Use of Implantable Venous Port Systems in the Treatment of Children with Orphan Diseases (Mucopolysaccharidosis and Pompe Disease): Case Series

Use of Implantable Venous Port Systems in the Treatment of Children with Orphan Diseases (Mucopolysaccharidosis and Pompe Disease): Case Series

Many orphan diseases in children require life-long and regular intravenous enzyme replacement therapy. The article describes the first Russian practice of implanting venous port systems in 12 patients with type I and II mucopolysaccharidosis and Pompe disease (6 months to 17 years old) to create lon...

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Bibliographic Details
Main Authors: M. Yu. Rykov, I. V. Filinov, E. I. Petrov, N. D. Vashakmadze, A. K. Gevorkyan, E. N. Arkhipova, I. V. Sil’nova, E. N. Basargina, N. V. Buchinskaya, A. I. Ivanov, E. A. Isupova, M. M. Kostik, N. A. Abramova, O. V. Kalashnikova, V. G. Chasnyk, A. E. Aleksandrov, D. A. Morozov, V. G. Polyakov
Format: Article
Language:English
Published: "Paediatrician" Publishers LLC 2015-09-01
Series:Вопросы современной педиатрии
Subjects:
orphan diseases
mucopolysaccharidoses
pompe disease
implantable venous port systems
venous access
Online Access:https://vsp.spr-journal.ru/jour/article/view/1198
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Summary:Many orphan diseases in children require life-long and regular intravenous enzyme replacement therapy. The article describes the first Russian practice of implanting venous port systems in 12 patients with type I and II mucopolysaccharidosis and Pompe disease (6 months to 17 years old) to create long-term venous access. Currently, implantable venous port systems are used in 9 (75%) of 12 patients. 4 cases of thrombosis are observed in 3 patients. All of them have been successfully treated. 1 patient had a rotation of the port camera with a membrane facing downwards due to violation of an implantation technique. The camera was adjusted during the second operation.
ISSN:1682-5527
1682-5535

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