RecruitingNot ApplicableNCT03645460

Gene Therapy for ADA-SCID Using an Improved Lentiviral Vector (Ivlv-ADA)

Gene Therapy Via Intravenous Injection of Lentiviral Vector (Ivlv-ADA) for the Treatment of Adenosine Deaminase-severe Combined Immunodeficiency (ADA-SCID)


Sponsor

Shenzhen Geno-Immune Medical Institute

Enrollment

10 participants

Start Date

Jun 30, 2024

Study Type

INTERVENTIONAL

Conditions

Summary

This is a Phase I/II trial of in vivo lentiviral gene therapy for treating adenosine deaminase severe combined immunodeficiency (ADA-SCID) using a self-inactivating lentiviral vector (LV) ivlv-ADA to functionally correct the genetic defect. The primary objectives are to evaluate the safety and efficacy of the direct intravenous (iv) LV gene therapy protocol.


Eligibility

Min Age: 1 Month

Inclusion Criteria9

  • Diagnosis of classical ADA-SCID based on:
  • A proven defective adenosine deaminase (ADA) gene as defined by direct sequencing of patient DNA.
  • T-cell immune deficiency defined as one or more of the following: CD3+ autologous T cells \< 300/ul, or less than 50% of normal value for in vitro mitogen stimulation, or absent proliferation in vitro to antigens.
  • With severe infections, including but not limited to: pneumonitis; protracted diarrhea requiring total parenteral nutrition; infection with herpes viruses or adenovirus or fungus; disseminated BCG infection.
  • No cytogenetic abnormalities (medullary karyotype) and no detection of main rearrangements associated with acute leukemia of children.
  • No prior allogeneic stem cell transplantation.
  • Life expectancy ≥ 2 months.
  • Negative for HIV infection.
  • Written, informed consent obtained prior to any study-specific procedures.

Exclusion Criteria1

  • None

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Interventions

GENETICDirect intravenous injection of ivlv-ADA lentiviral vector

Injection of ivlv-ADA lentiviral vector at \~1x10\^9 per kg body weight


Locations(2)

Shenzhen Geno-immune Medical Institute

Shenzhen, Guangdong, China

Guilin Hospital of Chinese Traditional and Western Medicine

Guilin, Guangxi, China

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NCT03645460