Plasma exchange and chelator therapy rescues acute liver failure in Wilson disease without liver transplantation.
Plasma exchange and chelator therapy rescues acute liver failure in Wilson disease without liver transplantation.
复制标题
血浆置换和螯合剂治疗无需肝移植即可挽救威尔逊病的急性肝功能衰竭。
DOI：
10.1111/hepr.12711
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发表时间：
2017
期刊：
影响因子：
--
通讯作者：
Nakamura K
中科院分区：
文献类型：
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作者：
Kido J;Matsumoto S;Momosaki K;Sakamoto R;Mitsubuchi H;Inomata Y;Endo F;Nakamura K
目的新威尔逊指数（NWI）评分≥ 11分的肝豆状核变性（WD）患者是致命的，这些患者是肝移植（LT）的良好候选者。然而，即使对于评分≥ 11的WD，血浆置换和螯合剂治疗也是必不可少的且有效的。此外，这些治疗的连续血液透析滤过（CHDF）对于WD伴肝性脑病的急性肝衰竭（ALF）至关重要，因为CHDF可以排除可能导致脑损伤的有毒代谢物。在这里，我们描述了四个获救的患者提出ALF在WD和讨论可用的治疗选项。MethodsWe经历了11名男性和8名女性患者提出WD在儿科，熊本大学医院1999年和2014年之间。1例男性和4例女性患者被诊断为WD与ALF使用的临床表现和生化tests.ResultsNWI评分≥ 11例1至3。对1、2例肝性脑病患者行血浆置换、CHDF、凝血因子替代治疗（CFRT）和LT，对3、4例无脑病患者行血浆置换、输血和CFRT后病情稳定，无LT。无肝性脑病的WD患者，NWI评分≥ 11分者，可采用保守治疗。因此，ALF合并肝性脑病本身是WD LT的适应症。
AimWilson disease (WD) in patients with a New Wilson Index (NWI) score ≥ 11 is fatal, and these patients are good candidates for liver transplantation (LT). However, plasma exchange and chelator therapy are indispensable and effective even for WD with a score ≥ 11. Moreover, continuous hemodiafiltration (CHDF) with these treatments is essential for acute liver failure (ALF) in WD with hepatic encephalopathy because CHDF can exclude toxic metabolites that may cause damage to the brain. Here, we describe four rescued patients presenting with ALF in WD and discuss the available treatment options.MethodsWe have experienced 11 male and 8 female patients presenting with WD at the Department of Pediatrics, Kumamoto University Hospital between 1999 and 2014. A male and 4 female patients were diagnosed as WD with ALF using a combination of clinical findings and biochemical tests.ResultsThe NWI score was ≥ 11 in cases 1 to 3. Cases 1 and 2 with hepatic encephalopathy received plasma exchange, CHDF, coagulation factor replacement treatment (CFRT) and LT. Cases 3 and 4 without encephalopathy obtained stable status without LT by plasma exchange, blood infusion, and CFRT.ConclusionsIt is better to undergo LT for WD patients with a NWI score ≥ 11, however, there is a possibility of remission by plasma exchange and medical therapy even without LT. WD patients with a NWI score ≥ 11can be rescued by conservative therapy when the ALF of WD does not present with ALF and hepatic encephalopathy. Therefore, ALF with hepatic encephalopathy itself is an indication for LT in WD.