Liver transplantation for the treatment of hepatolenticular degeneration

doi:10.3969/j.issn.2095-4344.2013.18.006

Abstract

Abstract:

BACKGROUND: In recent years, the treatment of hepatolenticular degeneration with liver transplantation has achieved great improvement, and a small amount of researches also optimistic reminder that the improvement can be sustained stably. Because of the patients, doctors, and other uncontrollable factors, the recovery of the patients after transplantation is different.
OBJECTIVE: To review the cases with liver transplantation in Department of Hepatobiliary Surgery, Fuzhou General Hospital of Nanjing Military Command, and to long-term follow-up the patients with nerves system damage before transplantation.
METHODS: From July 2005 to May 2010, nine cases (four males and five females) with a mean age of 24.5 years (14-44 years) undergoing liver transplantation for hepatolenticular degeneration were selected from Fuzhou General Hospital of Nanjing Military Command, and the clinical data of the nine patients were retrospective analyzed. All the recipients had low serum levels of ceruloplasmin (200-600 mg/L) before transplantation, and five of them demonstrated the neurological manifestations.
RESULTS AND CONCLUSION: There were eight patients underwent orthotopic liver transplantation and one patient received living donor liver transplantation in this study. Eight recipients recovered satisfactorily with excellent liver functions, while one patient died of multiple organ failure during perioperative period. Compared to the levels before transplantationthe, total bilirubin levels of the eight patients were significantly declined (P < 0.05), the serum levels of albumin, ceruloplasmin and platelet were obviously increased (P < 0.05); the serum copper levels were increased, but the difference was not significant (P > 0.05); the neurological manifestations were also partly relieved or disappeared after liver transplantation. K-P loop was receded in five patients at 1 month after transpantation, and K-P loop was disappeared in two patients at 7 and 11 months after transplantation. Orthotopic liver transplantation or living donor liver transplantation cannot only improve the copper metabolism of the patients with hepatolenticular degeneration, but also can significantly relieve the severe neurological manifestations.

Key words: organ transplantation, liver transplantation, orthotopic liver transplantation, living donor liver transplantation, hepatolenticular degeneration, K-P loop, human copper transporter 1, perioperative period, liver function, Child-Pugh classification, multiple organ dysfunction syndrome, provincial grants-supported paper

CLC Number:

R318

Yu Xiu-tao, Jiang Yi, Zhang Kun, Yang Fang, Cai Qiu-cheng . Liver transplantation for the treatment of hepatolenticular degeneration [J]. Chinese Journal of Tissue Engineering Research, 2013, 17(18): 3267-3274.

Figures/Tables 5

2.5 神经精神症状的改善情况及角膜K-F环变化有神经症状的5例患者中，1例14岁男性患者移植前语言障碍、四肢细震颤，移植后1个月余四肢震颤症状改善。 1例16岁男性患者移植前运动迟缓2年，加重伴右手震颤9个月，表情呆滞、注意力无法集中、智力进行性下降、性格暴躁，时有头晕，右手持物时震颤加重，写字不成形，左手指不自主搓动，肝移植后右手震颤症状减轻，神志清楚。 1例19岁男性患者构音障碍伴四肢震颤1年余，行活体肝移植后7个月左右构音障碍消失，四肢震颤明显好转。 1例44岁女性患者移植前反应迟钝，语速减慢，动作不协调，情绪易变化，双足不自觉的摆动，移植后不自主运动较前好转。 1例38岁女性患者移植前反复手抖10余年，乏力、尿黄20 d余，神志不清2 d。移植后12个月余入院复查，已无手抖症状，神志清楚，对答切题。移植前6例角膜K-F环阳性患者，除1例围手术期死亡外，5例患者角膜K-F环移植后1个月有不同程度变淡，其中2例患者分别于移植后7个月和11个月角膜K-F环消失。 2.6 典型病例资料患者男，17岁，2年前发现行动迟缓、双下肢僵硬及关节疼痛，多处检查未见异常，未行特殊治疗。9个月前，上述症状加重并伴右手震颤，倦怠、无力、食欲减退、表情呆滞、注意力无法集中、智力进行性下降、性格变暴躁，时有头晕，右手持物时震颤加重，写字不成形，左手指不自主搓动，查CT示“轻度脑萎缩”，予“盐酸苯海索+泼尼松”治疗后右手震颤症状有所减轻。激素治疗至入院前1周。现门诊眼科检查见K-F环，为进一步诊治，于2006年6月20日入住解放军南京军区福州总医院肝胆外科。入院检查：神智清楚，行动迟缓，目光呆滞，表情淡漠，反应较迟钝，计算力较差。面部呈满月脸。角膜可见K-F环。右侧肢体肌张力略高，左侧肢体肌张力正常。右上肢轻度细震颤。血常规：血小板 29×109 L-1，余正常。肝功能：总胆红素、丙氨酸转氨酶、谷草转氨酶、谷氨酰转肽酶、碱性磷酸酶、胆碱酯酶均增高，白蛋白34 g/L，血清铜蓝蛋白8.28 mg/L(150-600 mg/L)。HBsAg：阳性。Child-Pugh肝功能分级：B级。腹部彩超示：肝实质弥漫性损害。根据肝豆状核变性的诊断依据：①角膜色素环(K-F环)阳性。②血清铜蓝蛋白降低。③肝功能异常表现。④出现神经系统表现。具以上2项或以上者即可诊断为肝豆状核变性。该患者具备以上4条的临床表现，故可明确诊断为肝豆状核变性。手术方法：入院后经积极的保肝及其他对症处理之后于2006年7月13日在全麻下行“经典式原位肝移植术”。术前常规术野碘酒、乙醇消毒、铺巾。取右上腹部双侧肋缘下“T”切口，探查腹腔：见淡黄色清亮腹水，约200 mL，肝脏萎缩，灰红色、小结节型肝硬化，质地较硬，未扪及肿块，胆囊扩张，腹腔其他脏器未见异常。遂行“原位肝移植术”。供体为脑外伤患者，25岁，身体健康，肝功能及乙肝病毒两对半检测均正常，淋巴毒实验为3%。采用快速取肝法，肾灌注液灌注，UW液保存，热缺血时间约6 min，冷缺血时间约12 h，供肝质地柔软，无肝硬化结节及肿块。术中麻醉理想，手术顺利，渗血较少，输血400 mL。术中标本送病理检查表现为：肝小叶结构破坏，肝细胞内大部分细胞器均流失。肝细胞核无明显异常。窦间隙内枯否氏细胞增生，并见较多炎症细胞浸润。汇管区明显扩大，纤维组织增生，炎症细胞浸润。符合肝豆状核变性的病理表现。病理诊断为肝豆状核变性，见图1。"

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