Quantification of Elastin Markers Synthesis in Williams-Beuren Syndrome and 7q11.23 Micro-duplication Syndrome

NCT ID: NCT04051086

Last Updated: 2019-08-12

Study Results

Results pending

The study team has not published outcome measurements, participant flow, or safety data for this trial yet. Check back later for updates.

Basic Information

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Recruitment Status

UNKNOWN

Clinical Phase

NA

Total Enrollment

90 participants

Study Classification

INTERVENTIONAL

Study Start Date

2019-10-31

Study Completion Date

2021-10-31

Brief Summary

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Introduction: Williams-Beuren syndrome is a rare genetic disorder caused by a 7q11.23 microdeletion. The phenotype associates vasculopathy (arterial stenosis, hypertension), dimorphism and intellectual disability. Microdeletion includes several genes: ELN encodes for elastin and the haplo-insufficiency (only 1 functional copy) causes vasculopathy.

The primary objective is to quantify plasma and urinary levels of elastin peptides in Williams-Beuren patients and 7q11.23 micro-duplication syndrome patients in order to correlate the levels of these markers with the number of copies of ELN gene (proportional positive relationship "gene copy number - circulating levels of markers) Materials and Methods: This prospective study will be carried out in Lyon at the "Hôpital Femme-Mère-Enfant" for 2 years. 3 groups of patients will be studied: Williams-Beuren patients (N=20), micro-duplication 7q11.23 syndrome patients (N=10) and healthy patients (N=60). Subjects will be followed for 1 day.

Clinical examination (weight, height, blood pressure) and biological sample collection (blood and urine sample) will be carry out for Williams Beuren and micro-duplication 7q11.23 patients group. A large majority of visits will be part of patients' usual care. A large part of patients are systematically seen in consultation once a year. For healthy group, only biological sample collection will be carry out. The PE concentrations will be assessed and compared between the three groups of patients.

Detailed Description

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Conditions

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Williams-Beuren Syndrome Micro-duplication 7q11.23 Syndrome Vasculopathy

Study Design

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Allocation Method

NON_RANDOMIZED

Intervention Model

PARALLEL

Primary Study Purpose

OTHER

Blinding Strategy

NONE

Study Groups

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Williams Beuren

Subjects aged from 3 months to 60 years with a diagnosis confirmed with FISH of Williams Beuren syndrome.

Group Type OTHER

Physical examination and Urine and blood samples

Intervention Type BIOLOGICAL

Only one visit for each participant : A large majority of visits will be part of patients' usual care

* Medical examination : birth, weight, gender, blood pressure, medical history
* Urine and blood samples

Micro-duplication 7q11.23

Subjects aged from 3 months to 60 years with a diagnosis confirmed with CGHarray of micro-duplication 7q11.23 syndrome.

Group Type OTHER

Physical examination and Urine and blood samples

Intervention Type BIOLOGICAL

Only one visit for each participant : A large majority of visits will be part of patients' usual care

* Medical examination : birth, weight, gender, blood pressure, medical history
* Urine and blood samples

Healthy Group

Subjects without cardiovascular and neurological medical history.

Group Type OTHER

Urine and blood samples

Intervention Type BIOLOGICAL

Only one visit for each participant

* Medical history
* Urine and blood samples

Interventions

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Physical examination and Urine and blood samples

Only one visit for each participant : A large majority of visits will be part of patients' usual care

* Medical examination : birth, weight, gender, blood pressure, medical history
* Urine and blood samples

Intervention Type BIOLOGICAL

Urine and blood samples

Only one visit for each participant

* Medical history
* Urine and blood samples

Intervention Type BIOLOGICAL

Eligibility Criteria

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Inclusion Criteria

* Age : from 3 months to 60 years old
* Williams Beuren group : Diagnosis confirmed with FISH
* Micro-duplication 7q11.23 group : Diagnosis confirmed with CGHarray
* Healthy Group : no cardiovascular and neurological medical history
* Informed consent

Exclusion Criteria

* No social insurance
* Subject under judicial protection
* Subject participating in another research including an exclusion period still in progress
Minimum Eligible Age

3 Months

Maximum Eligible Age

60 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

Yes

Sponsors

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Hospices Civils de Lyon

OTHER

Sponsor Role lead

Responsible Party

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Responsibility Role SPONSOR

Locations

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Hôpital Femme Mère Enfant - Hospices Civils de Lyon

Bron, , France

Site Status

Countries

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France

Central Contacts

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Massimiliano ROSSI, Dr

Role: CONTACT

04 27 85 55 72 ext. +33

Linda PONS, Dr

Role: CONTACT

04.27.85.51.43 ext. +33

Facility Contacts

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Massimiliano ROSSI, Dr

Role: primary

04 27 85 55 72 ext. +33

Linda PONS, Dr

Role: backup

04.27.85.51.43 ext. +33

Other Identifiers

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69HCL18_0368

Identifier Type: -

Identifier Source: org_study_id

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