For younger patients, the clinical manifestations of Wilson's disease are mostly hepatic. Children with Wilson's disease often present with chronic liver disease and abnormal liver tests.10
As patients get older, neurologic and psychiatric symptoms occur more frequently.10 Some of the common neurologic manifestations of Wilson's disease include tremor, gait abnormalities, discoordination, dystonia, Parkinsonism, choreiform movements, drooling, dysphonia, dysarthria and dysphagia. Behavioral and psychiatric manifestations may include depression, altered behavior and personality, impulsiveness and labile mood, sexual exhibitionism and frank psychosis.4,10
Golden-brown pigment in cornea caused by copper deposits11
Facetious (false) smile, pseudo-laughter, open mouth and drooling saliva, reduced eye blinking and a dull look
Some people may not know about a family history of the condition because the mutation is often passed to a child by a parent who is a carrier.13
ALL CHILDREN OF A PARENT WITH WILSON'S DISEASE ARE CARRIERS OF THE DISEASE10
1. Olivarez L, Caggana M, Pass KA, Ferguson P, Brewer GJ. Estimate of the frequency of Wilson's disease in the US Caucasian population: a mutation analysis approach. Ann Hum Genet . 2001;65(Pt 5):459-463. doi:10.1017/S0003480001008764 2. Zimbrean PC, Schilsky ML. Psychiatric aspects of Wilson disease: a review. Gen Hosp Psychiatry. 2014;36(1):53-62. doi:10.1016/j.genhosppsych.2013.08.007 3. Hedera P. Update on the clinical management of Wilson's disease. Appl Clin Genet. 2017;10:9-19. doi:10.2147/TACG.S79121 4. Roberts EA, Schilsky ML. Diagnosis and treatment of Wilson disease: an update. Hepatology. 2008;47(6):2089-2111. doi:10.1002/hep.22261 5. Aggarwal A, Bhatt M. Update on Wilson disease. Int Rev Neurobiol. 2013;110:313-348. doi:10.1016/B978-0-12-410502-7.00014-4 6.Patil M, Sheth KA, Krishnamurthy AC, Devarbhavi H. A review and current perspective on Wilson disease. J Clin Exp Hepatol. 2013;3(4):321-336. doi:10.1016/j.jceh.2013.06.002 7.Millard H, Zimbrean P, Martin A. Delay in diagnosis of Wilson disease in children with insidious psychiatric symptoms: a case report and review of the literature. Psychosomatics. 2015;56(6):700-705. doi:10.1016/j.psym.2015.07.008 8. Srinivas K, Sinha S, Taly AB, et al. Dominant psychiatric manifestations in Wilson's disease: a diagnostic and therapeutic challenge! J Neurol Sci. 2008;266(1-2):104-108. doi:10.1016/j.jns.2007.09.009 9. Ferenci P. Whom and how to screen for Wilson disease. Expert Rev Gastroenterol Hepatol. 2014;8(5):513-520. doi:10.1586/17474124.2014.899898 10. Schilsky ML. Wilson disease: clinical manifestations, diagnosis, and treatment. Clin Liver Dis (Hoboken). 2014;3(5):104-107. doi:10.1002/cld.349 11. Joshi G, Dhingra D, Tekchandani U, Kaushik S. Kayser-Fleischer ring in Wilson's disease. QJM. 2019;112(8):629. doi:10.1093/qjmed/hcz027 12. Inheritance. Wilson Disease Association. Accessed April 9, 2020. https://www.wilsondisease.org/about-wilson-disease/inheritance 13. Schilsky ML, Ala A. Genetic testing for Wilson disease: availability and utility. Curr Gastroenterol Rep. 2010;12(1):57-61. doi:10.1007/s11894-009-0084-5
Wilson's disease (WD), the most common inherited disorder of copper metabolism, results from a failure of the copper excretory pathway. This leads to toxic accumulation of copper in the liver and eventually other organs.1 The worldwide prevalence of WD is estimated to be one in 30,000 individuals.2
The condition can be treated with a low copper diet and the use of chelating agents that bind copper to facilitate its excretion from the body. CLOVIQUETM (trientine hydrochloride) is a chelating agent indicated for treatment of patients with WD who are intolerant of the first-line treatment, penicillamine.3
CLOVIQUETM may be used to treat Wilson's disease in patients who cannot take the medication penicillamine. Clinical experience with trientine hydrochloride is limited and alternate dosing regimens have not been well-characterized; all endpoints in determining an individual patient's dose have not been well defined. CLOVIQUETM and penicillamine cannot be considered interchangeable. CLOVIQUETM should be used when continued treatment with penicillamine is no longer possible because of intolerable or life endangering side effects.
Unlike penicillamine, CLOVIQUETM is not recommended in cystinuria or rheumatoid arthritis. The absence of a sulfhydryl moiety renders it incapable of binding cystine and, therefore, it is of no use in cystinuria. In 15 patients with rheumatoid arthritis, trientine hydrochloride was reported not to be effective in improving any clinical or biochemical parameter after 12 weeks of treatment.
CLOVIQUETM is not indicated for treatment of biliary cirrhosis.
Please see full Prescribing Information for CLOVIQUETM Capsules.
You are encouraged to report negative side effects of prescription drugs to FDA. Visit www.FDA.gov/medwatch or call 1-800-FDA-1088. You may also contact Kadmon Pharmaceuticals, LLC at 1-877-377-7862 to report negative side effects.References:
Gaffney D, Fell GS, O'Reilly DS. ACP Best Practice No 163. Wilson's disease: acute and presymptomatic laboratory diagnosis and monitoring. J Clin Pathol. 2000;53(11):807-812.
European Association for Study of Liver. EASL clinical practice guidelines: Wilson's disease. J Hepatol. 2012;56(3):671-685.
Clovique. Package Insert. Kadmon Pharmaceuticals, LLC; 2018.