Functional Evaluation in Patients With Urea Cycle Disorders (UCD) During a Driving Task

NCT07701694 | Recruiting

• 1 other identifier: DRIVEUCD
• Study Type: observational
• Target: 60
• Locations: 1 country
• Sites: 1

Brief Summary

This study is being done to understand how the brain works while people with urea cycle disorder (UCD) perform driving tasks that range from easy to difficult and to look at how that compares to traditional tests of thinking and attention.

Conditions

Urea Cycle Disorder

Keywords

Participants with Urea Cycle Disorder; Healthy Volunteers; Cognitive function examination; Motor function examination; Simulated driving task with functional near-infrared spectroscopy monitoring

Outcome Measures

Primary Outcomes (1)

• Changes in Oxyhemoglobin (HbO), Deoxyhemoglobin (HbR) and Total Hemoglobin (HbT) concentrations in the right and left prefrontal cortex in participants with urea cycle disorders (UCD) compared to healthy control volunteers

  Investigators will compare prefrontal cortex hemoglobin concentrations during low cognitive load between patients with UCD and healthy age and sex matched control participants using functional near-infrared spectroscopy (fNIRS). Mean values of hemoglobin concentration differences on the right and left prefrontal cortex between individuals, with and without UCD, during the low cognitive load task will be calculated and compared using two sample t-tests (for normally distributed data) or Wilcoxon rank sum tests (for nonnormally distributed data). P values will be adjusted for multiple comparisons using the Bonferroni method.

  Day 1, during driving simulator task

Secondary Outcomes (1)

• Changes in HbO, HbR and HbT concentrations in the left and right parietal and occipital brain regions in participants with urea cycle disorders (UCD) compared to healthy control volunteers

  Day 1, during driving simulator task

Study Arms (2)

UCD group

Participants with urea cycle disorders (UCD) who meet the Eligibility Criteria and consent to participate

Control group

Healthy controls who meet the Eligibility Criteria and consent to participate

Eligibility Criteria

• Age: 16 Years - 40 Years
• Sex: all
• Healthy Volunteers: Yes
• Age Groups: Child (0-17), Adult (18-64)
• Sampling Method: Non-Probability Sample

Study Population

Individuals who meet the Eligibility Criteria and agree to participate.

You may qualify if:

• Individuals 16 to 40 years with molecular or biochemical confirmation of a urea cycle disorder.
• Healthy individuals will serve as an age and sex matched comparison group. Active driver's license for at least 6 months
• Read and understand English. as the tasks have not been validated in other languages
• IQ \>=70.

You may not qualify if:

• Skin disease that affects the scalp
• Past or present vascular disease
• Head trauma with loss of consciousness in the last year or evidence of cognitive impairment due to and persisting after head trauma
• Motor movement disorder which may cause excessive movement or impede use of the steering wheel and/or pedal
• Premature birth or birth before 32 weeks of gestation
• The participant is taking a stimulant or antidepressant
• Allergy to any component of the device, although expected to be rare
• History of psychosis
• Self-reported motion or cyber sickness
• Self-reported illicit substance use
• Vision disorder

Sponsors & Collaborators

• St. Jude Children's Research Hospital: lead

Study Sites (1)

St. Jude Children's Research Hospital

Memphis, Tennessee, 38105, United States

RECRUITING

Related Links

• St. Jude Children's Research Hospital
• Clinical Trials Open at St. Jude

MeSH Terms

Conditions

Urea Cycle Disorders, Inborn

Condition Hierarchy (Ancestors)

Brain Diseases, Metabolic, Inborn; Brain Diseases, Metabolic; Brain Diseases; Central Nervous System Diseases; Nervous System Diseases; Amino Acid Metabolism, Inborn Errors; Metabolism, Inborn Errors; Genetic Diseases, Inborn; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Metabolic Diseases; Nutritional and Metabolic Diseases

Study Officials

• Andrea Gropman, M.D.

  St. Jude Children's Research Hospital

  PRINCIPAL INVESTIGATOR

Central Study Contacts

Jean Laboe, RN

CONTACT

901-595-1693; jean.laboe@stjude.org

Study Design

• Study Type: observational
• Observational Model: CASE CONTROL
• Time Perspective: PROSPECTIVE
• Sponsor Type: OTHER
• Responsible Party: SPONSOR

Study Record Dates

• First Submitted: July 8, 2026
• First Posted: July 14, 2026
• Study Start: July 20, 2026
• Primary Completion (Estimated): August 1, 2031
• Study Completion (Estimated): August 1, 2032
• Last Updated: July 31, 2026

Record last verified: 2026-07

Locations

US (1)