Pediatric Hepatology, Gastroenterology and Transplantation, Ospedali Riuniti di Bergamo, Italy.
J Pediatr Gastroenterol Nutr. 2013 Feb;56(2):118-26. doi: 10.1097/MPG.0b013e318268a9e3.
Intestinal failure (IF) is a condition in which severe intestinal malabsorption mandates artificial nutrition through a parenteral route. Causes of severe protracted IF include short bowel syndrome, congenital diseases of enterocyte development, and severe motility disorders (total or subtotal aganglionosis or chronic intestinal pseudo-obstruction syndrome). IF can result in nutritional failure, defined as the long-term failure to nourish a child by natural or artificial means. Today, IF-associated liver disease is the most common cause of parenteral nutrition (PN) failure, but catheter-related sepsis and extensive vascular thrombosis may also jeopardize the health of those receiving PN. For a child with nutritional failure, intestinal transplantation, often in the form of a composite visceral graft, offers the only chance for long-term survival. The management of IF requires a multidisciplinary approach. There have been a number of recent advances in both medical and surgical treatments of IF. In particular, new intestinal lengthening techniques and the use of PN formulas rich in fish oil both have resulted in decreased rates of severe complications of IF and its treatments. In addition, better awareness of the risks and benefits of intestinal transplantation have resulted in better patient selection, and ultimately in improved patient survival, hence restricting the indication to transplantation only to patients with nutritional failure and no other chance to survive.
肠衰竭（IF）是一种严重的肠道吸收不良病症，需要通过肠外途径进行人工营养。导致严重迁延性 IF 的原因包括短肠综合征、肠细胞发育的先天性疾病和严重的运动障碍（全部或部分无神经节或慢性假性肠梗阻综合征）。IF 可导致营养衰竭，即长期无法通过自然或人工手段滋养儿童。如今，与 IF 相关的肝病是肠外营养（PN）失败的最常见原因，但导管相关败血症和广泛的血管血栓形成也可能危及接受 PN 治疗的患者的健康。对于营养衰竭的儿童，肠移植通常采用复合内脏移植物的形式，是长期生存的唯一机会。IF 的管理需要多学科方法。IF 的医疗和手术治疗都取得了一些新的进展。特别是新的肠道延长技术和富含鱼油的 PN 配方的使用，都降低了 IF 及其治疗的严重并发症的发生率。此外，对肠移植的风险和益处的认识提高，导致了更好的患者选择，最终提高了患者的生存率，因此，仅将移植的适应证限制在营养衰竭且没有其他生存机会的患者。
