小儿非胆道闭锁胆汁淤积性肝病超声与病理学特征对照

Correlative analysis of ultrasonographic and pathologic features in children with non-biliary atresia cholestatic diseases

目的:研究小儿非胆道闭锁(BA)胆汁淤积性肝病的超声、组织病理和超微病理特征,探讨其诊断意义。方法:对26例超声引导下肝穿活检及基因学检查的非BA胆汁淤积性肝病患儿进行回顾性研究,分析其超声、组织病理和超微病理特征。结果:26例非BA胆汁淤积性肝病患儿超声提示肝脏回声增强8例,肝内Glisson系统管壁增厚4例,肝脏回声增粗3例,单纯性肝肿大5例,肝无异常6例。普通病理包括炎性细胞浸润18例、肝细胞水样变性14例,肝细胞淤胆13例,其他主要为肝细胞肿胀,脂肪变性,纤维组织增生等。电镜超微结构显示肝细胞淤胆20例,毛细胆管扩张17例,糖原增多15例,其余以肝细胞脂肪变性、纤维组织增生及炎性细胞浸润等为主。结论:超声可反映非BA患儿各种病因导致的肝脏病理状态,指导临床诊断及判断预后.肝穿活检尤其是肝组织超微病理结构可明显提高遗传代谢病诊断率。

Objective：To study the correlation between the features of ultrasonography,histopathology and ultrastrucrural pathologic changes in children with non-biliary atresia （BA） cholestatic diseases. Methods： 26 children with non-biliary atresia （BA） eholestatic diseases were received liver biopsy guided by ultrasonography. The children were scanned by ultrasound before the biopsy, and the tissues obtained by biopsy were send for pathology or gene examination. Results： Ultrasonography of 26 children showed hyperechoic liver parenchyma in 8 cases, Glisson system wall thickening in 4 cases, coarse parenchyma in 3 cases,hepatomegaly in 5 cases,and no special changes in other 6 cases. Ordinary pathologic reports includ- ed 18 cases of inflammatory cell infiltration, 14 hepatocyte hydropic degeneration, 13 cholestasis,and others included hepatocyte swelling, fatty degeneration and fiber tissues proliferation. Ultrastructural pathologic changes showed 20 cases of hepatocyte cholestasis, 17 dilation of cholangiole and 15 increased glycogen. Other findings were as follows：fatty degeneration, fibrous tissues proliferation and inflammatory cell infiltration. Conclusion：US features can reflect the liver pathological state, and these can guide clinical diagnosis and estimate prognosis. The diagnosis of hereditary metabolic disorders could be made by liver biopsy,especially the ultrastructure pathology.