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

29. juli 2026 opdateret af: St. Jude Children's Research Hospital

Cognitive and Motor Function Examination in Patients With Urea Cycle Disorders (UCD) During a Driving Task Using Functional Near Infrared Spectroscopy (fNIRS)

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.

Studieoversigt

Status

Rekruttering

Betingelser

Urea cyklus lidelse

Detaljeret beskrivelse

Primary Objective

To use fNIRS to study brain functional behavior in subjects with UCD by measuring the changes in HbO, HbR and HbT concentrations versus healthy control volunteers during the baseline driving task on the left and right prefrontal cortex (LPFC, RPFC).

Secondary Objective

To include other cortical regions such as temporal and occipital regions, to investigate the neurocognitive changes in these regions which may or may not change with driving. Thus, we will measure changes in HbO, HbR and HbT concentrations during the baseline driving task in four more brain regions (left and right parietal and occipital regions, LPA, RPA, LOL, ROL).

This is a non-therapeutic, non-interventional study in which brain fNIRS (functional near-infrared spectroscopy) measures will be compared under simulated driving conditions between patients with UCD vs. age and sex-matched control participants.

Participants who agree to participate will have Information about their medical history collected, have a basic neurological exam and answer questions about their health, diet, and driving habits. They will complete some simple thinking and memory tests. These tests will help investigators understand skills like memory, attention, and problem-solving. Participants will also wear a soft cap with small sensors called an fNIRS (functional near-infrared spectroscopy) device while they complete a driving simulator activity. This cap is noninvasive and measures how the brain uses oxygen while they drive in the simulator.

All study activities will be completed on the day of enrollment.

Undersøgelsestype

Observationel

Tilmelding (Anslået)

Kontakter og lokationer

Dette afsnit indeholder kontaktoplysninger for dem, der udfører undersøgelsen, og oplysninger om, hvor denne undersøgelse udføres.

Studiekontakt

Navn: Jean Laboe, RN
Telefonnummer: 901-595-1693
E-mail: jean.laboe@stjude.org

Studiesteder

Forenede Stater
- Tennessee
  - Memphis, Tennessee, Forenede Stater, 38105
    - Rekruttering
    - St. Jude Children's Research Hospital
    - Kontakt:
      
      Jean Laboe, RN
      
      Telefonnummer: 901-595-1693
      
      E-mail: jean.laboe@stjude.org

Deltagelseskriterier

Forskere leder efter personer, der passer til en bestemt beskrivelse, kaldet berettigelseskriterier. Nogle eksempler på disse kriterier er en persons generelle helbredstilstand eller tidligere behandlinger.

Berettigelseskriterier

Aldre berettiget til at studere

Barn
Voksen

Tager imod sunde frivillige

Prøveudtagningsmetode

Ikke-sandsynlighedsprøve

Studiebefolkning

Individuals who meet the Eligibility Criteria and agree to participate.

Beskrivelse

Inclusion Criteria:

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.

Exclusion Criteria:

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

Studieplan

Dette afsnit indeholder detaljer om studieplanen, herunder hvordan undersøgelsen er designet, og hvad undersøgelsen måler.

Hvordan er undersøgelsen tilrettelagt?

Design detaljer

Antal grupper/kohorter

Kohorter og interventioner

Gruppe / kohorte
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

Hvad måler undersøgelsen?

Primære resultatmål

Resultatmål	Foranstaltningsbeskrivelse	Tidsramme
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 Tidsramme: Day 1, during driving simulator task	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

Sekundære resultatmål

Resultatmål	Foranstaltningsbeskrivelse	Tidsramme
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 Tidsramme: Day 1, during driving simulator task	Investigators will compare left and right parietal and occipital regions 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 left and right parietal and occipital regions 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

Samarbejdspartnere og efterforskere

Det er her, du vil finde personer og organisationer, der er involveret i denne undersøgelse.

Sponsor

St. Jude Children's Research Hospital

Efterforskere

Ledende efterforsker: Andrea Gropman, M.D., St. Jude Children's Research Hospital

Publikationer og nyttige links

Den person, der er ansvarlig for at indtaste oplysninger om undersøgelsen, leverer frivilligt disse publikationer. Disse kan handle om alt relateret til undersøgelsen.

Hjælpsomme links

Datoer for undersøgelser

Disse datoer sporer fremskridtene for indsendelser af undersøgelsesrekord og resumeresultater til ClinicalTrials.gov. Studieregistreringer og rapporterede resultater gennemgås af National Library of Medicine (NLM) for at sikre, at de opfylder specifikke kvalitetskontrolstandarder, før de offentliggøres på den offentlige hjemmeside.

Studer store datoer

Studiestart (Faktiske)

20. juli 2026

Primær færdiggørelse (Anslået)

1. august 2031

Studieafslutning (Anslået)

1. august 2032

Datoer for studieregistrering

Først indsendt

8. juli 2026

Først indsendt, der opfyldte QC-kriterier