Nayyar Navdeep, Mazariegos George, Ranganathan Sarangarajan, Soltys Kyle, Bond Geoffrey, Jaffe Ronald, Sun Qing, Nucci Anita, Kosmach Beverly, Squires Robert, Abu-Elmagd Kareem, Sindhi Rakesh
Hillman Center for Pediatric Transplantation, Children's Hospital of Pittsburgh, University of Pittsburgh, Pittsburgh, Pennsylvania, USA.
Semin Pediatr Surg. 2010 Feb;19(1):68-77. doi: 10.1053/j.sempedsurg.2009.11.009.
The multivisceral liver-intestine-pancreas-stomach allograft was first described by Starzl nearly 50 years ago. Since then, over 1000 children have received small bowel transplantation (SBTx), alone or with the liver and other organs, for refractory short gut syndrome (SGS) because of a variety of congenital conditions. In 2001, SBTx was approved as definitive therapy for SGS by Medicare. Currently, 1- and 5-year graft survival routinely exceeds 90% and 80%, respectively. The expected outcomes also include freedom from parenteral nutrition, normalization of growth parameters, and quality of life. However, recurrent rejection, complications of high-dose immunosuppression, or chronic rejection, which is more likely to occur after SBTx without a liver graft, account for differences between early and late survival. Future efforts aimed at overcoming such challenges include preventing SBTx through early referral to comprehensive SGS management programs and understanding why the liver protects the small bowel allograft from rejection. Finally, inflammatory mechanisms, which predispose the highly immunogenic small bowel allograft to a protracted risk of resistant rejection must be elucidated, in order to ensure durable success.
多脏器肝-肠-胰-胃移植最早由斯塔兹于近50年前描述。从那时起，超过1000名儿童因各种先天性疾病导致的难治性短肠综合征（SGS）接受了单独或联合肝脏及其他器官的小肠移植（SBTx）。2001年，SBTx被医疗保险批准为难治性短肠综合征的确定性治疗方法。目前，1年和5年移植物存活率通常分别超过90%和80%。预期结果还包括摆脱肠外营养、生长参数正常化和生活质量提高。然而，复发排斥反应、大剂量免疫抑制的并发症或慢性排斥反应（在未进行肝移植的小肠移植后更易发生）是早期和晚期存活率存在差异的原因。未来旨在克服此类挑战的努力包括通过早期转诊至综合难治性短肠综合征管理项目来预防小肠移植，以及了解肝脏为何能保护小肠移植物免受排斥。最后，必须阐明炎症机制，这种机制使具有高度免疫原性的小肠移植物面临持续的抵抗性排斥风险，以确保持久成功。
