Impact of Glibenclamide on Neurodevelopment in iDEND Syndrome

This observational study is looking at how the diabetes medication glibenclamide (also known as glyburide) affects brain development in people with iDEND syndrome. iDEND syndrome is a condition involving developmental delay, epilepsy, and diabetes that starts in infancy, caused by a specific change (V59M mutation) in the KCNJ11 gene. The study wants to see if starting glibenclamide treatment in the first year of life leads to better neurodevelopmental outcomes compared to starting it later. You might be able to join if you are between 2 and 50 years old, have the V59M mutation in the KCNJ11 gene, and are successfully taking glibenclamide by mouth. The study will measure the number and types of neurodevelopmental and psychiatric problems, how difficult these problems are, and their impact on daily life up to two years after you join.

Study design
This is an observational study, meaning researchers will watch and collect information without giving new treatments. It plans to include 21 participants.
What's involved
Participants will undergo a neurodevelopmental assessment, which includes parents and teachers filling out questionnaires. Where possible, you may also have face-to-face neuropsychological testing.
Compensation
Not stated in the trial record.
Follow-up
Your neurodevelopmental outcomes will be measured at or up to two years after you join the study.

AI-generated from the public study record. Only the study team can confirm whether you're eligible — confirm details with them before making decisions.

NCT05751525

Impact of Sulphonylureas on Neurodevelopmental Outcomes in KCNJ11-related Intermediate Developmental Delay, Epilepsy and Neonatal Diabetes (iDEND) Syndrome

UNKNOWN
Not specifiedAges 2–50Observational
Royal Devon and Exeter NHS Foundation Trust
~21 participants
Updated 2024-06-13 on ClinicalTrials.gov
What's tested:Sulfonylurea

At a glance

Recruiting sites
4 of 4 listed sites are recruiting right now
RecruitingSuspended, closed, or not yet open
What they're measuring
Number and type of neurodevelopmental and psychiatric disorders
Measured over At or up to 2 years after recruitment
+3 more outcomes measured
Neurodevelopmental Disorders
Intellectual Disability
Development Delay
ADHD
Autism Spectrum Disorder
Epilepsy
4 sites across 4 states
Illinois1
Italy1
Norway1
United Kingdom1
  • Prof Andrew Hattersley · PRINCIPAL_INVESTIGATOR · University of Exeter / Royal Devon University Healthcare Trust
Dr Pamela Bowman, MBBS MSc PhD
Email the study team

Opens a ready-to-send draft in your own email app — review before sending.

Do you actually qualify for this trial?

Add a private profile and we'll compare every criterion below against your situation — and tell you which ones are met, uncertain, or excluding.

Check eligibility for this trial ~2 min · HIPAA-protected · delete anytime
Eligibility criteria

Inclusion

Current age ≥2 years
Heterozygous for a V59M mutation in the KCNJ11 gene
Successfully transferred to oral sulphonylurea therapy
Willing to participate

Exclusion

Never able to transfer to oral sulphonylurea therapy
Unwilling to participate
  • Number and type of neurodevelopmental and psychiatric disordersAt or up to 2 years after recruitment

    Measured by Development and Wellbeing Assessment (DAWBA)

  • Level of difficulty due to neurodevelopmental and psychiatric morbidityAt or up to 2 years after recruitment

    Measured by Strengths and Difficulties Questionnaire (SDQ) total difficulties score. Scores range from 0-40 with higher scores indicating greater difficulties. Scores will be converted to Z-scores using normative population data for the purposes of analysis.

  • Impact on daily lifeAt or up to 2 years after recruitment

    Measured by SDQ impact score. Scores range from 0-10 for parent and self-report and 0-6 for teacher report, with higher scores indicating greater difficulties. Scores will be converted to Z-scores using normative population data for the purposes of analysis.

  • IQ scoreUp to 3 years after recruitment

    Measured by Leiter-3 International Performance Scale