Department of Pediatrics, Division of Combined Internal Medicine and Pediatrics, University of Texas Southwestern Medical Center, Dallas, Texas, USA.
Pediatric Clinical Nutrition, Children's Health, Dallas, Texas, USA.
Nutr Clin Pract. 2024 Aug;39(4):903-910. doi: 10.1002/ncp.11142. Epub 2024 Mar 16.
Approximately 85% of patients with cystic fibrosis (CF) have exocrine pancreatic insufficiency (EPI) with 10% requiring supplemental nighttime enteral tube feedings. Administration of pancreatic enzyme replacement therapy (PERT) with nighttime feedings is fraught with challenges. RELiZORB (Alcresta Therapeutics, Inc), an in-line lipase cartridge, delivers PERT continuously with enteral feedings. Outcomes related to the use of this in-line lipase cartridge are lesser known. This project evaluated anthropometrics related to in-line lipase cartridge use among pediatric patients with CF already receiving oral PERT therapy prior to nighttime enteral feedings.
Retrospective chart review was performed on 29 patients with CF and EPI receiving supplemental tube feedings and utilizing in-line lipase cartridge for a continuous 12 month period between 2015 and 2019. Anthropometrics were evaluated 12 months before and after initiation of in-line lipase cartridge.
Compared with mean height z score at 6-months pre-in-line lipase cartridge, mean height z score at 6-months post-in-line-lipase cartridge (adjusted mean difference [AMD] = 0.2540; 95% CI = [0.0487, 0.4592]; P = 0.0153) and mean height z score at 12-months post-in-line lipase cartridge (AMD = 0.2684; 95% CI = [0.0203, 0.5166]; P = 0.0340) were significantly higher. Mean weight z score at 12-months post-in-line-lipase-cartridge neared statistical significance compared with 6-months pre-in-line lipase cartridge (AMD = 0.2816; 95% CI = [-0.0003, 0.5634]; P = 0.0502) when excluding seven patients with advanced lung disease (forced expiratory volume in the first second of expiration of 40%). Weight-for-length or body mass index did not significantly differ compared with pre-in-line lipase cartridge.
Use of in-line lipase cartridge with enteral feeds improved anthropometrics, especially height, in pediatric patients with CF.
大约 85%的囊性纤维化 (CF) 患者存在外分泌胰腺功能不全 (EPI),其中 10%需要补充夜间肠内管饲。在夜间喂养时给予胰酶替代治疗 (PERT) 存在诸多挑战。RELIZORB(Alcresta Therapeutics,Inc.)是一种在线脂肪酶试剂盒,可在肠内喂养的同时持续提供 PERT。关于这种在线脂肪酶试剂盒的应用结果鲜为人知。本项目评估了在接受口服 PERT 治疗的 CF 儿科患者中,使用夜间肠内喂养的在线脂肪酶试剂盒相关的人体测量学指标。
对 2015 年至 2019 年间接受补充管饲并使用在线脂肪酶试剂盒的 29 例 CF 合并 EPI 患者进行回顾性图表审查。在开始使用在线脂肪酶试剂盒前 12 个月和后 12 个月评估人体测量学指标。
与使用在线脂肪酶试剂盒前 6 个月的平均身高 z 评分相比,使用在线脂肪酶试剂盒后 6 个月的平均身高 z 评分(调整平均差值 [AMD] = 0.2540;95%CI [0.0487,0.4592];P = 0.0153)和使用在线脂肪酶试剂盒后 12 个月的平均身高 z 评分(AMD = 0.2684;95%CI [0.0203,0.5166];P = 0.0340)显著更高。在排除 7 例患有晚期肺部疾病(第一秒用力呼气量为预计值的 40%)的患者后,使用在线脂肪酶试剂盒后 12 个月的平均体重 z 评分接近统计学意义(AMD = 0.2816;95%CI [-0.0003,0.5634];P = 0.0502)。与使用在线脂肪酶试剂盒前相比,体重/身长或体重指数无显著差异。
在 CF 儿科患者中,使用在线脂肪酶试剂盒联合肠内喂养可改善人体测量学指标,尤其是身高。