P1198 WILSON’S DISEASE IN CHILDHOOD - PRESENTATION AND MANAGEMENT (4 YEARS RETROSPECTIVE STUDY)

Introduction: Objective of the study:to evaluate the effectiveness of different treatments methods with chelating agents,Zinc or Orthopic Liver Transplantation,based on hepatic or neurological manifestation at the pediatric patients with Wilson’s Disease. Methods: Between January 2000 and December 2003 14 children were Wilson’s Disease diagnosed:10 male and 4 female, with an average age of 10,2 years.The average duration from the beginning to the diagnosis was 14 months,longer at those ones with initial neurological disorder (20 months).The diagnosis was based on the low serum ceruloplasmin level (under 0,2g/l), increased of the copper in 24-hours urine and the presence of Kayser-Fleischer rings.The chelating drugs D-Penicillamine was the main stay of therapy for 10 patients (2 of them in association with Zinc); only 1 patient was treated with Zinc salts,one with Trientine and 2 benefited by Orthotopic Liver Transplantation. No severe side effects were noticed.We have regularly monitories (every 6 months):serum copper,ceruloplasmin,liver biochemistries and physical examination.We measured the copper excretion in 24-hours urine (under 0,5mg/l)every 6 months. Results: All patients had a hepatic disease at the beginning of the study:hepatic cirrhosis (6 cases),chronic hepatitis(3), fulminate hepatic failure(3). 5 patients had a neuropsychiatrical disorder.2 patients with fulminate hepatic failure and hemolytic anemia who benefited by Orthotopic Liver Transplantation had a good further evolution.A patient with fulminate hepatic failure was treated with Trientine during a month,without improvement and evolution to exitus-we suspect poor compliance at the treatment.At the 5 patients with hepatic and neurological manifestation a partial remition of the neurological symptoms was achieved and the hepatic disease was stabilized.The neurological improvement was faster at those who received Zinc as well.At the 6 patients with hepatic disease, treated with D-Penicillamine a hepatic function improvement was noticed (3 by those ones were at presymptomatic stage). Conclusion: 1. The liver disease (unknown origin) with positive family history should imply Wilson’s Disease strongly. 2. The faster the introduction of the chelating therapy is, the better the results are. 3. At the symptomatic patients with a good compliance at the treatment an improvement of the hepatic disease was obtained. 4. The patients at whom the chelating treatment was initiated before the beginning of the clinical symptoms had an excellent evolution: no appearance of the symptoms on the followed period. 5. For patients with fulminate hepatic failure and hemolytic anemia the only solution was the Orthotopic Liver Transplantation.

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