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Phase 3 N=173 Randomized Quadruple-blind Treatment

Long-Term Efficacy and Safety Extension Study of BMN 110 in Patients With Mucopolysaccharidosis IVA (Morquio A Syndrome)

Mucopolysaccharidosis IV A · Morquio A Syndrome · MPS IVA

Enrolled (actual)
173
Serious AEs
51.4%
Results posted
May 2017
Primary outcome: Primary: Change From Baseline in 6-minute Walk (6MW) Test - ITT — 219.7; 207.2; 205.7; 203.9 meters

Study Design & Population

Study type
Interventional
Phase
Phase 3
Interventions
BMN 110 - Weekly (Drug); BMN 110 - Every Other Week (Drug)
Age
Pediatric, Adult, Older Adult · 5+ yrs
Sex
All
Sponsor
BioMarin Pharmaceutical
Primary completion
Jun 2016

Outcome Measures

OutcomeResultp-value
PRIMARY
Change From Baseline in 6-minute Walk (6MW) Test - ITT
219.7; 207.2; 205.7; 203.9; 207.2; 23.8
PRIMARY
Change From Baseline in 6-minute Walk (6MW) Test - MPP
208.7; 190.2; 195.9; 208.8; 201.6; 30.3
SECONDARY
Change From Baseline in 3-minute Stair Climb Test - ITT
33.1; 26.9; 27.1; 29.6; 28.9; 4.7
SECONDARY
Change From Baseline in 3-minute Stair Climb Test - MPP
33.1; 24.8; 25.6; 31.3; 28.7; 3.4
SECONDARY
Change From Baseline in Urine Keratan Sulfate - ITT
22.7; 28.5; 28.6; 26.9; 27.1; 3.4
SECONDARY
Change From Baseline in Urine Keratan Sulfate - MPP
21.2; 27.5; 27.2; 24.9; 25.5; 1.2

Summary

This Phase 3 extension study will evaluate the long-term efficacy and safety of BMN 110 2.0 mg/kg/week and/or BMN 110 2.0 mg/kg/every other week in patients with mucopolysaccharidosis IVA (Morquio A Syndrome).

Eligibility Criteria

Inclusion Criteria

  • Must have completed MOR-004
  • Is willing and able to provide written, signed informed consent. Or in the case of patients under the age of 18 (or other age as defined by regional law or regulation), provide written assent (if required) and have written informed consent, signed by a legally authorize representative, after the nature of the study has been explained, and prior to performance of research-related procedures.
  • If sexually active, must be willing to use an acceptable method of contraception while participating in the study.
  • If female, of childbearing potential, must have a negative pregnancy test at Baseline and be willing to have additional pregnancy tests done during the study.

Exclusion Criteria

  • Is pregnant or breastfeeding, at Baseline, or planning to become pregnant (self or partner) at any time during the study.
  • Has used any investigational product (other than BMN 110 in MOR-004), or investigational medical device, within 30 days prior to Baseline; or is required to use any investigational agent prior to completion of all scheduled study assessments.
  • Was enrolled in a previous BMN 110 study, other than MOR-004.
  • Has a concurrent disease or condition, including but not limited to, symptomatic cervical spine instability, clinically significant spinal cord compression, or severe cardiac disease that would interfere with study participation, or pose a safety risk, as determined by the Investigator.
  • Has any condition that, in the view of the Investigator, places the patient at high risk of poor treatment compliance or of not completing the study.
View full record on ClinicalTrials.gov →

Data sourced from ClinicalTrials.gov (NCT01415427). Outcome figures and adverse-event rates are extracted automatically from the registry's posted results and are provided for clinician reference, not as a substitute for the primary publication.

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