Welsh Liam, Robertson Colin F, Ranganathan Sarath C
Respiratory Medicine, Royal Children's Hospital, Melbourne, Australia; Murdoch Children's Research Institute, Melbourne, Australia.
Pediatr Pulmonol. 2014 Sep;49(9):873-7. doi: 10.1002/ppul.22946. Epub 2013 Oct 31.
Though baseline lung function as measured by spirometry in children with cystic fibrosis (CF) has improved, the annual rate of decline has not changed significantly during the critical period of adolescence. The aim of this study was to describe factors associated with longitudinal decline in lung function in a contemporary cohort of children with CF.
Best annual lung function data from children attending the CF service of the Royal Children's Hospital Melbourne were reviewed to determine rate of decline in FEV(1) up until time of transfer to an adult center. Mixed models were used to determine the influence of age, sex, genotype, newborn screening, respiratory hospitalization, CF related diabetes mellitus (CFRD), pancreatic insufficiency, Pseudomonas aeruginosa (PsA) infection, and body mass index (BMI) on lung function decline.
Longitudinal lung function data (range 5-20 years) were obtained for 98 patients with CF (55 male). Overall, the annual rate of decline in FEV(1) % predicted for the entire cohort was 1.4% per annum though the greatest rate of FEV1 decline was seen during adolescence (2.6%). Increasing age, homozygous ΔF508 genotype, CFRD, mucoid PsA infection, pancreatic insufficiency and respiratory hospitalizations were all significant predictors of FEV1 decline.
FEV(1) declines at its sharpest rate during adolescence even in the presence of newborn screening. Genotype, increasing age, CFRD, PsA infection, pancreatic insufficiency and a greater number of respiratory hospitalizations are all associated with an increased rate of lung function decline in Australian children and adolescents with cystic fibrosis.
尽管通过肺活量测定法测得的囊性纤维化（CF）患儿的基线肺功能有所改善，但在青春期这一关键时期，其年下降率并未显著改变。本研究的目的是描述当代CF患儿队列中与肺功能纵向下降相关的因素。
回顾了墨尔本皇家儿童医院CF门诊患儿的最佳年度肺功能数据，以确定在转至成人中心之前FEV（1）的下降率。使用混合模型来确定年龄、性别、基因型、新生儿筛查、呼吸道住院、CF相关糖尿病（CFRD）、胰腺功能不全、铜绿假单胞菌（PsA）感染和体重指数（BMI）对肺功能下降的影响。
获得了98例CF患者（55例男性）的纵向肺功能数据（范围为5 - 20岁）。总体而言，整个队列预测的FEV（1）%的年下降率为每年1.4%，尽管在青春期FEV1下降率最高（2.6%）。年龄增加、纯合子ΔF508基因型、CFRD、黏液样PsA感染、胰腺功能不全和呼吸道住院都是FEV1下降的显著预测因素。
即使存在新生儿筛查，FEV（1）在青春期下降速度最快。在澳大利亚CF患儿和青少年中，基因型、年龄增加、CFRD、PsA感染、胰腺功能不全以及更多次的呼吸道住院都与肺功能下降率增加相关。
