文章摘要
Wilson病合并脾功能亢进患者脾切除术的临床疗效分析
Analysis of clinical efficacy of splenectomy in patients with Wilson's disease and hypersplenism
投稿时间:2025-08-27  
DOI:10.3969/j.issn.1000-0399.2026.03.003
中文关键词: Wilson病  脾功能亢进  脾切除术  倾向评分匹配  长期疗效
英文关键词: Wilson's disease  Hypersplenism  Splenectomy  Propensity score matching  Long-term efficacy
基金项目:安徽省高等学校自然科学科学研究项目(编号:2023AH050849); 大健康研究院新安医学与中医药现代化研究所专项资金资助(编号:2023CXMMTCM002)
作者单位E-mail
王乐淼 230038 安徽合肥 安徽中医药大学神经病学研究生院
230001 安徽合肥 安徽中医药大学神经病学研究所附属医院脑病四科 
 
吴大任 230038 安徽合肥 安徽中医药大学神经病学研究生院
230001 安徽合肥 安徽中医药大学神经病学研究所附属医院脑病四科 
 
王佳炜 230001 安徽合肥 安徽中医药大学神经病学研究所附属医院脑病四科  
汪世靖 230001 安徽合肥 安徽中医药大学神经病学研究所附属医院脑病四科  
徐陈陈 230001 安徽合肥 安徽中医药大学神经病学研究所附属医院脑病四科  
孙丹丹 230001 安徽合肥 安徽中医药大学神经病学研究所附属医院脑病四科  
王伟 230001 安徽合肥 安徽中医药大学神经病学研究所附属医院脑病四科  
韩永升 230001 安徽合肥 安徽中医药大学神经病学研究所附属医院脑病四科
241002 安徽芜湖 皖南医学院 
 
王训 230001 安徽合肥 安徽中医药大学神经病学研究所附属医院脑病四科 neurodoc@163.com 
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中文摘要:
      目的 评估Wilson病合并脾功能亢进患者脾切除术的短期与长期疗效。方法 回顾分析2018年1月至2024年12月就诊于安徽中医药大学神经病学研究所附属医院的130例WD合并脾功能亢进患者的临床资料,根据是否进行脾切除分为脾保留组(n=30)和脾切除组(n=100)。比较两组患者一般资料及临床特征的差异;收集脾切除组患者术前、术后短期(≤3个月)和术后长期(≥1年)的血细胞计数、肝功能、肝纤维化程度、门静脉高压及神经功能等指标,对比分析手术前后不同随访时间点的疗效变化;其次采用倾向评分匹配(PSM)平衡脾切除长期组与同期随访脾保留组的基线差异,比较脾切除组与脾保留组两组血细胞计数、肝功能ChildPugh评分、中文版统一WD评分量表(UWDRS)得分以及并发症变化。结果 脾切除组术后短期及长期与术前相比,血小板(PLT)、红细胞(RBC)、白细胞(WBC)、中性细胞计数(NEUT)、血红蛋白(Hb)均升高(P<0.05);术后长期肝功能Child-Pugh评分、凝血酶原时间(HA)、Ⅳ型胶原蛋白(CⅣ)及门静脉内径、肝脏硬度值(pSWE)均较术前改善(P<0.01),UWDRS评分较术前及术后短期下降(P<0.05)。经PSM匹配后,脾切除组术后长期在血细胞计数、Child-Pugh评分及肝硬化并发症控制上均优于脾保留组(P<0.05),两组患者UWDRS评分比较,差异无统计学意义(P>0.05)。结论 脾切除术对WD合并脾功能亢进患者疗效明确:短期显著缓解脾亢;长期持续改善肝功能、缓解肝纤维化进程、降低肝硬化并发症风险。
英文摘要:
      Objective To evaluate the short-term and long-term efficacy of splenectomy in patients with Wilson's disease and hypersplenism. Methods The clinical data of 130 patients with WD with hypersplenism who attended the Affiliated Hospital of the Institute of Neurology of Anhui University of Traditional Chinese Medicine from January 2018 to December 2024 was retrospectively analyzed, and the patients were divided into two groups according to whether splenectomy was performed, including 30 patients in the splenectomy group and 100 patients in the splenectomy group. The differences in general information and clinical characteristics between the two groups were compared. The blood counts, liver function, liver fibrosis, portal hypertension and neurological function before surgery, short-term(≤3 months) and longterm(≥1 year) after surgery were collected, and the changes in efficacy at different follow-up time points before and after surgery were compared. Secondly, the propensity score matching(PSM) was used to balance the baseline differences between the long-term splenectomy group and the spleen-sparing group at the same time, and the blood counts, liver function Child-Pugh score, Chinese version of the Unified WD Rating Scale(UWDRS) scores and complications were compared between the splenectomy group and the splen-sparing group. Results In the splenectomy group, PLT, RBC, WBC, NEUT, and Hb significantly increased in the short-term and long-term postoperative period compared with those before surgery(P<0.05). The long-term liver function after surgery, Child-Pugh score, HA, CⅣ, portal vein diameter, and pSWE were significantly improved compared with those before surgery(P<0.01), and the UWDRS score was reduced compared with that before and after surgery(P<0.05). After PSM matching, the long-term blood count, Child-Pugh score and control of cirrhosis complications in the splenectomy group were better than those in the spleen-sparing group(P<0.05), and there was no significant difference in UWDRS scores between the two groups(P>0.05). Conclusion The efficacy of splenectomy in patients with WD and hypersplenism is clear: short-term significant alleviation of hypersplenism, long-term continuous improvement of liver function, alleviation of liver fibrosis process, and reduction of the risk of complications of cirrhosis.
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