CAJ | 학술논문

介绍临床以肝脾肿大、门脉高压、上消化道出血为主要症状的一种少见的遗传病一先天性肝纤维化的概念、诊断与治疗.1993年以来收治先天性肝纤维化3例,均经临床及病理证实,行睥切除、贲门胃底断流术.术后随访1～3年,患儿发育、营养佳,无再出血,肝功能正常,近期疗效满意.复习文献结合本组病例诊治认为,临床发现肝脾肿大、门脉高压、肝内胆管多发性扩张、肝功能基本正常者应想到该病.肝脏组织学特点为汇管区纤维增生明显,肝小叶结构正常.为预防术后并发症应早期诊断,及时治疗.
개소림상이간비종대、문맥고압、상소화도출혈위주요증상적일충소견적유전병일선천성간섬유화적개념、진단여치료.1993년이래수치선천성간섬유화3례,균경림상급병리증실,행비절제、분문위저단류술.술후수방1～3년,환인발육、영양가,무재출혈,간공능정상,근기료효만의.복습문헌결합본조병례진치인위,림상발현간비종대、문맥고압、간내담관다발성확장、간공능기본정상자응상도해병.간장조직학특점위회관구섬유증생명현,간소협결구정상.위예방술후병발증응조기진단,급시치료.
Three cases of congenital hepatic fibrosis underwent splenectomy and cardiofundotransectomy are reported.The clinical manifestations included hepatosplenomegaly,portal hypertension and upper gastrointestinal hemorrhage.The diagnosis was confirmed by pathologic examination.They were followed-up for 1 to 3 years with normal physical development,hepatic functions,and without GI bleeding.The authors state that patients with hepatosplenomegaly,portal hypertension,multiple dilation of intrahepatic bile ducts but without abnormal liver functions,congenital hepatic fibrosis should be considered.The histologic characteristics include fibrous proliferation of interlobular portal areas,and normal structure of hepatic lobules.Early diagnosis and treatment should be stressed to prevent postoperative complications.