A Phase 2 Basket Study of Vosoritide in Children With Turner Syndrome, SHOX Deficiency and Noonan Syndrome With an Inadequate Response to Human Growth Hormone

NCT ID: NCT06668805

Last Updated: 2025-08-06

Study Results

Results pending

The study team has not published outcome measurements, participant flow, or safety data for this trial yet. Check back later for updates.

Basic Information

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Recruitment Status

RECRUITING

Clinical Phase

PHASE2

Total Enrollment

72 participants

Study Classification

INTERVENTIONAL

Study Start Date

2024-11-22

Study Completion Date

2041-09-30

Brief Summary

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The purpose of this basket study in children with Turner syndrome, SHOX deficiency, and Noonan syndrome is to evaluate the effect of 3 doses of vosoritide versus hGH on growth as measured by AGV after 6 months of treatment. The long-term efficacy and safety of vosoritide at the therapeutic dose will be evaluated up to FAH.

Detailed Description

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This is a Phase 2, randomized, active-controlled, multicenter, basket study of vosoritide in children with Turner syndrome, short stature homeobox-containing gene (SHOX) deficiency, or Noonan syndrome who have an inadequate response to human growth hormone (hGH) treatment. The study is intended to characterize the short-term efficacy and safety of 3 dosing regimens of vosoritide versus hGH. The efficacy and safety of the vosoritide therapeutic dose will be further evaluated, with a comparison to hGH after 2 years of treatment, and an analysis of the impact of vosoritide on final adult height (FAH).

Conditions

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Short Stature Homeobox- Containing Gene SHOX Deficiency Noonan Syndrome Turner Syndrome

Study Design

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Allocation Method

RANDOMIZED

Intervention Model

PARALLEL

Primary Study Purpose

TREATMENT

Blinding Strategy

TRIPLE

Participants Caregivers Investigators

Study Groups

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Vosoritide Dose 1 - Low Dose

Drug: Vosoritide - Dose 1 Injection

• Experimental Drug Lyophilized powder for reconstitution

Group Type EXPERIMENTAL

Vosoritide Injection

Intervention Type DRUG

Modified recombinant human C-type natriuretic peptide Vosoritide

Vosoritide Dose 2 - Medium Dose

Drug: Vosoritide - Dose 2 Injection

• Experimental Drug Lyophilized powder for reconstitution

Group Type EXPERIMENTAL

Vosoritide Injection

Intervention Type DRUG

Modified recombinant human C-type natriuretic peptide Vosoritide

Vosoritide Dose 3- High Dose

Drug: Vosoritide Dose 3 Injection

• Experimental Drug Lyophilized powder for reconstitution

Group Type EXPERIMENTAL

Vosoritide Injection

Intervention Type DRUG

Modified recombinant human C-type natriuretic peptide Vosoritide

Human Growth Hormone

Drug: Human Growth Hormone

Group Type ACTIVE_COMPARATOR

Human Growth Hormone

Intervention Type DRUG

Commercial product containing somatotropin.

Interventions

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Vosoritide Injection

Modified recombinant human C-type natriuretic peptide Vosoritide

Intervention Type DRUG

Human Growth Hormone

Commercial product containing somatotropin.

Intervention Type DRUG

Eligibility Criteria

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Inclusion Criteria

1. Participants must be ≥ 3 years old, and \< 11 years old (females) or \< 12 years old (males), at the time of signing the informed consent form
2. A genetically confirmed diagnosis of Turner syndrome, SHOX deficiency or Noonan syndrome.
3. A height assessment corresponding to a height Z-score of \> -2.00 SDs and ≤ -1.75 SDs (up to 20% of participants)/≤ -2.00 SDs (at least 80% of participants) in reference to the general population of the same age and sex.
4. Tanner Stage 1, at time of signing the ICF.
5. Have been receiving continuous hGH for the treatment of short stature associated with their condition for a minimum of 1 year immediately prior to enrollment and be receiving a dose of ≥ 0.35 mg/kg weekly, with no weight-based dosing changes in the last 6 months and none planned in the future.
6. Are willing to continue on hGH at their current dose for the Baseline Growth Phase, and for 2 years post randomization if randomized to the hGH arm.
7. Inadequate response to prior hGH treatment.

