Literature DB >> 32351182

Management Perspective of Wilson's Disease: Early Diagnosis and Individualized Therapy.

Xiang-Zhen Yuan1, Ren-Min Yang2, Xiao-Ping Wang1.   

Abstract

Wilson's disease (WD) is an inherited disease caused by mutations in ATP7B and is characterized by the pathological accumulation of copper in the liver and brain. Common clinical manifestations of WD include a wide range of liver disease and neurological symptoms. In some patients, psychiatric symptoms may be the only manifestation at the time of diagnosis. The clinical features of WD are highly variable and can mimic any disease of internal medicine. Therefore, for unexplained medical diseases, the possibility of WD should not be ignored. Early diagnosis and treatment can improve the prognosis of WD patients and reduce disability and early death. Gene sequencing is becoming a valuable method to diagnose WD, and if possible, all WD patients and their siblings should be genetically sequenced. Copper chelators including D-penicillamine, trientine, and dimercaptosuccinic acid can significantly improve the liver injury and symptoms of WD patients but may have a limited effect on neurological symptoms. Zinc salts may be more appropriate for the treatment of asymptomatic patients or for the maintenance treatment of symptomatic patients. High-quality clinical trials for the drug treatment of WD are still lacking, therefore, individualized treatment options for patients are recommended. Individualized treatment can be determined based on the clinical features of the WD patients, efficacy and adverse effects of the drugs, and the experience of the physician. Liver transplantation is the only effective method to save patients with acute liver failure or with severe liver disease who fail drug treatment. Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.net.

Entities:  

Keywords:  Wilson's disease; ceruloplasmin; copper homeostasis; diagnosis; genetics; management

Year:  2021        PMID: 32351182     DOI: 10.2174/1570159X18666200429233517

Source DB:  PubMed          Journal:  Curr Neuropharmacol        ISSN: 1570-159X            Impact factor:   7.363


  6 in total

1.  Research on Portal Venous Hemodynamics and Influencing Factors of Portal Vein System Thrombosis for Wilson's Disease after Splenectomy.

Authors:  Zhou Zheng; Qingsheng Yu; Hui Peng; Wanzong Zhang; Yi Shen; Hui Feng; Long Huang; Fuhai Zhou; Qi Zhang; Qin Wang
Journal:  Front Surg       Date:  2022-05-30

2.  Delayed Diagnosis of Wilson's Disease Report From 179 Newly Diagnosed Cases in China.

Authors:  Minling Yu; Linxiang Ren; Muxin Zheng; Mingfan Hong; Zhisheng Wei
Journal:  Front Neurol       Date:  2022-07-05       Impact factor: 4.086

3.  A Novel Mutation of ATP7B Gene in a Case of Wilson Disease.

Authors:  Cigdem Yuce Kahraman; Ali Islek; Abdulgani Tatar; Özlem Özdemir; Adil Mardinglu; Hasan Turkez
Journal:  Medicina (Kaunas)       Date:  2021-01-29       Impact factor: 2.430

4.  Evaluation of the Safety and Toxicity of the Original Copper Nanocomposite Based on Poly-N-vinylimidazole.

Authors:  Irina A Shurygina; Galina F Prozorova; Irina S Trukhan; Svetlana A Korzhova; Nataliya N Dremina; Artem I Emel'yanov; Olesya V Say; Nadezhda P Kuznetsova; Alexander S Pozdnyakov; Michael G Shurygin
Journal:  Nanomaterials (Basel)       Date:  2021-12-22       Impact factor: 5.076

5.  Liver transplantation as a treatment for Wilson's disease with neurological presentation: a systematic literature review.

Authors:  Tomasz Litwin; Jan Bembenek; Agnieszka Antos; Adam Przybyłkowski; Marta Skowrońska; Iwona Kurkowska-Jastrzębska; Anna Członkowska
Journal:  Acta Neurol Belg       Date:  2022-01-26       Impact factor: 2.396

6.  A rare giant intracranial arachnoid cyst confused the diagnosis and treatment of Wilson disease.

Authors:  Zhang Wenbin; Huang Yeqing; Liu Aiqun; Hong Mingfan; Wei Zhisheng
Journal:  Transl Neurosci       Date:  2022-03-07       Impact factor: 1.757

  6 in total

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