Yesmembetov Kakharman, Muratova Zhansaya, Borovskiy Sergey, Ten Irina, Kaliaskarova Kulpash
From the Department of Hepatology National Scientific Center for Oncology and Transplantology, Astana, Kazakhstan; and the Clinic of Hepatology, Gastroenterology, and Nutrition, Astana, Kazakhstan.
Exp Clin Transplant. 2018 Mar;16 Suppl 1(Suppl 1):158-161. doi: 10.6002/ect.TOND-TDTD2017.P44.
We report the clinical case of 23-year-old patient with liver cirrhosis of unknown genesis, significant resistant ascites, and 2 episodes of bleeding from esophageal varices. Evaluation did not find any cause of liver disease, and the patient was placed on the transplant wait list due to subcompensated liver function (Model for End-Stage Liver Disease score of 16, Child-Pugh class B) and poorly controlled severe portal hypertension. After treatment with diuretics, largevolume paracentesis, antibiotics, and vasoconstrictors, hepatorenal syndrome and spontaneous bacterial peritonitis resolved and liver function improved significantly. Because the patient showed consistently good liver function and resistant portal hypertension, liver transplant was delayed with decision to perform transjugular intrahepatic portosystemic shunting instead. During the attempt of shunting, occlusive thrombosis of the iliac veins, inferior vena cavae, and hepatic veins were diagnosed and the procedure was stopped. Therefore, considering preserved liver function and severe portal hypertension, diagnosis of Budd-Chiari syndrome with subsequent development of liver cirrhosis was made. The patient was recommended to undergo evaluation to exclude thrombophilia as a cause of thrombosis.
我们报告了一名23岁患者的临床病例,该患者患有病因不明的肝硬化、严重的难治性腹水以及2次食管静脉曲张出血。评估未发现任何肝脏疾病病因,由于肝功能代偿不全(终末期肝病模型评分16分,Child-Pugh B级)和严重门静脉高压控制不佳,该患者被列入肝移植等待名单。在接受利尿剂、大量腹腔穿刺放液、抗生素和血管收缩剂治疗后,肝肾综合征和自发性细菌性腹膜炎得到缓解,肝功能显著改善。由于患者肝功能持续良好且门静脉高压难治,肝移植被推迟,决定改为行经颈静脉肝内门体分流术。在进行分流术的尝试过程中,诊断出髂静脉、下腔静脉和肝静脉闭塞性血栓形成,手术停止。因此,考虑到肝功能保留和严重门静脉高压,诊断为布加综合征并继发肝硬化。建议该患者接受评估以排除血栓形成的原因——易栓症。