vspВопросы современной педиатрииCurrent Pediatrics1682-55271682-5535Издательство «ПедиатрЪ»10.15690/vsp.v12i6.870vsp-252Research ArticleОРИГИНАЛЬНЫЕ СТАТЬИORIGINAL ARTICLESОСОБЕННОСТИ МОРФОЛОГИЧЕСКИХ ИЗМЕНЕНИЙ ПЕЧЕНИ У ДЕТЕЙ С ГЛИКОГЕНОВОЙ БОЛЕЗНЬЮCHARACTERISTICS OF THE HEPATIC MORPHOLOGICAL CHANGES IN CHILDREN WITH GLYCOGENOSISСурковА. Н.SurkovA. N.

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

dr_sura@mail.ruПотаповА. С.PotapovA. S.dr_sura@mail.ruЛозоваторА. П.LozovatorA. L.dr_sura@mail.ruТумановаЕ. Л.TumanovaE. L.dr_sura@mail.ruНаучный центр здоровья детей РАМН, МоскваРоссияScientific Centre of Children Health of RAMS, MoscowRussian FederationНаучный центр здоровья детей РАМН, Москва; Первый Московский государственный медицинский университет им. И.М. СеченоваРоссияScientific Centre of Children Health of RAMS, Moscow; I.M. Sechenov First Moscow State Medical UniversityRussian FederationОбъединенная больница с поликлиникой Медицинского центра по Управлению делами Президента РФ, МоскваРоссияHospital Amalgamated with Out-patient Clinic of the Medical Centre on the Management of the President Proceedings, MoscowRussian Federation201307122013126Вопросы современной педиатрии2428Copyright © Сурков А.Н., Потапов А.С., Лозоватор А.П., Туманова Е.Л., 20132013Сурков А.Н., Потапов А.С., Лозоватор А.П., Туманова Е.Л.Surkov A.N., Potapov A.S., Lozovator A.L., Tumanova E.L.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://vsp.spr-journal.ru/jour/article/view/252

Цель исследования: определить особенности морфологической картины печени у детей с гликогеновой болезнью I, III и VI типа. Пациенты и методы: обследовано 50 детей с гликогеновой болезнью в возрасте 1–17 (6,5±0,6) лет; из них с I типом — 6, с III типом — 18, с VI типом — 26 человек. Всем детям выполнена биопсия печени с определением индекса гистологической активности (Knodell) и гистологического индекса склероза (Desmet). Результаты: у 100% пациентов паренхима органа напоминала «растительную» ткань. Воспалительные изменения в печени отсутствовали у 66,0% и были выражены минимально у 18,0% детей. У 14,0% больных отмечался перигепатит. Высокая степень гистологической активности зафиксирована у 2,0% детей. Портальный и перипортальный фиброз выявлен у 100%, порто-портальные септы — у 54%, порто-центральные септы — у 6,0%, цирроз печени — у 20,0% детей. У 100% пациентов при ШИК-реакции в гепатоцитах отмечалось неравномерное глыбчатое распределение гликогена, который вымывался в контроле амилазой. При оценке динамики фиброзирования у 3 детей с VI типом гликогеновой болезни обнаружены различные варианты течения заболевания. В двух случаях изменения в печени не прогрессировали, в одном случае гистологическая картина ухудшилась. Выводы: пункционная биопсия печени играет важную роль в диагностике гликогеновой болезни, позволяя установить ряд специфических изменений, что необходимо для проведения дифференциальной диагностики, назначения терапии и оценки прогноза заболевания.

Aim

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.

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The authors declare that there are no conflicts of interest present.