Ozçay Figen, Canan Oğuz, Bilezikçi Banu, Torgay Adnan, Karakayali Hamdi, Haberal Mehmet
Department of Pediatric Gastroenterology, Hepatology, and Nutrition, Baskent University Faculty of Medicine, Ankara, Turkey.
Clin Transplant. 2006 Nov-Dec;20(6):776-82. doi: 10.1111/j.1399-0012.2006.00571.x.
We described six children with heritable liver disease and hepatocellular carcinoma treated with living-related liver transplantation. Underlying liver diseases were type-1 tyrosinemia (three patients), progressive familial intrahepatic cholestasis type II (two patients), and Wilson's disease (one patient). Two of the tumors were found incidentally during liver transplantation. Number of nodules was 12, 15, 3, 2, and 1 (in two patients). Three patients were treated with chemotherapy before the procedure. Chemotherapy was not given to any patient after liver transplantation. The mean follow-up was 17.7 +/- 6 months (range: 7-24). All patients are tumor recurrence free. Both graft and patient survival rates are 100% at a median of 18.5 months follow-up. Physicians in charge of treating children with heritable liver disease should screen them periodically for the development of hepatocellular carcinoma. Liver transplantation may offer these children better survival rates.
我们描述了6例患有遗传性肝病并接受活体亲属肝移植治疗肝细胞癌的儿童。潜在的肝脏疾病为1型酪氨酸血症（3例患者）、进行性家族性肝内胆汁淤积症II型（2例患者）和威尔逊病（1例患者）。其中2例肿瘤是在肝移植期间偶然发现的。结节数量分别为12个、15个、3个、2个和1个（2例患者）。3例患者在手术前接受了化疗。肝移植后未对任何患者进行化疗。平均随访时间为17.7±6个月（范围：7 - 24个月）。所有患者均无肿瘤复发。在中位随访18.5个月时，移植物和患者生存率均为100%。负责治疗遗传性肝病儿童的医生应定期对他们进行肝细胞癌发生情况的筛查。肝移植可能为这些儿童提供更高的生存率。
