肝豆状核变性、非酒精性脂肪性肝病和自身免疫性肝炎儿童肝组织超微结构变化特征

Hepatic ultrastructural features of paediatric Wilson’s disease,NAFLD and autoimmune hepatitis

目的 分析比较肝豆状核变性(WD)、非酒精性脂肪性肝病(NAFLD)和自身免疫性肝炎(AIH)儿童肝组织超微结构变化特征。方法 2018年12月~2022年2月我院诊治的WD患儿37例、NAFLD患儿20例和AIH患儿17例,均接受超声引导下肝活检,行常规组织病理学和超微结构观察。结果 WD组肝纤维化和铜染色阳性占比分别为78.4%和29.7%,显著高于NAFLD组的10.0%和0.0%(P<0.05),而巨泡状脂肪变性和小脂滴比例分别为62.2%和75.7%,显著低于NAFLD组的95.0%和95.0%(P<0.05);WD组肝组织肝细胞核空泡样变比例为59.5%,显著高于AIH组的17.6%(P<0.05),而门脉炎症、界面性肝炎、小叶内炎症、浆细胞浸润(>30%)和肝细胞玫瑰花样结构比例分别为78.4%、10.8%、64.9%、5.4%和24.3%,显著低于AIH组的100.0%、88.2%、82.4%、64.7%和76.5%,差异均具有统计学意义(P<0.05);在超微结构方面,WD组线粒体多形性、嵴尖端扩张、膜复制和基质密度增高比例分别为81.1%、83.8%、62.2%和62.2%,显著高于NAFLD组的30.0%、15.0%、5.0%和5.0%(P<0.05)或AIH组的23.5%、11.8%、5.9%和5.9%(P<0.05),而夹杂准晶体比例为0.0%,显著低于NAFLD组的15.0%(P<0.05)。结论 WD、NAFLD和AIH患儿肝组织具有各自特征性的病理学变化,尤其是超微结构的特征变化可能有助于区分和鉴别诊断。

Objective The purpose of this study was to investigate the ultrastructural changes of liver tissues in children with Wilsons disease(WD),non-alcoholic fatty liver diseases(NAFLD)and autoimmune hepatitis(AIH).Methods 37 children with WD,20 children with NAFLD and 17 children with AIH were encountered in our hospital between December 2018 and February 2022,and all children underwent liver biopsies for routine and ultrastructural examination.Results The proportion of liver fibrosis and copper staining positive in children with WD were 78.4%and 29.7%,significantly higher than 10.0%and 0.0%(P<0.05)in children with NAFLD,while the proportion of macrovesicular steatosis and lipid droplets were 62.2%and 75.7%,significantly lower than 95.0%and 95.0%(P<0.05)in children with NAFLD;the proportion of hepatocyte nucleus vacuolar degeneration in children with WD was 59.5%,much higher than 17.6%(P<0.05)in children with AIH,while the proportion of portal inflammation,interfacial hepatitis,lobular inflammation,plasma cell infiltration and hepatocyte rosette structure were 78.4%,10.8%,64.9%,5.4%and 24.3%,significantly lower than 100.0%,88.2%,82.4%,64.7%and 76.5%in children with AIH(P<0.05);as for the ultrastructural changes,the proportion of mitochondrial pleomorphism,crest tip dilatation,membrane replication and increased matrix density in children with WD were 81.1%,83.8%,62.2%and 62.2%,significantly higher than 30.0%,15.0%,5.0%and 5.0%(P<0.05)in children with NAFLD or 23.5%,11.8%,5.9%and 5.9%(P<0.05)in children with AIH,while the proportion of quasicrystal inclusion was 0.0%,much lower than 15.0%(P<0.05)in children with NAFLD.Conclusion The liver histo-pathological changes,including ultrastructural features,in children with WD,NAFLD and AIH are specific,which could provide evidences for diagnosis of them.