亲体部分肝移植治疗肝豆状核变性  

Study on Living Related Liver Transplantation for Treating Wilson′s Disease in Children

作　　者：陈源[1] 张会丰[1] 冯琳[1] 李根山[1] 

机构地区：[1]河北医科大学第二医院儿科,石家庄050000

出　　处：《实用儿科临床杂志》2006年第8期505-506,共2页Journal of Applied Clinical Pediatrics

摘　　要：目的通过对儿童期肝豆状核变性(WD)1例的亲体部分肝移植术(LRLT)后的肝脏病理学研究,探讨WD患儿LRLT的手术适应证。方法采集WD患儿LRLT后的受体和供体的肝脏标本,进行HE、MASSON、Timm′s、Rubeanic染色。结果WD患儿肝脏HE、MASSON染色表现为不同程度的退行性变,胶原纤维增生明显及典型假小叶形成;Timm′s、Rubeanic染色发现不均匀分布黑色颗粒或团块样物质沉积,处于典型肝硬化阶段,与临床Child Pugh分级存在差异。结论肝脏病理对WD的手术时机选择有重要价值,在肝脏病理支持的基础上,手术指征可适当放宽。Objective To explore the liver pathology from live related liver transplatation （LRLT） of Wilson＇s disease（WD） in children, and evaluatethe indication of LRLT. Methods The sample of this study, including the donater and patient, came from the LRLT. It was observed with HE, MASSON, Timm＇s and Rubeanic staining. Results With HE and MASSON stainning, hepatocyte showed degeneration, fiber hyperplasie and false lobule forrned; Timm＇s and Rubeanic stainning showed typical black deposit of granules and conglomerations. Liver pathology revealed that liver cirrhosis appeared, and it was diffrentent front Child Pugh. Conclusions It is of great significance that the liver pathology is useful for the treatment of WD. If liver pathology supportes, the indication of LRLT can be measurably broadened.

关 键 词：肝豆状核变性 亲体部分肝移植 病理学 儿童 

分 类 号：R726.5[医药卫生—儿科]