Yasui M, Kohmoto J, Ota K, Shinmen K, Tanaka H, Nogami H
Division of Neurological Diseases, Wakayama Medical College, Japan.
No To Shinkei. 1998 Aug;50(8):767-72.
Histological findings and metal contents in the liver were studied in a patient with neurologic type of Wilson's disease. Copper and aluminum contents in the biopsied liver of the patient with Wilson's disease were measured simultaneously by neutron activation analysis at Research Reactor Institute, Kyoto University. Four cases of adult cirrhosis were selected as the control for cirrhosis and five cases of adult liver as the control for neurologically normal. The biopsied liver showed markedly increase in the copper content (814.4 micrograms/g: dry weight) and extremely high content of aluminum (479.4 micrograms/g: dry weight), compared to those of the controls. On the other hand, macroscopically no cirrhosis was observed and the characteristic appearances of macronodular cirrhosis failed to detect histologically. Interestingly the fibrosis or inflammation of the liver was seen faintly. It is likely that toxic metals in the liver such as aluminum, copper and manganese might be implicated in the pathogenesis of neurologic type of Wilson's disease.
对一名患有神经型威尔逊病的患者的肝脏组织学发现和金属含量进行了研究。通过京都大学研究反应堆研究所的中子活化分析，同时测量了威尔逊病患者活检肝脏中的铜和铝含量。选择4例成人肝硬化病例作为肝硬化对照，5例成人肝脏作为神经功能正常对照。与对照组相比，活检肝脏的铜含量显著增加（814.4微克/克：干重），铝含量极高（479.4微克/克：干重）。另一方面，宏观上未观察到肝硬化，组织学上也未检测到大结节性肝硬化的特征性表现。有趣的是，肝脏的纤维化或炎症很轻微。肝脏中的有毒金属如铝、铜和锰可能与神经型威尔逊病的发病机制有关。
