摘要
目的探讨肝豆状核变性患者肝功能及肝脏病理学改变。方法回顾性分析9例肝豆状核变性患者临床资料及肝脏病变特点。结果3例肝功能正常,6例肝功能轻度异常。肝组织病理改变差异极大,可见不同程度纤维化、细胞坏死、炎性细胞浸润、轻至重度脂肪变性。结论肝豆状核变性患者肝脏病理学改变程度较临床表现更为严重;早期的肝细胞脂肪变性可与不同程度炎性细胞浸润、肝纤维化同时存在。
Objective To explore the correlation of liver function and liver pathology in patients with Wilson＇s disease. Methods Clinical data and pathological changes of 9 patients with Wilson＇s disease were analyzed retrospectively. Results The liver function were mildly paranormal in 6 patiems and normal in 3 patiems. The pathological features of the liver biopsy showed various degree fibrosis and cellular necrosis associated with inflammatory infiltrates, mild to severe steatosis. Conclusions The pathologic changes of liver in patients with Wilson＇s disease are more severe than clinical manifestation. The earliest changes including glycogen deposition in the nuclei of periportal hepatocytes and fatty infiltration combined with various degree fibrosis and cellular necrosis associated with inflammatory infiltrates are detectable on light microscopy in patients with Wilson＇s disease.
出处
《中华消化杂志》
CAS
CSCD
北大核心
2007年第12期817-819,共3页
Chinese Journal of Digestion
关键词
肝豆状核变性
肝脏
病理
Hepatolenticular degeneration
Liver
Pathology
