Department of Gastroenterology, Lahey Clinic Medical Center, Burlington, Massachusetts, USA.
JPEN J Parenter Enteral Nutr. 2013 Mar;37(2):274-80. doi: 10.1177/0148607112450301. Epub 2012 Jun 8.
Patients with intestinal failure and short bowel syndrome usually require chronic parenteral nutrition (PN). PN is associated with risks, including infections, vascular thrombosis, and liver disease. PN-associated liver disease (PNALD) can progress from steatosis to chronic hepatitis and ultimately to cirrhosis. The etiology of PNALD is not completely understood. Therapies for PNALD include carbohydrate or lipid calorie reduction, antibiotics, or the use of ursodeoxycholic acid. When these efforts fail, therapeutic options are limited and liver transplantation may be required. The transition from a soybean- to a fish oil-based lipid formulation, such as the ω-3 parenteral lipid formulation (Omegaven), has shown a dramatic reversal of PNALD within the pediatric population. This is the first report of a PN-dependent adult in the United States complicated by PNALD and hepatic failure who had improvement of liver disease with an ω-3 fish oil-based parenteral formulation.
肠衰竭和短肠综合征患者通常需要长期接受肠外营养(PN)。PN 相关风险包括感染、血管血栓形成和肝病。PN 相关肝病(PNALD)可从脂肪变性进展为慢性肝炎,最终发展为肝硬化。PNALD 的病因尚不完全清楚。PNALD 的治疗包括减少碳水化合物或脂肪热量、使用抗生素或熊去氧胆酸。当这些方法都失败时,治疗选择有限,可能需要进行肝移植。从大豆油基到鱼油基的脂质配方(如ω-3 肠外脂质配方(Omegaven))的转变,在儿科人群中已显示出 PNALD 的显著逆转。这是首例美国以 PN 为基础的成人病例报告,该患者因 PNALD 和肝衰竭而复杂化,用 ω-3 鱼油基肠外配方治疗后肝病得到改善。