Status:

COMPLETED

A Study of the Safety, Efficacy and Pharmacokinetics of Glycerol Phenylbutyrate in Pediatric Subjects Under 2 Years of Age With Urea Cycle Disorders

Lead Sponsor:

Amgen

Conditions:

Urea Cycle Disorder

Eligibility:

All Genders

1-2 years

Phase:

PHASE4

Brief Summary

This is an open-label study consisting of a transition period to RAVICTI, followed by a safety extension period for at least 6 months and up to 24 months of treatment with RAVICTI, depending on age at...

Detailed Description

Study acquired from Horizon in 2024.

Eligibility Criteria

Inclusion

  • Male and female subjects up to 2 years of age
  • Signed informed consent by subject's parent/legal guardian
  • UCD diagnosis or suspected diagnosis of any subtype, except N-acetyl glutamate synthetase deficiency. If UCD has not been previously confirmed by genetic testing, consent must be obtained from parent/legal guardian prior to perform genetic testing. If genetic testing is inconsistent with or excludes a UCD diagnosis, the subject will be withdrawn from the study.

Exclusion

  • Use of any investigational drug within 30 days of Day 1
  • Uncontrolled infection (viral or bacterial) or any other condition known to precipitate hyperammonemic crises. Once these precipitating factors are medically controlled, patients presenting in crisis are eligible.
  • Any clinical or laboratory abnormality of Grade 3 or greater severity according to the Common Terminology Criteria for Adverse Events (CTCAE) v4.03, except Grade 3 elevations in ammonia and liver enzymes, defined as levels 5-20 times the upper limit of normal in alanine aminotransferase (ALT), aspartate aminotransferase (AST), or gamma glutamyl transpeptidase (GGT) in a clinically stable subject
  • Any clinical or laboratory abnormality or medical condition that, at the discretion of the Investigator, may put the subject at increased risk by participating in this study
  • Known hypersensitivity to phenylacetate (PAA) or phenylbutyrate (PBA)
  • Liver transplantation, including hepatocellular transplant
  • Subjects on hemodialysis at time of initiating RAVICTI
  • Subjects on RAVICTI for UCD management
  • Currently treated with Carbaglu® (carglumic acid)

Key Trial Info

Start Date :

December 31 2014

Trial Type :

INTERVENTIONAL

Allocation :

ACTUAL

End Date :

July 17 2017

Estimated Enrollment :

27 Patients enrolled

Trial Details

Trial ID

NCT02246218

Start Date

December 31 2014

End Date

July 17 2017

Last Update

July 1 2024

Active Locations (17)

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Page 1 of 5 (17 locations)

1

Kaiser Permanente Regional Metabolic Center

Los Angeles, California, United States, 90027

2

Stanford Center for Clinical & Translational Research & Education

Palo Alto, California, United States, 94034

3

Children's National Medical Center

Washington D.C., District of Columbia, United States, 20010

4

Shands at University of Florida

Gainesville, Florida, United States, 32610