中文｜English
ISSN 1001-5256 (Print)
ISSN 2097-3497 (Online)
CN 22-1108/R
Volume 39 Issue 9
Sep.  2023
Turn off MathJax
Article Contents
Article Navigation >  Journal of Clinical Hepatology  > 2023  >  39(9): 2145-2150

Effect of dimercaptosuccinic acid combined with zinc sulfate versus D－penicillamine in treatment of children with Wilson’s disease

DOI: 10.3969/j.issn.1001-5256.2023.09.017
  • 1.

    The Second Medical School of Southern Medical University，Guangzhou 510000，China

  • 2.

    Department of Hepatology，The Fifth Medical Center of Chinese PLA General Hospital，Beijing 100000，China

  • 3.

    Department of Pediatrics，Minle Maternal and Child Health Hospital，Zhangye，Gansu 734000，China

Research funding:

Subject of Capital Characteristics (Z181100001718030);

Captial Medical Development Fund (2022－1－2182)

More Information
  • Corresponding author: WANG Fuchuan， wfc－20002000@163.com （ORCID： 0009－0008－2320－0220）; ZHANG Min， gcmw2001@163.com （ORCID： 0000－0003－2497－6748）
  • Received Date: 2022-12-04
  • Accepted Date: 2023-02-09
  • Published Date: 2023-09-19
    Abstract
  •   Objective  To investigate the effect of dimercaptosuccinic acid （DMSA） combined with zinc sulfate versus D－penicillamine （DPA） monotherapy in the treatment of children with Wilson’s Disease （WD）， and to provide a reference for clinical medication.  Methods  A total of 111 children with WD， aged 2－18 years， who were admitted to the Fifth Medical Center of Chinese PLA General Hospital from May 2018 to May 2021 were enrolled in this study and were randomly divided into DMSA+zinc sulfate treatment group （combination group with 52 children） and DPA monotherapy group （monotherapy group with 59 children）， and the two groups were compared in terms of liver function parameters and 24－hour urinary copper at baseline and 6 and 12 months of treatment. The Mann－Whitney U test was used for comparison of continuous data between two groups， and the Wilson rank sum test was used for comparison within each group； the chi－square test or the Fisher’s test was used for comparison of categorical data between two groups.  Results  At 12 months of treatment， the combination group had a significantly lower loss to follow－up rate due to adverse drug reactions than the monotherapy group ［3.85% （2/52） vs 20.34% （12/59）， χ2=6.821， P=0.009］. At 6 and 12 months of treatment， both the combination group and the monotherapy group had significant improvements in alanine aminotransferase and aspartate aminotransferase （all P<0.001）， while there were no significant differences between the two groups after treatment （all P>0.05）. Both groups had an increase in 24－hour urinary copper at 6 months of treatment and a reduction at 12 months of treatment， with a level of 123.00 （80.25－204.04） μg/24 h in the combination group and 239.50 （171.50－490.25） μg/24 h in the monotherapy group， and the monotherapy group had a significantly higher level of 24－hour urinary copper than the combination group （Z=－3.090， P=0.002）. For both groups at 12 months of treatment， there was a reduction in LSM in the children with a liver stiffness measurement （LSM） of ≥7.3 kPa； LSM was reduced by 1.8 （0.3－7.2） kPa in the 22 children in the combination group and was reduced by 2.2 （0.9－7.0） kPa in the 13 children in the monotherapy group； the children with a reduction in LSM accounted for 81.82% and 84.62%， respectively， in the two groups， with no significant difference between the two groups （χ2=0.127， P=0.721）.  Conclusion  In the treatment of WD， although DMSA combined with zinc sulfate has lower urinary copper excretion than DPA monotherapy， there is no significant difference in clinical efficacy between the two regimens， and DMSA combined with zinc sulfate has better safety than DPA monotherapy.

     

    • FullText(HTML)
  • loading
    • References(13)
  • [1]
    Inherited Metabolic Liver Disease Collaboration Group, Chinese Society of Hepatology, Chinese Medical Association. Guidelines for the diagnosis and treatment of hepatolenticular degeneration(2022 edition)[J]. Chin J Hepatol, 2022, 30( 1): 9－ 20. DOI: 10.3760/cma.j.cn501113－20211217－00603.

    中华医学会肝病学分会遗传代谢性肝病协作组. 肝豆状核变性诊疗指南(2022年版)[J]. 中华肝脏病杂志, 2022, 30( 1): 9－ 20. DOI: 10.3760/cma.j.cn501113－20211217－00603.
    [2]
    REN MS, ZHANG Z, CAI YL, et al. Comparison of long－term therapeutic effects between succimer and penicillamine in hepatolenticular degeneration[J]. Chin J New Drugs Clin Remed, 2000, 19( 3): 166－ 169. DOI: 10.3969/j.issn.1007－7669.2000.03.003.

    任明山, 张志, 蔡永亮, 等. 二巯丁二酸与青霉胺长期维持治疗肝豆状核变性疗效比较[J]. 中国新药与临床杂志, 2000, 19( 3): 166－ 169. DOI: 10.3969/j.issn.1007－7669.2000.03.003.
    [3]
    KALITA J, KUMAR V, RANJAN A, et al. Role of oxidative stress in the worsening of neurologic Wilson disease following chelating therapy[J]. Neuromolecular Med, 2015, 17( 4): 364－ 372. DOI: 10.1007/s12017－015－8364－8.
    [4]
    BREWER GJ, ASKARI F, DICK RB, et al. Treatment of Wilson’s disease with tetrathiomolybdate: V. Control of free copper by tetrathiomolybdate and a comparison with trientine[J]. Transl Res, 2009, 154( 2): 70－ 77. DOI: 10.1016/j.trsl.2009.05.002.
    [5]
    LI WJ, WANG XP. The therapeutic drug for Wilson’s disease, dimercaptosuccinic acid(sodium)[J]. World Clin Drugs, 2012, 33( 9): 574－ 576.

