Clinical outcome in relation to care in centres specialising in cystic fibrosis: cross sectional study

Objectives: To assess the effect on clinical outcome of managing paediatric and adult patients with cystic fibrosis at specialised cystic fibrosis centres. Design: Cross sectional study. Setting: Two adult cystic fibrosis centres in the United Kingdom. Subjects: Patients from an adult cystic fibrosis centre in Manchester were subdivided into those who had received continuous care from paediatric and adult cystic fibrosis centres (group A), and those who had received paediatric care in a centre not specialising in cystic fibrosis followed by adult care in a cystic fibrosis centre (group B). Group C were referrals to the new adult cystic fibrosis centre in Cambridge who had received neither paediatric nor adult centre care for their cystic fibrosis. Main outcome measures: Body mass index (weight (kg)/height (m(2))), lung function (forced expiratory volume in one second (FEV(1) percentage of predicted)), the Northern chest x ray film score, and age at colonisation with Pseudomonas aeruginosa. Results: A prominent stepwise increase in body mass index was associated with increasing amounts of care at a cystic fibrosis centre; 18.3, 20.2, and 21.3 for groups C, B, and A respectively (P<0.001). Improved nutritional status was correlated with a higher FEV(1) and better (lower) chest x ray film scores; r=0.52 and −0.45 respectively (P<0.001 for both). Conclusion: These findings provide the first direct evidence that management of cystic fibrosis in paediatric and adult cystic fibrosis centres results in a better clinical outcome, and strongly supports the provision of these specialist services.

研究目标：评估在专科囊性纤维化（cystic fibrosis）诊疗中心管理儿童及成人囊性纤维化患者对其临床结局的影响。
研究设计：横断面研究（cross-sectional study）。
研究场景：英国两家成人囊性纤维化诊疗中心。
研究对象：来自曼彻斯特成人囊性纤维化诊疗中心的患者被分为两组：A组为曾在儿科及成人囊性纤维化诊疗中心接受持续诊疗的患者；B组为先在非囊性纤维化专科中心接受儿科诊疗，随后在囊性纤维化专科中心接受成人诊疗的患者。C组为转诊至剑桥市新建成人囊性纤维化诊疗中心的患者，其囊性纤维化既往未接受过儿科或成人专科中心的诊疗。
主要结局指标：身体质量指数（体重(kg)/身高(m²)）、肺功能（一秒用力呼气容积占预计值百分比（forced expiratory volume in one second, FEV₁））、诺森胸部X线评分（Northern chest X-ray film score）以及铜绿假单胞菌（Pseudomonas aeruginosa）定植时的年龄。
研究结果：身体质量指数随囊性纤维化专科诊疗时长的增加呈显著阶梯式升高，C、B、A组的数值分别为18.3、20.2和21.3（P<0.001）。营养状况改善与更高的FEV₁占预计值百分比及更优（更低）的胸部X线评分相关，相关系数r分别为0.52和-0.45（两项指标P均<0.001）。
研究结论：本研究结果首次直接证实，在儿科及成人囊性纤维化专科中心对囊性纤维化患者实施诊疗可获得更佳临床结局，有力支持了此类专科诊疗服务的开展。