ABSTRACT
The authors analyze their experience with diagnosis and treatment of 182 children with syndrome of portal hypertension (PH) from 1991 through 2010. Two groups of patients were considered. The first group included 74 newborns with high risk of the development of PH (infants after catheterization of the umbilical vein who endured omphalitis with USI diagnosed thrombosis of the portal vein, patients with cavernous transformation of the portal vein, hepatosplenomegaly). The second group consisted of 108 children aged from 6 months to 14 years with realized syndrome of PH. Investigation of hemostasis (international normalized ratio, fibrinogen, VIII and IX factors) immunogram of the 2nd level, determination of gene polymorphism (prothrombin, V factor, MTHFR, PAI-1), Bonacini index were included in the complex of examination, besides clinical and biochemical analyses of blood. The degree of disturbance of hemodynamics in the portal system was estimated by the data of USI, dopplerography, FEGS. It was established that children with extrahepatic portal hypertension have markers of hereditary thrombophilia in 98% of cases. Bonacini index allows determination of early signs of the development of secondary fibrosis of the liver in children with PH without using liver biopsy.
