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目的:探讨直接性肝内门腔分流术(DIPS)治疗布加综合征(Budd-Chiari syndrome，BCS)的疗效。方法:回顾性分析2015年1月1日至2017年6月31日于北京世纪坛医院介入治疗科、北京友谊医院肝病中心及北京地坛医院普通外科接受DIPS治疗的BCS患者临床资料，包括患者病史、体征如腹胀、腹水、肝脏肿大、肝性脑病等以及围手术期生化检验指标。对比患者术前和术后2周谷丙转氨酶、谷草转氨酶、总胆红素、直接胆红素等生化指标及门静脉压力梯度。术后随访患者临床症状、分流道通畅情况、有无肝脏肿瘤及生存状况。结果:共纳入67例BCS患者，男性45例，女性22例，年龄(38.12±23.22)岁。患者BCS分型包括肝静脉型65例，其中肝静脉广泛性阻塞型62例、肝静脉阻塞伴血栓形成3例；肝静脉下腔静脉混合型2例。67例患者均成功行DIPS，共置入支架93枚，其中43例患者术中行胃冠状静脉栓塞术，2例混合型BCS患者同期行下腔静脉支架术，无严重操作相关并发症发生。患者门静脉压力梯度由术前(22.17±9.16) mmHg(1 mmHg＝0.133 kPa)降至术后(9.87±4.75) mmHg，差异有统计学意义(n P<0.05)。术后1个月，患者腹胀均缓解，术后3个月腹水均消退，肝脏淤血肿大明显缓解。与术前相比，术后2周患者谷丙转氨酶由(65.28±27.75)U/L降至(28.43±13.46)U/L，谷草转氨酶由(68.75±29.23)U/L降至(26.92±13.33)U/L,总胆红素由(175.31±80.48)μmol/L降至(45.08±26.54)μmol/L，直接胆红素由(127.55±44.65)μmol/L降至(35.12±10.77)μmol/L，白蛋白由(31.56±7.22)g/L升至(44.18±11.36)g/L，差异均有统计学意义(n P<0.05)。所有患者均随访3年，5例(7.46%)出现分流道狭窄并行分流道扩张，2例(2.99%)出现食管胃底静脉曲张破裂出血，4例(5.97%)腹水复发，2例(2.99%)出现肝性脑病，无患者出现肝恶性肿瘤，无患者死亡。n 结论:通过合理选择适应证应用DIPS术治疗BCS患者可迅速降低门静脉压力，缓解肝淤血，恢复肝脏形态及功能，疗效确切，安全可靠。“,”Objective:To study the efficacy of direct intrahepatic portosystemic shunt (DIPS) in treatment of Budd-Chiari syndrome (BCS).Methods:From January 1, 2015 to June 31, 2017, consecutive patients with BCS who were treated with DIPS at the Department of Interventional Therapy of Beijing Shijitan Hospital, the Liver Disease Research Center of Beijing Friendship Hospital and the General Surgery Department of Beijing Ditan Hospital were retrospectively analyzed. The symptoms, physical signs (including abdominal distension, ascites, pleural effusion, splenomegaly, hepatic encephalopathy) and perioperative laboratory results of these patients were collected and analyzed. Biochemical indicators including alanine aminotransferase (ALT), aspartate aminotransferase (AST), total bilirubin (TBil), direct bilirubin (DBil), and portal pressure gradient were compared before and 2 weeks after treatment. The patients were followed up for at least 3 years to assess their clinical symptoms, patency of shunt, oncological status and survival.Results:Of 67 patients with BCS who were included in the study, there were 45 males and 22 females, aged (38.12±23.22) years. The BCS classification of these patients were hepatic vein type (n n=65), including 62 patients with complete hepatic vein obstruction, 3 patients with hepatic vein occlusion due to thrombosis, and 2 patients with mixed hepatic vein and inferior vena cava occlusion. All 67 patients underwent DIPS with 93 stents being implanted. In addition, 43 patients underwent gastric coronary vein embolization, and 2 patients with mixed type of BCS underwent inferior vena cava stenting. The portal pressure gradient decreased from (22.17±9.16) mmHg (1 mmHg=0.133 kPa) to (9.87±4.75) mmHg, the difference was statistically significant (n P<0.05). Abdominal distension was relieved, at one month and ascites completely subsided in 3 months after operation. The liver congestion and swelling were obviously relieved. Comparison of patients 2 weeks after operation and before operation, ALT decreased from (65.28±27.75) U/L to (28.43±13.46)U/L, AST from (68.75±29.23) U/L to (26.92±13.33)U/L, TBil from (175.31±80.48)μmol/L to (45.08±26.54)μmol/L, DBil from (127.55±44.65)μmol/L to (35.12±10.77)μmol/L, and albumin increased from (31.56±7.22) g/L to (44.18±11.36)g/L, the difference was statistically significant (alln P<0.05). All patients were followed up for at least 3 years. Shunt stenosis was detected in 5 patients (7.46%) with shunt expansion being performed, variceal bleeding in 2 patients (2.99%), ascites recurrence in 4 patients (5.97%) and hepatic encephalopathy in 2 patients (2.99%). No patients were diagnosed with hepatic cancer, and no patients died.n Conclusion:DIPS was efficacious, safe and reliable to that BCS patients. It rapidly reduced portal venous pressure, relieved liver congestion, and restored liver morphology and liver function in these patients.