Choudhary Narendra S, Saigal Sanjiv, Thummala Srikanth, Saraf Neeraj, Rastogi Amit, Bhangui Prashant, Srinivasan Thiagrajan, Yadav Sanjay K, Nundy Samiran, Soin Arvinder S
Institute of Liver Transplantation and Regenerative Medicine, Medanta the Medicity, Gurugram, Delhi (NCR), India.
Sir Ganga Ram Hospital, Delhi, India.
J Clin Exp Hepatol. 2020 Sep-Oct;10(5):442-447. doi: 10.1016/j.jceh.2020.02.002. Epub 2020 Feb 14.
Primary sclerosing cholangitis (PSC) is a progressive cholestatic disorder with liver transplantation (LT) being the only definitive treatment in end-stage disease. Recurrence of PSC after LT is a significant concern which can lead to graft loss. The aim of this study is to find out the disease recurrence and long-term outcome after living donor liver transplantation (LDLT) in PSC.
We conducted a retrospective review of all patients undergoing LDLT for PSC at our centre. Of 2268 adult LTs from August 2004 to July 2018, 32 (1.4%) patients underwent LDLT for PSC including 6 with PSC and autoimmune hepatitis overlap. The data were reviewed to look for PSC recurrence, complications, and overall survival. All patients received tacrolimus-based immunosuppression. Data are shown as number, percentage, median, and interquartile range (IQR).
The mean age of 32 LDLT recipients was 44 ± 12 years (males 22, females 10). At the time of transplantation, the mean child's score was 9 ± 1.6 and model for end-stage liver disease score was 18.9 ± 6.4. Ulcerative colitis was seen in 7 patients and none had cholangiocarcinoma. Majority of patients (n = 29) received right lobe graft and all but 3 underwent hepaticojejunostomy for biliary reconstruction. PSC recurrence was seen in 6 (20%) patients during a median follow-up of 59 (29-101) months, after exclusion of 2 patients with early mortality. A total of five patients died during follow-up, and one of these deaths was due to PSC recurrence. There were 2 perioperative deaths due to sepsis and 3 deaths on follow-up (sepsis in 2 and PSC recurrence in 1).
LDLT can be performed in PSC with good overall long-term outcomes.
原发性硬化性胆管炎（PSC）是一种进行性胆汁淤积性疾病，肝移植（LT）是终末期疾病的唯一确定性治疗方法。肝移植后PSC复发是一个重大问题，可能导致移植物丢失。本研究的目的是了解活体供肝肝移植（LDLT）治疗PSC后的疾病复发情况和长期预后。
我们对在本中心接受LDLT治疗PSC的所有患者进行了回顾性研究。在2004年8月至2018年7月的2268例成人肝移植中，32例（1.4%）患者接受了LDLT治疗PSC，其中6例为PSC与自身免疫性肝炎重叠。对数据进行回顾，以寻找PSC复发、并发症和总体生存率。所有患者均接受以他克莫司为基础的免疫抑制治疗。数据以数量、百分比、中位数和四分位间距（IQR）表示。
32例LDLT受者的平均年龄为44±12岁（男性22例，女性10例）。移植时，平均儿童评分9±1.6，终末期肝病模型评分18.9±6.4。7例患者患有溃疡性结肠炎，无一例患有胆管癌。大多数患者（n = 29）接受右叶移植，除3例患者外，所有患者均接受肝空肠吻合术进行胆道重建。在排除2例早期死亡患者后，中位随访59（29 - 101）个月期间，6例（20%）患者出现PSC复发。随访期间共有5例患者死亡，其中1例死亡是由于PSC复发。围手术期有2例因败血症死亡，随访期间有3例死亡（2例因败血症，1例因PSC复发）。
PSC患者可进行LDLT，总体长期预后良好。
