neurojourНеврологический журнал имени Л.О. БадалянаL.O. Badalyan Neurological Journal2686-89972712-794XФГАУ «НМИЦ здоровья детей» Минздрава России10.46563/2686-8997-2022-3-2-54-64neurojour-65Research ArticleОРИГИНАЛЬНЫЕ ИССЛЕДОВАНИЯORIGINAL INVESTIGATIONSКачество жизни детей с мукополисахаридозами на фоне ферментозаместительной терапииQuality of life of children with mucopolysaccharidoses on the background of enzyme replacement therapyhttps://orcid.org/0000-0002-6545-4121ОсиповаЛилия АлександровнаOsipovaLiliya A.

Невролог консультативного отделения консультативно-диагностического центра ФГАУ «НМИЦ здоровья детей», 119991, Москва.

e-mail: liliya-o@yandex.ru

MD, neurologist of consultative department of Consultative and Diagnostic center, National Medical Research Center of Children’s Health, Moscow, 119991, Russian Federation.

e-mail: liliya-o@yandex.ru

liliya-o@yandex.ruhttps://orcid.org/0000-0002-9562-3774КузенковаЛ. М.KuzenkovaLudmila M.noemail@neicon.ruhttps://orcid.org/0000-0002-8750-9285ЧерниковВ. В.ChernikovVladislav V.noemail@neicon.ruhttps://orcid.org/0000-0001-6415-156XПодклетноваТ. В.PodkletnovaTatyana V.noemail@neicon.ruФГАУ «Научный медицинский исследовательский центр здоровья детей» Минздрава РоссииNational Medical Research Center of Children’s HealthФГАУ «Научный медицинский исследовательский центр здоровья детей» Минздрава России; ФГАОУ ВО «Первый Московский государственный медицинский университет им. И.М. Сеченова» Минздрава России (Сеченовский Университет)National Medical Research Center of Children’s Health; I.M. Sechenov First Moscow State Medical University202221072022325464Copyright © Осипова Л.А., Кузенкова Л.М., Черников В.В., Подклетнова Т.В., 20222022Осипова Л.А., Кузенкова Л.М., Черников В.В., Подклетнова Т.В.Osipova L.A., Kuzenkova L.M., Chernikov V.V., Podkletnova T.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.neuro-journal.ru/jour/article/view/65Введение

Введение. Мукополисахаридозы (МПС) — группа редких лизосомных болезней накопления, приводящих к уменьшению продолжительность жизни и ухудшению её качества. Несмотря на длительное изучение эффективности патогенетической терапии данных заболеваний, анализу исходов, сообщаемых пациентами и их родителями, уделяется мало внимания. 

Цель исследования — изучить, используя валидизированные опросники, заполняемые родителями, влияние ферментозаместительной терапии (ФЗТ) на качество жизни детей с МПС. 

Материалы и методы

Материалы и методы. Родители 45 детей с МПС (27 — с нейронопатическим и 18 — с ненейронопатическим течением), из них 31 — на фоне ФЗТ, заполнили родительскую версию опросника «Pediatric Quality of Life Inventory™ 4.0 Generic Core Scales». Родители 17 пациентов, получающих ФЗТ, заполняли этот опросник 2 раза, дополнительно из этой группы родители 7 детей с ненейронопатическими МПС заполнили опросник «Childhood Health Assessment Questionnare» и визуально-аналоговые шкалы для оценки боли и общего состояния здоровья, родители 10 детей с нейронопатическими МПС — визуально-аналоговые шкалы для оценки боли и общего состояния здоровья. Проведён поперечный и динамический анализ полученных данных. 

Результаты

Результаты. У детей с МПС на фоне ФЗТ отмечена стабилизация и отсутствие значимого улучшения показателей функциональной недостаточности, качества жизни и оцененных по визуально-аналоговым шкалам показателей боли и общего состояния здоровья. На фоне ФЗТ у 57% пациентов с ненейронопатическими МПС снизился средний балл по шкале «функционирование в детском образовательном учреждении», у 80% детей с нейронопатическими МПС ухудшился показатель «физическое функционирование». У 50% детей с нейронопатическими МПС и у 57% детей с ненейронопатическими МПС отмечено нарастание среднего балла по шкале «эмоциональное функционирование».

Заключение

Заключение. Детям с МПС, получающим ФЗТ, необходима дополнительная психологическая и педагогическая помощь, показано проведение регулярной двигательной реабилитации.

Участие авторов

Участие авторов:Осипова Л.А. — концепция, сбор материала, обработка и анализ полученных данных, написание текста, редактирование текста;Кузенкова Л.М. — концепция, редактирование текста;Черников В.В. — концепция, обработка полученных данных;Подклетнова Т.В. — редактирование текста.Все соавторы — утверждение окончательного варианта статьи, ответственность за целостность всех частей статьи и за информацию об авторах.

Финансирование

Финансирование. Исследование не имело спонсорской поддержки.

Конфликт интересов

Конфликт интересов. Все авторы участвовали в образовательных обучающих мероприятиях при поддержке ООО «Такеда Фармасьютикалс».

Поступила 04

Поступила 04.04.2022 Принята к печати 12.05.2022Опубликована 30.06.2022

Introduction

Introduction. Mucopolysaccharidoses (MPSs) represent a group of rare lysosomal storage diseases, associated with the decline in  life expectancy and impairing  it’s quality. Despite prolonged evaluation of the effectiveness of pathogenic therapy, patient-reported outcomes are poorly defined. The aim of the study. To describe the impact of enzyme-replacement therapy (ERT) on the quality of life in MPS children, using parent-completed validated questionnaires.

Materials and methods

Materials and methods. Parents of forty five MPSs children (27 — with neuronopathic disease, 18- with non-neuronopathic disease, 31 among them were treated with ERT) completed parent proxy-report of Pediatric Quality of Life Inventory™ (PedsQL™) 4.0 Generic Core Scales. Parents of seventeen children (10 — with neuronopathic disease), treated with ERT, completed PedsQL™ 4.0 Generic Core Scales twice, parents of 7 children with non-neuronopathic disease, treated with ERT, completed Childhood Health Assessment Questionnare (CHAQ) and visual analogue scale (VAS) of pain and overall health status, parents of 10 children with neuronopathic disease, treated with ERT, completed VAS of pain and overall health status twice. Cross-sectional and dynamic analyses have been undertaken. 

Results

Results. Stabilization and lack of significant improvement of functional disability, quality of life and VAS scores of pain and overall health status have been demonstrated. In 57% of patients with non-neuronopathic disease, treated with ERT, mean score of «School Functioning» decreased, in 80% of patients with neuronopathic disease, treated with ERT, mean score of «Physical Functioning» decreased. In 50% of children with neuronopathic disease and in 57% of children with non-neuronopathic disease, mean score of «Emotional Functioning» improved. 

Conclusion

Conclusion. MPS children, treated with ERT, require additional psychological and educational help, as well as regular motor rehabilitation. 

Contribution

Contribution: Osipova L.A. — concept, data collection, data processing and analysis, text writing, text editing;Kuzenkova L.M. — concept, text editing; Chernikov V.V. — concept, data processing;Podkletnova T.V. — text editing.All co-authors are responsible for the integrity of all parts of the manuscript and approval of its final version.

Acknowledgements

Acknowledgements. The study had no sponsorship.

Conflict of interest

Conflict of interest. The authors declare participation in educational activities with the support of Takeda Pharmaceutical Company.

Received

Received: April 04, 2022Accepted:  May 12, 2022Published: June 30, 2022

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