RecruitingPhase 1Phase 2NCT06910813

DFT383 in Pediatric Participants With Nephropathic Cystinosis

An Open-label, Multi-center, Phase I/II Study to Assess Safety, Tolerability and Efficacy of DFT383 in Pediatric Participants With Nephropathic Cystinosis, Followed by a Long-term Extension Phase


Sponsor

Novartis Pharmaceuticals

Enrollment

30 participants

Start Date

Jun 2, 2025

Study Type

INTERVENTIONAL

Conditions

Summary

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.


Eligibility

Min Age: 2 YearsMax Age: 5 Years

Inclusion Criteria9

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

Exclusion Criteria6

  • A history of kidney transplantation
  • A prior or planned bone marrow or stem cell transplantation or prior treatment with gene therapy
  • History of malignancy
  • A severe or uncontrolled medical disorder
  • Major surgery within 90 days
  • \. Indomethacin within 2 weeks prior to Screening

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Interventions

GENETICDFT383

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


Locations(4)

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

San Diego, California, United States

Stanford University - Stanford Children's Health

Stanford, California, United States

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

Atlanta, Georgia, United States

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

Houston, Texas, United States

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NCT06910813


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