    李文杰, 王晓平. 肝豆状核变性治疗药物二巯丁二酸(钠)[J]. 世界临床药物, 2012, 33( 9): 574－ 576.
    [6]
    KEMP W, LEVY M, WELTMAN M, et al. Australian Liver Association(ALA) expert consensus recommendations for the use of transient elastography in chronic viral hepatitis[J]. J Gastroenterol Hepatol, 2015, 30( 3): 453－ 462. DOI: 10.1111/jgh.12865.
    [7]
    ZHOU SM, GUO LP, CAI WF, et al. Latest advances in the treatment of hepatolenticular degeneration[J]. J Clin Hepatol, 2020, 36( 1): 218－ 221. DOI: 10.3969/j.issn.1001－5256.2020.01.052.

    周思敏, 郭丽萍, 蔡王锋, 等. 肝豆状核变性的治疗现状[J]. 临床肝胆病杂志, 2020, 36( 1): 218－ 221. DOI: 10.3969/j.issn.1001－5256.2020.01.052.
    [8]
    XIE JJ, WU ZY. Wilson’s disease in China[J]. Neurosci Bull, 2017, 33( 3): 323－ 330. DOI: 10.1007/s12264－017－0107－4.
    [9]
    LI XH. Medical treatment of Wilson’s disease[J]. J Intern Med Concepts Pract, 2021, 16( 5): 289－ 293. DOI: 10.16138/j.1673－6087.2021.05.001.

    李洵桦. 肝豆状核变性的内科治疗[J]. 内科理论与实践, 2021, 16( 5): 289－ 293. DOI: 10.16138/j.1673－6087.2021.05.001.
    [10]
    CHEN DW, ZHANG M. Advance in diagnostic and treatment of Wilson’s disease[J]. Infect Dis Info, 2019, 32( 2): 158－ 161. DOI: 10.3969/j.issn.1007－8134.2019.02.016.

    陈大为, 张敏. 肝豆状核变性诊疗新进展[J]. 传染病信息, 2019, 32( 2): 158－ 161. DOI: 10.3969/j.issn.1007－8134.2019.02.016.
    [11]
    BREWER GJ, DICK RD, JOHNSON VD, et al. Treatment of Wilson’s disease with zinc: XV long－term follow－up studies[J]. J Lab Clin Med, 1998, 132( 4): 264－ 278. DOI: 10.1016/s0022－2143(98)90039－7.
    [12]
    Chinese Foundation for Hepatitis Prevention and Control; Chinese Society of Infectious Disease and Chinese Society of Hepatology, Chinese Medical Association; Liver Disease Committee of Chinese Research Hospital Association. Consensus on clinical application of transient elastography detecting liver fibrosis: a 2018 update[J]. Chin J Hepatol, 2019, 27( 3): 182－ 191. DOI: 10.3760/cma.j.issn.1007－3418.2019.03.004.

    中国肝炎防治基金会, 中华医学会感染病学分会, 中华医学会肝病学分会和中国研究型医院学会肝病专业委员会. 瞬时弹性成像技术诊断肝纤维化专家共识(2018年更新版)[J]. 中华肝脏病杂志, 2019, 27( 3): 182－ 191. DOI: 10.3760/cma.j.issn.1007－3418.2019.03.004.
    [13]
    ZHOU XX, PU XY, XIAO X, et al. Observation on the changes of clinical symptoms, blood and brain copper deposition in Wilson disease patients treated with dimercaptosuccinic acid for 2 years[J]. J Clin Neurosci, 2020, 81: 448－ 454. DOI: 10.1016/j.jocn.2020.09.017.
    • Relative Articles
    • Supplements(0)
    • Cited By
  • 加载中

Catalog

    通讯作者: 陈斌, bchen63@163.com
    • 1. 

      沈阳化工大学材料科学与工程学院 沈阳 110142

    1. 本站搜索
    2. 百度学术搜索
    3. 万方数据库搜索
    4. CNKI搜索

    Figures(2)  / Tables(4)

    Get Citation
    PDF
    XML

    Article Metrics

    Article views (262) PDF downloads(33) Cited by()
    Proportional views
    Related
        

    The first journal specializing in hepatobiliary and pancreatic diseases in China

    Supervisor：Ministry of Education of the People's Republic of China

    Sponsor：Jilin University

    Academic Support: Chinese Society of Hepatology，Chinese Medical Association

    Address: 461 Xinjiang Road, Changchun

    Submit：0431-88782044

    Peer review：0431-88783542

    Email：lcgdb@vip.163.com

    About Journal

    Submission Guideline

    Journal Online

    Hepatobiliary College

    Guidelines

    YesterdayIP[]YesterdayPV[]TodayIP[]TodayPV[]Online[]

    Website Design © 2020 Editorial Board of Journal of Clinical Hepatology 吉ICP备10000617号-1 Supported by: Beijing Renhe Information Technology Co. Ltd  

    /

    DownLoad:  Full-Size Img  PowerPoint
    Return
    Return