CHARACTERISTICS OF THE HEPATIC MORPHOLOGICAL CHANGES IN CHILDREN WITH GLYCOGENOSIS
https://doi.org/10.15690/vsp.v12i6.870
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.About the Authors
A. N. Surkov
Scientific Centre of Children Health of RAMS, Moscow
Russian Federation
Surkov Andrey Nikolaevich, Candidate of Medical Science, Senior Researcher, Gastroenterologist at the department of rehabilitation treatment of children with diseases of the digestive system at the Research Institute of preventive pediatrics and rehabilitation treatment FSBI, SCCH
Russian Federation
Surkov Andrey Nikolaevich, Candidate of Medical Science, Senior Researcher, Gastroenterologist at the department of rehabilitation treatment of children with diseases of the digestive system at the Research Institute of preventive pediatrics and rehabilitation treatment FSBI, SCCH
A. S. Potapov
Scientific Centre of Children Health of RAMS, Moscow;
I.M. Sechenov First Moscow State Medical University
Russian Federation
Russian Federation
A. L. Lozovator
Hospital Amalgamated with Out-patient Clinic of the Medical Centre on the Management of the President Proceedings, Moscow
Russian Federation
Russian Federation
E. L. Tumanova
Scientific Centre of Children Health of RAMS, Moscow
Russian Federation
Russian Federation
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For citations:
Surkov A.N., Potapov A.S., Lozovator A.L., Tumanova E.L. CHARACTERISTICS OF THE HEPATIC MORPHOLOGICAL CHANGES IN CHILDREN WITH GLYCOGENOSIS. Current Pediatrics. 2013;12(6):24-28. (In Russ.) https://doi.org/10.15690/vsp.v12i6.870
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