Supplemental technologies of liver ultrasound investigation at the 1-st type tyrosinemia in pediatrics

Abstract

Aim of investigation. To carry out objective estimation of liver parenchyma state in children with the 1-st type hereditary tyrosinemia (НТ-1) by ultrasound method with quantitative estimation of hepatic parenchyma pattern.Material and methods. Overall 14 children with the 1-st type tyrosinemia aged 3 months to 14 years (6 — with acute and subacute forms and 8 — with chronic form) were studied. Traditional ultrasound investigation and innovative non-invasive ultrasound technique of quantitative analysis of liver parenchyma acoustic pattern — Acoustic Structure Quantification (ASQ) were applied. Traditional US-investigation of hemodynamic parameters of portal system blood flow reveals early US-signs of portal hypertension. Color mapping (ASQ technique) allows visual estimation of fibrosis severity and amount of functioning parenchyma. Plotting of histograms and density probability functions of various zones of liver parenchyma allows to establish the stage of fibrosis. The density index provides quantitative characteristic of fibrosis stage.Results. Neither absence of fibrosis or minimal and moderately severe fibrosis was observed in children with НТ-1. The density index range at severe fibrosis stage was 2,62 (2,62-2,72) (2,62-2,72), at stage of liver cirrhosis — 3,37 (3,21–3,80) (3,00–4,58). In the areas of normal liver parenchyma no significant differences from the control group were found and the range was equal to 1,10–1,35.Conclusion. Obtained data may be used for objective estimation of the process stage in children with the 1-st type tyrosinemia when liver biopsy is contraindicated.

Keywords

• tyrosinemia
• liver fibrosis
• liver cirrhosis
• quantitative analysis of acoustic spectrum
• ultrasound diagnostics