肝豆状核变性患者肝纤维化水平与其临床表型、Child分级关系的研究

Study on the Level of Hepatic Fibrosis in Wilson's Disease Patients,and the Relatives to the Clinical Manifestation-trpes and the Child-Pugh Scores

目的 :探讨肝豆状核变性 (WD)患者的血清HA、LN、C Ⅳ及PCⅢ水平与其临床表型和Child Pugh分级的关系。方法 :对 85例首次住院未经驱铜治疗的WD患者及 3 0例健康者 (对照组 )分别使用放射免疫法 (RIA)检测HA、LN、C Ⅳ、PCⅢ ;并对WD患者采用Child Pugh分级 (A、B、C)、区分临床表型 (脑型、脑 内脏型、肝型 ) ,以了解其与肝纤维化水平的关系。结果 :WD患者的血清HA、LN、C Ⅳ、PCⅢ水平较健康对照组明显升高 ,在肝型患者中最高 ,其次是脑 内脏型 ,而脑型升高最低 ;在Child Pugh分级中以C级患者最高 ,B级次之 ,A级最轻。结论 :WD患者均存在血清HA、LN、C Ⅳ、PCⅢ水平不同程度的增高 ,其增高水平与临床表型、Child Pugh分级相一致。

Objective: To evaluate the level of serum HA?LN?C-Ⅳ and PCⅢ in Wilson's Disease(WD) paitents;the correlation of the level of serum HA?LN?C-Ⅳ and PCⅢ with the clinical manifestation-types and the Child-Pugh scores. Methods: Serum HA?LN?C-Ⅳ and PCⅢ were measured by radioimmunoassay(RIA)in 85 patients with WD and 30 healthy control group.85 patients with WD were divided into 3 groups(A?B?C) according to the Child-Pugh scores, and were divided into cerebral?hepatic and cerebral-internal organ types according to the main clinical manifestations. Results:The level of serum HA?LN?C-Ⅳ and PCⅢ in WD patietns increased significntly compared to the control group( P <0.01);The level of serum HA?LN?C-Ⅳ and PCⅢ in hepatic type and C group was higher than that in cerebral-internal organ type and B group, the lowest level of serum HA?LN?C-Ⅳ and PCⅢ was in cerebral type and A group. Conclusion: All the WD patients had high degree of serum HA?LN?C-Ⅳ?PCⅢ, which had significantly correlations to the Child-Pugh scores and the clinical manifestation-types.