Exclusion Criteria

1. Participants with Turner syndrome known to have Y-chromosome material unless they have undergone gonadectomy and have fully external female genitalia.
2. Diagnosis of systemic disease or condition that may cause short stature other than Turner syndrome, SHOX deficiency, or Noonan syndrome, eg, renal, neoplastic, pulmonary, cardiac, gastrointestinal, immunologic and metabolic disease.
3. Bone age advanced beyond chronological age by more than 2 years.
4. Uncorrected congenital heart disease which places the participant at increased risk of an adverse cardiac outcome in the setting of hypotension,
5. Have an unstable condition likely to require surgical intervention during the study.
6. Evidence of decreased growth velocity (AGV \< 1.5 cm/year) as assessed over a period of at least 6 months and growth plate closure assessed using bilateral lower extremity X-rays.
7. Previous limb-lengthening surgery, or planned or expected to have limb lengthening surgery during the study period.
8. Planned or expected bone-related surgery (ie, surgery involving disruption of bone cortex, excluding tooth extraction), during the study period.
Minimum Eligible Age

3 Years

Maximum Eligible Age

11 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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BioMarin Pharmaceutical

INDUSTRY

Sponsor Role lead

Responsible Party

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Responsibility Role SPONSOR

Principal Investigators

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Medical Director, MD, PhD

Role: STUDY_DIRECTOR

BioMarin Pharmaceutical

Locations

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Children's Hospital Colorado

Aurora, Colorado, United States

Site Status RECRUITING

Nemours Children's Hospital, Delaware (Alfred I. Dupont Hospital for Children)

Wilmington, Delaware, United States

Site Status RECRUITING

Children's National Medical Center

Washington D.C., District of Columbia, United States

Site Status RECRUITING

Nicklaus Children's Hospital

Miami, Florida, United States

Site Status NOT_YET_RECRUITING

St. Luke's Children's Endocrinology and Diabetes

Boise, Idaho, United States

Site Status RECRUITING

Kentucky Children's Hospital

Lexington, Kentucky, United States

Site Status RECRUITING

New York Medical College

Boston, Massachusetts, United States

Site Status RECRUITING

M Health Fairview Pediatric Specialty Clinic - Explorer

Minneapolis, Minnesota, United States

Site Status RECRUITING

Icahn School of Medicine at Mount Sinai

New York, New York, United States

Site Status RECRUITING

Children's Hospital at Montefiore

The Bronx, New York, United States

Site Status RECRUITING

Atrium Health Carolinas Medical Center

Charlotte, North Carolina, United States

Site Status RECRUITING

Children's Medical Center Dallas

Dallas, Texas, United States

Site Status RECRUITING

University of Texas Health Science Center at Houston (UT Health)

Houston, Texas, United States

Site Status RECRUITING

Hôpital de la Timone

Marseille, Bouches-du-Rhône, France

Site Status RECRUITING

Angers University Hospital Center

Angers, Maine-et-Loire, France

Site Status RECRUITING

CHU de Toulouse - Hôpital des Enfants

Toulouse, Occitanie, France

Site Status RECRUITING

South Paris University Hospitals - Bicetre Hospital

Le Kremlin-Bicêtre, Paris, France

Site Status RECRUITING

Hôpital Robert-Debré

Paris, Paris, France

Site Status RECRUITING

IRCCS Istituto Giannina Gaslini

Genova, Genoa, Italy

Site Status NOT_YET_RECRUITING

Central University Hospital of Asturias

Oviedo, Principality of Asturias, Spain

Site Status NOT_YET_RECRUITING

Countries

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United States France Italy Spain

Central Contacts

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Trial Specialist

Role: CONTACT

1-800-983-4587

Study Manager

Role: CONTACT

1-800-983-4587

Facility Contacts

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Shanlee Davis

Role: primary

720-777-5251

Chijioke Ikomi

Role: primary

302-651-5965

Andrew Dauber

Role: primary

Ana Maria Rodriguez Barreto

Role: primary

Daniel Flynn

Role: primary

2083817340

Yuri Zarate

Role: primary

859-257-1000

Shilpa Mehta

Role: primary

Kyriakie Sarafoglou

Role: primary

Cassie Mintz

Role: primary

Laurie Cohen

Role: primary

718-920-4664

Edwin Ferren

Role: primary

Nadia Merchant

Role: primary

214-456-5959

David Rodriguez Buritica

Role: primary

Rachel Reynaud

Role: primary

Regis Coutant

Role: primary

Thomas Edouard

Role: primary

Agnes Linglart

Role: primary

Jean-Claude Carel

Role: primary

Mohamad Maghnie

Role: primary

Isolina Riano Galan

Role: primary

Other Identifiers

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111-211

Identifier Type: -

Identifier Source: org_study_id

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