SCLEROSING CHOLANGITIS IN CHILDREN AND ADOLESCENTS: CURRENT STATE OF THE PROBLEM

Abstract

There are presented generalized data on modern methods of diagnosis and treatment of sclerosing cholangitis (SCh), a chronic inflammatory liver disease of unknown etiology affecting the intrahepatic and/or extrahepatic bile ducts resulting in cirrhosis of the liver. In children, 2 forms of SCh are distinguished: primary (PSCh) and autoimmune (ASCh). Diagnosis of SCh requires performing cholangiography. ACh responds to standard immunosuppressive treatment with prednisone/prednisone and azathioprine. Supportive immunosuppressive therapy should last at least 2-3 years. After the end of such treatment, the doctor should continue to monitor changes in the ratios of AST/ALT/IgG autoantibodies in the blood of patients every 3-month for at least 5 years. SCh is often associated with inflammatory bowel disease, which should be excluded in the debut by determining the content of fecal calprotectin and colonoscopy data. In children, liver transplantation is one of the options for the treatment of Ch. However, the frequency of relapses after transplantation remains high, especially in ACh patients.