Primary sclerotizing cholangitis (PSC) is a chronic, progressive cholestatic disease rarely encountered in children. The authors present 7 children and adolescents where PSC was diagnosed on the basis of cholangiograp- hic, histological and biochemical findings. The mean age of the patients at the time of diagnosis was 13 years, range 2.5 to 16.5 years. All patients had high transaminase (ALT, AST) values and levels of gammaglutamyl transferase (GMT) and elevated serum alkaline phosphatese values. Antibodies against cytoplasm of neutrophils were present in 6 patients (85.6 %), antinuclear antibodies and antibodies against smooth muscle were detected in 3 patients (42.8 %). All patients had high immunoglobulin G levels. Five patients had clinical symptoms of colitis which was confirmed by colonoscopic and histological examination (71.6 %), in the remaining 2 histological signs of microscopic colitis were present. Crohn’s disease was diagnosed in two patients with clinical signs of colitis. Endoscopic retrograde pancreatocholangiography was performed in all patients and cholangiographic findings were evaluated according to the classification recommended for child age. Affections of intrahepatic and extrahe- patic bile ducts were described in all patients. Liver biopsies were classified according to Ludwig’s criteria for liver affection in PSC. The findings in two children corresponded to grade 1, in the remaining 5 children they were on the borderline between grades 1 and 2 of the classification. In all children treatment was started with ursodeoxy- cholic acid, in 6 patients in combination with prednisone, in one moreover with azathioprine. After 14-month monotherapy with ursodeoxycholic acid in one patient treatment was supplemented by azathioprine. The response to treatment was evaluated according to criteria for the treatment of autoimmune hepatitis. In 5 patients the response was complete, in one partial and in one patient there was no response.

        Key words: primary sclerotizing cholangitis, endoscopic retrograde cholangiopancreatography, non-specific