Status:

COMPLETED

Neurologic Injuries in Adults With Urea Cycle Disorders

Lead Sponsor:

Andrea Gropman

Collaborating Sponsors:

Office of Rare Diseases (ORD)

Rare Diseases Clinical Research Network

Conditions:

Brain Diseases, Metabolic, Inborn

Urea Cycle Disorder

Eligibility:

All Genders

18-60 years

Brief Summary

Urea cycle disorders (UCDs) are a group of rare inherited metabolism disorders. The purpose of this study is to evaluate how UCD-related neurologic injuries affect adults with one of the most common t...

Detailed Description

UCDs are a group of rare genetic diseases that affect how protein is broken down in the body. The cause of UCDs is a deficiency in one of eight enzymes responsible for removing ammonia, a waste produc...

Eligibility Criteria

Inclusion

  • Inclusion Criteria for Participants with OTCD:
  • Diagnosis of OTCD or heterozygote state of OTCD by metabolic or molecular means. Female participants must be clinically stable and heterozygous for OTCD. Male participants must be hemizygous for late onset OTCD.
  • Inclusion Criteria for Healthy Controls:
  • No known medical or metabolic disorder
  • Inclusion Criteria for All Participants:
  • IQ of at least 80
  • Willing to travel to study site
  • English-speaking
  • Age between 18 and 60 years
  • Exclusion Criteria for All Participants:
  • Currently being treated for an acute illness
  • History of neuropsychiatric drug use
  • Unable to undergo MRI scanning without being sedated
  • Unable to participate in neurocognitive and/or motor testing
  • Metal device in body that might interfere with MRI scanning
  • Pregnancy or breastfeeding

Exclusion

    Key Trial Info

    Start Date :

    March 1 2007

    Trial Type :

    OBSERVATIONAL

    Allocation :

    ACTUAL

    End Date :

    July 1 2010

    Estimated Enrollment :

    46 Patients enrolled

    Trial Details

    Trial ID

    NCT00472732

    Start Date

    March 1 2007

    End Date

    July 1 2010

    Last Update

    June 24 2015

    Active Locations (2)

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    Page 1 of 1 (2 locations)

    1

    George Washington University School of Medicine

    Washington D.C., District of Columbia, United States, 20037

    2

    Georgetown University

    Washington D.C., District of Columbia, United States, 20057