**Abstract**

The growth hormone/insulin-like growth factor 1 (GH/IGF-1) system is the most important regulator of growth, regeneration, and metabolism in children and adults. Children with congenital cholestatic diseases have elevated GH blood levels, which is combined with growth failure and body mass deficit. Congenital cholestatic diseases lead to end-stage liver disease (ESLD), where GH bioavailability, mediated through IGF-1, is impaired. Blood IGF-1 levels are decreased due to impaired production by the liver. This study included 148 children up to 5 years (60 months) old with congenital cholestatic diseases. The patients underwent liver transplantation (LT) at a leading transplant center in Russia. The clinical significance of the GH/IGF-1 axis in pediatric liver recipients was investigated. Relationship between the patients' GH/IGF-1 levels and anthropometric parameters was analyzed before and after LT. It was shown that LT leads to renewal/recovery of GH-IGF-1 regulation and improved anthropometric parameters (body height and body mass) in pediatric recipients.

**Keywords:** GH, IGF-1, congenital cholestatic diseases, pediatric liver transplantation, anthropometric parameters
