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肠外营养相关性胆汁淤积症（PNAC）是接受肠外营养治疗时间超过2周新生儿常见且严重的并发症。PNAC的发生可能是多因素作用的结果。早产、低出生体重、长期肠外营养、缺乏肠道喂养、动脉导管未闭、坏死性小肠结肠炎、肠道细菌过度生长、细菌移位以及肠外营养液组分失衡或含有有毒成分等均是新生儿PNAC的危险因素。PNAC的发生机制尚不明确，可能与胆汁肠肝循环的改变、肠道菌群移位、脂肪乳剂使用不当、氨基酸和碳水化合物使用不当、微量元素过量等有关。PNAC的预防主要包括早期开展肠内营养、优化肠外营养组分、营养液使用全程避光等。针对PNAC的治疗方法有限，可以考虑使用熊去氧胆酸、红霉素、苯巴比妥及利福平等药物。“,”Parenteral nutrition-associated cholestasis (PNAC) is a common and serious complication of newborns receiving parenteral nutrition support for more than 2 weeks. The occurrence of PNAC may be the result of multiple factors. Premature birth, low birth weight, long-term parenteral nutrition, lack of enteral feeding, patent ductus arteriosus, necrotizing enterocolitis, intestinal bacterial overgrowth, bacterial translocation, and imbalance of parenteral nutrition components or containing toxic ingredients are all risk factors of neonatal PNAC. The mechanism of PNAC is not clear, which may be related to the change of bile salt enterohepatic circulation, intestinal flora translocation, improper use of fat emulsions, improper use of amino acids and carbohydrate, and excessive trace elements. The prevention measurements of PNAC mainly include early enteral feeding, optimization of parenteral nutrition components, light protection during the use of nutrient solution, and so on. The therapeutic strategies of PNAC are limited, drugs such as ursodeoxycholic acid, erythromycin, phenobarbital, and rifampicin can be considered.