Department of Pediatric Gastroenterology & Hepatology, Dr Rela Institute & Medical Centre, Bharath Institute of Higher Education and Research, Chennai, Tamil Nadu, India.
Department of Histopathology, Dr Rela Institute & Medical Centre, Bharath Institute of Higher Education & Research, Chennai, Tamil Nadu, India.
Liver Transpl. 2024 Jul 1;30(7):699-706. doi: 10.1097/LVT.0000000000000351. Epub 2024 Feb 15.
Patients post liver transplant (LT) with progressive familial intrahepatic cholestasis type 1 (PFIC-1) often develop progressive graft steatohepatitis, intractable diarrhea, and growth failure. A total internal biliary diversion (TIBD) during an LT may prevent or reverse these adverse events. Children with PFIC-1 who underwent an LT at our institute were divided into 2 groups, A and B based on the timeline where we started offering a TIBD in association with LT. Pre-LT parameters, intraoperative details, and posttransplant complications like graft steatosis and diarrhea were also analyzed between the 2 groups, and their growth velocity was measured in the follow-up period. Of 550 pediatric LT performed between 2011 and 2022, 13 children underwent LT for PFIC-1. Group A had 7 patients (A1-A7) and group B had 6 (B1-B6). Patients A1, A4, B4, and B5 had a failed partial internal biliary diversion before offering them an LT. Patients A1, A2, and A6 in group A died in the post-LT period (2 early allograft dysfunction and 1 posttransplant lymphoproliferative disorder) whereas A3, A4, and A5 had graft steatosis in the follow-up period. A4 was offered a TIBD 4 years after LT following which the graft steatosis fully resolved. In group B, B1, B2, B5, and B6 underwent TIBD during LT, and B3 and B4 had it 24 and 5 months subsequently for intractable diarrhea and graft steatosis. None of the patients in group B demonstrated graft steatosis or diarrhea and had good growth catch-up during follow-up. We demonstrate that simultaneous TIBD in patients undergoing LT should be a standard practice as it helps dramatically improve outcomes in PFIC-1 as it prevents graft steatosis and/or fibrosis, diarrhea, and improves growth catch-up.
肝移植（LT）后患有进行性家族性肝内胆汁淤积症 1 型（PFIC-1）的患者常发生进行性移植物脂肪性肝炎、难治性腹泻和生长发育迟缓。LT 期间进行全内胆管分流术（TIBD）可能预防或逆转这些不良事件。本研究所在 LT 中接受 PFIC-1 的儿童根据 LT 期间开始提供 TIBD 的时间线分为 A 组和 B 组。比较了两组间 LT 前参数、手术细节和移植后并发症（如移植物脂肪变性和腹泻），并在随访期间测量了他们的生长速度。在 2011 年至 2022 年间进行的 550 例小儿 LT 中，有 13 例接受 LT 治疗 PFIC-1。A 组有 7 例（A1-A7），B 组有 6 例（B1-B6）。A1、A4、B4 和 B5 患者在接受 LT 之前曾行部分内胆管分流术失败。A 组 A1、A2 和 A6 患者在 LT 后死亡（2 例早期移植物功能障碍和 1 例移植后淋巴增殖性疾病），而 A3、A4 和 A5 在随访期间出现移植物脂肪变性。A4 在 LT 后 4 年接受 TIBD，随后移植物脂肪变性完全缓解。B 组 B1、B2、B5 和 B6 在 LT 期间接受 TIBD，B3 和 B4 因难治性腹泻和移植物脂肪变性分别在 24 个月和 5 个月后接受 TIBD。B 组无患者出现移植物脂肪变性或腹泻，且在随访期间有良好的生长追赶。我们证明，在 LT 患者中同时进行 TIBD 应成为标准治疗方法，因为它可以显著改善 PFIC-1 的预后，防止移植物脂肪变性和/或纤维化、腹泻，并改善生长追赶。
