Transient elastography, ultrasound imaging and liver enzymes in diagnosis of cystic fibrosis-related liver disease in children

Abstract

Background. Liver disease is responsible for relevant morbidity and mortality in children with cystic fibrosis. We aimed to assess the predictive value of a combination of transient elastography, ultrasound imaging and serum liver enzymes for diagnosis of cystic fibrosis-related liver disease. Materials and methods. A total of 108 children aged 0–17 years with cystic fibrosis were examined. The fibrosis stage was determined using transient elastography. The activity of enzymes (alanine transaminase, aspartate transaminase, alkaline phosphatase, gamma-glutamyl transferase, lactate dehydrogenase-5), ultrasound parameters of the liver at different stages of liver fibrosis have been investigated. Results. Liver fibrosis of varying severity (F1-F4) was detected in 29.6 % of patients with cystic fibrosis. Liver cirrhosis was observed in 14.8 % of children with cystic fibrosis. The association between an elevated activity of alkaline phosphatase, gamma-glutamyl transferase, lactate dehydrogenase-5, an enlargement of the left lobe of the liver and the degree of fibrosis F1-F4 was found. Moreover, a significant increase in the size of the left lobe corresponded to a higher degree of fibrosis. A reverse correlation was detected between the k coefficient (the ratio of the sizes of the right and left lobes of the liver) and the degree of fibrosis, with a greater degree of fibrosis corresponding to a lower value of this parameter. The maximum enlargement of the left lobe of the liver, the lowest value of the k coefficient, and the dilation of the portal and splenic veins were observed in patients with liver cirrhosis. Conclusions. The transient elastography, changing of ultrasound liver parameters with elevated activity of the alkaline phosphatase, gamma-glutamyl transferase, lactate dehydrogenase-5 could be used for early diagnosis of cystic fibrosis-related liver disease.