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目的分析肝豆状核变性患者的临床特点,提高对本病的认识,为早期诊断该病提供依据。方法回顾性分析了我院24例肝豆状核变性患者的临床资料及诊治过程。结果 24例患者中K-F环检查阳性21例(87.5%);血清铜蓝蛋白均降低(100%),24h尿铜测定19例升高(79.2%),血清铜18例下降(75%),头部CT/MRI检查,基底节病变10例,双侧丘脑、脑干异常信号3例,8例脑萎缩,5例未见异常,经治疗12例患者出现不同程度的好转,10例患者病情无明显变化,1例患者病情较前加重,1例患者治疗期间因肝脏衰竭死亡。结论肝豆状核多青少年发病,临床表现复杂多样,误诊率较高,应提高对本病认识,早期诊治该病。
Objective To analyze the clinical features of patients with hepatolenticular degeneration and to improve their understanding of the disease so as to provide evidence for the early diagnosis of the disease. Methods Retrospective analysis of 24 cases of Wilson’s disease in our hospital clinical data and diagnosis and treatment process. Results Twenty-one patients (87.5%) were positive for KF-ring examination in 24 patients. Serum ceruloplasmin was reduced (100%), 24h urinary copper (19.2%) and serum copper Head CT / MRI, basal ganglia lesions in 10 cases, bilateral thalamus, brain stem abnormal signal in 3 cases, 8 cases of brain atrophy, 5 cases without abnormalities, the treatment of 12 patients showed varying degrees of improvement, 10 patients with the disease No significant change, one patient worse than the previous condition, one patient died of liver failure during treatment. Conclusions There are many adolescents with hepatolenticular disease. The clinical manifestations are complex and diverse, and the misdiagnosis rate is high. Therefore, we should know more about the disease and diagnose and treat the disease early.