Sauvat F, Dupic L, Caldari D, Lesage F, Cezard J P, Lacaille F, Ruemmele F, Hugot J P, Colomb V, Jan D, Hubert P, Revillon Y, Goulet O
UFR Necker-Enfants Malades, University René Descartes Paris V, FAMA de Transplantation Intestinale, AP-HP, 149 Rue de Sèvres, 757015 Paris, France.
Transplant Proc. 2006 Jul-Aug;38(6):1689-91. doi: 10.1016/j.transproceed.2006.05.033.
We evaluated 131 patients (6 months-14 years) who experienced 21 deaths before listing, 11 continuing on the waiting list, 38 well on home parenteral nutrition, 6 off parenteral nutrition and 59 transplanted (20 girls) aged 2.5 to 15 years, (18 >7 years). They received cadaveric isolated intestine (ITx, n = 31) or liver-small bowel (LITx, n = 32), including right colon (n = 43; 23 LITx) for short bowel (n = 19), enteropathy (n = 20), Hirschsprung (n = 14), or pseudo-obstruction (n = 6). Treatment included tacrolimus, steroids, azathioprine, or interleukin-2 blockers. After 6 months to 10.5 years, the patient and graft survivals were 75% and 54%. Sixteen patients (10 LITx) died within 3 months from surgery (n = 3), bacterial (n = 5) or fungal (n = 6) sepsis, or posttransplant lymphoproliferative disorder (n = 2). Rejection occurred in 27 patients, including 10 steroid-resistant episodes requiring antilymphoglobulins. The grafts were removed due to uncontrolled rejection in seven ITx recipients. Surgical complications were observed in 38 recipients (25 LSBTx) within 2 months, including bacterial (n = 22) or fungal (n = 11) sepsis, cytomegalovirus disease (n=12), adenovirus (n = 11), or posttransplant lymphoproliferative disorder (n = 12). Forty-two children (19 LSBTx) are alive. Weaning from parenteral nutrition was achieved after 42 days (median). Factors related to death or graft loss were pre-Tx surgery (P < .01), pseudo-obstruction (P < .01), age over 7 years (P < .03), fungal sepsis (P < .03), steroid resistant rejection (P < .05), hospitalized versus home patient (P < .01), and retransplantation (P < .05). Colon transplant did not affect the outcome. Interleukin-2 blockers improved isolated ITx (P < .05). Early referral and close monitoring of intestinal failure and related disorders are mandatory to achieve successful ITx.
我们评估了131例患者（年龄6个月至14岁），其中21例在列入移植名单前死亡，11例继续在等待名单上，38例接受家庭肠外营养情况良好，6例停用肠外营养，59例接受了移植（20例为女孩），年龄在2.5至15岁之间（18例年龄大于7岁）。他们接受了尸体来源的孤立肠移植（ITx，n = 31）或肝-小肠联合移植（LITx，n = 32），其中包括用于短肠综合征（n = 19）、肠病（n = 20）、先天性巨结肠（n = 14）或假性肠梗阻（n = 6）的右半结肠移植（n = 43；23例为LITx）。治疗包括使用他克莫司、类固醇、硫唑嘌呤或白细胞介素-2阻滞剂。6个月至10.5年后，患者和移植物存活率分别为75%和54%。16例患者（10例为LITx）在术后3个月内死亡，原因包括手术相关（n = 3）、细菌性（n = 5）或真菌性（n = 6）败血症或移植后淋巴细胞增殖性疾病（n = 2）。27例患者发生排斥反应，其中10例为类固醇抵抗性发作，需要使用抗淋巴细胞球蛋白治疗。7例ITx受者因无法控制的排斥反应而移除移植物。38例受者（25例为LITx）在术后2个月内出现手术并发症，包括细菌性（n = 22）或真菌性（n = 11）败血症、巨细胞病毒病（n = 12）、腺病毒感染（n = 11）或移植后淋巴细胞增殖性疾病（n = 12）。42例儿童（19例为LITx）存活。肠外营养在术后42天（中位数）后停用。与死亡或移植物丢失相关的因素包括移植前手术（P < .01）、假性肠梗阻（P < .01）、年龄大于7岁（P < .03）、真菌性败血症（P < .03）、类固醇抵抗性排斥反应（P < .05）、住院患者与家庭患者（P < .01）以及再次移植（P < .05）。结肠移植不影响预后。白细胞介素-2阻滞剂可改善孤立ITx的预后（P < .05）。对于成功进行ITx而言，早期转诊以及对肠衰竭和相关疾病进行密切监测是必不可少的。
