Evaluation of the Effects of Cross Linking on the Biomechanical Characteristics of Skin Samples From Patients With Classic or Hypermobile Ehlers-Danlos Syndrome

Not Applicable

Not yet recruiting

• Conditions: Ehlers-Danlos Syndrome
• Interventions: Procedure: skin biopsy

• Registration Number: NCT06336473
• Lead Sponsor: GCS Ramsay Santé pour l'Enseignement et la Recherche

Brief Summary

The main aim of this study is to evaluate the effect of cross linking on the elasticity of skin samples from patients with non-vascular Ehlers-Danlos Syndrome (hEDS or cEDS).

Detailed Description

This is a single-center study based on skin biopsies. The skin biopsies will be taken at the non-vascular Ehlers-Danlos syndromes reference center at the Raymond Poincaré Hospital (Garches), by Dr. Karelle Benistan. This center regularly performs this type of biopsy as part of its routine care, and has set up a fibroblast bank for Genethon (Authorization for biological collection at Genethon: SED BACG n° DC-2021-4628).

Samples will be transported to the Centre de Recherche Saint-Antoine (UMR-S 938) where they will be processed, cut and placed on histological slides.

Atomic force microscopy analyses will be carried out at the Laboratoire de Réactivité de Surface (CNRS/Sorbonne University).

Study Timeline

• Study First Submitted: 2024-03-21
• Study First Submitted QC: 2024-03-21
• Study First Posted: 2024-03-28
• Status Verified: 2024-06
• Last Update Submitted: 2024-06-21
• Last Update Posted: 2024-06-24
• Study Start: 2024-09-03
• Primary Completion: 2025-04-01
• Study Completion: 2025-07-31

Recruitment & Eligibility

• Status: NOT_YET_RECRUITING
• Sex: All
• Target Recruitment: 28

Inclusion Criteria

• Patient aged 18 or over, having read and signed the consent form for participation in the study
• Patient with classic or hypermobile Ehlers-Danlos syndrome
• Regularly followed-up at the non-vascular Ehlers-Danlos Syndrome Reference Center at Garches Hospital.

Exclusion Criteria

• Patient under court protection, guardianship or curatorship
• Pregnant or breast-feeding patient
• Patient not affiliated to the French social security system
• Impossibility of giving the subject informed information and/or written informed consent: dementia, psychosis, disturbed consciousness, non-French-speaking patient
• Contraindication to anaesthesia or allergy to local anaesthetic product

Study & Design

• Study Type: INTERVENTIONAL
• Study Design: SINGLE_GROUP

Arm && Interventions

Group	Intervention	Description
Skin biopsy	skin biopsy	-

Primary Outcome Measures

Name	Time	Method
Young's modulus quantification	Day 0	Atomic force microscopy (AFM), in contact mode, enables force curves to be obtained. The tip no longer scans the surface of the sample, but probes the surface at several indentation points.; A microlever deflection curve is generated for each indentation point.; Calculation of Young's modulus is based on Bilodeau's model, in which the tip is assimilated to a pyramid in contact with a flat surface.; F is the measured force, E the modulus of elasticity, α the face angle of the pyramid, δ the indentation depth and ν the Poisson's ratio of the sample, set at 0.5 (corresponding to an incompressible material).; Young's modulus will be quantified by AFM

Secondary Outcome Measures

Name	Time	Method
Collagen fibril diameter measurement	Day 0	Three 2 μm x 2 μm images with at least 100 scan lines will be acquired on each sample and used to describe the intrinsic characteristics of collagen fibrils.
Collagen fibril D-period length measurement	Day 0	Three 2 μm x 2 μm images with at least 100 scan lines will be acquired on each sample and used to describe the intrinsic characteristics of collagen fibrils.

Trial Locations

Locations (1)

Clinique Maussins-Nollet; 🇫🇷 Paris, France