Changing the Paradigm: Omegaven for the Treatment of Liver Failure in Pediatric Short Bowel Syndrome
Changing the Paradigm: Omegaven for the Treatment of Liver Failure in Pediatric Short Bowel Syndrome
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DOI：
10.1097/mpg.0b013e318182c8f6
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发表时间：
2009-02-01
影响因子：
2.9
通讯作者：
Wales, Paul W.
中科院分区：
文献类型：
--
作者：
Diamond, Ivan R.;Sterescu, Anca;Wales, Paul W.
背景：肠外omega-3脂肪酸，如Omegaven，可能使患有肠外营养相关性肝病（PNALD）的儿童短肠综合征（SBS）患者受益。患者和方法：回顾性队列研究，描述了所有12名接受Omegaven治疗的SBS和晚期PNALD儿童的结果（目标omega-6与omega-3脂肪酸比例= 1:1至2:1）。结果：中位年龄为7.5个月（3.6-46个月），开始使用欧米茄文前的中位肠外营养持续时间为28.4周（15.3-55.3周）。初始血清结合胆红素中位数为137（范围54-203）μ mol/L （8.06 [3.18-11.94] mg/dL）。在12例患者中，9例高胆红素血症在中位24周（范围7-37周）内完全持续消退，所有患者不再考虑肝移植。在这些患者中，肝脏炎症标志物和营养状况也有所改善。三名患者在服用omega - aven的同时接受了肝肠移植。没有并发症可归因于omega - aven。结论：欧米茄文与SBS和晚期肝病患者肝功能恢复相关。肠外omega-3脂肪酸，如Omegaven，有可能从根本上改变新生儿SBS的模式，从早期死亡或肝衰竭移植到更慢性的疾病。更多患有SBS的儿童应该达到完全的肠内耐受，那些不具备肠道适应能力的儿童应该能够存活下来，并在长大后接受肠道移植。[j] .中文信息学报，2009。
Background: Parenteral omega-3 fatty acids, such as Omegaven, may benefit patients with pediatric short bowel syndrome (SBS) who develop parenteral nutrition-associated liver disease (PNALD).Patients and Methods: Retrospective cohort describing the outcome of all 12 children with SBS and advanced PNALD who were treated with Omegaven (target omega-6 to omega-3 fatty acid ratio = 1:1 to 2:1).Results: The median age was 7.5 (range 3.6-46) months, and median parenteral nutrition duration before starting Omegaven was 28.4 (range 15.3-55.3) weeks. Median initial serum conjugated bilirubin was 137 (range54-203) mu mol/L (8.06 [3.18-11.94] mg/dL). Of the 12 patients, 9 had complete and sustained resolution of hyperbilirubinemia within a median of 24 (range 7-37) weeks, and all are no longer being considered for liver transplantation. Improvements in markers of hepatic inflammation as well as nutritional status also were noted in these patients. Three patients received a liver-intestine transplant while taking Omegaven. There were no complications attributable to Omegaven.Conclusions: Omegaven is associated with restoration of liver function in patients with SBS and advanced liver disease. Parenteral omega-3 fatty acids, such as Omegaven, have the potential to fundamentally alter the paradigm of neonatal SBS from one of early death or transplantation from liver failure to a more chronic disease. More children with SBS should achieve full enteral tolerance and those who do not have the capacity for intestinal adaptation should be able to Survive and receive an intestinal graft when older. JPGN 48:209-215, 2009.