Lexmond Willem S, Van Dael Carin M L, Scheenstra René, Goorhuis Joanne F, Sieders Egbert, Verkade Henkjan J, Van Rheenen Patrick F, Kömhoff Martin
Division of Pediatric Nephrology, Beatrix Children's Hospital, University Medical Center Groningen, Groningen, the Netherlands.
Liver Transpl. 2015 Mar;21(3):369-80. doi: 10.1002/lt.24037. Epub 2015 Feb 10.
For more than 10 years, children at our national center for pediatric liver transplantation (LT) have been treated with Molecular Adsorbent Recirculating System (MARS) liver dialysis as a bridging therapy to high-urgency LT. Treatment was reserved for 20 patients with the highest degrees of hepatic encephalopathy (HE; median grade = 3.5). Death from neurological sequelae was considered imminent for these patients, and this was further reflected in significantly higher international normalized ratios and ammonia levels and worse prognostic liver indices (Model for End-Stage Liver Disease/Pediatric End-Stage Liver Disease scores and liver injury units) in comparison with 32 wait-listed patients who did not receive MARS dialysis. MARS therapy was generally well tolerated, with a reduction in thrombocytes and hemorrhaging as the most common side effects. HE improvement was documented in 30% of the treated patients, but progression to grade IV encephalopathy occurred in 45% of the patients despite the treatment. Serum ammonia, bilirubin, bile acid, and creatinine levels significantly decreased during treatment. Eighty percent of MARS-treated patients survived to undergo LT, and their survival was equivalent to that of non-MARS-treated patients with severe liver failure (69%, P = 0.52). The heterogeneity between MARS-treated patients and non-MARS-treated patients in our cohort precluded a statistical evaluation of a benefit from MARS for patient survival. Our data demonstrate the safety of MARS even in the most severely ill patients awaiting LT, but strategies that promote the more rapid and widespread availability of high-quality donor organs remain of critical importance for improving patient survival in cases of severe acute liver failure.
十多年来，我们国家儿童肝移植中心的儿童一直接受分子吸附循环系统（MARS）肝透析治疗，作为高紧急度肝移植的桥接治疗。该治疗仅用于20例肝性脑病（HE）程度最高的患者（中位分级=3.5）。这些患者被认为即将死于神经后遗症，这进一步体现在与32例未接受MARS透析的等待名单患者相比，他们的国际标准化比值和氨水平显著更高，预后肝脏指标（终末期肝病/儿童终末期肝病评分和肝损伤单位）更差。MARS治疗一般耐受性良好，最常见的副作用是血小板减少和出血。30%的接受治疗患者的HE有所改善，但尽管接受了治疗，仍有45%的患者进展为IV级脑病。治疗期间血清氨、胆红素、胆汁酸和肌酐水平显著下降。80%接受MARS治疗的患者存活下来接受了肝移植，他们的生存率与未接受MARS治疗的严重肝衰竭患者相当（69%，P=0.52）。我们队列中接受MARS治疗的患者与未接受MARS治疗的患者之间的异质性使得无法对MARS对患者生存的益处进行统计学评估。我们的数据表明，即使在等待肝移植的最重症患者中，MARS也是安全的，但促进高质量供体器官更快、更广泛可得的策略对于改善严重急性肝衰竭病例的患者生存仍然至关重要。
