (CF) subjects (age 2-6 years) at a time of clinical stability were compared to (1) concurrent clinical status measures and (2) later spirometry outcomes. Abnormal Lung Clearance Index (LCI) was defined using published reference data (ULN for LCI 8.0).
RESULTS
LCI was abnormal in 56% (28/50), with mean (SD) LCI 8.61(1.85) at age 4.71(1.3) years. Abnormal LCI was associated with higher dornase alfa use, previous positive bacterial cultures and pF508.del homozygous genotype. Later spirometry (n = 44; mean (SD) 2.3(0.5) years after MBW) demonstrated that abnormal initial preschool LCI was a strong predictor of lower later spirometry outcomes.
CONCLUSION
Abnormal preschool LCI was associated with concurrent measures of clinical status and later spirometry deficits, suggesting early prognostic utility of MBW testing in this age range.
背景
学龄前多次呼吸冲洗（MBW）的临床和预后价值尚不清楚。
方法
在临床稳定期，比较初始 MBW 结果（EcoMedics AG 的 Exhalyzer® D）与（1）同期临床状态测量值和（2）随后的肺活量测定结果。异常肺清除指数（LCI）使用已发表的参考数据（LCI 的 ULN 为 8.0）定义。
结果
56%（28/50）的 LCI 异常，平均（SD）LCI 为 4.71（1.3）岁时为 8.61（1.85）。异常 LCI 与较高的脱氧核糖核酸酶α使用、先前的阳性细菌培养和 pF508.del 纯合基因型相关。随后的肺活量测定（n=44；MBW 后平均（SD）2.3（0.5）年）表明，初始学龄前异常 LCI 是后来肺活量测定结果较低的强烈预测因素。
结论
学龄前异常 LCI 与同期临床状态测量值和随后的肺活量测定缺陷相关，表明 MBW 测试在该年龄段具有早期预后价值。
Zotero 插件