Bueno J, Ohwada S, Kocoshis S, Mazariegos G V, Dvorchik I, Sigurdsson L, Di Lorenzo C, Abu-Elmagd K, Reyes J
Thomas E. Starzl Transplantation Institute, University of Pittsburgh and Children's Hospital of Pittsburgh Transplantation Surgery, PA 15213, USA.
J Pediatr Surg. 1999 Jan;34(1):27-32; discussion 32-3. doi: 10.1016/s0022-3468(99)90223-3.
The aim of this study was to analyze factors impacting on the survival of pediatric patients with intestinal failure referred for intestinal transplantation (ITx).
Two hundred fifty-seven children (mean age, 3.4+/-0.26 years) with intestinal failure were evaluated for ITx between 1990 and 1998. All patients were dependent on total parenteral nutrition (TPN) for a mean of 31+/-2.7 months. The mean follow-up time from the date of evaluation was 9.2+/-0.9 months.
Eighty-two (32%) children underwent ITx with a mean waiting time of 10.1+/-1.3 months (simultaneous liver-intestinal allograft in 68% instances). Of the 175 patients who did not undergo transplantation, 120 died, 23 were lost to follow-up, and 32 are alive. Younger patients (< or =1 year) had poorer survival rates than patients older than 1 year (P<.0001). The patients with the worse prognosis were those with necrotizing enterocolitis, and those with the best prognosis were those with Hirschsprung's disease. Patients with "surgical" causes had poorer survival rates than those with "nonsurgical" causes (P<.04). Patients with bridging fibrosis or established cirrhosis had an earlier mortality than patients with portal fibrosis (P<.003). The worst survival rate was found for patients with bilirubin levels of greater than 3 mg/dL (P<.0001), plateletcounts less than 100.000/mL (P<.0001), prothrombin time greater than 15 seconds (P = .03) or partial thromboplastin time greater than 40 seconds (P<.04). Children who at the time of evaluation needed only an isolated intestinal allograft had a better prognosis than those who required a combined liver-intestine allograft (P<.00001). With multivariate analysis independent prognosis risk factors of poor outcome were hyperbilirubinemia and severity of histopathologic damage.
Early referral for ITx should occur before the development of liver dysfunction, taking into consideration the aforementioned risk factors that would facilitate the development and ominous evolution to liver failure.
本研究旨在分析影响接受肠移植（ITx）的小儿肠衰竭患者生存的因素。
1990年至1998年间，对257例肠衰竭儿童（平均年龄3.4±0.26岁）进行了肠移植评估。所有患者平均依赖全胃肠外营养（TPN）31±2.7个月。从评估日期起的平均随访时间为9.2±0.9个月。
82例（32%）儿童接受了肠移植，平均等待时间为10.1±1.3个月（68%的病例为同期肝肠联合移植）。在175例未接受移植的患者中，120例死亡，23例失访，32例存活。年龄较小的患者（≤1岁）的生存率低于1岁以上的患者（P<0.0001）。预后较差的患者是患有坏死性小肠结肠炎的患者，预后最好的患者是患有先天性巨结肠的患者。“手术”原因导致的患者生存率低于“非手术”原因导致的患者（P<0.04）。有桥接纤维化或已确诊肝硬化的患者比有门静脉纤维化的患者更早死亡（P<0.003）。胆红素水平大于3mg/dL（P<0.0001）、血小板计数低于100000/mL（P<0.0001）、凝血酶原时间大于15秒（P = 0.03）或部分凝血活酶时间大于40秒（P<0.04）的患者生存率最差。评估时仅需要孤立肠移植的儿童比需要肝肠联合移植的儿童预后更好（P<0.00001）。多因素分析显示，预后不良的独立危险因素是高胆红素血症和组织病理学损伤的严重程度。
考虑到上述易导致肝功能障碍发展及向肝衰竭不良演变的危险因素，应在肝功能障碍出现之前尽早转诊进行肠移植。
