Wilson Disease – specific features in pediatric patients
Piotr Socha Medical Science Review - Hepatologia 2006; 6 51-54 aaICID: 449243
Article type: Original article
IC™ Value: 3.59
Abstract provided by Publisher
Wilson disease is not as commonly diagnosed in children as in adults due to the less severe symptoms at pediatric age. However, early diagnosis and treatment is essential to improve prognosis in these patients. This is why it is so important to include Wilson disease in the differential diagnosis of liver injury in children. Hepatomegaly, hepatitis, and acute or fulminant liver failure can be the first symptoms of Wilson disease. The most common presentation, at least in Poland, are abnormal liver tests detected by chance during general medical work-up. Increased transaminases are usually the reason to perform differential diagnosis to find the cause of chronic hepatitis. Failure to find a viral etiology for an acute or chronic hepatitis, or delayed or incomplete resolution, should raise the possibility of Wilson disease. Treatment started at an early stage of liver injury stops the progression of liver disease and prevents the appearance of neurological symptoms. The neurological manifestation of Wilson’s disease in childhood may include dysarthria and speech problems. Evident neurological symptoms are rarely detected. In clinical practice, Wilson disease is not recognized below the age of 3 years and its diagnosis can be rarely established below the age of 10. The same diagnostic tests and the same diagnostic criteria are used in children as in adults. Children also receive the same pharmacotherapy. Acute liver failure in the course of Wilson disease has a better prognosis, and the special scoring system of King’s College to evaluate prognosis may be used when a decision on liver transplantation must be made.