Department of Pediatrics, School of Medicine, Marmara University, Istanbul, Turkey.
Division of Pediatric Pulmonology, School of Medicine, Marmara University, Istanbul, Turkey.
Pediatr Int. 2021 Jun;63(6):704-709. doi: 10.1111/ped.14488. Epub 2021 May 26.
Congenital lung malformations (CLM) are rare disorders and surgical intervention is the definitive treatment. Our aim is to evaluate the long-term lung function of patients with CLM after surgery compared to healthy children.
Sixteen children with CLM (M/F: 9/7) and 30 age-matched, healthy controls (M/F: 13/17) were included in the study. Demographic data were recorded and both groups were compared by spirometry and the nitrogen-based Lung Clearance Index (LCI).
Mean ± SD age of the patients was 12.0 ± 5.4 years. The mean forced expiratory volume in 1 s (FEV ), forced vital capacity (FVC), FEV /FVC, and forced expiratory flow between 25% and 75% of force expiration (FEF ) predicted was, 86.68 ± 16.65, 88.00 ± 14.58, 97.44 ± 9.89, and 79.00 ± 26.41, respectively in the patient group. Patients with CLM had significantly lower values in FEV , FVC, FEF than healthy controls (P = 0.002, P 0.007, P 0.045). While the mean LCI value in patients' group was 8.33 ± 1.52, it was 7.28 ± 0.80 in healthy controls (P = 0.023). Strong inverse correlation between LCI and FEV , FEV /FVC was detected in the patient group (P = 0.023; r: -0.581, P 0.017; r: -0.606 respectively).
This study revealed that, in long-term follow-up, patients who had surgery because of CLM have impairment in the pulmonary function compared to healthy children and LCI may be more accurate in detecting airway diseases early than spirometry.
先天性肺畸形（CLM）是罕见的疾病，手术干预是其明确的治疗方法。我们的目的是评估 CLM 患者手术后的长期肺功能，并与健康儿童进行比较。
本研究纳入了 16 名 CLM 患儿（男/女：9/7）和 30 名年龄匹配的健康对照者（男/女：13/17）。记录了人口统计学数据，并通过肺活量测定法和基于氮的肺清除指数（LCI）对两组进行了比较。
患者的平均年龄为 12.0 ± 5.4 岁。患者组的用力呼气量 1 秒（FEV1）、用力肺活量（FVC）、FEV1/FVC 以及用力呼出 25%至 75%肺活量时的流速（FEF25-75）预测值分别为 86.68 ± 16.65、88.00 ± 14.58、97.44 ± 9.89 和 79.00 ± 26.41。CLM 患者的 FEV1、FVC 和 FEF 明显低于健康对照组（P = 0.002、P < 0.007、P = 0.045）。而患者组的平均 LCI 值为 8.33 ± 1.52，健康对照组为 7.28 ± 0.80（P = 0.023）。在患者组中，LCI 与 FEV1 和 FEV1/FVC 之间存在强烈的负相关（P = 0.023；r：-0.581，P = 0.017；r：-0.606）。
本研究表明，在长期随访中，因 CLM 而接受手术的患者的肺功能较健康儿童受损，LCI 可能比肺活量测定法更早、更准确地检测气道疾病。
