Lung Clearance Index at 4 Years Predicts Subsequent Lung Function in Children with Cystic Fibrosis
Lung Clearance Index at 4 Years Predicts Subsequent Lung Function in Children with Cystic Fibrosis
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DOI：
10.1164/rccm.200911-1646oc
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发表时间：
2011-03-15
影响因子：
24.7
通讯作者：
Stocks, Janet
中科院分区：
文献类型：
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作者：
Aurora, Paul;Stanojevic, Sanja;Stocks, Janet
理论基础：囊性纤维化(CF)儿童预期寿命的显著提高带来了新的挑战，因为肺部疾病的传统标记物在幼儿中通常是正常的。这阻止了确定谁可能从更积极的治疗中受益，并要求进行大量的研究和延长干预研究的持续时间。本研究的目的是确定学龄前多次呼吸冲洗(MBW)结果是否可以预测随后的肺功能异常。方法：对3~5岁的CF学龄前儿童和健康对照儿童进行肺活量测定和MBW检查，并在学龄前(6~10岁)重复检测。主要结果是肺活量测定的FEV1和MBW的肺清除指数(LCI)。测量和主要结果：48名CF儿童和45名健康儿童完成了测试。35名(73%)CF儿童在学龄前有异常的LCI，而只有5名FEV1异常。学龄前LCI对学龄期成绩异常的阳性预测值为94%，阴性预测值为62%。只有一名学龄前FEV1异常的儿童有正常的学龄前LCI。相比之下，学龄前儿童FEV1的阳性预测值为100%，而阴性预测值仅为25%。结论：本研究表明，学龄前LCI异常预示着随后的肺功能异常，而正常的学龄前LCI通常保持正常。MBW有潜力作为治疗儿童CF的临床和研究成果。
Rationale: The markedly improved life expectancy of children with cystic fibrosis (CF) has created a new challenge, as traditional markers of lung disease are frequently normal in young children. This prevents identification of individuals who may benefit from more aggressive therapy and also obliges large study numbers and prolonged duration for intervention studies. There is an urgent need for alternative surrogates that detect early lung disease and track through early childhood.Objectives: This study aimed to determine whether multiple-breath washout (MBW) results at preschool age can predict subsequent abnormal lung function.Methods: Preschool children (3-5 yr) with CF and healthy control subjects underwent spirometry and MBW with testing repeated during early school age (6-10 yr). Primary outcomes were FEV1 from spirometry and lung clearance index (LCI) from MBW.Measurements and Main Results: Forty-eight children with CF and 45 healthy children completed testing. Thirty-five (73%) children with CF had abnormal LCI at preschool age, whereas only five had abnormal FEV1. The positive predictive value of preschool LCI for predicting any abnormal school-age result was 94%, with a negative predictive value of 62%. Only one child with abnormal FEV1 at school age had had a normal preschool LCI. In contrast, for preschool FEV1 the positive predictive value was 100%, but negative predictive value was only 25%.Conclusions: This study demonstrates that an abnormal preschool LCI predicts subsequent lung function abnormalities, whereas a normal preschool LCI usually remains normal. MBW has potential as a clinical and research outcome in young children with CF.