Liver Transplantation in Childhood: A 2-Year Single Center Experience.

IF 0.8
Hasret Ayyıldız Civan, Ferhat Sarı, Feyza Sönmez Topçu, Aysel Taktak, Hüseyin İlksen, Adem Tunçer, Emrah Şahin, Halil Şahin, Veysel Esan, Bülent Ünal, Abuzer Dirican
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Abstract

Objective: Liver transplantation is currently the most treatment for fulminant hepatitis, end-stage liver failure, hepatocellular carcinoma, and liver-originated metabolic diseases in children. With technological advances, improvements in surgical techniques and immunosuppressive therapy protocols have increased 1-year survival rates to 80%-90%. Our center successfully performs both living donor and cadaveric liver transplants in children. This study retrospectively analyzed the preoperative and postoperative data of 72 pediatric patients who underwent liver transplantation between July 2022 and July 2024.

Methods: We included 72 patients who underwent liver transplantation between July 1, 2022, and July 1, 2024. Cases were evaluated based on demographic data, liver failure etiology, and postoperative complications.

Results: Of the cases, 37 were female (58%) and 35 male (42%), with a mean age of 6.6 years (ranging from 5 months to 17 years and 11 months). Indications included biliary atresia (25), autoimmune hepatitis (9), cryptogenic cirrhosis (7), PFIC (7), congenital hepatic fibrosis (1), Caroli disease (2), Wilson's disease (4), Alagille syndrome (3), hepatocellular carcinoma (2), primary hyperoxaluria type 1 (2), Crigler Najjar syndrome type 1 (3), Budd-Chiari syndrome (1), glycogen storage disease type 3 (1), portal vein thrombosis (1), and acute fulminant hepatitis (4). Mean PELD score for patients under 12 years was 18 (range 0-37), and MELD score for patients over 12 years was 19.3 (range 11-40). A total of 69 patients received orthotopic liver transplantation from living donors. Two patients received combined liver and kidney transplants, and 1 received a cadaveric liver transplant. Donors included 40 females and 32 males. Left lobe transplants were performed in 58 patients, and right lobe in 14. Immunosuppression included Tacrolimus + MMF in 70 patients and Cyclosporine in 2. Postoperative complications included biliary anastomosis stenosis (3), bile leakage (2), hepatic vein thrombosis (1), portal vein thrombosis (4), intestinal perforation secondary to Bogota syndrome (5), PRES syndrome due to Tacrolimus toxicity (1), primary graft dysfunction (1), and postoperative bleeding (2). Within the first month, 12 patients (16.6%) died, and 3 (4%) died between 1 month and 1 year postoperatively. The most common early cause of death was sepsis and multiorgan failure. One patient developed chronic rejection but recovered with steroid immunosuppression without the need for re-transplantation.

Conclusion: Liver transplantation is a high-risk procedure requiring lifelong medication and follow-up. It is, however, the most effective treatment method for several severe pediatric liver conditions.

儿童肝移植:2年单中心经验。
目的:肝移植是目前儿童重型肝炎、终末期肝功能衰竭、肝细胞癌和肝脏源性代谢性疾病最常用的治疗方法。随着技术的进步,手术技术和免疫抑制治疗方案的改进使1年生存率提高到80%-90%。本中心成功地为儿童进行了活体肝移植和尸体肝移植。本研究回顾性分析了2022年7月至2024年7月期间接受肝移植的72例儿童患者的术前和术后资料。方法:我们纳入了2022年7月1日至2024年7月1日期间接受肝移植的72例患者。病例评估基于人口统计学数据、肝功能衰竭病因和术后并发症。结果:女性37例(58%),男性35例(42%),平均年龄6.6岁(5个月~ 17岁11个月)。适应症包括胆道闭锁(25例)、自身免疫性肝炎(9例)、隐源性肝硬化(7例)、PFIC(7例)、先天性肝纤维化(1例)、Caroli病(2例)、Wilson病(4例)、Alagille综合征(3例)、肝细胞癌(2例)、原发性高草酸尿1型(2例)、Crigler Najjar综合征1型(3例)、Budd-Chiari综合征(1例)、糖原蓄积病3型(1例)、门静脉血栓形成(1例)、急性暴发性肝炎(4例)。12岁以下患者的平均PELD评分为18(范围0-37),12岁以上患者的MELD评分为19.3(范围11-40)。共有69例患者接受活体供体原位肝移植。2例患者接受肝肾联合移植,1例患者接受尸体肝移植。捐赠者包括40名女性和32名男性。左肺叶移植58例,右肺叶移植14例。免疫抑制包括他克莫司+ MMF 70例,环孢素2例。术后并发症包括胆道吻合口狭窄(3例)、胆漏(2例)、肝静脉血栓形成(1例)、门静脉血栓形成(4例)、波哥大综合征继发肠穿孔(5例)、他克莫司毒性引起的PRES综合征(1例)、原发性移骨功能障碍(1例)、术后出血(2例)。术后1个月内死亡12例(16.6%),术后1个月至1年内死亡3例(4%)。最常见的早期死亡原因是败血症和多器官衰竭。1例患者出现慢性排斥反应,但经类固醇免疫抑制后恢复,无需再次移植。结论:肝移植是一项高风险手术,需要终生服药和随访。然而,对于几种严重的儿童肝脏疾病,它是最有效的治疗方法。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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