Dysautonomic Phenotype in Male Patients With MECP2 Mutation

NCT ID: NCT04502199

Last Updated: 2020-08-06

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

Total Enrollment

20 participants

Study Classification

OBSERVATIONAL

Study Start Date

2020-08-15

Study Completion Date

2021-01-13

Brief Summary

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Dysautonomic signs are well known among girls with a Rett Syndrom. Rett syndrom is caused by a MECP2 mutation in 95% of cases. We want to search dysautonomic signs among boys with a MECP2 mutations because they are less studied than the girls and they have more varied phenotypes.

Detailed Description

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First, we will launch a national call for participation in all the genetics departments in France to research boys with a missense or a non-sens mutation in the MECP2 gene. Our inclusion criteria is : less than 16 years old or dead before 16 years old patients of masculin sex with a missense or non-sens mutation in the MECP2 gene. Exclusion criteria is : patient under judicial protection or participation refusal by one of the legal guardian. Inclusion will take place in August 2020.

We had conceived an excel spreadsheet (2007 version) to collect all the datas needed with general data such as age, genetic mutation with the HGVS nomenclature using the NM\_001110792.2, familial and personal history, mensurations, dysmorphic signs. To search dysautonomic signs, we used a system approach. We will look for neurological signs : abnormal movements, sleep disturbance, cardiologic signs :abnormal bradycardia or tachycardia, hypotension, rythms anomalies, respiratory signs : respiratory irregularities, apnea, hyperpnea , digestive signs : gastrooesophagal reflux, constipation, diarrhea, vomiting or subocclusif episodes, thermoregulation signs : abnormal hypo or hyperthermia, excessive sweating. Each time a dysautonomic sign will be reported in a letter of a patient, we will demand this sign to be objectived by a complementary exam, or by a precise device during an hospitalisation : electoencephalogram (EEG), polysomnography (PSG), Electrocardiogram (ECG), holter-ECG, digestive fibroscopies, scoped surveillance.

If we have a positive response, we will contact the main caregiver of the patient who can be a neurologist or a geneticist and will provid him a spreadsheet and an explanatory sheet. We will ask of him a systematic review of all the letters of the patient including letters of hospitalisation, of the specialists, complementary exams. A non-opposition letter will be sent to him in a way he can send it to the legal guardians of the children : a non-opposition of 15 days will be requested. Then, the filled spreadsheet will be sent back to the center of inclusion in Brest through a securised mailbox. Data will be kept on a secure database with a password.

Our primary endpoint is the presence of at least one dysautonomic sign. We intend to include between 10 and 20 patients in this multicentric study. To analyse our results, we will use a descriptive approach.

This protocole was validated by the Ethic Comitee of Brest in june 18, 2020

Conditions

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MECP2-Related Severe Neonatal Encephalopathy Dysautonomia Masculinity

Study Design

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Observational Model Type

COHORT

Study Time Perspective

RETROSPECTIVE

Interventions

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non interventional study

observational study

Intervention Type OTHER

Eligibility Criteria

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Exclusion Criteria

\-
Maximum Eligible Age

16 Years

Eligible Sex

MALE

Accepts Healthy Volunteers

No

Sponsors

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University Hospital, Brest

OTHER

Sponsor Role lead

Responsible Party

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Responsibility Role SPONSOR

Locations

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CHU de Besançon

Besançon, , France

Site Status

CHRU de Brest

Brest, , France

Site Status

CHRU de Lille

Lille, , France

Site Status

Hospices Civiles de Lyon

Lyon, , France

Site Status

Hôpital de la Timone

Marseille, , France

Site Status

CHU de NIMES

Nîmes, , France

Site Status

Hôpital Robert Debre

Paris, , France

Site Status

Countries

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France

Central Contacts

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Juliette ROPARS

Role: CONTACT

0298223657

Facility Contacts

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DELPLANCQ DELPLANCQ

Role: primary

Juliette ROPARS

Role: primary

PETIT PETIT

Role: primary

Gaetan LESCA,

Role: primary

Mathieu MILH

Role: primary

Marie FARMER,

Role: primary

Catherine SARRET

Role: primary

Other Identifiers

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MECP2BOYS (29BRC20.0182)

Identifier Type: -

Identifier Source: org_study_id

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