Abstract：
Most people with cystic fibrosis (pwCF) develop pancreatic insufficiency and are treated with pancreatic enzyme replacement therapy (PERT). We aimed to describe the use of PERT and assess the correlates of PERT dose in adult pwCF. In a cross-sectional study at the Copenhagen CF Centre, the participants reported PERT intake, gastrointestinal (GI) symptoms and the use of concomitant treatments. Demographic and clinical characteristics were extracted from the Danish CF Registry. We used linear regression to assess the correlates of PERT dose per kg bodyweight (U-lipase/kg). We included 120 pwCF with a median age of 32.9 years, 46% women and 72% F508delta homozygote. The PERT dose ranged from 0 to 6160 U-lipase/kg per main meal (mean 1828; SD 1115). The PERT dose was associated with participants\' sex (men vs. women: 661; 95% CI: 302; 1020 U-lipase/kg), age (-16; 95% CI: -31; -1 U-lipase/kg per year) and weight (-45; 95% CI: -58; -31 U-lipase/kg per kg). Having less frequent constipation and being lung transplanted were also associated with a higher PERT dose. A third of participants did not take PERT for snacks, and this was associated with the frequency of diarrhoea. These findings indicate that PERT intake may be improved to reduce GI symptoms.
摘要：
大多数囊性纤维化（pwCF）患者会出现胰腺功能不全，并接受胰酶替代疗法（PERT）治疗。我们旨在描述PERT的使用，并评估成人pwCF中PERT剂量的相关性。在哥本哈根CF中心的一项横断面研究中，参与者报告了PERT的摄入量，胃肠道（GI）症状和伴随治疗的使用。人口统计学和临床特征从丹麦CF登记处提取。我们使用线性回归来评估PERT剂量/kg体重(U-脂肪酶/kg)的相关性。我们纳入了120pwCF，中位年龄为32.9岁，46%的女性和72%的F508δ纯合子。PERT剂量范围为每主餐0至6160U-脂肪酶/kg（平均值1828；SD1115）。PERT剂量与参与者的性别相关(男性与女性：661；95%CI：302；1020U-脂肪酶/kg），年龄（-16；95%CI：-31；-1U-脂肪酶/kg/年）和体重（-45；95%CI：-58；-31U-脂肪酶/kg/kg）。便秘频率较低和进行肺移植也与较高的PERT剂量有关。三分之一的参与者没有吃PERT零食，这与腹泻的频率有关。这些发现表明，PERT的摄入可能会得到改善，以减轻胃肠道症状。
