Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
| Name | Class |
|---|---|
| University College London Hospitals | OTHER |
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Ehlers-Danlos Syndrome (EDS) is an inherited disease of collagen, found in connective tissues, such as skin. EDS patients suffer from joint and skin problems (skin hyperextensibility, joint hypermobility) along with a large range of other disorders, including, delayed wound healing with atrophic scarring, easy bruising, tissue fragility, gastrointestinal and gum problems. There are many different types of EDS, with different mechanisms of action, and not all of these are well understood. This study will used advanced microscopy techniques called atomic force microscopy (AFM) and scanning electron microscopy (SEM) to analyse the changes in collagen as a result of EDS, compared to normal collagen. These changes will be viewed at the micron and nanoscale level (between 1,000 to 100,000 x magnification), and will focus on the differences in collagen construction through a process called cross-linking. These changes could potentially help clinicians understand the root cause of EDS symptoms, and provide a deeper knowledge of cross-linking disorders in collagen. Increasing our knowledge of how collagen is affected in EDS patients, may lead to improved treatment options for patients.
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Skin | Patients with EDS diagnosis having surgery, have debrided skin retained for this research |
| |
| Tendon | Patients with EDS diagnosis having surgery, have debrided tendon retained for this research |
| |
| Uterine tissue | Patients with EDS diagnosis having surgery, have debrided uterine tissue retained for this research |
| |
| Vaginal tissue | Patients with EDS diagnosis having surgery, have debrided vaginal tissues retained for this research |
| |
| Ligaments | Patients with EDS diagnosis having surgery, have debrided ligaments retained for this research |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Orthopaedic & Gynaecology surgery | Procedure | Patients will have the surgery they require for their treatment. During surgery, debrided tissues will be retained for research. No treatment plans will be altered for this research. |
| Measure | Description | Time Frame |
|---|---|---|
| Histological changes in EDS compared with healthy collagen using light microscopy after staining | Light microscopy will be qualitatively used to observe colour changes after staining between healthy and EDS collagen | 1-5 years |
| Collagen morphological changes in EDS compared with healthy collagen using AFM and SEM | AFM and SEM will be used to qualitatively observe changes in orientation in collagen. | 1-5 years |
| Collagen topographical changes in EDS compared with healthy collagen using AFM and SEM | AFM and SEM will be used to observe changes in length, width and height of healthy and EDS collagen, as well as D-band length. This will be measured in meters (nm). | 1-5 years |
| Measure | Description | Time Frame |
|---|---|---|
| Collagen Young's modulus changes in EDS compared with healthy collagen using AFM | AFM will be used to calculate the Young's (elastic) modulus of the EDS and healthy collagen. This will be measured in Pascals (GPa). | 1-5 years |
| Collagen nanoscale adhesion changes in EDS compared with healthy collagen using AFM |
Not provided
Inclusion Criteria:
Exclusion Criteria:
Not provided
Not provided
Not provided
Eligible patients will be identified from screening of all new and existing Hypermobility, Orthopaedics and Gynaecology clinics at UCLH and invited to participate in the study. The appropriate information sheet will be given to the patients who have been identified for elective surgery as part of their treatment plan and meeting the inclusion.
Not provided
| Name | Affiliation | Role |
|---|---|---|
| Hanna Kazkaz | UCLH | Principal Investigator |
| Laurent Bozec | UCL | Principal Investigator |
| Adam Strange | UCL | Principal Investigator |
| Rodney Graham | UCL | Principal Investigator |
| Susan Parekh | UCLH | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| University Collage Hospital | London | NW1 2BU | United Kingdom |
No data will be shared outside of this research
Not provided
Not provided
Not provided
Not provided
Not provided
| ID | Term |
|---|---|
| D004535 | Ehlers-Danlos Syndrome |
| ID | Term |
|---|---|
| D020141 | Hemostatic Disorders |
| D014652 | Vascular Diseases |
| D002318 | Cardiovascular Diseases |
| D006474 | Hemorrhagic Disorders |
Not provided
Not provided
| ID | Term |
|---|---|
| D019637 | Orthopedic Procedures |
| ID | Term |
|---|---|
| D013812 | Therapeutics |
| D013514 | Surgical Procedures, Operative |
Not provided
Not provided
Not provided
Not provided
Not provided
Collagen in skin, tendon, ligament, vaginal tissue, uterine tissue.
AFM will be used to calculate the changes in adhesion force of the EDS and healthy collagen. This will be measured in Newtons (nN). Quantitative outcomes: changes in Young's (elastic) modulus, changes in adhesion force, changes in single molecule pulling force |
| 1-5 years |
| Collagen nanoscale single molecule pulling force in EDS compared with healthy collagen using AFM | AFM will be used to calculate the changes in pulling force of single molecules of EDS and healthy collagen. This will be measured in Newtons (nN). | 1-5 years |
| D006402 |
| Hematologic Diseases |
| D006425 | Hemic and Lymphatic Diseases |
| D012868 | Skin Abnormalities |
| D000013 | Congenital Abnormalities |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
| D012873 | Skin Diseases, Genetic |
| D030342 | Genetic Diseases, Inborn |
| D003095 | Collagen Diseases |
| D003240 | Connective Tissue Diseases |
| D017437 | Skin and Connective Tissue Diseases |
| D012871 | Skin Diseases |