Andrés Moreno A M, Ramos E, Hernández F, Encinas J L, Leal N, Gámez M L, Martínez L, Sarriá J, Molina M, Martínez-Ojinaga E, Murcia J, Frauca E, Delgado M, Prieto G, López Santamaría M, Tovar J A
Departamento de Cirugía Pediátrica, Sección de Trasplantes Digestivos, Hospital Infantil La Paz, Madrid.
Cir Pediatr. 2010 Jul;23(3):177-83.
To analyze the evolution of Small Bowel Transplantation program since the beginning of the program.
[corrected] All children who underwent intestinal transplantation between 1997 and 2009 were retrospectively reviewed: epidemiological data, status before transplant, surgical technique, immunosupression, results, survival and long.term quality of life were analysed.
Fifty-two intestinal transplants were performed in 46 children (20 isolated bowel, 20 combined liver and intestine, and 12 multivisceral); median age was 32m (range 7m-19a); weight 12,3 kg (range 3,9-60); 31 had short gut syndrome, 8 dismotility, 5 intractable diarrhea, and two were miscellaneous. Intestinal adaptation was initially attempted in 26 patients, without success, 20 were directly listed for transplant. The modality of transplant was modified in 17 while listed. Baseline immunosupression consisted of tacrolimus and steroids, although 5 required conversion to Sirolimus later. Six died during the first month, due to sepsis/multiorganic failure (poor status at transplant); 13 died during the long-term follow-up. Acute rejection was seen in 20, chronic rejection in 3, PTLD in 8 (6 died) and GVHD in 5 patients (3 died). Overall survival after 5 years of follow-up is 65,2 % (51,7% for the graft). From 2006 to 2008, overall patient/graft survival at 6 m, 1 and 3 years after transplant is 88,7/84,1, 81,2/81,2 and 81,2/71,1%, respectively. After a median follw-up of 39 +/- 29 months, 27 patients are alive (59%), off TPN, (70% had their ostomy taken down), go to school, are scarcely hospitalized and enjoy a good quality of life.
Intestinal transplantation has consolided itself as a good choice for irreversible intestinal failure, being feasible to achieve a normal life. Although overall survival diminishes over time, the center experience has improved the results. These patients need a very close follow-up, once transplant is over, in order to get an early diagnose of immunological complications.
分析小肠移植项目自启动以来的发展情况。
对1997年至2009年间接受肠道移植的所有儿童进行回顾性研究:分析流行病学数据、移植前状况、手术技术、免疫抑制、结果、生存率及长期生活质量。
46名儿童接受了52例肠道移植(20例孤立肠移植、20例肝肠联合移植、12例多脏器移植);中位年龄为32个月(范围7个月至19岁);体重12.3千克(范围3.9至60千克);31例患有短肠综合征,8例肠道运动障碍,5例顽固性腹泻,2例情况各异。26例患者最初尝试进行肠道适应性调整但未成功,20例直接列入移植名单。17例在列入名单期间移植方式发生改变。初始免疫抑制方案包括他克莫司和类固醇,不过5例后来需要转换为西罗莫司。6例在第一个月内死亡,原因是败血症/多器官功能衰竭(移植时状况不佳);13例在长期随访中死亡。20例发生急性排斥反应,3例发生慢性排斥反应,8例发生移植后淋巴增殖性疾病(6例死亡),5例发生移植物抗宿主病(3例死亡)。随访5年后总体生存率为65.2%(移植物生存率为51.7%)。2006年至2008年,移植后6个月、1年和3年的总体患者/移植物生存率分别为88.7/84.1、81.2/81.2和81.2/71.1%。中位随访39±29个月后,27例患者存活(59%),停用全胃肠外营养(70%患者的造口已拆除),上学,很少住院,生活质量良好。
肠道移植已成为不可逆肠道衰竭的良好选择,实现正常生活是可行的。尽管总体生存率随时间下降,但该中心的经验改善了结果。这些患者移植后需要密切随访,以便早期诊断免疫并发症。