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A Study of Vosoritide Versus Placebo in Children With Hypochondroplasia Aged 0 to < 36 Months

Not Applicable
Recruiting
Conditions
Hypochondroplasia
Interventions
Drug: Placebo
Registration Number
NCT07126262
Lead Sponsor
BioMarin Pharmaceutical
Brief Summary

The purpose of this study is to evaluate the safety and efficacy of daily administration of vosoritide in participants with HCH aged 0 to \< 36 months over a 52-week period.

Detailed Description

Study 111-212 is a Phase 2, randomized, double-blind, placebo-controlled, multicenter study to assess the safety and efficacy of vosoritide versus placebo in infants and young children with HCH.

Eligible participants with documented HCH confirmed by genetic testing will be randomized in a 1:1 ratio to receive vosoritide or placebo. Participants will receive study treatment daily for 52 weeks by subcutaneous (SC) injection, followed by a 2-week safety follow-up visit. Vosoritide dosing will follow a weight-band regimen.

Recruitment & Eligibility

Status
RECRUITING
Sex
All
Target Recruitment
60
Inclusion Criteria
  1. Participants must be 0 to < 36 months of age at randomization.
  2. Participants must have a confirmed genetic diagnosis of HCH (obtained via whole genome sequencing; presence of a FGFR3 pathogenic variant associated with HCH).
  3. Participants aged 0 to < 12 months must have a height Z-score of ≤ -1.0 SDS andparticipants aged ≥ 12 to < 36 months must have a height Z-score of ≤ -2.0 SDS in reference to the average stature of the same sex and age, as calculated using the Center for Disease Control and Prevention (CDC) growth charts.
  4. Participant's weight at the Day 1 visit (pre-treatment) must be ≥ 3 kg.

Key

Exclusion Criteria
  1. Short stature condition other than HCH (eg, ACH, trisomy 21, pseudoachondroplasia).
  2. Have an unstable medical condition likely to require surgical intervention during the study period.
  3. Taking any of the prohibited medications.
  4. Have been treated with growth hormone, insulin-like growth factor 1 (IGF-1), or anabolic steroids in the 6 months prior to Screening, or long-term treatment (> 3 months) at any time.
  5. Require any investigational agent prior to completion of study period.
  6. Have received another investigational product or investigational medical device within 30 days prior to the Screening visit.
  7. Have used any other investigational product or investigational medical device for the treatment of HCH or short stature at any time.
  8. Have current malignancy, history of malignancy, or currently under work-up for suspected malignancy.
  9. Have known hypersensitivity to vosoritide or its excipients.
  10. Have a condition or circumstance that, in the view of the investigator, places the participant at high risk for poor treatment compliance or for not completing the study.
  11. Have any concurrent disease or condition that, in the view of the investigator, will interfere with study participation or safety evaluations, for any reason.

Study & Design

Study Type
INTERVENTIONAL
Study Design
PARALLEL
Arm && Interventions
GroupInterventionDescription
Placebo injection with vial and syringePlaceboSubcutaneous injection of recommended dose of placebo
Vosoritide injection with vial and syringeVosoritideSubcutaneous injection of recommended dose of vosoritide based on weight-band dosing once daily.
Primary Outcome Measures
NameTimeMethod
Incidence of treatment-emergent adverse eventsFrom baseline to end of treatment at 52 weeks
Incidence of serious adverse events versus placebo over the course of the studyFrom baseline to end of treatment at 52 weeks
Changes from baseline in heart rateAt week 13, at week 26, at week 39, at week 52

Units of measure: bpm

Change from baseline in height Z-scoreAt week 52
Changes from baseline in standard clinical laboratory values (hematology, urinalysis, and chemistry)At week 26, at week 52
Changes from baseline in respiratory rateAt week 13, at week 26, at week 39, at week 52

Units of measure: breaths/min

Changes from baseline in temperatureAt week 13, at week 26, at week 39, at week 52

Units of measure: celsius

Changes from baseline in blood pressureAt week 13, at week 26, at week 39, at week 52

Units of measure: mmHg

Secondary Outcome Measures
NameTimeMethod
Change in heightAt week 52
Cumulative annualized growth velocity (AGV)At week 52
Change from baseline in lumbar spine BMC as measured by DXAAt week 52
Apparent volume of distribution of vosoritide based upon the terminal phase (Vz/F)At week 26, at week 52
Time vosoritide is present at maximum concentration (Tmax)At week 26, at week 52
Maximum concentration (Cmax) of vosoritide in plasmaAt week 26, at week 52
Change from pre-dose at pre-specified timepoints versus placebo in cyclic guanine monophosphate (cGMP)At week 26 and week 52
Incidence of otitis mediaFrom baseline to end of treatment at 52 weeks
Seizure frequency over the course of the studyFrom baseline to end of treatment at 52 weeks
6-month interval AGVAt week 26, at week 52
Change from baseline in upper to lower body segment ratioAt week 52
Change from baseline in lumbar spine BMD Z-scoreAt week 52
Change from baseline in arm spanAt week 52
Change from baseline in total body (less head) bone mineral content (BMC) as measured by DXAAt week 52
Elimination half-life of vosoritide (t½)At week 26, at week 52
Apparent clearance of vosoritideAt week 26, at week 52
Change from baseline in total body (less head) bone mineral density (BMD) Z-scoreAt week 52
Area under the plasma vosoritide concentration time-curve from time 0 to infinity (AUC0-∞)At week 26, at week 52
Area under the plasma vosoritide concentration time-curve from time 0 to the last measurable concentration (AUC0-t)At week 26, at week 52

Trial Locations

Locations (19)

Phoenix Children's Hospital - Thomas Campus (Main)

🇺🇸

Phoenix, Arizona, United States

Cedars-Sinai Medical Center

🇺🇸

Los Angeles, California, United States

Benioff Children's Hospital - Oakland

🇺🇸

Oakland, California, United States

Children's National Medical Center

🇺🇸

Washington, District of Columbia, United States

Ann & Robert H. Lurie Children's Hospital of Chicago

🇺🇸

Chicago, Illinois, United States

The Johns Hopkins University School of Medicine

🇺🇸

Baltimore, Maryland, United States

University of Minneasota Masonic Children's Hospital

🇺🇸

Minneapolis, Minnesota, United States

University of Missouri

🇺🇸

Columbia, Missouri, United States

Children's Wisconsin - Fox Valley Hospital

🇺🇸

Neenah, Wisconsin, United States

Royal Children's Hospital Melbourne

🇦🇺

Parkville, Victoria, Australia

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Phoenix Children's Hospital - Thomas Campus (Main)
🇺🇸Phoenix, Arizona, United States

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