Gupte Girish L, Beath Susan V, Protheroe Sue, Murphy M Stephen, Davies Paul, Sharif Khalid, McKiernan Patrick J, de Ville de Goyet Jean, Booth Ian W, Kelly Deirdre A
Liver Unit, Birmingham Children's Hospital, Birmingham, UK.
Arch Dis Child. 2007 Feb;92(2):147-52. doi: 10.1136/adc.2005.090068. Epub 2006 May 16.
To describe the outcome of children with intestinal failure referred to Birmingham Children's Hospital (BCH) for consideration of intestinal transplantation (ITx), to determine factors for an adverse outcome and to analyse the impact of post-1998 strategies on survival.
A retrospective analysis was performed of children referred for ITx assessment from January 1989 to December 2003. Children were assessed by a multidisciplinary team and categorised into: (a) stable on parenteral nutrition; (b) unsuitable for transplantation (Tx); and (c) recommended for Tx. To analyse the impact of the post-1998 strategies on survival, a comparison was made between the two eras (pre-1998 and post-1998).
152 children with chronic intestinal failure were identified (63M:89F, median age 10 months (range 1-170)). After assessment, 69 children were considered stable on parenteral nutrition (5-year survival 95%); 28 children were unsuitable for Tx (5-year survival 4%); and 55 children were recommended for Tx (5-year survival 35%, which includes 14 children who died waiting for size-matched organs). Twenty three ITx and nine isolated liver transplants (iLTx) were performed. In a multivariate analysis, the following factors in combination had an adverse effect on survival: the presence of a primary mucosal disorder (p = 0.007, OR ratio 3.16, 95% CI 1.37 to 7.31); absence of involvement of a nutritional care team at the referring hospital (p = 0.001, OR ratio 2.55, 95% CI 1.44 to 4.52); and a serum bilirubin>100 micromol/l (p = 0.001, OR ratio 3.70, 95% CI 1.84 to 7.47). Earlier referral (median serum bilirubin 78 micromol/l in the post-1998 era compared with 237 micromol/l in the pre-1998 era, p = 0.001) may be a contributory factor to improved survival. The strategies of combined en bloc reduced liver/small bowel transplantation and iLTx resulted in fewer deaths on the waiting list in the post-1998 era (2 deaths in post-1998 era v 12 deaths in pre-1998 era). The overall 3-year survival in the post-1998 era (69%) has improved compared with the pre-1998 era (31%; p<0.001) CONCLUSION: The changing characteristics at the time of referral, including earlier referral and innovative surgical strategies have resulted in improved long-term survival of children referred for ITx.
描述转诊至伯明翰儿童医院(BCH)考虑进行肠移植(ITx)的儿童的治疗结果,确定不良预后的因素,并分析1998年后策略对生存率的影响。
对1989年1月至2003年12月转诊进行ITx评估的儿童进行回顾性分析。由多学科团队对儿童进行评估,并分为:(a)肠外营养状况稳定;(b)不适合移植(Tx);(c)建议进行Tx。为分析1998年后策略对生存率的影响,对两个时期(1998年前和1998年后)进行了比较。
共确定152例慢性肠衰竭儿童(男63例,女89例,中位年龄10个月(范围1 - 170个月))。评估后,69例儿童被认为肠外营养状况稳定(5年生存率95%);28例儿童不适合Tx(5年生存率4%);55例儿童建议进行Tx(5年生存率35%,其中包括14例在等待合适大小器官时死亡的儿童)。进行了23例ITx和9例孤立肝移植(iLTx)。多因素分析显示,以下因素综合起来对生存率有不利影响:存在原发性黏膜疾病(p = 0.007,比值比3.16, 95%可信区间1.37至7.31);转诊医院没有营养护理团队参与(p = 0.001,比值比2.55, 95%可信区间1.44至4.52);血清胆红素>100 μmol/L(p = 0.001,比值比3.70, 95%可信区间1.84至7.47)。更早转诊(1998年后时期中位血清胆红素为78 μmol/L,而1998年前时期为237 μmol/L,p = 0.001)可能是生存率提高的一个促成因素。1998年后时期采用的整块切除联合肝/小肠移植和iLTx策略使等待名单上的死亡人数减少(1998年后时期2例死亡,1998年前时期12例死亡)。与前1998年时期(31%;p<0.001)相比,1998年后时期的总体3年生存率(69%)有所提高。结论:转诊时特征的变化,包括更早转诊和创新的手术策略,已使转诊进行ITx的儿童长期生存率得到提高。