Endothelial dysfunction and platelet function disorder in hemostasis in development of hepatic fibrosis in children with autoimmune hepatitis

Abstract

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 <0.001), the platelet count (r = -0.5, p = 0.003). The determination of the endothelin-1 concentration, the von Willebrand factor activity and the platelet count may be used to assess the hepatopathy severity in children with autoimmune hepatitis.
 Conclusion. In children with autoimmune hepatitis the endothelial dysfunction and platelet disorders are revealed in the hemostasis system correlating with the severity of the pathological process.

Experience of liver transplantation in the regional clinical hospital

“…В крайней степени, когда консервативная терапия неэффективна, требуется пересадка печени. Заболевания (состояния), при которых показана трансплантация печени: фульминантная печеночная недостаточность, терминальная стадия диффузных хронических заболеваний печени (гепатит С, В, аутоиммунный гепатит, алкогольный цирроз печени, первичный билиарный цирроз печени), нарушение метаболизма на фоне врожденных дефектов развития гепатоцита (болезнь Вильсона-Коновалова), очаговые образования печени (доброкачественные образования, поражающие всю печень (редко), гепатоцеллюлярная карцинома (согласно Миланским критериям)) [1,2,3].…”

“…Experience of liver transplantation in the regional clinical hospital O. V. Zaitsev 1,3 , A. R. Monakhov 2 , S. I. Zubenko 2 , A. V. Koshkina 1, 3…”