CAJ | 학술논문

目的探讨原发性硬化性胆管炎(PSC)的临床、实验室、影像和病理学特点,以及熊去氧胆酸(UDCA)治疗对预后的影响. 方法回顾性总结我院收治的22例PSC患者临床资料,对既往用药史、手术史、临床表现、实验室检查、影像学、肝组织学及UDCA治疗对预后影响等方面进行分析.数据比较用x2或校正x2检验.结果 22例患者中15例为男性,15例出现乏力、尿黄、体质量减轻,4例合并溃疡性结肠炎.22例患者的碱性磷酸酶(ALP)水平均明显升高,达(348±184)U/L;19例ALT水平升高至(94.0±67.0)U/L,AST水平升高至(98.0±67.0)U/L;15例总胆红素水平达(99.0±115.0)μmol/L,直接胆红素水平达(74.4±92.4)μmol/L.内镜逆行胰胆管造影检查可见肝内胆管节段性狭窄与扩张,胆管壁僵直,胆囊增大,胆总管及胰管显影不清;磁共振胆管造影可显示肝内胆管呈“串珠样”改变,胆管壁僵硬,胆总管扩张.组织病理学表现为胆管的纤维化和炎症及小胆管的增生,典型改变为呈“洋葱皮样”的纤维性闭塞性胆管炎.服用UDCA治疗组与未服用UDCA组比较,好转者分别为7例和5例,无效者均为5例,疗效差异无统计学意义(配对x2值及校正x2值分别为0.333和0.083,P值均＞0.05).结论 PSC患者男性居多,乏力、尿黄、体质量减轻为常见临床表现,血清生物化学指标升高明显,影像学检查有明显的特征性改变;UDCA治疗对PSC疗效不明显.
목적 탐토원발성경화성담관염(PSC)적림상、실험실、영상화병이학특점,이급웅거양담산(UDCA)치료대예후적영향. 방법 회고성총결아원수치적22례PSC환자림상자료,대기왕용약사、수술사、림상표현、실험실검사、영상학、간조직학급UDCA치료대예후영향등방면진행분석.수거비교용x2혹교정x2검험.결과 22례환자중15례위남성,15례출현핍력、뇨황、체질량감경,4례합병궤양성결장염.22례환자적감성린산매(ALP)수평균명현승고,체(348±184)U/L;19례ALT수평승고지(94.0±67.0)U/L,AST수평승고지(98.0±67.0)U/L;15례총담홍소수평체(99.0±115.0)μmol/L,직접담홍소수평체(74.4±92.4)μmol/L.내경역행이담관조영검사가견간내담관절단성협착여확장,담관벽강직,담낭증대,담총관급이관현영불청;자공진담관조영가현시간내담관정“천주양”개변,담관벽강경,담총관확장.조직병이학표현위담관적섬유화화염증급소담관적증생,전형개변위정“양총피양”적섬유성폐새성담관염.복용UDCA치료조여미복용UDCA조비교,호전자분별위7례화5례,무효자균위5례,료효차이무통계학의의(배대x2치급교정x2치분별위0.333화0.083,P치균＞0.05).결론 PSC환자남성거다,핍력、뇨황、체질량감경위상견림상표현,혈청생물화학지표승고명현,영상학검사유명현적특정성개변;UDCA치료대PSC료효불명현.
Objective To characterize the clinical,laboratory,imaging and pathological features of primary sclerosing cholangitis(PSC)and investigate the impact of ursodeoxycholic acid(UDCA)therapy on patient prognosis.Methods The medical records of 22 patients diagnosed with PSC between 2002 and 2011 were retrospectively reviewed.The PSC diagnosis had been made in patients with suspect biochemical abnormalities following evaluation by magnetic resonance cholangiopancreatography(MRCP)and/or endoscopic retrograde cholangiopancreatography(ERCP)and percutaneous transhepatic cholangiography (PTC).Fibrosis and inflammaton were assessed by immunohistochemical analyses of tissue biopsies.Outcome of patients treated with UDCA(13-15 mg/kg/day,oral)were compared to that of patients without UDCA treatment by the x2 or corrected x2 tests.Results Among the 22 PSC patients,the majority was male (n =15)and presented with fatigue,dark urine,and body weight loss(n =15).Four cases had ulcerative colitis.At admission,all 22 cases showed elevated levels of alkaline phosphatase[ALP:(348 ± 184)U/L],19 cases showed elevated alanine aminotransferase [ALT:(94.0 ± 67.0)U/L] and aspartate aminotransferase [AST:(98.0 ± 67.0)U/L],and 15 cases showed elevated levels of total bilirubin(99.0± 115.0)μmol/L and direct bilirubin(74.4 ± 92.4 μmol/L.ERCP examination showed segmental intrahepatic bile duct stenosis with expansion,and stiff and enlarged gallbladder bile ducts,but unclear findings for the common bile ducts and pancreatic ducts.MRCP showed beading of the intrahepatic bile duct,stiffness of the bile duct wall,and dilation of the common bile duct.Fibrosis and inflammation were observed in the bile ducts,along with hyperplasia and the typical features of "onion skin" fibrosis and fibrous obliterative cholangitis.Five of the 10 patients treated with UDCA improved,and seven of the 12 patients in the non-UDCA treatment group improved.There was no statistically significant difference in outcome between the groups(paired x2 =0.333,corrected x2 =0.083,P ＞ 0.05).Conclusion PSC patients were predominantly male and the common clinical manifestations were fatigue,dark urine,and body weight loss.At admission,serum biochemical indicators of cholangitis were increased significantly and subsequent imaging studies confirmed the suspected diagnosis by showing obvious characteristic changes.UDCA treatment did not significantly improve patient prognosis.