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Phase 2 N=11 Treatment

Safety and Dose-Finding Study of DTX301 (scAAV8OTC) in Adults With Late-Onset Ornithine Transcarbamylase (OTC) Deficiency

Ornithine Transcarbamylase (OTC) Deficiency

Enrolled (actual)
11
Serious AEs
9.1%
Results posted
Dec 2022
Primary outcome: Primary: Number of Participants With Adverse Events (AEs), Treatment-Emergent Adverse Events (TEAEs), Serious Adverse Events (SAEs), Deaths, and TEAEs Leading to Discontinuation — 1; 1; 0; 1 Participants

Study Design & Population

Study type
Interventional
Phase
Phase 2
Interventions
scAAV8OTC (Genetic); Reactive Corticosteroid Taper Regimen (Drug); Prophylactic Corticosteroid Taper Regimen (Drug)
Age
Adult, Older Adult · 18+ yrs
Sex
All
Sponsor
Ultragenyx Pharmaceutical Inc
Primary completion
Dec 2021

Outcome Measures

OutcomeResultp-value
PRIMARY
Number of Participants With Adverse Events (AEs), Treatment-Emergent Adverse Events (TEAEs), Serious Adverse Events (SAEs), Deaths, and TEAEs Leading to Discontinuation
1; 1; 0; 1; 3; 3
SECONDARY
Change From Baseline Over Time in Rate of Ureagenesis
41.139; 46.857; -29.017; 14.586; 2.363; 20.897
SECONDARY
Change From Baseline Over Time in Area Under the Curve From Time Zero to 24 Hours (AUC0-24) of Plasma Ammonia
97.69; -1334.27; -3790.83; -1095.20; -264.07; -1384.95

Summary

This is a Phase 1/2, open-label, single arm, multicenter, safety and dose finding study of DTX301 in adults with late-onset OTC deficiency. The primary objective of the study is to determine the safety of single intravenous (IV) doses of DTX301.

Eligibility Criteria

Key Inclusion Criteria

  • Males and females ≥18 years of age with documented diagnosis of late onset (defined as first manifestation of signs and symptoms at ≥1 month of age) OTC deficiency, confirmed via enzymatic, biochemical, or molecular testing
  • Documented history of ≥1 symptomatic hyperammonemia event with ammonia ≥100 µmol/L.
  • Subject's OTC deficiency is stable as evidenced by either a) no clinical symptoms of hyperammonemia OR b) an ammonia level 2.0 mg/dL.
  • Participation in another investigational medicine study (including another gene transfer trial) within 3 months of Screening
  • Pregnant or nursing

Note additional inclusion/exclusion criteria may apply, per protocol.

View full record on ClinicalTrials.gov →

Data sourced from ClinicalTrials.gov (NCT02991144). Outcome figures and adverse-event rates are extracted automatically from the registry's posted results and are provided for clinician reference, not as a substitute for the primary publication.

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