Early Diagnosis of Hepatobiliary System Lesions In Children With Cystic Fibrosis

Abstract

An increase in the life expectancy of cystic fibrosis patients contributes to the formation of severe hepatobiliary pathology, leading to the development of biliary cirrhosis with a fatal outcome. The purpose was to prospectively assess the predictive value of a combination of serum liver enzymes, ultrasound liver parameters, and transient elastography to diagnose clinically significant liver fibrosis. Materials and methods. 108 children aged 0-17 years with cystic fibrosis were examined. The fibrosis stage was determined using transient elastography on FibroScan®502 (Echosens, France). The activity of enzymes (alanine transaminase, aspartate transaminase, alkaline phosphatase, gammaglutamyl transferase, lactate dehydrogenase-5), ultrasound parameters of the liver at different stages of liver fibrosis have been investigated. Results. Liver fibrosis of varying severity was detected in 29.6% of patients with cystic fibrosis (liver elasticity ranged from 5.9 to 49.0 kPa). Liver cirrhosis was observed in 14.8% of children with cystic fibrosis. The dependence of an increase in the activity of alkaline phosphatase, gammaglutamyl transferase, lactate dehydrogenase-5, and an enlargement of the left lobe of the liver, a reduction in the k ratio of the sizes of the right and left lobes of the liver on the degree of fibrosis F1-F4 (p<0.05) was found. Conclusions. The combined use of transient elastography FibroScan with increased activity of the alkaline phosphatase, gamma-glutamyl transferase, lactate dehydrogenase-5, and changing of ultrasound liver parameters could be used for early diagnosis of hepatobiliary lesions in cystic fibrosis. The age of a patient with cystic fibrosis over 6 years old, male gender, and the presence of ΔF508 deletion in the genotype have a high positive predictive value for liver fibrosis and cirrhosis.