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
{"title":"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","authors":"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","doi":"10.1016/j.liver.2025.100272","DOIUrl":null,"url":null,"abstract":"<div><h3>Background</h3><div>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.</div></div><div><h3>Methods</h3><div>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.</div></div><div><h3>Results</h3><div>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 %.</div></div><div><h3>Conclusions</h3><div>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.</div></div>","PeriodicalId":100799,"journal":{"name":"Journal of Liver Transplantation","volume":"18 ","pages":"Article 100272"},"PeriodicalIF":0.0000,"publicationDate":"2025-04-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Pure living donor liver transplant for liver and polycystic kidney disease, outcomes and experiences from an Asian transplant unit\",\"authors\":\"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\",\"doi\":\"10.1016/j.liver.2025.100272\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<div><h3>Background</h3><div>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.</div></div><div><h3>Methods</h3><div>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.</div></div><div><h3>Results</h3><div>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 %.</div></div><div><h3>Conclusions</h3><div>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.</div></div>\",\"PeriodicalId\":100799,\"journal\":{\"name\":\"Journal of Liver Transplantation\",\"volume\":\"18 \",\"pages\":\"Article 100272\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2025-04-11\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Journal of Liver Transplantation\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://www.sciencedirect.com/science/article/pii/S2666967625000157\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Journal of Liver Transplantation","FirstCategoryId":"1085","ListUrlMain":"https://www.sciencedirect.com/science/article/pii/S2666967625000157","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
Pure living donor liver transplant for liver and polycystic kidney disease, outcomes and experiences from an Asian transplant unit
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.