Intestinal failure-associated liver disease: What do we know today?
Intestinal failure-associated liver disease: What do we know today?
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DOI：
10.1053/j.gastro.2005.10.066
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发表时间：
2006-02-01
期刊：
影响因子：
29.4
通讯作者：
Kelly, DA
中科院分区：
文献类型：
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作者：
Kelly, DA
40%至60%需要长期全面肠外营养（TPN）治疗肠衰竭的婴儿和15%至40%需要家庭肠外营养的成年人发生肠衰竭相关肝病。临床表现包括肝脂肪变性、胆汁淤积、胆石症和肝纤维化。进展为胆汁性肝硬化和发展为门脉高压和肝功能衰竭发生在少数，但在婴儿和新生儿中比在成人中更常见。发病机制是多因素的。在婴儿中，它与早产、低出生体重、PN持续时间、需要多次剖腹手术的短肠综合征和复发性败血症有关。其他重要机制包括缺乏肠内喂养，导致肠道激素分泌减少；胆汁流量减少，胆汁淤积，导致胆汁淤积；胆汁淤积和胆结石会加重肝功能障碍。在成人中，IFALD不太常见，与年龄、PN时间长短、总热量摄入以及脂质或葡萄糖过载有关。在早产儿中，牛磺酸或半胱氨酸的缺乏可能起作用，而在成人和儿童中，胆碱缺乏可能加剧IFALD。脂质乳剂、胆碱缺乏和锰毒性与成人和儿童的肝脂肪变性和胆汁淤积有关。预防肠衰竭引起的肝脏疾病的管理策略包括早期肠内喂养，多学科的肠外营养管理方法，以及减少败血症的无菌导管技术。在PN溶液中添加胆碱、牛磺酸和半胱氨酸也可能起作用。口服熊去氧胆酸可改善胆汁流动，减少胆囊淤积。孤立小肠移植或肝小肠联合移植后的5年生存率约为50%，使其成为成人和儿童不可逆肝和肠功能衰竭的可接受的治疗选择。
Intestinal failure-associated liver disease develops in 40% to 60% of infants who require long-term total parenteral nutrition (TPN) for intestinal failure and 15% to 40% of adults on home parenteral nutrition. The clinical spectrum includes hepatic steatosis, cholestasis, cholelithiasis, and hepatic fibrosis. Progression to biliary cirrhosis and the development of portal hypertension and liver failure occurs in a minority but is more common in infants and neonates than in adults. The pathogenesis is multifactorial. In infants it is related to prematurity, low birth weight, duration of PN, short bowel syndrome requiring multiple laparotomies, and recurrent sepsis. Other important mechanisms include lack of enteral feeding, which leads to reduced gut hormone secretion; reduction of bile flow and biliary stasis, which leads to the development of cholestasis; and biliary sludge and gallstones, which exacerbate hepatic dysfunction. In adults, IFALD is less common and related to age, length of time on PN, total caloric intake, and lipid or glucose overload. In preterm infants, a deficiency of taurine or cysteine may play a role, whereas in both adults and children, choline deficiency may exacerbate IFALD. Lipid emulsions, choline deficiency, and manganese toxicity are associated with both hepatic steatosis and cholestasis in adults and children. Management strategies for the prevention of intestinal failure-induced liver disease include early enteral feeding, a multidisciplinary approach to the management of parenteral nutrition, and aseptic catheter techniques to reduce sepsis. The addition of choline, taurine, and cysteine to PN solutions may also play a role. Oral administration of ursodeoxycholic acid may improve bile flow and reduce gallbladder stasis. Survival after either isolated small bowel or combined liver and small bowel transplantation is approximately 50% at 5 years, making this an acceptable therapeutic option in adults and children with irreversible liver and intestinal failure.