肝移植术后胆道铸型综合征的临床表现与病理分型

Clinical manifestation and patho-typing of biliary cast syndrome in patients after othotopic liver transplantation

目的总结肝移植术后胆道铸型综合征（BCS）的病理分型与临床表现。方法回顾性分析103例肝移植术后BCS患者的临床表现、辅助检查、治疗方案及转归。患者通过纤维胆道镜检查，按照胆道上皮损伤程度分出不同类型。全部病例随访12个月，统计各组非吻合口狭窄的发生时间、部位及程度。结果103例BCS患者中，武警总医院发生59例，该院总BCS发生率为9．1％。BCS多数有黄疸、尿色深、陶土色大便、皮肤瘙痒及发热等临床症状；部分无临床症状。化验指标可表现为谷氨酸转氨酶、γ-谷氨酰转肽酶、碱性磷酸酶、总胆红素、直接胆红素等升高，外周血白细胞总数升高等。胆道造影可表现为胆道树不同程度的充盈缺损，按照胆道树的形态及充盈缺损的范围，将造影结果归纳为4种类型。纤维胆道镜检查可见固体物质充填于胆道，部分患者伴有胆道上皮坏死。根据胆道镜检查按胆道上皮坏死范围将BCS由轻到重分为6种类型：Ⅰ型14例，Ⅱ型18例，Ⅲ型27例，Ⅳ型23例，Ⅴ型13例，Ⅵ型18例。从Ⅰ型至Ⅳ型患者出现临床症状及胆道狭窄概率逐渐增加，取出BC后需支撑时间逐渐延长，胆道造影见充盈缺损范围逐渐扩大。BCS总病死率13．6％，再次移植病死率44．0％。总治愈率54．0％、总好转率71．0％、总狭窄发生率29．0％。结论（1）根据胆道镜检查BCS的病理类型主要有6种，临床症状主要有黄疸、发热等，胆道造影主要有4种表现。（2）临床症状及胆道造影表现主要取决于病理类型。

Objective To summarize the Patho-typing and the clinical manifestation of biliary cast syndrome （BCS） in patients after othotopic liver transplantation. Methods The clinical manifestation, findings,therapeutic means and efficacy of 103 patients with biliary cast syndrome after orthotopic liver transplantation were retrospectively analyzed. According to the injury level of biliary duct epithelium, patients were derided into different groups. All cases were followed up for twelve months. The place, degree and time after operation would be recorded when non-anastomotic biliary stricture was found. Results There were 59 BCS cases in the general hospital of armed police force of China. The incidence rate of BCS was 9. 1%. Many BCS patients showed symptoms such as jaundice, deep urine color, gray sedes, itch of skin and fever. Some were asymptomatic. In laboratory test, the liver functional enzyme in serum were increased, the total white cell count in peripheral blood was increased either. Cholangiography via T tube of biliary tract might show filling defect. According to the change degree of the biliary tract tree, there were four types filling defect concluded from all the presentation in BCS patients. Solid obturation of biliary tract were found by the check with optical fiber choledochoscope in all BCS patients, necrosis of biliary tract epithelium were observed in partial BCS patients. According to the injury level of biliary duct epithelium （gradually aggravated）, BCS patients were devided into six groups （ type Ⅰ , type Ⅱ , type Ⅲ, type Ⅳ, type Ⅴ and type Ⅵ ）. Fourteen cases were found in type Ⅰ and 18 in type Ⅱ. No clinical symptom was found in these two groups, a few indicators in serum （alanine aminotransferase ALT, total bilirubin TBIL, direct bilirubin DBIL） were in normal range, and others （gamma-glutamyl transferase GGT,alkaline phosphatase ALP） were heightened in 5 patients. There was no biliary cast （ BC ） found anymore in the period of espial in two groups. No stricture was found in both group. Twenty-seven cases in type Ⅲ and 23 cases in type IV,it was found there were about 33.4% patients accompanied with fever and 25.9% accompanied with jaundice in type Ⅲ . Paralleled,there were about 30. 4% and 34. 8% patients in type Ⅳ. The liver functional enzyme in serum were found increased in both type. After supporting treatment for 3-6 months, there were 5 and 3 patients present as mild non-anastomotic biliary stricture in type Ⅲ and type Ⅳ group. In the group type Ⅴ, there were 13 patients. The detected liver functional indicators in serum were increased. After supporting treatment for 6-12 months ,there were 4 patients present as moderate non-anastomotic biliary stricture in this group. There were 18 patients in type Ⅵ group, all indicators of the liver functional enzyme in serum before the treatment were elevated conspicuously. All patients in this group were found serious stricture up to three places that have not been sustaind in the period of espial. Nine died of MOSF, 1 died of AOSC, 8 had undergone retransplantation. In the retransplantation patients ,4 died of post operation MOSF,3 recovered to normal, 1 patient was found BCS once more 15 d after the retransplantation and the third-transplantation was performed 7 months after the second transplantation, no BCS was found again. The deaths total rate was 13.6% ,death rate of retransplantation was 44. 0%, total cure rate was 54. 0%, total improvement rate was 71.0% and total stenosis rate was 29. 0%. Conclusions （ 1 ） According to the check with optical fiber choledochoscope, there are 6 types of patho-typing in BCS patients. The clinical manifestation includes jaundice and fever. The filling defect of the biliary tract tree might showed 4 appearances. （2）The pathotyping contributes to the clinical manifestation and the filling defect of the biliary tract tree.