摘要
目的:明确重庆地区胆汁淤积型婴儿肝炎综合征(Infantile hepatitis syndromeI,HS)的病因诊断。方法:ELISA常规检测血清HCMV-IgM、弓形体-IgM、RuV-IgM、HSV-IgM、甲乙丙戊肝炎病毒标志物,肝活检行光镜和电镜诊断,免疫组织化学染色检测肝组织HCMV极早期抗原(Immediate earlyantigenI,EA)和早期抗原(Earlyantigen,EA)、HBcAg、胆盐输出泵(Bile salt exportpump,BSEP)和多重耐药相关蛋白3(Multidrug resistance 3,MDR3)。结果:36例胆汁淤积型IHS中人巨细胞病毒(Human cy-tomegalovirus,HCMV)肝炎10例;HCMV肝炎合并风疹病毒(Rubella virus,RuV)感染1例;HCMV肝炎合并单纯疱诊病毒(Her-pds simplex virus,HSV)感染1例;HCMV、HSV和RuV混合感染1例;胆道闭锁7例[5例合并HCMV感染,1例合并HCMV和乙肝病毒(Hepatitis b virus,HBV)感染];糖原累积病2例;尼曼皮克病合并HCMV肝炎1例;分类不明脂质代谢障碍1例;HCMV肝炎合并疑诊线粒体肝病1例;HCMV肝炎合并疑诊铁沉积病1例;疑诊脂质代谢障碍、胆汁酸代谢障碍和进行性家族性肝内胆汁淤积Ⅰ型(Progressive familial intrahepatic cholestasis,PFICⅠ)各1例;7例病因仍不清。结论:HCMV肝炎是重庆地区胆汁淤积型IHS的首要原因,存在与其他病毒合并感染和参与代谢性肝病发病的现象。肝组织学检查可作为早期诊断胆道闭锁的补充,电镜检查有助于某些遗传代谢性肝病的诊断。暂未在重庆地区胆汁淤积型IHS中发现BSEP和MDR3基因变异。
Objective：To understand the causes of cholestatic infantile hepatitis syndrome （IHS）in Chongqing area. Methods：Serum HCMV-IgM,Tox-IgM,RuV-IgM,HSV-IgM and hepatitis virus markers（A,B,C and E）were detected by ELISA. Liver biopsy tissues were observed under light microscope and electromicroscope. Immediate early antigen（IEA）and early antigen（EA）of HCMV,HbcAg, BSEP and MDR3 were detected in liver tissues by immunohistochemistry stain assay. Results：The diagnoses in 36 patients with cholestatic IHS included HCMV hepatitis in 10 patients,HCMV and RuV co-infection in 1 patient,HCMV,HSV and RuV co-infection in 1 patient,Biliary atresia in 7 patients（5 complicated with HCMV infection and 1complicated with HCMV and HBV co-infection）,Glycogen storage disease in 2 patients,Niemann-Pick disease and HCMV hepatitis in 1 patient,and Lipid storage diseases with unknown type in 1 patient. Probable diagnoses included liver mitochondriopathy and HCMV hepatitis in 1 patient ,siderosis and HCMV hepatitis in 1 patient,dyslipidoses in 1 patient,bile acid synthetic defect in 1 patient and progressive familial intrahepatic cholestasis Ⅰin 1 patient. There were also 7 patients the causes did not found. Conclusion：HCMV hepatitis is the main cause of infantile hepatitis syndrome in Chongqing , but it may be co-infected with other virus and complicated with inheritant metabolism liver diseases. The pathologic changes of liver tissues are helpful in diagnoses of biliary atresia and inheritant metabolism liver diseases in cholestatic IHS. BSEP and MDR3 defect were not found in cholestatic IHS in Chongqing area.
出处
《重庆医科大学学报》
CAS
CSCD
北大核心
2010年第10期1547-1549,共3页
Journal of Chongqing Medical University
