Navarro J, Rainisio M, Harms H K, Hodson M E, Koch C, Mastella G, Strandvik B, McKenzie S G
Dept of Gastroenterology and Cystic Fibrosis, Robert Debré Hospital, AP/HP, Paris, France.
Eur Respir J. 2001 Aug;18(2):298-305. doi: 10.1183/09031936.01.00068901.
The European Epidemiologic Registry of Cystic Fibrosis began collecting longitudinal data on European cystic fibrosis patients in 1994. A cross-sectional analysis was performed to identify the factors associated with low values of % predicted forced expiratory volume in one second (FEV1) upon patient enrollment. Data from 7,010 patients aged > or =6 yrs were included. Clinical conditions, microbiological isolates and medications reported at enrollment or within the following 180 days were analysed for age-specific associations. Factors associated with FEV1 that were lower by >10% of pred values were: lower weight for age percentiles, haemoptysis, pneumothorax, pulmonary symptoms at presentation, Pseudomonas aeruginosa, Burkholderia cepacia, oral corticosteroids, nonsteroid anti-inflammatory drugs, dornase alfa, oxygen and assisted ventilation and, in patients >12 yrs old only, use of airway clearance techniques, inhaled bronchodilators, oral nutritional supplements, pancreatic enzymes and insulin or oral hypoglycaemics. Slightly impaired lung function (5-10%) was associated with: diabetes (> or = 18-yrs-old), gastro-oesophageal reflux, allergic bronchopulmonary aspergillosis, asthma-like symptoms, portal hypertension, Aspergillus spp. and Candida spp. Sex, Haemophilus influenzae and Staphylococcus aureus were not associated with impaired pulmonary status. Regular exercise (especially in older patients) and nasal polyposis were associated with slightly better FEV1. The results confirm those of previous studies and suggest selective prescribing in sicker patients.
欧洲囊性纤维化流行病学登记处自1994年开始收集欧洲囊性纤维化患者的纵向数据。进行了一项横断面分析，以确定患者入组时与一秒用力呼气量（FEV1）预测值低相关的因素。纳入了7010名年龄≥6岁的患者的数据。分析了入组时或随后180天内报告的临床状况、微生物分离株和药物的年龄特异性关联。与FEV1低于预测值>10%相关的因素有：年龄别体重百分位数较低、咯血、气胸、就诊时的肺部症状、铜绿假单胞菌、洋葱伯克霍尔德菌、口服皮质类固醇、非甾体抗炎药、重组人脱氧核糖核酸酶、吸氧和辅助通气，仅在12岁以上患者中，还有气道廓清技术的使用、吸入性支气管扩张剂、口服营养补充剂、胰酶以及胰岛素或口服降糖药。肺功能轻度受损（5-10%）与以下因素相关：糖尿病（≥18岁）、胃食管反流、变应性支气管肺曲霉病、哮喘样症状、门静脉高压、曲霉属和念珠菌属。性别、流感嗜血杆菌和金黄色葡萄球菌与肺功能受损无关。规律运动（尤其是老年患者）和鼻息肉与FEV1稍好相关。结果证实了先前研究的结果，并提示对病情较重的患者进行选择性用药。
