迟发型肝豆状核变性的临床特点和治疗转归.pdf

() 2009, 34 ( 1) http: / /xbyx. xysm. net 40 J C en t Sou th Un iv (M ed S ci) 张永红, 杨旭, 唐晓鹏, 罗虹雨, 雷建华 ( 中南大学湘雅二医院肝病研究中心, 长沙 4 10 011) [ ] : 探讨迟发型肝豆状核变性 (W ilson disease, WD ) 的临床特点和 疗转归, 为迟发型肝 豆状核变性的诊断和 疗提供科学依据: 分析 8例迟发型肝豆状核变性的临床特点铜代谢变化 及其 疗结果采用免疫散射比浊法测定铜蓝蛋白, 原子吸收光谱法测定血清铜尿铜和肝铜; 初期 疗用二巯基丙磺酸钠, 维持 疗用青霉胺或锌剂: 迟发型肝豆状核变性占肝豆状核变性总数的 7. 0% , 肝病型起病缓慢, 恶心纳差等不明显, 极易误诊血常规氨基转移酶多数正常, 均有 KF 环, 肝功能受损程度及铜代谢异常相差甚大二巯基丙磺酸钠 疗第 1 天平均排铜 4 07 2 g, 是 疗前的 18. 1倍, 是同等剂量青霉胺的 2. 5 倍, 未见明显副作用, 患者预后良好: 迟发型肝豆状核变性并非 少见, 极易误诊, 值得高度重视, 初期用二巯基丙磺酸钠 疗可取得良好疗效 [ ] 肝豆状核变性; KF 环; 铜蓝蛋白; 尿铜; 二巯基丙磺酸钠 [ ] R 575. 24 [ ] A [ ] 167 27347 ( 200 9 ) 0 100 400 5 Clinical character and therapeutic effect of lateonsetW ilson disease ZHANG Y onghong, YANG X , TANG X iaopeng, LUO Hongy , LEI Jianh a ( R esearch Center f or L iver D iseases, Second X iangya H osp ital, Central South Un iv er sity, Chang sha 4 10011, Ch ina ) Abstract: Objective To investigate the clin ical character and therape tic effect of lateonset W ilson disease, and to provide some evidence for its d iagnosis and treatment. ethods Clin ical character, changes of copper metabolism, and therape tic effect of 8 patients w ith lateonset W ilson disease w ere analyzed. Cer lop lasm in level w as meas red by nephelom etry, and the copper contents in the ser m, rine, and liver w ere meas red by flam e atom ic absorption spectroscopy. The in itial treatment w as sod i m dmi ercaptos lphonate, follow ed by D pen icillam ine and / or zinc. Results Pa tients w ith lateonsetW ilson disease acco nted for 7. 0% of all patients, Who presented live