Khan Faraz A, Squires Robert H, Litman Heather J, Balint Jane, Carter Beth A, Fisher Jeremy G, Horslen Simon P, Jaksic Tom, Kocoshis Samuel, Martinez J Andres, Mercer David, Rhee Susan, Rudolph Jeffrey A, Soden Jason, Sudan Debra, Superina Riccardo A, Teitelbaum Daniel H, Venick Robert, Wales Paul W, Duggan Christopher
Boston Children's Hospital, Boston, MA.
Children's Hospital of Pittsburgh of University of Pittsburgh Medical Center, Pittsburgh, PA.
J Pediatr. 2015 Jul;167(1):29-34.e1. doi: 10.1016/j.jpeds.2015.03.040. Epub 2015 Apr 25.
In a large cohort of children with intestinal failure (IF), we sought to determine the cumulative incidence of achieving enteral autonomy and identify patient and institutional characteristics associated with enteral autonomy.
A multicenter, retrospective cohort analysis from the Pediatric Intestinal Failure Consortium was performed. IF was defined as severe congenital or acquired gastrointestinal diseases during infancy with dependence on parenteral nutrition (PN) >60 days. Enteral autonomy was defined as PN discontinuation >3 months.
A total of 272 infants were followed for a median (IQR) of 33.5 (16.2-51.5) months. Enteral autonomy was achieved in 118 (43%); 36 (13%) remained PN dependent and 118 (43%) patients died or underwent transplantation. Multivariable analysis identified necrotizing enterocolitis (NEC; OR 2.42, 95% CI 1.33-4.47), care at an IF site without an associated intestinal transplantation program (OR 2.73, 95% CI 1.56-4.78), and an intact ileocecal valve (OR 2.80, 95% CI 1.63-4.83) as independent risk factors for enteral autonomy. A second model (n = 144) that included only patients with intraoperatively measured residual small bowel length found NEC (OR 3.44, 95% CI 1.36-8.71), care at a nonintestinal transplantation center (OR 6.56, 95% CI 2.53-16.98), and residual small bowel length (OR 1.04 cm, 95% CI 1.02-1.06 cm) to be independently associated with enteral autonomy.
A substantial proportion of infants with IF can achieve enteral autonomy. Underlying NEC, preserved ileocecal valve, and longer bowel length are associated with achieving enteral autonomy. It is likely that variations in institutional practices and referral patterns also affect outcomes in children with IF.
在一大群肠衰竭（IF）患儿中，我们试图确定实现肠内自主的累积发生率，并确定与肠内自主相关的患者和机构特征。
进行了一项来自儿科肠衰竭联盟的多中心回顾性队列分析。IF被定义为婴儿期严重的先天性或后天性胃肠道疾病，依赖肠外营养（PN）超过60天。肠内自主被定义为停用PN超过3个月。
共对272名婴儿进行了中位（四分位间距）33.5（16.2 - 51.5）个月的随访。118名（43%）实现了肠内自主；36名（13%）仍依赖PN，118名（43%）患者死亡或接受了移植。多变量分析确定坏死性小肠结肠炎（NEC；比值比2.42，95%置信区间1.33 - 4.47）、在没有相关肠移植项目的IF中心接受治疗（比值比2.73，95%置信区间1.56 - 4.78）以及完整的回盲瓣（比值比2.80，95%置信区间1.63 - 4.83）是肠内自主的独立危险因素。第二个模型（n = 144）仅纳入术中测量残余小肠长度的患者，发现NEC（比值比3.44，95%置信区间1.36 - 8.71）、在非肠移植中心接受治疗（比值比6.56，95%置信区间2.53 - 16.98）以及残余小肠长度（比值比1.04 cm，95%置信区间1.02 - 1.06 cm）与肠内自主独立相关。
相当一部分IF婴儿可实现肠内自主。潜在的NEC、保留的回盲瓣和较长的肠长度与实现肠内自主相关。机构实践和转诊模式的差异可能也会影响IF患儿的结局。
