For younger patients, the clinical manifestations of Wilson's disease are most commonly hepatic. Children with Wilson's disease often present with chronic liver disease and abnormal liver tests.2
As patients get older, neurologic and psychiatric symptoms more commonly occur. Some common neurologic manifestations of Wilson's disease include tremor, gait abnormalities, and discoordination, dystonia, Parkinsonism, choreiform movements, drooling, dysphonia, dysarthria, and dysphasia. Behavioral and psychiatric manifestations may include depression, altered behavior and personality, impulsiveness and labile mood, sexual exhibitionism, and frank psychosis.2
Golden-brownish pigment in cornea caused by copper deposits
Facetious (false) smile, pseudo-laughter, open mouth and drooling saliva, reduced eye blinking and a dull look
1. Srinivas, K. Sinha, S. Taly, A.B. et al. Dominant psychiatric manifestations in Wilson's disease: A diagnostic and therapeutic challenge. J Neurol Sci. 266 (2008) 104-108. 2. Schilsky M. Wilson Disease: Clinical Manifestations, Diagnosis, and Treatment. Clinical Liver Disease. Vol 3, No 5, May 2014. 3. Roberts EA, Schilsky ML. AASLD Practice Guidelines. Diagnosis and treatment of Wilson disease: an update. Hepatology 2008;47(6):2089-111. 4. European Association for the Study of the Liver. EASL Clinical Practice Guidelines: Wilson's Disease. J Hepatol 2012;56:671-685. 5. Aggarwal A, Bhatt M. Update on Wilson disease. In Metal Related Neurodegenerative Disease, Bhatia K, Schneider S (eds.). Int Rev Neurobiol 2013;110:313-48.
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.5
ALL CHILDREN OF A WILSON'S DISEASE PARENT ARE AUTOMATICALLY CARRIERS OF THE DISEASE
1. Roberts EA, Schilsky ML. AASLD Practice Guidelines. Diagnosis and treatment of Wilson disease: an update. Hepatology 2008;47(6):2089-111. 2. European Association for the Study of the Liver. EASL Clinical Practice Guidelines: Wilson’s Disease. J Hepatol 2012;56:671-685. 3. Aggarwal A, Bhatt M. Update on Wilson disease. In Metal Related Neurodegenerative Disease, Bhatia K, Schneider S (eds.). Int Rev Neurobiol 2013;110:313-48. 4. Ala A, Borjigin J, Rochwarger A, Schilsky M. Wilson disease in septuagenarian siblings: raising the bar for diagnosis. Hepatology 2005;41:668-70. 5. Schilsky, M. Ala, A. Genetic Testing for Wilson Disease: Availability and Utility. Curr Gastroenterol Rep (2010) 12:57–61. 6. National Institute of Diabetes and Digestive and Kidney Diseases. Wilson Disease. https://www.niddk.nih.gov/health-information/liver-disease/wilson-disease. Accessed April 18, 2018. 7. Ferenci P, Askari F. Whom and how to screen for Wilson disease. Expert Rev. Gastroenterol. Hepatol. 8(5), 513-520 (2014).
Please visit wilsondiseasefacts.com for more information.
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. CLOVIQUE™ (trientine hydrochloride) is a chelating agent indicated for treatment of patients with WD who are intolerant of the first-line treatment, penicillamine.3
CLOVIQUE™ 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. CLOVIQUE™ and penicillamine cannot be considered interchangeable. CLOVIQUE™ should be used when continued treatment with penicillamine is no longer possible because of intolerable or life endangering side effects.
Unlike penicillamine, CLOVIQUE™ 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.
CLOVIQUE™ is not indicated for treatment of biliary cirrhosis.
Please see full Prescribing Information for CLOVIQUE™ 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,
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