Pure living donor liver transplant for liver and polycystic kidney disease, outcomes and experiences from an Asian transplant unit

Darren W. Chua , Horng-ren Yang , Ping Chun Li , Sheng-Hsien Chen , Shih Chao Hsu , Te-Hong Chen , Hsueh-Chou Lai , Ching-Feng Wu , You-Cian Lin , Long-Bin Jeng
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Abstract

Background

Adult polycystic liver disease (PLD) is a rare genetic disorder. In instances of intractable symptoms from mass effect or recurrent cyst-related complications, liver transplant affords the best therapeutic solution.

Methods

This is a retrospective case series of patients who underwent isolated pure living donor liver transplantation (LDLT) for adult polycystic kidney disease (PCKD) associated PLD from 2012- 2022. Short-term peri‑operative outcomes including renal sequelae and long-term survival were analysed.

Results

From 2012–2022, a total of 9 patients underwent LDLT for PLD. The median explanted liver weight was 6950 g (IQR 5653–7700) with a median blood loss of was 5450mls (IQR 2400–9875). There was 1 mortality due to acute antibody-mediated rejection (11.1 %). Of the four patients with existing chronic kidney disease (CKD), 2 (50 %) developed end-stage renal failure and was renal replacement therapy (RRT) dependent at 3 months. The median overall survival in our series was 63.9 months with a 5-year survival of 88.9 %.

Conclusions

Despite its technical challenges, LDLT affords an excellent long-term prognosis with acceptable major morbidity. Pre-operative CKD appears to be a risk factor for early renal deterioration.
纯活体供肝移植治疗肝脏和多囊肾疾病,来自亚洲移植单位的结果和经验
背景成人多囊肝病(PLD)是一种罕见的遗传性疾病。方法这是一项回顾性病例系列研究,研究对象是2012-2022年期间因成人多囊肾病(PCKD)相关多囊肝病而接受离体纯活体肝移植(LDLT)的患者。结果从2012年到2022年,共有9名患者接受了LDLT治疗。切除肝脏的中位重量为 6950 克(IQR 5653-7700),中位失血量为 5450 毫升(IQR 2400-9875)。有一名患者因急性抗体介导的排斥反应而死亡(11.1%)。在4名患有慢性肾病(CKD)的患者中,有2人(50%)发展为终末期肾衰竭,3个月后需要接受肾脏替代治疗(RRT)。在我们的系列研究中,总生存期的中位数为 63.9 个月,5 年生存率为 88.9%。术前慢性肾脏病似乎是导致早期肾功能恶化的一个危险因素。
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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