目的了解肝豆状核变性误诊的原因。
Objective: To detect the reasons of misdiagnosis of Wilson disease.
目的了解肝豆状核变性误诊的原因。
Objective: To detect the reasons of misdiagnosis of Wilson′s disease.
目的探讨青霉胺治疗肝豆状核变性的最佳方案。
Objective To determine the best plan with penicillamine in treating the patients with hepatolenticular degeneration.
目的评价MRI对诊断肝豆状核变性(HLD)的意义。
Objective to assess the significance of MRI in the diagnosis of hepatolenticular degeneration (HLD).
目的探讨活体肝移植治疗小儿肝豆状核变性的临床疗效。
Objective To explore the efficacy of living donor liver transplantation in the treatment of children with Wilson's disease who had severe hepatic insufficiency.
目的回顾性分析肝豆状核变性的MRI诊断和主要鉴别诊断。
Objective MRI diagnosis and main differential diagnosis of hepatolenticular degeneration was analysed retrospectively.
目的总结肝豆状核变性并肾损害患者临床表现、诊断及治疗。
Objective To summarize clinical manifestation, diagnosis and treatment of wilson disease complicated with renal impairment.
还发现在汉字的认知加工过程中有豆状核、丘脑及小脑的参与。
Putamen, thalamus and cerebellum were found to involve in the cognitive process of Chinese characters.
目的总结亲属活体供肝移植治疗肝豆状核变性时选择供者的经验。
Objective To summarize experience of selection of donors of living related liver transplantation (LRLT) for Wilson's Disease.
全穿刺导致椎间盘高度下降，糖胺聚糖含量下降，和更高的豆状核变性。
Penetrative puncture resulted in a faster decrease in disc height, lower glycosaminoglycan content, and higher grades of histologic degeneration.
结果肝豆状核变性所致精神障碍以精神症状为首发病时，极易造成误诊。
Results Misdiagnoses is extremely easy to occur during the arise of Wilson s degeneration with dysphrenia heading by mental symptoms.
比较青霉胺及锌剂治疗肝豆状核变性（HLD）的疗效及对尿铜排泄的作用。
PURPOSE To compare the efficacy and urinary copper excretion in the treatment of hepatolenticular degeneration (HLD) with penicillamine and zinc.
目的分析小儿肝豆状核变性临床误诊的原因以及因误诊而对治疗效果的影响。
Objective To find out the relationship between curative effect and misdiagnosis of children with hepatolenticular degeneration(HLD).
目的比较和分析肝豆状核变性和病毒性肝炎患者铜代谢的差异及其临床意义。
Objective To compare and analyse the difference of copper metabolism in groups of WD (Wilson 's disease) and viral hepatitis patients.
方法：运用中医学络病理论与现代医学阐述肝豆状核变性毒损脉络的基本病机。
Methods: the basic pathogenesis process of the toxin impairing collaterals of hepatolenticular degeneration was described by the TCM collateral disease theory and modern medicine.
CT扫描显示，双侧大脑基底节区，尾状核、豆状核及丘脑内有对称性钙化灶。
CT scans revealed the bilateral and symmetric calcifications of the basal ganglia, caudate nucleus, lentiform nucleus and thalami.
研究脑脊液和血清中烯醇化酶（NSE）在肝豆状核变性（WD）中的临床意义。
To investigate the clinical significance of the neuron specific enolase (NSE) in cerebrospinal fluid (CSF) and serum of Wilson disease (WD).
目的分析肝豆状核变性（WD）ATP7B基因8号外显子在中国人中的突变特点。
Objective To analyze the mutation characterization in exon 8 of ATP7B gene in Chinese Patients with Wilson disease(WD).
目的探讨微量元素铜、锌及铜氧化酶含量与肝豆状核变性诊断及治疗转归的关系及临床意义。
Objective To explore the relationship and clinical meaning between trace chemical elements such as copper, zinc, copper-oxygenase and wilson(WD), diagnosis and treatment.
结论心理护理、人文关怀能提高肝豆状核变性患者的心理状况，促进HLD患者的全面康复。
Conclusion Humanistic care and mental nursing can improve overall mental health status of patients with HLD and promote comprehensive rehabilitation of them.
目的探讨背驼式肝移植术，解决肝豆状核变性铜代谢障碍，延长患者生存时间，改善患者的生存质量。
Objective Hepatic transplantation was performed on patients with Wilson disease to correct the disorder of copper metabolism, and to prolong lifetime and improve living quality.
材料和方法：对临床和生化证实30例肝豆状核变性的儿童进行治疗前后的脑部及腹部的MRI检查。
Materials and Methods:Pretreatment and follow up brain and abdomen MRI were performed in30children with clinically-and biochemically-proved HLD.
方法：对临床及实验室检查证实的110例肝豆状核变性患者均进行颅脑ct平扫，部分给予增强扫描。
Methods: 110 patients with HLD confirmed by clinical and laboratory data were performed plain CT scanning, and some of them underwent additional contrast enhanced CT.
结论：1中央前沟首次出现在豆状核及内囊前肢出现而前连合及背侧丘脑尚未出现的1 ~2个断面上。
Conclusion: the precentral sulcus appears on the 1 2 sections that the lenticular nucleus and anterior limb of internal capsule appear but the anterior commissure and dorsal thalamus doesn't appear.
目的研究中国人肝豆状核变性（WD）基因12号外显子的突变特征，为建立直接基因诊断的方法提供理论依据。
Objectives To study the feature of disease causing mutation of exon 12 of Wilson disease (WD) gene in Chinese and evaluate its value in direct gene diagnosis.
结果脾切除、贲门周围血管离断术对于治疗肝豆状核变性病人的上消化道出血、脾功能亢进效果明显，有利于恢复驱铜药物治疗。
Results After the treatment, the hemorrhage of upper digestive tract and hypersplenism were relieved and the patients resumed the pharmaceutical therapy for removing copper.
结论血铜含量及铜氧化酶含量对肝豆状核变性确诊有良好的指导意义，尿铜含量可帮助医生对临床疗效转归和用药选择做出正确评价。
Conclusion The content of serum copper and copper-oxygenase is of meaningful. The content assay of urine copper can help doctors choose drug and come to right evaluation.
结论血铜含量及铜氧化酶含量对肝豆状核变性确诊有良好的指导意义，尿铜含量可帮助医生对临床疗效转归和用药选择做出正确评价。
Conclusion The content of serum copper and copper-oxygenase is of meaningful. The content assay of urine copper can help doctors choose drug and come to right evaluation.
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