Departments of Clinical Pharmacy and Pediatrics, The University of Tennessee Health Science Center and Le Bonheur Children's Hospital, State of Tennessee Center of Excellence in Pediatric Pharmacokinetics and Therapeutics, Children's Foundation Research Institute at Le Bonheur Children's Hospital, 50 N Dunlap, Memphis, TN 38103, USA.
Nutr Clin Pract. 2013 Feb;28(1):30-9. doi: 10.1177/0884533612462900. Epub 2012 Oct 19.
Parenteral nutrition-associated liver disease (PNALD) is a complex disease that is diagnosed by clinical presentation, biochemical markers of liver injury, concurrent use of parenteral nutrition (PN), and negative workup for other causes of liver disease. Since the first case of PNALD was reported more than 30 years ago, clinicians have had few effective treatments for PNALD, and when disease progressed to liver cirrhosis, it was historically associated with poor outcomes. Within the past 5 years, there has been much excitement about new treatments for PNALD, including use of both parenteral and enteral ω-3 polyunsaturated long-chain fatty acids (ω-3 PUFA) as well as restricting dosing of ω-6 PUFA. Scientists are also interested in uncovering the mechanisms associated with liver injury seen in PNALD. This article reviews the recent literature relating to the pathophysiology and treatment of PNALD.
肠外营养相关性肝病（PNALD）是一种复杂的疾病，通过临床表现、肝损伤的生化标志物、肠外营养（PN）的同时使用以及对其他肝病病因的阴性检查来诊断。自 30 多年前首例 PNALD 病例报告以来，临床医生对 PNALD 几乎没有有效的治疗方法，当疾病进展到肝硬化时，其预后历来较差。在过去的 5 年中，人们对 PNALD 的新治疗方法产生了浓厚的兴趣，包括使用肠外和肠内ω-3 多不饱和长链脂肪酸（ω-3 PUFA）以及限制ω-6 PUFA 的剂量。科学家们也对揭示与 PNALD 中所见肝损伤相关的机制感兴趣。本文综述了与 PNALD 的病理生理学和治疗相关的最新文献。
