Longitudinal evaluation of bronchopulmonary disease in children with cystic fibrosis
Longitudinal evaluation of bronchopulmonary disease in children with cystic fibrosis
复制标题
DOI：
10.1002/ppul.10336
复制
发表时间：
2003-09-01
影响因子：
3.1
通讯作者：
Splaingard, ML
中科院分区：
文献类型：
--
作者：
Farrell, PM;Li, ZH;Splaingard, ML
患有囊性纤维化（CF）的儿童在不同的年龄发展为支气管肺疾病。然而，在婴幼儿中确定慢性肺病的流行病学并量化其严重程度一直很困难。作为威斯康星州CF新生儿筛查项目的一部分，我们有一个理想的机会来纵向评估CF呼吸道疾病的症状、体征和定量指标的演变。在新生儿筛查测试结果导致早期识别后，64名中位年龄为6.71周的患者被招募并在中位年龄为11.3岁时进行系统研究，以获得临床信息，胸部X光片和肺功能检查。我们的观察结果显示，有一半的患者在10.5个月大时就有频繁咳嗽的病史。定量胸部放射学（CXR评分）表明，潜在的不可逆的异常存在于一半的儿童2岁。威斯康星州和Brasfield CXR评分的严重程度与呼吸道感染相关。威斯康星州CXR评分的纵向进展与年龄（P < 0.001）、胰腺功能不全（P = 0.005）和呼吸道分泌物金黄色葡萄球菌培养阳性（P = 0.039）相关。相比之下，连续肺功能测定显示出有限的灵敏度，肺容量测定也是如此;在7岁之前，作为可接受的质量控制的重复测量，两者都不令人满意。事件发生时间分析显示，半数患者FEF 25 - 75%预测值和FEV 1/FVC值分别在10.7年和9.9年时大于80%。我们得出结论，在评价的方法中，定量胸部放射学是目前经常评估CF中支气管肺疾病的最佳方法，并且大约85%的患者在5岁时的放射学进展是明显的。我们的研究结果还表明，支气管扩张和其他慢性感染的放射学证据是明显的气道阻塞之前，年轻的CF患者。(C)2003 Wiley-Liss，Inc.
Children with cystic fibrosis (CF) develop bronchopulmonary disease at variable ages. Determining the epidemiology of chronic lung disease and quantifying its severity, however, have been difficult in infants and young children. As part of the Wisconsin CF Neonatal Screening Project, we were presented with an ideal opportunity to assess longitudinally the evolution of symptoms, signs, and quantitative measures of CF respiratory disease. After newborn screening test results led to early recognition, 64 patients diagnosed at a median age of 6.71 weeks were enrolled and studied systematically at a median age of 11.3 years to obtain clinical information, chest radiographs, and pulmonary function tests. Our observations revealed that a frequent cough by history is evident by 10.5 months of age in half the patients. Quantitative chest radiology (CXR scoring) demonstrated that potentially irreversible abnormalities are present in half the children by 2 years. The severity of Wisconsin and Brasfield CXR scores increased in association with respiratory infections. Longitudinal progression of Wisconsin CXR scores was related to age (P < 0.001), pancreatic insufficiency (P = 0.005), and respiratory secretion cultures positive for Staphylococus aureas (P = 0.039). In contrast, serial spirometry showed limited sensitivity, as did lung volume determinations; neither was satisfactory as repeated measures with acceptable quality control until after 7 years of age. Time to event analyses revealed that half the patients had % predicted FEF25-75 and FEV1/FVC values greater than 80% until 10.7 and 9.9 years, respectively We conclude that of the methods evaluated, quantitative chest radiology is currently the best procedure for frequent assessment of bronchopulmonary disease in CF, and that radiographic progression is evident in approximately 85% of patients by 5 years of age. Our results also suggest that bronchiectasis and other radiographic evidence of chronic infection are apparent prior to airways obstruction in young CF patients. (C) 2003 Wiley-Liss, Inc.