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Intervention Review

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Enzyme replacement therapy with idursulfase for mucopolysaccharidosis type II (Hunter syndrome)

  1. Edina MK da Silva1,*,
  2. Maria Wany Louzada Strufaldi2,
  3. Régis B Andriolo3,
  4. Laercio A Silva4

Editorial Group: Cochrane Cystic Fibrosis and Genetic Disorders Group

Published Online: 8 JAN 2014

Assessed as up-to-date: 6 NOV 2013

DOI: 10.1002/14651858.CD008185.pub3


How to Cite

da Silva EMK, Strufaldi MWL, Andriolo RB, Silva LA. Enzyme replacement therapy with idursulfase for mucopolysaccharidosis type II (Hunter syndrome). Cochrane Database of Systematic Reviews 2014, Issue 1. Art. No.: CD008185. DOI: 10.1002/14651858.CD008185.pub3.

Author Information

  1. 1

    Universidade Federal de São Paulo, Emergency Medicine and Evidence Based Medicine, São Paulo, São Paulo, Brazil

  2. 2

    Universidade Federal de São Paulo, São Paulo, São Paulo, Brazil

  3. 3

    Universidade do Estado do Pará, Department of Public Health, Belém, Pará, Brazil

  4. 4

    Universidade Federal de São Paulo, Department of Urology, São Paulo, São Paulo, Brazil

*Edina MK da Silva, Emergency Medicine and Evidence Based Medicine, Universidade Federal de São Paulo, Rua Borges Lagoa 564 cj 64, Vl. Clementino, São Paulo, São Paulo, 04038-000, Brazil. edinaksilva@terra.com.br.

Publication History

  1. Publication Status: New search for studies and content updated (no change to conclusions)
  2. Published Online: 8 JAN 2014

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This is not the most recent version of the article. View current version (05 FEB 2016)

[Figure 1]
Figure 1. Study flow diagram.
[Figure 2]
Figure 2. Forest plot of comparison: 1 Idursulfase 0.5 mg/kg versus placebo, outcome: 1.1 Primary outcome: Six-minute walk test (6MWT).
[Analysis 1.1]
Analysis 1.1. Comparison 1 Idursulfase 0.5 mg/kg versus placebo, Outcome 1 Six-minute walk test (6MWT) (53 weeks).
[Analysis 1.2]
Analysis 1.2. Comparison 1 Idursulfase 0.5 mg/kg versus placebo, Outcome 2 % predicted FVC (53 weeks).
[Analysis 1.3]
Analysis 1.3. Comparison 1 Idursulfase 0.5 mg/kg versus placebo, Outcome 3 Absolute FVC (L) (53 weeks).
[Analysis 1.4]
Analysis 1.4. Comparison 1 Idursulfase 0.5 mg/kg versus placebo, Outcome 4 6MWT+ % predicted FVC (composite score) (53 weeks).
[Analysis 1.5]
Analysis 1.5. Comparison 1 Idursulfase 0.5 mg/kg versus placebo, Outcome 5 Liver volume (%) (53 weeks).
[Analysis 1.6]
Analysis 1.6. Comparison 1 Idursulfase 0.5 mg/kg versus placebo, Outcome 6 Spleen volume (%) (53 weeks).
[Analysis 1.7]
Analysis 1.7. Comparison 1 Idursulfase 0.5 mg/kg versus placebo, Outcome 7 Urine GAG μg/mg creatinine (%)(53 weeks).
[Analysis 1.8]
Analysis 1.8. Comparison 1 Idursulfase 0.5 mg/kg versus placebo, Outcome 8 One or more infusion-related adverse events.
[Analysis 1.9]
Analysis 1.9. Comparison 1 Idursulfase 0.5 mg/kg versus placebo, Outcome 9 Serious adverse events.