接受肠道康复治疗的患者中，肠道衰竭相关肝病的严重程度与死亡率增加相关：一项对儿科肠道衰竭协会数据库的分析。
The extent of intestinal failure-associated liver disease in patients referred for intestinal rehabilitation is associated with increased mortality: an analysis of the Pediatric Intestinal Failure Consortium database.
作者信息
Javid Patrick J, Oron Assaf P, Duggan Christopher P, Squires Robert H, Horslen Simon P
机构信息
Seattle Children's Hospital and University of Washington School of Medicine, Seattle, WA.
Seattle Children's Hospital and University of Washington School of Medicine, Seattle, WA.
出版信息
J Pediatr Surg. 2018 Jul;53(7):1399-1402. doi: 10.1016/j.jpedsurg.2017.08.049. Epub 2017 Sep 5.
BACKGROUND
The advent of regional multidisciplinary intestinal rehabilitation programs has been associated with improved survival in pediatric intestinal failure. Yet, the optimal timing of referral for intestinal rehabilitation remains unknown. We hypothesized that the degree of intestinal failure-associated liver disease (IFALD) at initiation of intestinal rehabilitation would be associated with overall outcome.
METHODS
The multicenter, retrospective Pediatric Intestinal Failure Consortium (PIFCon) database was used to identify all subjects with baseline bilirubin data. Conjugated bilirubin (CBili) was used as a marker for IFALD, and we stratified baseline bilirubin values as CBili<2 mg/dL, CBili 2-4 mg/dL, and CBili>4 mg/dL. The association between baseline CBili and mortality was examined using Cox proportional hazards regression.
RESULTS
Of 272 subjects in the database, 191 (70%) children had baseline bilirubin data collected. 38% and 28% of patients had CBili >4 mg/dL and CBili <2 mg/dL, respectively, at baseline. All-cause mortality was 23%. On univariate analysis, mortality was associated with CBili 2-4 mg/dL, CBili >4 mg/dL, prematurity, race, and small bowel atresia. On regression analysis controlling for age, prematurity, and diagnosis, the risk of mortality was increased by 3-fold for baseline CBili 2-4 mg/dL (HR 3.25 [1.07-9.92], p=0.04) and 4-fold for baseline CBili >4 mg/dL (HR 4.24 [1.51-11.92], p=0.006). On secondary analysis, CBili >4 mg/dL at baseline was associated with a lower chance of attaining enteral autonomy.
CONCLUSION
In children with intestinal failure treated at intestinal rehabilitation programs, more advanced IFALD at referral is associated with increased mortality and decreased prospect of attaining enteral autonomy. Early referral of children with intestinal failure to intestinal rehabilitation programs should be strongly encouraged.
LEVEL OF EVIDENCE
Treatment Study, Level III.
背景
区域性多学科肠道康复项目的出现与小儿肠道衰竭患者生存率的提高相关。然而，肠道康复的最佳转诊时机仍不明确。我们推测肠道康复开始时肠道衰竭相关肝病（IFALD）的程度与总体预后相关。
方法
使用多中心回顾性小儿肠道衰竭联盟（PIFCon）数据库识别所有有基线胆红素数据的受试者。结合胆红素（CBili）用作IFALD的标志物，我们将基线胆红素值分层为CBili<2mg/dL、CBili 2 - 4mg/dL和CBili>4mg/dL。使用Cox比例风险回归分析基线CBili与死亡率之间的关联。
结果
数据库中的272名受试者中，191名（70%）儿童收集了基线胆红素数据。基线时分别有38%和28%的患者CBili>4mg/dL和CBili<2mg/dL。全因死亡率为23%。单因素分析显示，死亡率与CBili 2 - 4mg/dL、CBili>4mg/dL、早产、种族和小肠闭锁相关。在控制年龄、早产和诊断的回归分析中，基线CBili 2 - 4mg/dL时死亡风险增加3倍（HR 3.25[1.07 - 9.92]，p = 0.04），基线CBili>4mg/dL时死亡风险增加4倍（HR 4.24[1.51 - 11.92]，p = 0.006）。二次分析显示，基线CBili>4mg/dL与实现肠内自主的可能性较低相关。
结论
在接受肠道康复项目治疗的肠道衰竭儿童中，转诊时更严重的IFALD与死亡率增加和实现肠内自主的前景降低相关。应强烈鼓励肠道衰竭儿童尽早转诊至肠道康复项目。
证据水平
治疗研究，三级。
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