Fuchs Susanne I, Gappa Monika, Eder Johannes, Unsinn Karin M, Steinkamp Gratiana, Ellemunter Helmut
Department of Paediatrics and Research Institute, Marien-Hospital Wesel gGmbH, Pastor Janßen Straße 8-38, 46483 Wesel, Germany.
Department of Paediatrics and Research Institute, Marien-Hospital Wesel gGmbH, Pastor Janßen Straße 8-38, 46483 Wesel, Germany.
Respir Med. 2014 Jun;108(6):865-74. doi: 10.1016/j.rmed.2014.03.011. Epub 2014 Mar 25.
Lung disease remains the main cause of morbidity and mortality in patients with Cystic Fibrosis (CF). To detect lung disease before clinical symptoms become apparent, sensitive tools are essential. Spirometry is used for monitoring, but the FEV1 remains frequently normal throughout childhood. The Lung Clearance Index (LCI) calculated from Multiple Breath Washout (MBW) was introduced at the CF centre Innsbruck in 2007 for assessing ventilation inhomogeneity in patients with mild lung disease. We hypothesized that LCIs in 2007 are of prognostic value for the presence or absence of structural lung changes in later years.
Between 2007 and 2010 MBW, spirometry and ultra-low-dose HR-CT were prospectively tracked in 36 patients (6-53 years) with a mean FEV1 ≥ 80% predicted in 2007.
At study start the majority of patients had abnormal CT scores and LCI results. While CT and spirometry remained largely stable throughout the study, LCI results slightly improved but still correlated with CT scores in 2010. LCI results in 2007 correlated with CT scores in 2010 while FEV1 did not. In 86% the LCI value in 2007 was indicative for the presence or absence of structural lung changes in 2010.
The LCI is a sensitive tool for detecting and tracking pulmonary changes. Extended structural changes are unlikely if the LCI is normal. The LCI has the potential to be used for monitoring the progression of early CF lung disease and assessing the effect of treatment in both clinical care and research settings.
肺部疾病仍然是囊性纤维化（CF）患者发病和死亡的主要原因。为了在临床症状出现之前检测出肺部疾病，敏感的检测工具至关重要。肺活量测定法用于监测，但在整个儿童期FEV1通常仍保持正常。2007年因斯布鲁克CF中心引入了根据多次呼吸冲洗（MBW）计算得出的肺清除指数（LCI），用于评估轻度肺部疾病患者的通气不均匀性。我们假设2007年的LCI对随后几年肺部结构变化的有无具有预后价值。
2007年至2010年期间，对36例年龄在6至53岁之间、2007年平均FEV1≥预测值80%的患者进行了MBW、肺活量测定法和超低剂量HR-CT的前瞻性跟踪。
研究开始时，大多数患者的CT评分和LCI结果异常。虽然在整个研究过程中CT和肺活量测定法基本保持稳定，但LCI结果略有改善，但在2010年仍与CT评分相关。2007年的LCI结果与2010年的CT评分相关，而FEV1则不然。86%的患者2007年的LCI值可指示2010年肺部结构变化的有无。
LCI是检测和跟踪肺部变化的敏感工具。如果LCI正常，则不太可能出现广泛的结构变化。LCI有潜力用于监测早期CF肺部疾病的进展，并在临床护理和研究环境中评估治疗效果。
