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

29. Juli 2026 aktualisiert von: 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.

Studienübersicht

Status

Rekrutierung

Bedingungen

Störung des Harnstoffzyklus

Detaillierte Beschreibung

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.

Studientyp

Beobachtungs

Einschreibung (Geschätzt)

Kontakte und Standorte

Dieser Abschnitt enthält die Kontaktdaten derjenigen, die die Studie durchführen, und Informationen darüber, wo diese Studie durchgeführt wird.

Studienkontakt

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

Studienorte

Vereinigte Staaten
- Tennessee
  - Memphis, Tennessee, Vereinigte Staaten, 38105
    - Rekrutierung
    - St. Jude Children's Research Hospital
    - Kontakt:
      
      Jean Laboe, RN
      
      Telefonnummer: 901-595-1693
      
      E-Mail: jean.laboe@stjude.org

Teilnahmekriterien

Forscher suchen nach Personen, die einer bestimmten Beschreibung entsprechen, die als Auswahlkriterien bezeichnet werden. Einige Beispiele für diese Kriterien sind der allgemeine Gesundheitszustand einer Person oder frühere Behandlungen.

Zulassungskriterien

Studienberechtigtes Alter

Kind
Erwachsene

Akzeptiert gesunde Freiwillige

Probenahmeverfahren

Nicht-Wahrscheinlichkeitsprobe

Studienpopulation

Individuals who meet the Eligibility Criteria and agree to participate.

Beschreibung

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

Studienplan

Dieser Abschnitt enthält Einzelheiten zum Studienplan, einschließlich des Studiendesigns und der Messung der Studieninhalte.

Wie ist die Studie aufgebaut?

Designdetails

Anzahl der Gruppen / Kohorten

Kohorten und Interventionen

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

Was misst die Studie?

Primäre Ergebnismessungen

Ergebnis Maßnahme	Maßnahmenbeschreibung	Zeitfenster
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 Zeitfenster: 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

Sekundäre Ergebnismessungen

Ergebnis Maßnahme	Maßnahmenbeschreibung	Zeitfenster
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 Zeitfenster: 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

Mitarbeiter und Ermittler

Hier finden Sie Personen und Organisationen, die an dieser Studie beteiligt sind.

Sponsor

St. Jude Children's Research Hospital

Ermittler

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

Publikationen und hilfreiche Links

Die Bereitstellung dieser Publikationen erfolgt freiwillig durch die für die Eingabe von Informationen über die Studie verantwortliche Person. Diese können sich auf alles beziehen, was mit dem Studium zu tun hat.

Nützliche Links

Studienaufzeichnungsdaten

Diese Daten verfolgen den Fortschritt der Übermittlung von Studienaufzeichnungen und zusammenfassenden Ergebnissen an ClinicalTrials.gov. Studienaufzeichnungen und gemeldete Ergebnisse werden von der National Library of Medicine (NLM) überprüft, um sicherzustellen, dass sie bestimmten Qualitätskontrollstandards entsprechen, bevor sie auf der öffentlichen Website veröffentlicht werden.

Haupttermine studieren

Studienbeginn (Tatsächlich)

20. Juli 2026

Primärer Abschluss (Geschätzt)

1. August 2031

Studienabschluss (Geschätzt)

1. August 2032

Studienanmeldedaten

Zuerst eingereicht

8. Juli 2026

Zuerst eingereicht, das die QC-Kriterien erfüllt hat