Important problems in the diagnosis and treatment of primary sclerosing cholangitis (based on the Russian consensus on diagnosis and treatment autoimmune hepatitis. Moscow, 2018).

The article is published based on the results of the Russian Consensus on the diagnosis and treatment of primary sclerosing cholangitis (PSC), discussed at the 44th annual Scientific Session of the CNIIG "Personalized Medicine in the Era of Standards" (March 1, 2018). The aim of the review is to highlight the current issues of classification of diagnosis and treatment of patients with PSC, which causes the greatest interest of specialists. The urgency of the problem is determined by the multivariate nature of the clinical manifestations, by often asymptomatic flow, severe prognosis, complexity of diagnosis and insufficient study of PSC, the natural course of which in some cases can be considered as a function with many variables in terms of the nature and speed of progression with numerous possible clinical outcomes. In addition to progression to portal hypertension, cirrhosis and its complications, PSC can be accompanied by clinical manifestations of obstructive jaundice, bacterial cholangitis, cholangiocarcinoma and colorectal cancer. Magnetic resonance cholangiography is the main method of radial diagnostics of PSC, which allows to obtain an image of bile ducts in an un-invasive way. The use of liver biopsy is best justified when there is a suspicion of small-diameter PSC, autoimmune cross-syndrome PSC-AIG, IgG4-sclerosing cholangitis. Currently, a drug registered to treat primary sclerosing cholangitis which can significantly change the course and prognosis of the disease does not exist. There is no unified view on the effectiveness and usefulness of ursodeoxycholic acid and its dosage in PSC. Early diagnosis and determination of the phenotype of PSC is of clinical importance. It allows to determine the tactics of treatment, detection and prevention of complications.

[THE CONTENT OF BILE ACIDS IN THE SERUM OF PATIENTS WITH NONALCOHOLIC FATTY LIVER DISEASE].

AIM: Rate content of primary, secondary, tertiary and unconjugated bile acids in the blood of patients with NAFLD. METHODS: The study involved 74 patients with NAFLD (male--30, female--44) And 51 healthy individuals (male--14, female--37). All patients underwent anthropometry and they had a complete clinical, biochemical and instrumental examination (determination of the amount of fat in the subcutaneous fat layer). Patients with hepatic steatosis were--64 people, with steatohepatitis--10 people. The content of bile acids (primary: cholic, chenodeoxycholic; secondary: lithocholic, deoxycholic and tertiary: ursodeoxycholic) in serum were determined by gas-liquid chromatography, chromatography "Chromos GC-1000" (Russia). RESULTS: In the blood of healthy individuals and patients with NAFLD are determined unconjugated primary, secondary and tertiary LCD. In healthy individuals there are no gender differences in the content of the LCD. NAFLD patients LCD level higher than that of healthy individuals. There is a significant difference in the concentration of secondary and tertiary LCD in patients with hepatic steatosis and steatohepatitis. CONCLUSIONS: 1. The content of the bile acids in the blood of patients with NAFLD significantly higher than in healthy individuals. 2. When steatohepatitis compared with hepatic steatosis, there are more significant fluctuations in the blood content of the LCD according to gender and type of LCD. So, cholic, chenodeoxycholic and deoxycholic higher than that of men, while, lithocholic and UDCA below. 3. Significant difference in the content of fatty acids in the blood between patients with hepatic steatosis and steatohepatitis exists only in relation to the secondary and tertiary LCD. Thus, when steatohepatitis compared with hepatic steatosis litoheolevaya acid and UDCA more in men and deoxycholic below. Conversely, women and lithocholic UDCA below and above deoxycholic acid.