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Aim. To perform a comparative analysis of clinical and anamnestic data and of the condition of primary hemostasis in chronic liver diseases in children and to identify additional informative diagnostic criteria that reflect severity of the course of autoimmune hepatitis (AIH) and chronic viral hepatitis C (CVHC) in children. Materials and Methods. 91 Patients from 3 to 7 years old were examined, of them: 60 children with AIH and 31 children with CVHC. The control group included 15 children of I and II health groups. In all the patients the clinic-anamnestic data and the condition of the primary hemostasis were evaluated. Concentrations of endothelin-1 and homocysteine, activity of Willebrand factor, amount of platelets and their aggregation activity were determined. Results. AIH is characterized by a more aggressive course as compared to CVHC manifested by clinical signs of a severe liver damage, significant biochemical changes and a high rate of fibrosis within the first two years. In all the children there were found disorders in the primary hemostasis interrelated with the main clinical and laboratory syndromes reflecting the severity of the liver damage. AIH is characterized by a higher concentration of homocysteine (р=0.007) and of the activity of Willebrand factor (р=0.037) in comparison with CVHC. Conclusions. Signs of a severe liver damage are not characteristic of children with CVHC in the first 10 years of the disease, however, disorders of the primary hemostasis are present characterized by hyperaggregation of platelets and by endothelial dysfunction. AIH in children is characterized by aggressive course and more pronounced deviations of the primary hemostasis in comparison with CVHC. In AIH and CVHC, the pathology of the primary hemostasis is closely related to some clinical-laboratory symptoms that reflect severity of the disease.

The purpose is: To assess the clinical relevance of the endothelial condition and platelet aggregation in the development of hepatic fibrosis in children with autoimmune hepatitis. Materials and Methods. 35 patients aged from 3 to 17 years old were studied, including 19 girls - (54%) and 16 boys - (46%). The 1st group consisted of 17 children with the degree of fibrosis F 0-2 acc. to the METAVIR score; the 2nd group consisted of 18 patients with the degree of fibrosis F 3-4 (METAVIR) acc. to the METAVIR score based on the indirect elastometry data in children of the 2nd group hepatic cirrhosis was diagnosed; the control group consisted of 15 children with health group I or II. The endothelium state and the platelet aggregation activity were assessed in all the patients. To test statistical hypotheses, the Mann-Whitney U test, the Spearman correlation coefficient and the Fisher criterion were used. The critical value of the significance level is assumed to be 0.05. Quantitative data are presented as: median and the first; third quartile of Me (Q1; Q3). Results. In children with autoimmune hepatitis some signs of the various-degree fibrosis formation were revealed in the ¾ of the examined. Patients of the 2nd group have a more aggressive course as compared to the 1st group: in the disease debut there were mainly expressed asthenoneurotic complaints (p = 0.021), manifestations of the jaundice syndrome (p = 0.014), more frequently hepatic-cell insufficiency (p = 0.045) is diagnosed and followed by complications of the disease (hypersplenism (p = 0.014), varicose veins of the esophagus (p = 0.003)). All children with autoimmune hepatitis have the endothelial dysfunction, the enhancing platelet aggregation activity. The degree of fibrosis correlates with the concentration of endothelin-1 (r = 0.4, p = 0.004), the von Willebrand factor (vWF) activity (r = 0.5, p