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MUCOPOLYSACCHARIDOSIS TYPE II
- Source :
- Педиатрическая фармакология, Vol 8, Iss 3, Pp 66-68 (2011)
- Publication Year :
- 2011
- Publisher :
- Union of pediatricians of Russia, 2011.
-
Abstract
- Article is devoted to one of the orphan diseases — mucopolysaccharidosis (MPS), which is the result of any lysosomal enzyme deficiency (which determines the type of illness). The most common is the MPS type II (Hunter syndrome), developing as a result of deficiency of the enzyme alpha-L-iduronosulphatsulphataze. The authors are observing the largest group of children with this pathology in the Russian population — 40 patients. On the example of their own clinical cases the only existing on the date the pathogenetic treatment is provided — replacement therapy with idursulphase that significantly improves the disease prognosis.Key words: MPS, types, Hunter syndrome, clinical course, diagnosis, treatment, prognosis, children.
- Subjects :
- Pediatrics
RJ1-570
Therapeutics. Pharmacology
RM1-950
Subjects
Details
- Language :
- Russian
- ISSN :
- 17275776 and 25003089
- Volume :
- 8
- Issue :
- 3
- Database :
- Directory of Open Access Journals
- Journal :
- Педиатрическая фармакология
- Publication Type :
- Academic Journal
- Accession number :
- edsdoj.94e6f5f630bb433f8116ad3e53a6e49c
- Document Type :
- article