CHARACTERISTICS OF THE HEPATIC MORPHOLOGICAL CHANGES IN CHILDREN WITH GLYCOGENOSIS

Abstract

Aim: to determine characteristics of the hepatic morphology in children with I, III and VI types of glycogenosis. Patients and methods: 50 children with glycogenosis (6 with type I, 18 with type III and 26 with type VI) aged from 1 to 17 (6,5±0,6) years old were examined. All children were performed hepatobiopsy with determination of the histological activity index and histological index of sclerosis (Desmet). Results: in 100% of the patients hepatic parenchyma resembled «vegetative» tissue. Inflammatory changes in the liver were absent in 66,0% of the patients and were minimal in 18,0% of the children. Perihepatitis was observed in 14,0% of the patients. High degree of histological activity was found in 2,0% of the children. Portal and periportal fibrosis was detected in 100% of the patients, porto-portal bridges — in 54%, porto-central bridges — in 6,0%, hepatic cirrhosis — in 20% of the children. On PAS reaction in hepatocytes of 100% of the patients were found irregular lump-like deposits of glycogen, which was washed out on control tests with amylase. On assessment of progression of fibrosis in 3 children with VI type of glycogenosis different variants of the clinical course were observed. In 2 cases there was no progression of hepatic changes, in 1 case — there was worsening of the histological features. Conclusions: needle aspiration biopsy of the liver is very important in diagnostics of glycogenosis and allows to detect a number of specific changes, which is necessary for differential diagnosis, treatment choice and prognosis assessment.Key words: children, glycogenosis, liver, morphology.

Keywords

• children
• glycogenosis
• liver
• morphology