ppharmПедиатрическая фармакологияPediatric pharmacology1727-57762500-3089Издательство «ПедиатрЪ»10.15690/pf.v23i4.3073ppharm-2874Research ArticleОБЗОР ЛИТЕРАТУРЫREVIEWГликогеновая болезнь: приверженность лечению как социально-медицинская проблемаGlycogen Storage Disease: Compliance as Social and medical issuehttps://orcid.org/0009-0004-1751-1293ГетмановС. Д.GetmanovStanislav D.

Москва

MD.

1, Ostrovityanova Str., building 7, Moscow, 117513. +7 (900) 019-13-04

stas.getmanoff@yandex.ruhttps://orcid.org/0000-0002-3697-4283СурковА. Н.SurkovAndrey N.

Сурков Андрей Николаевич - д.м.н., профессор РАН, заведующий отделением-врач-гастроэнтеролог отделения гастроэнтерологии с дневным стационаром Стационара для детей, заведующий отделом научных основ детской гастроэнтерологии, гепатологии и метаболических нарушений НИИ педиатрии и охраны здоровья детей НКЦ №2 ФГБНУ «РНЦХ им. акад. Б.В. Петровского», профессор кафедры факультетской педиатрии Института материнства и детства РНИМУ им. Н.И. Пирогова (Пироговский Университет)

117593, Москва, Литовский бул., д. 1А, тел.: +7 (916) 656-31-27

MD, PhD, Professor of the RAS.

Moscow

surkov@gastrockb.ruhttps://orcid.org/0000-0002-2209-7531Намазова-БарановаЛ. С.Namazova-BaranovaLeyla S.

Намазова-Баранова Лейла Сеймуровна д.м.н., профессор, академик РАН.

Москва, Шэньчжэнь

MD, PhD, Professor, Academician of the RAS.

Moscow; Shenzhen

 

leyla.s.namazova@gmail.comhttps://orcid.org/0009-0008-0449-2053ФатуллаевС. Т.FatullaevSadig T.

Фатуллаев Садиг Талех оглы

Москва

Sadig T. Fatullaev, MD.

Moscow

dr.sadig@gastrockb.ruhttps://orcid.org/0000-0001-5549-857XБессоновЕ. Е.BessonovEvgeniy E.

Бессонов Евгений Евгеньевич

Москва

MD.

Moscow

bessonov@gastrockb.ruhttps://orcid.org/0000-0001-6614-6115ЖурковаН. В.ZhurkovaNataly V.

Журкова Наталия Вячеславовна - к.м.н.

Москва

MD, PhD.

Moscow

n1972z@yandex.ruhttps://orcid.org/0009-0002-8363-2582ШатроваА. М.ShatrovaAnna M.

Шатрова Анна Максимовна - студентка.

Москва

Student.

Moscow

shatrovanna@mail.ruhttps://orcid.org/0009-0009-3313-413XИлюхинаС. П.IlyukhinaSofiya P.

Илюхина Софья Павловна – студентка

Москва

Student.

Moscow

ilyuchina.s@gmail.comhttps://orcid.org/0009-0006-9999-8043КострыгинаМ. М.KostryginaMarina M.

Кострыгина Марина Михайловна – студентка.

Москва

Student.

Moscow

kmm1912@ya.ruhttps://orcid.org/0009-0009-7708-9051КостинаК. В.KostinaKarina V.

Костина Карина Владиславовна – студентка

Москва

Student.

Moscow

karinkostina@yandex.ruРоссийский национальный исследовательский медицинский университет им. Н.И. Пирогова (Пироговский Университет), Москва, Российская ФедерацияРоссияPirogov Russian National Research Medical UniversityRussian FederationРоссийский национальный исследовательский медицинский университет им. Н.И. Пирогова (Пироговский Университет), Москва, Российская Федерация; НИИ педиатрии и охраны здоровья детей НКЦ №2 ФГБНУ «РНЦХ им. акад. Б.В. Петровского»РоссияPirogov Russian National Research Medical University; Pediatrics and Child Health Research Institute in Petrovsky National Research Centre of SurgeryRussian FederationРоссийский национальный исследовательский медицинский университет им. Н.И. Пирогова (Пироговский Университет), Москва, Российская Федерация; НИИ педиатрии и охраны здоровья детей НКЦ №2 ФГБНУ «РНЦХ им. акад. Б.В. Петровского»; Университет МГУ-ППИ в ШэньчжэнеРоссияPirogov Russian National Research Medical University; Pediatrics and Child Health Research Institute in Petrovsky National Research Centre of Surgery; Shenzhen MSU-BIT UniversityRussian FederationНИИ педиатрии и охраны здоровья детей НКЦ №2 ФГБНУ «РНЦХ им. акад. Б.В. Петровского»РоссияPediatrics and Child Health Research Institute in Petrovsky National Research Centre of SurgeryRussian Federation202609102026234476489Copyright © Гетманов С.Д., Сурков А.Н., Намазова-Баранова Л.С., Фатуллаев С.Т., Бессонов Е.Е., Журкова Н.В., Шатрова А.М., Илюхина С.П., Кострыгина М.М., Костина К.В., 20262026Гетманов С.Д., Сурков А.Н., Намазова-Баранова Л.С., Фатуллаев С.Т., Бессонов Е.Е., Журкова Н.В., Шатрова А.М., Илюхина С.П., Кострыгина М.М., Костина К.В.Getmanov S.D., Surkov A.N., Namazova-Baranova L.S., Fatullaev S.T., Bessonov E.E., Zhurkova N.V., Shatrova A.M., Ilyukhina S.P., Kostrygina M.M., Kostina K.V.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.pedpharma.ru/jour/article/view/2874

Печеночные формы гликогеновой болезни (ГБ) — это группа наследственных метаболических расстройств, при которых пациентам требуются пожизненная диетотерапия, регулярный прием сырого кукурузного крахмала и строгий самоконтроль. Низкая приверженность лечению обусловлена социальными барьерами, недостаточной медицинской поддержкой и психологическими факторами, что приводит к прогрессированию патологии, развитию метаболической декомпенсации и ухудшению качества жизни пациентов. В статье рассмотрены ключевые аспекты патогенеза печеночных форм ГБ, их основные клинические проявления и лабораторные маркеры, а также современные подходы к терапии с акцентом на проблемы соблюдения режима. Особое внимание уделено анализу факторов низкой приверженности и ее последствий. Предложены практические социально-медицинские решения: телемедицинские платформы, образовательные программы для семей, алгоритмы междисциплинарного мониторинга и меры по повышению доступности сырого кукурузного крахмала с пролонгированным действием. Подчеркнута важность включения психолого-педагогической поддержки в клинические рекомендации и стандарты оказания медицинской помощи для оптимизации прогноза и социальной адаптации детей с ГБ.

Hepatic forms of glycogen storage disease (GSD) are a group of inherited metabolic disorders where patients require lifetime nutritional treatment, regular intake of raw cornstarch, and strict self-management. Social barriers, insufficient medical support, and psychological factors cause low compliance to treatment, and lead to disease progression, metabolic decompensation, and decrease in patients’ quality of life. This article covers key aspects of GSD hepatic forms pathogenesis, their major clinical signs and laboratory markers, as well as modern management approaches focused on compliance issues. Special attention is given to the analysis of low compliance factors and its consequences. Practical social and medical solutions were proposed: telemedicine platforms, educational programs for families, interdisciplinary monitoring algorithms, and measures to increase the availability of raw cornstarch with prolonged action. The importance of including psychological and pedagogical support in clinical guidelines and standards of medical care to optimize the prognosis and social adaptation of children with GSD was emphasized.

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