LEVEL OF TRANSFORMING GROWTH FACTOR BETA-1 RELATES TO CONGENITAL LIVER DISEASE SEVERITY IN CHILDREN OF EARLY AGE

• Published in: Vestnik transplantologii i iskusstvennykh organov Vol. 18; no. 3; pp. 16 - 21
• Main Authors: R. M. Kurabekova, O. P. Shevchenko, O. M. Tsiroulnikova, N. P. Mozheyko, I. E. Tsiroulnikova, A. R. Monakhov, S. V. Gautier
• Format: Journal Article
• Language: English; Russian
• Published: Federal Research Center of Transplantology and Artificial Organs named after V.I.Shumakov 17-11-2016
• Subjects: biomarker; cytokine; hepatic fi brosis; liver diseases; pediatric liver transplantation; tgf-β
• ISSN: 1995-1191
• DOI: 10.15825/1995-1191-2016-3-16-21

Aim. Analysis of relationship between transforming growth factor beta-1 (TGF-β1) level in blood and liver disease severity before and after liver transplantation in early age children with congenital liver diseases. Materials and methods. The study included 135 pediatric patients aged from 2 to 73 months with end-stage liver disease. Results. The level of TGF-β1 in the blood of children with liver failure on average was lower than in healthy children of the same age. The cytokine level depended on the liver disease etiology: in patients with biliary atresia, biliary hypoplasia, Alagille syndrome, Byler disease and other diseases it was lower than that at Caroli disease when it did not differ from the level in healthy children. The level of cytokine in the blood plasma of patients was associated with the severity of hepatic fi brosis: in fi brosis of grade 1 and 4 it was lower than in fi brosis of grade 2 and 3. The liver transplantation from related living donor resulted in increase of TGF-β1 level in the blood plasma of patients regardless of the initial etiology and severity of the liver disease. Conclusion. The blood level of TGF-β1 refl ects liver disease severity in children with congenital liver diseases and may be used as a marker of liver function state before and after liver transplantation.