Cystic Fibrosis Centre, Department of Infectious Diseases, Rigshospitalet, 2100 Copenhagen, Denmark.
Department of Nutrition, Exercise and Sports, University of Copenhagen, 1958 Frederiksberg, Denmark.
Nutrients. 2022 Mar 22;14(7):1330. doi: 10.3390/nu14071330.
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.
大多数囊性纤维化（CF）患者会出现胰腺功能不全，并接受胰酶替代治疗（PERT）。我们旨在描述 PERT 的使用情况，并评估成人 CF 患者 PERT 剂量的相关因素。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）症状以及同时使用的治疗方法。从丹麦 CF 登记处提取了人口统计学和临床特征。我们使用线性回归来评估 PERT 剂量与体重（U-脂肪酶/公斤）的相关性。我们纳入了 120 名 CF 患者，中位年龄为 32.9 岁，46%为女性，72%为 F508del 纯合子。PERT 剂量范围为每餐 0 至 6160 U-脂肪酶/公斤（平均 1828；SD 1115）。PERT 剂量与参与者的性别（男性与女性：661；95%CI：302；1020 U-脂肪酶/公斤）、年龄（-16；95%CI：-31；-1 U-脂肪酶/公斤/年）和体重（-45；95%CI：-58；-31 U-脂肪酶/公斤）相关。便秘发作频率较低和接受肺移植也与较高的 PERT 剂量相关。三分之一的参与者没有在零食时服用 PERT，这与腹泻的频率有关。这些发现表明，可以改善 PERT 的摄入以减少 GI 症状。
