Modern Approaches to the Management of Children with Mucopolysaccharidosis Type I

• Main Authors: Galina V. Baidakova (Author), Alexander A. Baranov (Author), Irina V. Vakhlova (Author), Nato D. Vashakmadze (Author), Elena Yu. Voskoboeva (Author), Ekaterina Yu. Zakharova (Author), Ludmila M. Kuzenkova (Author), Sergey I. Kutsev (Author), Valentina I. Larionova (Author), Tina V. Lobzhanidze (Author), Ludmila K. Mikhailova (Author), Svetlana V. Mikhailova (Author), Sergey V. Moiseev (Author), Leyla S. Namazova-Baranova (Author), Sergey S. Nikitin (Author), Natalya L. Pechatnikova (Author), Olga A. Polyakova (Author), Alla N. Semyachkina (Author), Olga V. Udalova (Author)
• Format: Book
• Published: Union of pediatricians of Russia, 2022-10-01T00:00:00Z.
• Subjects: article
• Online Access: Connect to this object online.

Summary

This article presents modern data on epidemiology, etiology, and clinical manifestations of mucopolysaccharidosis (MPS) type I in children. MPS develops due to deficiency of particular lysosomal enzyme which determines the disease type. The article considers in details disease's pathogenesis and classification. Evidence-based approaches to diagnosis (differential diagnosis included) are covered, moreover, special attention is paid to pathogenetic, symptomatic, and surgical treatment of MPS.

Item Description

1727-5776
2500-3089
10.15690/pf.v19i4.2443