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Neuropathology and pathophysiology of multiple system atrophy

机译：多系统萎缩的神经病理学和病理生理学

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摘要

The most common indication for pediatric LTx is biliary atresia with failed HPE, yet the effect of previous HPE on the outcome after LTx has not been well characterized. We retrospectively reviewed a single-center experience with 134 consecutive pediatric liver transplants for the treatment of biliary atresia from 1 May 1995 to 28 April 2008. Of 134 patients, 22 underwent LTx without prior HPE (NPE), while 112 patients underwent HPE first. HPE patients were grouped into EF, defined as need for LTx within the first year of life, and LF, defined as need for LTx beyond the first year of life. NPE and EF groups differed significantly from the LF group in age, weight, PELD, and ICU status (p < 0.05) with NPE having the highest PELD and ICU status. Patients who underwent salvage LTx after EF following HPE had a significantly higher incidence of post-operative bacteremia and septicemia (p < 0.05), and subsequently lower survival rates. One-year patient survival and graft survival were as follows: NPE 100%, EF 81%, and LF 96% (p < 0.05); and NPE 96%, EF 79%, and LF 96% (p < 0.05). Further investigation into the optimal treatment of biliary atresia should focus on identifying patients at high risk of EF who may benefit from proceeding directly to LTx given the increased risk of post-LTx bacteremia, sepsis, and death after failed HPE.
机译：儿科LTx最常见的适应症是HPE失败的胆道闭锁症，但以前的HPE对LTx术后预后的影响尚不明确。我们回顾性地回顾了1995年5月1日至2008年4月28日连续134例小儿肝移植治疗胆道闭锁的单中心经验。在134例患者中，有22例未接受过HPE（NPE）的患者接受了LTx治疗，而112例首先接受了HPE。 HPE患者分为EF（定义为生命的第一年内需要LTx）和LF（定义为生命的第一年后需要LTx）。 NPE和EF组的年龄，体重，PELD和ICU状态与LF组有显着差异（p <0.05），其中NPE的PELD和ICU状态最高。 HPE后在EF后接受LTx抢救的患者术后菌血症和败血病的发生率显着较高（p <0.05），随后存活率较低。一年患者存活率和移植物存活率如下：NPE 100％，EF 81％和LF 96％（p <0.05）;和NPE 96％，EF 79％和LF 96％（p <0.05）。胆道闭锁的最佳治疗的进一步研究应集中在确定高EF风险的患者中，考虑到HPE失败后LTx术后菌血症，败血症和死亡的风险增加，可以从直接进行LTx获益。

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