影响肝豆状核变性患者预后及疗效因素的研究

Prognostic and the effects of therapeutic factors on Wilson's disease

目的探讨可预测肝豆状核变性(WD)治疗效果及疾病预后的相关因素。方法依据Nazer肝功能评分标准及Young神经功能评分标准分别对肝型及脑型患者进行评分。根据预后将肝型患者分为肝A、肝B两组,分别分析40例肝型患者和40例脑型患者的临床表现及辅助检查结果与预后的相关性。结果肝B组患者的肝功能显著异常于肝A组,肝A、肝B两组黄疸和腹水有显著性差异,B组患者的肝功能评分全部≥7,多因素Logistic回归发现胆红素、腹水与预后的相关性有统计学意义(P<0.01,回归系数分别为0.025、3.546,95%可信区间分别为1.007～1.044,4.899～24.53);脑型患者在治疗1个月后,语言功能障碍、扭转痉挛的评分升高有显著性,治疗1年后,语言功能障碍、震颤、肌强直/运动迟缓、流涎的评分下降有显著性,吞咽功能障碍、扭转痉挛、舞蹈样不自主动作的评分无显著性差异。结论黄疸和腹水是评价肝型患者预后最有价值的两个指标,具有黄疸和腹水的病人预后较差,肝功能评分≥7的病人预后较差;脑型患者中具有吞咽功能障碍、扭转痉挛、舞蹈样不自主动作症状的患者预后较差。

Objective To find out the valuable related factors that can predict the prognosis of WD. Methods We used Nazer and Young scale methods to evaluate patients. According to its prognosis 40 liver type patients were divided respectively into two groups ： group A, group B, analyse the relationship between clinical performance, assisted check results and the prognosis. Results The liver functions of group B were significantly more abnormal than those of group A, Jaundice and ascites were significantly more common in group B, the prognosis score of group B were all≥7, in the logistic regression analysis we found that jaundice and ascites were the independent factors to the prognosis of liver type patient. After one month＇s treatment dysarthria and torsion spasm were significantly worse, after one year treatment, dysarthria,tremor, bradykinesia and salivat reversed significantly, swallowing dysfunction, torsion spasm and dancing involuntary movement reversed insignificantly, in the logistic regression analysis we found that the course of disease and neurological score have significant relationship with the prognosis. Conclusion With regard to the prognosis in Wilson disease the presence of jaundice and ascites were the only clinical signs of prognosis value, being associated with a significantly wore prognosis. The liver function score ≥7 is associated with a significautly wore prognosis. Dysarthria and torsion spasm are worsen easily, tremor, bradykinesia, salivat are easy to recover, the swallowing dysfunction, torsion spasm and dancing involuntary movement are the most resistant neurological signs.