Huang Yu, Takatsuki Mitsuhisa, Soyama Akihiko, Hidaka Masaaki, Ono Shinichiro, Adachi Tomohiko, Hara Takanobu, Okada Satomi, Hamada Takashi, Eguchi Susumu
Department of Surgery, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki City, Nagasaki, Japan.
Department of Hepatobiliary Surgery, Guangzhou First People's Hospital, Guangzhou Medical University, Guangzhou, Guangdong, China (mainland).
Am J Case Rep. 2018 Mar 17;19:304-308. doi: 10.12659/ajcr.907494.
BACKGROUND Liver transplantation is indicated for patients with Wilson's disease (WD) who present either with acute liver failure or with end-stage liver disease and severe hepatic insufficiency as the first sign of disease. However, almost all reported cases have been treated with death donor liver transplantation. Here we report the case of a patient with WD associated with fulminant hepatic failure (WD-FHF) who underwent living donor liver transplantation (LDLT). CASE REPORT A 17-year-old female was diagnosed with WD-FHF based on high uric copper (10 603 μg/day, normal <100 μg/day), low serum ceruloplasmin (15 mg/dL, normal >20 mg/dL) and Kayser-Fleischer (K-F) corneal ring, and acute liver failure (ALF), acute renal failure (ARF) and grade 2 hepatic encephalopathy (HE). The model for end-stage liver disease (MELD) score was 35. Due to her critical condition, the patient underwent LDLT utilizing a right liver graft from her 44-year-old mother. The right hepatic vein (RHV) and inferior right hepatic vein (iRHV) were reconstructed. She developed severe liver dysfunction due to a crooked hepatic vein caused by compression from the large graft. To straighten the bend, a reoperation was performed. During the operation, we tried to relieve the compressed hepatic vein by adjusting the graft location, but the benefits were limited. We therefore performed stenting in both the RHV and iRHV on postoperative day 9. The patient gradually improved, exhibiting good liver and renal functions, and was finally discharged on postoperative day 114. CONCLUSIONS When WD-FHF deteriorates too rapidly for conservative management, LDLT is an effective therapeutic strategy.
背景
对于以急性肝衰竭或终末期肝病及严重肝功能不全为疾病首发表现的威尔逊病（WD）患者，肝移植是一种治疗选择。然而，几乎所有报道的病例均接受了死体供肝肝移植。在此，我们报告一例WD合并暴发性肝衰竭（WD-FHF）患者接受活体供肝肝移植（LDLT）的病例。
病例报告
一名17岁女性，根据高尿铜（10603μg/天，正常<100μg/天）、低血清铜蓝蛋白（15mg/dL，正常>20mg/dL）及凯-弗（K-F）角膜环，以及急性肝衰竭（ALF）、急性肾衰竭（ARF）和2级肝性脑病（HE），被诊断为WD-FHF。终末期肝病模型（MELD）评分35分。鉴于其病情危急，患者接受了来自其44岁母亲的右肝活体肝移植。重建了右肝静脉（RHV）和右下肝静脉（iRHV）。由于大的移植肝压迫导致肝静脉扭曲，她出现了严重的肝功能障碍。为了矫正弯曲，进行了再次手术。手术过程中，我们试图通过调整移植肝位置来缓解受压的肝静脉，但效果有限。因此，我们在术后第9天对RHV和iRHV均进行了支架置入。患者逐渐好转，肝功能和肾功能良好，最终于术后第114天出院。
结论
当WD-FHF病情进展过快无法进行保守治疗时，LDLT是一种有效的治疗策略。
