Department of Pediatric Surgery, The New Children's Hospital, University of Helsinki, Stenbäckinkatu 9, 00029 HUS, Helsinki, Finland.
Department of Pediatric Surgery, University Children's Hospital, Uppsala University, Uppsala, Sweden.
Clin Nutr. 2023 Jul;42(7):1095-1103. doi: 10.1016/j.clnu.2023.05.017. Epub 2023 May 25.
BACKGROUND & AIMS: Despite advances in the management of short bowel syndrome related intestinal failure (SBS-IF), large-scale contemporary pediatric studies are scarce. The aim of this multicenter study was to assess key outcomes and clinical prognostic factors in a recent Nordic pediatric SBS-IF population.
Patients with SBS-IF treated during 2010-2019, whose parenteral support (PS) started at age <1 year and continued >60 consecutive days were included and retrospectively reviewed. All six participating centers followed multidisciplinary SBS-IF management. Risk factors for PS dependency, intestinal failure associated liver disease (IFALD) and mortality were assessed with Cox regression and Kaplan Meier analyses. IFALD was defined with serum liver biochemistry levels.
Among 208 patients, SBS-IF resulted from NEC in 49%, gastroschisis w/wo atresia in 14%, small bowel atresia in 12%, volvulus in 11%, and other diagnoses in 14%. Median age-adjusted small bowel length was 43% (IQR 21-80%). After median follow up of 4.4 years (IQR 2.5-6.9), enteral autonomy was reached by 76%, none had undergone intestinal transplantation, and overall survival was 96%. Half of deaths (4/8) were caused by septic complications. Although biochemical cholestasis occurred only in 3% at latest follow-up and none of deaths were directly caused by IFALD, elevated liver biochemistry (HR 0.136; P = 0.017) and shorter remaining small bowel (HR 0.941; P = 0.040) predicted mortality. Shorter remaining small bowel and colon, and presence of end-ostomy were the main predictors of PS dependency, but not IFALD. Patients with NEC reached enteral autonomy more efficiently and had decreased incidence of IFALD compared to other etiologies.
Although with current multidisciplinary management, prognosis of pediatric SBS is encouraging, septic complications and IFALD still associated with the remaining low mortality rate.
尽管短肠综合征相关肠衰竭（SBS-IF）的治疗取得了进展，但大规模的当代儿科研究仍然很少。本项多中心研究的目的是评估最近北欧儿科 SBS-IF 人群的关键结局和临床预后因素。
纳入 2010-2019 年接受治疗、开始肠外营养（PS）时年龄<1 岁且持续>60 天的 SBS-IF 患儿，并进行回顾性研究。所有参与中心均遵循多学科 SBS-IF 管理。使用 Cox 回归和 Kaplan-Meier 分析评估 PS 依赖、肠衰竭相关肝病（IFALD）和死亡率的危险因素。通过血清肝功能检测定义 IFALD。
在 208 例患者中，SBS-IF 由 NEC 引起的占 49%，胃破裂伴/不伴闭锁占 14%，小肠闭锁占 12%，肠扭转占 11%，其他诊断占 14%。年龄调整后的小肠长度中位数为 43%（IQR 21-80%）。中位随访 4.4 年后（IQR 2.5-6.9），76%的患者实现了肠内自主，无一例患者进行了肠移植，总体生存率为 96%。一半的死亡（4/8）是由感染性并发症引起的。尽管在最近的随访中只有 3%的患者出现生化性胆汁淤积，且没有死亡病例是直接由 IFALD 引起的，但肝功能升高（HR 0.136；P=0.017）和剩余小肠较短（HR 0.941；P=0.040）与死亡率相关。剩余小肠和结肠较短以及存在肠造口术是 PS 依赖的主要预测因素，但与 IFALD 无关。与其他病因相比，NEC 患儿达到肠内自主的效率更高，IFALD 的发生率更低。
尽管目前采用多学科管理，但儿科 SBS 的预后令人鼓舞，但感染性并发症和 IFALD 仍然与较低的死亡率相关。
