Status:

UNKNOWN

Evaluation of the Effects of KCNQ1 Mutation on Insulin Tolerance and Obsessive Compulsive Features in Long QT Romano-Ward Syndrome Patients.

Lead Sponsor:

Biotrial

Collaborating Sponsors:

European Union

Nantes University Hospital

Conditions:

Romano-Ward Syndrome

Long QT Syndrome

Eligibility:

All Genders

18-48 years

Phase:

NA

Brief Summary

The objectives of the study are to investigate if KCNQ1 mutation in Romano-Ward long QT patients can be associated with changes in insulin regulation and with psychological features of compulsivity, i...

Detailed Description

Romano-Ward Syndrome (RWS) is a rare disorder characterized by prolongation of the QT interval, as well as T-wave abnormalities and possibly polymorphic ventricular fibrillation. RWS is inherited in a...

Eligibility Criteria

Inclusion

  • In the investigator's opinion, the subject is generally healthy based on their medical records (subjects with KCNQ1 mutation only), medical history, physical examination, vital signs, body weight, ECG (except long QT if applicable), and based on the results of haematology, clinical chemistry, urinalysis, urine drug screen (UDS) and serology;
  • Subjects with a KCNQ1 mutation: genotyped as having a mutation on the KCNQ1 gene with or without phenotypic manifestation of long QT syndrome;
  • Relatives of subjects with a KCNQ1 mutation: KCNQ1-mutated family relatives (with or without phenotypic expression) of a subject carrying a KCNQ1 mutation (Romano-Ward patients or subjects without phenotypic manifestation of long QT syndrome);
  • Relatives of subjects with a KCNQ1 mutation must live in a different household than the subject with the KCNQ1 mutation;
  • All subjects: negative UDS by dipstick analysis: opiates, methadone, cocaine, amphetamines (including ecstasy), barbiturates, benzodiazepines, and cannabinoids at admission to the assessment visit;
  • All subjects: negative alcohol breath test at admission to the assessment visit.

Exclusion

  • All subjects: having taken within 1 year before the assessment visit or currently taking any of the following medications: a. Antidiabetics: metformin, pioglitazone, acarbose, miglitol, sitagliptin, vildagliptin, saxagliptin, exenatide, liraglutide, semaglutide, repaglinide, nateglinide, insulin. b. Medications interfering with the central nervous system (CNS) such as any antipsychotic, antidepressant or regular use of anxiolytic medications \> once a week, or any attention deficit/hyperactivity disorder (ADHD) medication (e.g. methylphenidate);
  • Healthy subjects and relatives of subjects with a KCNQ1 mutation not phenotypically affected: any of the following on a de novo ECG: a. Heart rate (HR) \< 40 bpm or \> 100 bpm; b. PR interval \<120 msec; c. Abnormal repolarization; d. QT interval corrected for HR using Fridericia's formula (QTcF) \> 450 msec for male subjects or \> 470 msec for female subjects.

Key Trial Info

Start Date :

January 8 2021

Trial Type :

INTERVENTIONAL

Allocation :

ESTIMATED

End Date :

December 1 2021

Estimated Enrollment :

100 Patients enrolled

Trial Details

Trial ID

NCT04715256

Start Date

January 8 2021

End Date

December 1 2021

Last Update

January 20 2021

Active Locations (2)

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Page 1 of 1 (2 locations)

1

L'Institut du Thorax, Nantes Hospital

Nantes, France, 44000

2

Biotrial Clinical Unit

Rennes, France, 35000