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.
