Mukolipidosis type II as the cause of pulmonary hypertension in children

The article describes the development of multifactorial pulmonary hypertension (PH) associated with hypoxemia on the basis of a long-term clinical observation of the child with type II mucosolipidosis (ML). The literature data on the etiology, pathogenesis, clinical symptoms, diagnostic methods, modern ML treatment are presented. Results of patients' examination in dynamics, disease course, prescribed therapy and response to it are described. The study substantiates the connection of PH development with hypoxemia and pulmonary hypoplasia, progression of multiple dysostosis, incl. facial dysmorphism and thoracic dysplasia in patients with ML. The algorithm for examining patients with ML should include monitoring of oxygen saturation and pressure in the pulmonary artery in order to timely diagnose and treat hypoxemia and PH. © 2019, Pediatria Ltd.. All rights reserved.

Авторы
Ovsyannikov D.Y. 1, 2 , Polyakova N.A.2 , Pechatnikova N.L.2 , Korovina O.A. 1, 2 , Shokin A.A.2 , Kantemirova M.G. 1, 2 , Frolov P.A. 1 , Zhestkova M.A. 1 , Shorokhova P.M. 2 , Ponomareva D.P.2 , Daniel-Abu M. 1 , Nguyen B.V. 1 , Alekseeva O.V. 1 , Illarionova T.Y. 1 , Abramyan M.A. 1, 2 , Petryaykina E.E. 1, 2
Номер выпуска
4
Язык
Русский
Страницы
116-121
Статус
Опубликовано
Том
98
Год
2019
Организации
  • 1 Peoples’ Friendship University of Russia, Moscow, Russian Federation
  • 2 Morozov Children’s City Clinical Hospital, Moscow, Russian Federation
Ключевые слова
Children; Hypoxemia; Mucolipidosis type II; Pulmonary hypertension; Therapy
Дата создания
24.12.2019
Дата изменения
24.12.2019
Постоянная ссылка
https://repository.rudn.ru/ru/records/article/record/55168/