CAJ | 학술논문

目的研究婴儿胆汁淤积性肝病肝活组织检查的病理及超微病理结构特征,并结合临床资料探讨其在诊断中的意义.方法对2007-2008年在重庆医科大学儿童医院就诊的36例胆汁淤积型婴儿肝炎综合征患儿进行肝活组织检查并随访,分析其肝组织病理及超微病理特征.结果 36例婴儿胆汁淤积性肝病肝活组织检查的光镜结果显示,肝细胞肿胀、变性、坏死,多核巨细胞形成,胆管增生,纤维组织增生,肝小叶和汇管区炎性细胞浸润为主要病理表现.肝细胞淤胆,假腺腔形成、羽毛状变性和毛细胆管胆栓形成为胆汁淤积的特征.7例影像学检查除外胆道闭锁病例的肝组织呈现典型胆道梗阻征象.电镜下常见核改变、胞质溶解、炎症细胞浸润、胶原纤维增生和溶酶体增多.形态学改变结合临床表现诊断肝糖原累积病2例,尼曼皮克病1例,分类不明脂质沉积病1例.结论婴儿胆汁淤积性肝病肝组织普通光镜和电镜下存在共同的病理改变,两者相结合增加了遗传代谢性疾病的检出率.部分影像学诊断困难的胆道闭锁患者,其肝组织形态学可提示胆道梗阻.
목적 연구영인담즙어적성간병간활조직검사적병리급초미병리결구특정,병결합림상자료탐토기재진단중적의의.방법 대2007-2008년재중경의과대학인동의원취진적36례담즙어적형영인간염종합정환인진행간활조직검사병수방,분석기간조직병리급초미병리특정.결과 36례영인담즙어적성간병간활조직검사적광경결과현시,간세포종창、변성、배사,다핵거세포형성,담관증생,섬유조직증생,간소협화회관구염성세포침윤위주요병리표현.간세포어담,가선강형성、우모상변성화모세담관담전형성위담즙어적적특정.7례영상학검사제외담도폐쇄병례적간조직정현전형담도경조정상.전경하상견핵개변、포질용해、염증세포침윤、효원섬유증생화용매체증다.형태학개변결합림상표현진단간당원루적병2례,니만피극병1례,분류불명지질침적병1례.결론 영인담즙어적성간병간조직보통광경화전경하존재공동적병리개변,량자상결합증가료유전대사성질병적검출솔.부분영상학진단곤난적담도폐쇄환자,기간조직형태학가제시담도경조.
Objective To study the features of histopathologic and ultrastructural pathologic changes of liver biopsy in patients with infantile cholestatic disease, and to investigate its diagnostic significance combining with the clinical data. Methods Thirty-six children diagnosed as infantile cholestatic disease and recived liver biopsy in Chongqing Medical University Children's Hospital from Jun 2007 to Oct 2008 were enrolled and the pathologic and ultrastructural pathologic changes of liver were analyzed. Results Morphologic changes under light microscope in liver tissues included hepatocyte swelling, hepatocyte denaturation,hepatocyte necrosis, multinucleated giant cell formation, bile duct proliferation, fiber tissues proliferation and inflammatory cells infiltration in liver lobules and portal regions. The characteristics of cholestasis including intralobular cholestasis, acinus formation, feather-like cytoplasmic filaments and bile stasis in bile canaliculi were observed. The morphologic changes of biliary atresia were observed in 7 cases whose image investigations showed no obstruction of biliary tract. Nuclear changes, resolution of cytoplasm, inflammatory cell infiltration, collagen fiber proliferation and increased number of lysosomes were observed under electromicroscope. Two cases of glycogen storage disease, 1 case of Niemann-Pick disease and 1 case of lipid storage disease with unknown cause were confirmed by the combination of histological changes and clinical manifestations. Conclusion Common pathologic changes of liver tissues existed under light microscope or electroscope. The diagnosis of hereditary metabolic disorders could be made increasingly by application of these two technologies in clinical practice. It is difficult to diagnose biliary atresia in early childhood by image investigations and the pathological changes of liver tissues are helpful.