Zhang Liling, Gan Linwang, Liu Qi, Li Ying, Lin Jiaru, Ou Santao
Department of nephropathy, The First Affiliated Hospital of Southwest Medical University. Luzhou, Sichuan, China.
Medicine (Baltimore). 2020 Apr;99(14):e19511. doi: 10.1097/MD.0000000000019511.
Polycystic liver disease (PLD) is an autosomal-dominant disorder that is commonly associated with autosomal-dominant polycystic kidney disease (PKD) but rarely complicated with polycystic lung. Here, we report the first case of severe obstructive jaundice caused by multiple liver cysts in a patient with PLD complicated by PKD and polycystic lung.
A 72-year-old man with a history of PLD complicated with polycystic kidney presented with progressive jaundice, hematuria, poor appetite, nausea, and weight loss since 3 months.
PLD complicated with PKD and polycystic lung was identified using computed tomography, and obstructive jaundice was identified using magnetic resonance imaging and magnetic resonance cholangiopancreatography.
The patient could not undergo surgery, and was therefore treated with combined bilirubin adsorption and continuous veno-venous hemofiltration.
The patient's symptoms and laboratory findings improved after bilirubin adsorption and continuous veno-venous hemofiltration. Unfortunately, the patient was unable to continue the treatment due to financial reasons, and died of shock most likely due to cyst rupture.
Imaging examination of the lungs is necessary for patients with PLD. Although infrequent, jaundice can occur in these patients and cause severe hyperbilirubinemia. When surgery is contraindicated, blood purification may serve as an alternative treatment for patients with PLD-related obstructive jaundice.
多囊肝病(PLD)是一种常染色体显性疾病,通常与常染色体显性多囊肾病(PKD)相关,但很少并发多囊肺。在此,我们报告首例患有PLD并合并PKD和多囊肺的患者因多个肝囊肿导致严重梗阻性黄疸的病例。
一名72岁男性,有PLD合并多囊肾病史,自3个月前以来出现进行性黄疸、血尿、食欲减退、恶心和体重减轻。
通过计算机断层扫描确定为PLD合并PKD和多囊肺,通过磁共振成像和磁共振胰胆管造影确定为梗阻性黄疸。
患者无法接受手术,因此接受了胆红素吸附和连续性静脉-静脉血液滤过联合治疗。
胆红素吸附和连续性静脉-静脉血液滤过后,患者的症状和实验室检查结果有所改善。不幸的是,患者因经济原因无法继续治疗,最有可能因囊肿破裂死于休克。
对于PLD患者,肺部的影像学检查是必要的。虽然此类患者黄疸发生率不高,但可能发生并导致严重高胆红素血症。当手术禁忌时,血液净化可作为PLD相关梗阻性黄疸患者的替代治疗方法。