Robinson Paul D, Cooper Peter, Van Asperen Peter, Fitzgerald Dominic, Selvadurai Hiran
Department of Respiratory Medicine, The Children's Hospital at Westmead, Westmead, NSW, Australia.
Pediatr Pulmonol. 2009 Aug;44(8):733-42. doi: 10.1002/ppul.20956.
The use of alternative more sensitive measures has become a focus of research in CF. The utility of indexes of ventilation, Lung Clearance Index (LCI) and peak aerobic capacity (peak VO(2)), were studied as assessment tools in gauging response to intravenous (IV) therapy in acute pulmonary exacerbation, in comparison to the more commonly used index of forced expiratory volume in 1 sec (FEV(1)). The utility of a previously published clinical score was further explored.
Patients aged 8-18 years admitted for IV antibiotic treatment of a pulmonary exacerbation were recruited. Spirometry, plethysmography, multiple breath nitrogen washout, exercise testing, and Cystic Fibrosis Clinical Score (CFCS) were performed on admission and prior to discharge.
Twenty-eight patients were recruited, with a mean (range) age of 13.7 (8; 17) years, 16 female and 12 male. Mean (range) admission FEV(1) was 61.4 (28; 92)% predicted, or z-score -3.09 (-6.15; -0.52), FVC 83.0 (38; 120)% predicted, or z-score -1.71 (-5.66; -1.17), and Shwachman-Kulczycki 68.9 (50; 90). FEV(1) increased by 7.0% (P < 0.01) from admission to discharge. Mean (range) admission LCI, 10.10 (6.87; 14.83), decreased by 3.8% (P = 0.03). Mean (range) admission peak VO(2) (ml/kg/min), 31.2 (23.4; 45.4), increased on discharge by 6.6% (P < 0.01). Proposed clinical thresholds, based on the available variability data, highlighted the heterogeneity of response in lung function tests. Mean (range) admission CFCS, 26.5 (19; 39), decreased to 19.9 (13; 31) on discharge, a 25.2% improvement (P < 0.01). CFCS demonstrated improvement in 27 of 28 patients. Changes in peak VO(2) (r = -0.50, P = 0.02) and LCI (r = 0.48, P = 0.01) correlated with CFCS change.
In children with mild-to-moderate CF, whilst statistically significant improvement in both LCI and peak VO(2) were seen, heterogeneity of response was evident. The most consistent improvement was seen in CFCS. Correlation of LCI and peak VO(2) with change in clinical score (CFCS) was seen. The full clinical significance of these changes in LCI and peak VO(2) needs to be evaluated further with additional variability data. The CFCS may be useful in the assessment of response to treatment in CF but requires formal validation.
采用更敏感的替代测量方法已成为囊性纤维化（CF）研究的一个重点。与更常用的1秒用力呼气量（FEV₁）指标相比，研究了通气指标、肺清除指数（LCI）和最大有氧能力峰值（峰值VO₂）作为评估急性肺部加重期静脉注射（IV）治疗反应的工具的效用。进一步探讨了先前发表的临床评分的效用。
招募年龄在8至18岁因肺部加重期接受静脉抗生素治疗的患者。入院时和出院前进行肺功能测定、体积描记法、多次呼吸氮洗脱、运动测试以及囊性纤维化临床评分（CFCS）。
招募了28名患者，平均（范围）年龄为13.7（8；17）岁，16名女性和12名男性。入院时FEV₁平均（范围）为预测值的61.4（28；92）%，或z评分-3.09（-6.15；-0.52），FVC为预测值的83.0（38；120）%，或z评分-1.71（-5.66；-1.17），Shwachman-Kulczycki评分为68.9（50；90）。从入院到出院FEV₁增加了7.0%（P < 0.01）。入院时LCI平均（范围）为10.10（6.87；14.83），下降了3.8%（P = 0.03）。入院时峰值VO₂平均（范围）（ml/kg/min）为31.2（23.4；45.4），出院时增加了6.6%（P < 0.01）。基于现有变异性数据提出的临床阈值突出了肺功能测试中反应的异质性。入院时CFCS平均（范围）为26.5（19；39），出院时降至19.9（13；31），改善了25.2%（P < 0.01）。28名患者中有27名CFCS有改善。峰值VO₂（r = -0.50，P = 0.02）和LCI（r = 0.48，P = 0.01）的变化与CFCS变化相关。
在轻度至中度CF儿童中，虽然LCI和峰值VO₂均有统计学上的显著改善，但反应的异质性明显。CFCS的改善最为一致。观察到LCI和峰值VO₂与临床评分（CFCS）变化相关。LCI和峰值VO₂这些变化的全部临床意义需要通过更多变异性数据进一步评估。CFCS可能有助于评估CF的治疗反应，但需要正式验证。
