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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 investigate the liver function and hepatic pathological changes in patients with hepatolenticular degeneration. Methods Retrospective analysis of 9 cases of hepatolenticular degeneration in patients with clinical data and liver lesions. Results 3 cases of normal liver function, 6 cases of mild liver dysfunction. Pathological changes of liver tissue vary greatly, showing varying degrees of fibrosis, necrosis, inflammatory cell infiltration, mild to severe steatosis. Conclusion The pathological changes of liver in patients with hepatolenticular degeneration are more serious than the clinical manifestations. The early fatty degeneration of hepatocytes may be associated with inflammatory cell infiltration and hepatic fibrosis.