Literature DB >> 30363895

The Pragmatic Treatment of Wilson's Disease.

Annu Aggarwal1, Mohit Bhatt1.   

Abstract

Wilson's disease (WD) is a potentially fatal disorder of chronic copper toxicity, primarily affecting the liver and the brain. Judicious treatment can restore health and longevity, even in patients with severe neurological impairment. However, the disease is associated with considerable morbidity and mortality resulting from delay in diagnosis, and difficulty in pacing the medical treatment. In this article, we briefly review the diagnosis and treatment options for WD and share our experience in managing patients with WD. We focus on decoppering (copper chelation) treatment of WD and outline pragmatic strategies for patient management designed to recognize and minimize adverse effects while ensuring treatment compliance and effectiveness.

Entities:  

Keywords:  GAS for WD; penicillamine; trientine

Year:  2014        PMID: 30363895      PMCID: PMC6183178          DOI: 10.1002/mdc3.12003

Source DB:  PubMed          Journal:  Mov Disord Clin Pract        ISSN: 2330-1619


  66 in total

Review 1.  Brief observations on the management of Wilson's disease.

Authors:  J M Walshe
Journal:  Proc R Soc Med       Date:  1977

2.  The conquest of Wilson's disease.

Authors:  J M Walshe
Journal:  Brain       Date:  2009-07-13       Impact factor: 13.501

3.  A study of Wilson disease mutations in Britain.

Authors:  D Curtis; M Durkie; P Balac (Morris); D Sheard; A Goodeve; I Peake; O Quarrell; S Tanner
Journal:  Hum Mutat       Date:  1999       Impact factor: 4.878

4.  Chelation treatment of neurological Wilson's disease.

Authors:  J M Walshe; M Yealland
Journal:  Q J Med       Date:  1993-03

5.  Identification of one novel and nine recurrent mutations of the ATP7B gene in 11 children with Wilson disease.

Authors:  Juan Geng; Jian Wang; Ru-En Yao; Xiao-Qing Liu; Qi-Hua Fu
Journal:  World J Pediatr       Date:  2012-12-29       Impact factor: 2.764

Review 6.  Wilson's disease and epilepsy.

Authors:  T R Dening; G E Berrios; J M Walshe
Journal:  Brain       Date:  1988-10       Impact factor: 13.501

7.  Delineation of the spectrum of Wilson disease mutations in the Greek population and the identification of six novel mutations.

Authors:  G Loudianos; M Lovicu; P Solinas; E Kanavakis; M Tzetis; N Manolaki; E Panagiotakaki; T Karpathios; A Cao
Journal:  Genet Test       Date:  2000

8.  Identification and analysis of mutations of the Wilson disease gene in Chinese population.

Authors:  Z Wu; N Wang; S Murong; M Lin
Journal:  Chin Med J (Engl)       Date:  2000-01       Impact factor: 2.628

9.  Progressive lenticular degeneration: a familial nervous disease associated with cirrhosis of the liver, by S. A. Kinnier Wilson, (From the National Hospital, and the Laboratory of the National Hospital, Queen Square, London) Brain 1912: 34; 295-509.

Authors:  Alastair Compston
Journal:  Brain       Date:  2009-08       Impact factor: 13.501

10.  Molecular characterization of wilson disease in the Sardinian population--evidence of a founder effect.

Authors:  G Loudianos; V Dessi; M Lovicu; A Angius; A Figus; F Lilliu; S De Virgiliis; A M Nurchi; A Deplano; P Moi; M Pirastu; A Cao
Journal:  Hum Mutat       Date:  1999       Impact factor: 4.878

View more
  1 in total

Review 1.  Cardiac Involvement in Movement Disorders.

Authors:  Malco Rossi; Nestor Wainsztein; Marcelo Merello
Journal:  Mov Disord Clin Pract       Date:  2021-04-07
  1 in total

北京卡尤迪生物科技股份有限公司 © 2022-2023.