Clinical and Biological Characterization of Patients and Collection of Samples

NCT ID: NCT04499040

Last Updated: 2020-08-05

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

100 participants

Study Classification

INTERVENTIONAL

Study Start Date

2020-09-01

Study Completion Date

2023-12-31

Brief Summary

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Objectives: Collect clinical and biological data about patients with SD/THE, collect samples of patients; create a secure on line database to collect worldwide data about SD/THE Partners : APHM, HCL, APHP

Currently10 patients (8 with TTC37 mutations and 2 with SKIV2l mutations) present a SD/THE and are managed in France in 5 different centers (Marseille, Paris Trousseau, Paris Necker, Paris Robert Debrés, and Lyon). Most of them are followed in hepato-gastro-enterology units for their intractable diarrhea. Three aspects of the disease: intractable diarrhea, immune defect and liver disease are responsible for the main part of the burden of the disease .For each aspect, the investigators will propose a close follow-up with collection of clinical, biochemical, functional and microbial data.

Collect of clinical date: during a programmed consultation clinical data about symptom will be collected twice a year. A detailed form will be used for better delineation of the symptoms. These data included growth, symptom (diarrhea, pain …), and clinical signs. Most of these children have recurrent sample for follow up. During them some blood will be take for study the immune side but also the platelet function.

Detailed Description

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Conditions

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Tricho Hepato Enteric Syndrome

Study Design

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Allocation Method

NON_RANDOMIZED

Intervention Model

PARALLEL

Primary Study Purpose

DIAGNOSTIC

Blinding Strategy

NONE

Study Groups

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patients with SD/THE

Group Type EXPERIMENTAL

Blood sample

Intervention Type BIOLOGICAL

Blood samples to assess biological parameters such as:

Fecal samples

Intervention Type OTHER

Microbiota analysis

Neuropsychological

Intervention Type BEHAVIORAL

Neuropsychological assessment

control group

Group Type PLACEBO_COMPARATOR

Blood sample

Intervention Type BIOLOGICAL

Blood samples to assess biological parameters such as:

Fecal samples

Intervention Type OTHER

Microbiota analysis

Interventions

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Blood sample

Blood samples to assess biological parameters such as:

Intervention Type BIOLOGICAL

Fecal samples

Microbiota analysis

Intervention Type OTHER

Neuropsychological

Neuropsychological assessment

Intervention Type BEHAVIORAL

Eligibility Criteria

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Inclusion Criteria

* patient \< 18 year-old
* patients with SD/THE

control group :

* patients with tufting enteropathy and short bowel syndrome.

Exclusion Criteria

* child among whom the parents or the legal representatives refused that their child participates in this study
Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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Assistance Publique Hopitaux De Marseille

OTHER

Sponsor Role lead

Responsible Party

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Responsibility Role SPONSOR

Principal Investigators

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Jean-Olivier Arnaud

Role: STUDY_DIRECTOR

Assistance Publique Hopitaux De Marseille

Locations

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Assistance Publique Hopitaux de Marseille

Marseille, , France

Site Status

Countries

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France

Central Contacts

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Alexandre FABRE

Role: CONTACT

491382747 ext. +33

DRS AP-HM, Sponsor

Role: CONTACT

491382747 ext. +33

Facility Contacts

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ALEXANDRE FABRE

Role: primary

Other Identifiers

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2016-42

Identifier Type: -

Identifier Source: org_study_id

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