Clinical Practice in Pediatrics

Violation of the liver structure and function in congenital cholestatic diseases in the young

Objective. To estimate the degree of violation of liver structure and liver function in children with congenital cholestatic diseases based on the International Classification of Functioning of Disability and Health (ICF).
Patients and methods. A retrospective analysis of congenital cholestatic diseases in 81 children was conducted: 20 children with biliary atresia (BA), 17 with hereditary tyrosinemia type 1 (HT1), 23 with progressive familial intrahepatic cholestasis type 1 and 2 (PFIC1-2) and 21 patients with Alagilla syndrome (AS). The analysis of biochemical parameters of blood, ultrasound examination of the abdominal cavity, fibroelastometry, histological parameters. Conducted determination of the degree of
violation of the structure and liver function. 
Results. Liver function in children with PFIC1-2 was reduced by 35.7 ± 3.2%, the structure of the liver was impaired by 30.5 ± 3.0%. In children with BA, the liver function was reduced by 32.2 ± 2.5%, the structure of the liver was impaired by 27.2 ± 2.5%. In children with AS the liver function was reduced by 21.3 ± 2.5%, the structure of the liver was impaired by 25.9 ± 2.0%. In children with HT1 the liver function was reduced by 34.1 ± 11.3%, the structure of the liver was impaired by 34.8 ± 6.3%. 
Conclusion. In congenital cholestatic diseases in children, disorders of the liver structure and function can be established, using point systems. This allows us to objectively evaluate the dynamics of the course of the disease and establish the need for liver transplantation.
Key words: liver structure and function, Alagille syndrome, progressive familial intrahepatic cholestasis, biliary atresia, type 1 hereditary tyrosinemia.

For citation: Volynets G.V., Nikitin A.V., Skvortsova T.A., Khavkin A.I. Violation of the liver structure and function in congenital cholestatic diseases in the young. Vopr. prakt. pediatr. (Clinical Practice in Pediatrics). 2019; 14(4): 15–20. (In Russian).

DOI: 10.20953/1817-7646-2019-4-15-20

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