DFT383 in Pediatric Participants With Nephropathic Cystinosis

NCT ID: NCT06910813

Last Updated: 2025-11-10

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

PHASE1/PHASE2

Total Enrollment

30 participants

Study Classification

INTERVENTIONAL

Study Start Date

2025-06-02

Study Completion Date

2044-03-14

Brief Summary

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An open-label, multi-center, phase I/II study to assess the safety, tolerability and efficacy of DFT383 in pediatric participants with nephropathic cystinosis, followed by a long-term extension phase.

The purpose of this clinical study is to assess safety, tolerability, and efficacy of DFT383 in participants aged 2 to 5 years with nephropathic cystinosis. The study consists of a Core Phase and a long-term Extension Phase. DFT383 is a cellular gene therapy.

This study includes an active arm (Cohort 1) of participants treated with study treatment DFT383 and a concurrent reference arm (Cohort 0). Participants in Cohort 0 will not receive study treatment and will only participate in the Core Phase of the study. The study is not randomized and Cohort 0 aims to collect prospective and concurrent data in this rare disease.

Detailed Description

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This study is an open-label, multi-center, phase I/II study to assess the safety, tolerability, and efficacy of DFT383 in participants aged 2 to 5 years with nephropathic cystinosis, followed by a long-term extension phase.

The study includes two Treatment Groups (Cohort 1 and Cohort 0) and consists of a Core Phase and a long-term Extension Phase.

Participants in Cohort 1 will receive DFT383 and participate in both the Core and Extension Phase. Participants in Cohort 0 will not receive study treatment and will participate in the Core Phase only.

The two cohorts will be run in parallel. Investigational sites may participate in one or both cohorts.

Cohort 1 Approximately 15 participants will receive treatment with DFT383 in 3 (sub) cohorts (1A, 1B and 1C) dosed in a staggered approach. The total study duration for a participant in Cohort 1 will be up to 32 months in the core phase and up to 13 years for the long-term extension phase.

Cohort 0 Approximately 15 participants meeting similar inclusion/exclusion criteria and receiving SoC will be enrolled. The Schedule of Activities will be reduced for this Cohort. This cohort 0 is not a direct control but will provide essential context for interpreting the results observed in the participants receiving DFT383. The total study duration for a participant in Cohort 0 will be up to 24 months.

Conditions

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Nephropathic Cystinosis

Keywords

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Cystinosis Nephropathic cystinosis Lysosomal storage disorder CTNS gene DFT383 Cellular gene therapy Cysteamine Renal Fanconi syndrome

Study Design

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Allocation Method

NON_RANDOMIZED

Intervention Model

PARALLEL

Primary Study Purpose

TREATMENT

Blinding Strategy

NONE

Study Groups

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Cohort 1 (DFT383)

Treatment with DFT383

Group Type EXPERIMENTAL

DFT383

Intervention Type GENETIC

DFT383 is an autologous hematopoietic stem cell (HSC) gene therapy.

Cohort 0 (SoC)

No study treatment, will continue with standard of care (cysteamine).

Group Type NO_INTERVENTION

No interventions assigned to this group

Interventions

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DFT383

DFT383 is an autologous hematopoietic stem cell (HSC) gene therapy.

Intervention Type GENETIC

Eligibility Criteria

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Inclusion Criteria

Participants eligible for inclusion in this study must meet all the following criteria:

1. Informed consent in writing from parent(s) or legal guardian(s) must be provided
2. 2 to 5 years of age (including 5 years and 364 days old) at Screening
3. Weight-for-stature is ≥ the third percentile, and is ≥ 10 kg
4. Oral cysteamine therapy for at least 6 months
5. Historic clinical diagnosis of nephropathic cystinosis
6. Laboratory evidence of of renal fanconi syndrome (RFS)
7. Relatively preserved kidney function (eGFR ≥ 60mL/min/1.73m2)
8. Received all age-appropriate vaccinations

Exclusion Criteria

1. A history of kidney transplantation
2. A prior or planned bone marrow or stem cell transplantation or prior treatment with gene therapy
3. History of malignancy
4. A severe or uncontrolled medical disorder
5. Major surgery within 90 days


1\. Indomethacin within 2 weeks prior to Screening
Minimum Eligible Age

2 Years

Maximum Eligible Age

5 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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Novartis Pharmaceuticals

INDUSTRY

Sponsor Role lead

Responsible Party

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Responsibility Role SPONSOR

Locations

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University of California at San Diego - Rady Children's Hospital

San Diego, California, United States

Site Status RECRUITING

Stanford University - Stanford Children's Health

Stanford, California, United States

Site Status RECRUITING

Emory University School of Medicine - Children's Healthcare of Atlanta (recuiting Cohort 0)

Atlanta, Georgia, United States

Site Status RECRUITING

Baylor College of Medicine - Texas Children's Hospital (recuiting Cohort 0)

Houston, Texas, United States

Site Status RECRUITING

Countries

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United States

Central Contacts

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Novartis Pharmaceuticals

Role: CONTACT

Phone: 1-888-669-6682

Email: [email protected]

Novartis Pharmaceuticals

Role: CONTACT

Facility Contacts

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Caitlin Carter

Role: primary

Research Nurse

Role: primary

Laurence (Larry) Greenbaum

Role: primary

Ewa Elenberg

Role: primary

Other Identifiers

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CDFT383A12101

Identifier Type: -

Identifier Source: org_study_id