A biochemical and physicochemical comparison of two recombinant enzymes used for enzyme replacement therapies of hunter syndrome

2014 ◽  
Vol 31 (4) ◽  
pp. 309-315 ◽  
Author(s):  
Yo Kyung Chung ◽  
Young Bae Sohn ◽  
Jong Mun Sohn ◽  
Jieun Lee ◽  
Mi Sun Chang ◽  
...  
Keyword(s):  
Hunter Syndrome ◽  
Recombinant Enzymes ◽  
Enzyme Replacement

scholarly journals Allergic Reactions at Enzyme Replacement Therapy in Children with Mucopolysaccharidosis Type II

2021 ◽  
Author(s):  
Julia G. Levina ◽  
Nato D. Vashakmadze ◽  
Leyla S. Namazova-Baranova ◽  
Elena A. Vishneva ◽  
Natalia V. Zhurkova ◽  
...  
Keyword(s):  
Enzyme Replacement Therapy ◽  
Replacement Therapy ◽  
Hunter Syndrome ◽  
Hereditary Disease ◽  
Mucopolysaccharidosis Type ◽  
Type Ii ◽  
Mucopolysaccharidosis Type Ii ◽  
Recombinant Enzymes ◽  
Enzyme Replacement ◽  
Mps Ii

Mucopolysaccharidosis type II (MPS II; Hunter syndrome) is rare hereditary disease caused by changes in the IDS gene and associated deficiency of lysosomal enzyme iduronate-2-sulfatase (I2S). The main treatment scheme for children with MPS II is enzyme replacement therapy (ERT) with recombinant human I2S. The major issue of ERT is development of allergic (sometimes up to severe anaphylaxis) reactions to recombinant enzymes. The article covers features of infusion-related reactions to ERT, it describes pathogenesis, diagnostic criteria management algorithm of anaphylaxis. Whereas, there is the need of further studies on allergic infusion-related reactions to ERT in children.

Long-term biomarker and cognitive follow-up of children with Hunter syndrome receiving intrathecal enzyme replacement therapy

2015 ◽  
Vol 114 (2) ◽  
pp. S83 ◽  
Author(s):  
Joseph Muenzer ◽  
Christian J. Hendriksz ◽  
Margot B. Stein ◽  
Zheng Fan ◽  
Shauna Kearney ◽  
...  
Keyword(s):  
Enzyme Replacement Therapy ◽  
Replacement Therapy ◽  
Hunter Syndrome ◽  
Enzyme Replacement
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