Department of Gastroenterology and Hepatology, Centre for Liver and Biliary Sciences, Hepatology and Liver Transplant Medicine Saket, Max Super Speciality Hospital, New Delhi, 110017, India.
Liver Transplant and Gastrointestinal Surgery, Centre for Liver and Biliary Sciences, Max Super Speciality Hospital, Saket, New Delhi, India.
Hepatol Int. 2023 Apr;17(2):499-506. doi: 10.1007/s12072-022-10442-4. Epub 2022 Nov 14.
Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease. In the absence of effective medical therapy, liver transplant is the definitive treatment for advanced stage. However, recurrence of PSC after liver transplant is of concern which can lead to graft failure and may require retransplant. There are limited data on outcomes of living donor liver transplant (LDLT) in PSC. Also, in LDLT as donors are genetically related there can be an increased risk of recurrence. We conducted this retrospective study to analyze the outcomes of LDLT in PSC at a tertiary liver transplant center in north India.
We conducted a retrospective analysis of 3213 transplant recipients who underwent LDLT from January 2006 to May 2021. Of these 26 (0.80%) patients had PSC as indication for liver transplantation (PSC = 24, PSC-AIH overlap = 2). Data analysis was done to look for baseline demographics, clinical details, transplant outcomes, PSC recurrence, and survival.
Mean age of study group was 42 (± 13.8) years and 19 patients (73.1%) were males. All patients had decompensated cirrhosis at the time of transplant. Mean CTP score and MELD score were 9.5 (± 1.8) and 18.9 (± 7.1), respectively. Sixteen patients received modified right lobe graft, seven extended right lobe graft and five patients received left lateral graft. Median graft weight and mean graft to recipient weight ratio (GRWR) were 633.5 (IQR 473.5-633.5) grams and 1.23 (± 0.42), respectively. Most common biliary anastomosis was hepaticojejunostomy, done in 19 (73.1%) while duct to duct anastomosis was performed in 7 (26.9%) patients. Median follow-up was 96 (36-123) months. One patient had ulcerative colitis and none had cholangiocarcinoma. Two (7.7%) patients had bile leak during early post-transplant period. Three (11.1%) patients developed graft rejection and were managed successfully with steroid pulses. Three patients died during early post-transplant period while seven deaths occurred during long-term follow-up including one death due to COVID-19. Five (21.73%) patients had recurrence of PSC of which two patients had graft loss including one after retransplantation. The one year graft and patient survival rate was 88.5%.
LDLT can be performed in PSC with good long-term outcomes with a risk of PSC recurrence in about one-fifth patients.
原发性硬化性胆管炎（PSC）是一种慢性胆汁淤积性肝病。在缺乏有效药物治疗的情况下，肝移植是治疗晚期疾病的最终手段。然而，肝移植后 PSC 的复发令人担忧，这可能导致移植物失功，并且可能需要再次移植。关于 PSC 患者接受活体肝移植（LDLT）的结果数据有限。此外，由于供者之间存在遗传关系，复发的风险可能会增加。我们进行了这项回顾性研究，以分析印度北部一家三级肝移植中心接受 LDLT 的 PSC 患者的结果。
我们对 2006 年 1 月至 2021 年 5 月期间接受 LDLT 的 3213 名移植受者进行了回顾性分析。其中 26 名（0.80%）患者因 PSC 而接受肝移植（PSC=24 例，PSC-AIH 重叠=2 例）。对数据进行分析，以寻找基线人口统计学、临床细节、移植结果、PSC 复发和生存率。
研究组的平均年龄为 42（±13.8）岁，19 名患者（73.1%）为男性。所有患者在移植时均患有失代偿性肝硬化。平均 CTP 评分和 MELD 评分分别为 9.5（±1.8）和 18.9（±7.1）。16 名患者接受改良右叶移植，7 名患者接受右三叶扩展移植，5 名患者接受左外侧叶移植。中位移植物重量和平均移植物与受体体重比（GRWR）分别为 633.5（IQR 473.5-633.5）克和 1.23（±0.42）。最常见的胆道吻合术是胆肠吻合术，19 例（73.1%）患者采用这种吻合术，7 例（26.9%）患者采用胆管对胆管吻合术。中位随访时间为 96（36-123）个月。1 名患者患有溃疡性结肠炎，无胆管癌患者。2 名（7.7%）患者在移植后早期出现胆漏。3 名（11.1%）患者发生移植物排斥反应，经类固醇脉冲治疗成功。3 名患者在移植后早期死亡，7 名患者在长期随访中死亡，包括 1 名因 COVID-19 死亡。5 名（21.73%）患者发生 PSC 复发，其中 2 名患者发生移植物失功，包括 1 例在再次移植后发生。1 年移植物和患者存活率为 88.5%。
LDLT 可用于 PSC 患者，长期结果良好，但约五分之一的患者有 PSC 复发的风险。
