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Phase 3 N=121 Randomized Triple-blind Treatment

A Study to Evaluate the Efficacy and Safety of BMN 111 in Children With Achondroplasia

Achondroplasia

Enrolled (actual)
121
Serious AEs
5.8%
Results posted
Mar 2022
Primary outcome: Primary: Change From Baseline in Annualized Growth Velocity (AGV) at Week 52 — 1.71; 0.13 cm/year — p=< 0.0001

Study Design & Population

Study type
Interventional
Phase
Phase 3
Interventions
BMN 111 (Drug); Placebo (Drug)
Age
Pediatric, Adult · 5+ yrs
Sex
All
Sponsor
BioMarin Pharmaceutical
Primary completion
Oct 2019

Outcome Measures

OutcomeResultp-value
PRIMARY
Change From Baseline in Annualized Growth Velocity (AGV) at Week 52
1.71; 0.13 < 0.0001 sig
SECONDARY
Change From Baseline in Height Z-score at Week 52
0.27; -0.01 < 0.0001 sig
SECONDARY
Change From Baseline in Upper to Lower Segment Body Ratio at Week 52
-0.03; -0.02 = 0.506
SECONDARY
Summary of Subjects Experiencing Adverse Events (AEs) During Treatment
59; 60; 3; 4; 53; 51

Summary

The intent and design of this Phase 3 study is to assess BMN 111 as a therapeutic option for the treatment of children with Achondroplasia.

Eligibility Criteria

Inclusion Criteria

  • Parent(s) or guardian(s) consent
  • 5 to 2 mg/dL
  • Chronic anemia
  • Baseline systolic blood pressure (BP) 450 msec
  • Have an unstable condition likely to require surgical intervention during the study (including progressive cervical medullary compression or severe untreated sleep apnea)
  • Decreased growth velocity (< 1.5 cm/yr) over a period of 6 months or evidence of growth plate closure (proximal tibia, distal femur)
  • Treated with growth hormone, insulin-like growth factor 1 (IGF-1), or anabolic steroids in the previous 6 months or treatment greater than 6 months at any time
  • Greater than 1 month treatment with oral corticosteroids (low-dose ongoing inhaled steroid for asthma, or intranasal steroids, are acceptable) in the previous 12 months
  • Planned or expected to have limb-lengthening surgery during the study period. Subjects with previous limb- lengthening surgery may enroll if surgery occurred at least 18 months prior to the study and healing is complete without sequelae.
  • Planned or expected bone-related surgery (ie. surgery involving disruption of bone cortex, excluding tooth extraction), during the study period. Subjects with previous bone-related surgery may enroll if surgery occurred at least 6 months prior to the study and healing is complete without sequelae.
  • Had a fracture of the long bones or spine within 6 months prior to screening
  • History of severe untreated sleep apnea
  • New initiation of sleep apnea treatment (e.g. CPAP or sleep apnea-mitigating surgery) in the previous 2 months prior to screening
  • History of hip surgery or hip dysplasia atypical for achondroplastic subjects
  • History of clinically significant hip injury in the 30 days prior to screening
  • History of slipped capital femoral epiphysis or avascular necrosis of the femoral head
  • Abnormal findings on baseline clinical hip exam or imaging assessments that are determined to be clinically significant
  • Concurrent disease or condition that would interfere with study participation or safety evaluations, for any reason
  • Condition or circumstance that places the subject at high risk for poor treatment compliance or for not completing the study
View full record on ClinicalTrials.gov →

Data sourced from ClinicalTrials.gov (NCT03197766). 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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