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Study of Idursulfase-beta (GC1111) in Hunter Syndrome

Phase 2
Conditions
Mucopolysaccharidosis II
Registration Number
NCT02663024
Lead Sponsor
Green Cross Corporation
Brief Summary

This study evaluates the efficacy and safety of three doses of GC1111 in patients with Hunter Syndrome. Participants will be randomized to one of three doses of GC1111 or comparator.

Detailed Description

This is a randomized, double-blind, active-controlled, dose-ranging study, where patient will receive one of the three doses of GC1111 (0.5 mg/kg, 1.0 mg/kg, and 1.5 mg/kg) or ELAPRASE 0.5 mg/kg. Approximately 20 patients will be administrated each study drug once every week as an iv infusion for 24 weeks. Efficacy of GC1111 will be evaluated in Six-Minute Walk Test (6MWT), urine Glycosaminoglycans(uGAG), liver and spleen volume, percent predicted Forced Vital Capacity(FVC), and cardiac size and function. Also immunogenicity, Pharmacokinetics(PK) and safety will be evaluated.

Recruitment & Eligibility

Status
UNKNOWN
Sex
Male
Target Recruitment
20
Inclusion Criteria
  • Male patients between 5 and 35 years of age
  • Informed consent form signed
  • Patients diagnosed with hunter syndrome
  • Previously untreated with an enzyme replacement therapy
Exclusion Criteria
  • History of tracheostomy, bone marrow transplant, or cord blood transplant
  • Treatment with another investigational product within 30 days prior to the start of study drug
  • Known hypersensitivity of any of the ingredients of study drug
  • Patient with severe hunter syndrome who cannot perform 6MWT
  • Female patients

Study & Design

Study Type
INTERVENTIONAL
Study Design
PARALLEL
Primary Outcome Measures
NameTimeMethod
Percent change from baseline in urinary GAG(Glycosaminoglycans) at Week 25Baseline to Week 25
Secondary Outcome Measures
NameTimeMethod
Safety changes from baseline in clinical laboratory tests, physical examination and vital signsBaseline to Week 25
ImmunogenicityBaseline to Week 25

anti-drug-antibody

Pharmacokinetic profile - Maximum observed peak plasma concentration (Cmax)1 and 17 week
Change from baseline in Six Minute Walk Test at Week 25Baseline to Week 25
Incidence of Adverse Events and Serious Adverse EventsBaseline to Week 25
Percent change from baseline in Six Minute Walk Test at Week 25Baseline to Week 25
Change from baseline in Liver volume at Week 25Baseline to Week 25

Liver volume measured by MRI

Change from baseline in Spleen volume at Week 25Baseline to Week 25

Spleen volume measured by MRI

Pharmacokinetic profile - Area under the serum concentration time curve (AUClast)1 and 17 week
Change from baseline in urinary GAG at Week 25Baseline to Week 25
Percent change from baseline in Liver volume at Week 25Baseline to Week 25

Liver volume measured by MRI

Percent change from baseline in Spleen volume at Week 25Baseline to Week 25

Spleen volume measured by MRI

Pharmacokinetic profile - Time at which Cmax is observed (Tmax)1 and 17 week

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