Liver transplantation for urea cycle disorders in pediatric patients: A single-center experience
Liver transplantation for urea cycle disorders in pediatric patients: A single-center experience
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DOI：
10.1111/petr.12041
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发表时间：
2013-03-01
影响因子：
1.3
通讯作者：
Esquivel, Carlos O.
中科院分区：
文献类型：
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作者：
Kim, Irene K.;Niemi, Anna-Kaisa;Esquivel, Carlos O.
LT 已成为 UCD 的一种手术治疗方法。我们假设 LT 可以安全、广泛地应用于儿科人群，以有效预防高氨血症危机并有可能改善神经认知结果。为了确定 LT 对 UCD 的长期结果，我们对 2001 年 7 月至 2012 年 5 月期间在学术机构接受 LT 的 UCD 儿童的图表进行了回顾性审查。共有 23 名 UCD 患者接受了 LT，平均年龄为 3.4 岁。 15 名患者 (65%) 接受了全肝移植，7 名患者 (30%) 接受了缩小尺寸的移植物，1 名患者接受了活体供体移植物。患者五年平均存活率为 100%，同种异体移植存活率为 96%。就诊时平均峰值血氨 (NH3) 为 772mol/L（中位数 500，范围 1782969，正常
LT has emerged as a surgical treatment for UCDs. We hypothesize that LT can be safely and broadly utilized in the pediatric population to effectively prevent hyperammonemic crises and potentially improve neurocognitive outcomes. To determine the long-term outcomes of LT for UCDs, charts of children with UCD who underwent LT were retrospectively reviewed at an academic institution between July 2001 and May 2012. A total of 23 patients with UCD underwent LT at a mean age of 3.4yr. Fifteen (65%) patients received a whole-liver graft, seven patients (30%) received a reduced-size graft, and one patient received a living donor graft. Mean five-yr patient survival was 100%, and allograft survival was 96%. Mean peak blood ammonia (NH3) at presentation was 772mol/L (median 500, range 1782969, normal