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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引用次数: 0
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.