Amsterdam UMC, University of Amsterdam, Emma Children's Hospital, Pediatric Gastroenterology, Amsterdam Gastroenterology, Endocrinology and Metabolism, Amsterdam Reproduction & Development.
Emma Children's Hospital, Amsterdam UMC, University of Amsterdam, Child and Adolescent Psychiatry & Psychosocial Care, Amsterdam Reproduction and Development, Amsterdam Public Health, Amsterdam.
J Pediatr Gastroenterol Nutr. 2022 Jan 1;74(1):116-122. doi: 10.1097/MPG.0000000000003329.
The aim of the study was to describe the longitudinal development of health-related quality of life (HRQOL) and fatigue in children with chronic intestinal failure (CIF) on home parenteral nutrition (PN) and compare these children to the general population.
Prospective, observational study conducted over 7 years in patients suffering from CIF receiving home PN from 2 tertiary hospitals in the Netherlands. Every 6 months, parents (if child <8 years old) or patients (if child ≥8 years old) completed 2 questionnaires: Pediatric Quality of Life Inventory 4.0 (PedsQL) Generic and Fatigue on the KLIK (kwaliteit van leven in kaart [Dutch Acronym for Quality of Life in Clinical Practice]) Patient Reported Outcome Measures portal, which were compared with the general population. Linear mixed models (LMMs) were constructed to investigate the course of HRQOL over time.
Thirty-five patients were included (40% girls). At time of last KLIK contact, patients received HPN for a median of 5.3 years (interquartile range [IQR]: 2.9-9.7). In total, 272 questionnaires were completed. PedsQL generic total score for ages 5 to 7 and 8 to 12 years was significantly lower than the general population (P < 0.01 for both age groups) with effect sizes of 0.73 and 0.71, respectively. PedsQL fatigue total score for ages 5 to 7 years was also significantly lower (P = 0.01; effect size 0.70). LMMs for PedsQL Generic and Fatigue total score 2 to 7 and 8 to 18 years showed no significant coefficient for duration of home PN.
Children suffering from CIF receiving home PN ages 5 to 12 years report lower HRQOL scores than the general population. HRQOL and fatigue do not change during long-term treatment with home PN in these children.
本研究旨在描述接受家庭肠外营养（PN）的慢性肠衰竭（CIF）儿童健康相关生活质量（HRQOL）和疲劳的纵向发展，并将这些儿童与普通人群进行比较。
本研究为前瞻性观察研究，在荷兰的 2 家三级医院中，对接受家庭 PN 的 CIF 患者进行了 7 年的随访。每 6 个月，由父母（如果患儿年龄<8 岁）或患儿（如果患儿年龄≥8 岁）填写 2 份问卷：儿科生活质量量表 4.0（PedsQL）通用量表和 KLIK 疲劳量表（荷兰临床实践中生活质量的缩写）患者报告结局测量工具（Patient Reported Outcome Measures portal），并与普通人群进行比较。采用线性混合模型（LMM）来研究 HRQOL 的随时间变化的过程。
共纳入 35 例患儿（40%为女孩）。截至最后一次 KLIK 联系时，患儿接受 HPN 的中位时间为 5.3 年（四分位距 [IQR]：2.9-9.7）。共完成了 272 份问卷。5-7 岁和 8-12 岁的 PedsQL 通用总分显著低于普通人群（两组 P 值均<0.01），效应量分别为 0.73 和 0.71。5-7 岁的 PedsQL 疲劳总分也显著较低（P=0.01；效应量为 0.70）。LMM 用于 2-7 岁和 8-18 岁的 PedsQL 通用和疲劳总分均显示家庭 PN 持续时间无显著系数。
接受家庭 PN 的 5-12 岁 CIF 患儿报告的 HRQOL 评分低于普通人群。在这些儿童中，长期接受家庭 PN 治疗不会导致 HRQOL 和疲劳发生变化。
