ppharmПедиатрическая фармакологияPediatric pharmacology1727-57762500-3089Издательство «ПедиатрЪ»10.15690/pf.v18i1.2221ppharm-1944Research ArticleКЛИНИЧЕСКИЙ СЛУЧАЙCASE REPORTОсобенности хирургического лечения патологии позвоночника у пациента с синдромом ГурлерAspects of Surgical Treatment of Spinal Pathology in the Patient with Gurler Syndromehttps://orcid.org/0000-0001-5172-4429ОчироваП. В.OchirovaPolina V.

Очирова Полина Вячеславовна, кандидат медицинских наук, травматолог-ортопед в ТОО № 9 клиники патологии позвоночника и редких заболеваний

640014, Курган, ул. М. Ульяновой, д. 6, тел.: +7 (3522) 45-10-08

poleen@yandex.ruhttps://orcid.org/0000-0001-6565-7052РябыхС. О.RyabykhSergey O.

Курган

https://orcid.org/0000-0002-9315-3035РябыхТ. В.RyabykhTatyana V.

Курган

Национальный медицинский исследовательский центр травматологии и ортопедии имени академика Г.А. ИлизароваРоссияThe Russian Ilizarov Scientific Center for Restorative Traumatology and OrthopaedicsRussian Federation2021040320211812330Copyright © Очирова П.В., Рябых С.О., Рябых Т.В., 20212021Очирова П.В., Рябых С.О., Рябых Т.В.Ochirova P.V., Ryabykh S.O., Ryabykh 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.pedpharma.ru/jour/article/view/1944

Описан клинический случай хирургического лечения комбинированной патологии позвоночника у ребенка с синдром Гурлер. Подробно описаны показания, основные факторы риска и особенности оперативного лечения патологии позвоночника при этой нозологии, проанализированы подходы по данным литературы. Обоснование. Приведенным клиническим примером мы хотим привлечь внимание коллег к этой проблеме, подчеркнуть особенности ортопедических проявлений у пациентов с мукополисахаридозом, тип IН (МПС IH). Одним из важных пунктов является своевременное их выявление и хирургическое лечение. Описание клинического случая. В данной статье приведено описание особенностей хирургического лечения патологии позвоночника у пациента с МПС IH — с прогрессирующей кифотической деформацией, стенозом и нестабильностью на уровне краниовертебрального перехода, выраженным неврологическим дефицитом, функциональными нарушениями со стороны внутренних органов. Заключение. Для пациентов с МПС характерна многоуровневая ортопедическая патология, которая требует непрерывного наблюдения в течение всей жизни одной многопрофильной командой специалистов.

The clinical case of surgical treatment of combined spinal pathology in the child with Gurler syndrome is described in the article. Indications, major risk factors and aspects of surgical treatment of spinal pathology at this nosology are described in detail; all the approaches are analyzed according to the literature. Background. We would like to draw the attention of our colleagues to this problem with this clinical case. We want to emphasize the peculiarities of orthopedic manifestations in patients with mucopolysaccharidosis type IH (MPS IH). The most crucial is timely detection and surgical treatment. Clinical case description. This article describes the aspects of surgical treatment of spinal pathology in the patient with MPS IH: progressive kyphotic deformation, stenosis and craniovertebral junction instability, significant neurological deficit, internal organs functional disorders. Conclusion. Patients with MPS are characterized by multilevel orthopedic pathology which requires continuous follow-up throughout the life by single multidisciplinary team of specialists.

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