Koch C, McKenzie S G, Kaplowitz H, Hodson M E, Harms H K, Navarro J, Mastella G
Department of Pediatrics, Rigshospitalet, Copenhagen, Denmark.
Pediatr Pulmonol. 1997 Aug;24(2):147-54; discussion 159-61. doi: 10.1002/(sici)1099-0496(199708)24:2<147::aid-ppul15>3.0.co;2-2.
The Epidemiologic Registry of Cystic Fibrosis provides clinical profiles for more than 6,800 patients and descriptions of practice patterns across eight European countries. Preliminary cross-sectional analysis has been performed by age and pulmonary function as an assessment of disease severity. In general, pulmonary treatments including inhaled bronchodilators and rhDNase increased as lung disease became more severe. Use of a number of treatments, including mucolytic agents and inhaled corticosteroids, varied markedly from country to country. Several widely used therapies are not yet supported by controlled clinical trials, particularly in patients under 6 years of age. Nutritional intervention was more common in patients with advanced lung disease regardless of age. Patients with nasal polyps had less severe lung disease at each age than patients without polyps. It is clear that studies of early interventions are needed to determine the optimal types of treatments and the ages at which to begin treatment.
囊性纤维化流行病学登记处提供了6800多名患者的临床资料以及八个欧洲国家的诊疗模式描述。已按年龄和肺功能进行了初步横断面分析，以此评估疾病严重程度。一般而言，随着肺部疾病加重，包括吸入性支气管扩张剂和重组人脱氧核糖核酸酶在内的肺部治疗方法使用增加。包括黏液溶解剂和吸入性糖皮质激素在内的多种治疗方法的使用在不同国家之间存在显著差异。一些广泛使用的疗法尚未得到对照临床试验的支持，尤其是在6岁以下的患者中。无论年龄大小，营养干预在晚期肺部疾病患者中更为常见。在每个年龄段，患有鼻息肉的患者肺部疾病比没有息肉的患者轻。显然，需要开展早期干预研究，以确定最佳治疗类型和开始治疗的年龄。
