Survival after liver transplantation in patients with hepatic iron overload: The National Hemochromatosis Transplant Registry
Survival after liver transplantation in patients with hepatic iron overload: The National Hemochromatosis Transplant Registry
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DOI：
10.1053/j.gastro.2005.05.004
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发表时间：
2005-08-01
期刊：
影响因子：
29.4
通讯作者：
Polissar, N
中科院分区：
文献类型：
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作者：
Kowdley, KV;Brandhagen, DJ;Polissar, N
背景与目的：之前的非对照研究表明，肝铁超载的患者在肝移植后预后较差。我们研究了 HFE 突变对肝铁超负荷患者移植后生存的影响。方法：纳入来自12个肝移植中心的260例终末期肝病合并肝铁超负荷患者。记录肝铁浓度（HIC）、肝铁指数（HHII）、HFE突变状态和肝移植后的存活率。结果：HFE 相关血色素沉着症 (HH) 定义为 C282Y 纯合性（n = 14，7.2%）突变或 C282Y/H63D 复合杂合性（n = 11，5.6%）突变在 12.8% 的患者中被发现。与单纯杂合子（C282Y/wt 或 H63D/wt）或野生型患者相比，HH 患者肝移植后的存活率显着较低（1 年、3 年和 5 年存活率分别为 64%、48%、34%）。调整年龄、器官共享联合网络状态、移植年份以及升高的 HII 或 HIC 后，HH 患者的死亡风险比为 2.6 (P = 0.002)。与接受肝移植的总体人群相比，肝铁超负荷的非 HH 患者的生存率也显着降低（OR = 1.4，95% Cl：1.15-1.61，P < .001）。结论：HFE 相关 HH 患者肝移植后的 1 年和 5 年生存率显着较低。我们的数据还表明，肝铁超负荷可能与肝移植后生存率降低有关，即使对于没有 HH 的患者也是如此。在肝移植前使用 HFE 基因检测和铁消耗来早期诊断肝铁过载可能会提高移植后生存率，特别是 HH 患者。
Background & Aims: Previous uncontrolled studies have suggested that patients with hepatic iron overload have a poor outcome after liver transplantation. We examined the effect of HFE mutations on post-transplantation survival in patients with hepatic iron overload. Methods: Two hundred sixty patients with end-stage liver disease and hepatic iron overload were enrolled from 12 liver transplantation centers. Hepatic iron concentration (HIC), hepatic iron index (HHII), HFE mutation status, and survival after liver transplantation were recorded. Results: HFE-associated hemochromatosis (HH) defined as homozygosity for the C282Y (n = 14, 7.2%) mutation or compound heterozygosity for the C282Y/H63D (n = 11, 5.6%) mutation was identified in 12.8% of patients. Survival postliver transplantation was significantly lower among patients with HH (1-, 3-, and 5-year survival rates of 64%, 48%, 34%, respectively) compared with simple heterozygotes (C282Y/wt or H63D/wt) or wildtype patients. Patients with HH had a hazard ratio for death of 2.6 (P =.002) after adjustment for age, United Network for Organ Sharing status, year of transplantation, and either elevated HII or HIC. Non-HH patients with hepatic iron overload also had significantly decreased survival when compared with the overall population undergoing liver transplantation (OR = 1.4, 95% Cl: 1.15-1.61, P < .001). Conclusions: One- and 5-year survivals after liver transplantation are significantly lower among patients with HFE-associated HH. Our data also suggest that hepatic iron overload may be associated with decreased survival after liver transplantation, even in patients without HH. Early diagnosis of hepatic iron overload using HFE gene testing and iron depletion prior to liver transplantation may improve posttransplantation survival, particularly among patients with HH.