布加综合征的临床分析

目的分析布加综合征的临床特点，以提高对该病的认识，减少误诊。方法回顾性分析19例布加综合征患者的病史、症状、体征、实验室检查、影像学检查及治疗随}方情况。结果布加综合征患者的职业多为农民及打工者（68．4％）。临床表现主要为腹胀（78．9％）、肝脾肿大（占47．4％及63．2％）、侧腹壁静脉曲张（57．9％）、腹水（36．9％）、下肢水肿（26．3％）、下肢静脉曲张、色素沉着或溃疡（31．6％）。彩色多普勒和磁共振血管成像的诊断率达80％和84．6％。16例患者行介入或手术治疗，其中12例介入治疗者测下腔静脉直径由术前（24．58±3．85）mm缩小为（17．92±3．05）mm（P〈0．01），下腔静脉压力由术前（3．08±0．38）kPa降为术后（1．89±0．28）kPa（P〈O．01）。结论门脉高压表现重或伴有下腔静脉高压而肝大、肝功能损害轻者，应高度怀疑本病。彩色多普勒和磁共振血管成像是重要的检查方法，早期诊断及治疗对预后有重要的影响。介入手术是有效的治疗手段。

Budd-Chiari Syndrome

Objective To explore the clinical features of Budd-Chiari syndrome for further understanding of the disease and decreasing the misdiagnosis. Methods Ninteen cases were analyzed retrospectively in history, symptoms, physical sign, laboratory examination, imaging examination, therapy and follow-up. Results Most of the patients were farmers and workers （ 68.4% ）. Abdominal distention （ 78.9% ）, hepatomegalia （ 47.4% ）, splenomegalia （ 63.2% ）, subcutaneous varicos vein of latero-abdominal wall （ 57.9% ）, ascites （ 36.9% ）, edema of lower extremity （ 26.3% ）, varicose veins, pigmentation and ulcer of lower extremity （ 31.6% ） was the most common clinical manifestation. The diagnostic coincidence of color Doppler and magnetic resonance angiography reached 80% and 84.6%, respectively. 16 cases accepted interventional therapy or surgical operation and had a good prognosis. The diameter of inderior vena cava in 12 cases who received interventional therapy falled from （ 24.58 ±3.85 ）mm before the operation to （ 17.92 ±3.05 ） mm after the operation （ P 〈 0.01 ）. And pressure of inderior vena cava decreased from （ 3.08 ± 0.38 ） kPa to （ 1.89 ± 0.28 ） kPa （ P 〈 0.01 ）. Conclusion We should think of the probability of Budd-Chiari syndrome when the patients have an obvious manifestation of portal hypertension, especially with hypertension of inferior vena and hepatauxe but slight damage of liver function. Color Doppler and magnetic resonance angiography is the effective examination for Budd-Chiari syndrome. The early diagnosis and treatment is quite important for the prognosis. The interventional therapy is an effective method.