Peculiarities of clinical course of Wilson's disease in children

Purpose — to discover peculiarities of Wilson disease course in children dependently on the variant liver affection. Materials and methods. Anamnesis clinical paraclinical with consideration affection severity have been studied 50 aged 5–17 years. Results. It was estimated that 52% had a form chronic hepatitis minimal symptoms, 44% patients cirrhosis predominant signs edematous ascitic syndrome 4% fulminant debut disease. Complicated family anamnesis detected patients. Syndrome cytolysis predominated damage (р<0.05). Typical biochemical changes cirrhotic were hepatocellular insufficiency hypoalbuminemia coagulopathy, hyperbilirubinemia hypertransaminasemia Children contrast reliably lower serum ceruloplasmin concentration higher excretion copper urine. Specific for Kayser—Fleischer rings found out 36% only. Conclusions. Wilson's is characterized by progressing either or or, rarely, hepatitis. Clinical symptoms are vary from The research carried accordance principles Helsinki declaration. study protocol approved Local Ethics Committee all participating institution. informed consent patient obtained conducting studies. No conflict interest declared authors. Key words: disease, children, hepatitis, cirrhosis,