Sallie R, Chiyende J, Tan K C, Bradley D, Portmann B, Williams R, Mowat A P, Mieli-Vergani G
Institute of Liver Studies, King's College Hospital, London.
Gut. 1994 Jun;35(6):849-53. doi: 10.1136/gut.35.6.849.
Fulminant hepatic failure resulting from hepatitis E and coexistent Wilson's disease was diagnosed in a six year old girl six weeks after returning from a holiday in India. Wilson's disease was diagnosed on the basis of histological evidence of hepatocellular copper deposition, confirmed by biochemical estimation of liver copper concentration. Although severely damaged, the liver was non-cirrhotic. Hepatitis E virus (HEV) was diagnosed by nested polymerase chain reaction, the specificity of which was confirmed by direct sequencing of amplified DNA. Replication of HEV within the liver at the time of diagnosis was confirmed by selective amplification of the antigenomic strand of the virus obtained from total liver RNA. The patient had an orthotopic liver transplantation without recurrence of hepatitis and remains well at 19 months. Viral excretion, recorded by serial amplification of HEV RNA extracted from stool samples, persisted for 30 days after liver grafting. Severe vitiligo, present preoperatively, dramatically improved after liver grafting and institution of immunosuppressive treatment. This case suggests that viral infection may play a part in the acute decompensation seen in some cases of Wilson's disease.
一名六岁女孩在从印度度假归来六周后,被诊断为戊型肝炎导致的暴发性肝衰竭并合并威尔逊病。威尔逊病是根据肝细胞铜沉积的组织学证据诊断的,并通过肝脏铜浓度的生化测定得到证实。尽管肝脏严重受损,但未出现肝硬化。通过巢式聚合酶链反应诊断出戊型肝炎病毒(HEV),通过对扩增DNA的直接测序证实了其特异性。通过从总肝RNA中选择性扩增病毒的反基因组链,证实了诊断时HEV在肝脏内的复制。该患者接受了原位肝移植,肝炎未复发,在19个月时情况良好。通过对粪便样本中提取的HEV RNA进行连续扩增记录的病毒排泄,在肝移植后持续了30天。术前出现的严重白癜风在肝移植和开始免疫抑制治疗后显著改善。该病例表明病毒感染可能在某些威尔逊病病例中出现的急性失代偿中起作用。