Subclinical Myocardial Dysfunction in Children With Wilson's Disease

Wilson's disease (WD) is one of the most common metabolic liver diseases in older children. The most frequent clinical presentation is liver disease. However, Wilson's disease (WD) is a multisystem disorder. It is concluded that four modes of cardiac manifestations in Wilson's disease (WD) include arrhythmias, cardiomyopathy, cardiac death, and autonomic dysfunction. Such possible cardiac involvement should be added to the clinical picture of Wilson's disease (WD) involving the hepatic and central nervous system(CNS). The data on cardiac manifestations in children is very limited and only few adult studies are available. In this study, the investigators aim to unveil subclinical cardiac dysfunction in children with Wilson's disease with apparently normal cardiac functions by conventional assessment.

Inclusion Criteria 1. Confirmed diagnosis of Wilson's disease based on Leipzig scoring criteria (including clinical signs, Kayser-Fleischer rings, low ceruloplasmin, or genetic analysis). 2. Age between 4 years and 18 years. 3. Written informed consent obtained from parents or legal guardians. Exclusion Criteria 1. Children with clinical evidence of overt heart failure or known congenital heart disease. 2. Children suffering from fulminant hepatitis. 3. Known co-existing primary liver diseases other than Wilson's disease. 4. Presence of syndromic disorders or major congenital anomalies.