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目的:研究Wilson病(WD)患者的血清铁代谢相关指标水平。方法:选取未接受过正规驱铜治疗的WD患者103例(WD组),WD组再依据MRI异常分为3个亚组:WD-MRI无异常组、WD-MRI长T2组和WD-MRI短T2组。另选取健康志愿者25名(正常对照组)和乙肝患者18例(乙肝对照组)为研究对象。WD组患者于入院时进行血清铜蓝蛋白以及血清铁代谢指标测定;正常对照组及乙肝对照组均检测铁代谢指标。结果:WD各亚组高铁铁蛋白水平与正常对照组比均显著增高,差异有统计学意义(P<0.05);与乙肝对照组比较则显著降低(P<0.01)。WD组转铁蛋白明显降低,与正常对照组和乙肝对照组比较,均差异有显著统计学意义(均P<0.01)。WD各亚组的人转铁蛋白受体与乙肝对照组比较,差异有显著统计学意义(P<0.01);乙肝对照组与正常对照组比较,差异有显著统计学意义(P<0.01)。结论:WD患者存在铁代谢异常,但不同于乙肝对照组的人转铁蛋白受体高表达,可能是转铁蛋白的低调节所致。
Objective: To study the level of serum iron metabolism in patients with Wilson’s disease (WD). Methods: A total of 103 WD patients were enrolled in this study. WD group was divided into 3 subgroups according to MRI abnormalities: WD-MRI non-abnormal group, WD-MRI long T2 group and WD-MRI Short T2 group. Another 25 healthy volunteers (normal control group) and 18 patients with hepatitis B (hepatitis B control group) as the research object. Patients in WD group were tested for serum ceruloplasmin and serum iron metabolism index at admission; iron metabolism index was detected in normal control group and hepatitis B control group. Results: The levels of ferritin in WD subgroups were significantly higher than those in normal control group (P <0.05), but were significantly lower than those in control group (P <0.01). Transferrin in WD group was significantly lower than that in normal control group and control group (all P <0.01). There were significant differences between WD subgroups of human transferrin receptor and hepatitis B control group (P <0.01). There was significant difference between hepatitis B control group and normal control group (P <0.01). Conclusion: There is abnormal iron metabolism in patients with WD, but high expression of human transferrin receptor in hepatitis B control group may be due to the low regulation of transferrin.