Gringeri E, D'Amico F E, Bassi D, Mescoli C, Bonsignore P, Boetto R, Lodo E, Noaro G, Polacco M, D'Amico F, Boccagni P, Zanus G, Brolese A, Cillo U
Hepatobiliary and Liver Transplant Unit, Department of Surgical and Gastroenterologic Sciences, University of Padova, Padova, Italy.
Transplant Proc. 2012 Sep;44(7):2038-40. doi: 10.1016/j.transproceed.2012.06.041.
Polycystic liver disease (PLD) is due to a genetic disorder and frequently coexists with polycystic kidney disease (PKD). If the cysts produce symptomatology owing to their number and size, many palliative treatments are available. When none of the liver parenchyma is spared, or kidney insufficiency is marked, the only potentially curable treatment is liver transplantation (LT).
A 49-year old woman, diagnosed with PLD and PKD, was listed in January 2008 for combined LT and kidney transplantation (KT). A compatible organ became available 8 months later. Despite preserved liver function, the patient's clinical condition was poor; she experienced dyspnea, advanced anorexia, abdominal pain, and severe ascites. At LT, which took 9 hours and was performed using the classic technique, the liver was hard, massive in size (15.5 kg), and not dissociable from the vena cava. The postoperative course was complicated by many septic episodes, the last one being fatal for the patient at 4 months after transplantation.
LT for PLD in many series shows a high mortality rate. The Model for End-Stage Liver Disease (MELD) score does not stage patients properly, because liver function is usually preserved. The liver can achieve a massive size causing many symptoms, especially malnutrition and ascites; in this setting LT is the only possible treatment. Patients with a low MELD score undergo LT with severe malnutrition that predisposes them to greater susceptibility to sepsis. To identify predictor factors, beyond MELD criteria that relate to the increased liver volume before development of late symptoms is essential to expeditiously treat patients with the poorest prognosis to improve their outcomes.
多囊肝疾病（PLD）是一种遗传性疾病，常与多囊肾病（PKD）共存。如果囊肿因其数量和大小而产生症状，有多种姑息治疗方法可供选择。当肝实质无一幸免，或肾功能严重不全时，唯一可能治愈的治疗方法是肝移植（LT）。
一名49岁女性，被诊断为PLD和PKD，于2008年1月被列入联合肝移植和肾移植（KT）名单。8个月后获得了一个匹配的器官。尽管肝功能保留，但患者的临床状况较差；她出现呼吸困难、严重厌食、腹痛和严重腹水。在采用经典技术进行的长达9小时的肝移植手术中，肝脏坚硬，体积巨大（15.5千克），且与腔静脉无法分离。术后病程因多次败血症发作而复杂化，最后一次发作导致患者在移植后4个月死亡。
许多系列研究中，PLD患者进行肝移植的死亡率很高。终末期肝病模型（MELD）评分不能准确对患者进行分期，因为肝功能通常保留。肝脏可肿大到引起许多症状，尤其是营养不良和腹水；在这种情况下，肝移植是唯一可能的治疗方法。MELD评分低的患者在严重营养不良的情况下接受肝移植，这使他们更容易发生败血症。识别预测因素，除了与晚期症状出现前肝脏体积增加相关的MELD标准外，对于迅速治疗预后最差的患者以改善其结局至关重要。
