CAJ | 학술논문

目的探讨青少年布加综合征(Budd-Chiari syndrome,BCS)临床特点并评估介入治疗的疗效.方法徐州医学院附属医院自1990年1月至2012年4月收治227例年龄在29岁以下的BCS患者,均经彩超及血管造影证实,其中下腔静脉型87例、肝静脉型105例、混合型35例.通过经皮血管腔内血管成形术(percutaneous transluminal angioplasty,PTA)、血管内支架置入术及置管溶栓术开通闭塞血管.术后给予抗凝治疗、定期随访.结果 227例患者均以门脉高压的症状和体征为最初临床表现.210例患者初次介入手术取得成功,其中下腔静脉阻塞型成功率100％,肝静脉阻塞型85.7％,混合型94.3％.介入治疗成功后的下腔静脉平均压力由术前的(26.52±8.16) cm H2O下降至术后(14.28 ±4.08) cm H2O(P＜0.05).肝静脉平均压力由术前(35.70±13.26) cm H2O下降至术后(18.36±8.16) cm H2O(P＜0.05).术后随访1个月至15年,平均(46±37)个月.再狭窄发生率为21.4％(45/210),其中下腔静脉型狭窄率为13.8％(12/87),肝静脉阻塞型31.1％ (28/90),混合型15.2％ (5/33),肝静脉型患者再狭窄发生率明显高于其他两型.再狭窄患者介入治疗方法同初次治疗,44例再狭窄患者再次介入治疗取得成功.结论青少年布加综合征患者以肝静脉阻塞型最多见,门脉高压症状和体征为主要临床表现特点,肝静脉型介入治疗后复发率高于其他两型.
목적 탐토청소년포가종합정(Budd-Chiari syndrome,BCS)림상특점병평고개입치료적료효.방법 서주의학원부속의원자1990년1월지2012년4월수치227례년령재29세이하적BCS환자,균경채초급혈관조영증실,기중하강정맥형87례、간정맥형105례、혼합형35례.통과경피혈관강내혈관성형술(percutaneous transluminal angioplasty,PTA)、혈관내지가치입술급치관용전술개통폐새혈관.술후급여항응치료、정기수방.결과 227례환자균이문맥고압적증상화체정위최초림상표현.210례환자초차개입수술취득성공,기중하강정맥조새형성공솔100％,간정맥조새형85.7％,혼합형94.3％.개입치료성공후적하강정맥평균압력유술전적(26.52±8.16) cm H2O하강지술후(14.28 ±4.08) cm H2O(P＜0.05).간정맥평균압력유술전(35.70±13.26) cm H2O하강지술후(18.36±8.16) cm H2O(P＜0.05).술후수방1개월지15년,평균(46±37)개월.재협착발생솔위21.4％(45/210),기중하강정맥형협착솔위13.8％(12/87),간정맥조새형31.1％ (28/90),혼합형15.2％ (5/33),간정맥형환자재협착발생솔명현고우기타량형.재협착환자개입치료방법동초차치료,44례재협착환자재차개입치료취득성공.결론 청소년포가종합정환자이간정맥조새형최다견,문맥고압증상화체정위주요림상표현특점,간정맥형개입치료후복발솔고우기타량형.
Objective To present the clinical features and to evaluate interventional therapy for Budd-Chiari syndrome in Chinese youth.Methods From January 1990 to April 2012,227 cases who hospitalized at the age ＜ 29 underwent color Doppler ultrasound scan and digital subtraction angiography (DSA).87 cases were with occlusive inferior vena cava (IVC type),105 cases with occlusive hepatic veins (HV type) and 35 cases with occlusive inferior vena cava and hepatic veins (MIX type).The occlusive veins were opened by percutaneous transluminal angioplasty (PTA),endovascular stent placement,intravenous catheter thrombolysis or combination.Postoperative anticoagulation was given to all patients.Results The symptoms and signs of portal hypertension disappeared or were alleviated in successful cases.Technical success was achieved in 210 patients.The success rate was 100％ in IVC type,85.7％ in HV type and 94.3％ in MIX type.IVC pressure decreased from (26.52 ± 8.16) cm H2O to (14.28 ±4.08) cmH2O(P ＜ 0.05) and HV pressure dropped from(35.70 ± 13.26) cm H2O to(18.36 ±8.16) cm H2O (P ＜0.05).Restenosis or occlusion was found in 21.4％ (45/210) patients after a follow-up of 1 month to 15 years.The rate was 13.8％ (12/87) in IVC type,31.1％ (28/90) in HV type and 15.2％ (5/33) in MIX type.These patients were managed by interventional procedures.Technical successwas achieved in 44 cases with restenosis.Conclusions Hepatic vein occlusion was the most common type of BCS in Chinese youth.The symptoms and signs of portal hypertension were the initial clinical manifestations.Postoperative recurrence rate in HV type was higher than that in the other two types.