Kaufman S S, Atkinson J B, Bianchi A, Goulet O J, Grant D, Langnas A N, McDiarmid S V, Mittal N, Reyes J, Tzakis A G
Joint Section of Pediatric Gastroenterology, Creighton University and University of Nebraska Medical Center, Omaha, Nebraska, USA.
Pediatr Transplant. 2001 Apr;5(2):80-7. doi: 10.1034/j.1399-3046.2001.005002080.x.
Parenteral nutrition represents standard therapy for children with short bowel syndrome and other causes of intestinal failure. Most infants with short bowel syndrome eventually wean from parenteral nutrition, and most of those who do not wean tolerate parenteral nutrition for protracted periods. However, a subset of children with intestinal failure remaining dependent on parenteral nutrition will develop life-threatening complications arising from therapy. Intestinal transplantation (Tx) can now be recommended for this select group. Life-threatening complications warranting consideration of intestinal Tx include parenteral nutrition-associated liver disease, recurrent sepsis, and threatened loss of central venous access. Because a critical shortage of donor organs exists, waiting times for intestinal Tx are prolonged. Therefore, it is essential that children with life-threatening complications of intestinal failure and parenteral nutrition therapy be identified comparatively early, i.e. in time to receive suitable donor organs before they become critically ill. Children with liver dysfunction should be considered for isolated intestinal Tx before irreversible, advanced bridging fibrosis or cirrhosis supervenes, for which a combined liver and intestinal transplant is necessary. Irreversible liver disease is suggested by hyperbilirubinemia persisting beyond 3-4 months of age combined with features of portal hypertension such as splenomegaly, thrombocytopenia, or prominent superficial abdominal veins; esophageal varices, ascites, and impaired synthetic function are not always present. Death resulting from complications of liver failure is especially common during the wait for a combined liver and intestinal transplant, and survival following combined liver and intestinal Tx is probably lower than following an isolated intestinal transplant. The incidence of morbidity and mortality following intestinal Tx is greater than that following liver or kidney Tx, but long-term survival following intestinal Tx is now at least 50-60%. It is probable that outcomes shall improve in the future with continued refinements in operative technique and post-operative management, including immunosuppression.
肠外营养是短肠综合征及其他肠道衰竭病因患儿的标准治疗方法。大多数短肠综合征婴儿最终可停用肠外营养，而那些无法停用的婴儿大多能长期耐受肠外营养。然而，一部分依赖肠外营养的肠道衰竭患儿会出现因治疗引发的危及生命的并发症。现在，对于这一特定群体可推荐进行肠道移植（Tx）。需要考虑进行肠道移植的危及生命的并发症包括肠外营养相关肝病、反复败血症以及中心静脉通路面临丧失风险。由于供体器官严重短缺，肠道移植的等待时间延长。因此，必须相对早期地识别出患有肠道衰竭和肠外营养治疗危及生命并发症的患儿，即要及时在他们病情危急之前获得合适的供体器官。对于肝功能不全的患儿，应在不可逆的晚期桥接纤维化或肝硬化出现之前考虑进行孤立性肠道移植，而出现这种情况时则需要进行肝肠联合移植。持续性高胆红素血症超过3 - 4个月龄并伴有门静脉高压特征（如脾肿大、血小板减少或明显的腹壁浅表静脉）提示存在不可逆肝病；食管静脉曲张、腹水和合成功能受损并非总是出现。在等待肝肠联合移植期间，因肝衰竭并发症导致的死亡尤为常见，并且肝肠联合移植后的生存率可能低于孤立性肠道移植。肠道移植后的发病率和死亡率高于肝移植或肾移植，但现在肠道移植后的长期生存率至少为50% - 60%。随着手术技术和术后管理（包括免疫抑制）的不断改进，未来的治疗效果可能会有所改善。
