Parenteral nutrition associated liver disease

Stuart S Kaufman , Gabriel E Gondolesi , Thomas M Fishbein
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引用次数: 43

Abstract

Liver disease is relatively common during parenteral nutrition (PN). Cholestasis predominates in infants, and ranges in severity from mild increases in plasma conjugated bilirubin to progressive liver failure that results in death of the patient. Severity of liver disease depends primarily on the magnitude of the underlying intestinal problem that indicated PN. Transient ileus resulting from a non-intestinal disorder usually results in trivial, self-limited liver injury. Removal of a large segment of the intestinal tract because of necrotizing enterocolitis or a congenital malformation predicts a more prolonged course with a guarded prognosis, particularly when initially complicated by sepsis. Pathogenesis of PN-associated liver disease is not completely understood. There is no proven treatment short of ending PN through adaptation of remnant intestine or intestinal transplantation, with or without a concurrent liver graft. Effective interventions that are less radical than transplantation are needed. Research that includes prospective trials of novel therapies in PN-associated liver disease is the key to improving outcome.

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肠外营养相关的肝病
肝脏疾病是相对常见的肠外营养(PN)。胆汁淤积症主要发生在婴儿身上,其严重程度从血浆结合胆红素轻度升高到导致患者死亡的进行性肝功能衰竭不等。肝脏疾病的严重程度主要取决于显示PN的潜在肠道问题的严重程度。由非肠道疾病引起的短暂性肠梗阻通常会导致轻微的自限性肝损伤。由于坏死性小肠结肠炎或先天性畸形而切除一大块肠道预示着更长的病程和谨慎的预后,特别是当最初并发败血症时。PN-associated肝脏疾病的发病机制并不完全理解。目前还没有证实的治疗方法可以通过残肠适应或肠道移植来结束PN,同时或不同时进行肝移植。有效的干预措施,那么激进的移植是必要的。包括对pn相关肝病新疗法的前瞻性试验在内的研究是改善预后的关键。
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