Krohn Paul S, Hillingsø Jens G, Kirkegaard Preben
Department of Surgery and Transplantation, Rigshospitalet, University of Copenhagen, Denmark.
Scand J Gastroenterol. 2008 Jan;43(1):89-94. doi: 10.1080/00365520701529360.
Polycystic liver disease (PLD) is a rare, hereditary, benign disorder. Hepatic failure is uncommon and symptoms are caused by mass effects leading to abdominal distension and pain. Liver transplantation (LTX) offers fully curative treatment, but there is still some controversy about whether it is a relevant modality considering the absence of liver failure, relative organ shortage, perioperative risks and lifelong immunosuppression. The purpose of this study was to review our experience of LTX for PLD and to compare the survival with the overall survival of patients who underwent LTX from 1992 to 2005.
A retrospective study of the journals of 440 patients, who underwent 506 LTXs between 1992 and 2005, showed that 14 patients underwent LTX for PLD. All patients had normal liver function. Three were receiving haemodialysis and thus underwent combined liver/kidney transplantation. One patient had undergone kidney transplantation 10 years earlier.
Median follow-up was 55 months. One patient who underwent combined transplantation died after 5.4 months because of multiorgan failure after re-LTX, and one patient, with well-functioning grafts, died of lymphoma after 7 months. At present 12 patients are alive, relieved of symptoms and with good graft function.
We conclude that patients treated for PLD by LTX have a good long-term prognosis and excellent relief of symptoms and that LTX might be considered in severe cases of PLD, where conventional surgery is not an option.
多囊肝病（PLD）是一种罕见的遗传性良性疾病。肝衰竭并不常见，症状由肿块效应引起，导致腹胀和疼痛。肝移植（LTX）提供了完全治愈性的治疗方法，但考虑到无肝衰竭、相对器官短缺、围手术期风险和终身免疫抑制，对于它是否是一种合适的治疗方式仍存在一些争议。本研究的目的是回顾我们对PLD进行LTX的经验，并将其生存率与1992年至2005年接受LTX的患者的总体生存率进行比较。
对1992年至2005年间接受506次LTX的440例患者的病历进行回顾性研究，结果显示14例患者因PLD接受了LTX。所有患者肝功能均正常。3例患者正在接受血液透析，因此接受了肝肾联合移植。1例患者10年前接受过肾移植。
中位随访时间为55个月。1例接受联合移植的患者在再次LTX后因多器官衰竭于5.4个月后死亡，1例移植肾功能良好的患者在7个月后死于淋巴瘤。目前12例患者存活，症状缓解，移植肾功能良好。
我们得出结论，通过LTX治疗的PLD患者具有良好的长期预后和症状的显著缓解，并且在PLD的严重病例中，当传统手术不可行时，可以考虑LTX。
