Aim To determine the degree of disruption of the structure of the liver and the severity of portal hypertension and its dynamics during therapy in patients with autoimmune hepatitis (AIH) and Wilson’s disease (WD) in children based on a points system determining the degree of disruption of the structure of the liver.

Methods Analysis of case histories of 176 child at the age from 1 to 17 years (mean age 11.8 ± 3.5 years) with WD (55.4%) and AIH (42.6%). Of them with cirrhosis – 40.4%, without cirrhosis – 59.6%. 49 children (27.8%) liver transplantation was performed.

Results In children with AIH degree of disruption of the liver structure was 20.4 ± 9.2%. In children with liver cirrhosis in the outcome of AIH – 24.8 ± 8.3%, without cirrhosis – 13.2 ± 5.5% (p < 0.001). In children with WD degree of disruption of the liver structure at first hospitalisation was 17.7 ± 12.1% (p = 0.305). In children with liver cirrhosis in the outcome WD – 32.4 ± 9.2%, without cirrhosis – 10.5 ± 4.0% (p < 0.001). In 49 children, who underwent liver transplantation, the degree of disruption of liver structure was 45.2 ± 16.2% (p < 0.001). The degree of disruption of liver structure with AIH after 6 months of therapy – 15.1 ± 8.0% (p = 0.007), in children with liver cirrhosis in the outcome of AIH – 16.9 ± 8.9% (p < 0.001), without cirrhosis – 11.0 ± 2.3% (p = 0.276). After 12 months of therapy, the degree of disruption of the structure of the liver in children with AIH –13.4 ± 5.2% (p < 0.001), in children with liver cirrhosis in the outcome of AIH – 15.6 ± 5.3% (p < 0.001), in children without cirrhosis was 9.8 ± 1.8% (p = 0.139).

Conclusion Scoring system degree of disturbance of the liver structure and severity of portal hypertension can be used as an objective criterion for evaluating the degree of disturbance of liver structure changes in their dynamics on the background of the therapy.