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分析16例肝豆状核变性患者的临床表现。结果表明发病年龄为15．8±8．3岁，首发症状以神经系统（56．25％）和消化系统（25％）为主；在疾病过程中有80％以上患者仍表现为上述两系统症状，其中以肢体震颤和肝损害为多见。几乎100％的患者出现血清铜和铜蓝蛋白含量减低、24小时尿铜含量升高及眼K－F环阳性；62．5％的患者经D－青霉胺等治疗有效。结论：肝豆状核变性主要发生于青少年，以神经和消化系统为主要表现；血清铜、铜蓝蛋白、尿铜及眼K－F环是其特征性改变。
Analysis of 16 cases of Wilson’s disease in patients with clinical manifestations. The results showed that the onset age was 15.8 ± 8.3 years. The first symptom was mainly nervous system (56.25%) and digestive system (25%). More than 80% of the patients in the course of the disease still showed the above two systems Symptoms, including limb tremor and liver damage are more common. Almost 100% of patients showed decreased serum copper and ceruloplasmin levels, elevated urinary copper levels at 24 hours, and positive K-F ring in the eye; 62.5% were treated with D-penicillamine. Conclusion: Wilson’s disease mainly occurs in adolescents with neurological and digestive system as the main manifestations. Serum copper, ceruloplasmin, urinary copper and K-F ring are the characteristic changes.